Radiologic Pathology: 2006-2007

Radiologic Pathology Fifth Edition VOLUME 1 Chest, Gastrointestinal, and Genitourinary Radiologic Pathology Correlation...

Author: Ellen M. Chung | Jeffrey R. Galvin | Kelly K. Koeller | Mark D. Murphey | Paula J. Woodward Angela D. Levy

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Radiologic Pathology Fifth Edition

VOLUME 1 Chest, Gastrointestinal, and Genitourinary Radiologic Pathology Correlation Editors

Angela D. Levy, COL, MC, USA Chairman and Registrar Chief, Gastrointestinal Radiology

Ellen M. Chung, LTC, MC, USA Chief, Pediatric Radiology

Jeffrey R. Galvin, MD Chief, Chest Radiology

Kelly K. Koeller, MD Chief, Neuroradiology

Mark D. Murphey, MD

Six Week Course Director Chief, Musculoskeletal Radiology

Paula J. Woodward, MD

Chief, Genitourinary Radiology

Associate Editor Jean-Claude Kurdziel, MD

2006 2007

Illustrators Aletta A. Frazier, MD Dianne D. Engelby, MAMS, RDMS Heike Blum, MFA

Department of Radiologic Pathology Armed Forces Institute of Pathology Washington DC, USA

American Registry of Pathology Armed Forces Institute of Pathology Washington, DC 20306-6000 _____________________________________

© Copyright 2006 by the American Registry of Pathology.

All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means: electronic, mechanical, photocopy, recording, or any other information storage and retrieval system without written permission of the publisher. Made in the United States of America _____________________________________

Great care has been taken to guarantee the accuracy of the information contained in this volume. However, neither the American Registry of Pathology, Armed Forces Institute of Pathology, nor the editors and contributors can be held responsible for errors or for any consequences arising from the use of the information contained herein.

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as representing the views of the Departments of the Army, Air Force, Navy, or Defense. 987654321

Library of Congress Cataloging-in-publication Data [in process] ISBN 1-933477-00-8

Preface

The Armed Forces Institute of Pathology’s Radiologic Pathologic Correlation course presented by the Department of Radiologic Pathology enters its 59th year of educating radiology residents worldwide. For the fifth year, our staff and visiting lecturers have contributed their lecture material and images to compile Radiologic Pathology 2006 – 2007, continuing the tradition of presenting richly illustrated material that teaches the pathologic basis of disease to improve our understanding of the imaging appearance of disease. We hope the efforts of our authors and editors have once again accomplished our goal of bringing the outstanding and unique Radiologic Pathologic Correlation course to your fingertips.

Acknowledgements The annual production of the Radiologic Pathologic Correlation course and syllabus is made possible through the tremendous support, dedication, and selfless service of countless individuals who work in the AFIP and the various institutions and organizations throughout the world that believe in the importance of teaching the principles of disease through radiologic pathologic correlation.

The Department of Radiologic Pathology of the Armed Forces Institute of Pathology expresses our deepest appreciation and sincerest gratitude to: - All radiologists and radiology residents who have contributed case material to the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of Pathology, - All pathologists in the AFIP who have donated their time and expertise to radiologic pathologic correlation, - All of our outstanding authors, illustrators, and department staff members who make the course and the syllabus happen effortlessly year after year, - And, to the extraordinary efforts of our production team, headed by JeanClaude Kurdziel, MD, who have tirelessly dedicated the spring and summer of the last five years to the production of this syllabus.

iii

Faculty – VOLUME 1 Chest Radiology

Marc S. Levine, MD

Jeffrey R. Galvin, MD

Professor of Radiology Hospital of the University of Pennsylvania Advisory Dean University of Pennsylvania School of Medicine Philadelphia, PA and Former Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Chief, Pulmonary and Mediastinal Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Professor of Radiology and Pulmonary Medicine University of Maryland Baltimore, MD

Gerald F. Abbott, MD

Director of Chest Radiology Rhode Island Hospital and Assistant Professor of Radiology Brown University School of Medicine Providence, RI

Deborah Rubens, MD Professor and Associate Chair Department of Imaging Sciences University of Rochester Medical Center Rochester, NY and Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Aletta A. Frazier, MD

Staff Radiologist and Medical Illustrator Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Clinical Associate Professor of Radiology University of Maryland School of Medicine Baltimore, MD

Francis J. Scholz, MD

Staff Radiologist Lahey Clinic Medical Center Burlington, MA and Clinical Professor of Radiology Tufts University School of Medicine Boston, MA

Leonard M. Glassman, MD

Washington Radiology Associates, PC Washington, DC and Clinical Professor Department of Radiology George Washington University Medical Center Washington, DC

Robert K. Zeman, MD

Chairman and Professor of Radiology George Washington University Washington, DC

Genitourinary Radiology

Melissa L. Rosado de Christenson, MD, FACR Clinical Professor of Radiology The Ohio State University Columbus, OH and Adjunct Professor of Radiology Uniformed Services University of the Health Sciences Bethesda, MD

Paula J. Woodward, MD

Acting Chief, Genitourinary Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Adjunct Professor of Radiology University of Utah School of Medicine Salt Lake City UT

Rosita M. Shah, MD

Clinical Associate Professor of Radiology Hospital of the University of Pennsylvania Philadelphia, PA

Peter L. Choyke, MD

Gastrointestinal Radiology

Chief Molecular Imaging Program National Cancer Institute Bethesda, MD and Professor of Radiology and Nuclear Medicine Uniformed University of the Health Sciences Bethesda, MD

Angela D. Levy, COL, MC, USA

Chairman and Gastrointestinal Radiology Section Chief Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Associate Professor of Radiology and Nuclear Medicine Uniformed Services University of the Health Sciences Bethesda, MD

William D. Craig, MD

Chief, Genitourinary Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Bruce P. Brown, MD

Associate Professor of Radiology University of Iowa Iowa City, IA iv

David S. Hartman, MD

Professor of Radiology Department of Radiology Pennsylvania State University M. S. Hershey Medical Center Hershey, PA

Deborah J. Rubens, MD Professor and Associate Chair Department of Imaging Sciences University of Rochester Medical Center Rochester, NY and Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC Brent J. Wagner, MD

Chairman, Department of Radiology The Reading Hospital and Medical Center West Reading Radiology Associates West Reading, PA

Jade J. Wong-You-Cheong, MD

Associate Professor of Diagnostic Radiology Director of Ultrasound University of Maryland School of Medicine Baltimore, MD

v

Table of Contents – VOLUME 1 Chest Radiology Jeffrey R. Galvin, MD

An Approach to Diffuse Lung Disease, Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3 The Idiopathic Interstitial Pneumonias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .14 Airways Disease: The Movement from Anatomic to Physiologic Assessment . . . . . . . . . . . . . . . . . . . . . . . .26 Inhalational Lung Disease (Asbestosis and Silicosis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .44 Pulmonary Lymphoid Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .54 Angiitis and Granulomatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .63 The Pulmonary Complications of Organ Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .75 The Diagnosis of Pulmonary Embolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .82 Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .93 Fungal Disease in the Thorax: Opportunistic and Primary Pathogens . . . . . . . . . . . . . . . . . . . . . . . . . . . . .100 Bronchogenic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .110 Chest Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .121 Chest Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .126

Aletta A. Frazier, MD

Pulmonary Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131 Pulmonary Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .138

Melissa L. Rosado de Christenson, MD, FACR

Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148 Chest Seminar: Where is the lesion? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .168 Chest Seminar: Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .173

Rosita M. Shah, MD

Pneumonia: Usual and Unusual Organisms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .178

Gerald F. Abbott, MD

Uncommon Malignant Tumors of the Lung . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .192 Benign Tumors of the Lung and Tumor-like Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .199 Pleural Disease I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .205 Pleural Disease II and Chest Wall . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .213

Leonard M. Glassman, MD (Mammography)

Classic Breast Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .220 Basic Breast Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .229 Ductal Carcinoma in Situ (DCIS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .238 Breast Abnormalities in Young Women . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .246 The Male Breast . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .257

Gastrointestinal Radiology Angela D. Levy, COL, MC, USA

Benign Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .267 Malignant Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .275 Hepatic Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .284 Imaging of Chronic Liver Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .293 Benign Biliary Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .303 Biliary Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .313 Pancreatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .321 Gastric Malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .332 Abdominal Non Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .344 Small Intestinal Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .353 Colorectal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .361 Mesenteric Masses and Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .372 Idiopathic Inflammatory Bowel Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .382 Approach to Inflammatory Diseases of the Colon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .391 GI Seminar 1: Abdominal Gas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .400 GI Seminar 2: Nonneoplastic Disease of the Stomach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .405 GI Seminar 3: Pancreatic Duct . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .411 GI Seminar 4: Hepatic Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .417 GI Seminar 5: Complications of Meckel Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .422 vi

GI Seminar 6: Beyond Appendicitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .427 GI Seminar 7: Tumors and Tumor-Like Lesions of the Gallbladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .432

Robert K. Zeman, MD

Cholelithiasis and Cholecystitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .438

Marc S. Levine, MD

Inflammatory Diseases of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .444 Tumors of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .450 Radiology of Peptic Ulcer Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .455

Bruce Brown, MD

Pancreatitis: Imaging Has Made a Difference . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .460 Gastrointestinal Bleeding In The Age of the Endoscope. What Does a Radiologist Have To Contribute? . .468

Francis J. Scholz, MD

Small Bowel Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .475 Acute Mesenteric Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .487 Malabsorption . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .505 Familial Polyposis and Other Such . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .519

Deborah J. Rubens, MD

The Spleen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .531 Portal Venous Doppler . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .542

Genitourinary Radiology Paula J. Woodward, MD

Imaging of Uterine Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .551 Approach to Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .561 Urinary Tract Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .573 Retroperitoneum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .579 Radiologic Evaluation of the Scrotum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .585 First Trimester Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .594 Fetal CNS Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .602 Fetal Body Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .607

Peter L. Choyke, MD

Cystic Diseases of the Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .614 Imaging of Prostate Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .620

William D. Craig, MD

Radiographic Evaluation of Urinary Stone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .624

Deborah J. Rubens, MD

Testicular Torsion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .630

Brent J. Wagner, MD

Imaging of Ovarian Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .637 Adrenal Imaging in Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .645 Imaging of the Urinary Bladder and Urethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .649

Jade Wong You Cheong, MD

Non-Neoplastic Disorders Of The Ovary And Adnexae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .653 Imaging of Solid Organ Transplants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .664

David S. Hartman, MD

The Neglected Nephrogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .674 Problem Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .681

Paula J. Woodward, MD

GU Seminar 1: MSAFP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .691 GU Seminar 2: Renal Calcifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .693

vii

Chest Radiology

An Approach to Diffuse Lung Disease, Sarcoidosis Jeffrey R. Galvin, MD

Describing Diffuse Lung Disease • •

The Alveolar vs. Interstitial Problem Alveolar or Interstitial ?

•

Radiograph ➢ Lung volumes ➢ Opacity ➢ Distribution ➢ Ancillary findings Computed tomography ➢ Opacity ➢ Distribution

An Approach to Diffuse Lung Disease

•

Figure 1-1-2

Figure 1-1-1

Radiology and pathology form a continuum of visualization Fibrosis results in reduced lung volumes

Lung Volumes •

•

Reduced [Figure 1-1-2] ➢ Pathology distal to the airway ➢ Fibrosis ➢ IPF, asbestosis, sarcoidosis, chronic hypersensitivity pneumonitis Increased [Figure 1-1-3] ➢ Pathology of the airway ➢ Emphysema, asthma, bronchitis, constrictive bronchiolitis, LAM

Figure 1-1-3

Airways disease results in increased lung volumes Chest Radiology

3

An Approach to Diffuse Lung Disease

Distribution: Upper vs Lower Plain Film and CT Opacities

Figure 1-1-4

[Figure 1-1-4]

• • • • •

Nodules Reticulation and Lines Ground glass Consolidation Cystic airspaces

•

Nodules ➢ Sarcoid ➢ Silicosis, coal-workers ➢ Hypersensitivity Pneumonitis ➢ Metastasis Reticulation and Lines ➢ Fibrosis ✧ IPF-lower, subpleural ✧ Asbestosis-lower, subpleural ✧ Sarcoidosis-peribronchovascular ✧ Chronic hypersensitivity pneumonitis-mid and upper lung zone Ground glass [Shah and Miller AJR 2003] ➢ Non-specific ✧ Airspace, interstitial, combined – DIP, NSIP, AIP, DAD (32%) ✧ Infection (32%) ✧ Drug toxicity (11%) ✧ Hemorrhage (3%) ✧ Ground glass with reticulation Chronic diseases tend to involve the upper lung – Fibrosis Consolidation ➢ Organizing Pneumonia (BOOP) ➢ Chronic eosinophilic pneumonia ➢ Lymphoma ➢ Bronchoalveolar cell carcinoma ➢ Infection ➢ Hemorrhage Cystic airspaces ➢ Mimics reticulation on plain radiographs ➢ Fibrosis and honeycombing ✧ IPF-Lower, subpleural ✧ LAM-Diffuse ✧ LCH-Upper

Plain Film and CT Opacities

•

•

•

•

Radiologic-Pathology Continuum Anatomy - Secondary Lobule •

•

As defined by Miller ➢ Polygonal ➢ 1-2.5 cm ➢ Smallest unit demarcated by connective tissue septa Most useful diagnostically ➢ Readily identified on: ✧ HRCT ✧ Gross examination ✧ Histologic section ➢ Explains HRCT appearance ✧ Broad range of lung diseases ✧ Especially interstitial disease

An Approach to Diffuse Lung Disease

4

Chest Radiology

Anatomy - Secondary Lobule [Figure 1-1-5] •

• •

Core structures ➢ Axial interstitium ➢ Bronchiole ➢ Pulmonary artery ➢ Lymphatics Septal structures ➢ Peripheral interstitium ➢ Pulmonary veins ➢ Lymphatics Parenchyma ➢ Alveolar interstitium ➢ Alveoli ➢ Pulmonary capillary bed

Figure 1-1-5

The secondary lobule with lymphatics in the interloblular septa and along the bronchovascular bundle

Figure 1-1-6

Septal pattern on HRCT and gross specimen Chest Radiology

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An Approach to Diffuse Lung Disease

Abnormal Patterns •

• • •

•

Bronchovascular ➢ Bronchus ✧ Asthma, CF, bronchitis, bronchiectasis [Figure 1-1-7] ➢ Lymphatic ✧ CA, lymphoma, sarcoidosis ✧ Edema Centrilobular [Figure 1-1-8] ➢ Airway related Panlobular [Figure 1-1-9] ➢ Nonspecific Septal ➢ Lymphatic ✧ CA, lymphoma, sarcoidosis [Figure 1-1-10and 1-1-11]

Edema Random [Figure 1-1-12] ➢ Hematogenous spread of tumor ➢ TB ✧

Sarcoidosis • • • •

• •

Figure 1-1-7

Bronchovascular pattern

Figure 1-1-9

Figure 1-1-8

Centrilobular pattern

Figure 1-1-10

Multisystem granulomatous disorder Unknown etiology Young and middle aged adults Bilateral hilar lymphadenopathy, pulmonary infiltration, eye and skin lesions Clinical and radiologic findings supported by evidence of noncaseating epithelioid granulomas Exclusion of granulomas of unknown cause and local sarcoid reactions

ATS Statement on Sarcoidosis 1999

Sarcoidosis: Epidemiology •

• •

•

Worldwide ➢ both sexes, all races, all ages Predilection for adults ➢ under 40 years ➢ peak 20-29 years U.S. prevalence ➢ 10 per 100,000 exams Highest disease ➢ African-American women

Panlobular pattern

Figure 1-1-11

Septal pattern

Figure 1-1-12

Sarcoidosis: Clinical Features •

• •

• •

•

Asymptomatic ➢ 15-50% Constitutional symptoms ➢ 33% Dyspnea, cough, chest pain ➢ 33-50% Palpable lymph nodes ➢ 33-75% Ocular involvement ➢ 11-83% Cutaneous involvement ➢ 20-30% Erythema nodosum, Lupus pernio

An Approach to Diffuse Lung Disease

Combined septal and bronchovascular pattern 6

Random nodule pattern Chest Radiology

Sarcoidosis: Laboratory Abnormalities •

• • • • •

Figure 1-1-13

BAL ➢ ↑ macrophages, ↓ proportions; ↑ CD4 helper cells Angiotensin-Converting Enzyme ➢ Nonspecific Produced by granuloma/macrophage ➢ ↑ 33-90% Hypercalcemia 10% Hypercalciuria 30% ➢ Macrophage/granuloma extrarenal sources of 1-25 Dihydroxyvitamin D Anergy Hypergammaglobulinemia

Sarcoidosis: Respiratory System [Figure 1-1-13]

• •

•

100% lung involvement Portal of entry ➢ Local lymph nodes ➢ Distant organs Disease distribution ➢ Alveolar wall ➢ Secondary lobule, ➢ Axial CT ➢ Radiograph

Non-Caseating Granuloma and Fibrosis

Sarcoidosis pathogenesis

Alveolar Distribution

Sarcoidosis and the Secondary Lobule [Figure 1-1-14]

Figure 1-1-14

Bronchovascular distribution of granulomas in Sarcoidosis

Chest Radiology

7

An Approach to Diffuse Lung Disease

Figure 1-1-15

Distribution of nodules in sarcoidosis

Masses in Sarcoidosis

Ground glass in Sarcoidosis

Conglomerate masses and fibrosis in sarcoidosis

Sarcoidosis: Computed Tomography [Figure 1-1-15] • • • •

• • •

Nodules Masses Ground Glass Fibrosis ➢ Conglomeration ➢ Distortion Emphysema Bulla Honeycombing

An Approach to Diffuse Lung Disease

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Chest Radiology

Parenchymal Disease: Radiography • • • • • • • • •

Bilateral Symmetrical Nodules Reticulonodular Masses Ground Glass Hilar Retraction Bulla Honeycombing

• • • • • • •

Node Group Hilar R. Paratracheal A-P Window Subcarinal Ant. Med. Post. Med.

Sarcoidosis: Adenopathy CXR 84 76 72 12 12 0

Figure 1-1-16

[Figures 1-1-16 and 1-1-17]

CT 88 100 92 64 48 16

Lymph node involvement is a hallmark of sarciodosis

Sarcoidosis: Staging based on Adenopathy and Parenchyma Stage 0 ➢ Normal Stage 1 ➢ Adenopathy Stage 2 ➢ Adenopathy & Parenchyma Stage 3 ➢ Parenchyma

Presentation 8

Resolution –

51

65

12

20

29

Figure 1-1-17

49

20% develop fibrosis or Stage 4 disease

Sarcoidosis Stage I

Sarcoidosis Stage II

Sarcoidosis Stage III

Sarcoidosis Stage IV

Bilateral calcified lymph nodes are common

Sarcoidosis Progression

Sarcoidosis and the Parenchyma: Computed Tomography • •

• • •

Thickened Bronchovascular Bundles Nodules ➢ Peribronchovascular ➢ Pleural, subpleural and septal Consolidation and Large Nodules Ground-Glass Opacities Fibrosis

Chest Radiology

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An Approach to Diffuse Lung Disease

Thickened Bronchovascular Bundles [Figure 1-1-18] Figure 1-1-18

Peribronchovascular opacities in sarcoidosis

Peribronchovascular Nodules

Peribronchovascular and Pleural Nodules Septal Lines

Ground Glass Opacities

Consolidation and Large Nodules Fibrosis

Bronchovascular Bundle Distortion [Figure 1-1-14] Conglomerate Mass

Fibrosis and Emphysema

Fibrosis and Honeycombing Sarcoidosis: Diagnosis • • • •

Typical clinical and radiologic manifestations Non-caseating granulomas Transbronchial Bx Endobronchial Bx

•

Infection ➢ Tuberculosis, Fungal (Histoplasmosis) Pneumoconiosis ➢ Silica, Beryllium Hypersensitivity Pneumonitis Malignancy ➢ Lymphoma

Sarcoidosis: Differential Diagnosis • • •

An Approach to Diffuse Lung Disease

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Chest Radiology

Miliary Tuberculosis

Transbronchial Spread of Tuberculosis Histoplasmosis Silicosis

Berylliosis

Extrinsic Allergic Alveolitis Sarcoidosis: Mortality • • • •

Mortality range 5-10% Cor Pulmonale related to fibrosis Cardiac Arrhythmia Pulmonary Hemorrhage ➢ Aspergilloma

Cor Pulmonale

Sarcoidosis: Cardiac Involvement •

• •

Clinical involvement 5% ➢ Heart block, arrhythmia, mitral regurgitation, CHF (dilated cardiomyopathy) and sudden death Autopsy involvement 20-30% Localized wall motion abnormalities ➢ Anterior and apical ➢ MRI, Echocardiograph, Thallium-201

Vignaux AJR 184 Jan 2005

Cardiac Sarcoidosis [Figure 1-1-19] Figure 1-1-19

Sarcoid infiltration on MRI is represented as focal zones of increased signal on T2 and early gadolinium images

Dilated Cardiomyopathy

Chest Radiology

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An Approach to Diffuse Lung Disease

Sarcoidosis: Mycetoma •

• •

Figure 1-1-20

Present in 40-50% of cystic lesions ➢ Bullae, cavities or bronchiectasis Hemorrhage Steroids may convert to invasive process

Mycetoma [Figure 1-1-20]

Sarcoidosis: Therapy • • •

• • •

Cardiac, CNS, eye involvement Hypercalcemia Corticosteroids ➢ Relief of symptoms; resolution of radiologic abnormalities; improved function Cytotoxic agents ➢ Methotrexate, Azathioprine Chlorambucil, cyclophosphamide, antimalarials Risk of recurrence

Sarcoidosis: Resolution Sarcoidosis: Prognosis •

•

Favorable ➢ Acute onset, erythema nodosum, ➢ > 80% spontaneous remission ➢ Löfgren syndrome ➢ Low stage Poor ➢ Chronic course, Lupus pernio ➢ Older age at presentation ➢ Hypercalcemia/nephrocalcinosis ➢ Black race, Extrathoracic involvement

Mycetoma in a cystic space caused by sarcoidosis

Sarcoidosis Conclusion References

General 1. Akira M, Hara H, Sakatani M. Interstitial lung disease in association with polymyositis- dermatomyositis: longterm follow-up CT evaluation in seven patients. Radiology 1999; 210(2):333-8. 2. Bergin CJ, Muller NL. CT of interstitial lung disease: a diagnostic approach. American Jounal of Roentgenology 1987; 148:8-15. 3. Bergin C, Roggli V, Coblentz C, Chiles C. The secondary pulmonary lobule:normal and abnormal CT appearances. American Journal of Roentgenology 1988; 15:21-25. 4. Epler GR, McLoud TC, Gaensler EA, Mikus JR Carrington CB. Normal chest roentgenograms in chronic diffuse infiltrative lung disease. N Engl I Med 1978:298(17):934-9. 5. Epler GR. Chest films: underused tool in interstial lung disease. Journal of Respiratory Diseases 1987; 8(6):1 4-24. 6. Felson B. A new look at pattern recognition of diffuse pulmonary disease. American Journal of Roentgenology 1979; 133:183-189. 7. Calvin JR. Mon M, Stanford W. High-resolution computed tomography and diffuse lung disease. Curr Probl Diagn Radiol 1992; 21(2):31-74. 8. Grenier P. Valeyre D, Cluze I R Brauner MW, Lenoir 5, Chastang C. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high- resolution CT. Radiology 1991; 179:123-132. 9. Gruden JF, Webb WR, Naidich DR, McGuinness G. Multinodular disease: anatomic localization at thin-section CT—multireader evaluation of a simple algorithm. Radiology 1999; 210(3):711-20. 10. Gurney JW, Schroeder BA. Upper lobe lung disease: physiologic correlates. Radiology 1988; 167:359-366. 11. Heitzman ER. The lung. Second ed. St. Louis: C.V. Mosby, 1984. 12. Johkoh T, Muller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, lchikado K, Ando M, Nakamura H. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999; 211 (2):555-60. 13. Mathieson JR. Mayo JR. Staples CA, Muller NL. Chronic diffuse infiltrative lung disease: comparison of dianostic accuracy of CT and chest radiography. Radiology 1989; 171:111-116. An Approach to Diffuse Lung Disease

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14. Mayo JR. Webb WR, Gould R, Stein MG, Bass I, Gamsu G, Goldberg H. High- resolution CT of the lungs: an optimal approach. Radiology 1987; 163:507-510. 15. McLoud TC, Carrington CB, Gaensler EA. Diffuse Infiltrative lung disease: a new scheme for description. Radiology 1983; 149(2):353-363. 16. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part 2. Am Rev Respir Dis 1990; 142(6 Pt 1 ):1440-8. 17. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part lAm Rev Respir Dis 1990; 142(5):1206-15. 18. Muller NE, Coiby TV. Idiopathic interstitial pneumonias: high-resolution CT and histologic findings. Radiographics 1997; 17(4): 1016-22. 19. Murata K, Itoh H, Todo G, Kanaoka M, Noma 5, Itoh T, Furuta M, Asamoto H, Torizuka K. Centrilobular lesions of the lung: demonstration by high-resolution CT and pathologic correlation. Radiology 1986; 161 :641-645. 20. Murata K, Khan A, Rojas KA, Herman PG. Optimization of computed tomography technique to demonstrate the fine structure of the lung. Investigative Radiology 1988; 23:170-175. 21. Murata K, Khan A, Herman R Pulmonary parenchymal disease: evaluation with high-resolution CT. Radiology 1989; 170:629-635. 22. Muller NI, Miller RR. Computed tomography of chronic diffuse lung disease. American Review of Respiratory Disease 1990; 142:1206-1215, 1440-1448. 23. Staples CA, Muller NE, Vedal S, Abboud R, Ostrow D, Miller RR. Usual interstitial Pneumonia: correlation of CT with clinical, functional, and radiologic findings. Radiology 1987; 162:377-381. 24. Webb WR. High resolution CT of lung parenchyma. Radiologic Clinics of North America 1989; 27(6):1085-1097. 25. Weibel ER. Looking into the lung: what can it tell us? American Journal of Roentgenology 1979; 133:1021-1031. 26. Weibel ER, Bachofen H. The Fiber Scaffold of Lung Parenchyma. In: Crystal RG, West JB, eds. The Lung. New York: Raven Press, 1991; 787-794. 27. Weibel ER, Crystal RG. Structural Organization of the Pulmonary Interstitium. In: Crystal RG, West JB, eds. The Lung. New York: Raven Press, 1991; 369-380. Sarcoidosis 1. Bergin CJ, Bell DY, Coblentz CL, Chiles C, Gamsu C, Maclntyre NR, Coleman RE, Putman CE. Sarcoidosis: correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests. Radiology 1989; 171(3):619-24. 2. Gawne-Cain ML, Hansell CM. The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis and tuberculosis: a CT study. Clin Radiol 1996; 51(4):263-7. 3. Gleeson FV, Traill ZC, Hansell CM. Evidence of expiratory CT scans of small- airway obstruction in sarcoidosis. AJRAm J Roentgenol 1996; 166(5):1052-4. 4. Hansell DM, Milne DC, Wilsher ME, Wells AU. Pulmonary sarcoidosis:morphologic associations of airflow obstruction at thin-section CT. Radiology 1998; 209(3):697-704. 5. Kuhlman JE, Fishman EK, Hamper UM, Knowles M, Siegelman SS. The computed tomographic spectrum of thoracic sarcoidosis. Radiographics 1989; 9(3):449-66. 6. Miller WT Jr, Shah RM. Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations. AJR Am J Roentgenol. 2005 Feb;184(2):613-22. 7. Muller NE, Kullnig P, Miller RR. The CT findings of pulmonary sarcoidosis: analysis of 25 patients. AJR Am J Roentgenol 1989; 152(6):1179-82. 8. Muller NE, Mawson JB, Mathieson JR. Abboud R, Ostrow DN, Champion P Sarcoidosis: correlation of extent of disease at CT with clinical, functional, and radiographic findings. Radiology 1989; 171 (3):61 3-8. 9. Murdoch J, Muller NE. Pulmonary sarcoidosis: changes on follow-up CT examination. AJR Am J Roentgenol 1992; 159(3):473-7. 10. Newman ES, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997 Jul 10;337(2):1391 [see comments]. N Engl J Med 1997; 336(17):1224-34. 11. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. Pulmonary sarcoidosis: correlation of CT and histopathologic findings [published erratum appears in Radiology 1994 Mar;190(3):907]. Radiology 1993; 189(1):105-9. 12. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. CT and pathological correlation of pulmonary sarcoidosis. Semin Ultrasound CT MR 1995; 16(5):361-70. 13. Padley SP, Padhani AR, Nicholson A, Hansell DM. Pulmonary sarcoidosis mimicking cryptogenic fibrosing alveolitis on CT. Clin Radio! 1996; 51(11):807-10. 14. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol 1985; 144(3):51328. 15. Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis 1987; 135(3):747-60. 16. Vignaux O. Pictorial Essay: Cardiac sarcoidosis: spectrum of MRI features. AJR Am J Roentgenol 2005 Jan;184(1):249-54. 17. Winterbauer RH, Belic N, Moores KD. Clinical interpretation of bilateral hilar adenopathy. Ann Intern Med 1973; 78(1 ):65-71. Chest Radiology

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An Approach to Diffuse Lung Disease

The Idiopathic Interstitial Pneumonias Jeffrey R. Galvin, MD

The Idiopathic Interstitial Pneumonias Chronic Diffuse Lung Disease [Figure 1-2-1 and 1-2-2] •

• • • •

Alveolar involvement ➢ Surrounding airways ➢ Fibrosis and/or cells ✧ Alveolar wall ✧ Alveolar space Restrictive physiology Decreased lung volumes Increased attenuation Subacute or chronic ➢ Weeks to months

Figure 1-2-1

Figure 1-2-2

The Idiopathic Interstitial Pneumonias involve the alveolar walls and spaces

The lung volumes are low and there are areas of increased density

The Idiopathic Interstitial Pneumonias • •

• •

Liebow 1975 Supporting lung structures ➢ Inflammation ➢ Fibrosis Not confined to interstitium Initiated within the airspace

Liebow, Prog Reps Dis 1975

The Idiopathic Interstitial Pneumonias Current List-ATS/ERS Consensus Classification • • • • • •

Idiopathic Pulmonary Fibrosis (IPF) ➢ Usual Interstitial Pneumonia (UIP) Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) Desquamative Interstitial Pneumonia (DIP) Acute Interstitial Pneumonia (AIP) Cryptogenic Organizing Pneumonia (COP) NonSpecific Interstitial Pneumonia (NSIP)

Travis et al. Am J Respir Crit Care 2002 The Idiopathic Interstitial Pneumonias

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Chest Radiology

Idiopathic Pulmonary Fibrosis • •

• •

•

Usual Interstitial Pneumonia: histologic pattern 5th-7th decade ➢ 66% > 60 years ➢ 7/100,000 women and 10/100,000 men Insidious onset of dyspnea ➢ 6 months before diagnosis ➢ Restrictive ventilatory defect ➢ Rales and clubbing Associations: ➢ Cigarette smoke ➢ Dusty environments: farming, wood dust, metal dust ➢ GE reflux ➢ Autoantibodies common (ANA, RA) Median survival 2.5-3.5 years

Figure 1-2-3

Usual Interstitial Pneumonia: Histology • •

• • •

Geographic variation Temporal variation ➢ Fibroblast foci ➢ Mature fibrous tissue Extensive fibrosis Inflammation ➢ Minimal ➢ No correlation outcome Abnormal wound healing Prognosis ➢ Fibroblast foci ✧ Presence and extent

Katzenstein, Am J Respir Crit Care Med 1998 Selman, Ann Int Med 2001 King, Am J Respir Crit Care Med 2001

Idiopathic Pulmonary Fibrosis Imaging [Figure 1-2-3] •

•

Radiograph abnormal-95% ➢ Volume loss ➢ Reticulonodular opacities ➢ Lower lobe ➢ Honeycombing Computed tomography ➢ Peripheral and lower lobe ➢ Reticulation and ground glass ✧ Progress to honeycombing ➢ Ground glass in areas of traction bronchiectasis

The abnormalities are predominantly peripheral and lower lung field. There is progressive volume loss

Figure 1-2-4

Hartman, Chest 1996

IPF-Progressive Volume Loss

Idiopathic Pulmonary Fibrosis [Figure 1-2-4] IPF and Emphysema

Typical peripheral reticulation and honeycombing and traction bronchiectasis in a patient with IPF

Chest Radiology

15

The Idiopathic Interstitial Pneumonias

Utilility of Biopsy for Diagnosis of IPF •

• • •

• • •

Prospective, multi-center study ➢ 91 patients suspected of IPF Clinical diagnosis ➢ Positive predictive value with a confident diagnosis-87% Imaging diagnosis ➢ Positive predictive value with a confident diagnosis-96% ➢ CT always abnormal in patients with proven IPF Histologic diagnosis ➢ Agreement regarding the presence or absence of IPF-85% ➢ Agreement in patients without IPF-48% ✧ Relevance to NSIP Uncertain diagnosis Discordant data Disease other than IPF ➢ Hypersensitivity pneumonitis ➢ Collagen-vascular disease ➢ Infection

Figure 1-2-5

Hunninghake, Am J Respir Crit Care Med 2001

IPF Rad-Path Discord

Smoking Related ILD Interstitial Lung Disease [Figure 1-2-5] •

• •

Respiratory bronchiolitis ➢ RB Respiratory bronchiolitis-interstitial lung disease ➢ RB-ILD Desquamative interstitial lung disease ➢ DIP

Smoking Related ILD RB • • •

Clinical ➢ Cigarette smoke or equivalent ➢ Asymptomatic Pathology ➢ Peribronchiolar macrophages ➢ Peribronchiolar fibrosis Imaging ➢ Centrilobular nodules ✧ Poorly defined 2-3 mm ✧ Uper lobe predominance ➢ Ground glass opacity ➢ Bronchial wall thickening ➢ Decreased attenuation ➢ Emphysema ➢ Air trapping ➢ Reticulation

Smoker’s macrophages

Niewoehner, NEJM 1974 ; Remy-Jardin, Radiology 1993

The Idiopathic Interstitial Pneumonias

16

Chest Radiology

Figure 1-2-6

Smoking Related ILD [Figure 1-2-6] RB-ILD •

• •

Clinical ➢ Cigarette smoke or equivalent ➢ Dyspnea ➢ Restrictive or mixed PFT’s ➢ Good prognosis Pathology ➢ Peribronchiolar macrophages ➢ Peribronchiolar fibrosis Imaging ➢ Centrilobular nodules ✧ Poorly defined 2-3 mm ✧ Uper lobe predominance ➢ Ground glass opacity ➢ Bronchial wall thickening ➢ Decreased attenuation ➢ Emphysema ➢ Air trapping ➢ Reticulation

Meyers, Am Rev Respir Dis 1987 Park, J Comput Assist Tomogr 2002

Small centrilobular nodules with an upper lobe predominance in RB-ILD

Smoking Related ILD DIP •

•

•

Clinical ➢ Cigarette smoke ➢ 4th and 5th decade ➢ Uncommon ➢ 70% survival-10 years ➢ Steroids Pathology ➢ Pigmented macrophages ➢ Interstitial infiltrate ✧ Plasma cells and eosinophils ➢ Fibrosis Imaging ➢ Ground glass ✧ Symmetrical ✧ Basal predominance ➢ Reticulation ➢ Cysts ✧ Alveolar ducts ✧ Bronchioles ✧ Emphysematous spaces

Figure 1-2-7

Carrington, NEJM 1978 ; Hartman, Radiology 1993

Desquamative Interstitial Pneumonia [Figure 1-2-7

Ground glass opacities in DIP Chest Radiology

17

The Idiopathic Interstitial Pneumonias

Dependent Density

Figure 1-2-8

Desquamative Interstitial Pneumonia RB and DIP

Smoking Related ILD [Figure 1-2-8] Acute Interstitial Pneumonia AIP • • • • • •

Hammon-Rich disease Rapidly progressive Days-weeks Antecedent flu-like syndrome Mean age 50 years 50% fatal at least

Vourlekis, Medicine 2000

Acute Interstitial Pneumonia Histology •

• • •

Exudative phase ➢ Hyaline membranes ➢ Edema ➢ Inflammation Collapse of alveoli Organizing phase ➢ Type II hyperplasia ➢ Loose fibrosis Diffuse Alveolar Damage

Smoking related interstitial lung disease with upper lobe indistinct nodules, reticulation and well defined emphysematous spaces combined with lowerlobe ground glass

Figure 1-2-9

Katzenstein, Am J Pathol 1986 ; Ichikado, AJR 1997

Diffuse Alveolar Damage

Acute Interstitial Pneumonia Radiography [Figure 1-2-9 and 1-2-10] • • • • •

Diffuse Airspace opacification Costal sparing Mechanical ventilation Resembles ARDS

Figure 1-2-10

AIP involves all 5 lobes

Most patients are intubated with diffuse opacities The Idiopathic Interstitial Pneumonias

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Chest Radiology

Acute Interstitial Pneumonia Computed Tomography • Exudative phase •

Figure 1-2-11

➢ Consolidation ➢ Bilateral ➢ Focal sparing Organizing phase ➢ Distortion ➢ Traction bronchiectasis ➢ Ground glass

Johkoh, Radiology 1999; Ichikado et al. Am J Respr Crit Care Med 2002

Acute Interstitial Pneumonia [Figure 1-2-11] Cryptogenic Organizing Pneumonia • • • • •

•

Non-specific inflammatory response Pattern of repair Self-perpetuating Cryptogenic Secondary ➢ Connective tissues disease, hematologic malignancy, drugs or organ transplantation Focal ➢ Bacteria, legionella, mycoplasma, mycobacterial, or infarction

Lohr, Arch Int Med 1997

Cryptogenic Organizing Pneumonia •

• • • • • •

Focal areas of sparing are common in AIP

Terminology problem ➢ Bronchiolitis obliterans OP (BOOP), bronchiolitis obliterans (BO), bronchiolitis interstitial pneumonia (BIP) Subacute presentation (3 months) M=F Cough, dyspnea, weight loss, fever Restrictive PFT’s Steroid responsive Relapse common

Figure 1-2-12

Epler, NEJM 1985

Cryptogenic Organizing Pneumonia Histology • • •

Fibroblastic plugs in alveoli Fibrosis in the alveolar space May be airway centered ➢ Bronchiolitis

Cryptogenic Organizing Pneumonia [Figure 1-2-12] Radiography •

• •

Consolidation ➢ Unlateral or bilateral Small Nodules ➢ 10-50% Lung volumes ➢ normal in 75%

Cryptogenic organizing pneumonia is characterized by focal areas of consolidation more common in the lower lung fields Chest Radiology

19

The Idiopathic Interstitial Pneumonias

Cryptogenic Organizing Pneumonia Computed Tomography • • • • • •

Figure 1-2-13

Consolidation 90% Ground glass 75% Bronchial thickening and dilatation Small nodules along bronchvascular bundles Large nodules (15%) ➢ Irregular margins ➢ Air bronchograms Reverse halo

Cryptogenic Organizing Pneumonia [Figure 1-2-13 and 1-2-14]

Figure 1-2-14 Typical findings in COP with peripheral areas of consolidation. The differential includes chronic eosinophilic pneumonia, bronchoalveolar cell carcinoma, lymphoma and infection

Diffuse nodules may also be seen in COP

Nonspecific Interstitial Pneumonia •

• • • •

•

Katzenstein ➢ Described in 1994 Does not fit definition of other IIPs ➢ UIP, RB-ILD, DIP, OP, AIP Represents a variety of etiologies ➢ Collagen vascular disease, drug reaction, inhaled antigen ➢ Inadequately sampled UIP or OP Median age 45 Onset gradual with wide range ➢ 6 months to 3 years Better prognosis

Katzenstein, Am J Resp Crit Care 1994 Nicholson, Am J Respir Crit Care Med 2001

The Idiopathic Interstitial Pneumonias

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Chest Radiology

Nonspecific Interstitial Pneumonitis Histology •

• • •

3 categories ➢ Cellular ➢ Fibrosing ➢ Mixed Prognosis=fibrosis OP common Temporally uniform

Nonspecific Interstitial Pneumonitis Imaging • •

• •

Few reports on chest radiography Wide variety of CT patterns ➢ Ground glass, consolidation, reticular and honeycombing Traction bronchiectasis=fibrosis CT pattern indistinguishable ➢ UIP 32% ➢ Hypersensitivity 20% ➢ OP 14% ➢ Other 12%

Hartman, Radiology 2000

Nonspecific Interstitial Pneumonia NSIP ATS Consensus Conference • • • • •

Pathologists Radiologists Pulmonalogists 300 cases submitted ➢ 11 cases agreed to be NSIP by all pathologists Imaging ➢ Lower lobe ➢ Peribronchiolar reticulation and distortion ➢ Subpleural clearing

NSIP Current View

NSIP Fibrosis with IPF Imaging • •

•

Areas of NSIP commonly found in proven cases of UIP NSIP and UIP ➢ Different severity of injury? ➢ Different mechanism of injury? Prognosis in these cases is driven by the imaging

Katzenstein AA et al, Amer J of Surg Path 2002

Figure 1-2-15

Hypersensitivity Pneumonitis NSIP in Cigarette Smokers

Nonspecific Interstitial Pneumonia OP-NSIP

[Figure 1-2-15]

OP-NSIP is peribronchovascular with UIP is peripheral Chest Radiology

21

The Idiopathic Interstitial Pneumonias

The Idiopathic Interstitial Pneumonias Current List •

• • • • •

Figure 1-2-16

Idiopathic Pulmonary Fibrosis (IPF) ➢ Usual Interstitial Pneumonia (UIP) Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) Desquamative Interstitial Pneumonia (DIP) Acute Interstitial Pneumonia (AIP) Cryptogenic Organizing Pneumonia (COP) NonSpecific Interstitial Pneumonia (NSIP)

Idiopathic Pulmonary Fibrosis [Figure 1-2-16]

RB/RB-ILD [Figure 1-2-17]

RB-ILD/DIP [Figure 1-2-18]

IPF

Figure 1-2-17

RB-ILD

Figure 1-2-18

DIP

Acute Interstitial Pneumonia [Figure 1-2-19 and 1-2-20] Figure 1-2-19

Figure 1-2-20

AIP early phase

The Idiopathic Interstitial Pneumonias

AIP late phase with organization and fibrosis 22

Chest Radiology

Organizing Pneumonia NSIP in the Literature

Figure 1-2-21

[Figure 1-2-21]

NSIP-IPF

NSIP-Cigarette Smokers [Figure 1-2-22] Figure 1-2-22

Organizing pneumonia

Patients with smoking related fibrosis may have a biopsy that demonstrates NSIP

NSIP-Hypersensitivity Pneumonitis

NSIP-Organizing Pneumonia [Figure 1-2-23] Figure 1-2-23

Patients with organizing pneumonia may have a biopsy that demonstrates NSIP

Chest Radiology

23

The Idiopathic Interstitial Pneumonias

References

General 1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165:277-304 2. Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic interstitial pneumonias. Radiographics. 2003 Sep-Oct;23(5):1057-71. IPF/UIP 1. Hansell DM, Wells AU. CT evaluation of fibrosing alveolitis—applications and insights. J Thorac Imaging 1996; 11(4):231-49. 2. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am U Respir Crit Care Med 1998; 157(4 Pt 1):1301-15. 3. Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases. Chest 1993; 103(6):1808-12. 4. Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Resp Res 1975; 8:133. 5. Tobin RW, Pope CE, 2nd, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 158(6): 1804-8. 6. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990; 176(755759). 7. Coxson HO, Hogg JC, Mayo JR, Behzad H, Whittall KP, Schwait DA, Hartley PC, Galvin JR, Wilson JS, Hunninghake SW. Quantification of idiopathic pulmonary fibrosis using computed tomography and histology. Am J Respir Crit Care Med 1997; 155(5):1649-56. 8. Gay SE, Kazerooni EA, Toews GB, Lynch UP, 3rd, Gross BH, Cascade PN, Spizarny DL, Flint A, Schork MA, Whyte RI, Popovich U, Hyzy R, Martinez FJ. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am U Respir Crit Care Med 1998; 157(4 Pt 1):1063-72. 9. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar Ho, Schroeder DR, Offord KR. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 157(1):1 99-203. DIP 1. Gaensler EA, Goff AM, Prowse CM. Desquamative interstitial pneumonia. N Engl U Med 1966; 274(3)113-28. 2. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA.Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest. 2005 Jan;127(1):178-84.

DAD/AIP 1. Bone RC. The ARDS lung. New insights from computed tomography [editorial; comment]. Jama 1993; 269(1 6):21 34-5. 2. Desai SR, Wells AU, Rubens MB, Evans TW, Hansell DM. Acute respiratory distress syndrome: CT abnormalities at long-term follow-up. Radiology 1999; 210(1):29-35. 3. Greene R. Adult respiratory distress syndrome: acute alveolar damage. Radiology 1987; 163(1):57-66. 4. Ichikado K, Johkoh T, Ikezoe U, Takeuchi N, Kohno N, Arisawa U, Nakamura H, Nagareda T, Itoh H, Ando M. Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AUR Am U Roentgenol 1997; 1 68(2):333-8. 5. Johkoh T, Muller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda 0, Tomiyama N, Nakamura H. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999; 211(3):859-63. 6. Katzenstein AL, Myers UL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell kinetic study. Am U Surg Pathol 1986; 10(4):256-67. 7. Olson U, Colby TV, Elliott CG. Hamman-Rich syndrome revisited [see comments]. Mayo Clin Proc 1990; 65(12):1538-48. 8. Primack SL, Hartman TE, Ikezoe U, Akira M, Sakatani M, Muller NL. Acute interstitial pneumonia: radiographic and CT findings in nine patients [see comments]. Radiology 1993; 188(3):817-20.

NSIP 1. Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JR Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 1998; 158(4):1286-93. 2. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994; 18(2):136-47. The Idiopathic Interstitial Pneumonias

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3.

Kim TS, Lee KS, Chung MP, Han J, Park JS, Hwang JH, Kwon OJ, Rhee OH. Nonspecific interstitial pneumonia with fibrosis: high-resolution CT and pathologic findings. AJR Am J Roentgenol 1998; 171(6): 1645-50.

BOOP/Organizing Pneumonia 1. Akira M, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple large nodules or masses. AJR Am J Roentgenol 1998; 170(2):291-5. 2. Carlson BA, Swensen SJ, O’Connell EJ, Edell ES. High-resolution computed tomography for obliterative bronchiolitis. Mayo Clin Proc 1993; 68(3):307-8. 3. Chandler PW, Shin MS, Friedman SE, Myers JL, Katzenstein AL. Radiographic manifestations of bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR Am J Roentgenol 1986; 147(5):899906. 4. Epler GR, Colby TV, McLoud TC, Carrington CB, Oaensler EA. Bronchiolitis obliterans organizing pneumonia. N Engl J Med 1985; 312(3):152-8. 5. Gosink RB, Friedman Pd, Liebow AA. Bronchiolitis obliterans. Roentgenologic¬pathologic correlation. Am J Roentgenol Radium Ther Nucl Med 1973; 11 7(4):81 6-32. 6. Haddock JA, Hansell DM. The radiology and terminology of cryptogenic organizing pneumonia. Br J Radiol 1992; 65(776):674-80. 7. Katzenstein AL, Myers JL, Prophet WD, Corley LS, 3rd, Shin MS. Bronchiolitis obliterans and usual interstitial pneumonia. A comparative clinicopathologic study. Am J Surg Pathol 1986; 10(6):373-81. 8. Lau DM, Siegel MJ, Hildebolt CF, Cohen AH. Bronchiolitis obliterans syndrome: thin-section CT diagnosis of obstructive changes in infants and young children after lung transplantation. Radiology 1998; 208(3):783-8. 9. Lee KS, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR Am J Roentgenol 1994; 162(3):543-6. 10. Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD. Organizing pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997; 157(12):1323-9. 11. McLoud TC, Epler GR, Colby TV, Gaensler EA, Carrington CB. Bronchiolitis obliterans. Radiology 1986; 159(1)1-8. 12. Muller NL, Cuerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. Radiology 1987; 162(1 Pt 1):151-6. 13. Muller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR Am J Roentgenol 1990; 154(5):983-7.

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The Idiopathic Interstitial Pneumonias

Airways Disease: The Movement from Anatomic to Physiologic Assessment Jeffrey R. Galvin, MD

Assessment of Dyspnea A Common Clinical Problem • • • •

55 million adult smokers 15 million meet criteria for bronchitis 5 million with airway obstruction 10 million with asthma

Figure 1-3-1

Gordon Snyder

Differential Diagnosis of Airways Obstruction •

•

Common Emphysema, bronchitis, bronchiectasis, asthma Uncommon ➢ LAM, BO, panbronchiolitis, sarcoid, alpha-1 deficiency, ABPA ➢

Diseases with Obstructive Physiology The Changing Role of Imaging • •

Diagnosis Functional assessment

Why Pulmonary Functions are Insensitive •

• •

PFT’s based on wide range of normal 80-120% predicted Diseases with opposing physiologic processes The “silent zone” of the lungs

➢

Small airway tethered to the pleural surface by alveolar walls

The “Silent Zone” of the Lungs [Figure 1-3-1 and 1-3-2]

“Small Airways” •

• •

Figure 1-3-2

Peter Macklem ➢ 1970’s No cartilage ➢ <2mm physiologists ➢ 1mm pathologists Tethered ➢ fiber skeleton ➢ pleura

Weibel

Mosaic attenuation on an expiratory CT in patient with constrictive bronchiolitis

Airways Disease

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Chest Radiology

Diffuse Lung Disease Airways [Figure 1-3-3] • • • •

Figure 1-3-3

Airways involvement Obstructive physiology Increased lung volumes Decreased attenuation

Figure 1-3-4

Airways Disease Direct Signs [Figure 1-3-4] • •

Changes Airway wall Airway lumen Opacities ➢ Tubular ➢ Nodular ➢ Branching ➢ ➢

Airways Disease Indirect Signs •

Mosaic density Air trapping Subsegmental atelectasis Ground glass

• •

➢

• • • • • • • • • • •

Emphysema Emphysema and Fibrosis Alpha-1 deficiency Langherhans Cell Histiocytosis Bronchiectasis Asthma Allergic Bronchopulmonary Aspergillosis Sarcoidosis Diffuse Panbronchiolitis Bronchiolitis Obliterans Lymphangioleiomyomatosis

•

Permanent enlargement of airspaces distal to the terminal bronchiole, accompanied by destruction of the walls without obvious fibrosis

Airways Disease

Airways involvement at the level of the secondary lobule

“Tree-in-bud” in a patient with a respiratory infection

Figure 1-3-5

Emphysema ATS Definition Emphysema

Emphysema Classification [Figure 1-3-5] •

• •

Proximal Acinar Centrilobular Resp bronchiole Cigarette smoke Upper lobes Panacinar ➢ Entire acinus ➢ Alpha-1 deficiency ➢ Lower lobes Distal Acinar ➢ Paraseptal ➢ Distal acinus ➢ Subpleura ➢ Pneumothorax ➢ ➢ ➢ ➢

Chest Radiology

Cigarette smoke related emphysema is most severe in the upper lobes 27

Airways Disease

Emphysema Clinical Presentation • •

• •

Figure 1-3-6

Cough, dyspnea and sputum production Hemoptysis rare ➢ R/O cancer Symptomatic air flow obstruction ➢ After age 50, 20-30 years of smoking Cor pulmonale (late) related to hypoxemia and loss of capillary bed

Emphysema and Cor Pulmonale Emphysema Pulmonary Functions • •

• • • •

Important to identify patients at risk Reduction in Fev1 ➢ Most reproducible RV increases followed by TLC Volumes and flows ➢ Insensitive to early changes Diffusing capacity ➢ Sensitive but non-specific Small airways tests

Saber trachea

Figure 1-3-7

Emphysema Radiographic Feature •

• •

•

Hyperinflation Concave diaphragm Increased A-P diameter Retrosternal airspace Arterial deficiency pattern Bulla ➢ Cystic airspaces > 1cm Radiography is insensitive ➢ 41% of moderate disease ➢ 66% of severe disease ➢ ➢ ➢

Saber Trachea [Figure 1-3-6]

Emphysema and Computed Tomography [Figure 1-3-7]

Typical low attenuation lesions of emphysema

The Diagnosis of Mild Emphysema Correlation of CT and Pathology Scores •

HRCT detects emphysema Before there is airflow limitation on PFT’s HRCT excludes emphysema ➢ Patients with moderate to severe airflow limitation ➢

•

Kuwano et al, Am Rev Respir Dis 1990

Early Emphysema

Airways Disease

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Chest Radiology

Respiratory Bronchiolitis [Figure 1-3-8] “Smoker’s Bronchiolitis” •

• • •

Figure 1-3-8

Common change all smokers Pigmented macrophages ➢ In respiratory bronchioles ➢ Surrounding alveoli Upper lobe predominance Usually asymptomatic ➢ May cause symptoms ➢

Relationship of RB and Emphysema • • •

•

Prospective study 111 subjects ➢ Followed for 5 years ✧ Imaged at inception TO and 5 years T1 ➢ Smokers, nonsmokers and quitters Micronodules at TO predisposes to the development of emphysema at T1 Micronodules and emphysema at TO predicts more rapid decline in lung function

Remy-Jardin, Radiology 2001

Relationship of RB and Emphysema Remy-Jardin, Radiology 2001

Respiratory bronchiolitis

Emphysema and Fibrosis • • •

14 patients Scanning electron microscopy Thick and thin walls ➢ Both fibrotic

Figure 1-3-9

Nagai & Thurlbeck, Am Rev Resp Dis 1985

Emphysema and Fibrosis

•

Normal lung volumes and Normal flow rates Reduced diffusing capacity ➢ Severe Minimal pulmonary reserve

• • • • •

TLC 119% VC 126% RV 109% FEV1/FVC 88% D/Va 28%

• • •

Emphysema and Fibrosis

Emphysema and Fibrosis [Figure 1-3-9]

Emphysema and fibrosis

Idiopathic Pulmonary Fibrosis Langerhans Cell Histiocytosis Clinical Presentation • • • • •

Almost exclusively cigarette smokers Slight male preponderance Cough and dyspnea most common May be asymptomatic Occasional bone lesion

Chest Radiology

29

Airways Disease

Langerhans Cell Histiocytosis Histology [Figure 1-3-10] • • •

•

Figure 1-3-10

Nodular Interstitial lesions Located near bronchioles Histiocytes, eosinophils, plasma cells and lymphocytes Diagnosis requires Langerhans cells ➢ Large histiocytes ➢ Folded nuclei ➢ Eosinophilic cytoplasm Path DDX ➢ Eos pneumonia, DIP,UIP ➢ ➢

Langherhans Cell Histiocytosis [Figures 1-3-11 and 1-3-12]

Figure 1-3-11

The range of findings in Langerhans Cell Histiocytosis

Figure 1-3-12

Cystic lesions in LCH

Typical nodules in LCH

Langherhans Cell Histiocytosis Radiographic Features [Figure 1-3-13] • •

• • • • •

Figure 1-3-13

Varies over time Upper lobe ➢ Predominance Nodules ➢ 0.5-1.0 cm in upper lobes ➢ Early Cysts replace nodules ➢ Later Honeycomb lung Pneumothorax 15% Adenopathy and effusion are unusual

LCH is characterized by low attenuation areas with bizarre shapes Airways Disease

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Chest Radiology

Langerhans Cell Histiocytosis EM and Immunohistochemistry •

• •

Immunoperoxidase staining CD1a, S-100 protein Cells in clusters in interstitium EM ➢ X-bodies ➢ Langerhans cell granules ➢ Birbeck granules ➢

Figure 1-3-14

Langerhans Cell Histiocytosis Clinical Course •

• • •

Clinical resolution Common Radiographic abnormalities ➢ Persist Occasional progression ➢ Fibrosis and honeycombing May be fatal ➢ Rapid progression ➢

Langherhans Cell Histiocytosis Alpha-1 Antitrypsin Deficiency Pathophysiology

• • • • • •

1-2% of emphysema in the US Alpha-1 antitrypsin inactivates neutrophil elastase Production controlled by 2 genes Level of antitrypsin dependent on allele ZZ homozygotes most severe Smoking accelerates the destruction

• • • •

Radiograph may be normal Lower lobe predominance Panacinar emphysema CT ➢ Upper lobe involvement ➢ Bronchiectasis ➢ Airway thickening common CT more sensitive

Alpha-1 Antitrypsin Deficiency Imaging Features

•

Alpha-1 antitrypsin deficiency

Alpha-1 Antitrypsin Deficiency [Figure 1-3-14]

Figure 1-3-15

Bronchiectasis Pathophysiology • • • • • •

Dilatation of bronchi Reversible form ➢ Infection ➢ Atelectasis Congenital ➢ tracheobronchomalacia Post-inflammatory Postobstructive Fibrotic ➢ IPF ➢ Sarcoid

Williams-Campbell [Figure 1-3-15] Chest Radiology

Williams-Campbell 31

Airways Disease

Mounier-Kuhn Syndrome

Figure 1-3-16

Bronchiectasis Postinflammatory •

• • • •

Primary Ciliary Dyskinesia Kartagener’s Immunodeficiency Postinfectious ➢ TB, Measles, pertussis, viral Post-toxic bronchitis ➢ gastric acid aspiration Immunologic ➢ ABPA ➢

Primary Ciliary Dyskinesia

Post Obstructive Bronchiectasis [Figure 1-3-16] • • • •

Neoplasm Foreign body Broncholith Lymph node enlargement

• • • • •

Cough Purulent sputum Hemoptysis (50%) Dyspnea Rare ➢ clubbing, brain abscess, amyloidosis

Figure 1-3-17

Bronchiectasis Clinical Presentation

Bronchiectasis Radiographic Features

• • • • •

Prominent markings Crowding of Vessels “Tram Tracks” Loss of volume Cystic spaces

• • • •

Bronchi in the periphery “Signet Rings” “Tram Tracks” Sensitivity ➢ Collimation

Post obstructive bronchiectasis in a patient with mucoepidermoid carcinoma

Upper lobe smoking related emphysema

Figure 1-3-18

Bronchiectasis CT Features

RB-ILD

Emphysema [Figure 1-3-17]

Figure 1-3-19

RB/RB-ILD [Figure 1-3-18]

Emphysema and Fibrosis [Figure 1-3-19]

Emphysema and fibrosis Airways Disease

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Chest Radiology

Langherhans Cell Histiocytosis [Figures 1-3-20 to 1-3-22] Alpha-1 Antitrypsin Deficiency [Figure 1-3-23]

Figure 1-3-21

Figure 1-3-20

Early LCH nodules

Late LCH Cysts and nodules

End-stage LCH

Lower lobe predominance in Alpha-1 antitrypsin

Figure 1-3-22

Figure 1-3-23

Bronchiectasis

Diffuse Lung Disease Airways • • • •

Airways involvement Obstructive physiology Increased lung volumes Decreased attenuation

• • •

Reversible airway disease Increased airway responsiveness Persistent airflow obstruction occurs in chronic asthmatic ➢ Why? 6% in the American population ➢ Rate has doubled in 20 years ➢ Higher incidence in large cities

Asthma ATS Definition

•

Asthma Extrinsic • • • • • •

Family history atopy Early onset <30 years Seasonal symptoms Increased IGE Positive skin tests Often remits

Chest Radiology

33

Airways Disease

Asthma Intrinsic •

• • • •

Figure 1-3-24

No atopy Absence of external triggers Older age group Increased blood eosinophils Increased sputum eosinophils Fixed airway obstruction ➢ Progressive ➢

Asthma Pathology •

• •

Airway smooth muscle Hypertrophy Airway wall ➢ Inflammation ➢ Edema Airway plugging ➢ Mucus ➢ Inflammatory exudate ➢

Airway thickening in asthma

Figure 1-3-25

Asthma Radiographic Features [Figure 1-3-24] •

• • • •

Chest roentgenogram Often normal Airway thickening ➢ Chronic disease Rapid attenuation of vessels ➢ hypoxemia Pneumomediastinum ➢ pneumothorax Hyperinflation ➢ Adaptive ➢ Later air trapping ➢

Silva AJR 183 September 2004

Asthma-Hyperinflation [Figure 1-3-25] Asthma [Figure 1-3-26] CT Features • •

•

More sensitive than CXR Reversible ➢ Consolidation ➢ Atelectasis ➢ Mucoid impaction ➢ Airway Narrowing ➢ Air Trapping Permanent ➢ Bronchial wall thickening ➢ Bronchiectasis ➢ Emphysema

Severe hyperinflation in which you can see the slips of the diaphragm as it inverts

Figure 1-3-26

Mucoid impaction in severe asthma Airways Disease

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Chest Radiology

Allergic Bronchopulmonary Aspergillosis Primary Criteria • • • • • • •

Figure 1-3-27

Asthma Eosinophilia Immediate skin test reactivity Precipitating antibodies (IgG) Elevated serum (IgE) Pulmonary infiltrates Central bronchiectasis

Allergic Bronchopulmonary Aspergillosis Presentation & Pathology [Figure 1-3-27] •

• • • • •

Atopic individuals Most common Cystic fibrosis Airways filled ➢ Fungus ➢ Inspissated mucous Presentation with ➢ Cough, fever ➢ Hemoptysis ➢ Worsening asthma Seen in stable asthmatics Good response to steroids ➢

ABPA represents a non-invasive colonization of the airways

Allergic Bronchopulmonary Aspergillosis Imaging [Figures 1-3-28 and 1-3-29] •

• • •

Figure 1-3-28

Bifurcating opacities ➢ “Gloved-finger” ➢ Mucous filled airways Central Bronchiectasis Fleeting infiltrates Pleural disease ➢ Uncommon

Sarcoidosis and the Airways Computed Tomography •

• •

Functional evidence of airways obstruction Obstructive PFT’s are common Endobronchial biopsies find granulomas Obstructive physiology correlates with ➢ Decreased attenuation on expiratory scans (small airways) ➢ Reticular pattern and advanced fibrotic disease (large airways) ➢

Hansell et al, Radiology 1998

Endobronchial Granulomas

Mucoid impaction in ABPA

Figure 1-3-29

Small Airway Distortion

Reticular Pattern and Fibrosis

Central bronchiectasis in ABPA

Chest Radiology

35

Airways Disease

Diffuse Panbronchiolitis • • • • •

Japan most common Rarely: Korea, China, Europe and North America HLA BW54 M-F 2:1 Presentation ➢ Dyspnea ➢ Cough Obstructive PFT’s Slowly progressive ➢ 15 yr mean survival Erythromycin ➢ May not be an antibacterial effect ➢ ➢ ➢

Diffuse Panbronchiolitis Pathology • •

•

Discrete nodules Early infiltration ➢ Interstitium ➢ Respiratory bronchioles ➢ Alveolar ducts ➢ Foamy histiocyte, lymphocyte and plasma cells Late secondary ectasia ➢ Proximal terminal bronchioles

Figure 1-3-30

Diffuse Panbronchiolitis Imaging Early • •

Radiography Nodules 5mm Hyperinflation Computed Tomography ➢ Centrilobular nodules ➢ Branching opacities ➢ Mosaic attenuation ➢ ➢

Early Diffuse Panbronchiolitis represented by widespread airways nodules

Diffuse Panbronchiolitis [Figure 1-3-30]

Figure 1-3-31

Diffuse Panbronchiolitis Imaging Late [Figure 1-3-31] • •

Radiography Nodules Cysts and bulla Hyperinflation Computed Tomography ➢ Centrilobular nodules ➢ Bronchiolectasis ➢ Bronchiectasis ➢ ➢ ➢

Constrictive Bronchiolitis Introduction •

Confusing Terminology Obliterative Bronchiolitis Bronchiolitis Obliterans Bronchiolitis Obliterans Organizing Pneumonia ✧ Different disease ✧ Cryptogenic organizing pneumonia Small Airways ➢ Fibrosis ➢ Inflammation Response to ➢ Inflammatory disorders ➢ Infectious disorders ➢ ➢ ➢

• •

Airways Disease

36

Severe airway involvement in panbronchiolitis Chest Radiology

Constrictive Bronchiolitis Clinical Presentation • • • •

Figure 1-3-32

Cough, dyspnea and malaise History ➢ prior infection ➢ exposure Hypoxemia Airway obstruction

Constrictive Bronchiolitis Classification •

• • • • •

Infection RSV, adenovirus and mycoplasma Toxic Inhalation ➢ Ammonia, acid and NO Aspiration: gastric acid Collagen Vascular: RA Organ Transplantation Unknown ➢

Constrictive Bronchiolitis Histology [Figure 1-3-32] •

• •

Obstruction Terminal bronchiole Respiratory bronchioles Polyps of fibrosis Cellular infiltration ➢ Lymphs ➢ Plasma cells ➢ Histiocytes

Constrictive bronchiolitis

➢ ➢

Constrictive Bronchiolitis Imaging [Figures 1-3-33 and 1-3-34] • • • • • •

Hyperinflation Localized Diffuse Discrete nodules ➢ Airway associated Mosaic pattern Airway thickening Bronchiectasis Air trapping ➢ ➢

Figure 1-3-34

Figure 1-3-33

Central bronchiectasis and mosaic attenuation in constrictive bronchiolitis

Mosaic attenuation in constrictive bronchiolitis Chest Radiology

37

Airways Disease

Swyer-James Syndrome [Figures 1-3-35 and 1-3-36] Figure 1-3-35

Unilateral hyperlucent lung in a patient with Swyer-James

Swyer-James Syndrome

Swyer-James Syndrome-Adenovirus

Figure 1-3-37

Lymphangioleiomyomatosis Clinical Presentation • • • • • •

Exclusively women Reproductive years Progressive dyspnea Chylous pleural effusions Hemoptysis Massive hemorrhage

• • • • • •

Obstructive defect FEV1 is decreased TLC and RV increased DLCO reduced Hypoxemia Hypocapnia

Figure 1-3-36

Lymphangioleiomyomatosis Function

Lymphangioleiomyomatosis Histology •

Smooth muscle proliferation Disorderly ➢ Bronchioles, alveolar septa, arteries, veins and lymphatics Small air filled cysts ➢ Air trapping ➢

•

Figure 1-3-38 Typical thin-walled cyst in lymphangioleiomyomatosis

Lymphangioleiomyomatosis Gross Features [Figures 1-3-37 and 1-3-38] •

• • •

Cysts 0.2-2cm Diffuse involvement Enlarged thoracic duct Enlarged lymph nodes

➢

The upper and lower lobes are equally involved in LAM Airways Disease

38

Chest Radiology

Lymphangioleiomyomatosis Radiographic Features •

• • • •

Reticulonodular opacities Basilar Lung volume ➢ Normal ➢ Increased Pleural effusion ➢ 60-75% Pneumothorax Honeycombing late ➢

Lymphangioleiomyomatosis CT Features [Figure 1-3-39] •

• • •

Figure 1-3-39

Thin-walled cysts More sensitive than plain film Diffuse Bilateral involvement Adenopathy

➢

Thin-walled cysts and a pneumothorax in patient with lymphangioleiomyomatosis

Figure 1-3-40

Lymphangioleiomyomatosis [Figure 1-3-40] Lymphangioleiomyomatosis Therapy and Prognosis • •

• •

Slowly progressive course Variable Progression ➢ Cor pulmonale ➢ Respiratory insufficiency 50-80% 5 year survival ➢ Average survival 10 years Hormonal therapy ➢ Oophorectomy, progesterone ➢

Tuberous Sclerosis [Figure 1-3-41]

Minimal disease in LAM may be difficult may be difficult to separate from emphysema

Emphysema

Emphysema and Fibrosis

Figure 1-3-41

Langherhans Cell Histiocytosis Alpha-1 Antitrypsin Deficiency Asthma [Figure 1-3-42]

Figure 1-3-42

LAM may represent partial expression of tuberous sclerosis

Asthma Chest Radiology

39

Airways Disease

ABPA [Figure 1-3-43]

Figure 1-3-43

Sarcoidosis [Figure 1-3-44]

Diffuse Panbronchiolitis [Figure 1-3-45]

Constrictive Bronchiolitis [Figure 1-3-46] Swyer-James Syndrome LAM [Figure 1-3-47]

Physiologic Measurement An Integral Part of Imaging •

Imaging provides physiologic information not available from pulmonary functions Air content and blood flow can be quantified

•

➢

• • • • • • • • • • •

Emphysema Emphysema and Fibrosis Alpha-1 deficiency Histiocytosis-X Bronchiectasis Asthma Allergic Bronchopulmonary Aspergillosis Sarcoidosis Diffuse Panbronchiolitis Bronchiolitis Obliterans Lymphangioleiomyomatosis

ABPA

Airways Disease

Sarcoidosis airways involvement

Figure 1-3-45

Figure 1-3-46

Mosaic attenuation in constrictive bronchiolitis

Diffuse Panbronchiolitis

Typical findings in LAM

Figure 1-3-47 Airways Disease

Figure 1-3-44

40

Chest Radiology

Diffuse Lung Disease Airways • • • •

Airways involvement Obstructive physiology Increased lung volumes Decreased attenuation

References

General 1. Hartman T, Primack 5, Lee K, Swensen S, Muller N. CT of bronchial and bronchiolar diseases. RadioGraphics 1994; 14:991-1003. 2. Hogg JO, Macklein PT, Thurlbeck WM. Site and nature of airway obstruction in chronic obstructive lung disease. N Engl J Med 1968; 278(25):1355-60. 3. King GO, Muller NE, Pare PD. Evaluation of airways in obstructive pulmonary disease using high- resolution computed tomography. Am J Respir Crit Care Med 1999; 159(3):992-1 004. 4 Lucidarme O, Coche E, Cluzel P, Mourey-Gerosa I, Howarth N, Grenier P. Expiratory CT scans for chronic airway disease: correlation with pulmonary function test results. AJR Am J Roentgenol 1998; 170(2)101-7. 5. . Macklem PT. Obstruction in small airways—a challenge to medicine. Am J Med 1972; 52(6):721-4. 6. Muller NE, Miller RR. Diseases of the bronchioles: CT and histopathologic findings. Radiology 1995; 196(1):3-12. 7. Naidich D, McCauley Dl, Khouri NF, al e. Computed tomography of bronchiectasis. Journal of Computer Assisted Tomography 1982; 6:437-444. 8. Neeld DA, Goodman LR, Gurney JW, Greenberger PA, Fink JN. Computerized tomography in the evaluation of allergic bronchopulmonary aspergillosis. American Review of Respiratory Disease 1990; 142:1200-1205. 9. Snider GE. Distinguishing among asthma, chronic bronchitis, and emphysema. Chest 1985; 87(1, supplement):35539S. 10. Stern EJ, Swensen S, Hartman T, Frank M. Ct mosaic pattern of lung attenuation: distinguishing different causes. American Journal of Roentgenology 1995; 165:813-816. 11. Teel G, Engeler C, Tahsijain J, duCret R. Imaging of small airways disease. RadioGraphics 1996; 16:27-41. 12. Weibel ER, Rachofen H. The Fiber Scaffold of Lung Parenchyma. In: Crystal RG, West JB, eds. The Lung. New York: Raven Press, 1991; 787-794. 13. Weibel ER, Crystal RG. Structural Organization of the Pulmonary Interstitium. In: Crystal RG, West JB, eds. The Lung. New York: Raven Press, 1991; 369-380. 14. Worthy SA, Muller NE, Hartman TE, Swensen S, Padley SF, Hansell CM. Mosaic attenuation pattern on thin-section CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause. Radiology 1997; 205(2):465-70.

Emphysema 1. Bankler AA De Maertelaer V, Keyzer C, Gevenois PA. Pulmonary emphysema: subjective visual grading versus objective quantification with macroscopic morphometry and thin-section CT densitometry. Radiology 1999; 211(3):8518. 2. Coxson HO, Rogers RM, Whittall KP, D'Yachkova Y, Pare PD, Sciurba FC, Hogg JC. A quantification of the lung surface area in emphysema using computed tomography. Am J Respir Crit Care Med 1999; 159(3):851-6. 3. Gelb AF, Hogg JC, Muller NE, Schein MJ, Kuei J, Tashkin DP, Epstein JD, Kollin J, Green Rh, Zamel N, Elliott WM, Hadjiaghai E. Contribution of emphysema and small airways in COPD. Chest 1996; 109(2):353-9. 4. Kinsella M, Muller NE, Staples C, Vedal S, Chan-Yeung M. Hyperinflation in asthma and emphysema. Assessment by pulmonary function testing and computed tomography. Chest 1988; 94(2):286-9. 5. Kinsella M, Muller NE, Abboud RT, Morrison NJ, DyBuncio A. Quantitation of emphysema by computed tomography using a "density mask" program and correlation with pulmonary function tests. Chest 1990; 97:315-321. 6. Klein JS, Gamsu G, Webb WR, Golden JA, Muller NE. High-resolution CT diagnosis of emphysema in symptomatic patients with normal chest radiographs and isolated low diffusing capacity. Radiology 1992; 182(3):817-21. 7. Kondoh Y, Taniguchi H, Yokoyama S, Taki F, Takagi K, Satake I Emphysematous change in chronic asthma in relation to cigarette smoking: assessment by computed tomography. Chest 1990; 97:845-849. 8. Kuwano K, Matsuba K, Ikeda T, Murakami J, Araki A, Nishitani H, Ishida T, Yasumoto K, Shigematsu N. The diagnosis of mild emphysema. Correlation of computed tomography and pathology scores. Am Rev Respir Dis 1990; 141(1):16978. 9. Miller RR, Muller NE, Vedal S, Morrison NJ, Staples CA. Limitations of computed tomography in the assessment of emphysema. American Review of Respiratory Disease 1989; 139:980-983. 10. Muller NE, Thurlbeck WM. Thin-section CT, emphysema, air trapping, and airway obstruction [editorial;comment]. Radiology 1996; 1 99(3):621 -2. 11.Muller NE, Staples CA, Miller RR, Abboud RT. Density Mask” An objective method to quantitate emphysema using computed tomography. Chest 1988; 94:782-787. Chest Radiology

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12. Nagao M, Murase K, Yasuhara Y, Ikezoe J. Quantitative analysis of pulmonary emphysema: three-dimensional fractal analysis of single-photon emission computed tomography images obtained with a carbon particle radioaerosol. AJR Am J Roentgenol 1998; 171(6):1657-63. 13. Park KJ, Bergin CJ, Clausen JE. Quantitation of emphysema with three-dimensional CT densitometry: comparison with two-dimensional analysis, visual emphysema scores, and pulmonary function test results. Radiology 1999; 211(2):541-7 14. Remy-Jardin M, Remy J, Gosselin B, Becette V, Edme J. Lung parenchymal changes secondary to cigarette smoking: pathologic-ct correlations. Radiology 1993; 186:643-651. 15. Snider GE, Kleinerman J, Thurlbeck WM, Bengali Zl-i. The definition of emphysema. Report of the National Heart, Blood and Eung Institute, Division of Eung Diseases Workshop. American Review of Respiratory Diseases 1985; 132:182-1 85. 16. Sutinen S, Christoforidis AJ, Klugh GA, Pratt PC. Roentgenologic criteria for the recognitiion of nonsymptomatic pulmonary emphysema. American Review of Respiratory Disease 1965; 91:69-76. 17. Uppaluri R, Mitsa T, Sonka M, Hoffman EA, McLennan G. Quantification of pulmonary emphysema from lung computed tomography images. Am J Respir Crit Care Med 1997; 156(1:248-54. Alpha-1 Antitrypsin 1. Brantly ME, Paul ED, Miller BH, Falk RT, Wu M, Crystal RG, Clinical features and history of the destructive lung disease associated with alpha-1-antitrypsin deficiency of adults with pulmonary symptoms. Am Rev Respir Dis 1988; 138(2):327-36. 2. Brantly M, Nukiwa T, Crystal RG. Molecular basis of alpha-1-antitrypsin deficiency. Am J Med 1988; 84(6A):1331. 3. Guest PJ, Hansell CM. High resolution computed tomography (HRCT) in emphysema associated with alpha-1antitrypsin deficiency. Clin Radiol 1992; 45(4):260-6. 4. Kueppers F, Black ER Alphal-antitrypsin and its deficiency. Am Rev Respir Dis 1974; 110(2):176-94.

Eosinophilic Granuloma 1. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with highresolution CT. Radiology 1989; 172(1):255-8. 2. Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in the adult. Medicine (Baltimore) 1981; 60(6):385-96. 3. Lacronique J, Roth C, Battesti JP, Basset F, Chretien J. Chest radiological features of pulmonary histiocytosis X: a report based on 50 adult cases. Thorax 1982; 37(2):104-9. 4. Moore AD, Godwin JO, Muller NE, Naidich DP, Hammar SR Buschman DE, Takasugi JE, de Carvalho CR. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989; 172(1):249-54. 5. Stern EJ, Webb WR, Golden JA, Gamsu G. Cystic lung disease associated with eosinophilic granuloma and tuberous sclerosis: air trapping at dynamic ultrafast high-resolution CT. Radiology 1992; 182(2):325-9. Asthma 1. Backman KS, Greenberger PA, Patterson R. Airways obstruction in patients with long-term asthma consistent with irreversible asthma’. Chest 1997; 112(5): 1234-40. 2. Brown RH, Herold CJ, Hirshman CA, Zerhouni EA, Mitzner W. In vivo measurements of airway reactivity using highresolution computed tomography. Am Rev RespirDis 1991; 144(1):208-12. 3. Haraguchi M, Shimura S, Shirato K. Morphometric analysis of bronchial cartilage in chronic obstructive pulmonary disease and bronchial asthma. Am J Respir Crit Care Med 1999; 159(3):1005-13. 4. Kinsella M, Muller NE, Staples C, Vedal S, Chan-Yeung M. Hyperinflation in asthma and emphysema. Assessment by pulmonary function testing and computed tomography. Chest 1988; 94(2):286-9. 5. Martin J, Powell E, Shore S, Emrich J, Engel EA. The role of respiratory muscles in the hyperinflation of bronchial asthma. Am Rev Respir Dis 1980; 121(3):441-7. 6. Paganin F, Trussard V, Seneterre E, Chanez R Giron J, Godard R Senac JP, Michel FB, Bousquet J. Chest radiography and high resolution computed tomography of the lungs in asthma. Am Rev Respir Dis 1992; 146(4):1084-7. 7. Silva CI, Colby TV, Muller NL. Asthma and associated conditions: high-resolution CT and pathologic findings. AJR Am J Roentgenol. 2004 Sep;183(3):817-24. Allergic Bronchopulmonary Aspergillosis 1. Neeld CA, Goodman LR, Gurney JW, Greenberger PA, Fink JN. Computerized tomography in the evaluation of allergic bronchopulmonary aspergillosis. Am Rev Respir Dis 1990; 142(5):1200-5.

Lymphangioleiomyomatosis 1. Aberle DR, Hansell CM, Brown K, Tashkin DP. Lymphangiomyomatosis: CT, chest radiographic, and functional correlations. Radiology 1990; 176(2):381-7.

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2.

3. 4. 5.

6.

Chu SC, Horiba K, Usuki J, Avila NA, Chen CC, Travis WD, Ferrans VJ, Moss J. Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis. Chest 1999; 115(4):1041 -52. Corrin B, Liebow PA, Friedman Pd. Pulmonary lymphangiomyomatosis. A review. Am J Pathol 1975; 79(2):348-82. Lenoir 5, Grenier P, Brauner MW, Frija J, Remy-Jardin M, Revel D, Cordier J. Pulmonary lymphangiomyomatosis and tuberous sclerosis: Comparison of radiographic and thin-section CT findings. Radiology 1990; 175:329-334. Muller NE, Chiles C, Kullnig P. Pulmonary lymphangiomyomatosis: correlation of CT with radiographic and functional findings. Radiology 1990; 175(2):335-9. Sullivan Ed. Lymphangioleiomyomatosis a review. Chest 1998; 114(6):1689-703.

Chest Radiology

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Airways Disease

Inhalational Lung Disease (Asbestosis and Silicosis) Jeffrey R. Galvin, MD Pneumonokoniosis • •

“It will then be necessary to embrace under a single title all essentially identical forms of disease …the pneumonokoniosis (from Konis, dust) recommends itself”

Zenker 1866 Hematite Mining

Figure 1-4-1

Inorganic Dusts • • • • •

Silica Asbestos Coal Iron Beryllium

• • • • • •

Dust macules Diffuse interstitial fibrosis Diffuse alveolar damage Alveolar proteinosis Giant cell (GIP) Granulomatous inflammation

Pneumoconiosis The accumulation of dust in the lungs and the tissue reaction to its presence

Types and Sizes of Common Aerosols

Particle Deposition Inertial impaction, sedimentation and diffusion [Figure 1-

4-1]

• • • •

10,000-20,000 liters/day Deposition related to particle size >10 microns deposit in nasopharynx and large airways (100%) 1-5 micron particles deposit in lung parenchyma (20%)

Airway Velocity Inertial impaction, sedimentation and diffusion

Particles less that 5 microns can be deposited beyond the conducting airways in the alveolar spaces.

Figure 1-4-2

Particle Clearance [Figure 1-4-2] Cough, tracheobronchial and alveolar transport •

• • •

Most important ➢ Deposition less critical Mucociliary escalator ➢ Outer gel, inner liquid sol 90% of particles removed within 2 hrs Alveolar transport ➢ Dissolution, engulfed by macrophages, removed to lymphatics

Early Basal Deposition

Inhalational Lung Disease

Macrophages remove small particles to regional lymph nodes.

44

Chest Radiology

Removal to Lymph Nodes

Physiologic Gradients-Airflow FRC [Figure 1-4-3] Physiologic Gradients-Airflow TLC [Figure 1-4-4] Figure 1-4-3

Alveoli in the bases are smaller than those in the apex.

Physiologic Gradients-Blood Flow [Figure 1-4-5]

Figure 1-4-4

The smaller alveoli in the bases enlarge to a greater degree than those in the apex. Therefore most airflow is directed towards the bases

Physiologic Gradients-Lymphatic Flow [Figure 1-4-6] Figure 1-4-5

There is increased blood flow and hydrostatic pressure in the dependent vessels

Figure 1-4-6

The lymphatics are driven by hydrostatic pressure. Therefore lymphatic flow is best in the dependent lung.

Figure 1-4-7

Removal to Lymph Nodes [Figure 1-4-7]

This explains the tendency for chronic diseases to be upper lobe Chest Radiology

45

Inhalational Lung Disease

Tuberculosis

Figure 1-4-8

Silicosis Mineralogy •

• •

Silicon ➢ Element Silica (SiO2) ➢ Mineral Silicone ➢ Synthetic polymer

Figure 1-4-9

Adenopathy with peripheral calcification is a hallmark of silicosis

Figure 1-4-10

Nodules with an upper lobe predominance is typical

Figure 1-4-11

Silicoproteinosis is an acute lower lobe process

Silicosis predisposes a patient to having active tuberculosis

Inhalational Lung Disease

46

Chest Radiology

Silicosis Epidemiology

Figure 1-4-12

• • • •

Occupational exposure predominates ➢ 3 million workers Mining, stonecutting, engraving and foundry work Males more commonly affected Degree of exposure underestimated Increased risk of neoplasia and scleroderma

• • • • •

5 million particles/cubic foot-lower threshold 100 million particles/cubic foot-100% affected > 5 micron particle removed in nares and upper airways 80% of particles removed in hours to days Retained particles consistently .5-.7 microns

• • • •

Macrophages and polys concentrate Macrophages generate oxygen-free radicals Macrophages generate fibrogenic proteins Immune related tissue damage ➢ Rheumatoid factor, ANA and gamma globulin

•

Silicosis Pathogenesis

Silicosis Pathogenesis

The silicotic nodule is typical response to inhaled silica

Silicosis [Figures 1-4-8 to 1-4-11] Clinical manifestations •

• • •

•

Diagnosis ➢ Typical imaging pattern of adenopathy and nodules ➢ Exposure to high concentration of silica ➢ 10-20 years of exposure Simple silicosis ➢ Asymptomatic Symptoms with PMF Intense exposure ➢ Silicoproteinosis TB and cancer

Figure 1-4-13

Simple Silicosis Pathology [Figure 1-4-12] •

Progress to mature nodules: 3 zones ➢ Central dense fibrosis ➢ Mid-zone concentric collagen ➢ Peripheral dust laden cells

Simple Silicosis Imaging manifestations [Figure 1-4-13] • • •

Adenopathy common Calcification 10-20% Calcification 5-10% ➢ Eggshell pattern

Simple Silicosis Imaging manifestations •

• •

Well-circumscribed nodules ➢ 1-10 mm Upper lobe and posterior ➢ Lymphatic gradient ➢ CT more sensitive Pleural lesions ➢ Candle-wax or pseudoplaques

Chest Radiology

Eggshell calcification 47

Inhalational Lung Disease

Progressive Massive Fibrosis Pathology • • • •

Figure 1-4-14

Conglomeration of nodular lesions Pathology definition ➢ 2 cm Radiology definition ➢ 1 cm Upper lung zones ➢ Posterior

Progressive Massive Fibrosis Imaging manifestations [Figure 1-4-14] • •

• • •

Progression after exposure May fill entire upper lobe ➢ Posterior Usually bilateral Carcinoma mimic ➢ Solitary ➢ Lower lobe Associated emphysema ➢ Not always smoking related ➢ Scar emphysema

Progressive massive fibrosis

Figure 1-4-15

Silicotic Alveolar Proteinosis Pathology [Figure 1-4-15] • • • • •

High concentration of particulate silica Acute onset ➢ Weeks-months Alveoli filled with PAS+ material Similar to surfactant Type II cell hyperplasia

Silicosis and Tuberculosis [Figure 1-4-16] Silicosis Computed tomographic technique

• • •

Thick sections of value in nodular diseases ➢ Small nodules easier to differentiate from vessels Thin sections 1-2 mm collimation at 10 mm intervals or 3-5 selected images with prior thick section CT High spatial frequency algorithm Supine No contrast

• • • • • •

Adenopathy Nodules PMF Silicoproteinosis Tuberculosis Cancer

•

•

Crazy paving pattern associated with alveolar proteinosis

Figure 1-4-16

Silica and Lung Disease

Tuberculosis in a patient with silicosis Inhalational Lung Disease

48

Chest Radiology

Asbestos Introduction •

• • • •

Group of naturally occurring mineral fibers ➢ Hydrated fibrous silicate Flexible and strong Corrosion, thermal and electrical resistance 500 tons - 3 million tons ➢ 60 years 9 million people exposed ➢ Primary (mining) ➢ Secondary (industrial) ➢ Nonoccupational (air)

Figure 1-4-17

Figure 1-4-18

Nonoccupational Exposure

Asbestos Bodies [Figures 1-4-17 and 1-4-18] • • • • •

• •

Indicates exposure Transparent fiber core Iron and mucopolysaccharide coat Predominantly amphiboles Longer fibers are coated ➢ < 20 microns not coated ➢ Uncoated fibers are pathogenic ➢ 7-5000 X’s more uncoated fibers Fibers cannot be removed Lower posterior disease

Asbestos Serpentine: chrysotile • • • • •

•

Asbestos bodies are commonly found in urban dwellers

Figure 1-4-19

95% of commercial use Curly and pliable Textile manufacture Fragments easily Chemically unstable ➢ Dissolves easily Less pathogenic

Asbestos fibers are much larger than macrophages and therefore cannot be removed to regional lymph nodes

Figure 1-4-20

Asbestos Amphiboles: amosite, crocidolite, anthophilite, tremolite and actinolite • • • • • •

5% of commercial use Straight, broad fiber Do not fragment easily Long fibers (>20 microns) ➢ Not cleared More likely coated Higher carcinogenic potential

Pleural effusions are the most common early complication of asbestos exposure

Figure 1-4-21

Asbestos Related Chest Disease [Figures 1-4-19 to 1-4-21]

• • • •

• • •

Pleural effusions Pleural plaques Round atelectsis Pleural thickening ➢ Diffuse Mesothelioma Asbestosis Lung cancer

Chest Radiology

Rounded atelectasis is usually preceded by a pleural effusion

Asbestosis is a lower lobe subpleural process 49

Inhalational Lung Disease

Pleural Plaques Pathology •

• • • • •

Figure 1-4-22

Common autopsy finding ➢ (50-10%) Dense bands of collagen ➢ “Basket weave” Asbestos bodies absent Uncoated fibers in dissolved lung tissue Dose response ➢ Between parenchymal asbestos bodies and presence of plaques Pathogenesis uncertain

Pleural Plaques • • •

• • • • •

Postero-lateral parietal pleura Central diaphragm Absent ➢ Apices and costophrenic angles Almost always bilateral Sharply demarcated Millimeters to 10 cm May calcify extensively Highly suggestive of asbestos exposure

The visceral pleural stripe is best seen between the ribs

Figure 1-4-23

Roberts, AJCP 1971

Pleural Anatomy [Figure 1-4-22] Pleural Plaques Imaging •

• •

Radiography insensitive ➢ (8-40% of autopsy cases) Companion shadows ➢ Fat and muscle HRCT ➢ Best sensitivity and specificity

Pleural Fat [Figure 1-4-23]

Pleural Plaques [Figure 1-4-24]

Fat may mimic fibrotic pleural disease

Diffuse Pleural Thickening •

• • • •

•

Smooth pleural density ➢ CXR: > 25% of the length of the chest wall ➢ CT: 3 mm thick, 8 cm wide, 5 cm craniocaudal Posteromedial lower lobes Involves costophrenic angle Mediastinal pleural involvement ➢ Rare ➢ Suggests mesothelioma Visceral and parietal pleura ➢ Adhesions Sequela of prior effusion?

Figure 1-4-24

Pleural Effusion Definition • •

• •

History of exposure to asbestos Confirmation of effusion ➢ Imaging of thoracentesis Absence of other disease related to effusion Absence of malignant tumor for 3 years

Epler, JAMA 1982

Inhalational Lung Disease

Visceral pleural plaques 50

Chest Radiology

Pleural Effusion Clinical presentation

Figure 1-4-25

• • • • •

Most common abnormality ➢ First 20 yrs 3% prevalence ➢ Asbestos exposed Small < 500 ml Serosanguinous Persist for weeks to 6 months 66% asymptomatic 28% recur

• • • •

Lung cancer Tuberculosis Benign asbestos effusion Mesothelioma

• • • •

•

Described 1928 Loeschke Usually asymptomatic Folded lung vs inflammatory reaction Associated conditions ➢ Asbestos exposure, CHF, infarct, TB and histoplasmosis Preceded by effusion

• • • •

Irregular fibrous thickening of the visceral pleura Extensive pleural folding beneath the fibrosis Layers of invaginated pleura bound by fibrous adhesions Surrounding lung collapsed or fibrotic

•

•

Pleural Effusion Differential diagnosis

Round Atelectasis

Round atelectasis is associated with pleural effusion

Round Atelectasis Histology [Figure 1-4-25]

Menzies, AJSP 1987

Figure 1-4-26

Round Atelectasis Imaging criteria [Figure 1-4-26] • • • • •

Well-circumscribed Round or oval opacity “Comet tail” sign Pleural thickening Volume loss

•

Interstitial fibrosis ➢ Associated with asbestos bodies Biopsy ➢ Not the standard of practice

Asbestosis Pathologic definition •

Asbestosis • • • • •

Dose-response relationship Probable exposure threshold Latency period inversely proportional to exposure level Latency is several decades Cigarette smoke may act synergistically

Chest Radiology

51

Round atelectasis

Inhalational Lung Disease

Asbestosis ATS criteria 1986 • • •

• •

Reliable history of exposure Latency of at least 10 years Restrictive pulmonary functions ➢ DLco and VC <80% Appropriate physical findings ➢ Inspiratory crackles, clubbing Chest radiographic abnormalities ➢ ILO perfusion > 1/0 (s, t or u)

Asbestosis Histology •

• •

Early ➢ Fibrosis of respiratory bronchioles Progression ➢ Terminal bronchioles, alveolar ducts and alveolar septa Minimum 2 asbestos bodies in area of fibrosis

Craighead, Arch Pathol Lab Med, 1982

Asbestosis Chest radiography •

• •

Lower lobe ➢ Irregular opacities ➢ Nonspecific ➢ Associated pleural disease Large inter-observer variation ➢ Low perfusion Normal in 26% of path proven cases

Asbestosis and Cigarette Smoking Small irregular opacities •

• •

•

Small opacities are related to ➢ Dust exposure, cigarette smoke, age, radiographic technique and obesity Cigarette smoke causes ➢ Interstitial fibrosis ➢ Emphysema ➢ Bronchiolar thickening Asbestos causes ➢ Interstitial fibrosis Asbestos workers who smoke ➢ Have more opacities ➢ Related to dust exposure and cigarettes

Asbestosis High-resolution CT •

• • • •

Figure 1-4-27

Lower lobe and posterior ➢ Reticulonodular opacities ➢ Parenchymal bands ➢ Curvilinear subpleural line ➢ Interstitial short lines ➢ Honeycombing High sensitivity Nonspecific Specificity increases with # of abnormalities Prone imaging is key!

Parenchymal Bands [Figure 1-4-27]

Inhalational Lung Disease

Parenchymal bands in a patient with asbestosis 52

Chest Radiology

Reticulonodular Opacites

Curvilinear Subpleural Line Short Lines

Honeycombing

Asbestosis vs UIP • • • • •

Asbestos exposure in the last 30 years is low Clinical asbestosis requires substantial exposure Asbestos exposed individuals can have other interstitial lung diseases Band like opacities merging with the pleura are rare in UIP Upper zone fibrosis and ground glass are rare in asbestosis

Gaensler, ARRD, 1991 – Al-Jarad, Thorax, 1992

Asbestosis High-resolution CT • • • • •

Short lines and parenchymal bands are statistically most significant Strong association with diffuse pleural disease Multifocal HRCT finds asbestosis in exposed individuals with normal radiographs and PFT’s Obstructive PFT’s correlate with emphysema

Aberle, AJR, 1988 – Aberle, Radiology, 1988 – Staples, ARRD, 1989

Asbestosis Dependent density •

•

Posterior blood flow ➢ 5X’s greater Posterior alveoli ➢ Smaller or collapsed ➢ Less steep ventilatory gradient ➢ Closing volumes (10-40% of VC)

Asbestosis Computed tomographic technique

• • • • •

1.5-2 mm collimation 10 mm interval High spatial frequency algorithm Prone Thick section supine: CA screen?

Asbestos Related Chest Disease Tuberculosis Silicosis

Asbestosis

Particle Deposition and Clearance Cough, tracheobronchial and alveolar transport

Chest Radiology

53

Inhalational Lung Disease

Pulmonary Lymphoid Disorders Jeffrey R. Galvin, MD

The Pulmonary Lymphoid System • • •

• • • •

Lymphatics Lymph nodes BALT ➢ Bronchus Associated Lymphoid Tissue Lymphoid aggregates Lymphocytes Dendritic cells Langerhans cells

Figure 1-5-1

Figure 1-5-2

The Pulmonary Lymphoid System [Figures 1-5-1 and 1-5-2]

•

• • • • • •

Lymphatics ➢ Originate in the pleura ➢ Valves ➢ Drain towards hilum ➢ Follow interlobular septa ➢ Accompany blood vessels Lymph Nodes BALT Lymphoid aggregates Lymphocytes Dendritic cells Langerhans cells

The Pulmonary Lymphoid System [Figures 1-5-3 and 1-5-4]

• •

• • • • •

One set of lymphatics originate in the Lymphatic channels continue along the visceral pleura as demonstrated by the pulmonary veins to the hilum. A second lines on the surface of the lung. These set of lymphatics originates near the lymphatics enter the lung and follow the center of the secondary lobule and interloblular septa in the periphery of the follows the pulmonary arteries lung

Lymphatics Lymph Nodes ➢ Encapsulated lymph nodes ✧ Proximal bronchi ✧ Bifurcations ➢ Reactive lymph nodes ✧ Peripheral and septal ✧ Cigarettes or dust BALT Lymphoid aggregates Lymphocytes Dendritic cells Langerhans cells

Figure 1-5-3

Figure 1-5-4

Reactive Lymph Nodes

The Pulmonary Lymphoid System • • •

• • • •

Lymphatics Lymph nodes BALT ➢ Bronchus Associated Lymphoid Tissue Lymphoid aggregates Lymphocytes Dendritic cells Langerhans cells

Pulmonary Lymphoid Disorders

Classic encapsulated intrapulmonary lymph nodes are found at the bifurcations of the first 3-4 orders of bronchi and are demonstrated adhering to the right main pulmonary 54

The majority of intrapulmonary lymph nodes are probably not visible radiographically. Almost all of these lymph nodes are subpleural and inferior to the carina

Chest Radiology

BALT – The organizing principle [Figure 1-5-5] •

• • •

Figure 1-5-5

Lymphoid collections ➢ Bronchial epithelium ➢ Bifurcations Absent in the normal adult ➢ Absent at birth ➢ Common in young children Reappears with antigenic stimulation ➢ Cigarette smoke ➢ Connective tissue disease ➢ AIDS Basis of pulmonary lymphoid disorders

Pulmonary Lymphoid Disorders – Derivations of BALT •

• •

Hyperplasias of BALT ➢ Follicular hyperplasia ✧ Follicular bronchitis ➢ Diffuse hyperplasia ✧ Lymphoid Interstitial Pneumonia ➢ Nodular Lymphoid Hyperplasia ✧ Pseudolymphoma Non-Hodgkin’s lymphomas ➢ Low-Grade B Cell lymphomas ➢ Lymphomatoid granulomatois Immune impairment ➢ PTLD, AIDS and other

Bronchus associated lymphoid tissue or BALT is found along the bronchiole, interlobular septa and pleura. It is normally found only in young children.

Koss, Sem Diag Pathol 1995

Follicular Hyperplasias of BALT – Hyperplasia of BALT [Figure 1-5-6]

• •

•

Follicular bronchitis and bronchiolitis Pathologic features ➢ Antigenic stimulation of BALT ➢ Lymphoid aggregates ➢ Peribronchial ➢ Reactive follicles ➢ Minimal alveolar extension Clinical

Figure 1-5-6

Follicular Hyperplasias of BALT – Hyperplasia of BALT • •

Pathologic features Clinical ➢ Young adults (44 yrs.) ➢ Cough and dyspnea ✧ Fever and weight loss ➢ Immune deficiencies ✧ AIDS ✧ Congenital ➢ Collagen vascular diseases ✧ Sjogren’s ✧ Rheumatoid arthritis ➢ Uncertain Etiology ✧ Hypersensitivity reactions?

Chest Radiology

Follicular bronchitis is characterized by hyperplastic lymphoid follicles with reactive germinal centers distributed along bronchioles and to a lesser extent bronchi.

55

Pulmonary Lymphoid Disorders

Follicular Hyperplasias of BALT – Hyperplasia of BALT: Imaging [Figure 1-5-7]

•

•

Radiography ➢ Diffuse ➢ Reticulonodular CT ➢ Nodules 3-12 mm ✧ Centrilobular ✧ Peribronchial ➢ Ground Glass ➢ Air Trapping

Follicular Hyperplasia – Differential Diagnosis • • • • •

Respiratory Bronchiolitis Hypersensitivity pneumonitis Diffuse panbronchiolitis Cystic Fibrosis Primary ciliary dyskinesia

Diffuse Hyperplasias of BALT – Hyperplasia of BALT [Figure 1-5-8]

• •

•

Lymphocytic Interstitial Pneumonia Pathologic Features ➢ Alveolar septal infiltration ✧ Lymphocytes (T-cells) ✧ Diffuse ➢ Lymphoid follicles (B-cells) ✧ Germinal centers ✧ Peribronchial distribution ✧ Spectrum with follicular hyperplasia of BALT (Follicular Bronchitis) Clinical

Figure 1-5-7

Almost all patients with follicular bronchitis have centrilobular nodes that are less than 3mm. These nodules correlate with the peribronchiolar distribution of hyperplastic lymphoid follicles shown on the histology section to your left

Figure 1-5-8

Diffuse Hyperplasias of BALT – Lymphoid Interstitial Pneumonia LIP- Hyperplasia of BALT • •

Pathologic features Clinical ➢ Women>men ➢ 4th-6th decade ➢ Cough and dyspnea ➢ Collagen vascular disease ✧ Sjogrens, RA, and SLE ➢ Bone marrow transplantation ➢ AIDS rare in adults ✧ Common in children ➢ Dysproteinemia ➢ Restrictive lung functions

Pulmonary Lymphoid Disorders

LIP is characterized by diffuse infiltration of the alveolar septa

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Chest Radiology

Diffuse Hyperplasia of BALT – Hyperplasia of BALT: Imaging

Figure 1-5-9

[Figures 1-5-9 and 1-5-10]

•

•

Radiography ➢ Lower lung zone ➢ Reticulonodular CT ➢ Ground Glass ➢ Nodules ✧ Centrilobular ✧ Poorly defined ➢ Cystic air spaces ➢ Thickened BVB’s ➢ Adenopathy

LIP vs Lymphoma LIP Cysts 82% Consolidation 18% Large Nodules 6% Effusions 0%

The lung windows demonstrate diffuse hazy ground glass that correlates with diffuse alveolar wall thickening. The alveolar wall thickening is primarily the result of lymphoid infiltation

Lymphoma 2% 66% 41% 25%

Figure 1-5-10

Nodular Lymphoid Hyperplasia – Hyperplasia of BALT [Figure 1-5-11]

• •

•

Pseudolymphoma Pathologic Features ➢ Solitary, subpleural mass ➢ Lymphoid proliferation ✧ Interstitial ✧ Perivascular ✧ B and T cells ✧ Polyclonal pattern – Benign ➢ Reactive germinal centers ➢ Difficult to separate from lymphoma Clinical

Nodular Lymphoid Hyperplasia – Hyperplasia of BALT • • •

Pseudolymphoma Pathologic features Clinical ➢ Rare entity ➢ Most cases were lymphomas ✧ Monoclonal B cell proliferation ➢ Middle age ✧ Asymptomatic ➢ Autoimmune Diseases 15% ✧ Sjorgren’s ✧ SLE ✧ Transverse myelitis ➢ Surgical excision curative

Chest Radiology

Thin walled cysts are often found deep within the lung parenchyma. Previous reports have suggested that airway narrowing or obliteration results in these cystic lesions. The histology on the left demonstrates complete obliteration of the bronchiole by lymphoid infiltration. The accompanying arteriole is identified by its typical wall.

Figure 1-5-11

Nodular lymphoid hyperplasia or pseudolymphoma presents as a solitary subpleural mass of lymphoid tissue with numerous reactive germinal centers

57

Pulmonary Lymphoid Disorders

Nodular Lymphoid Hyperplasia Hyperplasia of BALT: Imaging

Figure 1-5-12

[Figures 1-5-12 and 1-5-13]

•

•

Radiography ➢ Solitary Nodule ➢ Focal Consolidation CT ➢ Air bronchograms ✧ 100% ➢ Indistinct margins ➢ Occasionally multiple ➢ Adenopathy and/or effusion suggests lymphoma

Pulmonary Lymphoid Disorders Derivations of BALT • •

•

Hyperplasias of BALT Non-Hodgkin’s lymphomas ➢ Low-Grade B Cell lymphomas ➢ Lymphomatoid granulomatosis Immune impairment

Figure 1-5-13

Low-Grade B-Cell Lymphoma •

Pathologic features ➢ Lymphocytic infiltration ➢ Small lymphocytes ✧ Alveolar wall ✧ Peribronchiolar ✧ Perivascular ➢ Immunologic evidence of malignancy ✧ Monoclonality ✧ B-cell markers CD20 ➢ Germinal Centers

Low-Grade B-Cell Lymphoma •

The CT demonstrates the typical subpleural, solitary lesion with indistinct margins. The bulk of the lesion consists of a mass of lymphoid tissue with multiple reactive germinal centers.

Clinical ➢ Similar presentation to nodular lymphoid hyperplasia ➢ 5th-6th decade ➢ Male=Female ➢ Asymptomatic 50% ➢ 5 year survival 85-95% ➢ Surgical resection ✧ Rare recurrence

Air bronchograms are universally present and the lymphoid infiltration gradually diminishes resulting in the classical indistinct margin

Figure 1-5-14

Low-Grade B-Cell Lymphoma [Figure 1-5-14

•

Imaging ➢ Radiography ✧ Solitary nodule/mass – Multiple ✧ Consolidation ✧ Air bronchogram – 50% ✧ Slow Growth ➢ CT ✧ Consolidation ✧ Air bronchograms ✧ Airway narrowing or “stretching”

Pulmonary Lymphoid Disorders

Grossly low grade B-cell lymphoma usually presents as a single white tan lesion that can be either well circumscribed or indistinct. This is well demonstrated by the gross specimen on the left from the AFIP archive. The disease can, however, be multifocal as shown on the right and has been reported as a primarily endobronchial lesion. 58

Chest Radiology

Primary Tracheal Lymphoma • • • •

Figure 1-5-15

Extremely rare BALT derivative Extensive at diagnosis Potentially curable

Lymphomatoid Granulomatosis [Figure 1-5-15]

•

Pathologic features ➢ Majority of cases are B-cell lymphomas ➢ Reactive small T-cells ➢ Malignant B-cells ✧ Majority of infiltrate ➢ Epstein-Barr Virus ➢ Angiocentric infiltration ➢ Necrosis ✧ Peribronchovascular ✧ Peripheral

Lymphomatoid Granulomatosis •

Clinical ➢ 7-85 years (mean 48 yrs) ➢ Male:Female (2:1) ➢ Malaise and weight loss ➢ Lung involvement 100% ➢ Cough and dyspnea ➢ Skin 39-53% ✧ Nodules, ulcers and rash ➢ CNS 37-53% ➢ Renal 32-40% ➢ High mortality rate 53-90% ➢ Most proceed to lymphoma

Lymphomatoid Granulomatosis is an angiocentric B-cell lymphoma which often demonstrates areas of necrosis.

Figure 1-5-16

Lymphomatoid Granulomatosis •

Imaging ➢ Nodules 80% ✧ Multiple ✧ Bilateral (80%) ➢ Mid and lower lobes ➢ Cavitation 20% ➢ Large masses ✧ Correspond to infarcts ➢ Diffuse reticulonodular opacities ➢ Hilar adenopathy 25%

Chest CT on the left demonstrates a bronchovascular distribution of nodules that are shown to be areas of infarction on gross examination.

LYG [Figure 1-5-16]

Pulmonary Lymphoid Disorders – Derivations of BALT • • •

Hyperplasias of BALT Non-Hodgkin’s lymphomas Immune impairment ➢ Posttransplantation Lymphoproliferative Disease (PTLD) ➢ AIDS ➢ Other forms of prolonged immune suppression

Chest Radiology

59

Pulmonary Lymphoid Disorders

Lymphoma and Immune Impairment [Figure 1-5-17] •

Pathologic Features ➢ B-cell non-Hodgkin’s lymphoma ✧ Driven by Epstein-Barr Virus infection ✧ Diffuse polyclonal expansion – Reduced T-cell control ✧ Malignant transformation

Figure 1-5-17

Lymphoma and Immune Impairment •

Clinical ➢ Spectrum of benign to malignant ✧ Infectious mono-like ✧ PTLD polymorphic ✧ PTLD monomorphic ➢ Cyclosporin shortens induction (<1 year) ➢ May respond to reduction in immunosuppression, anti-virals and surgery ➢ Chemotherapy should be avoided ➢ Heart-lung up 20%

Lymphoma and Immune Impairment •

Imaging ➢ Nodules ✧ May cavitate ✧ Halo ✧ Along bronchovascular bundles ➢ Lymph node ➢ Ground glass ➢ Septa thickening ➢ Consolidation ➢ Effusion

Post transplant lymphomas are driven by EpsteinBarr virus, reduced T-cell surveillance and malignant transformation. Genetic mutation may eventually result in malignant transformation of one of these clones, represented in purple.

PTLD

[Figure 1-15-18]

Bone Marrow Transplant

Prolonged Chemotherapy

Figure 1-5-18

Pulmonary Lymphoid Disorders Derivations of BALT • • •

Hyperplasias of BALT Non-Hodgkin lymphomas Immune impairment ➢ Posttransplantation Lymphoproliferative Disease PTLD ➢ AIDS ➢ Other

58 year old male with alpha 1 antitrypsin deficiency who underwent a double lung transplant 7 years prior to presenting with shortness of breath, cough, fever and chills for 2 months. The CT reveals multiple multiple indistinct nodules in a characteristic distribution along the bronchovascular bundles Pulmonary Lymphoid Disorders

60

Chest Radiology

Follicular Hyperplasia [Figure 1-5-19]

Diffuse Hyperplasia of BALT-LIP [Figure 1-5-20]

Figure 1-5-19

Nodular Lymphoid Hyperplasia [Figure 1-5-21] Low-Grade B-Cell Lymphoma [Figure 1-5-22] LYG [Figure 1-5-23]

Figure 1-5-20

Follicular Bronchitis.

Figure 1-5-21

Lymphocytic Interstitial Pneumonia (LIP).

Figure 1-5-22

Nodular Lymphoid Hyperplasia which was formerly known as Pseudolymphoma.

Figure 1-5-23

Low grade B-cell Lymphoma

Lymphomatoid Granulomatosis

Chest Radiology

61

Pulmonary Lymphoid Disorders

Immune Impairment-PTLD [Figure 1-5-24]

Figure 1-5-24

BALT - The organizing principle • •

Lymphoid collections Basis of pulmonary lymphoid disorders

Post Transplant Lymphoproliferative Disorder

References

General 1. Koss MN. Pulmonary lymphoid disorders. Semin Diagn Pathol. 1995 May;12(2):158-71. 2. Travis WD, Galvin JR.Non-neoplastic pulmonary lymphoid lesions. Thorax. 2001 Dec;56(12):964-71.

Pulmonary Lymphoid Disorders

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Chest Radiology

Angiitis and Granulomatosis Jeffrey R. Galvin, MD

Angiitis and Granulomatosis • • •

•

First characterized by Averill Liebow 1973 Unknown etiology Angiitis ➢ Cellular infiltration of blood vessel Granulomatosis ➢ Necrosis of lung parenchyma not related to blood vessel occlusion

Angiitis and Granulomatosis: Current List • • •

Wegener’s granulomatosis Churg-Strauss syndrome ➢ Allergic granulomatosis Necrotizing sarcoid granulomatosis Bronchocentric granulomatosis Lymphomatoid granulomatosis

• • • • •

Etiology remains unknown Inflammatory vs. lymphoproliferative Clinical and laboratory findings key to Dx Adequate tissue samples are important Must R/O infection: mycobacterial or fungal

• •

Angiitis and Granulomatosis: General Concepts

Pathogenesis of Vasculitis

Angiitis and Granulomatosis: Differential Multiple vessel associated nodules [Figure 1-6-1] •

• • • • •

Metastatic disease ➢ Squamous Multifocal infection ➢ Fungus, TB, bacteria Septic emboli Multiple pulmonary infarcts Langerhans’ cell histiocytosis Rheumatoid nodules

Figure 1-6-1

Wegener’s Granulomatosis: Classic Pathology Triad •

•

• •

•

Vasculitis described 1852 ➢ Von Rokitansky Wegener described 1936 ➢ Wegener’s Focal vasculitis of ➢ Arteries and veins Necrotising granulomas ➢ Upper and lower airways Necrotising glomerulitis ➢ Focal

Vasculitis in the lung fits into the differential of vessel associated nodules Chest Radiology

63

Angiitis and Granulomatosis

Wegener’s Granulomatosis: Gross Pathology [Figures 1-6-2 and 1-6-3]

•

• • •

Necrotic nodules ➢ With and without cavitation Parenchymal consolidation Massive hemorrhage Airway narrowing

Figure 1-6-2

Wegener’s Granulomatosis: Demographics •

• • • •

Rare ➢ 3/100,000 in US 2nd-8th decades of life Average age-50 years Male=Female ➢ Slight male predominance (4:3) May occur in children

Wegener’s Granulomatosis: Limited • •

• •

Involvement of lungs alone Clinical sparing ➢ Kidneys ➢ Upper respiratory tract Biopsy positive ➢ When clinically normal Better prognosis

Wegener’s Granulomatosis: Clinical Presentation •

• •

•

Classic triad ➢ Sinusitis ➢ Pulmonary symptoms ➢ Renal insufficiency Variable onset and course Chronic URI symptoms ➢ May persist for years before pulmonary disease Overwhelming vasculitis ➢ Diffuse

Solid and cavitary nodules often coexist in patients with Wegener’s granulomatosis

Figure 1-6-3

Upper Airway •

• • • • • •

Chronic nasal obstruction ➢ Chronic discharge Destruction of cartilaginous nasal septum “Saddle nose deformity” Laryngeal involvement ➢ Subglottic stricture Eustachian tube obstruction Otitis media Cochlear nerve vasculitis

Pulmonary • • • •

• •

Most commonly affected (94%) Multiple bilateral nodules or masses Cavitation common (30-50%) Occasionally solitary mass or nodule ➢ Dx difficult ➢ All patients progress Less common ➢ Diffuse alveolar hemorrhage Pleural lesion and effusions are rare

Angiitis and Granulomatosis

Airway narrowing is a common complication

64

Chest Radiology

Renal

Figure 1-6-4

• • • •

Tempo: insidious to explosive Segmental necrotizing glomerulonephritis UA: erythrocyte casts and proteinuria Large vessel vasculitis

•

Skin (50%) ➢ Symmetric papulonecrotic lesion of extremities Eye and orbit (30%) ➢ Scleritis, conjunctivitis, optic nerve and retro-orbital mass Nervous system (30%) ➢ Mononeuritis multiplex Joints ➢ Acute arthritis follows activity of disease (+RA latex)

Wegener’s Granulomatosis: Other Organ Involvement • •

•

Wegener’s Granulomatosis: Airway Involvement [Figures 1-6-4 and 1-6-5]

•

• • • •

•

Endobronchial abnormalities ➢ 59% bronchoscopy Subglottic stenosis Tracheobronchitis ➢ Ulcerating Tracheal or bronchial stenosis Often multifocal ➢ Variable length of involvement CT key for evaluation ➢ CXR often normal

Focal airway narrowing is a common complication in Wegener’s

Wegener’s Granulomatosis: Radiography •

• • • •

•

Earliest lesions ➢ Bilateral reticulo-nodular opacities Multifocal nodules ➢ Bilateral ➢ 5mm-10cm Sharply marginated Cavitation 20-50% Evolution ➢ Thick walls to thin walled cysts with treatment Airspace consolidation

Figure 1-6-5

Changing Presentation

Necrosis and Hemorrhage [Figure 1-6-6] Figure 1-6-6

Collapse due to airway narrowing in Wegener’s

Massive necrosis and hemorrhage in Wegener’s Chest Radiology

65

Angiitis and Granulomatosis

Evolution with Treatment

[Figures 1-6-7 and 1-6-8]

Figure 1-6-8

Figure 1-6-7

Infections are a common complication

Figure 1-6-9

Nodules in varying stages

Wegener’s Granulomatosis: Computed Tomography [Figure 1-6-9] •

•

• • •

•

Feeding vessels ➢ 88% Cavitation ➢ Nodules greater than 2cm Subpleural location ➢ Predominant CT “halo sign” Pleural based lesions ➢ Mimic infarcts Reveals more nodules

Diffuse Pulmonary Hemorrhage: Capillaritis [Figure 1-6-10]

•

•

Common ➢ Microscopic polyangiitis ➢ Wegener’s granulomatosis ➢ SLE Uncommon ➢ Goodpastures ✧ Anti-GBM ➢ Collagen vascular ➢ Idiopathic pulmonary hemorrhage ➢ Churg Strauss syndrome ➢ Behcet’s syndrome ➢ IgA Nephropathy

Figure 1-6-10

Nodules with feeding vessels are common in vasculitis

Pulmonary hemorrhage in capillaritis Angiitis and Granulomatosis

66

Chest Radiology

Microscopic Polyangiitis [Figure 1-6-11]

• • • • •

Microscopic polyarteritis nodosa Most common cause of pulmonary-renal syndrome 5th decade Male > Female Renal, muskuloskeletal, pulmonary, GI and cutaneous

Microscopic polyangiitis

Wegener’s Granulomatosis: Laboratory • • •

Figure 1-6-11

ANCA ➢ Serum Antineutrophil Cytoplasmic Autoantibody c-ANCA cytoplasmic pattern ➢ Proteinase 3 ➢ 99% specificity and 96% sensitivity in active disease ➢ Positivity drops to 30% in remission p-ANCA perinuclear pattern ➢ Reacts with myeloperoxidase ➢ positive in collagen vascular diseases

Wegener’s Granulomatosis: Treatment and Prognosis • • •

• • •

Universally fatal without treatment Trimethoprim/Sulfa effective in localized disease Steroids and cyclophosphamide ➢ Remission in 93% 5 year survival 90-95% Infectious complications ➢ Relapse and drug toxicity require close monitoring and follow-up imaging Relapse has different manifestations from presentation

Churg-Strauss Syndrome: Allergic Angiitis and Granulomatosis • • • •

Described by Churg and Strauss ➢ 1951 True systemic vasculitis Associated ➢ Asthma ➢ Allergic rhinitis ➢ Blood eosinophilia Hypersensitivity response to inhaled antigen?

Chest Radiology

67

Angiitis and Granulomatosis

Churg-Strauss Syndrome: Pathology • • •

Necrotizing vasculitis Eosinophilic tissue infiltration “Allergic granulomas” ➢ Extravascular ➢ Eosinophils ➢ Multinucleated giant cells

Churg-Strauss Syndrome: Demographics • • • •

2nd-4th decades 28 years mean age of onset Male=Female Excellent response to steroids

•

Late onset asthma ➢ 100% Precedes CSS by weeks to years (30) Severe rhinitis and sinusitis ➢ 70%

Churg-Strauss Syndrome: Background • •

Churg-Strauss Syndrome: Prodromal Stage • • • • • • • •

Infiltration of tissues with eosinophils Blood eosinophilia Elevated IgE + rheumatoid factor Progressive asthma, sinus pain, myocardial involvement Loffler’s like fleeting infiltrates Abdominal pain ➢ Diarrhea and eosinophilic peritonitis Myalgias and neuritis

Churg-Strauss Syndrome: Vasculitic Stage • •

•

•

Increasingly severe and widespread symptoms Lung ➢ Eosinophilic consolidation, miliary to 2 cm nodules (without cavitation), and diffuse hemorrhage Cardiac ➢ Coronary vasculitis and eosinophilic myocarditis (50% of mortality) GI ➢ Ulcerations, perforations and peritonitis

Churg-Strauss Syndrome: Computed Tomography •

• • •

•

Parenchymal opacification ➢ Predominantly peripheral 59% ➢ Effusions Nodules ➢ 12% Bronchial thickening Dilatation ➢ 12% Interlobular septal thickening ➢ 6%

Worthy et. Al. AJR Feb. 1998

Churg-Strauss Syndrome: Comparison with Wegener’s •

CSS ➢ High incidence of asthma ➢ High incidence of cardiac involvement (47%) ➢ Less severe renal and sinus disease ➢ Associated with P-ANCA

Angiitis and Granulomatosis

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Chest Radiology

Churg-Strauss Syndrome: Therapy and Prognosis • • • •

Prognosis relates to early diagnosis and therapy High dose steroids usually effective Cyclophosphamide in resistant cases Therapy stopped after 6-12 months of remission

•

A distinct entity? ➢ Katzenstein Some reported cases are undiagnosed infections Those with extrapulmonary involvement ➢ Sarcoidosis

Necrotizing Sarcoid Granulomatosis How is this related to sarcoidosis? • •

Necrotizing Sarcoid Granulomatosis: Demographics • • •

3rd to 7th decades Mean age 49 years Female:male ➢ 2.2:1

Figure 1-6-12

Necrotizing Sarcoid Granulomatosis: Pathology •

•

•

Non-caseating granulomas ➢ Similar to sarcoidosis Vasculitis ➢ Pulmonary arteries ➢ Pulmonary veins ➢ Found in areas away from parenchymal granulomas Coagulative necrosis ➢ Widespread ➢ Main distinction from sarcoidosis

Necrotizing Sarcoid Granulomatosis: Clinical Presentation • • • • •

• •

100% lung involvement Cough most common symptom Chest pain, fever and dyspnea Weight loss and fatigue May be asymptomatic ➢ 15-40% Rare extrapulmonary involvement ➢ 13% Aspergillus antigens in some patients

Koss et al, Human Pathology 1980

Necrotizing Sarcoid Granulomatosis: Imaging [Figure 1-6-12]

•

• •

Hilar adenopathy ➢ Variable ➢ Up to 79% Nodules ➢ Cavitation is common ➢ Subpleural ➢ Perivascular Parenchymal opacities ➢ Same distribution

Necrotizing Sarcoid Granulomatosis: Prognosis and Therapy • • •

May require no therapy Prompt response to steroids No reported deaths

Chest Radiology

Typical nodules in NSG

69

Angiitis and Granulomatosis

Lymphomatoid Granulomatosis: Etiology and Demographics [Figure 1-6-13]

• • •

• • •

•

Majority of cases are B-cell lymphomas Epstein-Barr Virus Reactive small T-cells ➢ Majority of infiltrate Malignant B Cells Age range ➢ 7-85 years Mean age of onset ➢ 48 years Male:Female (2:1)

Figure 1-6-13

Lymphomatoid Granulomatosis: Pathology • • • •

Angiocentric infiltration ➢ Mixed cell population ✧ Atypical lymphocytes, plasma cells, histiocytes Vascular invasion Vascular destruction Necrosis ➢ Peribronchovascular ➢ Peripheral

Lymphomatoid Granulomatosis: Clinical Presentation •

• •

• •

Lung involvement ➢ 100% ➢ Cough and dyspnea Skin ➢ 39-53% ➢ Nodules, ulcers and rash CNS ➢ 37-53% Renal ➢ 32-40% Malaise and weight loss ➢ 35%

LYG is a large B-cell lymphoma

Figure 1-6-14

Lymphomatoid Granulomatosis: Imaging [Figure 1-6-14]

•

• • • • •

Nodules ➢ 80% ➢ Multiple ➢ Bilateral (80%) Mid and lower lobes Cavitation ➢ 20% Large masses ➢ Correspond to infarcts Diffuse reticulonodular opacities Hilar adenopathy ➢ 25%

Lymphomatoid Granulomatosis: Treatment and Prognosis •

• •

Peripheral opacities in LYG

Mortality rate ➢ 53-90% Long term remissions reported ➢ Cyclophosphamide and steroids All who fail therapy proceed to develop lymphoma ➢ 12-47%

Angiitis and Granulomatosis

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Chest Radiology

Bronchocentric Granulomatosis Clinical and Demographics – Asthmatics • • • • •

Average age 22 years Tissue manifestation of ABPA Dyspnea, cough, fever, malaise and hemoptysis Peripheral and tissue eosinophilia No extrapulmonary findings

• • •

Average age 50 years Males=Females Fungal infections ➢ Histo, blastomyces, aspergillus Mycobacterial infections Rheumatoid arthritis Wegener’s granulomatosis Idiopathic

Figure 1-6-15

Bronchocentric Granulomatosis Clinical and Demographics – Non-Asthmatics

• • • •

Bronchocentric Granulomatosis: Pathology • •

• •

•

Nonspecific reaction Early invasion of mucosa ➢ Histiocytes ➢ Eosinophils ✧ Asthmatics ➢ Neutrophils ✧ Non-asthmatics Secondary involvement of adjacent arteries Granulomatous destruction ➢ Bronchial walls Bronchopneumonia ➢ Distal to affected airways

Mucoid impaction in patients with BCG

Bronchocentric Granulomatosis: Imaging [Figure 1-6-15] •

• • •

Most often unilateral ➢ 75% Multiple or solitary nodules Parenchymal consolidation ➢ Upper lobe predominance Associated findings of ABPA ➢ Bronchiectasis ➢ Mucoid impaction

Figure 1-6-16

BCG and Tuberculosis

BCG and Aspergillus [Figure 1-6-16]

Bronchocentric Granulomatosis Treatment and Prognosis • • •

ABPA may be clinically unsuspected Asthmatics respond to steroids Some cases remit without treatment Must rule out treatable infection and Wegener’s granulomatosis

•

Metastatic disease ➢ Squamous Multifocal infection ➢ Fungus, TB, bacteria Septic emboli Multiple pulmonary infarcts Langerhans’ cell histiocytosis Rheumatoid nodules

Angiitis and Granulomatosis: Differential Multiple vessel associated nodules •

• • • •

Chest Radiology

71

Angiitis and Granulomatosis

BCG?

Fungal Infection ?

Angiitis and Granulomatosis: Conclusion • • •

Wegener’s granulomatosis Churg-Strauss syndrome ➢ Allergic granulomatosis Necrotizing sarcoid granulomatosis Bronchocentric granulomatosis Lymphomatoid granulomatosis

• • •

Etiology Prognosis Therapy

• •

“Until specific causes are found ………we must devise syndromes”

References

1. Thurlbeck WM, Churg AM, eds. Pathology of the lung, second edition. New York: Thieme Medical Publishers, 1995; 401-435. 2. Godman GC, Churg J. Wegener’s granulomatosis: Pathology and review of the literature. A.M.A. Arch Pathol, 1954; 58(6): 533-553 3. Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest 1995; 108(2):320-323. 4. Liebow AA. The J. Burns Amberson Lecture: pulmonary angiitis and granulomatosis. Am Rev Respir Dis 1973; 108:118. 5. Travis WD, Fleming MV. Vasculitis of the lung. Pathology: State of the Art Reviews 1996; 4(1): 23-41. 6. Travis WD. Pathology of pulmonary granulomatous vasculitis. Sarcoidosis Vasc and Diffuse Lung Dis 1996; 13:1427. 7. Leavitt RY, Fauci AS. Pulmonary vasculitis. Am Rev Respir Dis 1986; 134:149-166. 8. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: Prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983; 98:76-85. 9. Kornblut AD, Fauci AS. Conversations on allergy and immunology; Cutis 1985; 35:27-34. 10. McDonald TJ, DeRemee RA. Wegener’s granulomatosis. Laryngoscope 1983; 93: 220-231. 11. Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis: a clinical and imaging study of 77 cases. Chest 1990; 97:906-912. 12. Daum TE, Specks U, Colby TV, et al. Tracheobronchial involvement in Wegener’s granulomatosis. Am J Respir Crit Care Med 1995; 151: 522-526. 13. Aberle DR, Gamsu G, Lynch D. Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology 1990; 174:703-709. 14. Nölle B, Specks U, Lüdemann J, Rohrbach MS, DeRemee RA, Gross WL. Anticytoplasmic autoantibodies: Their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med 1989; 111:28-40. 15. Travis WD, Carpenter HA, Lie JT. Diffuse pulmonary hemorrhage: an uncommon manifestation of Wegener’s granulomatosis. Am J Surg Pathol 1987; 11(9): 702-708. 16. Staples CA. Pulmonary angiitis and granulomatosis. Radiol Clin North Am 1991; 29(5): 973-982. 17. Kornblut AD, Wolff SM, DeFries HO, Fauci AS. Symposium on granulomatous disorders of the head and neck: Wegener’s granulomatosis. Otol Clin North Am 1982; 15(3):673-683. 18. Allen NB, Bressler PB. Diagnosis and treatment of the systemic and cutaneous necrotizing vasculitis syndromes. Med Clin North Am 1997; 81(1): 243-259. 19. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener’s granulomatosis: review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 1991; 15(4): 315-333. 20. Katzenstein AA, Locke WK. Solitary lung lesions in Wegener’s granulomatosis: Pathologic findings and clinical significance in 25 cases. Am J Surg Pathol 1995; 19(5): 545-552. 21. Feigin DS. Vasculitis in the lung. J Thorac Imag 1988; 3(1):33-48. 22. Epstein DM, Gefter WB, Miller WT, Gohel V, Bonavita JA. Spontaneous pneumothorax: an uncommon manifestation of Wegener granulomatosis. Radiol 1980; 135:327-328. 23. Fraser RS, Pare JAP, Fraser PD, eds. Synopsis of diseases of the chest, second edition. Philadelphia: WB Saunders Company, 1994; 411-419. Angiitis and Granulomatosis

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24. Farrelly CA. Wegener’s granulomatosis: a radiological review of the pulmonary manifestations at initial presentation and during relapse. Clin Radiol 1982; 33:545-551. 25. Lee SJ, Berry GJ, Husari AW. Wegener’s granulomatosis presenting as right middle lobe obstruction. Chest 1993; 103(5):1623-1624. 26. Travis WD, Colby TV, Lombard C, Carpenter HA. A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation. Am J Surg Pathol 1990; 14(12):1112-1136. 27. Wadsworth DT, Siegel MJ, Day DL. Wegener’s granulomatosis in children: chest radiographic manifestations. AJR 1994;163:901-904. 28. McHugh K. Wegener’s granulomatosis in children. [Letter] AJR 1995; 165(3):743. 29. Maguire R, Fauci AS, Doppman JL, Wolff SM. Unusual radiographic features of Wegener’s granulomatosis. AJR 1978; 141:233-238. 30. Maskell GF, Lockwood CM, Flower CDR. Computed tomography of the lung in Wegener’s granulomatosis. Clin Radiol 1993; 48:377-380. 31. Papiris SA, Manoussakis MN, Drosos AA, Kontogiannis D, Constantopoulos SH, Moutsopoulos HM. Imaging of thoracic Wegener’s granulomatosis: the computed tomographic appearance. Am J Med 1992; 93: 529-536. 32. Jaspan T, Davison AM, Walker WC. Spontaneous pneumothorax in Wegener’s granulomatosis. Thorax 1982; 37:774775 33. Grotz W, Mundinger A, Würtemberger G, Peter HH, Schollmeyer P. Radiographic course of pulmonary manifestations in Wegener’s granulomatosis under immunosuppressive therapy. Chest 1994;105(2):509-513. 34. Weir IH, Muller NL, Chiles C, Godwin JD, Lee SH, Kullnig P. Wegener’s granulomatosis: findings from computed tomography of the chest in 10 patients. Can Assoc Radiol J 1992; 43(1):31-34. 35. Kuhlman JE, Hruban RH, Fishman EK. Wegener granulomatosis: CT features of parenchymal lung disease. J Comput Assist Tomogr 1991; 15(6):948-952. 36. Erzurum SC, Underwood GA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest 1989; 95(6):1357-1359. 37. Primack SL, Hartman TE, Lee KS, Müller NL. Pulmonary nodules and the CT halo sign. Radiol 1994; 190:513-515. 38. Connolly S, Manson D, Eberhard A, Laxer RM, Smith C. CT appearance of pulmonary vasculitis in children. AJR 1996; 167:901-904. 39. Foo SS, Weisbrod GL, Herman SJ, Chamberlain DW. Wegener granulomatosis presenting on CT with atypical bronchovasocentric distribution. J Comput Assist Tomogr 1990; 14(6):1004-1006. 40. Stokes TC, McCann BG, Rees RT, Sims EH, Harrison BDW. Acute fulminating intrapulmonary haemorrhage in Wegener’s granulomatosis. Thorax 1982; 37:315-316. 41. Dugowson CE, Aitken ML. Unusual presentation of recurrent Wegener’s granulomatosis. Chest 1991; 99(3):781-784. 42. Erzurum SC, Underwood GA, Hamilos DL, Waldron JA. Pleural effusion in Churg-Strauss syndrome. Chest 1989; 95 (6):1357-1359. 43. Amundson DF. Cavitary pulmonary cryptococcosis complicating Churg-Strauss vasculitis. Southern Med J 1992; 85(7):700-702. 44. Buschman DL, Waldron JA, King TE. Churg-Strauss pulmonary vasculitis: high-resolution computed tomography scanning and pathologic findings. Am Rev Respir Dis 1990; 142:458-461. 45. Liebow AA, Carrington CRB, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol 1972; 3(4):457-558. 46. Katzenstein AA, Carrington CB, Liebow AA. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases. Cancer 1979; 43:360-373. 47. Fauci AS, Haynes BF, Costa J, Katz P, Wolff SM. Lymphomatoid granulomatosis: prospective clinical and therapeutic experience over 10 years. N Engl J Med 1982; 306(2):68-74. 48. Bragg DG, Chor PJ, Murray KA, Kjeldsberg CR. Lymphoproliferative disorders of the lung: histopathology, clinical manifestations, and imaging features. AJR 1994; 163:273-281. 49. Fauci AS, Haynes BF, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic, and therapeutic considerations. Ann Intern Med 1978; 89(1):660-676. 50. Dee PM, Arora NS, Innes DJ. The pulmonary manifestations of lymphomatoid granulomatosis. Radiol 1982; 143: 613-618. 51. Prenovault JMN, Weisbrod GL, Herman SJ. Lymphomatoid granulomatosis: a review of 12 cases. J Can Assoc Radiol 1988; 39:263-266. 52. Koss MN. Pulmonary lymphoid disorders. Semin Diag Pathol 1995; 12(2):158-171. 53. Guinee D, Jaffe E, Kingma D, Fishback N, et al. Pulmonary lymphomatoid granulomatosis: evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T-cell component and vasculitis. Am J Surg Pathol 1994; 18(8): 753-764. 54. Hicken R, Dobie JC, Frew E. The radiology of lymphomatoid granulomatosis in the lung. Clin Radiol 1979; 30: 661664. 55. Scully RE, Mark EJ, McNeely WF, Ebeling SH. Case records of the Massachusetts General Hospital. New Engl J Med 1996; 335(20):1514-1521. Chest Radiology

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56. Niimi H, Hartman TE, Müller NL. Necrotizing sarcoid granulomatosis: computed tomography and pathologic findings. J Comput Assist Tomogr 1995;19(6):920-923. 57. Warren J, Pitchenik AE, Saldana MJ. Granulomatous vasculitides of the lung: a clinicopathologic approach to diagnosis and treatment. South Med J 1989; 82(4):481-491. 58. Chittock DR, Joseph MG, Paterson NAM, McFadden RG. Necrotizing sarcoid granulomatosis with pleural involvement: clinical and radiographic features. Chest 1994; 106:672-676. 59. Weisbrod CL. Pulmonary angiitis and granulomatosis: a review . J Can Assoc Radiol 1989; 40:138-134. 60. Sadoun D, Kambouchner M, Tazi A, et al. Granulomatose necrosante sarcoid-like: à propos de 4 observations. Ann Med Interne 1994; 145(4) 230-233. 61. Myers JL, Katzenstein AA. Granulomatous infection mimicking bronchocentric granulomatosis. Am J Surg Pathol 1986; 10(5):317-322. 62. Koss MN, Robinson RG, Hochholzer L. Bronchocentric granulomatosis. Hum Pathol 1981; 12(7):632-638. 63. Sulavik SB. Bronchocentric granulomatosis and allergic bronchopulmonary aspergillosis. Clin Chest Med 1988; 9(4):609-621. 64. Berendsen HH, Hofstee N, Kapsenberg PD, Siewertsz Van Reesema DR, Klein JJ. Bronchocentric granulomatosis associated with seropositive polyarthritis. Thorax 1985; 40:396-397. 65. Clee MD, Lamb D, Urbaniak SJ, Clark RA. Progressive bronchocentric granulomatosis: case report. Thorax 1982; 37:947-949. 66. Felson B, Reeder MM. Gamuts in radiology, second edition. Cincinnati: Audiovisual Radiology of Cincinnati, Inc, 1967: 561-562. 67. Albelda SM, Gefter WB, Epstein DM, Miller WT. Diffuse pulmonary hemorrhage: a review and classification. Radiology 1985; 154: 289-29

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The Pulmonary Complications of Organ Transplantation Jeffrey R. Galvin, MD Introduction • • •

•

Organ transplant first performed the 1960’s Solid organ for vital organ failure Hematopoietic stem cell (HSC) ➢ Standard therapy ✧ Malignant, hematologic, autoimmune and genetic diseases Not proven in breast cancer

Pulmonary Complications in 40-60%: Multifactorial Cause • • • •

Underlying disease Therapy for underlying disease Graft-vs-host disease Conditioning regimen ➢ Chemotherapy and radiation

Solid Organ Transplant: Typical Schedule [Figure 1-7-1]

Figure 1-7-1

Bone Marrow Transplant: Typical Schedule

Figure 1-7-2

Solid organ transplant complications begin predominantly after the first month post-transplant

[Figure 1-7-2]

Complications are usually separated into those that occur before and after there first 100 day

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Early Pulmonary Complications • • • • •

• • •

Figure 1-7-3

Pulmonary edema Fungal infection Diffuse alveolar hemorrhage Bacterial infection Viral infection ➢ CMV and herpes Pneumocystis carinii Acute graft-vs-host disease Idiopathic pulmonary syndrome ➢ ARDS, DAD

Late Pulmonary Complications

•

Chronic graft-vs-host Obstructive airways disease ➢ Bronchiolitis obliterans Organizing Pneumonia ➢ BOOP Restrictive ventilatory defect Late bacterial infections ➢ Sinopulmonary Herpes varicella zoster

• •

Residual tumor Occult infection

•

Donor aspiration ➢ General anesthesia 150-200 aspirates Marrow strained Immunocompetent T-cells ➢ Depleted with monoclonal reagents Infusion of marrow ➢ 400-800 ml

• •

• • •

Pretransplant Considerations

Traditionally, allogeneic transplantation used bone marrow grafts. From 1999-2002 there was a steady increase in peripheral blood stem cell grafts

Operative Technique [Figure 1-7-3] • • •

•

Figure 1-7-4

Immunologic Impact • •

•

Profound neutropenia Prolonged depression ➢ Cellular function ➢ Humoral function Graft-vs-host ➢ Direct effect ➢ Steroids

Pulmonary Edema • • •

• • • • • •

[Figure 1-7-4]

Common complication 2nd-3rd week posttransplantation Rapid onset ➢ Dyspnea and hypoxemia Reticulo-nodular markings Fluid overload ➢ Blood products, antibiotics and TPN Cardiac Renal dysfunction Decreased albumin Often accompanied by fever

Pulmonary Complications of Organ Transplantation

Pulmonary edema in a bone marrow transplant patient demonstrating interlobular septal thickening and enlarged pulmonary veins 76

Chest Radiology

Fungal Infections •

• • • • • • •

Up to 45% of BMT patients ➢ 85% mortality Aspergillus is most common Occurs in the first 30 days posttransplantation Symptoms: ➢ Fever, dyspnea, cough, chest pain and hemoptysis Predisposition ➢ Prolonged granulocytopenia ➢ Broad spectrum antibiotics Nodules Early “Halo-Sign” Late “Air Crescent”

Fungal Infections

[Figures 1-7-5 to 1-7-7]

Invasive Aspergillosis: Diagnosis • • • •

Figure 1-7-5

Fungi normally invade the lung via the airway

Figure 1-7-6

BAL (69%) Tissue Biopsy (60%) Antigen (83%) Computed Tomography (92%)

Caillot, J Clin Oncol. 1997

Imaging and Survival: Invasive Aspergillosis Caillot, J Clin Oncol. 1997

Pulmonary Hemorrhage • • • •

• • • •

21% of BMT patients 12th day posttransplantation Neutrophil recovery Sudden onset: ➢ Dyspnea, cough, fever and hypoxemia Rare hemoptysis Mortality 50-80% Radiographic abnormalities before symptoms Bilateral ground glass opacities ➢ May be localized

Typical infarct with a halo of blood in an aspergillus infection

Figure 1-7-7

Air-crescent sign in a patient with recovering cell counts

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Pulmonary Complications of Organ Transplantation

Pulmonary Hemorrhage [Figure 1-7-8]

Figure 1-7-8

Cytomegalovirus Pneumonia • • • •

•

10-40% of BMT patients 6-12 weeks posttransplantation Mortality rate of 85% Reactivation of latent virus in 70% ➢ Remainder infected by “CMV positive” blood products Anti-viral therapy improves prognosis

Cytomegalovirus Pneumonia [Figure 1-7-9] • • • •

Bilateral Ground glass Nodules Consolidation ➢ Alone

66% 59% 59% 3%

Franquet et al AJR Oct 2003

Pneumocystis Jiroveci Pneumonia • •

• • • •

<10% of BMT patients Effective prophylaxis ➢ Trimethoprim/sulfa Rapid progression Severe dyspnea Bilateral perihilar Ground-glass opacities

Aspergillus infection may be a cause of pulmonary hemorrhage

Figure 1-7-9

Noninfectious Pulmonary Complication •

• • •

•

Late-onset Noninfectious Pulmonary Complications ➢ LONIPC’s ➢ After the first 3 months ➢ 10-23% of allogeneic grafts Idiopathic pulmonary syndrome ➢ Diffuse alveolar damage (DAD) Bronchiolitis obliterans Organizing pneumonia ➢ BOOP Associate with GVHD ➢ Sicca syndrome ➢ Acive donor T-cells

Sakaida, Blood Vol 102 2003

Typical lower lobe nodules of CMV

Idiopathic Pulmonary Syndrome • •

• • • • • •

Diffuse lung injury posttransplantation Histology ➢ Interstitial mononuclear infiltrate ➢ DAD 12% of allogenic BMT 40-80 days posttransplantation Risk factors ➢ GVHD ➢ Radiation Fever, cough and hypoxemia Mortality rate of 70% Diffuse Opacities

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Idiopathic Pulmonary Syndrome

Figure 1-7-10

[Figures 1-7-10 and 1-7-11]

Diffuse Alveolar Damage •

• •

• • • • •

Infectious agents ➢ Legionella, mycoplasma, viruses Inhalants ➢ Ammonia, chlorine, HS Drugs ➢ Cytoxan, BCNU, Bleomycin Ingestants ➢ Kerosene, Paraquat Shock/trauma Sepsis Radiation Idiopathic ➢ Hammon-Rich or AIP

Patients with IPS present with diffuse opacities involving all 5 lobes

Viral Infection

Figure 1-7-11

Idiopathic Pulmonary Syndrome [Figure 1-7-12]

Graft-vs-Host Disease (GVHD) : Donor T-lymphocytes recognize the recipient’s tissue as foreign •

•

Acute GVHD ➢ 20-100 days posttransplantation ➢ 25-75% of patients ➢ skin, gut and liver dysfunction ➢ 10% mortality Chronic GVHD ➢ 1> 100 days posttransplantation ➢ 20-45% of patients ➢ Features of autoimmune diseases ➢ Sjogren’s, scleroderma, biliary cirrhosis and airway obstruction

GVHD and IPS

GVHD and Infection

In the early phase (exudative) there is diffuse consolidation and ground glass often with peripheral clearing

Figure 1-7-12

GVHD and IPS

Radiation Pneumonitis • • • •

Related to dose of TBI Presents within 90 days Cough, fever, and dyspnea Threshold lowered by chemotherapy

•

Correlates ➢ Idiopathic interstitial pneumonia Increased likelihood with more radiation Not a serious complication by itself May be a harbinger of pneumothorax

Mediastinal Emphysema • • •

The late phase of IPS demonstrates traction bronchiectasis consistent with fibrosis Chest Radiology

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Pulmonary Complications of Organ Transplantation

Secondary Malignancies • •

• • • •

Figure 1-7-13

0.02% incidence 7X’s increase ➢ Over the general population 1 year after transplantation ➢ Median Hodgkin’s 45% Leukemia 17% Solid tumors 38%

Lymphoma and Immune Impairment •

Pathologic Features ➢ B-cell non-Hodgkin ’s ✧ Driven by Epstein-Barr virus infection ✧ Diffuse polyclonal expansion – Reduced T-cell control ✧ Malignant transformation

Bone Marrow Transplant Bronchiolitis Obliterans • • • • • •

• •

Bronchiolitis obliterans demonstrating mosaic attenuation

2-13% of BMT’s Low immunoglobulin level Chronic GVHD ➢ Sicca syndrome 100 days posttransplantation Gradual deterioration of PFT’S Airflow obstruction ➢ Fixed Reduction in diffusing capacity Imaging ➢ Mosaic attenuation ➢ Expiratory accentuation ➢ Centrilobular nodules ➢ Patchy consolidation

Figure 1-7-14

Bronchiolitis Obliterans [Figures 1-7-13 to 1-7-15] Segmental or Lobar Consolidation •

Infection

• • • • •

Pulmonary edema Hemorrhage Diffuse alveolar damage Viral pneumonia Pneumocystis pneumonia

• • •

Bacterial pneumonia Pulmonary edema Hemorrhage

• • • •

Aspergillus Pneumocystis Diffuse alveolar damage CMV

Diffuse Opacities

Bronchiolitis obliterans demonstrating indistinct nodules and branching opacities

Figure 1-7-15

Rapid Progression Over 24 Hours Progression Over Days

Bronchiolitis obliterans demonstrating bronchiectasis Pulmonary Complications of Organ Transplantation

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Diffuse Opacities Fluid Overload

Organizing Pneumonia

BMTP: dyspnea and cough

BMTP Lymphangitic Spread BMTP

BMTP Aspergillus

BMTP: dyspnea and cough BMTP: Edema

Bone Marrow Transplant: Typical Schedule References

General 1. Franquet T, Muller NL, Lee KS, Gimenez A, Flint JD.High-resolution CT and pathologic findings of noninfectious pulmonary complications after hematopoietic stem cell transplantation. AJR Am J Roentgenol. 2005 Feb;184(2):62937 2. Kotloff RM, Ahya VN, Crawford SW.Pulmonary complications of solid organ and hematopoietic stem cell transplantation. Am J Respir Crit Care Med. 2004 Jul 1;170(1):22-48. Epub 2004 Apr 7

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The Diagnosis of Pulmonary Embolism Jeffrey R. Galvin, MD Pulmonary Embolus • • •

Frequent Potentially fatal Largely undiagnosed

Baglin, J Clin Path, 1997

Pulmonary Embolus: Epidemiology • • • •

5 million episodes of DVT 300,000 embolic events 50,000 deaths 100/100,000 new cases

• • • • • • • •

Predisposing factors Pathology Signs and symptoms Radiography Arterial blood gases V/Q scanning Computed tomography Arteriography

The “Clinical Picture” of PE

Pulmonary Embolism is a Complication of Deep Venous Thrombosis Hull, Annals of Internal Med 1983

Sources of Pulmonary Emboli •

• •

•

Majority of clots ➢ Lower extremity veins Increasing number of clots ➢ Upper extremities, cardiac chambers and catheters A negative venous study ➢ Does not rule out PE < 50% of PE patients ➢ Positive lower extremity study

Kelly, Ann Int Med, 1991

Embolic Events: Predisposing Causes • • •

Stasis Trauma Hypercoagulable states

• • • • • • • • •

1° thrombophlebitis Bed rest Recent surgery Venous insufficiency Recent fracture Myocardial infarction Malignancy CHF No Predisposition

Predisposing Causes

Pulmonary Embolism

39% 32% 31% 25% 15% 12% 8% 5% 6%

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PE and Malignancy: 10-15% of unexplained phlebitis • • •

Gastrointestinal Pulmonary Genitourinary

The History and Physical are Non-Specific Symptoms in Patients with Non-Fatal PE • • • • • • •

Chest Pain Dyspnea Apprehension Cough Hemoptysis Sweats Syncope

88% 84% 59% 53% 30% 27% 13%

• • • • • • • • •

RR> 16 Rales HR> 100 T> 37.8°C Diaphoresis Gallop Phlebitis Murmur Cyanosis

92% 58% 44% 43% 36% 34% 32% 23% 19%

Signs in Patients with Non-Fatal PE

Figure 1-8-1

PE and Underlying Lung Disease

The History and Physical are Insensitive We do not know the Prevalence of PE. Diagnostic Algorithm and Clinical Suspicion [Figure 1-8-1]

“Among the various causes of an incorrect diagnosis, most important are: the failure to suspect PE and, the protean nature of the disease.” Morpurgo, Chest 1995

The clinical diagnosis of PE is unreliable. Many patients are symptomatic

History and Physical

The Role of Clinical Suspicion •

Less than 35% of fatal emboli were diagnosed antemortem

• • • • • • • • • •

Dyspnea Syncope Altered mentation Apprehension Chest pain Sweatiness Pleuritic Pain Cough Hemoptysis Arrest

Symptoms in Patients with Fatal PE

Chest Radiology

59% 27% 20% 17% 10% 9% 8% 3% 3% 8%

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Pulmonary Embolism

Signs in Patients with Fatal PE • • • • • • • • • •

RR> 16 HR> 100 Rales T> 37.8°C Edema Hypotension Cyanosis Gallop Diaphoresis Phlebitis

66% 54% 42% 30% 26% 20% 12% 10% 10% 7%

Figure 1-8-2

The Chest X-Ray is Usually Abnormal

The Chest X-Ray in Pulmonary Embolism •

84% had abnormal radiographs PE(%) ➢ Atelectasis/Infiltrate 68 ➢ Pleural Effusion 48 ➢ Pleural Opacity 35 ➢ Elevated Diaphragm 24 ➢ Decreased Vascularity 21 ➢ Prominent PA 17 ➢ Cardiomegaly 12 ➢ Westermark’s Sign 7 ➢ Pulmonary Edema 4

PIOPED

NoPE(%) 48 31 21 19 12 28 11 2 13

Common Radiographic Abnormalities

• • • • • • • •

Infiltrate Pleural Effusion Atelectasis Diaphragm Up 2 or More CHF Focal Oligemia Normal

• • • • • •

Atelectasis 100% Consolidation 57% Hampton’s hump 50% Ground glass 57% Pleural Effusions 87% Mosaic attenuation

Chest CT Findings

54% 51% 27% 17% 44% 17% 2% 7%

Truong, ARRS, 1998

Radiographic and CT Findings

[Figure 1-8-2]

Peripheral opacities may raise suspicion for clinically unsuspected PE

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Pathology [Figure 1-8-3] • • •

Figure 1-8-3

Edema Hemorrhage Infarction

Normal Arterial Oxygenation Does Not Exclude Pulmonary Embolism Arterial Blood Gases • • •

10-15% will have a PO2 >85mm HG A low arterial PO2 is non-specific A respiratory alkalosis is most common

•

V/Q abnormalities ➢ Variable Complete vascular occlusion ➢ Rare Complete shunt 2° to ➢ Atelectasis ➢ Hemorrhage Autoregulation ➢ Hypoxic vasoconstriction ➢ Hypocapnic bronchoconstriction

The Physiology of Pulmonary Embolism • • •

Hemorrhage and edema are common sequela of PE. Infarct is less common and is more likely to occur in patients with CHF

Levy, JAP, 1974 Dantzker, Circulation Res, 1974 Dantzker, Chest Vol. 91 no. 5

Physiologic Change with Heparinization •

•

Ventilation ➢ Returns more rapidly than perfusion Perfusion ➢ May return before ventilation

Santolicandro, Am J Res Crit Care Med, 1995

V/Q Physiology

Ventilation/Perfusion Scanning • • • • •

High Intermediate Low Normal Total

PIOPED

Clinical Science Probability (%) 80-100 20-79 0-19 All Probabilities 96% 88% 56% 87% (103/118) 66% 28% 16% 30% (104/345) 40% 16% 4% 14% (40/296) 0% 6% 2% 4% (5/128) 68% 30% 9% 28% (252/887)

The Basis of “Clinical Science Probability” • • • • • • • • •

Dyspnea Pleuritic Pain Cough Leg Swelling Hemoptysis Palpitations Wheezing “Angina”

PIOPED

Chest Radiology

PE(%) 73 66 37 28 13 10 9 4

No PE(%) 72 59 36 22 8 18 11 6

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Pulmonary Embolism

Traditional Approach [Figure 1-8-4]

Figure 1-8-4

The Low-Probability Lung Scan •

• •

“There is an 8% mortality rate in patients with a “low probability” V/Q scan and limited cardiopulmonary reserve.” Hull, Archives of Internal Medicine, 1995 “There is a 25%-30% disagreement between expert readers in interpreting INTERMEDIATE and LOW probability V/Q scans.” PIOPED, JAMA, 1990 A Potentially Lethal Reading ➢ “Pulmonary embolism cannot be diagnosed on clinical grounds; it can only be suspected.” Bone, Archives of Internal Medicine, 1993

The Goal of Imaging – Visualization of the clot The Role of Pulmonary CT Angiography • • •

Initial screening Detection of unexpected emboli Detection of other pathology

CT Angiography

Pulmonary CT Angiography – Sensitivity and Specificity • • • •

Accurately identifies emboli ➢ Main, lobar and segmental vessels Misses some subsegmental emboli Indeterminate 8-10% Constantly changing ➢ Related to collimation, scanner speed and prevalence ➢ Sensitivity 66-93% ➢ Specificity 89-97%

Eng, AJR 183; 2004

Pulmonary CT Angiography – Sensitivity and Specificity • • • •

3mm visualizes 40% of subsegmental arteries 3mm visualizes 75% of segmental arteries 1.25mm visualizes 75% of subsegmental arteries 1.23mm visualizes 90% of segmental arteries

Patel, Radiology, 2003

Significance of Small Emboli – Standard Angiography •

•

Good outcome ➢ Patients with “negative angio” 1.5% embolize when followed 1 year ➢ 691 patients

Novelline, Radiology, 1978

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Distribution of Pulmonary Emboli •

•

•

Multiple locations ➢ > 55% Marked preference for ➢ Right lung and lower lobes Subsegmental only ➢ 6-30%

PIOPED, JAMA, 1990; Oser, Radiology, 1996; Morpurgo, Chest, 1995

Significance of Small Emboli - CTA •

•

Small emboli that need treatment ➢ Poor cardiopulmonary reserve ➢ Coexisting acute DVT ➢ Recurrent PE Small emboli that may not need treatment ➢ Subsegmental clots without evidence of DVT ➢ Indeterminate scan without evidence of DVT ➢ Normal cardiovascular status ➢ Follow-up DVT scan in 1 week

Goodman, Radiology 234; 2005

Pulmonary CT Angiography Negative Predictive Value of a Normal CT • •

No prospective, consecutive studies “The safety of withholding anticoagulants…is uncertain”

Pulmonary CT Angiography Negative Predictive Value of a Normal CT

n Mayo 69 Feretti 109 Garg 78 Loomis 81 Goodmann 198 Remy-Jardin 71 Tillie-Leblond 185 Kavanagh 85

Follow-up 3m 3m 6m 6m 3m 3m 12m 9m

NPV 97% 97% 99% 100% 99% 97% 98% 99%

Pulmonary CT Angiography – Intra and Interobserver Variability • • • •

Radiology’s Achilles’ Heel Related to clot size Exacerbated by poor exam Related to reader experience

Mayo, Radiology, 1997; Chartrand-Lefebre, AJR, 1999

Pulmonary CT Angiography •

•

Alternate diagnoses ➢ 11-33% Unexpected emboli ➢ 1-4%

Storto, AJR:184 2005

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Pulmonary Embolism

Alternative Diagnoses Figure 1-8-5

[Figure 1-8-5 and 1-8-6]

Figure 1-8-6

Pulmonary infection is a common alternative diagnosis is patients suspected of pulmonary embolus

Adenocarcinoma is an important predisposition for hypercoaguability and PE

Pitfalls in Helical CT [Figure 1-8-7 and 1-8-8] •

• • •

Partial volume ➢ Obliquely oriented arteries Suboptimal contrast enhancement Breathing artifacts Lymph nodes

Figure 1-8-8

Figure 1-8-7

Adenopathy can mimic PE

Breathing artifacts should be assessed before reading a CTA for PE

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Technical Improvements • • • •

•

Multi-channel CT Narrower collimation: 1mm Subsecond scanning Contrast timing ➢ “Smart prep” ➢ Test bolus: peak + 5 sec ➢ 20 seconds normal cardiac output ➢ Caudal-cranial scanning Workstation viewing ➢ Cine Mode (PACS or Workstation) ➢ Adjust window and levels for each case ➢ Multi-planar reconstruction ➢ Breathing artifact-coronal lung windows

Figure 1-8-9

16 Channel CT [Figure 1-8-9]

Coronal reconstrution helps separate pulmonary arteries and veins

Paddlewheel Reformation Simon, AJR:177 July 2001

Combined Pulmonary CTA and Venography • • •

Increases detection of thromboembolic disease by 20% Contiguous sections 1cm collimation

Cham, Radiology;234 2005

Combined Pulmonary CTA and Venography Year Loud 00 Duwe 00 Garg 00 Cham 00 Peterson 01

N 150 74 70 116 136

Sensitivity 97% 89% 100% 100% 71%

Specificity 100% 94% 97% 96% 93%

A Diagnostic Algorithm for Pulmonary Embolism

Pulmonary Embolus and Prognosis

The prognosis in PE patients is closely related to the presence and extent of clot in the peripheral veins

Chest Pain-Dyspnea Screening Conclusion

• • • • • •

The clinical diagnosis of PE is unreliable The chest radiograph is usually abnormal V/Q readings restricted to “reliable categories” Small clots are a problem for all modalities Outcome studies are key CT angiography is the modality of choice

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Pulmonary Embolism

References

General 1. Robin E, D. Overdiagnosis and overtreatment of pulmonary embolism: the emperor may have no clothes. Annals of Internal Medicine 1977; 87(6):775-781. 2. Morgenthaler TI, Ryu JH. Clinical characteristics of fatal pulmonary embolism in a referral hospital. Mayo Clinic Proceedings 1995; 70(5):417-424. 3. Baglin TP, White K, Charles A. Fatal pulmonary embolism in hospitalised medical patients. J Clin Pathol 1997; 50(7):609-10. 4. Goldhaber SZ. Pulmonary embolism. N Engl J Med 1998; 339(2):93-104. 5. Huisman MV, Buller HR, ten Cate JW, van Royen EA, Vreeken J, Kersten M-J, Bakx R. Unexpected high prevalence of silent pulmonary embolism in patients with deep venous thrombosis. Chest 1989; 95(3):498-502. 6. Patriquin L, Khorasani R, Polak JF. Correlation of diagnostic imaging and subsequent autopsy findings in patients with pulmonary embolism [see comments]. AJR Am J Roentgenol 1998; 171(2):347-9. 7. Shatz DV. Statewide, population-based, time-series analysis of the frequency and outcome of pulmonary embolus in 318,554 trauma patients [letter; comment]. J Trauma 1998; 44(1):239. Physiology 1. Dalen JE, Haffajee CI, Alpert JS, Howe JP, Ockene IS, Paraskos JA. Pulmonary embolism, pulmonary hemorrhage and pulmonary infarction. New England Journal of Medicine 1977; 296(25):1431-1435. 2. Dantzker DR, Bower JS. Clinical significance of pulmonary function tests: alterations in gas exchange following pulmonary thromboembolism. Chest 1982; 81(4):495-501. 3. Dantzker DR. Ventilation-perfusion inequality in lung disease. Chest 1987; 91:749-754. 4. Santolicandro A, Prediletto R, Fornai E, Formichi B, Begliomini E, Giannella-Neto A, Giuntini C. Mechanisms of hypoxemia and hypocapnia in pulmonary embolism. Am J Respir Crit Care Med 1995; 152(1):336-47.

Radiography 1. Moses DC, Silver TM, Bookstein JJ. The complementary roles of chest radiography, lung scanning, and selective pulmonary angiography in the diagnosis of pulmonary embolism. Circulation 1974:179-188. 2. Bynum LJ, Wilson JE. Radiographic features of pleural effusions in pulmonary embolism. American Review of Respiratory Disease 1978; 117:829-834. 3. Buckner CB, Walker CW, Purnell GL. Pulmonary embolism: chest radiographic abnormalities. Journal of Thoracic Imaging 1989; 4(4):23-27. 4. Sasahara AA, Hyers TM. The urokinase pulmonary embolus trial-A national cooperative study. Circulation 1973; 47(suppl 2):1-108. Scintigraphy 1. Alderson PO, Rujanavech N, Secker-Walker RH, Mcknight RC. The role of 133Xe ventilation studies in the scintigraphic detection of pulmonary embolism. Radiology 1976; 120(633-640). 2. Hirsh J. Diagnosis of venous thrombosis and pulmonary embolism. American Journal of Cardiology 1990; 65:45C49C. 3. Hull RD, Hirsh J, Carter CJ, Jay RM, Dodd PE, Ockelford PA, Coates G, Gill G, Turpie AG, Doyle DJ, Buller HR, Raskob GE. Pulmonary angiography, ventilation lung scanning, and venography for clinically suspected pulmonary embolism with abnormal perfusion lung scan. Annal of Internal Medicine 1983; 98(6):891-899. 4. Hull RD, Hirsh J, Carter CJ, Raskob GE, Gill GJ, Jay RM, Leclerc JR, David M, Coates G. Diagnostic Value of ventilation-perfusion lung scanning in patients with suspected pulmonary embolism. Chest 1985; 88(6):819-828. 5. Hull R, Raskob G, Ginsberg J. A noninvasive strategy for the treatment of patients with supected pulmonary embolism. Archives of Internal Medicine 1994; 154:289-97. 6. Hull RD, Raskob GE, Coates G, Panju AA. Clinical validity of a normal perfusion lung scan in patients with suspected pulmonary embolism. Chest 1990; 97(1):23-26. 7. Hull R, Raskob G, Pineo G, Brant R. The low-probability lung scan: a need for change in the nomenclature. Archives of Internal Medicine 1995; 155(1845-1851). 8. Schluger N, Henschke C, King T, Russo R, Binkert B, Rackson M, Hayt D. Diagnosis of pulmonary embolism at a large teaching hospital. Journal of Thoracic Imaging 1994; 9:180-184. 9. Pioped Investigators. Value of the ventialtion/perfusion scan in acute pulmonary embolism. Journal of The American Medical Association 1990; 263:2753-2759. Angiography 1. Novelline R, Baltarowich O, Athanasoulis C, Greenfield A, McKusick K. The clinical course of patient with suspected pulmonary embolism and a negative pulmonary angiogram. Radiology 1978; 126:561-567. 2. Quinn MF, Lundell CJ, Klotz TA, Finck EJ, Pentecost M, McGehee WG, Garnic JD. Reliability of selective pulmonary arteriography in the diagnosis of pulmonary embolism. American Journal of Roentgenology 1987; 149:479-471. Pulmonary Embolism

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3. Stein PD, Athanasoulis C, Alavi A, Greenspan RH, Hales CA, Saltzman HA, Vreim CE, Terrin ML, Weg JG. Complications and validity of pulmonary angiography in acute pulmonary embolus. Circulation 1992; 85(462468). 4. Stein PD, Henry JW, Gottschalk A. Reassessment of pulmonary angiography for the diagnosis of pulmonary embolism: relation of interpreter agreement to the order of the involved pulmonary arterial branch. Radiology 1999; 210(3):689-91.

Computed Tomography 1. Balakrishnan J, Meziane MA, Siegelman SS, Fishman EK. Pulmonary infarction: CT appearance with pathologic correlation. Journal of Computer Assisted Tomography 1989; 13(6):941-945. 2. Beigelman C, Chartrand-Lefebvre C, Howarth N, Grenier P Pitfalls in diagnosis of pulmonary embolism with helical CT angiography. AJR Am J Roentgenol; 1998; 171(3):579-85. 3. Cham MD, Yankelevitz DF, Henschke CI.Thromboembolic disease detection at indirect CT venography versus CT pulmonary angiography. Radiology. 2005 Feb;234(2):591-4. 4. Coche EE, Muller NL, Kim KI, Wiggs BR, Mayo JR. Acute pulmonary embolism: ancillary findings at spiral CT. Radiology 1998; 207(3):753-8. 5. Eng J, Krishnan JA, Segal JB, Bolger DT, Tamariz LJ, Streiff MB, Jenckes MW, Bass EB. Accuracy of CT in the diagnosis of pulmonary embolism: a systematic literature review. AJR Am J Roentgenol. 2004 Dec;183(6):181927. 6. Eyer BA, Goodman LR, Washington L. Clinicians' response to radiologists' reports of isolated subsegmental pulmonary embolism or inconclusive interpretation of pulmonary embolism using MDCT. AJR Am J Roentgenol. 2005 Feb;184(2):623-8. 7. Falashci F, Palla A, Formichi B, Sbragia P Petruzzelli S, Guintini C, Bartolozzi C. CT evaluation of chronic thromboembolic pulmonary hypertension. Journal of Computer Assisted Tomography 1992; 16:897-903. 8. Garg K, Welsh CH, Feyerabend AJ, Subber SW, Russ PD, Johnston RJ, Durham JD, Lynch DA. Pulmonary embolism: diagnosis with spiral CT and ventilation-perfusion scanning—correlation with pulmonary angiographic results or clinical outcome. Radiology 1998; 208(1):201-8. 9. Gefter W, Hatabu H, Holland G, Gupta K, Henschke C, Pavelsky H. Pulmonary Thromboembolism: recent developments in diagnosis with CT and MR imaging. Radiology 1995; 197:561-574. 10. Geraghty JJ, Stanford W, Landas S, Galvin J. Ultrafast computed tomography in experimental pulmonary embolism. Investigative Radiology 1991; 27:60-63. 11. Goodman LR. Small pulmonary emboli: what do we know? Radiology. 2005 Mar;234(3):654-8. 12. Goodman LR, Curtin JJ, Mewissen MW, Foley WD, Lipchik RJ, Crain MR, Sagar KB, Collier BD. Detection of pulmonary embolism in patients with unresolved clinical and scintigraphic diagnosis: helical ct versus angiography. American Journal of Roentgenology 1995; 164:1369-1 374. 13. Goodman LR, Lipchik RJ. Diagnosis of acute pulmonary embolism: time for a new approach. Radiology 1996; 199:25-27. 14. Goodman LR, Lipochik RJ, Kuzo RS. Acute pulmonary embolism: the role of computed tomographic imaging. Journal of Thoracic Imaging 1997; 12(2):83-86. 15. Goodman LR. Helical CT for initial imaging of pulmonary embolus. AJR Am 3 Roentgenol 1998; 171(4):1153-4. 16. Gurney JW. No fooling around: direct visualization of pulmonary embolism. Radiology 1993; 188:618-619. 17. Kim KI, Muller NL, Mayo JR. Clinically suspected pulmonary embolism: utility of spiral CT. Radiology 1999; 210(3):693-7. 18. Mayo JR. Remy-Jardin M, Muller NL, Remy J, Worsley DF, Hossein-Foucher C, Kwong JS, Brown MJ. Pulmonary embolism: prospective comparison of spiral CT with ventilation-perfusion scintigraphy. Radiology 1997; 205(2):447-52. 19. Remy-Jardin M, Remy J, Artaud D, Deschildre F, Fribourg M, Beregi JP. Spiral CT of pulmonary embolism: technical considerations and interpretive pitfalls. J Thorac Imaging 1997; 12(2):103-17. 20. Remy-Jardin M, Remy J, Deschildre F, Artaud D, Beregi JP Hossien-Foucher C, Marchdise X, Duhamel A. Diagnosis of pulmonary embolism with spiral ct:comparison with pulmonary angiography and scintigraphy. Radiology 1996;200:699-706. 21. Remy-Jardin M, Remy J, Wattinne L, Giraud F. Central pulmonary thromboembolism: diagnosis with spiral volumetric ct with single-breath-hold technique-comparison with pulmonary angiography. Radiology 1992; 185:381-387. 22. Ren H, Kuhlman JE, Hruban RH, Fishman EK, Wheeler PS, Hutchins GM. CT of inflation-fixed lungs: wedgeshaped density and vascular sign in the diagnosis of infarction. Journal of Computer Assisted Tomography 1990;14(1):82-86. 23. Revel MP, Petrover D, Hernigou A, Lefort C, Meyer G, Frija G. Diagnosing pulmonary embolism with fourdetector row helical CT: prospective evaluation of 216 outpatients and inpatients. Radiology. 2005 Jan;234(1):26573. Chest Radiology

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24. Storto ML, Di Credico A, Guido F, Larici AR, Bonomo L.Incidental detection of pulmonary emboli on routine MDCT of the chest. AJR Am J Roentgenol. 2005 Jan;184(1):264-7. 25. Tardivon AA, Musset D, Maitre S, Brenot F Dartevelle P, Simonneau G, Lobrune M. Role of ct in chronic pulmonary embolism: comparison with pulmonary angiography. Journal of Computer Assisted Tomography 1993;17:345-351. 26. Teigen C, Maus TP, Sheedy PF, Johnson CM, Stanson AW, Welch TJ. Pulmonary embolism: diagnosis with electon-beam ct. Radiology 1993;188:839-845. 27. Teigen CL, Maus TR Sheedy PR Stanson AW, Johnson CM. Preen JR Mckusick MA. Pulmonary embolism: diagnosis with contrast-enhanced electron-beam ct and comparison with pulmonary angiography. Radiology 1995; 194:313-319. 28. Van Erkel A, van Possum A, Bloem J, Mali W, Pattynama P. Cost-effectiveness of the us of spiral CT angiography to determine suspected pulmonary embolism. Radiology 1995; 197(P):303-304. 29. Van Rossum AB, Pattynama PMT, Tjin ER, Treurniet FE, Arndt J-W, van Eck B, Kieft GJ. Pulmonary embolism: validation of spiral ct angiography in 149 patients. Radiology 1996; 201 :467-470. 30. Winer-Muram HT, Rydberg J, Johnson MS, Tarver RD, Williams MD, Shah H, Namyslowski J, Conces D, Jennings SG, Ying J, Trerotola SO, Kopecky KK.Suspected acute pulmonary embolism: evaluation with multidetector row CT versus digital subtraction pulmonary arteriography.Radiology. 2004 Dec;233(3):806-15. 31. Winston C, Wechsler RJ, Salazar AM, Kurtz AB, Spirn PW. incidental pulmonary emboli detected at helical ct: effect on patient care. Radiology 1996;201:23-27. Magnetic Resonance 1. Hatabu H, Gaa J, Kim D, Li W, Prasad PV, Edelman R. Pulmonary perfusion and angiography: evaluation with breath-hold enhanced three-dimensional fast imaging steady-state precession mr imaging with short tr and te. AJR 1996; 167:653-655. 2. Hatabu H, Gaa J, Kim D, Li W, Prasad P, Edelman RR. Pulmonary perfusion: qualitative assessment with dynamic contrast-enhanced mri using ultra short TE and inversin recovery turbo FLASH. Magnetic Resonance in Medicine 1996; 36:503-508. 3. Gefter W, Hatabu H, Holland G, Gupta K, Henschke C, Pavelsky H. Pulmonary Thromboembolism: recent developments in diagnosis with CT and MR imaging. Radiology 1995; 197:561-574. 4. Gefter WB, Hatabu H, Dinsmore BJ, Axel L, Palevsky H, Reichik N, Schiebler ML, Kressel HY. Pulmonary vascular cine MR imaging: a noninvasive approach to dynamic imaging of the pulmonary circulation. Radiology 1990; 176(3):761-770. 5. Meaney JFM, Weg JG, Chenevert TL, Stafford-Johnson D, Hamilton BH, Prince MR. Diagnosis of pulmonary embolism with magnetic resonance angiography. The New England Journal Resonance 1997; 336:1422-1427.

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Tuberculosis

Jeffrey R. Galvin, MD Tuberculosis • • • • • • •

Leading cause of death from infectious disease 8-10 million new cases/year 2-3 million deaths/year 1/3 of world population infected > 90% of new cases in developing countries 80% 15-59 years of age Highest incidence ➢ Southeast Asia: 247/100,000 ➢ Sub-Saharan Africa: 191/100,000 ✧ HIV co-infection: 60% of children, 70% of adults

Tuberculosis: History • • • •

Ancient disease 1882: Robert Koch ➢ Isolation of M. tuberculosis 1944: streptomycin 1952: INH

Tuberculosis Pre-Antibiotic Era Tuberculosis: United States •

• •

1953: 84,304 cases ➢ 19,707 deaths 1985: 22,201 cases ➢ 1,752 deaths 1986-1992: 20% increase in reported cases ➢ HIV ➢ Immigration ➢ Congregate settings ➢ Deteriorating TB services ➢ MDR-TB ➢ Decreasing TB research

Tuberculosis: United States •

•

• •

2002: 15,075 cases ➢ 5.2/100,000 ➢ 43% decrease from 1992 ➢ 4-6% of population infected ✧ 15 million people 51% Foreign-born ➢ Mexico, the Philippines, Vietnam, India and China U.S. -born ➢ African Americans 25% of all cases ➢ homeless, immunocompromised, elderly Urban areas, coastal states, states bordering Mexico

NMWR,March 21, 2003 Vol 52

Mycobacteria •

•

• •

Tuberculosis complex ➢ M. tuberculosis, M. bovis, M. africnum, M. microti M. tuberculosis and M. bovis ➢ > 95% of pulmonary mycobacterioses Slow growth Person-to-person transmission

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Tuberculosis

M. tuberculosis: Pathologic features • •

•

• •

Gram positive pleomorphic rod Acid fast: ➢ Resists decolorization with acid alcohol Virulence related to cell wall ➢ No endotoxin or enzymes Caseous necrosis Caseating granuloma ➢ Central caseous necrosis ➢ Rim of histiocytes, giant cells

Figure 1-9-1

Caseous Necrosis [Figure 1-9-1]

Tuberculosis: Pathogenesis •

Inhaled bacteria [Figure 1-9-2] ➢ Mid to lower lung zones ➢ Ghon focus

Figure 1-9-2

Even though we tend to think of TB as an upper lobe disease, we inhale most bacteria into the mid and lower lung zones

The caseating granuloma is the hallmark of TB. The actively growing bacilli reside in the macrophages in the periphery

Figure 1-9-3

Physiologic Gradients-Airflow FRC Tuberculosis: Pathogenesis •

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• • • •

Inhaled bacteria ➢ Mid to lower lung zones ➢ Ghon focus Regional lymph node spread [Figures 1-9-3] ➢ Ranke complex Lymphatic/hematogenous dissemination Cell-mediated immunity Delayed hypersensitivity ➢ Caseous necrosis ➢ 2-10 weeks Healing

Tuberculosis

In primary tuberculosis the ineffective macrophages carry bacteria to regional lymph nodes where they proliferate and disseminate

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Tuberculosis: Pathogenesis •

•

•

Latent TB infection ➢ +PPD ➢ No active signs of infection Survival of organisms [Figures 1-9-4] ➢ Apical/posterior upper lobe ➢ Superior segment lower lobe ✧ Oxygen gradient ✧ Lymphatic gradient ✧ Bucket handle rib motion Active TB infection [Figures 1-9-5] ➢ 5% within 2 years ➢ 5-10% lifetime risk ➢ HIV: 50% within 2 years ➢ Pulmonary fibrotic lesions, underweight, silicosis, DM, renal failure, gastrectomy, jejunoileal bypass, transplantation, head and neck cancer, prolonged immunosuppressive therapy

Tuberculosis: Clinical features • • •

Primary TB Postprimary TB Disseminated TB

•

Asymptomatic 65% ➢ Nonspecific symptoms when present Progressive primary complex ➢ Fever, cough, hemoptysis, weight loss

The lymphatic gradient helps explain the upper lobe distribution of reactivation tuberculosis

Figure 1-9-5

Primary Tuberculosis: Clinical features •

Figure 1-9-4

Primary Tuberculosis: Radiologic features • • •

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Lymphadenopathy [Figure 1-9-6] ➢ Children 95%, young adults 43%, elderly 10% ➢ Right paratracheal, hilar ➢ Peripheral enhancement, central low-attenuation Atelectasis, overinflation [Figure 1-9-7] ➢ Children ➢ Anterior segements upper lobes ➢ Medial segment middle lobe Consolidation ➢ Unifocal 75% Figure 1-9-6 ➢ Segmental, lobar, multifocal ➢ Homogeneous, patchy, linear, nodular Pleural effusion ➢ Adults 38%, children 11%

Leung, Radiology 1999, Vol 210

The lymphatic gradient helps explain the upper lobe distribution of reactivation tuberculosis

Lymphadenopathy is hallmark of primary TB and is more common in children Chest Radiology

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Tuberculosis

Postprimary Tuberculosis: Clinical Features •

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Reactivation ➢ Fever, malaise, anorexia, weight loss, anorexia, night sweats ➢ Dyspnea, cough, chest pain, hemoptysis Active TB infection ➢ 5% within 2 years ➢ 5-10% lifetime risk ➢ HIV: 50% within 2 years ➢ Pulmonary fibrotic lesions, underweight, silicosis, DM, renal failure, gastrectomy, jejunoileal bypass, transplantation, head and neck cancer, prolonged immunosuppressive therapy

Figure 1-9-7

Postprimary Tuberculosis: Pathogenesis [Figures 1-9-8 and 1-9-9] • • •

Delayed hypersensitivity Liquifaction Cavitation ➢ Airway ➢ Vessel ➢ Pleura

Figure 1-9-8

The lymph nodes which surround airways may cause narrowing that results in atelectasis

Figure 1-9-9

Postprimary TB implies reactivation of dormant bacilli. It is characterized by tissue destruction

Cavitation and necrosis enables spread via the airway, blood stream or pleura

Postprimary Tuberculosis: Radiologic features • • • •

Consolidation 50-70% Cavitation 40-45% Nodules Airways involvement

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Postprimary Tuberculosis: Radiologic features •

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•

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Consolidation 50-70% ➢ Heterogeneous, nodular, linear ➢ Apical, posterior 85%, Superior segments 14% Cavitation 40-45% [Figure 1-9-10] ➢ Thin or thick walls, air-fluid levels 20% Nodules ➢ Tuberculoma ✧ SPN: variable borders, satellite lesions, upper lobes ➢ Endobronchial spread [Figures 1-9-11 to 1-9-13] ✧ Centrilobular, tree-in-bud, 100% by CT ➢ Hematogenous spread ✧ Miliary 1-3mm, random Airways involvement ➢ Bronchiectasis, bronchitis, airway narrowing

Figure 1-9-11

Figure 1-9-10

Cavitation implies a large number of bacilli speeds the progression of disease

Figure 1-9-12

Endobronchial spread leads to airways nodules Endobronchial spread leads to airways nodules

Figure 1-9-13

Postprimary Tuberculosis-Cavitation Thoracoplasty Oleothorax

Plumbage [Figure 1-9-14]

Postprimary Tuberculosis-Nodules Postprimary Tuberculosis-Airways

Endobronchial spread leads to airways nodules

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Tuberculosis

Postprimary Tuberculosis: Assessment of Activity • •

• •

Cannot discern activity from a single film Inactive disease ➢ radiographic stability ➢ 6mos Negative cultures Suggestive of active disease ➢ Cavitation ➢ Consolidation ➢ Ground glass ➢ Centrilobular opacities

Figure 1-9-14

Lee et al, Chest, 1996

Tuberculosis: Complications • •

• • •

End-stage disease Hemoptysis ➢ Bronchial arteries in chronic cavities ➢ Mycetoma ➢ Rassmussen (pulmonary artery) aneurysm Chest wall involvement Pericardial involvement Empyema ➢ BPF, empyema necessitatis

Images demonstrate broncho-esophageal fistula which was a complication of plumbage

Hemoptysis-Bronchial Artery Hemoptysis-Mycetoma End-Stage Lung

Tuberculosis-Chest Wall

Tuberculosis-Pericardial Tuberculosis: HIV/AIDS • • •

CD4>200 ➢ Well formed granulomas ➢ Upper lobe cavities, consolidation and nodules CD4<200 ➢ Poorly formed granulomas ➢ Adenopathy, consolidation and miliary disease CD4<60 ➢ No hypersensitivity reaction ➢ Organisms spread from GI tract ➢ Miliary Disease

Tuberculosis and AIDS

Tuberculosis and AIDS-Low CD4 Tuberculosis: Diagnosis •

• • •

Conventional methods ➢ Acid-fast smear: 1 day ➢ Culture: 1-2 weeks ➢ Identification: 2-3 weeks ➢ Drug susceptibility testing: 3-4 weeks Radiometric methods Polymerase chain reaction (PCR) HPLC

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Summary •

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Primary TB ➢ Consolidation ➢ Ipsilateral lymphadenopathy ➢ Pleural effusion Postprimary TB ➢ Consolidation ➢ Cavitation ➢ Apical/posterior upper lobe nodules ➢ Tracheobronchial spread

Tuberculosis Pre-Antibiotic Era References

General 1. Leung AN.Pulmonary tuberculosis: the essentials. Radiology. 1999 Feb;210(2):307-22

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Tuberculosis

Fungal Disease in the Thorax: Opportunistic and Primary Pathogens Jeffrey R. Galvin, MD

Fungal Disease in the Thorax: Overview •

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Opportunistic invaders ➢ Aspergillus species ➢ Candida ➢ Mucormycosis Primary pathogens ➢ Histoplasma capsulatum ➢ Blastomyces dermatitidis ➢ Coccidioides immitis

Opportunistic Invaders •

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Immunocompromised host ➢ Mucosal disruption ➢ Reduced cellular and/or humoral immunity Ubiquitous Lack dimorphism Multiple organisms may occur

Figure 1-10-1

Primary Pathogens • •

• • •

May infect healthy individuals Dimorphism ➢ Saprophytes in the soil ➢ Spores via germination Most disease mild or subclinical Fulminant or chronic disease may occur Specific geographic regions ➢ Endemic

Coccidioidomycosis Blastomycosis

Histoplasmosis

Histoplasmosis: Epidemiology and Ecology •

• •

The mycelial form of Histoplasmosis is found in soil that has been enriched with bird droppings. The fungus then releases conidia or spores

Endemic fungal disease ➢ Ohio, Mississippi and St. Lawrence river valleys ➢ Reported worldwide but relatively rare outside of the United States ➢ Infection rate up to 95% in endemic areas ➢ Point sources associated with aerosolization ✧ Earth moving, bird husbandry and spelunking Dimorphic fungus Clinical

Histoplasmosis: Epidemiology and Ecology • • •

Endemic fungal disease Dimorphic fungus ➢ Mycelial form in high nitrogen soil ✧ Guano from birds and bats ➢ Yeast within the infected host Clinica

Histoplasmosis [Figure 1-10-1] Fungal Diseases

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Histoplasmosis: Pathology [Figure 1-10-2] •

Early sequence of infection ➢ Mycelia produce micronidia ➢ Micronidia reach alveolar spaces ✧ 2-5 microns

Histoplasmosis: Pathology [Figure 1-10-3 and 1-10-4] •

•

Early sequence of infection ➢ Lymphocytes and macrophages replace polys ➢ Micronidia transform to conidia or spores ➢ Spores transform into budding yeast ➢ Macrophages phagocytose and kill yeast Late sequence of infection ➢ Lymphocyte-mediated cellular immunity ➢ Granulomatous inflammation ➢ Necrosis ➢ Fibrosis

The fungal spores are able to reach the alveolar level, bypassing the upper airway defenses because of their small size which is less than 5 microns

Histoplasmosis: Pathology •

Distinction from tuberculosis ➢ Histoplasmosis relatively benign ➢ Immunity to histoplasmosis short lived ✧ 20% lose immunity each year ✧ Continuous reinfection – Primary and postprimary not appropriate

Histoplasmosis: Clinical •

•

•

Asymptomatic ➢ 95-99% of infection in endemic areas ➢ Parenchymal opacities in 10-25% ➢ Small inoculum or prior infection (cellular immunity) and moderate inoculum Symptomatic ➢ Acute ✧ Moderate vs large inoculum ➢ Chronic ➢ Disseminated Late complications ➢ Histoplasmoma ➢ Broncholithiasis ➢ Mediastinal granuloma ➢ Mediastinal fibrosis

Histoplasmosis: Acute Clinical •

•

Signs and symptoms ➢ “Flulike”: fever, chills, cough ➢ Retrosternal pain ✧ Mediastinal lymph node involvement ➢ Erythema nodosum in women ➢ Arthralgia Shorter incubation with prior exposure

Histoplasmosis: Acute Radiology • • •

Poorly defined areas of consolidation ✧ Single or multiple Hilar lymph node enlargement Numerous discrete nodular shadows in heavy exposure ✧ 3-4 mm ✧ Symptoms precede radiographic change ✧ Nodules change to punctate calcifications

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From three to 5 days following inhalation the spores germinate and release yeast forms. The yeast within the alveoli are rapidly phagacytosed by macrophages

Lymphocytemediated cellular immunity develops at 1014 days controlling the infection through a necrotizing granulomatous response

Figure 1-10-2

Figure 1-10-3

Figure 1-10-4

Fungal Diseases

Acute Histoplasmosis [Figures 1-10-5]

Figure 1-10-5

Acute Histoplasmosis – large inoculum Acute Histoplasmosis [Figure 1-10-6]

Histoplasmosis: Chronic Pulmonary Histoplasmosis • • •

Emphysema and bullous disease a common predisposition Upper lobe predominance Two possible mechanisms ➢ Hypersensitivity reaction in preexisting emphysematous space ✧ Few organisms ✧ Colonization or minimal invasion ✧ Thick walled bulla filled with fluid may clear spontaneously ✧ Progressive loss of volume ➢ Similar to TB ✧ Fibrosis, cavitation and granulomatous inflammation

Acute histoplasmosis is associated with areas of consolidation and ipsilateral hilar and mediastinal enlargement

Chronic Histoplasmosis [Figure 1-10-7]

Figure 1-10-6

Histoplasmosis Disseminated •

Clinical ➢ Rare entity (1/100,000-1/500,000) ➢ Most patients immunocompromised ✧ 30% infants < 2 years ✧ 20% immunocompromised ✧ 50% apparently normal (transient compromise) ➢ Reduced macrophage function ✧ Parasitization of macrophages ✧ Intracellular survival and multiplication ➢ Radiology ✧ Miliary nodules (1-3 mm) ✧ 50% of disease associated with AIDS purely extrathoracic – Normal radiograph – Positive blood or bone marrow biopsy

Fungal Diseases

Bilateral soft tissue nodules imply a large innoculum. The nodules disappear over months leaving behind small calcific densities

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Disseminated Histoplasmosis [Figure 1-10-8]

Figure 1-10-7

Histoplasmosis: Late Complications • • • •

Histoplasmoma Broncholithiasis Mediastinal granuloma Mediastinal fibrosis

•

Solitary nodule (.5-3 cm) ➢ Sharply defined ➢ Smaller satellite lesions ➢ Central or diffuse calcification ✧ Diagnostic of benign lesion if less that 3 cm ➢ May increase in size ✧ Similar reaction to fibrosing mediastinitis Hilar calcification common on ipsilateral side Fungal nodules account for 30% of all solitary nodules 87% are less than 2.5 cm in diameter

Histoplasmosis: Histoplasmoma

• • •

Histoplasmoma [Figure 1-10-9] Broncholith

Histoplasmosis: Mediastinal granuloma • • •

Pathology ➢ Direct infection of hilar and mediastinal lymph nodes Clinical ➢ Often asypmtomatic with discovery of a mediastinal mass on chest radiograph ➢ SVC or esophageal obstruction less common Radiology ➢ Middle mediastinal mass ✧ Subcarinal or paratracheal ➢ Enhancing capsule with low attenuation center ➢ Mass may be low signal on T2 weighted MR because of fibrous tissue or calcification

Chronic histoplasmosis resembles post-primary TB but usually represents a hypersensitivity reaction in patients with emphysema

Figure 1-10-8

Disseminated Histoplasmosis presents with miliary nodules and macrophages filled with organisms

Figure 1-10-9

Histoplasmomas are the residua of a prior area of pneumonitis. They typically demonstrate concentric rings of calcification but may remain uncalcified especially in older individuals Chest Radiology

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Fungal Diseases

Mediastinal Granuloma [Figure 1-10-10]

Figure 1-10-10

Histoplasmosis: Fibrosing Mediastinitis •

•

Pathology ➢ Proliferation of acellular collagen and fibrous tissue within the mediastinum ➢ Most cases in the United States are an immunological response to H. capsulatum ✧ Focal form: paratracheal and subcarinal ✧ Calcification ➢ Idiopathic form ✧ Diffuse, infiltrating ✧ Noncalcified ✧ Multiple mediastinal compartments Clinical ➢ Signs and symptoms of obstruction to mediastinal structures ✧ Superior vena cava, pulmonary veins or arteries, central airway or esophagus

Mediastinal granuloma is the result of direct infection of mediastinal lymph nodes. Acutely the lymph nodes demonstrate low attenuation with an enhancing capsule

Figure 1-10-11

Fibrosing Mediastinitis [Figure 1-10-11] Blastomycosis

Blastomycosis: Epidemiology and Ecology •

•

•

Ecological niche [Figure 1-10-12] ➢ Difficult to establish ➢ Saprophyte in an unidentified resevoir within reach of man and dogs ➢ Survives only in wet soil with a high PH and high organic content ➢ Soil probably contaminated rather than the natural resevoir ➢ Point sources in dead and decaying material near rivers, streams and swamps Dimorphic fungus ➢ Mycelium in natural habitat ➢ Releases spores (conidia) into the air ➢ Budding yeast ✧ (8-15 microns) in vivo ✧ Broad based Clinical ➢ Less common than Histoplasmosis ➢ High risk of symptomatic disease although most cases are probably asymptomatic ➢ Males more commonly affected (3:1-15:1) ➢ Exposure in heavily wooded areas ➢ Variable course ✧ Symptoms of acute pneumonia – Abrupt onset, fever, chills, cough and pleuritic pain – Occasional rapid progression > Hematogenous dissemination: skin, bone and genitourinary tract > ARDS ✧ Chronic disease similar to tuberculosis

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Fibrosing mediastinitis due to h. capsulatum usually presents as a mediastinal mass associated with calcification

Figure 1-10-12

Blastomycosis is usually associated with activity in damp, wooded areas Chest Radiology

Blastomycosis: Pathology • • • • •

Initial inflammatory response is neutrophilic ➢ Small collections of cells to hundreds of milliliters of pus Rapidly followed by chronic inflammatory response ➢ Lymphocyte, histiocytes and plasma cells ➢ Langhans’ giant cells ➢ Granulomas Both responses may coexist ➢ Organisms more common in supperative area Progression ➢ Coalescence of patchy consolidation ➢ Airway perforation ➢ Cavitation Ulcerative bronchitis is common

Blastomycosis: Pathology • • • • •

Initial response is neutrophilic Chronic inflammatory response Both responses may coexist Progression ➢ Coalescence of patchy consolidation ➢ Airway perforation ➢ Cavitation Ulcerative bronchitis is common

Blastomycosis: Radiologic Manifestations •

• •

Consolidation most common ➢ Upper lobe 2:1 ➢ Rounded, ill-defined ➢ Masslike opacities ✧ Central or paramediastinal ✧ Carinoma mimic ✧ Solitary nodules ➢ Air bronchograms (88% CT) ➢ Cavitation Nodules ➢ Intermediate size ➢ Remote from consolidation ➢ Satellite lesions Miliary disease ➢ Hematogenous dissemination

Figure 1-10-13

Blastomycosis often with an upper lobe areas of masslike consolidation

Asymptomatic Mass [Figure 1-10-13]

Figure 1-10-14

Consolidation

Solitary Pulmonary Nodule Similar to Postprimary TB

Disseminated Disease [Figure 1-10-14] Mass and Dissemination

Blastomycosis: Treatment • • •

Pulmonary disease may be self-limited even if extensive Extrapulmonary disease requires treatment Amphotericin B IV or oral Keotconazole

Chest Radiology

Miliary disease may complicate infection with Blastomycosis 105

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Coccidioidomycosis [Figure 1-10-15]

Coccidioidomycosis: Epidemiology and Ecology • • •

Figure 1-10-15

Ecological niche Dimorphic fungus Clinical ➢ Acute Disease ✧ 100,000 new cases each year, essentially all in the southwest ✧ No racial, sex or age predilection in acute disease ✧ Most inhabitants of the endemic area infected in the first year of exposure ✧ Incubation period 10-16 days ✧ 60% are asymptomatic ✧ Symptoms when present include – Fever, pleuritic chest pain, cough – Valley Fever: allergic form with erythema nodosum or multiforme ✧ Severity of disease related to immune status Coccidioidomycosis is associated exclusively with the desert southwest and race – Filipinos, African Americans and Hispanics more likely to suffer dissemination ➢ Chronic Disease (5%) ✧ Symptoms persist without dissemination ✧ May be mildly immunocompromised ➢ Dissemination ✧ Rare occurrence – Immunocompromise – Non-Caucasian (Filipino, African American and Hispanic) – Early dissemination more common and carries a poor prognosis – Mortality rate or 50% even with early treatment

Coccidioidomycosis: Pathology • • • •

Lung the usual portal of entry Neutrophilic response early ➢ Especially in response to ruptured spherules ➢ Spherules ingested by macrophages Granulomatous and giant cell reaction follows Necrosis may occur

Coccidioidomycosis: Radiologic Manifestations •

•

Acute Disease ➢ Consolidation most common (75%) ✧ Usually unilateral, hilar or basal ✧ Segmental or lobar ➢ Multifocal nodular or patchy opacities ➢ Peribronchiolar thickening ➢ Hilar or mediastinal adenopathy (20%) ✧ Mediastinal adenopathy may herald dissemination ➢ Pleural effusion 20% ✧ Small, unilateral Coccidioidoma

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Acute Disease

Coccidioidoma [Figure 1-10-16]

Coccidioidomycosis: Radiologic Manifestations • •

•

Acute Disease Coccidioidoma ➢ Area of prior consolidation ➢ Round and well circumscribed ➢ 1.5cm average (up to 6cm) ➢ Usually single ➢ Marked enhancement with contrast CT ➢ Caseating chronic granulomatous inflammation Chronic Disease ➢ Cavitation ✧ Occur in areas of consolidation ✧ May be thin or thick walled ✧ Pneumothorax or empyema may result ➢ Chronic progressive pneumonia

Chronic Coccidioidomycosis [Figure 1-10-17] Coccidioidomycosis: Radiologic Manifestations •

•

Figure 1-10-16

A coccidioidoma is the resdua of an area of infectious consolidation

Chronic Disease ➢ Cavitation ➢ Chronic progressive pneumonia ✧ Indolent course similar to TB ✧ Biapical fibronodular lesions ✧ Hilar and mediastinal adenopathy ✧ Hilar retraction ✧ Persistently positive sputum ✧ High complement fixing antibody titer ✧ Non-Caucasian Disseminated Disease ➢ Miliary or reticular nodular pattern ✧ Less well circumscribed that TB ➢ Lymphadenopathy is common ➢ Pericardial effusion ➢ Skin, bone, meninges or upper genitourinary tract

Figure 1-10-17

Thin-walled cavities are suggestive of chronic coccidioidomycosis

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Fungal Diseases

Dissemination – Miliary Nodules Histoplasmosis

Acute Histoplasmosis [Figure 1-10-18]

Histoplasmosis – Solitary Nodule [Figures 1-10-19 and 1-10-20]

Histoplasmosis – large inoculum [Figure 1-10-21] Histoplasmosis [Figure 1-10-22]

Disseminated Histoplasmosis [Figure 1-10-23] Chronic Histoplasmosis [Figure 1-10-24] Fibrosing Mediastinitis [Figure 1-10-25] Blastomycosis

Figure 1-10-18

Coccidioidomycosis

Fungal Disease in the Thorax: Overview • •

Opportunistic invaders ➢ Aspergillus species ➢ Candida ➢ Mucormycosis Primary pathogens ➢ Histoplasma capsulatum ➢ Blastomyces dermatitidis ➢ Coccidioides immitis ➢ Paracoccidioides brasiliensis

Symptomatic patients with acute histoplasmosis may present with solitary or multifocal areas of consolidation and associated adenopathy

Figure 1-10-19

Figure 1-10-20

As the infection heals the inflammatory area rounds up and is surrounded by a fibrous capsule. Over a prolonged period calcification may develop in the nodule and regional lymph nodes

Fungal Diseases

The nodule may enlarge by adding fibrous tissue to the periphery

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Figure 1-10-21

Figure 1-10-22

If the patient inhales a large number of spores then numerous patches of consolidation may round up into well circumscribed nodules

Figure 1-10-23

As they heal the patient will be left with numerous calcifications

Figure 1-10-24

Patients with reduced immune function may present with hematogenous spread of disease and miliary nodules

Figure 1-10-25 Patients with underlying emphysema may develop chronic histoplasmosis which in most cases represents a hypersensitivity reaction to a small number of organisms

Fibrosing mediastinitis represents an exuberant fibrous reaction within the mediastinum which may result in damage to mediastinal structures. Most focal cases of fibrosing mediastintis in the United States are due to H. capsulatum

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Bronchogenic Carcinoma Jeffrey R. Galvin, MD A 20th Century Disaster

Histological Classification of Tumors

• • • •

World Health Organization Lung tumor editions ➢ 1967 ➢ 1981 ➢ 1999 ➢ 2004 Improve communication Consistent treatment Basis for comparative studies Prognosis

• • • • • •

Subclasses of adenomas Preinvasive lesions Adenocarcinoma Definition of BAC Neuroendocrine tumors Biphasic and pleomorphic tumors

• •

Changes in the 1999/2004 WHO

Incidence of Lung Cancer Gazdar, Semin Oncol, 1988

Histological Typing of Lung Tumors • • • •

Based on light microscopic criteria Classified by the best differentiated region Graded by the most poorly differentiated region Histologic heterogeneity is the “rule”

• • •

Prognosis: Small cell vs. non-small cell Stage determines prognosis in non small cell >95% of 1° lung tumors ➢ Adeno ➢ Squamous ➢ Large cell ➢ Small cell ➢ Combination of above

Histological Typing of Lung Tumors

Lung Cancer Demographics • • •

Most common cancer in males world-wide Leading cause of cancer mortality in women and men (United States) Mortality rates in women began increasing in 1935 and surpassed breast ca in 1987

Age-Adjusted Cancer Death Rates – Males vs Females Cancer 49: 1999

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Lung Cancer Etiology – Cigarette smoking • • •

85-90% of lung cancer deaths 25% of lung cancer in non-smokers attributed to passive smoke Risk related to: ➢ Number of cigarettes smoked ➢ Depth of inhalation ➢ Age at which smoking began

Clinical Presentation •

• • • •

Central tumors ➢ Cough ➢ Wheezing ➢ Hemoptysis ➢ Pneumonia Extrapulmonary invasion ➢ Pain ➢ Pancoast Syndrome ➢ SVC Syndrome Metastases Paraneoplastic Syndromes Asymptomatic 10%

Paraneoplastic Syndromes Cachexia, malaise and fever Ectopic hormone production ➢ ACTH ➢ ADH ➢ Hypercalcemia ➢ Clubbing and HPO ➢ Thrombotic endocarditis ➢ Non-bacterial • Migratory thrombophlebitis

• •

Lung Cancer and Clotting

Squamous Cell Carcinoma •

• • •

Terminology ➢ Squamous ✧ Flattened cells ➢ Epidermoid ✧ Mimics differentiation of the epidermis Rapid local growth Distant metastases later Strong association ➢ Cigarette smoking

Squamous Cell Carcinoma • •

•

Pancoast Syndrome Hyperparathyroidism ➢ Parathyroid-like substance Most common to present as radiographically occult

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Preinvasive Lesions: Squamous Dysplasia • • •

• •

Similar to cervical Ca Squamous metaplasia Progression ➢ Dysplastic epithelium Carcinoma in situ ➢ Full thickness dysplasia Precursor ➢ Invasive squamous cell Ca

Figure 1-11-1

Squamous Cell Carcinoma: Microscopic features •

• •

Individual cell keratinization ➢ Eosinophilia Keratin pearls ➢ Well differentiated tumors Intercellular bridges

Figure 1-11-2

Squamous Cell Carcinoma: Gross Features [Figure 1-11-1] •

• • •

Central lesion ➢ Polypoid, endobronchial, exophytic growth Central necrosis common Bronchial wall invasion ➢ Common ➢ Positive cytology Proximal growth ➢ Along bronchial mucosa

The majority of squamous cell cancers are central lesions

Squamous Cell Carcinoma: Radiologic Features [Figure 1-11-2] • •

•

• •

•

Hilar or perihilar mass Bronchial wall thickening ➢ Often focal Consolidation ➢ Must clear completely Atelectasis Peripheral nodule or mass ➢ 30% Cavitation

Squamous cell cancers are predominantly central and endobronchial as exemplified by this tomogram

Atelectasis [Figures 1-11-3 to 1-11-5]

Figure 1-11-3

Figure 1-11-4

Atelectasis in an adult smoker is lung cancer until proven otherwise Bonchogenic Carcinoma

Typical central squamous cell carcinoma 112

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Cavitation [Figure 1-11-6]

Figure 1-11-5

Pancoast Tumor: Superior Sulcus Tumor •

• • •

Characteristic pain ➢ 8th cervical ➢ 2nd thoracic trunk Horner’s Syndrome Destruction of bone Hand muscle atrophy

Pancoast, JAMA, 1992

Small Cell Lung Cancer • • • •

Rapid growth Considered metastatic at presentation Poorest survival Strongest association with cigarette smoking

Small Cell Lung Cancer •

• •

Golden’s S sign

Small cell carcinoma ➢ Pure histology Variant ➢ Combined Elimination ➢ Oat cell ➢ Intermediate type

WHO, 2004

Small Cell Lung Cancer: Microscopic Features • • •

• • • •

Small, uniform cells Scant cytoplasm Necrosis is common ➢ Often extensive >10 mitosis per 10 HPF ➢ Average 60-70 Neuroendocrine morphology Neuroendocrine markers ➢ 75% Light microscopy diagnosis

Figure 1-11-6

WHO, 2004

Cavitation is most common in squamous cell cancer Chest Radiology

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Small Cell Lung Cancer: Gross Features • • • • • • •

Figure 1-11-7

Large Central mass (90%) Bronchial compression No endobronchial lesion Proximal growth ➢ Along submucosa Extensive necrosis Hemorrhage

Small Cell Lung Cancer: Radiologic Features [Figures 1-11-7 and 1-11-8]

• • •

•

Hilar or perihilar mass Mediastinal adenopathy Primary tumor ➢ Rarely evident Cavitation ➢ Extremely rare

Small cell tends to spread along the peribronchovascular lymphatics without endobronchial invasion

Small Cell Lung Cancer • • • •

Cushing Syndrome SIADH Eaton Lambert Most common cause ➢ SVC Syndrome

Figure 1-11-8

Small Cell Lung Cancer: Therapy • • • •

Response to chemotherapy and radiotherapy Untreated: median survival 2-4 months Treated: median survival 9-18 months Limited stage – 15-25% survive 2 years

• • • • •

Rapid growth Location ➢ Segmental ➢ Subsegmental Early metastases Poor prognosis Strong association with cigarette smoking

• • • • •

Large cells Prominent nucleoli Poorly differentiated Diagnosis of exclusion Neuroendocrine features

•

Large and bulky ➢ Greater that 3 cm Soft Large areas of necrosis

Large Cell Carcinoma

Large Cell Carcinoma: Microscopic Features

Small cell most commonly presents as a mediastinal mass

Large Cell Carcinoma: Gross Features • •

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Large Cell Carcinoma: Radiologic Features • •

Usually peripheral 70% of tumors ➢ > 4 cm at presentation

Figure 1-11-9

Large Cell Carcinoma [Figure 1-11-9] Adenocarcinoma: Etiology •

Cigarette smoke causatively linked to lung cancer ➢ 1950 ➢ Squamous cell 18X’s Adeno ➢ Squamous cell: central

Adenocarcinoma: Etiology [Figure 1-11-10]

• •

•

•

Cigarette smoke causatively linked to lung cancer Adenocarcinoma most common ➢ Peripheral Filtered low-yield cigarettes ➢ Smaller particles ➢ Reduced nicotine ➢ Greater depth of puffs ➢ Increased number of puffs ➢ N-nitrosamines Other factors - 10% ➢ Passive smoke ➢ Particulates ➢ Cooking practices

Large cell cancers are commonly necrotic but rarely cavitate

Figure 1-11-10

Adenocarcinoma: Microscopic Features • • •

• • • •

Glands Papillary structures Mucin ➢ Intracellular ➢ Extracellular Prominent nucleoli Moderate cytoplasm Desmoplastic reaction “Scar carcinoma” ➢ Rare!

Adenocarcinoma: Radiologic Features • • • • • •

• •

Peripheral (75%) Solitary mass or nodule Upper lobes 3:2 Right lung 3:2 Lobulated Borders ➢ Ill-defined ➢ Well-defined Spiculated Obstructive pneumonitis (25%)

Chest Radiology

Adenocarinoma, the most common lung cancer is predominantly a peripheral lesion

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Spiculation and Retraction [Figure 1-11-11]

Figure 1-11-11

Scar Carcinoma [Figure 1-11-12] Necrosis

Air Bronchogram [Figure 1-11-13]

Adenocarinomas are commonly spiculated peripheral nodules

Figure 1-11-12

In scar carcinomas the scar is usually a reaction to the malignancy

Figure 1-11-13

Air bronchograms are commonly seen in adenocarcinomas Bonchogenic Carcinoma

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Slow Growth

Atypical Adenomatous Hyperplasia: Preinvasive lesion [Figures 1-11-14 and 1-11-15]

•

• • • •

Atypical cuboidal epithelium ➢ Lining alveoli ➢ Lining bronchioles Found in lung cancer resection specimens Probable precursor ➢ BAC ➢ Invasive adenocarcinoma Patchy ground glass 5mm or less

Figure 1-11-14

Kitamura, AJCP, 1999

AAH

Bronchioloalveolar Carcinoma: Microscopic Features • •

• •

Lepidic growth pattern No evidence ➢ Stromal invasion ➢ Vascular invasion ➢ Pleural invasion Diagnosis cannot be made on a small biopsy Requires thorough sampling of resected specimen

WHO, 1999

Bronchioloalveolar Carcinoma: Mucinous Type • • •

Alveolar spaces distended with mucin Aerogenous spread is common Multifocal consolidation

•

Alveoli lined with ➢ Clara cells ➢ Type II cells Central alveolar fibrosis ➢ Common Close association ➢ AAH

Bronchioloalveolar Carcinoma: Non-mucinous Type • •

The diagnosis of AAH may be difficult and the differentiation form a small BAC may be problematic

Figure 1-11-15

In AAH the architecture of the lung is not disturbed

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Bronchioloalveolar Carcinoma: Gross Features [Figure 1-11-16]

•

•

Consolidation ➢ Focal ➢ Multifocal Architecture ➢ Preserved

Figure 1-11-16

Bronchioloalveolar Carcinoma: Radiologic Features [Figure 1-11-17]

•

• • • •

Solitary nodule ➢ Excellent prognosis ➢ Resection Consolidation ➢ May be multifocal Ground glass Multiple nodules May cavitate?

Noguchi, Cancer 1995

Bronchioloalveolar Carcinoma BAC Recurrence

BAC vs Adenocarcinoma

BAC - Adenocarcinoma: CT, Histology and Doubling Time • • • •

Type A Ground glass Localized BAC Doubling time ➢ Mean: 880 days ➢ Range: 662-1486 days

BAC often presents as as area of consolidation

Figure 1-11-17

Aoki et al, AJR, 2000

BAC - Adenocarcinoma: CT, Histology and Doubling Time • • • • •

Type B Ground glass Focal increased attenuation Localized BAC Doubling time ➢ Mean: 880 days ➢ Range: 662-1486 days

Aoki et al, AJR, 2000

BAC - Adenocarcinoma: CT, Histology and Doubling Time • • • • • •

•

Type C Solid attenuation Focal ground glass Spiculation Pleural tag Localized BAC ➢ Active fibroblastic proliferation Doubling time ➢ Range: 42-1346 days

BAC usually presents as an area of consolidation

Aoki et al, AJR, 2000

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BAC - Adenocarcinoma: CT, Histology and Doubling Time • • • • • •

Type D Solid attenuation only Spiculation Pleural tag Poorly differentiated adenocarcinoma Doubling time ➢ Mean: 252 days ➢ Range: 124-402 days

Figure 1-11-18

Aoki et al, AJR, 2000

AdenoCa Appearance and Prognosis Aoki et al, AJR, 2001

Adenocarcinoma [Figure 1-11-18]

One form of adenocarcinoma begins as an invasive process and presents with a solid nodule

BAC

AAH [Figure 1-11-19] BAC [Figure 1-11-20]

Figure 1-11-20

Figure 1-11-19

The precursor lesion to BAC is AAH

Adenocarcinoma - BAC Prognosis [Figure 1-11-21]

BAC demonstrates lepidic growth and presents as an area of ground glass and/or consolidation

Figure 1-11-21

Survival decreases with increasing amount of consolidation and less ground glass opacity Chest Radiology

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References

General 1. Travis W, Colby T, Shimasato Y, Brambilla E. Histological Typing of Lung and Pleural Tumors., International Classification of Tumors. Third ed. Berlin: Springer Verlag, 1999. 2. Colby T, Koss M, Travis W. Tumors of the Lower Respiratory Tract, Atlas of Tumor Pathology. Third ed. Washington, DC: Armed Forces Institute of Pathology, 1999. 3. Travis WD, Brambilla E, et al: Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (WHO Classification of Tumours), IARC Press, 2004 (Oxford). 4. Patel AM, Peters SG. Clinical manifestations of lung cancer Mayo Clin Proc 1993; 68(3):273-7. 5. Davila DG, Williams DE. The etiology of lung cancer. Mayo Clin Proc 1993; 68(2): 170-82. 6. Travis WD, Lubin J, Ries L, Devesa S. United States lung carcinoma incidence trends: declining for most histologic types among males, increasing among females. Cancer 1996; 77(12):2464-70. 7. Travis WD, Travis LB, Devesa SS. Lung cancer [published erratum appears in Cancer 1995 Jun 15;75(12):2979]. Cancer 1995; 75(1 Suppl):191-2O2. 8. Pisani RJ. Bronchogenic carcinoma: immunologic aspects. Mayo Clin Proc 1993; 68(4):386-92. 9. Whitesell PL, Drage CW. Occupational lung cancer Mayo Clin Proc 1993; 68(2):1 83-8. 10. Patel AM, Davila DG, Peters SG. Paraneoplastic syndromes associated with lung cancer [see comments]. Mayo Clin Proc 1993; 68(3):278-87. 11. Morabia A, Wynder EL. Cigarette smoking and lung cancer cell types. Cancer 1991; 68(9):2074-8. 12. Ko YC, Lee CH, Chen MJ, Huang CC, Chang WY, Lin HJ, Wang HZ, Chang PY. Risk factors for primary lung cancer among non-smoking women in Taiwan. Int J Epidemiol 1997; 26(1):24-31. 13. Kitamura H, Kameda Y, Ito T, Hayashi H. Atypical adenomatous hyperplasia of the lung. Implications for the pathogenesis of peripheral lung adenocarcinoma [see comments]. Am J CIin Pathol 1999; 111(5):610-22. 14. Karsell PR, McDougall JC. Diagnostic tests for lung cancer. Mayo Clin Proc 1993; 68(3):288-96. 15. Dalager NA, Pickle LW, Mason TJ, Correa P, Fontham E, Stemhagen A, Buffler PA, Ziegler RG, Fraumeni JF, Jr. The relation of passive smoking to lung cancer Cancer Res 1986; 46(9):4808-11. 16. Charloux A, Hedelin G, Dietemann A, Ifoundza T, Roeslin N, Pauli G, Quoix E. Prognostic value of histology in patients with non-small cell lung cancer. Lung Cancer 1997; 17(1):123-34. 17. Charloux A, Ouoix E, Wolkove N, Small D, Pauli G, Kreisman H. The increasing incidence of lung adenocarcinoma: reality or artefact? A review of the epidemiology of lung adenocarcinoma. Int J Epidemiol 1997; 26(1 ):14-23. 18. Muller NL, Miller RR. Neuroendocrine carcinomas of the lung. Semin Roentgenol 1990; 25(1 ):96-1 04. 19. Travis WD, Rush W, Flieder DB, Falk E=R, Fleming MV, Gal AA, Koss MN. Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid. Am J Surg Pathol 1998; 22(8):934-44. 20. Hardy J, Smith I, Cherryman G, Vincent M, Judson I, Perren T, Williams M. The value of computed tomographic (CT) scan surveillance in the detection and management of brain metastases in patients with small cell lung cancer Br J Cancer 1990; 62(4):684-6. 21. Sone S, Takashima S, Li F, Yang Z, Honda T, Maruyama Y, Hasegawa M, Yamanda T, Kubo K, Hanamura K, Asakura K. Mass screening for lung cancer with mobile spiral computed tomography scanner [see comments]. Lancet 1998; 351 (9111):1242-5.

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Chest Seminar 1

Jeffrey R. Galvin, MD Case 1: This 57 year old male with a long history of smoking cigarettes. He now complains of a chronic cough

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Case 2: This 20 year old Caucasian female presented 7 years prior to the current admission with sudden onset of shortness of breath. The original chest radiograph revealed a pneumothorax. The patient now presents with increasing shortness of breath.

Chest Seminar 1

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Case 3: This 58 year old Caucasion female presented with a one month history of hemoptysis

Chest Radiology

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Case 4: This 38 year old African American female presented with a history of chronic asthma and increasing cough and shortness of breath

Chest Seminar 1

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Case 5: This 72 year old Caucasian female presented with cough and occasional fever. She was treated intermittently with antibiotics for 6 months. Open biopsy was obtained because of progressive symptoms.

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Chest Seminar 2

Jeffrey R. Galvin, MD Case 1: 15 year old female was admitted to the ER with an overdose of Nefazodone and other unknown pills. Activated charcoal was administered after which she developed vomiting and gagging. Respiratory distress required intubation. Bronchial lavage was performed and immunosuppressants were started. The patient developed progressive dyspnea and obstructive pulmonary functions over the next 6 months. Bilateral lung transplantation was done 19 months later.

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Case 2: 59 year old Caucasian female with history of breast cancer 12 years prior to admission and local recurrence treated with radiation 7 years later. She presented with a right lung mass.

Chest Radiology

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Case 3: 46 year old Caucasian male with long standing year history of shortness of breath. He presents with a 3 months of worsening dyspnea.

He demonstrated a mild leukocytosis and an increase in serum LDH.

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Case 4: 57 year old Caucasian male with a new history of cough and a new abnormality on chest radiograph. A chest CT was done based on the abnormalities found on the chest radiograph. A bronchoscopy was performed.

Chest Radiology

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Case 5: 61 year old female worked as a hospital storage room manager.

She presented to the ER complaining of fatigue, increasing shortness of breath, chest pain, and cough productive of blood tinged sputum.

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Pulmonary Hypertension Aletta Ann Frazier, MD Key Points • • •

Radiologic findings distinguish precapillary (arterial) from postcapillary (venous) pulmonary hypertension Idiopathic and secondary conditions are included in the differential diagnosis Vascular histopathology and secondary cardiac changes are often reflected in the radiology of pulmonary hypertension

Precapillary Pulmonary Circulation

[Figure 1-14-1]

Figure 1-14-1

Precapillary (arterial) circulation and vascular anatomy. Arterial vessels accompany the dichotomously branching airways of the lung

Postcapillary Pulmonary Circulation [Figure 1-14-2]

Figure 1-14-2

Postcapillary (venous) circulation drains the capillary beds of the alveoli. Veins and venules course back to the left atrium within interlobular septa

Normal Pulmonary Circulation • • •

Low pressure system with high degree of capacitance (recruitment and distension) Less than one tenth the resistance to flow in comparison to systemic circulation (low vasomotor tone) Right ventricle expends minimal energy to perfuse the pulmonary vascular bed

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Precapillary (Arterial) Pulmonary Hypertension • • • •

Insidious: dyspnea, chest pain, syncope Right heart pressure overload develops late Imaging reveals pulmonary HTN & clues to etiology NIH Criteria (cardiac cath): Mean PA pressure > 25 mm Hg at rest (normal 10)

• • • •

PV regurgitation RV hypertrophy & enlargement TV regurgitation & RAE Dilated IVC, hepatic veins

Cor Pulmonale… the best predictor of clinical outcome

Figure 1-14-3

Normal Heart vs. Cor Pulmonale

American College of Chest Physicians: “Venice Classification” • • • • •

PAH PH with left-sided heart dz PH with lung dz, hypoxemia PH due to thromboembolic dz Miscellaneous

Simonneau G. et al. J Am Coll Cardiol. 2004 Jun 16;43 (12 Suppl S):5S-12S.

Pulmonary Hypertension: Precapillary Etiologies • •

Idiopathic Secondary ➢ Chronic thromboembolic disease ➢ Sickle cell disease ➢ Eisenmenger physiology ➢ Mediastinal fibrosis ➢ Connective tissue disease ➢ Chronic hypoxia (COPD, IPF)

Photomicrograph demonstrates a muscular artery (adjacent to airway) narrowed by medial hypertrophy and obstructed by intravascular thrombus

Pulmonary Arterial Hypertension: Histology [Figure 1-14-3] • • • •

Medial hypertrophy Intimal proliferation Thrombosis Arteritis

• • • • •

Dilated central arteries Pruning of peripheral vessels Mosaic perfusion Cor pulmonale PA atherosclerosis

Precapillary Hypertension: Imaging

Idiopathic Pulmonary Hypertension [Figures 1-14-4 to 1-14-6]

Figure 1-14-4

Radiographic features of pulmonary hypertension: enlarged main pulmonary artery, dilated central hilar vessels, and peripheral oligemia

Figure 1-14-6

Figure 1-14-5

CT criteria for enlarged main PA in precapillary pulmonary hypertension: transverse diameter exceeds 29 mm Pulmonary Hypertension

132

CT manifestations of cor pulmonale: dilated RA and RV, thickened anterior RV wall, and flattened interventricular septum Chest Radiology

Cor Pulmonale • •

Figure 1-14-7

Axial White Blood Cine Sagittal White Blood Cine

Courtesy Laura Heyneman, MD

Idiopathic Pulmonary Hypertension • •

•

•

Mean age 45, F>M (3:1) 6% “familial” ➢ Autosomal dominant, incomplete penetrance Associations ➢ HIV infection ➢ Appetite suppressants ➢ Cocaine abuse ➢ Chronic liver disease Mean survival 2.8 years without treatment

Longstanding uncorrected ASD with acquired Eisenmenger physiology in 35 y.o. female [Courtesy of Melissa Rosado de Christenson, MD]

Atrial Septal Defect [Figures 1-14-7 to 1-14-9]

Figure 1-14-9

Figure 1-14-8

Lung specimen radiograph demonstrates calcified atherosclerotic plaques in the main PA [Courtesy of Melissa Rosado de Christenson, MD]

Vascular pruning pattern [Courtesy of Melissa Rosado de Christenson, MD]

Eisenmenger Physiology •

• •

Congenital L-to-R shunt ➢ VSD, ASD, PDA ➢ Endocardial Cushion Defect Shunt reversal (R-to-L) follows sustained elevation in PVR PAH irreversible & requires lung transplantation

Chronic Thromboembolic Disease [Figures 1-14-10 to 1-14-12]

Figure 1-14-10

Chronic Thromboembolic Disease • • • • •

Approx 4% of cases acute PE; presents within 2 years Symptomatic with >60% vascular bed occlusion 5-year survival rate <35% (without surgery) V/Q scan - high probability (helps to exclude IPH) CT ➢ Enlarged central PA ➢ Eccentric & linear filling defects +/- calcification ➢ Abrupt cut-offs (pruning) ➢ Bronchial arteries (50% of cases) ➢ Mosaic perfusion ➢ Pleural tags (healed infarcts)

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Broad-based intravascular soft tissue density in the right PA, combined with multiple bronchial arterial collateral vessels, suggests CTEPH Pulmonary Hypertension

Chronic Thromboembolic Disease •

•

Figure 1-14-11

CT Angio ➢ 94-100% sensitivity, 96-98% specificity for CTEPH ➢ More sensitive than PA angio for proximal disease ➢ Non-invasive modality for pre- and post-op assessment ➢ Multiplanar & curved multiplanar reconstructions further characterize disease extent Cardiac MR ➢ Cine imaging ✧ Right heart function ➢ Phase-contrast imaging ✧ low velocities in L & R PA’s ✧ shunt vol from bronchial arteries to pulmonary venous circulation ✧ R-to-L shunt via patent foramen ovale

“Recanalized” Chronic Thrombus “Organizing” Chronic Thrombus

Pulmonary Thromboendarterectomy • • • • •

15-30% CTEPH patients are candidates Thrombi from main to segmental or subsegmental level Intima & superficial media removed w/ thrombi Operative mortality 8-23% Improved long term survival - 75% at six years

Other thromboembolic materials Intravenous Talcosis

Chronic thromboembolic disease: mural-based soft tissue masses and calcium in the lumen of right main PA

• • •

Chronic IV injection of crushed tablets (Methadone, amphetamines) Thrombogenic pharmaceutical binding agent: magnesium silicate Granulomas coalesce into birefringent particles

•

EARLY ➢ Diffuse micronodular opacities LATE ➢ Fibrosis ➢ High density perihilar masses ➢ Emphysema

Figure 1-14-12

Intravenous Talcosis: Imaging •

Postcapillary Pulmonary Circulation

Pulmonary Hypertension: Postcapillary Etiologies •

•

Mosaic attenuation reflects geographic variations in blood flow

Idiopathic ➢ Pulmonary veno-occlusive disease (PVOD) ➢ Pulmonary capillary hemangiomatosis (PCH) Secondary ➢ Mitral valve stenosis ➢ Left ventricular failure ➢ Mediastinal fibrosis ➢ Left atrial mass / thrombus ➢ Venous constriction / invasion by tumor

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Pulmonary Venous Hypertension

Figure 1-14-13

• • • • • • • • • •

Acute or chronic onset Elevated wedge pressures (with exceptions) PAH - secondary Venous dilatation Venous “arterialization” Septal edema, fibrosis + Interlobular septa Subpleural thickening Ground glass opacities Pleural effusion

• • •

Capillary proliferation & dilatation Venous medial hypertrophy & intimal proliferation Recanalized thrombus in veins & venules

• • • •

Young adults (M:F is 2:1) Pediatric in 1/3 of cases Fatal within 3-5 years Associations ➢ Chemotherapy ➢ Prior viremia ➢ HIV ➢ ? Toxic exposure Current Debate ➢ Separate entities? ➢ Contiguous spectrum of injury? ➢ Cause-effect scenario?

PCH/PVOD PCH/PVOD

•

PVOD, a rare cause of postcapillary pulmonary hypertension: prominent interlobular septa and subpleural edema

PCH/PVOD [Figure 1-14-13] • •

Difficult to discern from primary PAH clinically Vasodilators contraindicated: severe pulmonary edema

• • •

Difficult to discern from primary PAH clinically Vasodilators contraindicated: severe pulmonary edema CT clues ➢ Septal lines ➢ GG nodules (+/-) ➢ Normal left atrium

Figure 1-4-14

PVOD/PCH

Mediastinal Fibrosis [Figure 1-14-14] Mediastinal Fibrosis: Imaging • • • •

Mediastinal contours abnormal Coarse calcium Soft tissue replaces mediastinal fat Constriction, encasement of mediastinal structures Two coronal CT reconstructions show mediastinal fibrosis constricting pulmonary venous drainage at their entrance to left atrium, thereby creating unilateral postcapillary pulmonary hypertension. (Note that precapiillary hypertension is also evident: the main PA is dilated and there is extrinsic compression of the right PA by mediastinal fibrosis)

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Mitral Stenosis

Diagnostic Strategy - Recommended Imaging Studies by ACCP • • •

CXR Echocardiography with Doppler V/Q (if CTEPH suspected) ➢ PA gram if positive (resectability)

ACCP Evidence-based Clinical Practice Guidelines. Chest 2004;126:14S-34S

Diagnostic Strategy - Imaging Studies •

•

From the ACCP: ➢ CXR ➢ Echocardiography with Doppler ➢ V/Q We also suggest: ➢ Chest CT/CTA ✧ Precapillary vs. postcapillary origin ✧ Clues to underlying etiology ➢ MRI ✧ Cardiac anatomy/function ✧ Future: hemodynamics of lung perfusion

Go With the Flow …. •

•

Pulmonary Arterial Hypertension ➢ Enlarged central and hilar vessels ➢ Pruned peripheral vessels ➢ Mosaic perfusion ➢ Por pulmonale ➢ PA atherosclerosis Pulmonary Venous Hypertension ➢ Septal lines ➢ Smooth pleural thickening ➢ Ground glass opacities ➢ Pleural effusion

References 1.

Bergin CJ, Rios G, King MA, Belezzuoli E, Luna J, Auger WR. Accuracy of high-resolution CT in identifying chronic pulmonary thromboembolic disease. AJR Am J Roentgenol 1996; 166:1371-1377. 2. Benjamin MS, Drucker EA, Mcloud TC, Shepard JO. Small pulmonary nodules: Detection at chest CT and outcome. Radiology 2003; 226:489-493. 3. Botticelli JT, Schlueter DP, Lange RL. Pulmonary venous and arterial hypertension due to chronic fibrous mediastinitis. Hemodynamics and pulmonary function. Circulation 1966; 33:862-871. 4. Burke AP, Virmani R. Mini-symposium: Pulmonary pathology: Evaluation of pulmonary hypertension in biopsies of the lung. Current Diagnostic Pathology 1996; 3:14-26. 5. Jones AT, Hansell DM, Evans TW. Quantifying pulmonary perfusion in primary pulmonary hypertension using electron-beam computed tomography. Eur Respir J 2004; 23:202-207. 6. King MA, Ysrael M, Bergin CJ. Chronic thromboembolic pulmonary hypertension: CT findings. AJR Am J Roentgenol 1998; 170:955-960. 7. Krowka MJ. Pulmonary hypertension: diagnostics and therapeutics. Mayo Clin Proc 2000; 75:625-630. 8. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:14S-34S. 9. Maltby JD, Gouverne ML. CT findings in pulmonary venoocclusive disease. J Comput Assist Tomogr 1984; 8:758-761. 10. Ng CS, Wells AU, Padley SP. A CT sign of chronic pulmonary arterial hypertension: the ratio of main pulmonary artery to aortic diameter. J Thorac Imaging 1999; 14:270-278. 11. Primack SL, Muller NL, Mayo JR, Remy-Jardin M, Remy J. Pulmonary parenchymal abnormalities of vascular origin: high-resolution CT findings. Radiographics 1994; 14:739-746. 12. Randall PA, Heitzman ER, Bull MJ, et al. Pulmonary arterial hypertension: a contemporary review. Radiographics 1989; 9:905-927. Pulmonary Hypertension

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13. Remy-Jardin M, Remy J, Louvegny S, Artaud D, Deschildre F, Duhamel A. Airway changes in chronic pulmonary embolism: CT findings in 33 patients. Radiology 1997; 203:355-360. 14. Resten A, Maitre S, Humbert M, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol 2004; 183:65-70. 15. Schoepf UJ, Costello P. Multidetector-row CT imaging of pulmonary embolism. Semin Roentgenol 2003; 38:106114. 16. Sherrick AD, Swensen SJ, Hartman TE. Mosaic pattern of lung attenuation on CT scans: frequency among patients with pulmonary artery hypertension of different causes. AJR Am J Roentgenol 1997; 169:79-82. 17. Simonneau G et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004 Jun 16;43 (12 Suppl S): 5S-12S. 18. Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Medical College of Wisconsin Lung Transplant Group. Chest 1998; 113:1250-1256. 19. Worthy SA, Muller NL, Hartman TE, Swensen SJ, Padley SP, Hansell DM. Mosaic attenuation pattern on thinsection CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause. Radiology 1997; 205:465-470.

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Pulmonary Metastases Aletta Ann Frazier, MD Key Points • • •

The pathogenesis of pulmonary metastases is complex The spectrum of radiological manifestations reflects pathways of spread Many extrathoracic malignancies produce characteristic radiologic patterns of pulmonary metastases

Metastatic Disease to the Lung • • •

Most common lung neoplasm Incidence: 20-55% of patients dying from extrathoracic malignancy Lung is the ONLY site of metastatic disease in 15-25% of these patients

• • • • •

Pulmonary & bronchial arteries Pulmonary & pleural lymphatics Thoracic duct Airways Transdiaphragmatic lymphatics

• • • • • • • •

Breast Colon Uterus Kidney Prostate Oropharynx Stomach Pancreas

• • • • • • •

Choriocarcinoma Osteosarcoma Testicular tumors Melanoma Ewing’s sarcoma Thyroid carcinoma Kaposi’s sarcoma

•

Certain tumors seed the lung directly, others first drain via another filtration organ (bone or liver) Systemic venous drainage directly to lung ➢ Melanoma ➢ Sarcomas ➢ Choriocarcinoma ➢ Thyroid ➢ Kidney, Testes, Adrenal Gland ➢ Oropharynx Venous drainage via liver ➢ Colon ➢ Pancreas ➢ Stomach Venous drainage via bone ➢ Prostate Dual venous drainage (simultaneous seeding) ➢ Kidney, Bladder, Ureters ➢ Uterus, Cervix

Metastatic Disease to the Lung:Routes of Spread

What are the most likely primary extrathoracic malignancies?

Which malignancies are the most highly predisposed?

Principle: “Generalizing Sites” •

• •

•

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•

➢ Anus, Rectum Complex venous (and lymphatic) drainage ➢Breast

Pathogenesis of Hematogenous Metastases [Figure 1-15-1] • • •

Tumor cells penetrate draining venules Enter systemic venous circulation Filtered by pulmonary arterial circulation

Pathogenesis of Hematogenous Metastases

Figure 1-15-1

[Figure 1-15-2]

• • •

Adherence, extravasation in distal arterioles Expansile growth in interstitium and alveoli Vascularization ➢ Pulmonary arteries ➢ Bronchial circulation ➢ Transpleural collaterals

Parenchymal Nodules: Histology • • • •

Well-defined Homogeneous cell population Adjacent to arteries and arterioles Alveolar septa compressed or obliterated

• • • • •

Rounded, coalescent or multilobulated Multiple Peripheral, basilar Variable sizes Mixed areas of viability, necrosis, hemorrhage

•

High sensitivity ➢ 95% for nodules >1cm ➢ 91% for nodules .5-1cm Low specificity (60% in 40-65 y.o. adults) ➢ intrapulmonary lymph nodes ➢ granulomatous diseases ✧ sarcoidosis ✧ silicosis ➢ amyloidosis ➢ infection

Nodular Metastases

Hematogenous metastases arise from tumor cells which penetrate vessels and lymphatics at the primary site, and are transported to the right heart via the systemic venous circulation

Parenchymal Nodules: Multidetector Chest CT •

Figure 1-15-2

Bloodborne tumor cells arrest in distal arterioles of the pulmonary circulation, extravasate into the interstitium, and establish nodules by expansile growth

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Parenchymal Nodular Metastases: [Figure 1-15-3] HRCT-Pathologic Correlation: Nodules <1cm • • • •

Peribronchovascular (12%) Periseptal (28%) Intermediate (68%) Angiocentric (76%) ➢ Directly-centered on feeding vessel (18%) ➢ Eccentric to feeding vessel (58%)

Figure 1-15-3

Hirakata et al. AJR 1993;161:37-43

Parenchymal Nodules: [Figure 1-15-4] Imaging Features, Chest CT • • • • • • •

Multiple Peripheral, basilar Variable in size “Random” - eccentrically located between BVB & interlobular septa Occasionally angiocentric Less commonly - “cannonball” or miliary Rarely ➢ cavitary ➢ calcified ➢ solitary ➢ ground glass halo (hemorrhagic) ✧ angiosarcoma ✧ choriocarcinoma ✧ post therapy

Figure 1-15-4

Secondary pulmonary lobule: hematogenous metastases may be angiocentric but are random with respect to the secondary pulmonary lobular architecture

Figure 1-15-5

Metastatic colon carcinoma in middle aged male: variable-sized nodules are random, peripheral, occasionally angiocentric in location

“Cannonball” Metastases [Figures 1-15-5 and 1-15-6] • • • •

Colorectal carcinoma Renal cell carcinoma Sarcomas Melanoma

Cannonball metastases (gross lung) in young adult male with soft tissue sarcoma Pulmonary Metastases

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Micronodular (Miliary) Metastases [Figure 1-15-7] •

• •

Malignancies ➢ Thyroid CA (papillary) ➢ Choriocarcinoma Opacities may persist post-treatment (“sterile”) DDX ➢ Miliary tuberculosis ➢ Viral pneumonia ➢ Sarcoidosis

Figure 1-15-6

Pulmonary Metastases: Unusual Manifestations • • • • •

Cavitary, calcified or solitary pulmonary nodules Lymphangitic carcinomatosis Tumor thromboembolism Endobronchial metastases Pleural-based metastases

• •

Incidence 4% (vs. 9% of lung primaries) Malignancies ➢ Squamous cell neoplasms (head and neck; cervix) - 69% ➢ Adenocarcinomas (colon, breast) - 31% ➢ Sarcomas (bone) - spontaneous ptx Wall thickness NOT indicative of benignity DDX ➢ septic emboli ➢ vasculitis ➢ collagen vascular disease

Cavitation in Metastases [Figure 1-15-8]

• •

Figure 1-15-7

Cannonball metastases in a young adult male with a soft tissue sarcoma (scout; axial lung and mediastinal CT images)

Figure 1-15-8

Micronodular metastases in middle aged female with thyroid cancer

Cavitary metastases in elderly male with oropharyngeal cancer

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Calcification in Metastases [Figure 1-15-9] •

• •

Figure 1-15-9

Malignancies ➢ Osteosarcoma, chondrosarcoma, and synovial sarcomas ➢ Papillary/mucinous adenocarcinomas (ovary, thyroid, GI) Post-chemotherapy or post-radiation Variable content ➢ Osteoid matrix ➢ Dystrophic calcification ➢ Psammoma body formation

Solitary Metastasis • • •

Unusual: 1-28% of all metastatic lesions 3-10% of all SPNs are solitary metastases Variable margins ➢ well-defined ➢ multilobulated ➢ spiculated

Calcified metastases in middle-aged female with ovarian cancer

Solitary Metastasis vs. Lung Primary •

“In patients with known primary malignancies and single parenchymal nodules, the overall incidence of second primary lung carcinoma is greater than that of solitary metastases”

Coppage et al: J Thorac Imaging 1987; 2(4):24-37

Solitary Metastasis vs. Lung Primary •

“The likelihood of a primary lung cancer versus a metastasis depends on the histologic characteristics of the extrapulmonary neoplasm and the patient’s smoking history”

Quint et al: Radiology 2000; 217: 257-61

Solitary Metastasis vs. Lung Primary •

A SPN is more likely to be bronchogenic CA than a solitary met if the patient has carcinoma of: ➢ Head and neck ➢ Bladder ➢ Esophagus ➢ Breast ➢ Cervix ➢ Bile Ducts ➢ Ovary ➢ Prostate ➢ Stomach

Solitary Metastasis vs. Lung Primary •

•

The incidence is fairly equal in patients with carcinoma of: ➢ Kidney ➢ Colon ➢ Adrenal gland ➢ Uterus ➢ Salivary or parotid gland ➢ Thyroid gland SPN is more likely solitary metastasis in: ➢ Melanoma ➢ Sarcoma (soft tissue, bone) ➢ Testicular carcinoma

Solitary Metastasis

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Lymphangitic Carcinomatosis •

• • • •

Figure 1-15-10

Adenocarcinomas in 80%: ➢ Lung ➢ Breast ➢ Stomach ➢ Pancreas ➢ Prostate ➢ Colon Incidence 6-55% Symptoms: gradual onset dyspnea, cough PFT’s: reduced lung compliance & diffusing capacity Diagnosis: bronchial lavage or TBB

Lymphangitic Carcinomatosis [Figure 1-15-10]

• •

Blood-borne tumor cells extravasate and invade lymphatic channels Tumor also enters lymphatics “retrograde” via mediastinal, hilar lymph nodes (25%) Lymphatics expand with tumorlets and edema Clusters or cords of tumor in lymphatics of the interlobular septa and peribronchovascular interstitium Edema and desmoplastic reaction accentuate interstitial thickening Pleural involvement: 2/3 Nodal involvement: 1/3

• • • • • • •

Normal (50%) Kerley B lines Reticulonodular opacities Subpleural edema Pleural effusion (30-50%) Hilar,mediastinal lymphadenopathy (20-40%) Bilateral or unilateral findings

•

Smooth or nodular thickening of ➢ Bronchovascular bundles ➢ Interlobular septa (Kerley’s lines; polygonal arcades) ➢ Lobar fissures (subpleural edema) Ground glass opacities Focal or unilateral distribution (50%) ➢ lung or breast CA Pleural effusion Lymphadenopathy (up to 50%)

• • • • •

Lymphangitic Carcinomatosis: Imaging Features - Chest radiograph

Secondary pulmonary lobule: lymphangitic carcinomatosis produces smooth and nodular expansion of bronchovascular bundle sheaths and interlobular septa

Figure 1-15-11

Lymphangitic Carcinomatosis Imaging Features: Chest CT [Figure 1-15-11]

• • • •

Lymphangitic carcinomatosis in a middle aged female with breast cancer

Tumor Embolism • • • •

•

Lodges in distal arterioles (100-200 micron diameter) 26% cancer pts (at autopsy) <1% clinically significant Complications ➢ Cor pulmonale (PAH) ➢ Lung infarction ➢ Lung hemorrhage Parenchymal or lymphatic mets if extravasation

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Tumor Embolism •

Figure 1-15-12

Malignancies ➢ Stomach ➢ Lung (esp. adenoCA) ➢ Breast ➢ ChorioCA ➢ Ovary ➢ Prostate ➢ Liver ➢ Kidney ➢ Lymphoma ➢ Right atrial myxoma

Tumor Embolism [Figure 1-15-12] •

•

CXR ➢ typically normal ➢ if widespread: nodules, airspace opacities CT ➢ “beading” of peripheral pulmonary arteries ➢ mosaic perfusion ➢ wedge-shaped peripheral opacities ➢ if extravasation: nodules, lymphangitic carcinomatosis

Endobronchial Metastases

• • • •

• • •

Tumor arrives via bronchial arteries & peribronchial lymphatics Rarely, via airways (BAC) 2-5% incidence in pts dying from metastases Malignancies ➢ Kidney ➢ Colon, Rectum ➢ Breast ➢ Melanoma ➢ Pancreas Mean interval from diagnosis of primary: 65 months Mean survival after discovery: 15.5 months Treatment options ➢ Radiation, ➢ Chemotherapy ➢ Surgery ➢ Interventional bronchoscopy (stenting, laser or mechanical resection, brachytherapy, photodynamic therapy)

Chan et al. Curr Opin Pulm Med 2003;9:301-308

Tumor thromboembolism may produce beading along peripheral bronchovascular bundles, as well as pulmonary infarction [Image courtesy of Mark Gosselin, MD]

Endobronchial Metastases: Imaging Features [Figure 1-15-13] • • • • •

Intraluminal soft tissue mass Atelectasis or post-obstructive pneumonia Serpiginous or nodular opacities (distal mucoid impaction) Hilar mass (if adjacent mediastinal invasion) DDX: bronchogenic carcinoma

Figure 1-15-13

Endobronchial metastases with left upper lobe collapse (renal cell carcinoma)

Figure 1-15-14

Pleural Metastases [Figures 1-15-14 to 1-15-16] •

•

Malignancies ➢ Lung ➢ Breast (50% of patients) ➢ Ovary ➢ Stomach ➢ Lymphoma Arise from lymphangitic or vascular invasion

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Large pleural effusion and pleural-based nodule in patient with breast cancer

Chest Radiology

•

• • • • •

Figure 1-15-16

Typically manifest as exudative pleural effusion ➢ Pleural nodules less common Scattered nodules on pleural surface Visceral & parietal pleura typically both involved Radiologic DDX ➢ asbestos exposure ➢ splenosis Rind-like or sheet-like pattern Radiologic DDX ➢ mesothelioma ➢ post-inflammatory fibrothorax

Figure 1-15-15

Pleural rind-like metastases in elderly female with NSCLC

Large pleural effusion and pleural-based nodule in patient with breast cancer

Do we impact the management of cancer patients with metastatic disease to the lung? Statement from the Fleischner Society: Guidelines for Management of Pulmonary Nodules MacMahon H et al. Radiology 2005;237:395-400

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Parenchymal Nodules Assessing Therapeutic Response •

•

Tumor doubling time ➢ Definition: 25% increase in tumor diameter separated by time (Tdt) ➢ Nodule growth rates vary widely ✧ according to histologic tumor cell type ✧ within the same patient ➢ Proposed reasons for differences ✧ Not all nodules are tumor (benign or inflammatory) ✧ Smaller mets grow at faster rates than larger mets ✧ Nodules represent different monoclonal cell populations (with variable responses to treatment) Document changes in several nodules in the same patient for optimal therapeutic assessment - impacts treatment strategy & participation in clinical trials

Chojniak et al. Am J Clin Oncol 2003;26(4):374-377

Parenchymal Nodules Indications for Metastasectomy •

•

To cure, but only if ➢ Complete resection possible ➢ No extrathoracic metastases (EXCEPT colon CA with liver mets) ➢ No therapeutic alternative (chemotherapy-insensitive tumors) ➢ Multiple nodules NOT necessarily a contraindication To prolong 5-year survival ➢ Colorectal cancer: up to 20-50% ➢ Osteogenic & soft tissue sarcomas: up to 40% ➢ Melanoma, renal cell, head & neck, female GU: up to 30% ➢ Thyroid, parathyroid: up to 61%

Yoneda et al. Curr Opin Pulm Med 2000;6:356-363.

“A Sarcoid-like Reaction”

• •

•

• •

Rare but well-documented Follows resection or treatment ➢ Lymphoma (Hodgkin & Non-Hodgkin) ➢ AML ➢ Lung CA ➢ Testicular CA ➢ Gastric CA ➢ Renal CA Radiologic manifestations ➢ Mediastinal, hilar lymphadenopathy ➢ Pulmonary nodules or consolidations ➢ Systemic sarcoidosis absent ➢ Positive on FDG-PET: mimics recurrence Biopsy required for confirmation ? Local immunologic response to tumor cells

Patterns of Metastatic Disease to the Lung Overview •

• •

Parenchymal nodules ➢ Well-circumscribed, random or angiocentric, basilar>apical ➢ Unusual: cavitary, calcified, solitary Lymphangitic carcinomatosis ➢ Septal lines, nodular/thickened fissures, GGO ➢ Pleural effusion ➢ Lymphadenopathy Tumor thromboembolism ➢ Beading of peripheral arteries ➢ Mosaic perfusion ➢ Pleural-based opacity (infarction)

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• •

Endobronchial nodule ➢ Rounded defect in airway, or cut-off of airway lumen ➢ Post-obstructive atelectasis, pneumonia, mucoid impaction Pleural-based metastases ➢ Pleural effusion ➢ Nodules on pleural surface ➢ Variation: rind-like pattern mimics mesothelioma

References 1. 2. 3. 4. 5. 6. 7.

8. 9.

10. 11.

12.

13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23.

Chan AL, Yoneda KY, Allen RP, Albertson TE. Advances in the management of endobronchial lung malignancies. Curr Opin Pulm Med 2003; 9:301-308. Chojniak R, Younes RN. Pulmonary metastases doubling time: Assessment by computed tomography. Am J Clin Oncol 2003; 26(4):374-377. Coppage L, Shaw C, Curtis A. Metastatic disease to the chest in patients with extrathoracic malignancy. J Thorac Imag 1987; 2(4):24-37. David SD, Westcott J, Fleishon H, Gefter WB, Henschke CI, McLoud TC, Pugatch RD, Sostman HD, Tocino I, White CD, Yankelevitz D, Bode FR. Screening for pulmonary metastases. American College of Radiology. ACR Appropriateness Criteria. Radiology 2000; 215 Suppl:655-62. Davis S. CT evaluation for pulmonary metastases in patients with extrathoracic malignancy. Radiology 1991; 180:112. Heffner JE, Milam MG. Sarcoid-like hilar and mediastinal lymphadenopathy in a patient with metastatic testicular cancer. Cancer 1987; Oct 1;60(7):1545-7. Hirakata K, Nakata H, Haratake J. Appearance of pulmonary metastases on high-resolution CT scans: comparison with histopathologic findings on autopsy specimens. Am J Roentgenol 1993; 161:37-43. Jungraithmayr W, Hasse J, Stoelben E. Completion pneumonectomy for lung metastases. EJSO 2004; 30:1113-1117. Kozuka T, Johkoh T, Hamada S, Maito H, Tomiyama N et al. Detection of pulmonary metastases with multi-detector row CT scans of 5-mm nominal section thickness: Autopsy lung study. Radiology 2003; 226:231-234. Libshitz HI, North LB. Pulmonary metastases. Radiol Clin North Am 1982; 20:437-451. MacMahon H et al. Guidelines for management of small pulmonary nodules detected on CT scans: A statement from the Fleischner Society. Radiology 2005; 237:395-400. Marglin S, Mortimer J, Castellino R. Radiologic investigation of thoracic metastases from unknown primary sites. J Thorac Imag 1987; 2(4):38-43. Milne EC, Zerhouni EA. Blood supply of pulmonary metastases. J Thorac Imag 1987; 2(4):15-23. Murata K, Takahashi M, Mori M, Kawaguchi N et al. Pulmonary metastatic nodules: CT-pathologic correlation. Radiology 1992; 182:331-335. Parra ER et al. Pulmonary and mediastinal “sarcoidosis” following surgical resection of cancer. Pathol Res Pract 2004; 200(10);701-5. Poste G, Fidler I. The pathogenesis of cancer metastasis. Nature 1980; 283:139-145. Pugatch RD. Radiologic Evaluation in Chest Malignancies. Chest 1995; 107:294S-297S. Quint L, Park C, Iannettoni M. Solitary pulmonary nodules in patients with extrapulmonary neoplasms. Radiology 2000; 217:257-261. Seo JB, Im J, Goo JM, Chung MJ, Kim M. Atypical pulmonary metastases: spectrum of radiologic findings. RadioGraphics 2001; 21:403-417. Snyder BJ, Pugatch RD. Imaging characteristics of metastatic disease to the chest. Chest Surg Clin N Am 1998; 8(1):29-48. Woodard PK, Dehdashti F, Putman CE. Radiologic diagnosis of extrathoracic metastases to the lung. Oncology 1998; 12(3): 441-444. Yoneda KY, Louie S, Shelton D. Approach to pulmonary metastases. Current Opinion in Pulmonary Medicine 2000; 6(4):356-363. Zhao B, Schwartz LH, Moskowitz CS et al. Pulmonary metastases: Effect of CT section thickness on measurement – initial experience. Radiology 2005; 234:934-939.

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Differential Diagnosis of Mediastinal Masses Melissa L. Rosado de Christenson, MD, FACR Learning Objectives: • • • • •

To define the mediastinum and describe the mediastinal compartments To provide a classification for a practical approach to the imaging diagnosis of mediastinal masses To list clinical and cross-sectional imaging features that allow a focused differential diagnosis To describe lesions with pathognomonic imaging features To differentiate neoplastic from non-neoplastic conditions with emphasis on management

Mediastinal Compartments [Figure 1-16-1] • • • • •

Mediastinum - space between pleural surfaces and lungs Bound by sternum and vertebrae From thoracic inlet to diaphragm Thymus, lymph nodes, heart, great vessels,trachea, esophagus, nerves and other soft tissues Arbitrary division into compartments - no anatomic boundaries

The Mediastinal Compartments • • • •

[Figures 1-16-2 and 1-16-3]

Anatomic - Superior, anterior, middle, posterior Excludes paravertebral areas Surgical - Superior, anterior, middle, posterior Includes paravertebral areas Radiographic (Felson) - Anterior, middle, posterior Radiographic (Fraser, Müller, Colman, Paré) - Anterior, middle-posterior, paravertebral

Figure 1-16-2

The mediastinum

Figure 1-16-3

Anatomic and Surgical Mediastinal Compartments

Mediastinal Masses

Figure 1-16-1

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Mediastinal Masses • • • • •

Patients are often asymptomatic 83% of asymptomatic masses are benign 57% of symptomatic masses are malignant Approximately 1/3 are malignant Approximately 1/10 are vascular

• • • •

Neurogenic tumor Thymoma Cysts Lymphoma Teratoma Granuloma Mediastinal goiter

Mediastinal Masses – Mayo Clinic (N=1,064) 20% 19% 18% 30%

Wychulis et al. J. Thorac Cardiovasc Surg 1971

Approach to Mediastinal Masses •

• •

Clinical ➢ Demographics (age, gender) / Symptoms Radiography ➢ Mediastinal compartment / Adjacent structures ➢ Focal mass vs. diffuse mediastinal enlargement ➢ Lesion contours / Density Cross-sectional Imaging ➢ Location / Relationship to normal structures ➢ Morphologic features / Associated findings

Mediastinal Masses • • • • •

Neoplasia ➢ Malignant (secondary / diffuse) ➢ Benign and malignant (primary / focal) Congenital cysts Glandular enlargement Vascular lesions Herniations / Esophageal abnormalities Miscellaneous conditions

• • • • • •

Small cell / poorly differentiated lung cancer Elderly smokers; males and females Cough, dyspnea, SVC syndrome, weight loss Mediastinal mass, lymphadenopathy, local invasion Primary neoplasm may not be evident Non-surgical lesion

• • • • • •

Known malignancy Renal cell carcinoma Testicular carcinoma Head and neck cancer Breast carcinoma Melanoma

•

Figure 1-16-4

Malignant Neoplasia – Lung Cancer

Malignant Neoplasia - Metastases [Figure 1-16-4]

Chest Radiology

Metastatic renal cell carcinoma

149

Mediastinal Masses

Malignant Neoplasia – Lymphoma • • • • •

• •

Figure 1-16-5

Hodgkin disease / Non-Hodgkin lymphoma All age groups (young patients); Males and females Palpable lymphadenopathy, constitutional symptoms Lobular / diffuse mediastinal enlargement Prevascular, paratracheal lymphadenopathy ➢ Nodal coalescence, local invasion Primary mediastinal lymphoma Non-surgical lesion

Lymphoma • •

• •

Non-Hodgkin lymphoma - 75% of all cases 50-70% of mediastinal lymphoma is Hodgkin disease ➢ 15-21% is non-Hodgkin lymphoma Hodgkin – 66% intrathoracic at presentation ➢ Non-Hodgkin – 37% intrathoracic at presentation Treatment: Radiotherapy, chemotherapy

Lymphoma: Clinical Features •

•

Hodgkin Disease: Microscopic Features

Hodgkin Disease ➢ Males = Females (NSHD, 2 X more common in females) ➢ Bimodal distribution: 2nd to 3rd and > 5th decades ➢ Lymphadenopathy: cervical, supraclavicular ➢ 20-30%; fever, night sweats, wt. loss Non-Hodgkin lymphoma ➢ Systemic disease with constitutional symptoms: lymphadenopathy, local invasion ➢ Lymphoblastic - male children / adolescents ➢ Diffuse large-B cell - young adult females

Figure 1-16-6

Lymphoma: Pathologic Features [Figures 1-16-5 and 1-16-6] •

•

Hodgkin Disease ➢ Nodal cellular infiltrate, collagenous connective tissue (NS), ReedSternberg cell ➢ Lymphadenopathy, nodal coalescence, primary thymic involvement, cystic change, hemorrhage, necrosis ➢ Local invasion (including chest wall), hemorrhage, necrosis Non-Hodgkin Lymphoma ➢ Lymphoblastic (precursor T-lymphoblastic) -lymphoblasts ➢ Diffuse large B-cell (primary mediastinal [thymic] large B-cell); large cells, vesicular nuclei, prominent nucleoli ➢ Large, infiltrative, locally invasive mass, necrosis

Lymphoma: Imaging Features [Figures 1-16-7 to 1-16-9] • •

• • •

Lobulated unilateral/bilateraldiffuse mediastinal enlargement Hodgkin Disease ➢ Intrathoracic involvement in 85% ➢ Lymphadenopathy; prevascular, paratracheal ➢ Nodal coalescence (homogeneous or heterogeneous) ➢ Ca++; 1% - 1 year post-therapy, rare pre-therapy Non-Hodgkin ➢ Prevascular, paratracheal adenopathy ➢ Isolated involvement of other mediastinal lymph nodes Local invasion Primary mediastinal lymphoma

Hodgkin Disease: Gross Features

Figure 1-16-7

Hodgkin Disease: Imaging Features

Mediastinal Masses

150

Chest Radiology

Figure 1-16-8

Figure 1-16-9

Non-Hodgkin lymphoma – nodal coalescence with low attenuation corresponding to necrosis

Hodgkin disease – prevascular and paratracheal lymphadenopathy and left pleural effusion

Secondary Neoplasia

• • • • •

Diffuse, bilateral mediastinal enlargement Lymphadenopathy Local invasion Metastases Other imaging features of malignancy

•

Infection ➢ Fungal: Mediastinal fibrosis; Calcification ➢ Other granulomatous infections Sarcoidosis ➢ Bilateral symmetric hilar lymphadenopathy ➢ Typical lung parenchymal involvement Castleman disease ➢ Enhancement / calcification (10%)

Non-Neoplastic Lymphadenopathy • •

Mediastinal Fibrosis • •

• • •

Granulomatous lymphadenopathy Young patients with signs and symptoms of obstruction ➢ Trachea, bronchi, esophagus, vessels Mediastinal mass, circumscribed or locally invasive, calcification Systemic antifungal agents, excision, dilatation, bypass graft 30% mortality

Figure 1-16-10

Castleman Disease [Figure 1-16-10] • • • • •

Angiofollicular or giant lymph node hyperplasia Hyaline vascular type (> 90%) vs. plasma cell variant Localized vs. systemic Adult females (M:F - 4:1) may be asymptomatic Middle mediastinal / hilar mass ➢ Solitary mass ➢ Dominant mass with lymphadenopathy ➢ Multiple enlarged lymph nodes ➢ Enhancement, calcification (10%)

Chest Radiology

151

Castleman Disease: Enhancing mediastinal lymphadenopathy Mediastinal Masses

Primary Neoplasia •

•

Figure 1-16-11

Thymus ➢ Thymoma ➢ Thymic malignancy ➢ Thymolipoma ➢ Germ cell neoplasm Neurogenic neoplasms

Thymoma • • • • • •

Epithelial neoplasm, most common primary thymic neoplasm Slow growth, “benign” behavior M=F; 70% in the 5th and 6th decades Most patients asymptomatic 25-30% with symptoms of compression/invasion Associated parathymic syndromes: ➢ Myasthenia gravis ➢ Pure red cell aplasia ➢ Hypogammaglobulinemia

Thymoma and Myasthenia Gravis • • • •

Myasthenia gravis (MG) – autoimmune neurological disorder 85% of patients with MG have follicular thymic hyperplasia 15% of patients with MG have a thymoma Of all patients with thymoma, 30-50% have MG

• •

Lymphocytes and epithelial cells in varying proportions WHO 1999 classification (morphology and lymphocyte-to-epithelial cell ratios) Types A, AB, B1, B2, B3 Tumor lobules compartmentalized by fibrous septa Encapsulated vs. Invasive Spherical mass, variable size, lobular contours, typically encapsulated Hemorrhage, necrosis, cystic change (mural nodules) Invasive thymoma - microscopic documentation of capsular invasion, local invasion, tumor implants, metastases

Thymoma: Pathologic Features [Figures 1-16-11 to 1-16-13] • • • • • •

Figure 1-16-13

Thymoma: Microscopic Features

Figure 1-16-12

Thymoma: pathologic features – tumor lobules compartmentalized by fibrous bands

Thymoma: Gross Features Mediastinal Masses

152

Chest Radiology

Thymoma: Imaging Features [Figures 1-16-14 to 1-16-18] • • • • • •

Anterior mediastinal mass; lobular, unilateral, variable size Normal radiographs in 25% (occult thymoma) Focal, spherical, homogeneous or heterogeneous ➢ Necrosis, cystic (mural nodules), calcification (typically curvilinear and peripheral) No lymphadenopathy Exclude local invasion of fat, cardiovascular structures, lung Pleural implants (may cause diffuse pleural thickening)

Figure 1-16-14

Thymoma: Unilateral, lobular, left anterior mediastinal mass

Figure 1-16-15

Figure 1-16-16

Occult Thymoma: Well-defined, lobular, unilateral, prevascular mass Thymoma: Well-defined right cardiophrenic angle mass with irregular low attenuation corresponding to necrosis

Figure 1-16-18

Figure 1-16-17

Cystic thymoma: Unilateral spherical cystic mass with peripheral curvilinear calcification and mural nodules Invasive thymoma: Direct invasion of left brachiocephalic vein Chest Radiology

153

Mediastinal Masses

Thymoma: Staging (Masaoka) (10 - year survival)

Figure 1-16-19

I II

Encapsulated / no microscopic capsular invasion (86-100%) Microscopic invasion into surrounding fat / mediastinal pleura, microscopic capsular invasion (55-100%) III Macroscopic invasion of adjacent organs, pericardium, heart, great vessels, lung (47-60%) IVa Pleural / pericardial dissemination (0-11%) IVb Lymphatic / hematogenous dissemination

Thymoma: Therapy / Prognosis •

• •

Encapsulated; complete excision ➢ Best prognosis ➢ Occasional local recurrence, distant metastases Post-operative radiation for invasive thymoma to decrease local Thymic Carcinoid: Mediastinal mass with recurrence adjacent lymphadenopathy in a patient Chemotherapy for for progression after surgery and unresectable with ACTH production and MEN 1 lesions

Thymic Malignancy: Carcinoid / Carcinoma • • •

Rare malignant epithelial neoplasms Symptomatic patients Poor prognosis

• • •

Neuroendocrine neoplasm; atypical carcinoid (necrosis / mitoses / invasion) Males > Females; 3:1; wide age range (average, 43 yrs) 50% functionally active ➢ ACTH – Cushing syndrome (33-40%) MEN type 1 – (Wermer syndrome) (19-25%) ➢ Hyperparathyroidism (90%), islet cell tumor of pancreas (80%) pituitary adenoma (65%)

Thymic Carcinoid

•

Figure 1-16-20

Thymic Carcinoma • •

•

Male > Female; wide age range (mean: 5th decade) Several cell types identical to primary lung cancer; R/O metastases WHO Type C thymoma

Thymic Carcinoid / Carcinoma: Imaging Features [Figure 1-16-19]

• • • • •

Large anterior mediastinal mass (R/O thymoma) R/O metastatic lung malignancy (histology) Lymphadenopathy Local invasion, pleural or pericardial effusion/implantation, metastases Carcinoid ➢ Octreotide imaging for diagnosis for occult (non-specific metastases and other neoplasms)

Thymolipoma • • •

Rare benign thymic neoplasm M=F; wide age range (average age, 28 yrs) Asymptomatic patients: 50% ➢ Symptoms with large tumors

Thymolipoma, microscopic features: Thymic tissue admixed with mature adipose tissue

Thymolipoma: Pathologic Features [Figure 1-16-20] • •

Encapsulated, soft, lobular, yellow Mature adipose tissue and thymic tissue in variable proportions

Mediastinal Masses

154

Chest Radiology

Thymolipoma: Imaging Features [Figure 1-16-21] •

• • •

Well-defined anterior / inferior mediastinal mass ➢ Unilateral or bilateral, slow growth May conform to shape of structures ➢ R/O cardiac enlargement / diaphragmatic elevation ➢ Positional change in shape Anatomic connection to the thymus (pedicle) Mixed fat and soft tissue attenuation/signal

Figure 1-16-21

Germ Cell Neoplasms • • • •

Most common in the gonad Extragonadal germ cell neoplasms; midline locations, most commonly the mediastinum Postulated origin in multipotential primitive germ cells “misplaced” during embryogenesis Cell types: ➢ Teratoma (mature, immature [immature neuroectoderm], “malignant” [mixed malignant germ cell neoplasm]) ➢ Seminoma ➢ Non-seminomatous germ cell neoplasms

Mature Teratoma

• • • • •

Thymolipoma, imaging features – anterior mediastinal mass with anatomic connection with the thymus and mixture of fat and soft tissue attenuation

Figure 1-16-22

60-75% of mediastinal germ cell neoplasms Males=Females Children and young adults (< 40 yrs) Often asymptomatic Symptoms of compression or rupture

Mature Teratoma: Pathologic Features [Figures 1-16-22 and 1-16-23]

•

• •

More than one embryonic germ cell layer ➢ Ectoderm – skin, dermal appendages ➢ Mesoderm – bone, cartilage, muscle ➢ Endoderm – GI, respiratory tissue, mucus glands Spherical, encapsulated, lobulated Multilocular or unilocular cyst ➢ Oily, sebaceous, gelatinous material (lipid) ➢ Focal solid areas: hair, teeth, bone

Figure 1-16-23

Teratoma, microscopic features: cystic neoplasm with ectodermal, mesodermal and endodermal components

Teratoma, gross features: multilocular cystic mass Chest Radiology

155

Mediastinal Masses

Mature Teratoma: Imaging Features [Figure 1-16-24] • • • •

Figure 1-16-24

Unilateral anterior mediastinal mass Spherical, lobular contours, well-defined Multilocular cystic - 85% Attenuation: ➢ Fluid 89%, Fat 76%, Ca++ 53% ➢ Fat fluid level - 11% ➢ ST/FL/FAT/Ca++ 39% ➢ ST/FL/FAT 24% ➢ ST/FL 15%

Mature Teratoma: Therapy and Prognosis • •

Complete excision is curative Excellent prognosis ➢ Near 100% five-year survival

Teratoma, imaging features: Unilateral, anterior mediastinal, multilocular cystic mass with intrinsic fluid, soft tissue, fat and calcium

Germ Cell Neoplasms • • • •

Most common in the gonad Extragonadal germ cell neoplasms; midline locations, most commonly the mediastinum Postulated origin in multipotential primitive germ cells “misplaced” during embryogenenesis Cell types: ➢ Teratoma (mature, immature, “malignant”) ➢ Seminoma ➢ Non-seminomatous germ cell neoplasms

Seminoma •

• •

• •

40% of malignant germ cell neoplasms of a single histology ➢ Caucasian males, third to fourth decades Most patients are symptomatic Rounded cells with sharp borders, clear cytoplasm, fibrous bands, lymphocytes, plasma cells, granulomas Homogeneous soft tissue mass Radiation therapy / Cisplatin-based chemotherapy ➢ 60-80% long-term survival

Seminoma: Imaging Features [Figure 1-16-25] •

•

Anterior mediastinal mass (both sides of midline) ➢ Large, bulky, well-defined, lobulated, locally invasive CT: ➢ Homogeneous soft tissue mass ➢ Mimics nodal coalescence ➢ Slight homogeneous contrast-enhancement ➢ Rarely necrosis / cystic change (8%)

Non-Seminomatous Malignant Germ Cell Neoplasms • • • • •

• • • •

Yolk sac (endodermal sinus) tumor Embryonal carcinoma Choriocarcinoma Mixed germ cell neoplasm Males, 90% symptomatic ➢ Klinefelter syndrome (20%); hematologic malignancy Alpha-fetoprotein (EST, EC) ➢ B-human chorionic gonadotropin (choriocarcinoma) ➢ LDH (60%) tumor burden Large, unencapsulated Hemorrhage, necrosis, “cyst” formation Cisplatin-based chemotherapy; excision of residual tumor

Mediastinal Masses

156

Figure 1-16-25

Seminoma, imaging features: Diffuse homogeneous anterior mediastinal mass with mass effect

Figure 1-16-26

Non-seminomatous malignant germ cell neoplasm, imaging features: Large anterior mediastinal locally invasive heterogeneous mass Chest Radiology

Non-Seminomatous GCN: Imaging Features

Figure 1-16-27

[Figure 1-16-26]

•

• •

Large, well or poorly-defined anterior mediastinal mass ➢ Extends to both sides of midline Heterogeneous ➢ Large areas of central low attenuation ➢ Frond-like peripheral soft tissue Loss of tissue planes ➢ Local invasion, lymphadenopathy

Neurogenic Neoplasms [Figure 1-16-27] •

• • •

20% of primary mediastinal neoplasms ➢ 35% in children 70–80% benign Peripheral nerves ➢ Schwannoma ➢ Neurofibroma ➢ Malignant peripheral nerve sheath tumor Sympathetic ganglia ➢ Ganglioneuroma ➢ Ganglioneuroblastoma ➢ Neuroblastoma

Neurogenic neoplasms may arise from peripheral nerves or sympathetic ganglia

Figure 1-16-28

Schwannoma / Neurofibroma [Figure 1-16-28] • • • • • •

Schwannoma – Most common mediastinal neurogenic neoplasm ➢ Spherical, encapsulated ➢ Cellular and less cellular areas (Antoni A / B) Neurofibroma – second most common mediastinal neurogenic neoplasm ➢ Spherical/fusiform, unencapsulated Calcification, cystic change, hemorrhage Young adults; 3rd and 4th decades Most (65%) asymptomatic Symptoms and signs of compression

Schwannoma / Neurofibroma: Imaging Features [Figures 1-16-29 and 1-16-30]

• • •

•

Spherical, smooth / lobular, well-defined paravertebral mass Osseous findings (50%): pressure erosion/deformity of ribs or vertebrae; expanded neuroforamen Homogeneous/heterogeneous ➢ Heterogeneous enhancement; Ca++ in 10% ➢ Growth into spinal canal in 10% MR Imaging – R/O spinal involvement ➢ T1 – Low-to-intermediate signal ➢ T2 – Foci of high signal

Figure 1-16-30

Figure 1-16-29

Schwannoma, imaging features: Unilateral paravertebral spherical mass Chest Radiology

Schwannoma, gross features: Heterogeneous spherical mass

Schwannoma, imaging features: Intraspinal extension 157

Mediastinal Masses

Neurofibromatosis (NF1) • • • • •

Multiple neoplasms (including ganglioneuroma) Plexiform neurofibroma Vagus nerve, sympathetic chain, phrenic nerve Diffuse enlargement of peripheral nerve Multiple masses along a nerve

• • •

Most frequent in the paravertebral region Rare among neurogenic neoplasms Large (> 5 cm) spherical mass ➢ Central low attenuation – necrosis ➢ Calcification ➢ May exhibit local invasion

Malignant Peripheral Nerve Sheath Tumor

Peripheral Nerve Neoplasms: Therapy and Prognosis • •

•

Excision Schwannoma/Neurofibroma ➢ Excellent prognosis Malignant peripheral nerve sheath tumor ➢ Solitary – 75% five-year survival ➢ Neurofibromatosis – 30% five-year survival

Thoracic Meningocele

• • • •

Intrathoracic extrusion of meninges and their fluid content Well-defined spherical paravertebral mass Enlarged neuroforamen, pressure erosion, sclerosis Homogeneous, fluid attenuation / signal

•

Children, adolescents, young adults ➢ Asymptomatic patients De novo; maturation of neuroblastoma Benign paravertebral neoplasm Mature ganglion cells, Schwann cells, nerve fibers Encapsulated, elongate mass ➢ Gray / yellow with lobular surface

Ganglioneuroma [Figure 1-16-31] • • • •

Figure 1-16-31

Ganglioneuroma: Imaging Features • • •

•

Well-defined, oblong paravertebral mass Osseous erosion / displacement Homogeneous or heterogeneous ➢ Calcification in 25% MR: Homogeneous intermediate signal on T1 / T2 ➢ R/O intraspinal extension

Ganglioneuroblastoma/Neuroblastoma • • •

•

Ganglioneuroma, gross features: Elongate paravertebral mass

Infants and young children Asymptomatic; chest wall pain, paraplegia, Horner syndrome, diarrhea, hemothorax Elevation of urine catecholamines ➢ Elevation of urine/serum VMA (screening) Neuroblastoma – Elongate paravertebral mass ➢ 50% < 2 years ➢ 90% < 5 years ➢ May be congenital

Mediastinal Masses

158

Chest Radiology

Ganglioneuroblastoma/Neuroblastoma: Pathologic Features •

• •

Adrenal – most common location ➢ Paravertebral – second most common location Ganglioneuroblastoma: Neuroblasts and ganglion cells ➢ Well / poorly differentiated Neuroblastoma: Neuroblasts, Homer-Wright pseudorosettes ➢ Well / poorly differentiated

Neuroblastoma: Imaging Features

Figure 1-16-32

[Figure 1-16-32]

• • • • •

Well-defined large elongate paravertebral mass Radiographic evidence of Ca++ in 10% Osseous erosion R/O intraspinal growth Local soft tissue invasion

•

Ganglioneuroma ➢ Excision is curative Ganglioneuroblastoma ➢ Five-year survival near 90% Neuroblastoma ➢ Five-year survival – 30% ➢ More favorable course with: age < 2 yrs, mediastinal ➢ Spontaneous maturation to ganglioneuroma

Sympathetic Ganglia Tumors • •

Paraganglioma •

• •

•

Middle mediastinum: Aortopulmonary paraganglia ➢ Paravertebral: Aortico sympathetic paraganglia ➢ Heart Adults (average age 30-40 yrs) ➢ Males > Females; 2:1 ➢ Asymptomatic; excess catecholamines Well-defined spherical mass ➢ Homogeneous/heterogeneous Marked contrast enhancement ➢ 90% uptake of I131 or I123 MIBG

Neuroblastoma, imaging features: Unilateral calcified paravertebral mass in a neonate with intraspinal extension

Primary Neoplasia •

•

Benign ➢ Focal, unilateral mass ➢ No lymphadenopathy ➢ No local invasion Malignant (invasive) ➢ Focal, unilateral mass ➢ Lymphadenopathy ➢ Local invasion

Figure1-16-33

Bronchogenic Cyst [Figure 1-16-33] • • • •

Most common congenital cyst of the mediastinum Abnormal ventral foregut bud Failure to induce mesenchymal development to lung parenchyma Mediastinum (85%), pericardium, diaphragm, pleura and lung

Bronchogenic Cyst: Clinical Features • • • • •

Rare in infants, infrequent in children Young adults Asymptomatic – incidental finding Symptomatic – chest pain, mass effect, obstruction, infection Excision, observation, drainage, sterile alcohol ablation

Chest Radiology

159

Bronchogenic cyst: Typical subcarinal location Mediastinal Masses

Bronchogenic Cyst : Pathologic Features [Figures 1-16-34 and 1-16-35]

• • • • • •

Respiratory epithelium Wall: bronchial glands, cartilage, smooth muscle Closed foregut connection Spherical, ovoid, unilocular Thin wall Fluid variable: clear, turbid, hemorrhagic, serous, viscous

Figure 1-16-34

Bronchogenic Cyst: Imaging Features [Figures 1-16-36 and 1-16-37]

• • •

• •

Well-defined, spherical, middle mediastinal mass Near trachea, carina, stem bronchi CT: ➢ Thin smooth wall (enhancement) ➢ Water (40%) or soft tissue (43%) attenuation ➢ Homogeneous / heterogeneous, non-enhancing contents MR: ➢ T1 - variable (slightly hyperintense to muscle) ➢ T2 - isointense or hyperintense to CSF Thin-walled pulmonary cyst; air, fluid, air-fluid level

Other Congenital Cysts • • • •

Foregut cysts Esophageal - within esophageal; ectopic gastric mucosa Neuroenteric - Associated spinal anomaly Pericardial - Cardiophrenic angle, imperceptible wall, fluid attenuation; asymptomatic patients

Bronchogenic cyst, microscopic features: respiratory epithelium with cartilage and smooth muscle in wall

Figure 1-16-35

Figure 1-16-36

Bronchogenic cyst, imaging features: Subcarinal spherical mass with extension to the right

Bronchogenic cyst, gross features: Thinwalled unilocular cyst

Figure 1-16-37

Bronchogenic cyst, imaging features: spherical subcarinal mass that may not exhibit water attenuation Mediastinal Masses

160

Chest Radiology

Thymic Cyst [Figures 1-16-38 and 1-16-39]

• • •

Uncommon (3% of mediastinal masses) Acquired vs. Congenital Children / young adults Association with neoplasia, AIDS Diffuse infiltrative lymphocytosis syndrome (DILS) Epithelial lining and thymus in cyst wall Multilocular / unilocular R/O cystic neoplasm

• • • • • •

Focal, spherical Unilocular Thin-walled No mural nodules No lymphadenopathy Along foregut-derived structures

•

Lymphoid hyperplasia (lymphofollicular / autoimmune thymitis) secondary follicles with germinal centers; may not produce thymus enlargement Myasthenia gravis, hyperthyroidism, lupus, scleroderma, RA, cirrhosis True hyperplasia - global increase in the size and weight of the thymus Rebound hyperplasia - following chemotherapy (2 weeks to 14 months), steroids or severe insult Ant. mediastinal widening Homogeneous soft tissue Maximal thickness ➢ Under 20 years – 1.8 cm ➢ Over 20 years – 1.3 cm Follicular thymic hyperplasia – normal or mildly enlarged thymus

• • • •

Figure 1-16-38

Congenital Cysts

Thymic Hyperplasia • • •

• • • •

Mediastinal Goiter [Figure 1-16-40] • •

• • •

20% of cervical goiters Asymptomatic females: incidental finding ➢ May produce symptoms by mass effect Adenomatous goiter; rarely malignancy or thyroiditis Fibrous capsule; nodules composed of thyroid follicles Hemorrhage, calcification, cystic change

Thymic cyst, gross features: multilocular cyst

Figure 1-16-39

Thymic cyst, imaging features: Multilocular cyst

Figure 1-16-40

Mediastinal goiter, pathologic features: iodine content, well-defined lobular soft tissue mass Chest Radiology

161

Mediastinal Masses

Mediastinal Goiter: Imaging Features

Figure 1-16-41

[Figures 1-16-41 and 1-16-42]

•

• • • • •

Unilateral anterior mediastinal mass (80%) ➢ Other compartments also affected, R > L Well-defined lobular borders Cervico-thoracic sign ➢ Continuity with cervical thyroid Calcification - punctate, coarse, curvilinear Cystic change High attenuation ➢ Intense, sustained contrast enhancement

Figure 1-16-42

Mediastinal goiter, imaging features: Large calcified unilateral mass with cervicothoracic sign

Figure 1-16-43

Mediastinal goiter, imaging features: Continuity between cervical and mediastinal portions of the mass, high attenuation and calcification

Parathyroid Adenoma

•

Ectopic parathyroid glands: superior pole of thymus (39%), mediastinum (2%), intrathyroid (0.2-3.5%) Primary hyperparathyroidism post surgical parathyroidectomy MEN I Imaging ➢ Tc99m / Tl201 subtraction imaging ➢ T123 / Tl201 ➢ Tc99m - Sestamibi (mitochondria) ➢ Single radionuclide/Dual radionuclide CT/MRI correlation of mediastinal uptake

• • •

Anatomically related to normal gland Continuity with normal gland Function similar to that of normal gland

• • • • •

Benign mesenchymal mediastinal tumor Proliferation of lymphatic vessels without communication with lymphatic tree Developmental vs. neoplasm vs. hamartoma Asymptomatic / symptoms of compression Mediastinal extension of cystic hygroma (10%), soft palpable mass; 90% diagnosed in infancy Mediastinal mass in asymptomatic child / adult

•

• • •

Glandular Enlargement

Lymphangioma, microscopic features: Interconnecting endothelial lined vascular channels

Lymphangioma

•

Lymphangioma: Pathologic Features [Figures 1-16-43 and 1-16-44]

• • • •

Intercommunicating spaces of variable size lined by endothelial cells Soft, cystic mass Cystic hygroma– large vascular spaces Cavernous lymphangioma – small vascular spaces

Mediastinal Masses

162

Chest Radiology

Lymphangioma: Imaging Features [Figures 1-16-45 and 1-16-46] • • • • •

Anterosuperior mediastinum; other compartments affected Cervical / axillary / chest wall mass; mediastinal extension Spherical, lobular, well-defined borders Circumscribed mass / infiltrative mass Multilocular, cystic, heterogeneous ➢ Solid components, tissue septa

Figure 1-16-44

Figure 1-16-45

Lymphangioma, imaging features: Multilocular cystic mediastinal mass with extension into the axilla

Figure 1-16-46

Lymphangioma, gross features: Multilocular cystic appearance due to enlargement of vascular channels

Lymphangioma, imaging features: Infiltrative or localized multilocular cystic mediastinal mass

Hemangioma [Figure 1-16-47] •

• • • • • •

Rare vascular mediastinal tumor ➢ Neoplasm vs. developmental Young patients; 75% < 35 yrs. Asymptomatic; 1/3-1/2 with symptoms of compression ➢ Rendu-Osler-Weber syndrome Communicating vascular spaces ➢ Endothelial lining, organized thrombi, Ca++, phleboliths Anterior mediastinal mass (also in other compartments) Spherical, well-defined, Ca++ 28%, punctate, phleboliths Heterogeneous intense enhancement

Chest Radiology

Figure 1-16-47

Hemangioma, imaging features: Anterior mediastinal mass with intrinsic phleboliths and intense heterogeneous enhancement

163

Mediastinal Masses

Vascular Lesion - Aneurysm • • • • •

Abnormal mediastinal contour contiguous with vascular structures Saccular aneurysms may resemble other primary mediastinal masses Curvilinear peripheral calcification Contrast enhancement Continuity with vascular lumen

• •

Esophageal / paraesophageal Severe liver disease and portal hypertension; Left gastric – portosystemic collaterals Visible on radiography in 10% Middle-posterior-paravertebral cluster of serpiginous vessels with intense enhancement

Vascular Lesion - Varices • •

Vascular Lesions • •

• •

Focal vs. infiltrative Lymphatic ➢ Multilocular cystic ➢ Extramediastinal involvement Blood vessels ➢ Intense, heterogeneous / serpiginous enhancement Aneurysms ➢ Focal vascular enlargement

Figure 1-16-48

Herniations – Hiatus Hernia [Figure 1-16-48] • • • • •

Gastric herniation through enlarged esophageal hiatus Increased intra-abdominal pressure / Increased prevalence with increasing age Asymptomatic; Reflux / bleeding Retrocardiac mass, homogeneous, air-filled, air-fluid Identification of abdominal contents in hernia sac

Herniation - Morgagni • • • •

Hiatus hernia, imaging features: herniation of abdominal contents through esophageal hiatus

Developmental defect in right anteromedial hemidiaphragm Asymptomatic / Abdominal pain Right cardiophrenic angle mass Demonstration of internal fat (omentum), bowel loops or abdominal organs (liver)

Herniations •

• •

Intrathoracic extension of abdominal contents ➢ Bowel ➢ Omental fat Esophageal hiatus Morgagni hernias

Miscellaneous – Achalasia • • • •

Absent peristalsis and incomplete relaxation of esophageal sphincter Primary – deficiency of ganglion cells in myenteric plexus Secondary – (pseudo achalasia) Chagas disease and primary or secondary malignancy at the GE junction Esophageal dilatation with air-fluid levels ➢ Esophageal displacement to the right, mass effect on mediastinum, pulmonary consolidation (aspiration)

Miscellaneous - EMH • • • • •

Extramedullary hematopoiesis Compensatory formation of blood elements outside osseous medulla Hemolytic anemia Unilateral or bilateral paravertebral mass; may exhibit internal fat attenuation Adjacent medullary expansion

Mediastinal Masses

164

Chest Radiology

Miscellaneous – Acute Mediastinitis • • • •

Surgery, instrumentation with esophageal perforation Ill patients with fever, chills and chest pain Focal or diffuse mediastinal widening, pneumomediastinum, pleural effusion, pneumothorax Abscess, abnormal mediastinal air, extraluminal ingested contrast, obliteration of tissue planes

Mediastinal Masses: Pathognomonic • • • • • • • •

Lateral thoracic meningocele Extramedullary hematopoiesis Aneurysm Esophageal varices Teratoma Lipomatosis Congenital cyst (BC, PC) Mediastinal goiter

• • •

• • • • •

Thymoma (mural nodules) Congenital cysts (unilocular, middle, posterior mediastinum) Neurogenic neoplasm (associated osseous erosion) ➢ Meningocele (NF1, continuity with spinal canal, homogeneous water attenuation / signal) Mature teratoma (multilocular cystic mass with internal fat) Lymphoma (lymphadenopathy) Lymphangioma (multilocular cysts - vascular channels) Esophageal enlargement (achalasia) Mediastinal goiter (high attenuation, continuity with thyroid)

• • • • •

Lipomatosis (diffuse, no mass effect) Lipoma Thymolipoma (fat / soft tissue connecting to thymus) Mature teratoma (cystic) Morgagni hernia (CPA, right, continuous with abdominal fat)

• • • • •

Mediastinal goiter (continuity with cervical thyroid) Hemangioma (phleboliths, follows vascular enhancement) Castleman disease (enhancing lymphadenopathy) Paraganglioma (catecholamine production) Aneurysm / Varices

Mediastinal Masses: Cystic

Mediastinal Masses: Fat

Mediastinal Masses: Intense Enhancement

References

General 1. Aquino SL, Duncan G, Taber KH, Sharma A, Hayman LA. Reconciliation of the anatomic, surgical, and radiographic classifications of the mediastinum. J Comput Assist Tomogr 2001; 25: 489-492. 2. Armstrong P. Mediastinal and hilar disorders. In: Armstrong P, Wilson AG, Dee P, Hansell DM, eds. Imaging of Diseases of the Chest. Third edition. London: Mosby, 2000; 789-892. 3. Felson B. Chest Roentgenology. Philadelphia: Saunders, 1973: 380-420. 4. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the anterior compartment. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré's Diagnosis of Diseases of the Chest, Fourth edition. Philadelphia: Saunders, 1999: 2875-2937. 5. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the middle-posterior mediastinal compartment. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2938-2973. 6. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the paravertebral region. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2974-2983. Chest Radiology

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7. 8. 9.

10. 11. 12. 13.

Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics 2002; 22: S79S93. Rosado de Christenson ML. Abnormalities and Diseases of the Mediastinum. In: Parker MS, Rosado de Christenson ML, Abbott GF. Teaching Atlas of Chest Imaging. New York: Thieme 2006; 621-702. Rosai J, Sobin LH. Histological Typing of Tumours of the Thymus. International Histological Classification of Tumours, Second edition. New York: Springer 1999. Shimosato Y, Mukai K. Tumors of the thymus and related lesions. In: Rosai J, ed. Atlas of Tumor Pathology: Tumors of the Mediastinum, fasc 21, ser 3. Washington, DC: American Registry of Pathology and Armed Forces Institute of Pathology, 1997: 33-247. Shimosato Y, Mukai K. Tumors of the mediastinum excluding the thymus, heart and great vessels. In: Shimosato Y, Mukai K, eds. Atlas of Tumor Pathology: Tumors of the Mediastinum, fasc 21, ser 3. Washington, DC: Armed Forces Institute of Pathology, 1997; 249-273. Strollo DC, Rosado-de-Christenson ML. Tumors of the thymus. J Thorac Imag 1999; 14: 152-171. Woodburne RT, Burkel WE. Essentials of Human Anatomy, Ninth edition. New York: Oxford University Press, 1994: 370-371.

Thymoma 1. Rosado-de-Christenson ML, Galobardes J, Moran CA. Thymoma: Radiologic-Pathologic Correlation. RadioGraphics 1992; 12: 151-168. 2. Thomas CR, Wright CD, Loehrer PJ, Sr. Thymoma: state of the art. J Clin Oncol 1999; 17: 2280-2289. 3. Tomiyama N, Müller NL, Ellis SJ, et al. Invasive and noninvasive thymoma: distinctive CT features. J Comput Assist Tomogr 2001; 25: 388-393.

Thymic Malignancy 1. Jung K-J, Lee KS, Han J, Kim J, Kim TS, Kim EA. Malignant thymic epithelial tumors: CT-pathologic correlation. AJR 2001: 176: 433-439. 2. Rosado-de-Christenson ML, Abbott GF, Kirejczyk WM, Galvin JR, Travis WD. Thoracic carcinoids: Radiologicpathologic correlation. RadioGraphics 1999; 19: 707-736. Thymolipoma 1. Rosado-de-Christenson ML, Pugatch RD, Moran CA, Galobardes J. Thymolipoma: analysis of 27 cases. Radiology 1994; 193: 121-126. Thymic Hyperplasia 1. Budavari AI, Whitaker MD, Helmers RA. Thymic hyperplasia presenting as anterior mediastinal mass in 2 patients with Graves disease. Mayo Clin Proc 2002; 77: 495-499. 2. Hara M, McAdams HP, Vredenburgh JJ, Herndon JE, Patz EF Jr. Thymic hyperplasia after high-dose chemotherapy and autologous stem cell transplantation: incidence and significance in patients with breast cancer. AJR 1999; 173: 1341-1344.

Germ Cell Neoplasms 1. Choi S-J, Lee JS, Song KS, Lim T-H. Mediastinal teratoma: CT differentiation of ruptured and unruptured tumors. AJR 1998; 171: 591-594. 2. Moeller KH, Rosado-de-Christenson ML, Templeton PA. Mediastinal mature teratoma: imaging features. AJR 1997; 169: 985-990. 3. Strollo DC, Rosado-de-Christenson ML. Primary mediastinal malignant germ cell neoplasms: imaging features. Chest Surg Clin N Am 2003; 12: 645-658. Lymphoma 1. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the international lymphoma study group. Blood 1994; 84: 1361-1392. 2. Fraser RS, Müller NL, Colman N, Paré PD. Lymphoproliferative disorders and leukemia. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 1269-1330.

Non-Neoplastic Lymphadenopathy 1. Atasoy C, Fitoz S, Erguvan B, Akyar S. Tuberculous fibrosing mediastinitis: CT and MRI findings. J Thorac Imag 2001; 16: 191-193. 2. McAdams HP, Rosado de Christenson ML, Fishback NF, Templeton PA. Castleman disease of the thorax: radiologic features with clinical and histopathologic correlation. Radiology 1998; 209: 221-228. 3. Rossi SE, McAdams HP, Rosado-de-Christenson ML, Franks TJ, Galvin JR. Fibrosing mediastinitis. RadioGraphics 2001; 21:737-757. Mediastinal Masses

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Mediastinal Cysts 1. Choi YW, McAdams HP, Jeon SC, et al. Idiopathic multilocular thymic cyst: CT features with clinical and histopathologic correlation. AJR 2001; 177: 881-885. 2. Jeung M-Y, Gasser B, Gangi A, et al. Imaging of cystic masses of the mediastinum. RadioGraphics 2002; 22: S79-S93. 3. McAdams HP, Kirejczyk WM, Rosado-de-Christenson ML, Matsumoto S. Bronchogenic cyst: imaging features with clinical and histopathologic correlation. Radiology 2000; 217: 441-446

Neurogenic Neoplasms 1. Ichikawa T, Ohtomo K, Araki T, et al. Ganglioneuroma: computed tomography and magnetic resonance features. Br J Radiol 1996; 69: 114-121. 2. Marchevsky AM. Mediastinal tumors of peripheral nervous system origin. Semin Diagn Pathol 1999; 16: 65-78. 3. Moon WK, Im J-G, Han MC. Malignant schwannomas of the thorax: CT findings. J Comput Assist Tomogr 1993; 17: 274-276. 4. Rossi SE, Erasmus JJ, McAdams HP, Donnelly LF. Thoracic manifestations of neurofibromatosis-I. AJR 1999; 173: 1631-1638. Endocrine Lesions 1. Buckley JA, Stark P. Intrathoracic mediastinal thyroid goiter: imaging manifestations. AJR 1999; 173: 471-475. 2. Fraser RS, Müller NL, Colman N, Paré PD. Masses situated predominantly in the anterior mediastinal compartment. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. 3. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2875-2937. 4. Hopkins CR, Reading CC. Thyroid and parathyroid imaging. Semin US CT MRI 1995; 16: 279-295.

Vascular Lesions 1. Charruau L, Parrens M, Jougon J, et al. Mediastinal lymphangioma in adults: CT and MR imaging features. Eur Radiol 2000; 10: 1310-1314. 2. Miyake H, Shiga M, Takaki H, Hata H, Osini R, Mori H. Mediastinal lymphangiomas in adults: CT findings. J Thorac Imaging 1996; 11: 83-85. 3. McAdams HP, Rosado-de-Christenson ML, Moran CA. Mediastinal hemangioma: radiographic and CT features in 14 patients. Radiology 1994; 193: 399-402. 4. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, Farver CF. Thoracic lymphangioma in adults: CT and MR imaging features. AJR 1994; 162:283-29-89. 5. Henseler KP, Pozniak MA, Lee FT Jr, Winter TC III. Three-dimensional CT angiography of spontaneous portosystemic shunts. RadioGraphics 2001; 21: 691-704. 6. Ibukuro K, Tsukiyama T, Mori K, Inoue Y. Preaortic esophageal veins: CT appearance. AJR 1998; 170: 15351538. 7. Ibukuro K, Tsukiyama T, Mori K, Inoue Y. Precaval draining vein from paraesophageal varices: Radiologicanatomic correlation. AJR 1999; 172: 651-654. 8. Kim M-J, Mitchell DG, Ito K. Portosystemic collaterals of the upper abdomen: Review of anatomy and demonstration on MR imaging. Abdom Imaging 2000; 25: 462-470. 9. Lee SJ, Lee KS, Kim SA, Kim TS, Hwang JH, Lim JH. Computed radiography of the chest in patients with paraesophageal varices: Diagnostic accuracy and characteristic findings. AJR 1998; 170: 1527-1531.

Miscellaneous Lesions 1. Fraser RS, Müller NL, Colman N, Paré PD. The diaphragm. In: Fraser RS, Müller NL, Colman N, Paré PD, eds. Fraser and Paré’s Diagnosis of Diseases of the Chest. Fourth edition. Philadelphia: Saunders, 1999; 2987-3010. 2. Mueller CF, Klecker RJ, King MA. Case 3. Achalasia. AJR 2000; 175: 867; 870-871 3. Woodfield CA, Levine MS, Rubesin SE, Langlotz CP, Laufer I. Diagnosis of primary versus secondary achalasia. Reassessment of clinical and radiographic criteria. AJR 2000; 175: 727-731. 4. Dunnick NR. Image interpretation session: 1999. Extramedullary hematopoiesis in a patient with beta thalassemia. RadioGraphics 2000; 20: 266-268. 5. Gilkeson RC, Basile V, Sands MJ, Hsu JT. Chest case of the day. Extramedullary hematopoiesis (EMH). AJR 1997; 169: 267, 270-273. 6. Moellers M-C, Bader JB, Alexander C, Samnick S, Kirsch C-M. Localization of extramedullary hematopoiesis with Tc-99m-labeled monoclonal antibodies (BW 250/183). Clin Nuc Med 2002; 27: 354-357.

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Chest Seminar: Where is the Lesion? Melissa L. Rosado de Christenson, MD, FACR Learning Objectives • •

To review the radiologic features of thoracic radiologic abnormalities based on location To enumerate the radiologic characteristics that allow lesion localization and the formulation of a focused radiologic differential diagnosis

Case 1: 38-year-old woman with cough • • • •

Location Differential diagnosis Next best study Diagnosis:

Solitary Lung Mass •

•

• •

Lung cancer ➢ Size / frequency ➢ Stage ? Carcinoid tumor ➢ Borders / bronchus Solitary metastasis ➢ Lower lobe location / shape Hamartoma / Infection ➢ Borders

Solitary Lung Mass • • • •

Young, relatively asymptomatic woman Mass with well-defined lobular borders Lower lobe location Abutting bronchus

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Case 2: 16-year-old girl with cough • • • •

Location Differential diagnosis Next best study Diagnosis:

Anterior Mediastinal Mass • • • • •

Young girl, relatively asymptomatic Well-defined, unilateral mass with peripheral calcification and lobular borders No lymphadenopathy Central water attenuation Low attenuation mural nodule

•

Mature teratoma ➢ Fluid / fat / Ca++ Thymic cyst ➢ Fluid / Ca++ Lymphoma ➢ Age group … but no lymphadenopathy Thymoma ➢ Fluid, calcium, mural nodule … but fat

Anterior Mediastinal Mass • •

•

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Case 3: 58-year-old man with chest pain and hemoptysis • • • •

Location Differential diagnosis Next best study Diagnosis:

Lung Mass with Cavitation and Chest Wall Involvement • • • •

Symptomatic older male Chest wall invasion (rib destruction) Upper lobe location Cavitation

•

Bronchogenic carcinoma ➢ Chest wall invasion ➢ Stage? Infection ➢ Actinomycosis, tuberculosis, fungus Primary chest wall tumor / Metastasis Other

Chest Wall Mass / Cavitation •

• •

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Case 4: Asymptomatic 40-year-old male; pre-operative radiograph • • • •

Location Differential diagnosis Next best study Diagnosis:

Multifocal Pleural Nodules • • • •

Asymptomatic patient No known malignancy Well-defined peripheral pleural-based nodules Associated findings - Abdominal abnormalities?

•

Splenosis ➢ Where is the spleen? Metastases Malignant pleural mesothelioma Other

Multifocal Pleural Nodules • • •

Splenosis • • •

Auto-transplantation of splenic tissue typically following splenic rupture Most common manifestation: Multiple peritoneal nodules Thoracic splenosis: ➢ Multiple pleural-based nodules ➢ May be missed on radiography ➢ 99mTC-tagged heated RBC scintigraphy ➢ Liver-spleen scan

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Chest Seminar: Where is the Lesion

Case 5: 34-year-old man with left chest pain for many years • • • •

Location Differential diagnosis Next best study Diagnosis:

Multifocal Chest Wall and Mediastinal Masses • • • • •

Chronic lesions with minimal symptoms Unilateral or bilateral? Benign pressure erosion Pulmonary involvement Other chest wall / mediastinal involvement

•

Neurofibromatosis ➢ Malignant potential Vascular lesions Metastases Other

Multifocal Chest Wall and Mediastinal Masses • • •

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Chest Seminar: Differential Diagnosis of Mediastinal Masses Melissa L. Rosado de Christenson, MD, FACR Learning Objectives • •

To review concepts of differential diagnosis of mediastinal masses To emphasize importance of demographics, location and morphology in the formulation of a focused differential diagnosis

Case 1: Elderly man with chest pain • • • • •

Location Characterization Next study Biopsy Differential diagnosis

Heterogeneous Middle Mediastinal Mass; Rim CA++ • • •

•

Neoplasia ➢ Carcinoma ➢ Lymphoma Congenital Cyst … but heterogeneous Vascular lesion ➢ Aneurysm – rim CA++ Other

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Case 2: Asymptomatic 52-year-old man • •

Differential diagnosis Next best study

Spherical Paravertebral Mass with Pressure Erosion •

• •

Neurogenic Neoplasm ➢ Next study? Lateral Thoracic Meningocele ➢ History? Other

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Case 3: 40-year-old woman with difficulty swallowing • •

Differential diagnosis Next best study

Unilateral Cystic Anterior Mediastinal Mass; Mural CA++ •

• •

•

Cystic Thymoma ➢ Mural nodule Cystic Teratoma ➢ No fat no calcium Thymic Cyst / Pericardial Cyst ➢ but mural nodule Cystic Lymphoma ➢ but no lymphadenopathy

Unilateral Cystic Anterior Mediastinal Mass; Mural CA++ and mural nodule • • •

Symptomatic woman ➢ Symptoms related to function of voluntary musculature Age over 40 Pattern of enhancement ➢ Mural nodule

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Chest Seminar: Differential Diagnosis of Mediastinal Masses

Case 4: 24-year-old man with chronic abdominal discomfort • • •

Differential diagnosis Diagnosis Should the lesion be excised?

Right Cardiophrenic Angle Mass of Fat and Soft Tissue Attenuation •

• •

•

Thymolipoma ➢ Fat / soft tissue ➢ Does not conform to adjacent structures / thymus? Lipoma ➢ Fat / soft tissue Mature Teratoma ➢ But...No fluid Morgagni Hernia ➢ Continuity with abdominal fat

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Case 5: 29-year-old woman with fatigue and cough • • •

Differential diagnosis Diagnosis Should the lesion be excised? ➢ Biopsied?

Anterior Mediastinal Mass (Cystic change, Ca++) •

• •

•

Lymphoma: ➢ Age, local invasion, lymphadenopathy ➢ Cystic change, Ca++ ? Thymoma ➢ Cystic change, Ca++,local invasion ➢ But…lymphadenopathy Teratoma ➢ But…lymphadenopathy, local invasion and soft tissue predominant Malignant GCN ➢ But…wrong gender

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Pneumonia: Usual and Unusual Organisms Rosita M. Shah, MD

Classification of Pulmonary Infection •

• •

Community-acquired Pneumonia ➢ S. pneumoniae LOBAR ➢ Mycoplasma LOBULAR ➢ Influenzae INTERSTITIAL Nosocomial Pneumonia Unusual Pulmonary Infections

Pulmonary Infection: Classification: Morphology •

3 radiographic and pathologic patterns ➢ Lobar ➢ Lobular (bronchopneumonia) ➢ Interstitial

Pulmonary Infection: Classification • •

Lobar and lobular pneumonias both produce air space filling Significant differences include: ➢ Site of initial inflammation ➢ Degree of lobular opacification ➢ Radiographic pattern ➢ Etiologic agents

Alveolar Filling Pneumonias •

Site of initial infection varies ➢ Alveolar level in lobar pneumonia ➢ Bronchiolar level in bronchopneumonia

Alveolar Filling Pneumonias •

Degree of opacification of secondary lobule is different ➢ Complete in lobar pneumonia ➢ Incomplete in bronchopneumonia

Alveolar Filling Pneumonias •

Radiographic pattern will vary ➢ Lobar pattern ➢ Bronchopneumonia pattern

Alveolar Filling Pneumonias • Etiologic agent may vary Lobar Pneumonia S.pneumoniae K.pneumoniae also seen with Legionella Mycoplasma H.influenzae

Pneumonia

Bronchopneumonia

Gram –’s, anaerobes Legionella Actinomycosis Nocardia Mycoplasma Typical, atypical TB Parasites

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Alveolar Filling Pneumonias ·

· ·

Accurate pattern recognition depends on: ➢ Early imaging ➢ Normal lung structure Organisms may produce more than one pattern Basic pattern differentiation may be difficult ➢ Interstitial vs bronchopneumonia

Community-acquired Pneumonia: Epidemiology · ·

2–10 /1000 annual incidence 22-50% hospitalization rate ➢ Outpatient mortality 1-5% ➢ Inpatient mortality 25%

Community-acquired Pneumonia: Etiology · ·

In up to 50%, no definitive organism isolated Most common isolates: ➢ S. pneumoniae ➢ M. pneumoniae ➢ K. pneumoniae ➢ H. influenzae ➢ L. pneumophila ➢ Respiratory viruses

S. pneumoniae: Demographics · ·

S. pneumoniae most frequent isolate in CAP ➢ 8-76% incidence Recognized risk factors ➢ alcoholism, splenic dysfunction, viral pneumonia, congenital and acquired immune deficiencies

S. pneumoniae: Demographics · ·

25% incidence of bacteremia 25-40% mortality, unchanged >30y ➢ Age >65 ➢ CHF,DM ➢ Alcoholism ➢ Thrombocytopenia ➢ Renal dysfunction ➢ Number of lobes

Chest 1993; 103:1152-56

S. pneumoniae: Pathology · · · ·

Aspiration to peripheral air spaces Alveolus represents site of initial inflammatory lesion Spread occurs by contignous involvement of adjacent alveoli 3 pathologic stages

S. pneumoniae: Pathology ·

· ·

ACUTE RESPONSE ➢ Increased capillary permeability ➢ Protein rich edema ➢ Contiguous alveolar filling via Pores of Kohn and Canals of Lambert RED HEPATIZATION ➢ PMN infiltration and intra-alveolar hemorrhage GRAY HEPATIZATION ➢ Macrophage infiltration and uptake of blood products

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S. pneumoniae: Radiology [Figure 1-19-1] ·

LOBAR pattern ➢ Homogeneous, confluent density ➢ Nonsegmental distributions

S. pneumoniae: Radiology · ·

Spread at alveolar level results in nonsegmental distributions characteristic of early lobar pneumonia Round pneumonia ➢ Manifestation of nonsegmental distribution ➢ Most common in pediatric infection with S.pneumoniae

Figure 1-19-1

S. pneumoniae: Radiology ·

LOBAR pattern ➢ Prominent air bronchograms ➢ Preserved volume

S. pneumoniae: Radiology · · · ·

48% of consecutive hospitalized pts demonstrated focal lobar patterns 33%, multifocal lobar patterns 16% lobular pattern Dominant pattern did not vary with immune status or disease severity

AJR 2000;175:1533

Lobar pattern consolidation due to S. pneumoniae

S. pneumoniae: Radiology · ·

·

Small pleural effusions up to 60% Infrequent cavitation ➢ Associated with serotype 3 Most frequent organism in pulmonary gangrene ➢ Vascular thrombosis from severe necrosis ➢ Intracavitary mass (sloughed lung)

M. pneumoniae: Demographics · · · ·

15-35% of CAP ➢ 50% of CAP during summer months Peak age 5-25 yo Self limited ➢ Few fatal cases associated with ARDS ➢ Increased severity in sickle cell anemia Most frequent etiology in Atypical Pneumonia Syndrome ➢ Atypical radiographic features ➢ Prominent extrapulmonary complaints

M. pneumoniae: Pathology ·

·

Eaton agent-1944 ➢ Gram -- filamentous rod ➢ Absent cell wall Acute cellular bronchiolitis ➢ Superficial inflammation involving luminal surface of bronchi, bronchioles ➢ Associated interstitial infiltrates

Pneumonia

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M. pneumoniae: Radiology [Figure 1-19-2] · ·

· · ·

Figure 1-19-2

LOBULAR pattern Bronchopneumonia Heterogeneous, patchy consolidation ➢ Minimal exudate into centrilobular alveoli Segmental distribution ➢ Spread at bronchiolar level Volume loss Minimal air bronchograms ➢ Peribronchial thickening

M. pneumoniae: Radiology ·

CT Findings [Figure 1-19-3] ➢ 86% centrilobular nodules ➢ 82% bronchovascular thickening ➢ 59% consolidation with lobular distribution

Reittner, AJR 2000; 174:37

Bronchopneumonia pattern due to M. pneumoniae

Respiratory Viruses · · · · · ·

Influenzae A,B,C Para-influenzae Respiratory syncytial virus Adenovirus Herpes viruses SARS

Figure 1-19-3

Influenzae A: Demographics · · ·

·

10-20% CAP 10,000-40,000 deaths/ influenzae epidemic Peak incidence ➢ Pediatric population Highest mortality-adult and aged ➢ Superinfection ➢ S.aureus ➢ S.pneumoniae

Influenzae A: Pathology · · ·

St 1 infection of epithelial cells, proliferation and necrosis St 2 bronchial and alveolar wall edema,hemorrhage Ulceration, bacterial infection

HRCT of M. pneumoniae

Influenzae A: Radiology ·

INTERSTITIAL pattern ➢ Reticular ➢ Nodular ➢ Peribronchial thickening ➢ Subpleural edema ➢ Hilar haze

Figure 1-19-4

Influenzae A: Radiology [Figure 1-19-4]

· · ·

Bilateral, parahilar, lower lobe Air trapping Prominent GGO

(left) CXR (right) HRCT Influenzae pneumonia mimicking edema

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Pneumonia

Influenzae A: Radiology · ·

Pleural effusions, cavitation uncommon without bacterial superinfection Rapid deterioration should suggest superinfection

Adenovirus [Figure 1-19-5] · ·

·

Interstitial pneumonia with prominent necrotizing bronchiolitis Potential infection in immune competent and suppressed hosts with high mortality ➢ Pediatric population ➢ Military epidemics ➢ Transplant recipients Swyer James, Macleod’s syndrome ➢ Bronchiolitis obliterans following viral infection in early childhood

Figure 1-19-5

Respiratory Herpesviruses ·

·

HSV-1, HSV-2, VZV, EBV, CMV ➢ Primary infection, latency, reactivation ➢ Up to 40% mortality Risk factors ➢ Immune-suppression, lung transplantation, airway management, pregnancy

Swyer James Syndrome due to pediatric viral pneumonia

Varicella Pneumonia ·

·

Figure 1-19-6

Complication of adult chickenpox ➢ 5-50% incidence Prominent acinar opacities ➢ 5-10mm nodules, coalescence ➢ Patchy GGO

Kim AJR 1999;172:113 · May heal with miliary calcifications

Varicella Pneumonia [Figure 1-19-6] ·

Prominent acinar opacities

Severe Acute Respiratory Syndrome · · · · ·

SARS-CoV (corona virus) Initial cases Nov 2002-June 2003, rapid spread from Asia 20-50% require mechanical ventilation 10% mortality, age dependant Severe DAD

Severe Acute Respiratory Syndrome ·

· · · ·

Acinar nodules in varicella pneumonia

Predominant consolidation 1-2weeks ➢ Focal (39%), multifocal (28%), diffuse (14%) Ground glass opacity Reticulation Bronchiolar dilation Residual changes in 50% at 4wks

Ooi GC. Radiology 2004;230:836; Paul NS. AJR 2003;182:493

Severe Community-acquired Pneumonia: Definition · · ·

Impending respiratory failure Hemodynamic instability Radiographic assessment ➢ Bilateral or multilobar involvemnt ➢ 50% increase in size of opacity within 48hr

Pneumonia

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Severe Community-acquired Pneumonia: Etiology

· · · ·

S. pneumoniae L. pneumophila S. aureus P. aeruginosa in patients with bronchiectasis

L. pneumophila: Demographics ·

· ·

15% of CAP ➢ Epidemic and sporadic forms ➢ Legionnaire’s disease= pneumonic form ➢ Peak summer Aerobic Gram -- bacillus Proliferates in warm, humid environments

Figure 1-19-7

L. pneumophila: Pathology ·

Bronchocentric inflammation

L. pneumophila: Demographics · ·

· ·

Acute onset Prominent extrapulmonary symptoms ➢ Neurologic manifestations, diarrhea, renal insufficiency 10% mechanical ventilation 15% mortality in cases requiring hospitalization

L. pneumophila: Radiology [Figure 1-19-7] · · · · · ·

Bronchopneumonia pattern Pleural effusions in 2/3 Bilateral and multifocal in 50% May produce lobar or mass-like consolidation Cavitation uncommon without immunosupression Delayed resolution

K. pneumoniae: Demographics · · · · ·

Nosocomial or community acquired 5-10% lobar pneumonias 25% bacteremic, 50% mortality Males, >60yo Risk factors: alcoholism, COPD, DM

K. pneumoniae: Pathology ·

Gram -- bacillus ➢ Abundant PMN infiltration of alveoli, edema ➢ Lobar expansion - Friedlander’s pneumonia ➢ Massive necrosis ➢ Common association with gangrene

HRCT in Legionella pneumonia demonstrating bronchocentric nodules and pleural effusion

K. pneumoniae: Radiology ·

· ·

Lobar pattern ➢ Bulging fissures Abscess 30-50% Necrotizing pneumonia at CT ➢ Low density areas with small cavities

Moon JCAT 1995;19:176

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S.aureus: Demographics ·

· · · · ·

Figure 1-19-8

30-50% colonization rates in healthy adults ➢ DM ➢ IVDA ➢ HIV ➢ Surgical pts Methicillin resistance 1944 ➢ Increasing incidence of resistant community-acq infection Antecedant viral pneumonia Frequent cause of nosocomial infection Extremes of age ➢ Nursing home population Risk factors ➢ Debilitated states, mechanical ventilation, burns, indwelling catheters, IVDA

S. aureus: Radiology · · · · ·

Aerogenous infection [Figures 1-19-8 and 1-19-9] ➢ Multifocal Broncho-pneumonia Hematogenous infection [Figure 1-19-10] ➢ Multifocal, discrete nodular or wedge shaped abnormality with normal intervening lung Cavitation / abscess (25-75%) Pneumatoceles (60% ped infection) Pleural effusions / empyema (50%)

CXR and HRCT bronchopneumonia pattern due to S. aureus

Figure 1-19-9

Necrotizing bronchopneumonia due to S. aureus

Pneumonia

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P. aeruginosa in Cystic Fibrosis ·

Figure 1-19-10

Chronic colonization with P.aeruginosa ➢ Mucoid variant ➢ ABX resistance ➢ Elastase production ➢ Bronchiectasis

The Practical Points · · · · ·

S.pneumoniae and K.pneumoniae most commonly associated with lobar pattern and pulmonary gangrene M.pneumoniae, L.pneumophilus most commonly associated with broncho-pneumonia pattern and atypical pneumonia syndrome Viral pneumonias associated with interstitial pattern Pathologic in immune-competent and suppressed hosts Prominent bronchiolitis seen with mycoplasma, adeno and other respiratory viruses

Septic emboli

Nosocomial Pneumonia · ·

Rising incidence parallels usage of antibiotics Gram negative infections ➢ 40-50% increase 1950-60 ➢ 55-65% nosocomial infections ➢ 50% nosocomial pneumonia ➢ 75% ICU pneumonia

Nosocomial Pneumonia: Definition ·

Pneumonia developing >48hr sp admission, intubation or discharge

Diagnosis ·

Quantitative cultures ➢ Tracheal aspirate 10 5-6 cfu/ml ➢ BAL 10 4 cfu/ml ➢ Protected specimen brush 10 3 cfu/ml ➢ False negative and false positive rates 20-30%

Baughman Chest 2000

Pathophysiology · · ·

Direct inhalation Hematogenous spread Aspiration ➢ 45% incidence in sleep ➢ Altered gag reflex, consciousness, GI motility ➢ NG / ET

Pathophysiology ·

· · ·

Abnormal gram negative airway colonization ➢ 25% 24hr ➢ 40% 7d Gastric alkalinization Serious illness Antibiotic TX

Johanson Ann Intern Med 1972

Pathophysiology · · ·

Repetitive aspiration leads to Bronchiolitis Lobular (broncho) pneumonia ➢ Peribronchiolar neutrophilic infiltrate (104 cfu/g)

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Nosocomial Pneumonia in the ICU · ·

·

Ventilator-associated Pneumonia (VAP) Most common nosocomial infection in ICU ➢ 3-21x greater incidence in intubated patients ✧ > 1 intubation, >3 days ➢ 10-65% ICU patients acquire VAP ➢ 1/3-3/4 ARDS patients acquire VAP 20-80% mortality rate

Figure 1-19-11

Ventilator-associated Pneumonia ·

Prognosis depends: ➢ Organism ✧ Highest mortality: P.aeruginosa, MRSA ➢ Population ✧ Highest mortality: medical ICU ✧ 10-20% mortality: trauma ICU ➢ Late onset ✧ MDR

Nosocomial Pneumonia and Aspiration [Figure 1-19-11] ·

Pneumonitis (Mendelson Syndrome) ➢ pH < 2.5, >0.3 ml/kg ➢ Biphasic inflammatory response ✧ 1-2 hr permeability edema ✧ 4-6 hr acute inflammation ➢ 30% mortality ➢ Rapid clearing

Nosocomial Pneumonia and Aspiration · · · · ·

Sterile vs Normal flora (<5d) vs Gram – bacilli(>5d) Anaerobes seen in late aspiration Aspiration accounts for upto 15% of CAP

Dependent aspiration pneumonia complicated by ARDS

Marik NEJM 2001

Microbiology · · · · · ·

Normal flora Gram – bacilli S. aureus Anaerobes Legionella Respiratory viruses

Microbiology ·

·

Early <5d ➢ H. influenzae ➢ S. pneumoniae ➢ S. aureus Late >5d ➢ S. aureus ➢ P. aeruginosa ➢ Enterobacteriaceae ➢ Acinetobacter spp. ➢ Stenotrophomonas maltophilia

Pneumonia

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Chest Radiology

P. aeruginosa • • • •

Most common ICU isolate 70% TX failure rate Distal airway colonization, hematogenous dissemination Increased severity in neutropenia, bacteremia

• •

Micro-abscesses Necrotic vasculits ➢ Sm-med pulmonary arteries Hemorrhage

P. aeruginosa: Pathology •

P. aeruginosa: Radiology •

• •

Bronchopneumonia pattern ➢ Discrete nodules may be indicative of vasculitis Frequent cavitation Pleural effusions/empyema

Nosocomial Viral Pneumonia • • • •

Rate of infection assoc with length of hospitalization Hospital worker as carrier Frequently unsuspected High mortality rates ➢ RSV 30-100% ➢ Parainfluenzae 15-30%

Nodular or Mass-like Consolidations • •

Nonsegmental distribution ➢ ‘round’ pneumonia Granulomatous infection ➢ M. tuberculosis ➢ Fungi ➢ Actinomycosis ➢ Nocardia

A. Israelii; Demographics • •

•

Figure 1-19-12

Normal oral flora Sites of infection: ➢ Cervicofacial 55% ➢ Abdomen 20% ➢ Pulmonary 25% Risk factors: poor oral hygiene, aspiration

Smego RA. Clin Infec Dis 1998;26:1255

A. Israelii: Pathology • • •

Multifocal abscesses Interconnecting sinus tracts Sulphur granule ➢ Spoke-wheel arrangement of neutrophils surrounding filamentous organism

A. Israelii: Radiology [Figure 1-19-12] •

•

Pneumonia with chest wall involvement due to A. israelli

Consolidation ➢ Mass-like ➢ Cavitary Pleural, chest wall and osseous involvement ➢ Up to 50%

Chest Radiology

187

Pneumonia

N. Asteroides: Demographics

· · ·

Ubiquitous distribution 50% of patients are immunocompetent Risk factors: ➢ Neutropenia ✧ Steroids, late HIV, hemetologic malignancy, alveolar proteinosis

N. Asteroides: Pathology · ·

Peribronchial abscesses, granulomatous inflammation Extensive necrosis ➢ May mimic M.TB or fungal infection

N. Asteroides: Radiology ·

Extrapulmonary disease 50% with 40-90% mortality ➢ CNS 25% ➢ Skin and subcutaneous abscesses

N. Asteroides: Radiology ·

· ·

Consolidation ➢ Mass-like ➢ Cavitary Pleural and chest wall involvement 30-50% Adenopathy 40%

Cavitary Pneumonia in AIDS ·

N. asteroides

Alveolar Proteinosis and N. asteroides Parasitic Infection ·

Pulmonary involvement due to hypersensitivity or direct invasion ➢ Echinococcosis ➢ Paragonimiasis ➢ Ascariasis ➢ Strongyloidiasis

Figure 1-19-13

Parasitic Infection ·

Radiographic findings may overlap with other infections ➢ Fleeting, patchy infiltrates ➢ Reticulonodular opacities ➢ Bronchopneumonia ➢ Atelectasis

Echinococcus granulosus · ·

Cestode (tapeworm), endemic to S.America, Australia, Middle East, Africa and Mediterranean Definitive host - dog,wolf Intermediate host - sheep, cow, deer, moose

Echinococcus granulosus ·

Duodenum - portal venous system liver ➢ 45-75% isolated liver involvement ➢ 15-35% pulmonary involvement

Intact (right lung) and ruptured (left lung) echinococcal cysts

Echinococcus granulosus [Figure 1-19-13] · · · ·

Pulmonary cysts acquired in childhood Diagnosis 30-40yo Intact cyst - asymptomatic Eosinophilia 25-40%

Pneumonia

188

Chest Radiology

Echinococcus granulosus: Pathology ·

Hydatid cyst consists of 3 layers ➢ Pericyst – host inflammatory cells ➢ Exocyst – acellular laminated membrane ➢ Endocyst – fluid-filled germinal center, daughter cysts

Echinococcus granulosus: Radiology ·

Intact cyst ➢ Well demarcated, homogeneous mass ➢ Spherical when central, ovoid when peripheral ➢ Multiple 20-30% ➢ Lower lobes 60%

Echinococcus granulosus: Radiology ·

·

Impending Rupture ➢ Crescent sign - air between pericyst and laminated membrane Ruptured cyst ➢ Water lily sign – rupture of endocyst

Paragonimiasis westermani ·

· ·

Trematode (lung fluke) ➢ endemic to Asia ➢ Contaminated freshwater crab Jejunum – peritoneal cavity – diaphragm – pleura – lung Chronic granulomatous reaction

Paragonimiasis westermani: Radiology ·

·

Pulmonary findings dependant on stage of infection ➢ PTX and pleural infection during pleural penetration by juvenile worms ➢ Transient, patchy consolidation and linear tracts during larval migration Peribronchial cysts associated with mature worm

Ascariasis lumbricoides · ·

· ·

Roundworm infection Most common parasitic infection ➢ Endemic worldwide ➢ 25-95% prevalence ➢ Highest incidence in children Large iingestion associated with pneumonitis Small bowel – systemic circulation – alveoli - trachea – small bowel

Strongyloides stercoralis · ·

Round worm Skin – systemic circulation – alveoli – trachea – small bowel

Ascariasis Strongyloides: Radiology · · ·

Bronchopneumonia Patchy, transient consolidation Eosinophilic pneumonia

B. Anthracis: Anthrax ·

· ·

Gram+ spore forming rod ➢ Dormant spores are virulent Infection typical in livestock Exotoxin production associated with hemorrhagic mediastinitis, edema and pleuritis

Earls Radiology:222:305, 2001

Chest Radiology

189

Pneumonia

Complications of Pneumonia [Figure 1-19-14] · ·

Figure 1-19-14

Pleural Infection Empyema ➢ Purulent exudate ➢ WBC>25,000 ➢ pH<7.0 ➢ + organisms

S.mitis Empyema in 51yo male with IVDA hx Complications of Pneumonia ·

Right empyema and LUL septic embolus due to S. aureus

Cavitation ➢ Cavitary pneumonia ➢ Lung abscess ➢ Pneumatocele ➢ Gangrene ➢ DDX bronchopleural fitula

Complications of Pneumonia ·

·

Pneumatocele ➢ Ball-valve mechanism ➢ Rapid evolution ➢ No lung destruction Most common with S.aureus ➢ 60% of peds infection

Figure 1-19-15

Complications of Pneumonia [Figure 1-19-15] ·

· ·

Pulmonary Gangrene ➢ Lung necrosis due to vascular thrombosis Most common with S.pneumoniae K.pneumoniae Bronchiectasis ➢ Irreversible dilation ✧ Should not be diagnosed < 4 m of acute infection ➢ Colonization with atypical TB, aspergillus ➢ Advanced course in HIV ➢ +/- antecedant infection

CXR and CT pulmonary gangrene due to K.pneumoniae

A.fumigatus complicating post-infectious bronchiectasis The Role of Imaging in Pneumonia ·

Diagnosis of infection ➢ Presence of centrilobular nodules in acute parenchymal disease favors pneumonia Tomiyama N. AJR 2000;174:1745 ➢ Thin section CT allows earlier diagnosis of pneumonia in immunosuppressed pts (5 days)

·

Heussel CP. AJR 1997;169:1347 Recognition of complications ➢ Decreased enhancement in pneumonia indicates severe necrosis Donnelly LF. Radiology 1997;205:817

The Practical Points · ·

·

Organisms may produce more than one pattern Bacterial, viral and fungal pneumonia have similar CT findings post lung transplantation Collins AJR 2000;175:811 Consider clinical setting

Pneumonia

190

Chest Radiology

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16.

Kim JS, Ryu CW, Lee SI, Sung DW, Park CK. High-resolution CT findings of varicella-zoster pneumonia. AJR Am J Roentgenol. 1999 Jan;172(1):113-6. Moon WK, Im JG, Yeon KM, Han MC. Complications of Klebsiella pneumonia: CT evaluation. J Comput Assist Tomogr. 1995 Mar-Apr;19(2):176-81. Ooi GC, Khong PL, Muller NL, Yiu WC, Zhou LJ, Ho JC, Lam B, Nicolaou S, Tsang KW. Severe acute respiratory syndrome: temporal lung changes at thin-section CT in 30 patients. Radiology. 2004 Mar;230(3):83644. Paul NS, Chung T, Konen E, Roberts HC, Rao TN, Gold WL, Mehta S, Tomlinson GA, Boylan CE, Grossman H, Hong HH, Weisbrod GL. Prognostic significance of the radiographic pattern of disease in patients with severe acute respiratory syndrome. AJR Am J Roentgenol. 2004 Feb;182(2):493-8. Paul NS, Roberts H, Butany J, Chung T, Gold W, Mehta S, Konen E, Rao A, Provost Y, Hong HH, Zelovitsky L, Weisbrod GL. Radiologic pattern of disease in patients with severe acute respiratory syndrome: the Toronto experience. Radiographics. 2004 Mar-Apr;24(2):553-63. Review. Reittner P, Muller NL, Heyneman L, Johkoh T, Park JS, Lee KS, Honda O, Tomiyama N. Mycoplasma pneumoniae pneumonia: radiographic and high-resolution CT features in 28 patients. AJR Am J Roentgenol. 2000 Jan;174(1):37-41. Shah RM, Gupta S, Angeid-Backman E, O'Donnell J. Pneumococcal pneumonia in patients requiring hospitalization: effects of bacteremia and HIV seropositivity on radiographic appearance. AJR Am J Roentgenol. 2000 Dec;175(6):1533-6. Watanakunakorn C, Greifenstein A, Stroh K, Jarjoura DG, Blend D, Cugino A, Ognibene. AJ. Pneumococcal bacteremia in three community teaching hospitals from 1980 to 1989.Chest. 1993 Apr;103(4):1152-6. Collins J, Muller NL, Kazerooni EA, Paciocco G. CT findings of pneumonia after lung transplantation. AJR Am J Roentgenol. 2000 Sep;175(3):811-8. Donnelly LF, Klosterman LA. Pneumonia in children: decreased parenchymal contrast enhancement--CT sign of intense illness and impending cavitary necrosis. Radiology. 1997 Dec;205(3):817-20. Earls JP, Cerva D Jr, Berman E, Rosenthal J, Fatteh N, Wolfe PP, Clayton R, Murphy C, Pauze D, Mayer T, Bersoff-Matcha S, Urban B. Inhalational anthrax after bioterrorism exposure: spectrum of imaging findings in two surviving patients. Radiology. 2002 Feb;222(2):305-12. Fartoukh M, Azoulay E, Galliot R, Le Gall JR, Baud F, Chevret S, Schlemmer B. Clinically documented pleural effusions in medical ICU patients: how useful is routine thoracentesis? Chest. 2002 Jan;121(1):178-84. Heussel CP, Kauczor HU, Heussel G, Fischer B, Mildenberger P, Thelen M. Early detection of pneumonia in febrile neutropenic patients: use of thin-section CT. AJR Am J Roentgenol. 1997 Nov;169(5):1347-53. Johanson WG Jr, Pierce AK, Sanford JP, Thomas GD. Nosocomial respiratory infections with gram-negative bacilli. The significance of colonization of the respiratory tract. Ann Intern Med. 1972 Nov;77(5):701-6. Smego RA Jr, Foglia G. Actinomycosis. Clin Infect Dis. 1998 Jun;26(6):1255-61; quiz 1262-3. Review. Tomiyama N, Muller NL, Johkoh T, Honda O, Mihara N, Kozuka T, Hamada S, Nakamura H, Akira M, Ichikado K. Acute parenchymal lung disease in immunocompetent patients: diagnostic accuracy of high-resolution CT. AJR Am J Roentgenol. 2000 Jun;174(6):1745-50.

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Uncommon Malignant Tumors of the Lung Gerald F. Abbott, MD

Uncommon Malignant Tumors of the Lung: • • • • •

Bronchial Carcinoid - most common “Uncommon” Adenoid Cystic Carcinoma ----> Salivary Gland Tumors Mucoepidermoid Carcinoma----> Salivary Gland Tumors Carcinosarcoma ----> Mixed Tumors Pulmonary Blastoma ----> Mixed Tumors

•

• •

Term formerly referred to: ➢ Bronchial Carcinoid ➢ Adenoid Cystic Carcinoma ➢ Mucoepidermoid Carcinoma A misnomer These tumors are not benign

• • • • •

Gastrointestinal tract 90% Lung Thymus Biliary tract Ovarian teratomas

•

Typical carcinoid ➢ Low-grade malignancy Atypical carcinoid ➢ Moderate-grade malignancy

“Bronchial Adenoma”: History Lesson

Carcinoids

Bronchial Carcinoid •

Typical Carcinoid

•

0.6-2.4% of all pulmonary neoplasms Low grade malignancy Good prognosis ➢ 95% five-year survival Not associated with smoking

• • •

Males = Females Wide age range. Median age: 50 years Symptoms: cough, hemoptysis, dyspnea

• • • • • • •

Uniform cells Forming nests, ribbons, rosettes, trabeculae Stroma highly vascular May exhibit calcification or osseous metaplasia Polygonal cells, pale cytoplasm, stippled nuclear chromatin Rare mitoses Ultrastructure: Neurosecretory granules

• • • •

Cytoplasmic neurosecretory granules Central or eccentric dense core Thin lucent halo May contain biologically active peptides

• • •

Typical Carcinoid: Demographics Typical Carcinoid: Microscopy

Neuroendocrine Cells and Tumors: Electron microscopy

Uncommon Malignant Tumors

192

Chest Radiology

Neuroendocrine Markers: Immunohistochemistry • • •

Chromogranin Synaptophysin Neural cell adhesion molecules (NCAM)

•

Similarities: ➢ Neurosecretory granules ➢ Rosette and trabecula formation Differences: ➢ Fewer granules in Small Cell Carcinoma ➢ Carcinoid not associated with smoking

Carcinoid: Relationship to Small Cell Carcinoma •

Tumors with NE Morphology • • • •

A spectrum by light micropscopy Low-grade Intermediate High-grade

Typical Carcinoid Atypical Carcinoid Small cell lung carcinoma Large cell neuroendocrine carcinoma

Neuroendocrine Tumors: World Health Organization criteria (1999) [Figures1-20-1]

• • • •

Typical carcinoid: <2 mitoses per 10 HPF Atypical carcinoid: 2-10 mitoses per 10 HPF Large cell neuroendocrine ca: 11 or more mitoses per 10 HPF (median 70) Small cell ca: 11 or more mitoses per 10 HPF (median 80)

Figure 1-20-1 NE Tumors: Metastases

Atypical Carcinoid: Histopathologic criteria • • • •

Poor architectural organization Cellular pleomorphism Focal necrosis Increased mitotic activity

Arrigoni J Thorac Cardiovasc Surg 1972

Atypical Carcinoid • • • • •

Morphology between Typical Carcinoid and Small Cell Ca Tend to be larger, more invasive, peripheral Age: Decade older than Typical Symptoms: similar to Typical Imaging: similar to Typical

Chest Radiology

193

Uncommon Malignant Tumors

Atypical Carcinoid • • • • • •

10% of bronchial carcinoid Peripheral Increased mitoses Aggressive behavior; early metastases Osteoblastic bone metastases Pathology DDx: Small Cell Carcinoma

Bronchial Carcinoid: Gross Pathology [Figure 1-20-2]

• • • •

Usually seen at bronchoscopy Soft, fleshy, endobronchial mass Sessile. May be pedunculated Often extend through wall

Figure 1-20-2

Growth patterns of bronchial carcinoid tumors. Partially endobronchial tumors (“iceberg” configuration) most common

Bronchial Carcinoid: Central • • •

Bronchiectasis Mucoid impaction Obstructive pneumonia

• • •

Usually asymptomatic Late presentation Discovered incidentally

Bronchial Carcinoid: Peripheral

Uncommon Malignant Tumors

194

Chest Radiology

Bronchial Carcinoid: Radiologic Findings

Figure 1-20-3

[Figures 1-20-3 and 1-20-4]

• • • •

Central tumors in 80% Lobar, segmental, subsegmental bronchi Consolidation, atelectasis Pleural effusion

Figure 1-20-4

Small endobronchial carcinoid tumor in distal left mainstem bronchus

Bronchial Carcinoid: CT Features [Figures 1-20-5 to 1-20-7] • • • • • • •

•

Bronchial relationship in 83% Partially endobronchial Completely endobronchial Abutting a bronchus Sharply marginated, lobulated mass May enhance or demonstrate Ca++ Atelectasis, consolidation, bronchiectasis, mucoid impaction Lymphadenopathy

Figure 1-20-6

Bronchial carcinoid manifesting as a central, well-defined left perihilar mass

Figure 1-20-5

Bronchial carcinoid. Partially endobronchial (‘iceberg”) tumor in left upper lobe

Figure 1-20-7

Bronchial carcinoid obstructing left upper lobe bronchus with distal atelectasis, pneumonitis and associated left hilar lymphadenopathy

Bronchial carcinoid in a 34-year-old male. Unenhanced CT (left) demonstrates central tumor and distal, peripheral consolidation. Contrast enhanced CT (right) shows diffuse contrast enhancement Chest Radiology

195

Uncommon Malignant Tumors

Bronchial Gland Tumors • • •

Adenoid Cystic Carcinoma Mucoepidermoid Carcinoma Equivalent to salivary gland tumors of same name

• • • •

Synonym - Cylindroma 80% of bronchial gland tumors 20 to 35% of all tracheal tumors Second most common tracheal malignancy (after Squamous cell carcinoma)

Figure 1-20-8

Adenoid Cystic Carcinoma [Figure 1-20-8]

Adenoid Cystic Carcinoma • • • •

Guarded prognosis Common local recurrence Occasional metastases to regional nodes Rarely extrathoracic spread

• • •

Males = Females Wide age range - Average age: 40 - 50 Symptomatic patients: cough, wheezing, dyspnea, hemoptysis

• • • • • •

Mucin-containing cysts Varying in caliber Within larger tumor tubules Surrounds, invades nerves Encases vessels, infiltrates bronchi Few mitoses

•

Misdiagnosis ➢ Adenocaracinoma ➢ Pleomorphic adenoma ➢ Small cell carcinoma Metastatic salivary gland tumor Solid pattern on small biopsy

Adenoid Cystic Carcinoma: Demographics

Adenoidcystic carcinoma partially occluding the trachea and extending into the adjacent soft tissues of the mediastinum

Adenoid Cystic Carcinoma: Microscopy

Figure 1-20-9

Adenoid Cystic Carcinoma: Pathologists’ Pitfalls

• •

Adenoid Cystic Carcinoma: Gross •

• • •

Endobronchial mass ➢ Trachea and main bronchi Sessile, polypoid, annular growth Proximal & distal spread Extension into mediastinum

Adenoid Cystic Carcinoma: Radiology [Figure

1-20-9]

Adenoidcystic carcinoma. Coronal (left) and saggital

• • • • •

Central: trachea and main bronchi (right) surface-rendered CT images of the trachea Intraluminal nodule or mass demonstrate both nodular and circumferential deformity Constriction of tracheal/bronchial lumen of the air-column in the lower trachea 10 - 15% in lung periphery CT / MRI: length of involvement; mediastinal involvement

• • • •

Male=Female. Wide age range Cough, fever, hemoptysis, pneumonia, atelectasis Low-grade: excellent prognosis High-grade: better prognosis than Bronchogenic Ca

Mucoepidermoid Carcinoma: Demographics and Prognosis

Uncommon Malignant Tumors

196

Chest Radiology

Mucoepidermoid Carcinoma [Figure 1-20-10]

Figure 1-20-10

Mucoepidermoid Carcinoma: Microscopy •

•

Low grade: ➢ Mucinous cysts ➢ Solid collections of squamous cells High grade: ➢ Solid sheets of tumor ➢ Mitoses and necrosis

Mucoepidermoid Carcinoma: Gross • • •

Submucosal, smooth surfaced Endobronchial, exophytic, polypoid High grade may have ragged invasive appearance

Mucoepidermoid Carcinoma: Radiologic Findings [Figures 1-20-11 and 1-20-12]

Mucoepidermoid carcinoma arising in the right mainstem bronchus at the origin of the right upper lobe bronchus

• •

Solitary nodule or mass Most in main or lobar bronchi Few in trachea Distal effects: ➢ Atelectasis, pneumonia Central lesions Atelectasis, pneumonia

• •

Carcinosarcoma Pulmonary Blastoma

• • • •

Rare - 0.3% of all lung neoplasms Middle aged and elderly males Poor prognosis Aggressive: local invasion, widespread metastases, and rapid death

• • • •

Figure 1-20-11

Mixed Tumors: Neoplasms with malignant epithelial and mesenchymal components Carcinosarcoma

Carcinosarcoma: Microscopy •

•

Mucoepidermoid carcinoma manifesting as a solitary pulmonary nodule on chest radiography

Epithelial component: ➢ Squamous Cell Carcinoma ➢ Adenocarcinoma ➢ Undifferentiated Carcinoma Mesenchymal component: ➢ Usually dominant ➢ Spindle cell (common) ➢ Chondrosarcoma ➢ Osteosarcoma ➢ Rhabdomyosarcoma

Figure 1-20-12

Carcinosarcoma: Gross •

•

Peripheral ➢ Large mass ➢ Average diameter 6 cms. ➢ Frequent necrosis and hemorrhage Central ➢ Endobronchial growth ➢ May extend to adjacent parenchyma ➢ Tumor-distended bronchi may resemble mucus plugs

Central mucoepidermoid carcinoma manifesting as mild prominence of the suprerior aspect of the right hilum with associated atelectasis of the right upper lobe

Chest Radiology

197

Uncommon Malignant Tumors

Carcinosarcoma: Imaging [Figure 1-20-13] •

•

Figure 1-20-13

Peripheral ➢ Large ➢ Well-circumscribed mass Central ➢ Atelectasis, pneumonia ➢ Tumor “mucus plugs” ➢ Upper lobe predominance ➢ Direct extension to pleura, chest wall, and mediastinum

Pulmonary Blastoma • • • • •

Primary lung tumor Mix of epithelial and mesenchymal components Both components blastomatous and immature Morphologic mimic of embryonal lung ? a variant of carcinosarcoma

Pulmonary Blastoma: Demographics and Prognosis • • • •

Predominantly males Biphasic age distribution: first and seventh decades Symptoms: cough, hemoptysis, dyspnea, chest pain Poor survival

•

• •

Mixture: ➢ Epithelial-lined tubules ➢ Primitive stroma Resembles embryonal lung Metastases: mesenchymal, epithelial, or mixed

• • •

Large mass Unencapsulated and soft Abundant central necrosis and hemorrhage

• • • •

Large peripheral mass Well-circumscribed May show pleural invasion May metastasize

• • • • • • • • • • •

Squamous cell ca Adenocarcinoma Small cell ca (rare) Carcinoid Adenoid cystic ca Mucoepidermoid ca Carcinosarcoma Pulmonary blastoma Sarcoma (10%) Endobronchial metastasis Lymphoid malignancies (NHL>HD)

Pulmonary Blastoma: Microscopy

Carcinosarcoma. Contrast enhanced CT demonstrates a peripheral mass with irregular and lobulated borders in the right upper lobe

Figure 1-20-14

Pulmonary Blastoma: Gross

Pulmonary Blastoma: Radiology [Figure 1-20-14]

Pulmonary blastoma manifesting as a large, heterogeneous mass in the left lower lobe

Endobronchial Tumors: Malignant

Uncommon Malignant Tumors

198

Chest Radiology

Benign Tumors of the Lung and Tumor-like Lesions Gerald F. Abbott, MD

Benign Tumors and Tumor-like lesions • • • •

Hamartoma Papilloma / Papillomatosis Inflammatory pseudotumor Granuloma

• • • • •

Albrecht, 1904 Tumor-like malformation Tissues normal to location In excess or disarray (disorganized) “Adult”, “Classic”, “Local” hamartoma

• • • •

Acquired lesion Disorganized growth of tissues normally found in lung Benign neoplastic proliferation Probably derived from bronchial wall mesenchymal cell (“benign mesenchymoma”)

“Hamartoma”

Hamartoma

Hamartoma • • • •

Most common benign tumor of lung 77% of benign lung tumors 8% of SPNs 3% of all lung tumors

• • • • •

Onset in adult life Often adults with previously normal CXR Almost never seen in infants Histology: passive entrapement of epithelium Cytogenetics: Chromosome 12: abnormal q13-q15 regions (as in other benign soft-tissue neoplasms)

Hamartoma: Evidence of Acquired Lesion

Hamartoma: Demographics • • • •

Age range: 30-70 years Peak incidence: 6th decade Female: Male = 3:2 (1:1 for endobronchial hamartoma) Asymptomatic in 90% < 8% obstructive symptoms

Hamartoma: Clinical • • • •

Most are peripheral and asymptomatic If symptomatic: hemoptysis If bronchial obstruction: pneumonitis Fever, cough, expectoration, chest pain

• • • •

Cartilage nests (lobules) in 95% Surrounded by fibrous tissue Mature fat cells Cleft-like invaginations of entrapped respiratory epithelium

Hamartoma: Microscopic

Chest Radiology

199

Benign Tumors

Hamartoma: Gross • • • • • •

Solitary 1 – 3 cm (rarely “Giant”) Rounded, well-circumscribed, lobulated Firm lesions. Usually cartilaginous May see areas of fat Easily “shelled-out”

• • •

Peripheral > Central 80 – 90 % Peripheral No lobar predilection

• • • • • •

Sharply defined, lobulated subpleural Most < 3 cm Calcification on CXR (10-15% ) Rarely see fat on CXR May enlarge on serial CXRs Up to 3 to 5 mm per year

• • •

10 – 15% speckled or “Popcorn” “Popcorn” less frequent than once thought Diagnostic when present ➢ Nodular growths within lesion ➢ Protrude in different directions

Figure 1-21-1

Hamartoma: Distribution

Hamartoma: Radiographic

Hamartoma. Unenhanced chest CT demonstrates a peripheral solitary nodule with focal fat attenuation

Figure 1-21-2

Hamartoma: Calcification

Hamartoma: Computed Tomography

•

Distinguishes fat and cartilage Most are 2.5 cm or less Smooth edge No fat / Focal fat alone / Fat with areas of calcification Cavitation: rare

• • • • •

Thin sections (2mm) Smoothly contoured nodule = or < 2.5 cm diameter Focal fat in 8 voxels or more Or fat with calcification

• • • •

Hamartoma. Unenhanced chest CT demonstrates a central mass in the left lower lobe with a lobular area of “popcorn” calcification

Hamartoma: Computed Tomography [Figures 1-21-1 to 1-21-3]

Figure 1-21-3

Siegelman. Radiology 1986; 160:313-317.

Hamartoma: Computed Tomography • • • • •

no fat or calcification 36% 4% diffuse calcification 38% areas of fat 21% calcium and fat Occasionally: focal calcification, no fat

• • • • • •

Gastric smooth muscle tumors Extra-adrenal paraganglioma Pulmonary chondroma Association unclear Young females < 20 years May have only 2/3 of the triad

“Carney’s Triad”

Hamartoma. Unenhanced chest CT demonstrates speckled calcification in a central endobronchial tumor with associated loss of volume in the left lung

Carney JA. Cancer 1979

Benign Tumors

200

Chest Radiology

Chondroma • • • • • •

Rare Benign cartilaginous tissue Parenchymal or endobronchial Lack epithelial-lined clefts seen in hamartomas In young female Search for Carney’s Triad

• • • •

Morphologically identical to parenchymal Often polypoid. Sessile or thin pedicle Manifest by airway obstruction Micro: more fat, lack clefts, cartilage scant or absent

• • •

Benign Surgical excision = Cure Exceptional cases: additional hamartomas

• • • • •

Branching or coarsely lobulated tumor Arise from and project above an epithelial surface Rare pulmonary tumors Solitary (rare) or Multiple (papillomatosis) Proximal or peripheral

• • • • • •

Rare Usually in adults Papillary exophytic growth Trachea, main or lobar bronchi Males >40 years of age Post-obstructive pneumonia, bronchiectasis

• • •

Children 18 months to 3 years of age Majority remain localized, disappear spontaneously May spread distally and obstruct airways ➢ 5% Spread remains limited to trachea ➢ 1% Develop lung disease - 10 years after laryngeal disease (extension to bronchi, bronchioles, alveolar airspaces)

Hamartoma: Endobronchial

Hamartoma: Treatment and Prognosis Papillomas

Solitary Papillomas

Juvenile Laryngeal Papillomatosis

Laryngeal Papillomatosis: Demographics and Etiology • • • • •

Human papilloma virus - HPV types 6 and 11 0.1% of infants develop LP. Predilection for first-born infants 50% of their mothers have genital tract involvement HPV spread transvaginally at birth Infects oropharyngeal secretions of child

• • •

Non-keratinizing squamous cells Fibrovascular core Form papillomatous projections

• • •

Cauliflower-like excresences Protrude into bronchial lumens May extend into parcenchyma as nodules or cavities

Papillomas: Microscopic Papillomatosis: Gross

Chest Radiology

201

Benign Tumors

Laryngeal Papillomatosis • •

Majority remain localized 5% spread to trachea and distal airways

• • • •

Many remain limited to trachea 1% Develop lung disease Patients with lung disease may develop Squamous cell carcinoma

• • • •

Implantation of inhaled fragments from larynx? Multifocal viral infection? Trauma-induced by tracheostomy? In children, papillomas in bronchi and lung associated with multiple papillomas of trachea or larynx

Tracheobronchial Papillomatosis

Tracheobronchial Papillomatosis: Pathogenesis of lower respiratory tract involvement:

Figure 1-21-4

Papillomatosis: Imaging • • •

Multiple nodules Cavities, 2-3 mm thick walls Air-fluid levels

• • • • • •

Multiple, well-defined nodules Perihilar, posterior thorax Grow to several centimeters Cavitate, 2-3 mm thick walls Air-fluid levels may develop Cavities may represent: ➢ Papillomatosis ➢ Squamous cell carcinoma ➢ Abscess (obstructive pneumonitis)

Papillomatosis: Imaging [Figures 1-21-4 and 1-21-5]

Papillomatosis: Treatment and Prognosis • • • • •

Multiple recurrences Multiple excisions Tracheostomy 37.5% mortality if spread to lungs Worse if malignant degeneration occurs

• • • • •

Plasma cell granuloma Histiocytoma Fibroxanthoma, Xanthoma Myofibroblastic tumor Mast cell granuloma

• • •

Uncommon. Reactive or neoplastic process? May begin as organizing pneumonia May have aggressive features: ➢ Vascular invasion ➢ Vertebral destruction ➢ Recurrence

Papillomatosis. Chest CT demonstrates nodular and cystic opacities that predominantly involve the dorsal aspects of both lungs

Figure 1-21-5

Inflammatory Pseudotumor: Synonyms

Inflammatory Pseudotumor

Papillomatosis and squamous cell carcinoma. Contrast-enhanced Chest CT demonstrates central squamous cell carcinoma in the left lower lobe with distal pneumonitis Benign Tumors

202

Chest Radiology

Inflammatory Pseudotumor • • • • •

WHO 1999: histologic spectrum of fibroblastic and myofibroblastic proliferations With varying infiltrate of inflammatory cells Usually solitary, tumefactive lesion Destroys underlying lung architecture Reactive or Neoplastic ?

Inflammatory Pseudotumor: Demographics

• •

Males = Females Wide age range: 1 to 77 years. Average: 29.5 years 60% <40 years ➢ Children: peak 6-7 years o ➢ Most common 1 lung mass in children 74% asymptomatic Many patients have history of respiratory infection

• • •

Variable. A continuum from plasma cell granuloma to fibrohistiocytic Mixture of collagen, fibroblasts, myofibroblasts, and chronic inflammatory cells

• • • • •

SPN or Mass Well-defined. Firm. Lobulated Lack a capsule Cut-surface: whorled, heterogeneous 1-10cm, 4.4 cm mean

• • • • •

Solitary nodule or mass in 70% Well-defined May manifest as consolidation May mimic neoplasm Endobronchial lesions occur in 10%

• • • • • • •

Solitary, well-defined nodule or mass Endobronchial lesions occur May extend into mediastinum Parenchymal consolidation Calcification, cavitation infrequent May mimic malignant neoplasm Usually no or slow growth. May regress

• • • • • • •

Solitary nodule or mass Sharply circumscribed Lobulated Heterogeneous or homogeneous Enhancement: / calcification variable, nonspecific Calcification: variable Endobronchial lesions occur

• • •

Diagnosed and treated by surgical excision Excellent prognosis after resection Recurrence in 5% ➢ Especially if mediastinal or chest wall involvement DDx: fibrous histiocytoma, sarcomatoid carcinoma

• • •

Inflammatory Pseudotumor: Microscopic Inflammatory Pseudotumor: Gross

Inflammatory Pseudotumor

Figure 1-21-6

Inflammatory Pseudotumor: Radiographic 70% 10% 5% 6%

Inflammatory Pseudotumor: CT [Figure 1-21-6]

Inflammatory pseudotumor. Contrast-enhanced chest CT demonstrates an irregular, heterogeneous mass in the left upper lobe

Inflammatory Pseudotumor: Therapy and Prognosis

•

Chest Radiology

203

Benign Tumors

Granulomas • • •

Infectious Sarcoid (necrotizing granuolomatosis) Hypersensitivity pneumonitis

• • •

Mycobacterial Fungal Parasitic

• • • •

Tuberculoma or Histoplasmoma Satellite lesions common Usually small, smooth Often calcified when healed

• • • • •

TB Histoplasmosis Coccidioidomycosis Cryptococcosis Aspergillosis

• • • • •

<5 mm, micronodular, military Histoplasmosis Blastomycosis Cryptococcosis Coccidioidomycosis

•

Malignant ➢ Primary carcinoma ➢ Metastases ➢ Other 1° malignancy Benign ➢ Non-neoplastic lesion ➢ Tumor ( Hamartoma 8% )

Infectious Granulomas 64% 30% 6%

Granuloma: Infectious

Granuloma – Well-defined Pulmonary Nodule Multiple Ill-defined Pulmonary Nodules

Granulomas – Tiny nodules

Solitary Pulmonary Nodule (n = 955)

•

49% 38% 9% 2% 51% 37% 14%

Toomes H. The coin lesion of the lung. Cancer 1983.

Benign Tumors

204

Chest Radiology

Pleural Disease I Gerald F. Abbott, MD

Pleural Disease I and II: Objectives • • • •

Anatomy and physiology Non-neoplastic and neoplastic pleural disease Chest wall disease Radiologic-Pathologic correlation

•

Normal anatomy ➢ Standard fissures ➢ Accessory fissures Non-neoplastic pleural disease ➢ Effusions ➢ Fibrosis ➢ Pneumothoraces

Figure 1-22-1

Pleural Disease I •

Standard (solid lines) and accessory (dashed lines) fissures

Pleural Anatomy •

•

Parietal Pleura ➢ Covers non-pulmonary surfaces ➢ Systemic supply/drainage ➢ Lymphatics communicate with pleural space ➢ Pain fibers ➢ 5-15 ml of pleural fluid Visceral Pleura [Figure 1-22-1] ➢ Covers lung surface ➢ Dual supply/drainage ➢ Vagus nerve/sympathetic trunks ➢ Lymphatics do not communicate with pleural space

Figure 1-22-2

Pleural Imaging •

•

Radiography / CT ➢ Inconspicuous ➢ Visceral + Parietal = 0.2 mm Thin-collimation ➢ 1-2 mm thick line ➢ Intercostal regions ➢ Normal fluid ➢ Endothoracic fascia ➢ Innermost intercostal m.

Pneumothorax in a supine patient manifesting as a “deep sulcus” and hyperlucency overlying the left hemidiaphragm

Pleural Anatomy [Figure 1-22-2]

Caudal limit of pleura lower than lung Costal and diaphragmatic pleura contact to form costophrenic recess

Pleural Anatomy • •

Junction Lines Apposition of layers of pleura ➢ Anterior ➢ Posterior

Pleural Anatomy - Fissures • • • •

Visceral pleura Variable depth into parenchyma Complete Incomplete

Chest Radiology

205

Pleural Disease I

Incomplete Major Fissure: CT • • • • •

More frequent Right RUL / RLL 70% RML / RLL 47% LUL / LLL 40% Lingula / LLL 46%

• • • •

Major (oblique) fissures Best seen on lateral CXR Origin: T4 Left, T5 Right Right fissure more oblique

•

80-90% of standard CT ➢ Lucent band ➢ Line ➢ Dense band

Major Fissure: Radiography Figure 1-22-3

Major Fissure: CT

Major Fissure: CT Propeller-like morphology [Figure 1-22-3] •

•

Upper thorax ➢ Anterior concave ➢ Lateral-facing Inferior thorax ➢ Anterior convex ➢ Medial-facing

Chest CT (lung window) demonstrates right upper lobe loss-of-volume manifested by displacement of the right major fissure. An endobronchial carcinoid obstructs the origin of the right upper lobe bronchus

Standard Fissures: Radiography •

Minor fissure ➢ “Lights up” in CHF ➢ Interstitial edema (Subpleural interstitium)

Figure 1-22-4

Minor Fissure: CT • • • • •

Lucent area Devoid of vasculature 44% triangular 8% round / ovoid Ground glass attenuation

• • • •

Curvilinear line or band Increased attenuation C-shaped Fusion between RUL / RML (60-90%)

•

10% CXR / 20% CT (50% of anatomic specimens) ➢ Azygos, Superior, Inferior, Left minor

Incomplete Minor Fissure: HRCT

Accessory Fissures: Radiography [Figure 1-22-4] Accessory Fissures: Azygos • • • •

Abnormal migration of posterior cardinal vein Four layers of pleural 1% population 2M : 1F

Pleural Disease I

Diagram illustrates position of inferior (I), superior (S) and azygos (az) accessory fissures

206

Chest Radiology

Accessory Fissures: CT Azygos [Figure 1-22-5]

Accessory Fissures: Inferior Accessory [Figures 1-22-6 and 1-22-7] • • • • • •

Separates medial basal segment from remaining basilar segments Most common 30 - 45% anatomic specimens CXR: 5 - 10% 80% Right-sided CT: 15%

•

Common in RUL or LUL atelectasis ➢ Less common in RML Seen post upper lobectomy Small triangular opacity Projects upward from diaphragm Related to inferior accessory fissure

Figure 1-22-5

Indirect Signs of Atelectasis: Juxtaphrenic Peak • • • •

Accessory Fissures: Superior Accessory • • • •

Separates superior segment from basilar segments 6% anatomic specimens Right > Left Horizontal course ➢ Inferior to minor fissure

Chest CT demonstrates an azygos fissure forming the lateral margin of an azygos lobe

Figure 1-22-6

Accessory Fissures: Left Minor Fissure • • • • •

Separates lingula from remainder of upper lobe 8-18% anatomic specimens 1.5% of chest radiographs Oblique course More cephalad

• • •

Formed by Parietal & Visceral pleura Courses inferiorly & posteriorly Contains bronchial veins, lymphatics, nodes

Pulmonary Ligament

Figure 1-22-7

Coned-down view of right lung demonstrates an inferior accessory fissure separating the medial basal segment from the remaining basal segments of the right lower lobe

Chest CT demonstrates an inferior accessory fissure (arrow). Chest Radiology

207

Pleural Disease I

Pulmonary Ligament: Imaging [Figure 1-22-8] • •

CXR: not visualized CT: 60%-70%

• • • • •

Most common cause Increased hydrostatic pressure Bilateral >80% Unilateral = right-sided Pseudotumor

Figure 1-22-8

Pleural Effusion Cardiac Decompensation [Figure 1-22-9]

Figure 1-22-9

Lateral chest radiograph demonstrates lenticular opacity of fluid accumulation in the minor fissure (pseudotumor)

Chest CT demonstrates inferior accessory fissure (arrowhead) and right and left pulmonary ligaments (arrows)

Figure 1-22-10

Pleural Effusion: Bacterial Pneumonia • • • •

Parapneumonic effusions 40% Exudate 10% require drainage Complications ➢ Loculation ➢ Empyema

Ultrasound demonstrates multiple septations within a loculated fluid collection representing empyema

Pleural Effusion: Empyema [Figure 1-22-10] •

Figure 1-22-11

Three phases ➢ Exudative ➢ Fibrinopurulent ➢ Organizing

Pleural Effusion: Empyema [Figure 1-22-11] • • • •

Lenticular shape Obtuse margins Compress lung Split pleura sign

Pleural Effusion: Lung abscess or Empyema ? Pleural Effusion: Empyema • • • • •

Lenticular shape Obtuse margins Compress lung Split pleura sign Disparity in length of air-fluid level

Pleural Disease I

Contrast enhanced chest CT demonstrates smoothly thickened parietal and visceral pleura enclosing a fluid collection of empyema (“split pleura sign”) 208

Chest Radiology

Pleural Effusion: Lung abscess • • •

Round shape Does not compress lung Equal length of air-fluid level

•

Treatment ➢ Tube thoracostomy ➢ Fibrinolytics ➢ Decortication

Pleural Effusion: Empyema

Pleural Effusion: Empyema necessitatis • • • • v •

Inadequate treatment Drainage into chest wall Tuberculosis 73% Bacterial / Fungal Malignancy Immunocompromised patients

•

Exudate ➢ ↑ lymphocyte count ➢ ↓ glucose level Unilateral Small to moderate

Pleural Effusion: Tuberculosis • •

Pleural Effusion: Subpulmonic • • •

Fluid accumulates between lung base and diaphragm Shifts apex of diaphragm laterally Usually transudate ➢ Cardiac decompensation ➢ Renal failure ➢ Cirrhosis with ascites

Pleural Effusion: Subpulmonic •

Imaging ➢ Apparent elevation diaphragm ➢ Ill-defined costophrenic angle ➢ Diaphragmatic spur ➢ Mobile fluid ➢ Displace gastric bubble ➢“Rock of Gibraltar” on lateral

Pleural Effusion and Ascites •

CT Features ➢ Effusion = outside of hemidiaphragm ➢ Ascites = inside of hemidiaphragm

Pleural Effusion: Connective Tissue Disease • • • • • •

Rheumatoid arthritis Most common thoracic manifestation Middle aged males Antedates clinical disease Exudate / chyliform / low glucose Imaging ➢ Unilateral ➢ Chronic ➢ Transient / relapse ➢ Fibrothorax / decortication

Chest Radiology

209

Pleural Disease I

Pleural Effusion: Asbestos Exposure • • • • • • • •

Diagnosis of exclusion Occupational exposure No malignancy within 3 yrs 10 yrs post-exposure Exudate 1/3 patients have chest pain Recurrent 15 - 30% Small (<500 ml)

•

Associated with diffuse pleural thickening ➢ Involves C-P angle Implicated in formation of Rounded Atelectasis

Pleural Effusion - Asbestos Exposure •

Round Atelectasis: CXR • • • •

•

Peripheral mass Abuts thickened pleura 3.5 to 7 cm Posterior lower lobe most common ➢ Other lobe, diaphragms Bronchovascular bundles converge, forming “comet tail”

Figure 1-22-12

Round Atelectasis: CT • • • • •

Rounded subpleural mass Broadly abuts contiguous pleural thickening Air-bronchogram hilar aspect Bronchovascular “comet tail” Loss of volume in same lobe

Round Atelectasis: Required CT Findings [Figure 1-22-12]

• • • •

Subpleural mass Thickened pleura Loss of volume Comet tail

• • • • • • • •

Asbestos exposure Pleural effusion Atelectasis Pleural adherence Effusion subsides Lung re-expands Pleural fibrosis Contraction

• • • • •

2nd most common pleural abnormality Result of many primary diseases of the pleura Complication of inflammatory disease Most localized to single area Less often diffuse ➢ May have functional abnormalities

Chest CT (mediastinal and lung windows) demonstrates the CT findings of round atelectasis

Round Atelectasis: Pathogenesis

Pleural Fibrosis

Pleural Fibrosis: Focal • • •

Healed Pleuritis Bacterial pleuritis/trauma Imaging ➢ Blunt posteriolateral CP sulci ➢ Rule-out small effusion

Pleural Disease I

210

Chest Radiology

Pleural Fibrosis: Focal [Figure 1-22-13] • • • • • •

Pleural Plaques Serpentine (chrysotile) asbestos Dense hyalinized collagen Parietal pleural surface Asbestos exposure Asymptomatic

• • •

Pleural Plaques 50% of exposed individuals Visible plaques ➢ 15 years non-calcified ➢ 20 years calcified

Figure 1-22-13

Pleural Fibrosis: Focal

Pleural Fibrosis: Imaging • • • • • •

Bilateral (80%) Lateral chest wall 4th to 8th ribs Tendinous diaphragm Spares apices and CPAs En face “Holly leaf”

•

Fibrous obliteration of normal pleural space ➢ Tuberculosis/bacterial empyema ➢ Hemothorax ➢ Asbestos-related pleural effusions ➢ Rheumatoid effusions Volume loss/restrictive disease

Chest CT (lung window) demonstrates multiple bilateral pleural plaques

Figure 1-22-14

Pleural Fibrosis: Diffuse Fibrothorax Figure 1-22-14]

•

Pleural Fibrosis: Diffuse Fibrothorax •

➢ ➢ ➢ ➢ ➢

Radiographic definition Smooth/uninterrupted 25% or more of chest wall May obliterate c-p suclus ≤ 2.0 cm thickness +/- calcification

Pleural Fibrosis: Diffuse Fibrothorax •

Pleural fibrosis. Chest radiograph demonstrates pleural thickening and calcification in the right hemithorax

Imaging CT ➢ Extends > 8.0 cm cranio-caudal ➢ Pleura > 3 mm thick ➢ Extrapleural fat hypertrophy ➢ +/- Pleural calcification ➢ Mediastinal pleura spared

Figure 1-22-15

Pneumothorax

•

Air within the pleural space Spontaneous ➢ Primary ➢ Secondary Traumatic

• • • • • •

M:F=5:1 3rd - 4th decade Right-side predominance 30% ipsilateral recurrence 10% contralateral recurrence Rupture of apical bleb/bulla

• •

Pneumothorax: Primary Spontaneous [Figure 1-22-15]

Chest Radiology

Chest CT demonstrates left pneumothorax and a bleb along the visceral pleural surface of the collapsed lung 211

Pleural Disease I

Pneumothorax: Secondary Spontaneous • • • • • •

COPD Most common concurrent condition 0.5% per year 45-65 years of age Peripheral emphysematous lung Mortality rate ~3%

• • •

Pneumocystis Jiroveci Pneumonia (PCP) Destruction of alveolar septa → bulla Subpleural necrosis → cystic degeneration / bulla

• • • • •

Pneumocystis Jiroveci Pneumonia (PCP) Complicates in 12% Refractory “air-leaks” Poor prognosis Death in 8 weeks (<57%)

• • • •

•

Lymphangioleiomyomatosis (LAM) Women child-bearing age Proliferation of immature smooth muscle Bronchiolar obstruction ➢ Cysts → PTX Recurrence ~40%

• • • •

Langerhans Cell Histiocytosis Smokers Cysts rupture Recurrent ptx (25%)

Pneumothorax: Secondary Spontaneous Pneumothorax: Secondary Spontaneous

Pneumothorax: Secondary Spontaneous

Pneumothorax: Secondary Spontaneous

Pleural Disease I

212

Chest Radiology

Pleural Disease II and Chest Wall Gerald F. Abbott, MD

Malignant Pleural Effusion • • • • • • •

Most common manifestation of metastatic involvement Exudative effusion Lung Ca 36% Breast Ca 25% Lymphoma 10% Ovarian 5% Gastric Ca 2%

•

Malignant Effusion = T4 N0 N1 N2 IA IIA IIIA IB IIB IIIA IIB IIIA IIIA IIIB IIIB IIIB

TNM Staging of Lung Cancer T1 T2 T3 T4

MI=IV

N3 IIIB IIIB IIIB IIIB

Malignant Pleural Effusion: Diagnosis and Prognosis • • • • •

Combined pleural cytology and pleural biopsy Multiple thoracenteses / pleural biopsies Poor prognosis Lung Carcinoma - mean survival 2 to 3 months Breast Carcinoma - mean survival 7 to 15 months

• • • •

Discrepant margins on orthogonal views Elliptical shapes Obtuse angles Cross boundaries

• • • •

Localized fibrous tumor Malignant mesothelioma Secondary Pleural metastases ➢ Bronchogenic carcinoma ➢ Other carcinomas ➢ Lymphoma ➢ Invasive thymoma

Pleural / Chest Wall Mass

Pleural Neoplasms • Primary

Localized Fibrous Tumor • • • • • • • •

Rare (< 5% of pleural neoplasms) Not related to asbestos M=F Mean age: 50 years Symptoms in 54% Cough, chest pain, dyspnea HPO 0 - 35% Hypoglycemia 4%

Chest Radiology

213

Pleural Disease II

Localized Fibrous Tumor: Microscopic

Figure 1-23-1

• • • •

Variable patterns Disorderly arrangement Spindle cells and collagen High mitotic activity suggests malignancy 20%

• • • • •

2 - 40 cm 80% visceral / 20% parietal Lobular, encapsulated Pedicle: good prognosis Cut-surface: whorled, nodular, fibrous hemorrhage, necrosis, cysts

Localized Fibrous Tumor: Gross

Localized Fibrous Tumor: CXR [Figures 1-23-1 to 1-23-3] • • • • •

Incidental finding Solitary rounded, lobular mass Mid to inferior thorax Obtuse or acute angles at interfaces Pedunculated tumors ➢ Positional mobility ➢ Pathognomonic

Localized fibrous tumor. PA chest radiograph demonstrates a pleural mass a peripheral mass in the right lower hemithorax with incomplete borders

Figure 1-23-2

Figure 1-23-3

Localized fibrous tumor. Chest CT (lung windows) demonstrates a pleural mass that forms obtuse angles at its interface with the chest wall

Localized fibrous tumor. PA chest radiograph demonstrates a large mass that opacifies most of the right hemithorax and produces mass effect with contralateral shift of the mediastinum

Localized Fibrous Tumor: CT [Figure 1-23-4] • • • • •

Figure 1-23-4

Well-defined, smooth, lobular Abutting pleural surface Elongated, lenticular Heterogeneity: ➢ hemorrhage, necrosis, cysts Contrast enhancement

Localized fibrous tumor. Contrast enhanced chest CT demonstrates a large heterogeneous mass in the right lower hemithorax with lobulated contours Pleural Disease II

214

Chest Radiology

Localized Fibrous Tumor Therapy and Prognosis • • • • •

Treatment of choice: complete excision 90% cure rate Symptoms usually resolve post-op Recur with tumor recurrence Recurrence in 10% of patients

• • • • • • • •

Most common primary pleural neoplasm 2,000 to 3,000 cases / year in USA 10% of exposed individuals Shipyards / asbestos plants Sixth to eighth decades Male : Female 3-6 : 1 Amphiboles most tumorigenic Latency: 30-40 years

• • • •

Insidious onset of symptoms 6-8 months prior to Dx Dyspnea, chest pain, cough, weight loss Rarely: SVC Syndrome, Horner Syndrome, dysphagia, vocal cord paralysis, HPO, clubbing, hypoglycemia

Malignant Mesothelioma

Malignant Mesothelioma: Clinical

Figure 1-23-5

Malignant Mesothelioma - Microscopic • • • •

Epithelioid Sarcomatous Biphasic Interobserver agreement

50 % 15 % 25 % 50%

• • • • • • •

Sheets, plaques, masses Parietal > Visceral Bulk in inferior hemithorax Lung encasement Fissural growth Parenchymal involvement Mediastinal, chest wall, diaphragmatic invasion

Malignant Mesothelioma: Gross

Malignant mesothelioma. PA chest radiograph demonstrates circumferential, nodular and contiguous pleural masses throughout the left hemithorax

Malignant Mesothelioma: Radiographic

Figure 1-23-6

[Figure 1-23-5]

• • • • •

Pleural effusion Pleural masses Circumferential Mediastinal shift Pleural plaques 25%

•

Malignant mesothelioma cannot be reliably differentiated from pleural metastases

Malignant Mesothelioma: Radiographic Malignant Mesothelioma: CT [Figure 1-23-6] • • • • • •

Pleural thickening Fissural thickening Pleural effusion Ipsilateral ↓ volume Pleural calcification Ipsilateral ↑ volume

Kawashima AJR 1990

Chest Radiology

92% 86% 74% 42% 20% 14%

Chest CT demonstrates circumferential, nodular pleural thickening in the left hemithorax that extends into the major interlobar fissure. Calcified pleural plaques are demonstrated bilaterally 215

Pleural Disease II

Malignant Mesothelioma: DX • •

Video-Assisted-Thoracoscopic-Surgery: Sensitivity 98% ➢ Complication: tumor seeding along entry ports Open biopsy: when adhesions preclude VATS Cytology and FNA Bx of limited value

• • • •

Staging Comparable / superior to CT Tumor enhancement Increased signal intensity

• •

Median survival: 10 months Best prognosis: ➢ 25-30% 5-year survival ➢ Negative margins ➢ Epithelial cell type ➢ No metastases Extrapleural pneumonectomy ➢ High mortality / morbidity

•

Malignant Mesothelioma: MR

Malignant Mesothelioma: Treatment and Prognosis

•

Pleural Metastases Most common pleural neoplasm • Common •

•

➢ Adenocarcinoma ✧ Lung, breast, ovary, stomach Less common: ➢ Lymphoma, Thymoma Imaging ➢ Pleural effusion ➢ Pleural masses ➢ Or both

Figure 1-23-7

Pleural Metastases • •

•

•

Hematogenous / Lymphatic Direct extension ➢ Lung ca, breast ca Drop metastases ➢ Invasive thymoma May be bilateral

Pleural Thickening

Malignant Pleural Thickening [Figure 1-23-7]

• • • •

Circumferential Nodular > 1 cm Mediastinal pleura

Pleural metastases. Chest CT demonstrates adenocarcinoma arising from a right upper lobe bronchus and pleural metastases that are nodular, circumferential, and involve the mediastinal pleura

Leung et al AJR 1990

Chest Wall • • • •

Congenital and developmental anomalies Inflammatory and infectious diseases Non-neoplastic conditions Neoplasia: benign and malignant

Pleural Disease II

216

Chest Radiology

Chest Wall: Congenital / Developmental Anomalies [Figures 1-23-8 and 1-23-9]

• • • •

Pectus deformities Anomalous ribs Cleidocranial dysostosis Poland syndrome

• • • •

Hematogenous Direct extension Pyogenic infection: S. aureus, P. aeruginosa Imaging ➢ Osseous destruction if advanced ➢ CT / MR for better delineation ➢ CT for biopsy and/or drainage

Figure 1-23-8

Chest Wall: Inflammatory / Infectious Diseases

Chest Wall:Tuberculosis [Figure 1-23-10] • • • • •

Uncommon Hematogenous spread Contiguous spread Abscess / sinus tract 25% Imaging ➢ Bone / cartilage destruction ➢ Soft-tissue mass ➢ Calcification ➢ Peripheral enhancement

Lateral chest radiograph demonstrates pectus carinatum

Figure 1-23-9

Chest Wall: Inflammatory / Infectious Diseases

Chest Wall: Actinomycosis [Figure 1-23-11] • • • • • •

Actinomyces israelii Anaerobic gram-positive Lung → Pleura → Chest Wall Proteolysis → Fistulas Diagnosis: anaerobic cultures Imaging: soft-tissue mass draining sinus, periostitis

Figure 1-23-11

Figure 1-23-10

Tuberculosis. Contrast enhanced chest CT demonstrates an anterior chest wall abscess with subtle peripheral enhancement and right pleural effusion and pleural thickening

Actinomycosis. Contrast enhanced chest CT demonstrates peripheral consolidation in the left upper lobe with contiguous soft-tissue density extending into the adjacent mediastinum and anterior chest wall Chest Radiology

Poland syndrome. Chest CT demonstrates congenital absence of the right pectoralis muscles

217

Pleural Disease II

Chest Wall: Neoplasms • •

•

Figure 1-23-12

Adults Benign: ➢ Lipoma ➢ Other mesenchymal neoplasms Malignant: ➢ Fibrosarcoma ➢ Malignant fibrous histiocytoma ➢ Other mesenchymal neoplasms ➢ Lymphoma

Chest Wall Neoplasms: Lipoma • • • • •

Common Subcutaneous Intrathoracic Both Diagnostic CT number

• • • • •

Aggressive fibromatosis Most: second to fourth decades Shoulder, chest wall Soft tissue mass Frequent recurrence

• •

Adults Malignant: ➢ Fibrosarcoma ➢ Malignant fibrous histiocytoma

Fibrosarcoma. Chest CT demonstrates postsurgical changes of right mastectomy and a softtissue mass in the left chest wall. The patient received radiation therapy through ports that included the left posterolateral chest wall.

Chest Wall Neoplasms: Desmoid (Fibromatosis)

Chest Wall Neoplasms: Soft Tissue Involvement [Figure 1-23-12]

Chest Wall Neoplasms: Osseous Involvement [Figure 1-23-13] • •

•

Rib expansion ➢ Fibrous Dysplasia ➢ Enchondroma Pressure erosion ➢ Neurogenic (slow growth) Rib destruction: ➢ Metastatic or Primary ➢ Inflammatory

Chest Wall Neoplasms: Osseous Destruction • Adult •

➢ Metastatic disease (Lung, Breast, Prostate, etc.) ➢ Multiple myeloma ➢ Chondrosarcoma Child ➢ Ewing sarcoma ➢ Neuroblastoma ➢ Lymphoma ➢ Askin tumor (PPNET)

Chest Wall Neoplasms: Myeloma • • • • •

Figure 1-23-13

Males > Females 5th - 7th decades Axial skeleton Multiple or solitary Imaging: ➢ Osseous destruction ➢ Soft-tissue mass

Pleural Disease II

Fibrous dysplasia. Chest CT demonstrates an expansile lesion with intact cortical margins nvolving a right rib

218

Chest Radiology

Chest Wall Neoplasms: Chondrosarcoma • • • •

Adults Painful, palpable mass Costochondral junction, rib, sternum Imaging: ➢ Expansile, destructive ➢ Chondroid calcification ➢ Soft-tissue mass

PNET “Askin Tumor”: Primitive Neuroectodermal Tumor • • • • • • •

Malignant small round cell tumor Children, adolescents Female: Male = 3:1 Unilateral Rib destruction 2/3 Pleural effusion Poor prognosis

Chest Radiology

219

Pleural Disease II

Classic Breast Lesions Leonard M. Glassman, MD

Figure 1-24-1

Most Lesions are Non-specific [Figure 1-24-1] • • •

Differentials can be given High likelihood diagnoses can be made Is this a cyst or a solid mass?

• •

Pectoralis major Lymph nodes

Normal Variants

Pectoralis Major [Figure 1-24-2] Figure 1-24-2

CC view showing a rounded density medially, the medial end of the pectoralis major muscle

Sternalis [Figure 1-24-3]

Non specific lobulated mass which could be a cyst, benign solid mass or a carcinoma

Sharply marginated medial density, smaller than the pectoralis major, is the sternalis muscle

Figure 1-24-3

Intramammary Lymph Nodes

• • • • • • •

Normal finding 28% of breasts May enlarge and shrink in size Circumscribed with hilar notch or fatty hilum Usually less than 15 mm in size Not related to the usual lymphatic drainage patterns Usually upper outer quadrant

Figure 1-24-4

Intramammary Lymph Nodes Figures 1-24-4 to 1-24-6]

Classic Breast Lesions

220

Left: Medial lymph node Right: Typical lymph node with fatty hilum

Chest Radiology

Figure 1-24-5

Figure 1-24-6

Left: Lymph node with hilar notch Right: Core biopsy specimen of normal lymph node

Six examples of benign lymph nodes. The top three are with traditional real time scanning and the bottom three with compound scanning

Congenital Anomalies •

•

Polythelia ➢ Accessory nipples ➢ 2.4% of neonates Polymastia ➢ 2-3% of women ➢ Axillary breast tissue most common ➢ Inframammary fold and labia next most common

Figure 1-24-7

Figure 1-24-8

Polythelia [Figures 1-24-7 and 1-24-8]

Polymastia [Figures 1-24-9 and 1-24-10] •

Can be palpable or visible

• • • •

Fatty lesions Gynecomastia Fibrocystic changes Foreign bodies

• • • •

Hamartoma Lipoma Fat necrosis Galactocele

• •

Very rare hamartomas 1 Phyllodes with liposarcoma

Benign Abnormalities

Fatty Lesions

Accessory nipple seen medially

Accessory nipple in a patient at the inframammary crease

All Lesions that Contain Fat are Benign Except

Figure 1-24-9

Figure 1-24-10

Palpable axillary accessory breast tissue Chest Radiology

Visible bilateral axillary accessory breast tissue 221

Classic Breast Lesions

Hamartoma

[Figures 1-24-11 to 1-24-14]

Fibroadenolipoma Palpable mass or mammographic finding ➢ Can be large and not palpable • Encapsulated normal breast elements

• •

Figure 1-24-11

Figure 1-24-12

Figure 1-24-13

Large hamartoma containing fat, glandular tissue and fibrous tissue

Small hamartoma

Hamartoma showing mixed echogenicity

Lipoma • • •

Hamartoma presenting as an intermediate density mass without visible fat

Figure 1-24-15

[Figure 1-24-15]

Benign tumor Usually not palpable because it is soft Liposarcoma usually water density

Liposarcoma

Figure 1-24-14

Figure 1-24-16

[Figure 1-24-16]

Lipoma in the axillary region

Left: Liposarcoma is water density, not fatty Right: Water density liposarcoma

Fat Necrosis • • • • •

Figure 1-24-17

Figure 1-24-18

Figure 1-24-19

50% have history of trauma ➢ Including surgery & XRT Oil cyst Partially calcified lesion Can be spiculated Can progress from fatty to spiculated

Oil Cyst

[Figures 1-24-17 to 1-24-19]

Classic Breast Lesions

Oil cyst 222

Oil cyst with scarring and dystrophic calcification

Heavily calcified oil cyst Chest Radiology

Fat Necrosis - Progression [Figure 1-24-20]

Figure 1-24-21

Figure 1-24-20

Left: Oil cyst with scarring Right: Several years later the scarring predominates and the fatty part of the lesion disappears

Galactocele [Figure 1-24-21] • • • •

Pregnant or breast feeding Cystic lesion Fat fluid level on horizontal beam film Aspiration usually curative

•

Exaggerated physiologic phenomenon ➢ Cysts ➢ Apocrine metaplasia and hyperplasia ➢ Stromal alterations ➢ Mild epithelial hyperplasia ➢ Mild adenosis

Two galactoceles with layering of milk (fat rising and calcium dropping)

Figure 1-24-22

Fibrocystic Changes [Figures 1-24-22 and 1-24-23]

Figure 1-24-23

Apocrine Metaplasia [Figure 1-24-24] Cyst [Figure 1-24-25] • • • • •

Cystic lobular involution Anechoic with sharp back wall Enhanced thru-transmission of sound 10% atypical Diagnosable on ultrasound or aspiration

Figure 1-24-24

Specimen showing multiple small cysts with a characteristic blue color (blue domed cysts)

Figure 1-24-25

Multiple small hypoechoic cysts and textural irregularity of fibrocystic change

Figure 1-24-26

Microcystic nodule of apocrine metaplasia

Pneumocystography [Figure 1-24-26]

Chest Radiology

Anechoic mass with sharp borders and enhanced transmission of sound, a classic cyst

223

Pneumocystogram of a benign cyst Classic Breast Lesions

Foreign Body •

• •

Figure 1-24-27

Silicone or paraffin ➢ Free injection ➢ Leakage from implants Surgical drain Wire fragments

Figure 1-24-28

Free Silicone (Implant Rupture) [Figure 1-24-27]

Free Silicone or Paraffin [Figure 1-24-28] Free Silicone [Figure 1-24-29]

Silicone in the tissue from implant rupture

Figure 1-24-29

Figure 1-24-30

Left: Multiple high density globules of injected silicone for augmentation Right: Low power view of “holes” in the tissue from silicone

Surgical Drain [Figure 1-24-30]

Penrose drain remaining after biopsy

Wire Fragment [Figure 1-24-31] Thickened Skin Pattern • • • • • •

Diffuse fibrosis and calcification from injected silicone or paraffin for augmentation

Figure 1-24-31

Wire fragment left behind after breast biopsy

Figure 1-24-32

Edema Mastitis Inflammatory carcinoma Post-radiation change Obstruction to lymphatic drainage in the axilla or superior mediastinum Lymphoma

Thickened Skin Pattern – Mastitis [Figure 1-24-32]

Prominent thickened skin pattern as compared to the normal side

Classic Breast Lesions

224

Chest Radiology

Thickened Skin Pattern – Radiation Therapy [Figure 1-24-33]

Figure 1-24-33

Inflammatory Carcinoma [Figure 1-24-34] •

• • •

•

Clinical findings ➢ Heavy firm breast ➢ Red skin ➢ Warm skin ➢ Peau d’orange Can not differentiate from acute mastitis Far advanced local disease ➢ Usually poorly differentiated ductal carcinoma Radiographic findings ➢ Obstruction of dermal lymphatics ✧ Can diagnose with a skin biopsy ➢ Diffuse lymphatic invasion within the breast ➢ Increased density ➢ Trabecular thickening 50% five year survival ➢ Pre-op chemo, mastectomy and radiation

Mammographic Findings

• • • • •

Skin thickening Diffuse increased density Trabecular thickening Adenopathy Signs of carcinoma ➢ Mass, calcification, asymmetry, distortion

Extensive edema and increased density after radiation therapy

Figure 1-24-34

Inflammatory Carcinoma [Figure 1-24-35] • •

Axillary nodes Supraclavicular node

Figure 1-24-35

Far advanced inflammatory breast cancer with skin necrosis

Left: Enlarged abnormal axillary nodes in inflammatory breast cancer Right: Abnormal supraclavicular node

Figure 1-24-36

Inflammatory Carcinoma [Figure 1-24-36] Classically Benign Calcifications • • • • • • • •

Lobular Sutural Fibroadenoma Skin Vascular Secretory Lucent centered Egg shell

Chest Radiology

Left: Extremely dense breast in inflammatory breast cancer. Right: Inflammatory breast cancer with enlargement, and extensive skin thickening

225

Classic Breast Lesions

Lobular Calcifications [Figure 1-24-37] • • •

Tightly clustered Round Fit together like a jigsaw puzzle

• •

Look like sutures Usually post radiation therapy

• •

Coarse or “popcorn-like” Calcification generally peripheral

Figure 1-24-37

Sutural Calcifications [Figure 1-24-38] Figure 1-24-38

Calcified Fibroadenoma

Tight cluster of smooth calcifications, a benign lobular cluster

Peripheral Calcification [Figure 1-24-39]

Figure 1-24-39

Dystrophic calcification with calcified suture

Figure 1-24-40

Surface calcification in a fibroadenoma

Calcified Fibroadenoma [Figures 1-24-40 and 1-24-41]

Skin Calcifications • •

Faint peripheral clusters with lucent centers Tangent view

Left: Dense calcification (popcorn like) in a fibroadenoma Right: Sclerotic fibroadenoma with calcification

Dermal Localization [Figure 1-24-42]

Figure 1-24-41

Figure 1-24-42

Left: Marker over cluster for dermal localization Right: Tangential film showing cluster in skin

Classic Breast Lesions

Extremely dense calcification in a fibroadenoma 226

Chest Radiology

Vascular Calcifications • • • •

Figure 1-24-43

Parallel tracks associated with blood vessels Calcifications are on the outside of the tube Diabetes ? Heart Disease ?

Vascular / Ductal [Figure 1-24-43] Secretory Calcifications •

Large rods ➢ Luminal calcifications ➢ Oriented toward nipple ➢ Relatively smooth surface ➢ May branch

Loa Loa [Figure 1-24-44 • • • •

Also called eye worm Found in rain forest and swamps of West Africa, especially Cameroon Transmitted by day biting Chrysops flies Loa loa filarial nematode Larvae develop over 1 year Mature worms up to 3-6 cm x 0.5 cm

• • • •

Primary in the breast 27 to 89 years old Median 64.5 years Highly aggressive tumors

• •

Left: Calcification in artery

Figure 1-24-44

Right: Calcification in duct

Figure 1-24-45

Osteosarcoma

Primary Osteosarcoma [Figure 1-24-45]

Calcified loa loa worm

National Flower of the Radiologist is the Hedge Some Diagnoses Can be Made •

Mass with dense osteoid matrix

Make them when you can

References 1. 2.

Adler DD, Jeffries DO, Helvie MA. Sonographic features of breast hamartomas. J Ultrasound Med 1990; 9:85-90. Ahern V, Brennan M, Ung O, Kefford R. Locally advanced and inflammatory breast cancer. Aust Fam Physician 2005; 34:1027-1032. 3. Bassett LW, Gold RH, Cove HC. Mammographic spectrum of traumatic fat necrosis: the fallibility of "pathognomonic" signs of carcinoma. AJR Am J Roentgenol 1978; 130:119-122. 4. Berg WA, Campassi CI, Ioffe OB. Cystic lesions of the breast: sonographic-pathologic correlation. Radiology 2003; 227:183-191. 5. Bosch X. Unique features of inflammatory breast carcinoma. Lancet Oncol 2005; 6:549. 6. Dershaw DD, Moore MP, Liberman L, Deutch BM. Inflammatory breast carcinoma: mammographic findings. Radiology 1994; 190:831-834. 7. Ellis DL, Teitelbaum SL. Inflammatory carcinoma of the breast. A pathologic definition. Cancer 1974; 33:10451047. 8. Erguvan-Dogan B, Yang WT. Direct injection of paraffin into the breast: mammographic, sonographic, and MRI features of early complications. AJR Am J Roentgenol 2006; 186:888-894. 9. Ganott MA, Harris KM, Ilkhanipour ZS, Costa-Greco MA. Augmentation mammoplasty: normal and abnormal findings with mammography and US. Radiographics 1992; 12:281-295. 10. Hance KW, Anderson WF, Devesa SS, Young HA, Levine PH. Trends in inflammatory breast carcinoma incidence and survival: the surveillance, epidemiology, and end results program at the National Cancer Institute. J Natl Cancer Inst 2005; 97:966-975. 11. Helvie MA, Adler DD, Rebner M, Oberman HA. Breast hamartomas: variable mammographic appearance. Radiology 1989; 170:417-421. Chest Radiology

227

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12. Hogge JP, Robinson RE, Magnant CM, Zuurbier RA. The mammographic spectrum of fat necrosis of the breast. Radiographics 1995; 15:1347-1356. 13. Kushwaha AC, O'Toole M, Sneige N, Stelling CB, Dryden MJ. Mammographic-pathologic correlation of apocrine metaplasia diagnosed using vacuum-assisted stereotactic core-needle biopsy: our 4-year experience. AJR Am J Roentgenol 2003; 180:795-798. 14. Levitan LH, Witten DM, Harrison EG, Jr. Calcification In Breast Disease Mammographic-Pathologic Correlation. Am J Roentgenol Radium Ther Nucl Med 1964; 92:29-39. 15. Mester J, Simmons RM, Vazquez MF, Rosenblatt R. In situ and infiltrating ductal carcinoma arising in a breast hamartoma. AJR Am J Roentgenol 2000; 175:64-66. 16. Sheppard DG, Whitman GJ, Huynh PT, Sahin AA, Fornage BD, Stelling CB. Tubular carcinoma of the breast: mammographic and sonographic features. AJR Am J Roentgenol 2000; 174:253-257. 17. Soo MS, Kornguth PJ, Hertzberg BS. Fat necrosis in the breast: sonographic features. Radiology 1998; 206:261269. 18. Stacey-Clear A, McCarthy KA, Hall DA, et al. Calcified suture material in the breast after radiation therapy. Radiology 1992; 183:207-208. 19. Svane G, Franzen S. Radiologic appearance of nonpalpable intramammary lymph nodes. Acta Radiol 1993; 34:577-580.

Classic Breast Lesions

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Basic Breast Imaging Leonard M. Glassman, MD There are Two Diseases

Figure 1-25-1

•

Cancer and no cancer

•

Mass and calcification

• •

Anatomy is simple Physiology is almost irrelevant

• •

90% of cancers present as calcification, mass or both 10% present as ➢ Focal asymmetry, developing or neodensities ➢ Dilated duct ➢ Thickened skin pattern ➢ Architectural distortion ➢ Paget’s disease

Cancer has Two Predominant Signs You Have the Opposite Side for Comparison What You Need to Remember

(left) Ductogram showing normal ducts; (right) : Diffuse tubular parenchymal pattern caused by periductal fibrosis not ductal dilatation

Rad Path Correlation: What You Need to Remember

• •

The mass edge interface with the surrounding tissue reflects the aggressiveness of the underlying lesion Benign masses tend to be less aggressive than malignant masses The shape of the calcification represents a cast of an underling anatomic or pathologic space Benign processes often cause smooth spaces Necrosis (benign or malignant) causes irregular spaces

• • • •

Skin Nipple and areola Subcutaneous fat Premammary fascia

•

• • • •

Glandular cone ➢ Breast disease occurs here Retromammary fascia Retromammary fat Muscle Ribs

•

15 - 20 lobes or segments

• • •

Normal Anatomy

Figure 1-25-2

Normal Anatomy

(left) Normal ductal distribution from nipple to lobules; (right) : Normal duct with single cell epithelial layer and clear lumen

Segmental Anatomy

Normal Ducts [Figures 1-25-1 and 1-25-2]

Terminal Duct Lobular Unit (TDLU) [Figure 1-25-3] • • • •

Short segment of terminal duct and a cluster of ductules (acini) Functional unit of the breast Ductal and lobular cancers begin here Explains mixed ductal & lobular features in the same neoplastic lesion

Chest Radiology

229

Figure 1-25-3

(left) Drawing of TDLU (right) Microscopic view of TDLU Basic Breast Imaging

Embryology •

Milk ridges ➢ Ventral ectodermal thickenings from the axillary to the inguinal region ➢ Usually limited to the pectoral regions by the ninth week of embryonic life

Congenital Anomalies •

•

Athelia ➢ Rarest anomaly of the breast ➢ Absence of the nipple Amastia ➢ Agenesis of breast & nipple ➢ Associated with hypoplasia of the ipsilateral pectoral muscles in 90% ➢ Can be iatrogenic

Congenital Anomalies • •

Polythelia Polymastia

•

Increased estrogen & progesterone ➢ Estrogen promotes ductal growth ➢ Progesterone promotes lobular growth and breast secretion Hyperplasia and hypertrophy Extremely dense breast pattern Can still see calcifications on mammography Can see masses on sonography

Figure 1-25-4

Pregnancy Changes [Figure 1-25-4] • • • •

Mastitis • •

3% of primary diagnoses at biopsy Many different types ➢ Infection ➢ Systemic ➢ Antigen-antibody reaction ➢ Idiopathic

Very dense parenchyma in a normal pregnant patient

Figure 1-25-5

Mastitis [Figures 1-25-5 and 1-25-6] •

•

Acute mastitis ➢ Usually in lactating women with a cracked nipple ➢ Can go on to abscess Chronic mastitis

Chronic Mastitis •

•

•

Chronic infection ➢ TB ➢ Fungus Immunologic ➢ Diabetes ➢ Amyloid ➢ Wegener granulomatosis ➢ Sarcoid ➢ Churg – Strauss Idiopathic ➢ Necrobiotic xanthogranulomatosis ➢ Granulomatous mastitis

Focal parenchymal density from acute bacterial mastitis

Figure 1-25-6

Most Common Benign Neoplasms •

• •

Fibroadenoma ➢ Biphasic tumor Intraductal papilloma Hamartoma

Basic Breast Imaging

(left) Early abscess with accumulation of pus in small spaces. (right) Later stage with larger area of pus

230

Chest Radiology

Biphasic Tumors •

Figure 1-25-7

Epithelial & stromal elements ➢ Fibroadenoma ❖ Benign epithelial and stromal elements ➢ Phyllodes tumor ❖ Benign epithelial & hyperplastic or sarcomatous stroma ➢ Carcinosarcoma ❖ Both elements malignant

Fibroadenoma [Figure 1-25-7] • • • • • • •

(left) Circumscribed mass (fibroadenoma) showing Begins in TDLU sharp border since there is no tissue invasion. Response to unopposed estrogen in young women (right) Fibroadenoma showing surface devoid of Oval or round circumscribed nodule adherent surrounding tissue May have coarse calcification, especially in periphery Growth pushes surrounding tissue without invasion Figure 1-25-9 7-16% of patients have multiple tumors Polyclonal cell population ➢ Begins as local fibroadenomatiod change which coalesces into a fibroadenoma

Figure 1-25-8

Fibroadenoma [Figures 1-25-8 to 1-25-10] • •

Fibroadenoma Carcinoma

• •

Rare Most often lobular neoplasia (LCIS) or DCIS Invasive carcinoma very rare ➢ Usually grows in from outside

Carcinoma Arising in a Fibroadenoma •

Fibroadenoma Phyllodes

(left) Fibroadenoma showing pushing Fibroadenoma showing but not invasion of surrounding tissue. sharp borders and (right) Invasive carcinoma without the sharp border because of invasion, not enhanced thru transmission of sound pushing.

Phyllodes Tumor [Figures 1-25-11 and 1-25-12] • •

• •

Benign epithelial elements and cellular spindle cell stroma Can act malignant ➢ Local recurrence ➢ Distant blood born metastases ➢ Lymph node enlargement reactive usually Well circumscribed lobulated mass Similar appearance on sonography ➢ May have cystic spaces

Figure 1-25-10

Figure 1-25-11

(left) Densely calcified fibroadenomas. (right) Irregular calcification and ill defined density from degenerated fibroadenoma (left) Phyllodes tumor as a macrolobulated circumscribed mass similar to a fibroadenoma. (right) Phyllodes tumor similar to a fibroadenoma except note the small cystic clefts. Chest Radiology

231

Basic Breast Imaging

Figure 1-25-12

Figure 1-25-13

(left) Mass with nonvascular cystic clefts in this Phyllodes tumor. (right) Phyllodes tumor showing clefts on low power microscopy.

Ill defined subareolar and UOQ mass in this carcinosarcoma

Figure 1-25-14

Carcinosarcoma [Figure 1-25-13]

Papilloma [Figures 1-25-14 to 1-25-16] •

•

Papillary growth pattern supported by a fibrovascular stalk ➢ Arises centrally ➢ Usually solitary Papillomatosis ➢ Arises peripherally in the TDLU ➢ Usually multiple

Figure 1-25-15

(left) Filling defect in duct from papilloma (right) Specimen of papilloma in a dilated duct.

Figure 1-25-16

(left) Unusual presentation of a papilloma as a discrete mass. (right) Filling defect in a dilated duct from a papilloma

(left) Lobulated mass representing a dilated duct. (right) Dilated duct with papilloma showing prominent blood flow

Lobular Neoplasia • • •

No mammographic signs usually Incidental finding on biopsy Includes atypical lobular hyperplasia and LCIS

•

High incidence of bilaterality and multifocality ➢ Consider it a bilateral disease High risk marker for the development of invasive carcinoma ➢ Up to 15% in either breast equally within 20 years ➢ Lobular or ductal features If found on core biopsy 19% upgraded to carcinoma on excision ➢ Usually LN2 or LN3 Pleomorphic and florid LCIS (subset of LN3) diagnosis carries highest risk

Lobular Neoplasia • •

•

Basic Breast Imaging

232

Chest Radiology

➢ Pleomorphic type causes necrosis and can present as irregular calcifications

Figure 1-25-17

Invasive Ductal Cancer [Figures 1-25-17 to 1-25-25] •

• • • • • • • • •

NOS (not otherwise specified) ➢ 50 to 75% of invasive cancers Medullary Papillary Colloid (Mucinous) Tubular Metaplastic Cribriform Adenoid cystic Paget's disease Inflammatory

Figure 1-25-19

Figure 1-25-18 Diffusely infiltrative invasive ductal carcinoma

(left) Spiculated mass in this invasive ductal carcinoma (right) Specimen of spiculated invasive ductal carcinoma

(left) Irregular calcification and spiculation in this invasive ductal carcinoma (right) Specimen of this case showing irregular calcifications in irregular lumens and necrotic tissue spaces

(left) Irregular calcifications in invasive ductal carcinoma (right) Specimen showing irregular calcifications in irregular ductal lumens in this invasive ductal carcinoma

Figure 1-25-22

Figure 1-25-21

(left) Mass with indistinct borders in invasive ductal carcinoma (right) Spiculated mass in this invasive ductal carcinoma

MR showing enhancing mass in invasive ductal carcinoma

Chest Radiology

Figure 1-25-20

233

Basic Breast Imaging

Figure 1-25-23

Figure 1-25-24

Irregular shaped mass with lobulations and an acute angle on the right side in this invasive ductal carcinoma

(left) Invasive ductal carcinoma presenting as a thick walled cyst (the “cyst” is central necrosis) (right) Dense shadowing by invasive ductal carcinoma

Figure 1-25-25

Paget’s Disease [Figures 1-25-26 and 1-25-27] • • • • •

Red nipple and areola Scaling eczematoid reaction 50% have a palpable mass Must have Paget’s cells in skin Usually has underlying carcinoma

Figure 1-25-26

Many irregular calcifications in this invasive ductal carcinoma

Figure 1-25-27

(left) Paget’s disease with red moist nipple (right) Paget’s disease with dry scaly nipple

Types of Invasive Ductal Carcinoma With Rounded Expansile Periphery • • • •

Medullary Papillary Cribriform Colloid

• • • • • •

Medullary Papillary Cribriform Colloid Tubular Adenoid cystic

(left) Invasive ductal carcinoma calcifications behind nipple in Paget's disease (right) Specimen confirming calcifications in invasive ductal carcinoma. Paget’s cells in skin

Types of Invasive Ductal Carcinoma with Improved Prognosis Figure 1-25-28

Medullary Carcinoma [Figure 1-25-28] (left) Medullary carcinoma with slightly more indistinct borders than fibroadenoma (right) Minimal invasion of surrounding tissue (bottom right) by medullary carcinoma Basic Breast Imaging

234

Chest Radiology

Papillary Carcinoma [Figure 1-25-29]

Figure 1-25-29

Tubular Carcinoma [Figures 1-25-30 and 1-25-31] • • •

Typically spiculated Must have 75 - 100% tubular formation Less than 75% acts like usual invasive carcinoma

Figure 1-25-30

Circumscribed macrolobulated mass was a papillary carcinoma

Figure 1-25-31

Very spiculated mass is typical tubular carcinoma

Adenoid Cystic Carcinoma

(left) Tubule formation in tubular carcinoma (right) Spiculations in tubular carcinoma

[Figure 1-25-32]

Invasive Lobular Cancer • • •

Prognosis similar to invasive ductal cancer Most commonly a spiculated mass Some are more difficult to see as they are diffusely infiltrating ➢ Present as asymmetric density

Figure 1-25-32

Invasive Lobular Cancer [Figures 1-25-33 to 1-25-35] • •

Invasive lobular Invasive ductal

Figure 1-25-33

(left) Tumor cells of invasive lobular carcinoma in single file (right) Tumor cells of invasive ductal carcinoma in enlarged thick walled ducts

Chest Radiology

Figure 1-25-34

Typical adenoid cystic carcinoma with lobulations but no spiculation

Spiculkated mass is the most common presentation of invasive lobular carcinoma 235

Basic Breast Imaging

Sarcoma • •

• • •

1% of breast malignant tumors Breast contains fat, fibrous tissue, blood vessels, etc. ➢ Angiosarcoma, malignant fibrous hystiocytoma, chondrosarcoma, rhabdomyosarcoma etc. Metaplasia can occur Malignant transformation can occur Often after chest or breast irradiation

Figure 1-25-35

Fibrosarcoma [Figure 1-25-36]

Spindle Cell Sarcoma [Figure 1-25-37] Angiosarcoma [Figure 1-25-38]

What You Need to Remember • •

The mass edge represents the aggressiveness of the underlying abnormality The shape of the calcification represents a cast of an underlying space

Figure 1-25-36

Focal asymmetric density in invasive lobular carcinoma often causes misdiagnosis

Figure 1-25-37

(left) Non specific mass in fibrosarcoma. (right) Specimen of fibrosarcoma

(left) Irregular non specific mass in spindle cell sarcoma. (right) Specimen of spindle cell sarcoma

Figure 1-25-38

Angiosarcoma

Basic Breast Imaging

236

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References 1.

2. 3. 4. 5. 6. 7. 8.

9. 10. 11.

12. 13. 14. 15.

16.

17.

18.

19.

20. 21. 22. 23.

24. 25. 26. 27. 28. 29. 30.

Alleva DQ, Smetherman DH, Farr GH, Jr., Cederbom GJ. Radial scar of the breast: radiologic-pathologic correlation in 22 cases. Radiographics 1999; 19 Spec No:S27-35; discussion S36-37. Bartella L, Liberman L, Morris EA, Dershaw DD. Nonpalpable mammographically occult invasive breast cancers detected by MRI. AJR Am J Roentgenol 2006; 186:865-870. Bassett LW. Imaging of breast masses. Radiol Clin North Am 2000; 38:669-691. Chao TC, Lo YF, Chen SC, Chen MF. Phyllodes tumors of the breast. Eur Radiol 2003; 13:88-93. Chen SC, Cheung YC, Su CH, Chen MF, Hwang TL, Hsueh S. Analysis of sonographic features for the differentiation of benign and malignant breast tumors of different sizes. Ultrasound Obstet Gynecol 2004; 23:188-193. Espinosa LA, Daniel BL, Vidarsson L, Zakhour M, Ikeda DM, Herfkens RJ. The lactating breast: contrast-enhanced MR imaging of normal tissue and cancer. Radiology 2005; 237:429-436. Feder JM, de Paredes ES, Hogge JP, Wilken JJ. Unusual breast lesions: radiologic-pathologic correlation. Radiographics 1999; 19 Spec No:S11-26. Goel NB, Knight TE, Pandey S, Riddick-Young M, de Paredes ES, Trivedi A. Fibrous lesions of the breast: imagingpathologic correlation. Radiographics 2005; 25:1547-1559. Jackson VP, Bassett LW. Breast sonography. Breast Dis 1998; 10:55-66. Kolb TM, Lichy J, Newhouse JH. Comparison of the performance of screening mammography, physical examination, and breast US and evaluation of factors that influence them: an analysis of 27,825 patient evaluations. Radiology 2002; 225:165-175. Kriege M, Brekelmans CT, Boetes C, et al. Efficacy of MRI and mammography for breast-cancer screening in women with a familial or genetic predisposition. N Engl J Med 2004; 351:427-437. Lehman CD, Blume JD, Weatherall P, et al. Screening women at high risk for breast cancer with mammography and magnetic resonance imaging. Cancer 2005; 103:1898-1905. Liberman L, Drotman M, Morris EA, et al. Imaging-histologic discordance at percutaneous breast biopsy. Cancer 2000; 89:2538-2546. Liberman L, Mason G, Morris EA, Dershaw DD. Does size matter? Positive predictive value of MRI-detected breast lesions as a function of lesion size. AJR Am J Roentgenol 2006; 186:426-430. Mercado CL, Hamele-Bena D, Oken SM, Singer CI, Cangiarella J. Papillary lesions of the breast at percutaneous core-needle biopsy. Radiology 2006; 238:801-808. Park JM, Han BK, Moon WK, Choe YH, Ahn SH, Gong G. Metaplastic carcinoma of the breast: mammographic and sonographic findings. J Clin Ultrasound 2000; 28:179-186. Pisano ED, Parham CA. Digital mammography, sestamibi breast scintigraphy, and positron emission tomography breast imaging. Radiol Clin North Am 2000; 38:861-869. Sabate JM, Clotet M, Gomez A, De Las Heras P, Torrubia S, Salinas T. Radiologic evaluation of uncommon inflammatory and reactive breast disorders. Radiographics 2005; 25:411-424. Samardar P, de Paredes ES, Grimes MM, Wilson JD. Focal asymmetric densities seen at mammography: US and pathologic correlation. Radiographics 2002; 22:19-33. Schnall MD, Blume J, Bluemke DA, et al. Diagnostic architectural and dynamic features at breast MR imaging: multicenter study. Radiology 2006; 238:42-53. Sewell CW. Pathology of benign and malignant breast disorders. Radiol Clin North Am 1995; 33:1067-1080. Sheppard DG, Whitman GJ, Huynh PT, Sahin AA, Fornage BD, Stelling CB. Tubular carcinoma of the breast: mammographic and sonographic features. AJR Am J Roentgenol 2000; 174:253-257. Shetty MK, Shah YP, Sharman RS. Prospective evaluation of the value of combined mammographic and sonographic assessment in patients with palpable abnormalities of the breast. J Ultrasound Med 2003; 22:263-268; quiz 269-270. Sickles EA. Management of probably benign breast lesions. Radiol Clin North Am 1995; 33:1123-1130. Sickles EA. Probably benign breast lesions: when should follow-up be recommended and what is the optimal followup protocol? Radiology 1999; 213:11-14. Slawson SH, Johnson BA. Ductography: how to and what if? Radiographics 2001; 21:133-150. Soo MS, Williford ME, Walsh R, Bentley RC, Kornguth PJ. Papillary carcinoma of the breast: imaging findings. AJR 1995; 164:321-326. Stavros AT, Thickman D, Rapp CL, Dennis MA, Parker SH, Sisney GA. Solid breast nodules: use of sonography to distinguish between benign and malignant lesions. Radiology 1995; 196:123-134. Stavros T, Rapp CL, Parker SH. Sonography of mammary implants. Ultrasound Q 2004; 20:217-260. van den Bosch MA, Daniel BL, Mariano MN, et al. Magnetic resonance imaging characteristics of fibrocystic change of the breast. Invest Radiol 2005; 40:436-441

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Ductal Carcinoma in Situ (DCIS) Leonard M. Glassman, MD Ductal Carcinoma in Situ (DCIS) •

Also called intraductal carcinoma ➢ Not invasive ductal carcinoma ➢ DCIS is “benign” ❖ Disease is confined to the breast ❖ Patients die from metastatic disease secondary to invasive carcinoma

Figure 1-26-1

Relative Risk of Invasive Carcinoma (Biopsy Findings) •

• • • •

•

Mild intraductal hyperplasia (IDH) ➢ No increased risk Moderate or florid IDH ➢ Slight increased risk Atypical hyperplasia (ductal or lobular) with no family history and/or postmenopausal ➢ Mild to moderate increased risk Atypical hyperplasia (ductal or lobular) with positive family history and/or premenopausal ➢ High risk Ductal carcinoma in situ (left) Thickened duct walls with necrosis and comedo ➢ High risk secretion in the lumen. Lobular carcinoma in situ (top right) Stretching and thinning if duct lumen by early ➢ High risk DCIS

Confined to the Duct [Figure 1-26-1] • •

(bottom right) Dilated duct with wall irregularity in DCIS

No spread to blood or lymph nodes Less than 1% positive axillary nodes ➢ Probably unrecognized invasion ➢ Most likely in large lesions and palpable lesions

The Problems • • • •

How big is the problem? How do we classify the disease? How do we diagnose it? What is adequate treatment?

•

USA data ➢ 50% eligible women get screening annually after 40 ➢ 240,000 new breast cancers annually ❖ Includes invasive and intraductal 1980 5% of new breast cancers were DCIS ➢ Usually a palpable lump or nipple discharge 2000 20% of new breast cancers ➢ Usually microcalcifications on mammography Age-adjusted incidence increasing ➢ 2.4 per 100,000 in 1973 ➢ 15.8 per 100,000 in 1992

How Big is the Problem?

•

• •

Is It Malignant? • •

30 to 60% of underdiagnosed DCIS becomes invasive cancer within 25 years Usually in the same breast and near the biopsy site

Ductal Carcinoma in Situ

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Chest Radiology

Invasive Cancer after DCIS •

NASBP B17 (790 women) at 5 years ➢ Women treated with breast conservation ➢ 115 recurrent cancers ❖ 80% same breast ❖ 15.7% opposite breast ❖ 4.3% regional or distant metastases ➢ 12.1% recurrence with lumpectomy + radiation (36% invasive) ➢ 26.8% recurrence with lumpectomy alone (51% invasive)

Indicators of Recurrence after Conservative Treatment • • • • • •

Tumor size Nuclear grade Necrosis Margin status Multifocality Lymphocytic infiltrate

•

Same risks as for invasive cancer ➢ Increasing age ➢ Early menarche ➢ Family history ➢ Previous breast biopsy ➢ Nulliparity or late age at first birth

Epidemiology

Pathologic Classification of DCIS • •

No uniform agreement on single scheme Interobserver agreement between schemes is poor

• • • • • • •

DIN European Commission Working Group Lagios Modified Lagios Nottingham UKNBCSP Van Nuys

•

Comedo ➢ Needs comedonecrosis and high nuclear grade Non-comedo ➢ Cribriform ➢ Micropapillary ➢ Papillary ➢ Solid Special type ➢ Apocrine ➢ Clear cell ➢ Signet ring cell ➢ Small cell ➢ Endocrine ➢ Spindle cell

Classifications of DCIS

Architectural Classification of DCIS •

•

Intraductal Carcinoma (DCIS) •

•

Histology may be able to predict recurrence risk ➢ High grade, large cell, comedo have higher recurrence ➢ Low grade, small cell, noncomedo (cribriform, micropapillary) Poor correlation of calcification type and extent with grade and extent of tumor

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Is DCIS One Disease? Low Grade ER PR HER-2/neu p53 bcl-2

• • • •

Associated invasive carcinoma shows marker phenotype like precursor DCIS Low grade DCIS yields low grade invasive tumors High grade DCIS yields high grade invasive tumors Theory: Low grade and high grade DCIS are different from the start

• •

Mass Mammographic calcifications ➢ Can’t distinguish from invasive carcinoma ➢ Associated mass usually invasive disease

Is DCIS One Disease?

+++ +++ + + +

High Grade

• • • • •

+ + +++ ++ –

Figure 1-26-2

Diagnosis of DCIS

Mass • •

Rare today Usually small

Small indistinct mass represents DCIS

DCIS Mass Close-up [Figure 1-26-2]

Figure 1-26-3

DCIS Mass [Figures 1-26-3 to 1-26-5]

Intraductal Carcinoma (DCIS) • • • • •

Microcalcification usually without mass Particles < 1 mm Varying size shape and density Clustered May coexist with benign calcifications

• • • • •

Size Number Distribution Shape Change over time

•

< 1 mm ➢ Evaluate malignant potential by smallest particles in the abnormality

Calcification

(left) Large obscured mass was entirely DCIS. (right) Small vertically oriented solid mass represents DCIS

Figure 1-26-5

Size of Particles Number •

Cluster is 5 particles or more in 1 cubic cm.

Figure 1-26-4

Irregular filling defect in duct represents DCIS

MRI with large area of enhancement was DCIS on biopsy Ductal Carcinoma in Situ

240

Chest Radiology

Distribution [Figure 1-26-6] • •

•

Figure 1-26-6

Cluster Linear ➢ DCIS involves a duct ➢ Linear distribution toward nipple ➢ High grade is continuous involvement ➢ Low grade has skip areas Segmental ➢ Involvement of an entire ductal system

Segmental Intraductal Carcinoma [Figure 1-26-7]

(left) Cluster of irregular calcifications was DCIS on biopsy (right) Broken rod shaped calcification in a linear distribution represents DCIS

Shape is Most Important •

•

Irregular ➢ Not smooth round or hollow Heterogeneous or pleomorphic ➢ Not all the same

Figure 1-26-7

Pleomorphic [Figure 1-26-8]

Calcifications represent the caste of a space • •

Irregular duct Necrotic tissue space

Intraductal Carcinoma [Figure 1-26-9] Figure 1-26-9

Segmental distribution of granular calcifications was DCIS

Figure 1-26-8

Left: Small calcifications filling the residual lumen made irregular by wall thickening of DCIS Right: Duct calcification shape is related to the contour of the duct wall

Shapes of DCIS Calcification • • • •

Granular Irregular rods Casting Irregular ➢ Branching ➢ Comma shaped ➢ Arrow shaped or pointed

Chest Radiology

Multiple irregular calcifications in biopsy proven DCIS

241

Ductal Carcinoma in Situ

Granular [Figure 1-26-10]

Figure 1-26-10

• • • •

Very small (<0.5 mm) Need magnifying glass to evaluate Too small to see true shape “Grains of sand”

• •

•

Made up of many tiny pieces on magnification Not solid large rods ➢ Secretory disease Often branch

• •

Regular Irregular

• •

Granular calcifications filling the lumen of an irregular duct Often branch

• • • •

Castes of necrotic spaces Branching Comma shaped Arrow shaped or pointed

Irregular Rods

Extremely small calcifications represent a finding of DCIS

Rods [Figure 1-26-11] Casting

Irregular

Figure 1-26-11

[Figure 1-26-12]

Figure 1-26-12

(left) Smooth rods of secretory disease (right) Broken rods of DCIS

Figure 1-26-13

(left) Diffuse microcalcifications of DCIS. (right) Ultrasound shows calcifications and parenchymal change in DCIS

Small irregular clustered calcifications were biopsied yielding DCIS

Intraductal Carcinoma [Figures 1-26-13 to 1-26-15]

Figure 1-26-14 Figure 1-26-15

(left) Clustered irregular calcifications. (right) Calcification in necrotic wall of duct involved with DCIS Ductal Carcinoma in Situ

242

Diffuse amorphous calcifications of DCIS Chest Radiology

Change Over Time • • •

Benign processes can change Malignant processes almost always change within 3 years Short interval follow-up ➢ Probably benign findings ➢ 6 months unilateral, annual bilateral for 3 years ❖ No scientific basis

Biopsy •

•

Imaging guided biopsy with specimen radiography ➢ Usually stereotactic Wire guided excision with specimen radiography

Pathologic Findings Needed in Any Report •

• • • • •

Nuclear grade ➢ Low, intermediate or high Necrosis ➢ Comedo or punctate Architectural pattern Lesion size Margin assessment Specimen processing ➢ Report should include ❖ Presence of calcification ❖ Correlation with specimen radiograph and/or mammogram

Extent of Calcification Does Not Correspond to the Extent of the Tumor • •

Best correlation is with comedo type but not good enough ➢ Poor correlation with cribriform and micropapillary Must use histologic margins to define true extent

Treatment •

•

Simple mastectomy without axillary dissection ➢ 25% of patients choose this option ➢ Large lesions in small breasts ➢ Multiple lesions ➢ No radiation ❖ Unavailability ❖ Prior radiation ❖ Collagen vascular disease ➢ Patient preference Reconstruction

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Breast Conservation •

• •

•

•

Wide local excision without axillary dissection ➢ Sentinel node when large or palpable lesions Post -excision mammogram with magnification views of biopsy site ➢ May be done 2 or 3 months after excision ➢ Not necessary if specimen radiograph shows complete excision with 10 mm margin Radiation is standard ➢ Helps in all patients ➢ Benefit may be small in a subset of patients ❖ Small lesions ❖ Low grade histology ❖ Wide clear margins Local excision alone without radiation ➢ Controversial ➢ Less than 2 - 3 cm lesion ➢ Margins should be 10 mm or greater ➢ Nuclear grade low or intermediate Recurrence risk 1% per year

What is a Clear Margin? •

Relative risk of recurrence after excision and radiation ➢ 10 mm or greater 1.14 ➢ 1 – 9 mm 1.49 ➢ <1 mm 2.54

Radiation Therapy

• • • •

1.8 to 2.0 Gy fractions Monday through Friday 45 to 50 Gy total dose Boost 10 to 20 Gy to surgical bed No axillary radiation

• •

No cytotoxic drugs Tamoxifen 20 mg daily for 5 years ➢ Newer drugs possible with fewer side effects ➢ Decreases invasive recurrences ➢ No change in survival ❖ Survival is over 90% without chemotherapy

Chemotherapy

Treatment of Recurrence •

•

DCIS ➢ Mastectomy if radiation given previously ➢ Mastectomy or wide excision with radiation Invasive carcinoma ➢ Treat like any invasive cancer ➢ Can not give radiation twice

Follow-up • •

Lifetime Annual mammography ➢ First exam 6 months after completion of treatment ➢ Every 6 months for the first two years? ➢ Use of magnification views common ❖ Most common in first exam after treatment

Summary • • •

DCIS is carcinoma without the ability to spread YET It is detected on mammography as calcification Adequate detection and treatment decreases the incidence of invasive cancer and therefore death

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References 1.

2. 3. 4. 5. 6. 7. 8. 9.

10. 11.

12.

13.

14. 15.

16.

Consensus Conference on the classification of ductal carcinoma in situ. The Consensus Conference Committee. Cancer 1997; 80:1798-1802. Cornfield DB, Palazzo JP, Schwartz GF, et al. The prognostic significance of multiple morphologic features and biologic markers in ductal carcinoma in situ of the breast: a study of a large cohort of patients treated with surgery alone. Cancer 2004; 100:2317-2327. Hashimoto BE, Kramer DJ, Picozzi VJ. High detection rate of breast ductal carcinoma in situ calcifications on mammographically directed high-resolution sonography. J Ultrasound Med 2001; 20:501-508. Hermann G, Keller RJ, Drossman S, et al. Mammographic pattern of microcalcifications in the preoperative diagnosis of comedo ductal carcinoma in situ: histopathologic correlation. Can Assoc Radiol J 1999; 50:235-240. Hermann G, Keller RJ, Halton K, Schwartz I, Rabinowitz JG, Tartter P. Nonpalpable ductal carcinoma in situ versus infiltrating carcinoma of the breast--can they be differentiated by mammography? Can Assoc Radiol J 1991; 42:219222. Holland R, Hendriks JH. Microcalcifications associated with ductal carcinoma in situ: mammographic-pathologic correlation. Semin Diagn Pathol 1994; 11:181-192. Ikeda DM, Birdwell RL, Daniel BL. Potential role of magnetic resonance imaging and other modalities in ductal carcinoma in situ detection. Magn Reson Imaging Clin N Am 2001; 9:345-356, vii. Moon WK, Myung JS, Lee YJ, Park IA, Noh DY, Im JG. US of ductal carcinoma in situ. Radiographics 2002; 22:269280; discussion 280-281. Morris EA, Liberman L, Ballon DJ, et al. MRI of occult breast carcinoma in a high-risk population. AJR Am J Roentgenol 2003; 181:619-626. Page DL, Lagios MD. Pathology and clinical evolution of ductal carcinoma in situ (DCIS) of the breast. Cancer Lett 1994; 86:1-4. Schwartz GF, Solin LJ, Olivotto IA, Ernster VL, Pressman PI. Consensus Conference on the Treatment of In Situ Ductal Carcinoma of the Breast, April 22-25, 1999. Cancer 2000; 88:946-954. Sewell CW. Pathology of high-risk breast lesions and ductal carcinoma in situ. Radiol Clin North Am 2004; 42:821830. Silverstein MJ, Lagios MD, Groshen S, et al. The influence of margin width on local control of ductal carcinoma in situ of the breast. N Engl J Med 1999; 340:1455-1461. Stomper PC, Connolly JL, Meyer JE, Harris JR. Clinically occult ductal carcinoma in situ detected with mammography: analysis of 100 cases with radiologic-pathologic correlation. Radiology 1989; 172:235-241. Waldman FM, DeVries S, Chew KL, Moore DH, 2nd, Kerlikowske K, Ljung BM. Chromosomal alterations in ductal carcinomas in situ and their in situ recurrences. J Natl Cancer Inst 2000; 92:313-320. Yen TW, Hunt KK, Mirza NQ, et al. Physician recommendations regarding tamoxifen and patient utilization of tamoxifen after surgery for ductal carcinoma in situ. Cancer 2004; 100:942-949.

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Breast Abnormalities in Young Women Leonard M. Glassman, MD

Lesions in Young Women are Rare • •

Very few patients seen by the average radiologist Women 21 years of age and younger ➢ Fibroadenoma (up to 95%) ➢ Juvenile hypertrophy ➢ Abscess and mastitis ➢ Phyllodes tumor ➢ Malignancy ❖ Primary ❖ Metastatic ➢ Cysts are rare

Lesions in Young Women are Rare •

Women over 21 years of age ➢ Fibroadenoma ➢ Abscess and mastitis ➢ Phyllodes tumor ➢ Cysts ❖ More common as age approaches 35 ➢ Malignancy

Diagnosis in Young Women •

•

Ultrasound is primary modality ➢ Breasts are dense after puberty ➢ Radiation has a small risk Mammography is used in select older patients ➢ High risk screening ➢ Masses in patients over 30 ➢ Malignant looking lesions

Breast Cancer Incidence in Young Women •

% of all breast cancers ➢ Age <20 ❖ 0% ➢ Age <30 ❖ 0.3% ➢ Age 20 – 34 ❖ 2% Invasive ❖ 1% DCIS ➢ Age 40 – 49 ❖ 19%

High Risk Screening •

• • •

Multiple first degree family members ➢ Begin mammography 10 years before the earliest affected relative ❖ Interval uncertain but every 1-2 years usual BRCA 1 and 2 ➢ Begin at 25 ❖ Interval uncertain but annual is common Ultrasound can be useful MRI can be useful ➢ More sensitive than mammography in high risk groups

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Ultrasound in Young Women •

Good test in dense breasts BUT ➢ Many benign non palpable masses in young women ➢ Very few cancers in young women

Breast MRI in Young Women •

•

Diagnosis ➢ Proven cancer ❖ Multifocality ➢ Difficult imaging ❖ Is biopsy needed? Screening ➢ Strong family history ➢ Gene positive BRCA 1 BRCA 2

Presenting Signs & Symptoms • • •

Mass Pain Screening ➢ BRCA 1 ➢ BRCA 2 ➢ Family history

Benign Lesions Occurring Multiple Times • • • • • • • • • • • •

Fibroadenoma ➢ Juvenile ➢ Giant Phyllodes low grade Granular cell tumor Lactating adenoma Hamartoma Normal breast Fibrocystic change Intraductal papilloma Juvenile papillomatosis Vascular Mastitis Juvenile hypertrophy Diabetic mastopathy

• • • • • • • • • •

PASH Granulomatous mastitis Fibromatosis Adenosis Intraductal papilloma Fibroadenomatoid hyperplasia Fibrosis Mondor’s disease Varix Rosai Dorfman disease (Sinus histiocytosis with lymphadenopathy)

•

Age 0-35 ➢ 3 Age 5-9 ➢ 8 Age 10-14 ➢ 3 Age 15-19

•

Benign Lesions Occurring Once

32 Fibroadenoma

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Breast Abnormalities in Young Women

Fibroadenoma •

•

Second most common breast lesion ➢ Fibrocystic change is first Begins in TDLU ➢ Caused by unopposed estrogenic stimulation ➢ Rare in men ❖ Must have hyperestrogenic state

Fibroadenoma • • •

Figure 1-27-1

Multiple in 16 – 25% of patients clinically Found in 25% of breasts examined microscopically Can undergo myxoid change or hyalinization ➢ Gelatinous nodule ➢ Calcification

Fibroadenoma • •

Originate in lobules (TDLU) Stages of development (left) Two intermediate density circumscribed ➢ Proliferation of epithelial and stromal elements in fibroadenomas. multiple lobules (right) Sharply bordered horizontally oriented oval ➢ Confluence of the hyperplastic lobules solid mass representing a fibroadenoma ➢ Formation of fibroadenomatous nodules ➢ Nodules coalesce to form FA

Fibroadenoma (32) [Figure 1-27-1]

Fibroadenoma vs Giant Fibroadenoma vs Juvenile Fibroadenoma • •

•

Fibroadenoma Giant fibroadenoma ➢ Large lesion usually > 10 cm Juvenile fibroadenoma ➢ Age 20 years or younger ➢ Typically rapid growth and large size ➢ Usually pericanicular type with cellular stroma

Giant Fibroadenoma (32) [Figure 1-27-2]

Juvenile Fibroadenoma (14) [Figure 1-27-3]

Figure 1-27-2

Figure 1-27-3 (left) Large circumscribed intermediate density mass which is a giant fibroadenoma. (upper right) Same lesion on ultrasound. It is sharply bordered and homogeneous (lower right) Surgical specimen without adherent surrounding tissue

(left) Large solid sharply bordered mass in a 14 year old, a juvenile fibroadenoma. (right) Gross specimen of a juvenile fibroadenoma Breast Abnormalities in Young Women

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Phyllodes Tumor • •

• •

Benign epithelial elements and cellular spindle cell stroma Can act malignant ➢ Local recurrence ➢ Distant blood born metastases ➢ Lymph node enlargement reactive usually Well circumscribed lobulated mass Similar appearance on sonography to fibroadenoma ➢ May have cystic spaces

Figure 1-27-4

Phyllodes Tumor •

•

Low grade ➢ Pushing margins ➢ Mild atypia ➢ May recur locally ➢ Rare metastases High grade ➢ Invasive margin ➢ Moderate to severe atypia ➢ Common local recurrence ➢ Hematogenous metastases

(left) CT scan showing left breast mass with areas of liquefaction necrosis. (right) Gross specimen of low grade Phyllodes with areas of necrosis

Figure 1-27-5

Phyllodes Tumor •

Treatment ➢ Wide local excision

6 Phyllodes Low Grade •

• • • •

Age 25-35 ➢ 3 Age 25-29 ➢ 3 Age 30-35 Can occur in girls under 10 years old Usually older than 10 years Tendency to recur but not metastasize Pushing margins without invasion

(left) Partially obscured large non calcified mass in the upper breast. (right) Specimen shows mass with only minimal adherent tissue

Phyllodes Low Grade (34) [Figure 1-27-4] Phyllodes Low Grade (31) [Figure 1-27-5] 6 Granular Cell Tumor •

• •

•

•

Age 15-35 ➢ 1 Age 15-19 ➢ 1 Age 20-24 ➢ 2 Age 25-29 ➢ 2 Age 30-35 Neural cell origin ➢ First described in tongue ➢ 6% in the breast ➢ 1/1000 incidence of invasive ductal carcinoma Wide age range (17-75 years) ➢ Average age 30’s Discrete round mass or spiculated mass ➢ Push or invasive margin Rare metastasis to axillary nodes ➢ One case in literature of lung metastases

Granular Cell Tumor (33) [Figure 1-27-6]

Chest Radiology

Figure 1-27-6

Circumscribed mass was granular cell tumor on biopsy 249

Breast Abnormalities in Young Women

Granular Cell Tumor (35) [Figure 1-27-7]

Figure 1-27-7

Lactating Adenoma [Figures 1-27-8 and 1-27-9] • • •

Young women Pregnant or lactating women Circumscribed lobulated masses

Figure 1-27-8

(left) Spiculated granular cell tumor. (right) Irregular solid markedly hypoechoic mass with microlobulations and spiculation in this granular cell tumor

Figure 1-27-9 (left) Lobulated solid mass is a biopsy proven lactating adenoma. (right) Lactating adenoma presenting as a smooth intermediate density non-calcified mass

Hamartoma • • •

Fibroadenolipoma Palpable mass or mammographic finding ➢ Can be large and not palpable Encapsulated normal breast elements

Hamartoma (34) [Figure 1-27-10]

Solid mass with an angular border on the right side which was proven to be a lactating adenoma

Juvenile Papillomatosis •

• •

•

Firm discrete mass ➢ Localized cystically dilated ducts with intraductal proliferation 2/3 less than 20 years old Association with family history of breast carcinoma ➢ 10% develop carcinoma within 10 years Treat with excisional biopsy

Figure 1-27-10

Juvenile Papillomatosis (14) [Figure 1-27-11] Figure 1-27-11

(left) Hamartoma as a smooth large noncalcified mass with fat in it. (right) Lobulated mildly inhomogeneous solid mass corresponding to the hamartoma on the last figure

(left) Multicystic mass with many small cystic spaces represents classic juvenile papillomatosis in a 14 year old. (right) Pathologic specimen of juvenile papillomatosis

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Juvenile Hypertrophy [Figure 1-27-12]

Figure 1-27-12

• • • • •

Usually age 11-14 Usually coincides with first menses Usually lasts 3-6 months Unilateral or bilateral palpable mass Iatrogenic amastia if removed

• •

Focal fibrosis in the breast Diabetes mellitus type 1 since childhood ➢ Poorly controlled ➢ Complications from vasculitis elsewhere Occurs in young to middle age

Diabetic Mastopathy

•

Diabetic Mastopathy (33) [Figure 1-27-13]

Figure 1-27-13

Diabetic Mastopathy (28) [Figure 1-27-14] Figure 1-27-14

Increased tissue behind the nipple in this 12 year old girl was juvenile hypertrophy

Very irregular hypoechoic mass with shadowing was also diabetic mastopathy in a 28 year old

PASH (Pseudoangiomatous Stromal Hyperplasia) • • • •

Dense mass in a 33 year old Type 1 diabetic was diabetic mastopathy

Wide age range Focal lesion usually Histologically shows slit-like separation of stromal cells Exaggerated stromal response to hormone stimulation

PASH (35) [Figure 1-27-15]

Figure 1-27-15

(left) Circumscribed mass in PASH in a 35 year old. (center) Large cystic spaces in PASH (right) Specimen showing large cystic spaces in PASH

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Granulomatous Mastitis [Figure 1-27-16] • • • •

Figure 1-27-16

Usually in reproductive age Often within 3 years of pregnancy Idiopathic Specific causes must be excluded ➢ TB or other bacteria ➢ Sarcoid ➢ Fat necrosis ➢ Foreign body

Granulomatous Mastitis (26) [Figures 1-27-17 and 1-27-18] Figure 1-27-17

Swelling and redness in granulomatous mastitis

Figure 1-27-18

Spiculated mass in granulomatous mastitis

Irregular hypoechoic mass was granulomatous mastitis

Malignant • • •

• • •

No malignant lesion under age 15 in our series Invasive ductal carcinoma ➢ DCIS Sarcoma ➢ Angiosarcoma most common High grade phyllodes Lymphoma Metastasis

Figure 1-27-19

Ductal Carcinoma

•

Age 15-35 ➢ 2 age 15-19 ➢ 2 Age 20-24 ➢ 4 Age 25-29 ➢ 18 Age 30-35 2 Secretory carcinoma

• • •

Most common carcinoma Youngest patient 6 years (not in this series) Signs similar to older patients

•

Invasive Ductal Carcinoma [Figure 1-27-19]

Breast Abnormalities in Young Women

Clustered amorphous calcifications of invasive ductal carcinoma in a 30 year old 252

Chest Radiology

Invasive Ductal Carcinoma (28) [Figure 1-27-20] Medullary Carcinoma (24) [Figure 1-27-21]

Figure 1-27-21

Figure 1-27-20

Medullary Carcinoma (17) [Figure 1-27-22]

Figure 1-27-22

Microlobulated mass with spiculations in a 28 year old represents invasive ductal carcinoma

Seventeen year old patient with medullary carcinoma

Secretory Carcinoma • • •

Irregular shaped mass with Previously called juvenile spiculations in a 24 year old patient carcinoma with medullary carcinoma Initial report age 3-15 ➢ Oldest patient 87 Limited aggressiveness in younger patients

Secretory Carcinoma (23) [Figure 1-27-23]

Figure 1-27-23

DCIS (26) [Figure 1-27-24] • •

Found on screening mammography in high risk patients Found as mass rarely or nipple discharge

Figure 1-27-24

Slightly irregular mass of secretory carcinoma in a 23 year old

Amorphous calcifications of DCIS in a 26 year old

Sarcoma • •

• •

Malignant mesenchymal tumors 1% of malignant tumors in all ages ➢ Higher % in young women After radiation therapy 2-15 years Many histological subtypes

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Sarcoma •

• • •

Age 15-35 ➢ 2 Age 15-19 ➢ 2 Age 20-24 ➢ 2 Age 25-29 ➢ 4 Age 30-35 7 Angiosarcoma 2 Granulocytic sarcoma 1 Myosarcoma

Figure 1-27-25

Angiosarcoma •

• •

14 – 82 years ➢ Mean of 35 Lobulated mass Highly aggressive lesion ➢ Axillary metastasis rare ➢ Hematogenous metastasis usual

Angiosarcoma (34) [Figure 1-2725]

(left) Large mass replacing the entire breast in angiosarcoma (right) Angiosarcoma

Phyllodes High Grade •

• • • • •

Figure 1-27-26

Age 20-35 ➢ 2 Age 20-24 ➢ 1 Age 25-30 ➢ 2 Age 30-35 Usually older than 10 years Tendency to recur and metastasize Invasive margins Axillary adenopathy usually reactive Metastases hematogenous

Phyllodes High Grade (31) [Figure 1-27-26] Lymphoma • •

(left) Lobulated mass representing a high grade phyllodes tumor (right) Lobulated edge of high grade phyllodes tumor well seen on ultrasound

Primary or secondary Focal mass or diffuse process

Lymphoma (27) [Figure 1-27-27] Metastatic Disease • • •

Neurofibrosarcoma Medulloblastoma In adults (male and female) ➢ Melanoma ➢ Lung ➢ Prostate ➢ Lymphoma

Figure 1-27-27

Irregular mass of primary lymphoma in a 27 year old

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Metastatic Disease (29) [Figure 1-27-28] •

Neurofibrosarcoma

•

Medulloblastoma

Figure 1-27-28

Metastatic Disease (35) [Figure 1-27-29] Figure 1-27-29

(left) Well marginated oval solid mass in a metastatic neurofibrosarcoma (right) Specimen shows sharply marginated mass without invasion of surrounding tissue (left) Partially obscured noncalcified mass in metastatic medulloblastoma (right) Well marginated lobulated metastatic medulloblastoma

Conclusions • •

• • • • •

Ultrasound is the primary modality in this age group Mammography is reserved for screening, likely malignant lesions and the older patients in this group MRI indications are evolving Cysts are rare especially in the younger age groups Most solid lesions are benign ➢ Fibroadenoma most common Juvenile hypertrophy and juvenile papillomatosis are unique to this age group and have specific appearances on imaging Malignant lesions occur and look like malignant lesions in older women ➢ Invasive ductal carcinoma most common

References 1.

Bock K, et.al. Pathologic Breast Conditions in Childhood and Adolescence. Evaluation by Sonographic Diagnosis. J Ultrasound Med 2005; 24:1347-1354. 2. Chateil JF, Arboucalot F, Perel Y, Brun M, Boisserie-Lacroix M, Diard F. Breast metastases in adolescent girls: US findings. Pediatr Radiol 1998; 28:832-835. 3. Ciftci AO, Tanyel FC, Buyukpamukcu N, Hicsonmez A. Female breast masses during childhood: a 25-year review. Eur J Pediatr Surg 1998; 8:67-70. 4. Elsheikh A, Keramopoulos A, Lazaris D, Ambela C, Louvrou N, Michalas S. Breast tumors during adolescence. Eur J Gynaecol Oncol 2000; 21:408-410. 5. El-Tamer MB, Song M, Wait RB. Breast masses in African American teenage girls. J Pediatr Surg 1999; 34:14011404. 6. Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: report of seven cases. Am J Surg Pathol 1997; 21:664-668. 7. Greydanus DE, Parks DS, Farrell EG. Breast disorders in children and adolescents. Pediatr Clin North Am 1989; 36:601-638. 8. Harris VJ, Jackson VP. Indications for breast imaging in women under age 35 years. Radiology 1989; 172:445-448. 9. Karl SR, Ballantine TV, Zaino R. Juvenile secretory carcinoma of the breast. J Pediatr Surg 1985; 20:368-371. 10. Kronemer KA, Rhee K, Siegel MJ, Sievert L, Hildebolt CF. Gray scale sonography of breast masses in adolescent girls. J Ultrasound Med 2001; 20:491-496; quiz 498. 11. Murphy JJ, Morzaria S, Gow KW, Magee JF. Breast cancer in a 6-year-old child. J Pediatr Surg 2000; 35:765-767. 12. Pettinato G, Manivel JC, Kelly DR, Wold LE, Dehner LP. Lesions of the breast in children exclusive of typical fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases. Pathol Annu 1989; 24 Pt 2:296-328. Chest Radiology

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13. Raganoonan C, Fairbairn JK, Williams S, Hughes LE. Giant breast tumours of adolescence. Aust N Z J Surg 1987; 57:243-247. 14. Raju GC, Jankey N, Naraynsingh V. Breast disease in young West Indian women: an analysis of 1051 consecutive cases. Postgrad Med J 1985; 61:977-978. 15. Rogers DA, Lobe TE, Rao BN, et al. Breast malignancy in children. J Pediatr Surg 1994; 29:48-51. 16. Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ. Juvenile papillomatosis and breast carcinoma. Cancer 1985; 55:1345-1352. 17. Simmons PS. Diagnostic considerations in breast disorders of children and adolescents. Obstet Gynecol Clin North Am 1992; 19:91-102. 18. Simmons PS. Breast disorders in adolescent females. Curr Opin Obstet Gynecol 2001; 13:459-461. 19. Squire R, Bianchi A, Jakate SM. Radiation-induced sarcoma of the breast in a female adolescent. Case report with histologic and therapeutic considerations. Cancer 1988; 61:2444-2447. 20. Templeman C, Hertweck SP. Breast disorders in the pediatric and adolescent patient. Obstet Gynecol Clin North Am 2000; 27:19-34. 21. Weinstein SP, Conant EF, Orel SG, Zuckerman JA, Bellah R. Spectrum of US findings in pediatric and adolescent patients with palpable breast masses. Radiographics 2000; 20:1613-1621.

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The Male Breast

Leonard M. Glassman, MD

Figure 1-28-1

Development •

Birth to puberty same as female

• • •

Major ducts with little branching Connective tissue and fat Almost no lobules

•

Less than 1% of breast imaging ➢ Mammography ➢ Ultrasound ➢ MRI ➢ CT

Anatomy [Figure 1-28-1] Imaging

Normal Male Mammogram [Figures 1-28-2 to 1-28-5] Figure 1-28-2

Normal male with minimal subareolar tissue

Figure 1-28-3

Normal male with small focus of subareolar tissue

Figure 1-28-4

Normal male with intramammary node

Male Breast Disease • • •

Presents as mass, swelling or pain Presents as nipple discharge Can be benign or malignant

• • • • •

Gynecomastia Pseudogynecomastia Papilloma Adenoma Myofibroblastoma ➢ More common in men than women Granular cell tumor Fibrocystic change

Normal male with no tissue seen except fat

Figure 1-28-5

Large amount of subareolar tissue in an asymptomatic male

Benign Disease

• •

Chest Radiology

257

The Male Breast

Benign Disease • • • • • • •

Diabetic mastopathy Epitheal inclusion cyst Cystic Lymphangioma Pleomorphic hyalinizing angioectatic tumor of soft parts Varix Leiomyoma Lipoma

•

No lactating adenomas ➢ No pregnancy Rare lobular tumors ➢ No lobules without progesterone ➢ Rare invasive lobular carcinomas reported

Benign Disease •

Benign Disease •

Rare biphasic tumors ➢ Fibroadenoma, phyllodes, carcinosarcoma ➢ Lesions begin in TDLU (lobules) ✧ Lobular development rare in men

Gynecomastia • • •

Potentially reversible enlargement of the male breast Presents as soft mobile tender subareolar mass Simultaneous proliferation ducts and stroma without encapsulation ➢ Florid (early) phase ✧ Begins as increased number of ducts and epithelial proliferation with edema and cellular fibroblastic stroma ✧ Reversible phase ➢ Fibrotic (late) stage ✧ Progresses to dilated ducts, moderate epithelial proliferation and fibrosis

Gynecomastia • •

• •

2cm or more of subareolar tissue in non obese male Common “normal” finding ➢ 55% of men at autopsy ➢ Peak incidence 60 – 69 years ➢ Significant if new or symptomatic Palpable unilateral or bilateral subareolar mass ➢ Often conical shape 65% of breast lesions in elderly males ➢ 25% Carcinoma ➢ 10% Other lesions

Gynecomastia • •

Response to hyperestrogenism or estrogen like response Absolute increase in estrogen HCG or estrogen precursors ➢ Secretion by tumors ✧ Leydig cell tumor ✧ Germ cell tumors ✧ Hepatoma ✧ Adrenal cortical tumors ✧ Pituitary tumors

Gynecomastia •

Absolute increase in estrogen HCG or estrogen precursors ➢ Estrogen therapy ✧ Prostate carcinoma ✧ Topical estradiol to scalp

The Male Breast

258

Chest Radiology

Figure 1-28-6

➢ Increase in estrogen precursors ✧ Cirrhosis ✧ Hyperthyroidism

Gynecomastia •

Relative increase in estrogen ➢ Testicular failure or atrophy ✧ Idiopathic ✧ Cytotoxic chemotherapy ➢ Puberty and senescence ✧ Transient in puberty (1 – 2 years) ➢ Klinefelter’s syndrome (XXY) ➢ Testicular feminization syndrome

Gynecomastia •

• •

Hyperthyroidism ➢ Reverses when the patient is euthyroid Refeeding after malnutrition or starvation Onset of hemodialysis

Symptomatic male with prominent subareolar tissue

Gynecomastia •

Figure 1-28-7

Drugs (partial list) ➢ Spironolactone ➢ Reserpine ➢ Digitalis ➢ Ergot ➢ Thyroid extract ➢ Dilantin ➢ Thiazide diuretics ➢ Cimetadine ➢ Marijuana

Figure 1-28-8

Gynecomastia •

Mammographic patterns ➢ Nodular glandular (florid phase) ➢ Dendritic (fibrotic phase) ➢ Diffuse glandular (very high estrogen levels)

Irregular dense tissue behind the nipple

Nodular Pattern [Figure 1-28-6] •

Fan shaped density radiating from the nipple ➢ May be more prominent in UOQ ➢ Blends into surrounding fat

Dendritic Pattern [Figure 1-28-7] •

Subareolar density with prominent extensions into fat ➢ Density smaller than nodular pattern

Symptomatic male with diffusely dense pattern

Figure 1-28-9

Diffuse Pattern [Figure 1-28-8] •

Small heterogeneously dense breast

• • •

Usually bilateral No palpable mass Excessive fat deposition in breast area ➢ Normal variant ➢ Obesity ➢ Neurifibromatosis

Pseudogynecomastia [Figure 1-28-9]

Chest Radiology

Male with enlarged fatty breast 259

The Male Breast

Myofibroblastoma [Figures 1-28-10 and 1-28-11] •

• • • • •

Solitary palpable firm mass ➢ Rarely bilateral ➢ No calcifications Freely moveable More common in men than women Mean age late 50’s Circumscribed lobulated mass without calcification Treated with local excision

Figure 1-28-10

Granular Cell Tumor [Figures 1-28-12 and 1-28-13] • • • •

Benign tumor of neural origin 6% in breast Typical age is 30’s Bimodal appearance ➢ Spiculated or circumscribed ✧ Usually circumscribed in males

Figure 1-28-13

Figure 1-28-12

Figure 1-28-11

Typical appearance of a myofibroblastoma

Oval solid mass proven to be myofibroblastoma

Granular cell tumor with smooth margins

Granular cell tumor with irregular margins

Figure 1-28-14

Epidermal Inclusion Cyst [Figure 1-28-14] • •

Skin lesion Round well circumscribed dense mass

Cystic Lymphangioma [Figure 1-28-15]

Figure 1-28-15

Left: Smooth circumscribed superficial mass proven to be an epidermal inclusion cyst. Right: Ultrasound shows epidermal origin of the mass Non specific lobulated mass proven to be a cystic lymphangioma The Male Breast

260

Chest Radiology

Granulomatous Mastitis • •

Figure 1-28-16

Idiopathic Specific causes must be excluded ➢ TB or other bacteria ➢ Sarcoid ➢ Fat necrosis ➢ Foreign body

Pleomorphic Hyalinizing Angioectatic Tumor of Soft Parts [Figure 1-28-16] Varix [Figure 1-28-17]

Leiomyoma [Figure 1-28-18] • •

Complex mass on MRI with non specific appearance proven to be a pleomorphic hyalinizing angioectatic tumor of soft parts

Circumscribed mass No mitotic activity ➢ >2 mitoses/hpf is leiomyosarcoma

Malignant Disease

Figure 1-28-17

• • • •

Carcinoma Metastasis Lymphoma Sarcoma

•

1690 new cases estimated in 2005 in USA ➢ 460 men will die of disease in 2005 1% of all invasive mammary cancers ➢ Less than 0.1% of male cancers ➢ Higher incidence in China and Africa ✧ High incidence of hyperestrogenism secondary to parasitic liver disease

Male Breast Cancer •

Male Breast Cancer •

•

•

Occurs in 60’s (10 years after women) ➢ Reported in ages 5 - 93 Unilateral painless subareolar mass ➢ Bilateral in 2% of cases ➢ Can present as bloody nipple discharge Most invasive ductal cancer including special types ➢ Invasive lobular cancer very rare ➢ DCIS rare (no screening) ➢ 80% ER positive

Large vascular structure with venous flow (not shown)

Figure 1-28-18

Male Breast Cancer •

Frequently located subareolar ➢ Most common presentation is a painless subareolar mass ➢ Mass usually eccentric to the nipple ➢ Mass round, oval or irregular ➢ Calcifications rare and coarser than in women

Male Breast Cancer • • •

Paget’s disease and skin ulceration more common than in women Axillary metastases similar to women at same stage Found at later stage than women

•

Infiltrating ductal carcinoma including special types ➢ 93.7% invasive ductal (usually NOS) ➢ 2.6 % papillary ➢ 1.8% colloid ➢ 1.5% lobular

Male Breast Cancer

Chest Radiology

261

Leiomyoma with typical appearance as a circumscribed non specific mass

The Male Breast

Male Breast Cancer

Figure 1-28-19

• • •

DCIS ➢ 10% of cases ➢ Usually papillary type ➢ Comedo type very rare Liposarcoma Lymphoblastic lymphoma Metastasis

• • • • • • • •

Advanced age Family history Jewish heritage Chest wall irradiation Hyperestrogenism Hyperthyroidism Exposure to hepatotoxins Occupational exposure to high heat

•

BRCA 2 in 4 – 16% of cancer patients ➢ 40% in Iceland Undescended testes Orchiectomy and orchitis Klinefelter’s syndrome ➢ 47, XXY ➢ 6% of male breast cancer ➢ 3% lifetime risk

•

Carcinoma Risk Factors

Irregular lobulations in a subareolar invasive ductal carcinoma

Carcinoma Risk Factors • • •

Figure 1-28-20

Invasive Ductal Carcinoma [Figures 1-28-19 to 1-28-21] Papillary Carcinoma [Figure 1-28-22]

Figure 1-28-21

Irregular mass with spiculations is proven invasive ductal carcinoma

Figure 1-28-22 Ultrasound of irregular lobulated vascular mass typical of invasive ductal carcinoma

Metastasis • • •

•

Prostate most common in males Hematogenous spread from primary Usually in patients with widespread disease ➢ Occasionally solitary Usually round or oval circumscribed lobulated non-calcified mass Ductal involvement by a papillary carcinoma

The Male Breast

262

Chest Radiology

Metastasis Small Cell Carcinoma Lung [Figure 1-28-23]

Figure 1-28-23

Lymphoma • •

Primary or secondary Usually a unilateral mass ➢ Can be diffuse thickening rarely ➢ No calcification or retraction

Liposarcoma [Figures 1-28-24 and 1-28-25] • •

Very rare sarcoma Slowly enlarging painful mass

• • • • •

Age 60’s Soft Mobile Tender usually Subareolar ➢ Central Unilateral or bilateral Nodular, fibrotic or diffuse

Gynecomastia

• •

Gynecomastia •

•

Nodular ➢ Fan shaped Fibrotic ➢ Subareolar density with extensions into fat

Carcinoma

Age 60’s Soft or hard Mobile or fixed Tender or painless Subareolar Eccentric usually Unilateral usually Mass, large or small

Typical rounded masses in metastatic disease

Figure 1-28-24

Carcinoma

Large mass Lobulated border Small mass Spiculations

Gynecomastia / Carcinoma [Figures 1-28-26] Conclusion • • •

•

Disease presents as mass pain or nipple discharge Gynecomastia and invasive ductal cancer are the most common lesions in the male breast ➢ There are other rarer benign and malignant lesions Gynecomastia and carcinoma can look similar ➢ Biopsy is sometimes necessary to separate gynecomastia from carcinoma All lesions eccentric to the nipple need biopsy unless they are characteristically benign ➢ Contain fat ➢ Lymph node

Figure 1-28-26

Figure 1-28-25

CT scan showing water density mass in this male patient

Left: Gynecomastia. Right: Carcinoma

Chest Radiology

Large water density mass in a male breast with a preexisting lipoma

263

The Male Breast

References 1. 2. 3. 4. 5. 6. 7. 8. 9.

10. 11.

12. 13. 14. 15.

16. 17.

Appelbaum AH, Evans GF, Levy KR, Amirkhan RH, Schumpert TD. Mammographic appearances of male breast disease. Radiographics 1999; 19:559-568. Braunstein GD, Glassman HA. Gynecomastia. Curr Ther Endocrinol Metab 1997; 6:401-404. Chantra PK, So GJ, Wollman JS, Bassett LW. Mammography of the male breast. AJR Am J Roentgenol 1995; 164:853-858. Dershaw DD, Borgen PI, Deutch BM, Liberman L. Mammographic findings in men with breast cancer. AJR Am J Roentgenol 1993; 160:267-270. Evans GF, Anthony T, Turnage RH, et al. The diagnostic accuracy of mammography in the evaluation of male breast disease. Am J Surg 2001; 181:96-100. Fentiman IS, Fourquet A, Hortobagyi GN. Male breast cancer. Lancet 2006; 367:595-604. Giordano SH. A review of the diagnosis and management of male breast cancer. Oncologist 2005; 10:471-479. Giordano SH, Cohen DS, Buzdar AU, Perkins G, Hortobagyi GN. Breast carcinoma in men: a population-based study. Cancer 2004; 101:51-57. Gunhan-Bilgen I, Bozkaya H, Ustun EE, Memis A. Male breast disease: clinical, mammographic, and ultrasonographic features. Eur J Radiol 2002; 43:246-255. Haraldsson K, Loman N, Zhang QX, Johannsson O, Olsson H, Borg A. BRCA2 germ-line mutations are frequent in male breast cancer patients without a family history of the disease. Cancer Res 1998; 58:1367-1371. Hill TD, Khamis HJ, Tyczynski JE, Berkel HJ. Comparison of male and female breast cancer incidence trends, tumor characteristics, and survival. Ann Epidemiol 2005; 15:773-780. Hodgson NC, Button JH, Franceschi D, Moffat FL, Livingstone AS. Male breast cancer: is the incidence increasing? Ann Surg Oncol 2004; 11:751-755. Iredale R, Brain K, Williams B, France E, Gray J. The experiences of men with breast cancer in the United Kingdom. Eur J Cancer 2006; 42:334-341. Jellici E, Malago R, Remo A, Bonetti F, Pozzi Mucelli R. Imaging of the male breast. Radiol Med (Torino) 2005; 110:574-588. Michels LG, Gold RH, Arndt RD. Radiography of gynecomastia and other disorders of the male breast. Radiology 1977; 122:117-122. Pappo I, Wasserman I, Halevy A. Ductal carcinoma in situ of the breast in men: a review. Clin Breast Cancer 2005; 6:310-314. Shi AA, Georgian-Smith D, Cornell LD, et al. Radiological reasoning: male breast mass with calcifications. AJR Am J Roentgenol 2005; 185:S205-210.

The Male Breast

264

Chest Radiology

Gastrointestinal Radiology

266

Gastrointestinal Radiology

Benign Hepatic Neoplasms Angela D. Levy, COL, MC, USA

AFIP Classification - Tumors of the Liver and Intrahepatic Bile Ducts •

• •

Hepatocellular origin ➢ Hepatocellular adenoma, focal nodular hyperplasia, nodular regenerative hyperplasia ➢ Hepatocellular carcinoma, fibrolamellar carcinoma, hepatoblastoma Cholangiocellular origin ➢ Bile duct cyst, biliary cystadenoma, bile duct adenoma ➢ Cholangiocarcinoma, biliary cystadenocarcinoma Mesenchymal origin ➢ Hemangioma, angiomyolipoma, myelolipoma, mesenchymal hamartoma ➢ Angiosarcoma, epithelioid hemangioendothelioma

Benign Hepatic Neoplasms - Objectives •

•

Benign neoplasms ➢ Hemangioma ➢ Focal nodular hyperplasia (FNH) ➢ Hepatocellular adenoma ➢ Bile duct cyst ➢ Biliary cystadenoma/cystadenocarcinoma ➢ Lipomatous tumors Surgical vs. nonsurgical neoplasms

Hemangioma •

• • •

Most common benign hepatic tumor ➢ Likely a hamartoma rather than true neoplasm 1% to 7% of the population ➢ Most common in adult women ➢ Least common in pediatric population More common in women, 5:1 ➢ Estrogen influences ➢ May enlarge during pregnancy Symptoms ➢ 85% asymptomatic ➢ Pain ➢ Palpable mass ➢ Rupture

Hemangioma • •

•

Kasabach-Merritt syndrome ➢ Hemolytic anemia and consumptive coagulopathy Erythropoietin secretion ➢ Erythrocytosis Associations ➢ Focal nodular hyperplasia ➢ Tuberous sclerosis

Hemangioma - Pathology • • • •

Peripheral feeding vessels Blood filled spaces Endothelial lining Fibrosis from ➢ Slow flowing blood ➢ Thrombosis ➢ Hyalinization ➢ Scar formation

Gastrointestinal Radiology

267

Benign Hepatic Neoplasms

Hemangioma - Sonography [Figure 2-1-1] • • •

Homogeneous, hyperechoic Minimal posterior acoustic enhancement Atypical features ➢ Hypoechoic center ➢ Echogenic border ➢ Scalloped borders ➢ Heterogeneous hypoechoic

Hemangioma - Hypoechoic Foci Hemangioma - Scalloped, echogenic border

Figure 2-1-1

Hepatic hemangioma on sonography shows a well-defined mass that is homogenously hyperechoic. Histologically, the tumor is composed of multiple blood filled spaces that provide interfaces to produce an echogenic mass on sonography

Figure 2-1-2

Hemangioma Heterogeneous, hypoechoic Hemangioma - CT and MR • • • •

Peripheral globular enhancement in arterial phase Slow centripetal filling during portal venous/equilibrium Rapid enhancement pattern ➢ Capillary hemangiomas Classic appearance of hemangioma on CT. ➢ "Flash fill" phenomenon There is discontinuous, nodular, peripheral enhancement and gradual MR contrast filling in the lesion ➢ Homogenous hyperintense T2 Figure 2-1-3 ➢ Progressive hyperintensity as TE increases ➢ "Light bulb" phenomenon

Hemangioma - CT

Hemangioma - MR

[Figure 2-1-2]

Hemangioma - Pedunculated Hemangioma - Edematous scar

Atypical hemangioma on MR due to large size and central hyalinization. Tagged-RBC nuclear medicine imaging is positive confirming the diagnosis of hemangioma

Hemangioma Heterogeneous with Fibrosis [Figure 2-1-3]

Figure 2-1-4

Hemangioma - Multiplicity [Figure 2-1-4]

Focal Nodular Hyperplasia • •

• •

Second most common benign liver neoplasm 80% to 95% occur in women ➢ Peak age, 20 to 40 years 80% asymptomatic Associations ➢ Hepatic hemangiomas ➢ Intracranial aneurysms ➢ Dysplastic system arteries ➢ Intracranial neoplasms: meningioma, astrocytoma

Benign Hepatic Neoplasms

Multiple hemangiomas shown on MR 268

Gastrointestinal Radiology

• •

Pathogenesis ➢ Hyperplastic response to a vascular malformation ➢ Central artery ➢ Central scar Gross Pathology ➢ Central scar ➢ Nodular with fibrous septa ➢ No hemorrhage or necrosis ➢ No capsule

Figure 2-1-5

Focal Nodular Hyperplasia •

Histology ➢ Fibrous septa FNH is often isoechoic to normal liver on sonography and may show ➢ Large arteries marked flow on color doppler of power doppler imaging ➢ Normal hepatocytes ➢ Kupffer cells ➢ No portal tracts or central veins

Focal Nodular Hyperplasia - Sonography [Figure 2-1-5] •

• •

Subtle ➢ Similar texture to normal liver ➢ “Stealth lesion” Scar is hypoechoic Doppler ➢ Peripheral and central vessels

Figure 2-1-6

Focal Nodular Hyperplasia - CT •

• •

Noncontrast ➢ Iso- or hypodense ➢ Hypodense scar Arterial ➢ Rapid enhancement ➢ Hypodense scar Portal venous ➢ Iso- or hypo- or hyperdense ➢ Delayed enhancement of scar ➢ Peripheral capsule-like vessels

FNH [Figures 2-1-6 and 2-1-7]

FNH shows contrast enhancement during the arterial phase and near isoattenuation during the portal venous phase

Figure 2-1-7

FNH - Sulfur Colloid • • •

Normal uptake 60% Defect 30% Increased uptake 10%

•

T1 isointense ➢ Low signal scar T2 iso or slightly hyperintense ➢ High signal scar FNH on CT showing late enhancement of the central scar and Gd-DTPA peripheral vessels ➢ Rapid homogeneous enhancement ➢ May have flash enhancement ➢ Delayed enhancement of the scar ➢ Rim-like enhancement late T2 with ferumoxide ➢ Lesion decreases signal ➢ Except scar

FNH - MR • •

•

Gastrointestinal Radiology

269

Benign Hepatic Neoplasms

FNH [Figure 2-1-8]

FNH - Flash Enhancement

FNH - Ferumoxide-enhanced MR Paley MR, et al. AJR 2000; 175:1: 159-63

Figure 2-1-8

FNH - Atypical Imaging Features • • • • • •

Multiplicity Absent scar Very large scar Fat Hemorrhage Calcification (very rare)

Atypical FNH - Absent Scar

Atypical FNH - Hemorrhage

Typical appearance of FNH on MR

Hepatocellular Adenoma • • •

• •

Third most common benign liver tumor Composed of benign hepatocytes Almost always occur in women ➢ Mean age, 30 years ➢ History of oral contraceptive use ➢ Declining incidence Surgical resection ➢ Risk of hemorrhage ➢ Small risk of malignant transformation to HCC Hepatocyte proliferation ➢ Exogenous estrogens ➢ Ovarian tumors ➢ Anabolic steroids ➢ Antiestrogens ➢ Glycogenosis, type Ia and III ➢ Hurler syndrome

Hepatocellular Adenoma - Clinical Features •

• •

Acute abdominal pain 40% ➢ Hemorrhage within tumor ➢ Intraperitoneal hemorrhage Palpable mass 35% Incidental 10%

Hepatocellular Adenoma - Pathologic Features • •

Histology ➢ Benign hepatocytes ➢ Rich in glycogen ➢ Kupffer cells Gross ➢ Solitary ➢ Multiple (up to 50%) ➢ Capsule (25%) ➢ Peripheral vessels ➢ Central fat ➢ Necrosis, infarcts, hemorrhage

Benign Hepatic Neoplasms

270

Gastrointestinal Radiology

Hepatocellular Adenoma - CT and MR • • •

• • •

Capsule Heterogeneous Hemorrhage (25% to 50%) ➢ Acute, high density on unenhanced CT ➢ Chronic, hemosiderin rings on MR Focal fat Enhancement ➢ Variable Intracellular glycogen/fat ➢ Diffuse low attenuation on CT ➢ Loss of signal on out-ofphase MR

Figure 2-1-9

Hemorrhagic hepatocellular adenoma

Figure 2-1-10

Hepatocellular Adenoma Acute Hemorrhage [Figure 2-1-9]

Hepatocellular Adenoma Hemosiderin Rings Hepatocellular Adenoma Focal Fat and Capsule [Figure 2-1-10]

Hepatocellular Adenoma Diffuse Low Attenuation [Figure 2-1-11]

Hepatocellular adenoma with focal fat and a capsule on CT with the corresponding gross specimen

Hepatocellular Adenoma - Out-of-Phase MR

Figure 2-1-11

Hepatocellular Adenoma Fat Suppression

Hepatocellular Adenoma Imaging Difficulties • • •

Nonhemorrhagic Fibrosis/scar formation Multiple ➢ Glycogenosis ➢ Hepatocellular adenomatosis Diffuse low attenuation in hepatocellular adenoma due to intracellular glycogen

Hepatocellular Adenoma Multifocality • •

Multiple estrogen-associated adenomas Hepatocellular adenomatosis

• • • • •

Affects men and women Unrelated to estrogens Abnormal LFT's Biopsy for diagnosis Treated symptomatically

Hepatocellular Adenomatosis [Figure 2-1-12]

Figure 2-1-12

Hepatocellular adenomatosis Gastrointestinal Radiology

271

Benign Hepatic Neoplasms

Bile Duct (Hepatic) Cyst • •

•

Common Congenital/developmental origin ➢ Lined by a single layer of columnar cells Affect all age groups ➢ Majority occur in 4th to 6th decades of life ➢ Rare in children

Bile Duct (Hepatic) Cyst •

• •

Asymptomatic ➢ Majority of cases ➢ Incidental discovery Symptomatic ➢ Large size ➢ Secondary hemorrhage or infection ➢ Treated with drainage, sclerotherapy, or excision Imaging ➢ Unilocular, simple cyst ➢ Septations, debris when complicated by infection or hemorrhage

Bile Duct (Hepatic) Cyst •

Complex cyst differential ➢ Echinococcal cyst ➢ Simple cyst with hemorrhage/infection ➢ Post-traumatic cyst ➢ Abscess ➢ Ciliated hepatic foregut cyst ➢ Peliosis ➢ Biliary cystadenoma ➢ Biliary cystadenocarcinoma ➢ Cystic metastasis ➢ Teratoma

Biliary Cystadenoma • • •

Benign tumor, but ➢ May recur after excision ➢ May develop into cystadenocarcinoma Middle-aged women ➢ 42 - 55 years ➢ Ovarian stroma histologically Cystic neoplasms ➢ Unilocular or multilocular ➢ Septations ➢ Mural nodules ➢ Calcification

Biliary Cystadenoma - Imaging Features •

• • •

•

Cystic neoplasms ➢ Unilocular or multilocular ➢ Cyst fluid variable composition Septations Mural nodules ➢ May enhance Calcification ➢ Punctate or linear May communicate or extend into biliary system

Benign Hepatic Neoplasms

272

Gastrointestinal Radiology

Biliary Cystadenoma [Figure 2- 1-13]

Figure 2-1-13

Lipomatous Tumors •

•

Angiomyolipoma ➢ Benign ➢ Composed of adipose, smooth muscle, and blood vessels ➢ Most cases sporadic ➢ Tuberous sclerosis in 6% Myelolipoma ➢ Rare ➢ Benign ➢ Composed of myeloid, adipose, and blood vessels

Angiomyolipoma [Figures 2-1-14 and 2-1-15] Myelolipoma

Summary - Benign Hepatic Neoplasms •

•

Nonsurgical lesions ➢ Hemangioma ➢ Focal nodular hyperplasia Surgical lesions ➢ Hepatocellular adenoma ➢ Biliary cystadenoma

Biliary cystadenoma

Figure 2-1-14

Summary - Hemangioma •

• •

Sonography ➢ Homogenous ➢ Hyperechoic CT/MR ➢ Peripheral nodular enhancement Tagged-RBC

Summary - FNH •

•

CT/MR ➢ Rapid enhancement ➢ Homogenous tumor ➢ Hypodense/intense scar ➢ Delayed scar enhancement ➢ Delayed peripheral enhancement Sulfur colloid

Summary - HCA •

• •

Echogenic angiomyolipomas on sonography

For imaging diagnosis ➢ Female patient ➢ Oral contraceptive use ➢ Evidence of hemorrhage Suggest HCA ➢ Diffuse low attenuation ➢ Diffuse fat on MR ➢ Appropriate patient BIOPSY !

Figure 2-1-15

Summary - Biliary Cystadenoma •

•

Cystic neoplasm ➢ Septations ➢ Nodules ➢ Calcification Most common in middle-aged women

Gastrointestinal Radiology

Multiple hepatic angiomyolipomas in a patient with tuberous sclerosis who has angiomyolipomas in the right kidney and a history of left nephrectomy for a hemorrhagic angiomyolipoma 273

Benign Hepatic Neoplasms

References

Hemangioma 1. Freeny PC, Marks WM. Patterns of contrast enhancement of benign and malignant hepatic neoplasms during bolus dynamic and delayed CT. Radiology 1986; 160:613-618. 2. Birnbaum BA, Noz ME, Chapnick J, et al. Hepatic hemangiomas: diagnosis with fusion of MR, CT, and Tc-99mlabeled red blood cell SPECT images. Radiology 1991; 181:469-474. 3. Quinn SF, Benjamin GG. Hepatic cavernous hemangiomas: simple diagnostic sign with dynamic bolus CT. Radiology 1992; 182:545-548. 4. Yamashita Y, Ogata I, Urata J, Takahashi M. Cavernous hemangioma of the liver: pathologic correlation with dynamic CT findings. Radiology 1997; 203:121-125. 5. Kim T, Federle MP, Baron RL, Peterson MS, Kawamori Y. Discrimination of small hepatic hemangiomas from hypervascular malignant tumors smaller than 3 cm with three-phase helical CT. Radiology 2001; 219:699-706. Focal Nodular Hyperplasia 1. Mattison GR, Glazer GM, Quint LE, Francis IR, Bree RL, Ensminger WD. MR imaging of hepatic focal nodular hyperplasia: characterization and distinction from primary malignant hepatic tumors. AJR Am J Roentgenol 1987; 148:711-715. 2. Rummeny E, Weissleder R, Sironi S, et al. Central scars in primary liver tumors: MR features, specificity, and pathologic correlation. Radiology 1989; 171:323-326. 3. Buetow PC, Pantongrag-Brown L, Buck JL, Ros PR, Goodman ZD. Focal nodular hyperplasia of the liver: radiologic-pathologic correlation. RadioGraphics 1996; 16:369-388. 4. Paley MR, Mergo PJ, Torres GM, Ros PR. Characterization of focal hepatic lesions with ferumoxides-enhanced T2-weighted MR imaging. AJR Am J Roentgenol 2000; 175:159-163. 5. Brancatelli G, Federle MP, Grazioli L, Blachar A, Peterson MS, Thaete L. Focal nodular hyperplasia: CT findings with emphasis on multiphasic helical CT in 78 patients. Radiology 2001; 219:61-68. 6. Ruppert-Kohlmayr AJ, Uggowitzer MM, Kugler C, Zebedin D, Schaffler G, Ruppert GS. Focal nodular hyperplasia and hepatocellular adenoma of the liver: differentiation with multiphasic helical CT. AJR Am J Roentgenol 2001; 176:1493-1498. 7. Hussain SM, Terkivatan T, Zondervan PE, et al. Focal nodular hyperplasia: findings at state-of-the-art MR imaging, US, CT, and pathologic analysis. Radiographics 2004; 24:3-17; discussion 18-19. Hepatocellular Adenoma 1. al-Otaibi L, Whitman GJ, Chew FS. Hepatocellular adenoma. AJR Am J Roentgenol 1995; 165:1426. 2. Casillas VJ, Amendola MA, Gascue A, Pinnar N, Levi JU, Perez JM. Imaging of nontraumatic hemorrhagic hepatic lesions. Radiographics 2000; 20:367-378. 3. Grazioli L, Federle MP, Ichikawa T, Balzano E, Nalesnik M, Madariaga J. Liver adenomatosis: clinical, histopathologic, and imaging findings in 15 patients. Radiology 2000; 216:395-402. 4. Ichikawa T, Federle MP, Grazioli L, Nalesnik M. Hepatocellular adenoma: multiphasic CT and histopathologic findings in 25 patients. Radiology 2000; 214:861-868.

Biliary Cystadenoma 1. Palacios E, Shannon M, Solomon C, Guzman M. Biliary cystadenoma: ultrasound, CT, and MRI. Gastrointest Radiol 1990; 15:313-316. 2. Buetow PC, Buck JL, Pantongrag-Brown L, et al. Biliary cystadenoma and cystadenocarcinoma: clinical-imagingpathologic correlations with emphasis on the importance of ovarian stroma. Radiology 1995; 196:805-810. 3. Levy AD, Murakata LA, Abbott RM, Rohrmann CA, Jr. From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics 2002; 22:387-413.

Benign Hepatic Neoplasms

274

Gastrointestinal Radiology

Malignant Hepatic Neoplasms Angela D. Levy, COL, MC, USA

Malignant Hepatic Neoplasms - Objectives

•

Malignant neoplasms ➢ Hepatocellular carcinoma (HCC) ➢ Fibrolamellar carcinoma (FLC) ➢ Intrahepatic cholangiocarcinoma ➢ Angiosarcoma ➢ Epithelioid hemangioendothelioma Approach to the incidentally discovered liver mass

• •

Neoplasm composed of malignant hepatocytes Fifth most common cancer worldwide

•

High incidence areas ➢ Sub-Saharan Africa, Asia ➢ 30 to 45 years old ➢ Hepatitis B and C, aflatoxins ➢ Aggressive Low incidence areas ➢ Western hemisphere ➢ 70 to 80 years old ➢ Alcoholic cirrhosis, hepatitis C, hemochromatosis ➢ Insidious

•

Hepatocellular Carcinoma

Hepatocellular Carcinoma - Geographic Variation

•

Hepatocellular Carcinoma - Etiology •

Strong association with chronic liver disease ➢ Cirrhosis ➢ Hepatitis B ➢ Hepatitis C

Hepatocellular Carcinoma - Other Etiologies • • •

Aflatoxin B1 Metabolic Diseases ➢ Hemochromatosis (25%) ➢ Hereditary tyrosinemia (20%) ➢ Alpha-1-antitrypsin deficiency (15%) Anabolic steroids

Hepatocellular Carcinoma - Clinical Features •

• •

More common in men ➢ 2:1 to 5:1 Elevated alpha-fetoprotein (AFP) ➢ Elevated in 70%-90% Paraneoplastic syndromes ➢ Hypoglycemia ➢ Erythrocytosis ➢ Hypercholesterolemia ➢ Rare, hypercalcemia, precocious puberty, gynecomastia, carcinoid syndrome, osteoporosis, hypertrophic pulmonary osteoarthropathy

Gastrointestinal Radiology

275

Malignant Hepatic Neoplasms

Hepatocellular Carcinoma - Pathophysiology •

• •

Key feature relevant to imaging ➢ Angiogenesis Normal liver blood supply ➢ ~80% portal venous ➢ ~20% hepatic artery HCC blood supply ➢ ~100% hepatic artery ➢ Rarely, hypovascular

Figure 2-2-1

Hepatocellular Carcinoma Gross Pathology •

Key features relevant to imaging ➢ Capsule ➢ Necrosis/hemorrhage/fibrosis ➢ Vascular invasion ➢ Macroscopic fat ➢ No calcification when HCC occurs in chronic liver disease

Hepatocellular Carcinoma - Gross Pathology • • • • •

Solitary and Encapsulated Macroscopic fat Hemorrhage and necrosis Multifocal Vascular invasion

• • •

Trabecular growth Occasional Kupffer cells Vascular invasion

•

Variable and nonspecific ➢ Small lesions, hypoechoic and uniform ➢ Large lesions, focal and heterogeneous ➢ Diffuse, multinodular pattern Suggestive features ➢ High velocity arterial flow ➢ Peripheral hypoechoic rim

Hepatocellular Carcinoma - Histologic Features Hepatocellular Carcinoma Sonographic Features

•

Sonographic appearance of a small HCC. The lesion is well defined and hypoechoic. On CT, the mass is hypervascular in the arterial phase of contrast enhancement

Figure 2-2-2

Mosaic appearance of HCC on sonography, CT, and gross pathology

Hepatocellular Carcinoma Hypoechoic [Figure 2-2-1]

Figure 2-2-3

Hepatocellular Carcinoma - Peripheral Hypoechoic Rim

Hepatocellular Carcinoma - Mosaic Pattern [Figure 2-2-2]

Hepatocellular Carcinoma - Multifocal with Portal Vein Invasion [Figure 2-2-3]

Multifocal HCC with portal vein invasion on sonography Malignant Hepatic Neoplasms

276

Gastrointestinal Radiology

Hepatocellular Carcinoma - CT and MR Features •

• •

Arterial phase ➢ Rapid enhancement in small HCC ➢ Late arterial phase better than early arterial phase Portal venous phase ➢ Heterogeneous, "mosaic pattern" Suggestive features ➢ Capsular enhancement ➢ Central fibrosis ➢ Fatty change ➢ Vascular invasion ➢ Arterioportal shunting

Figure 2-2-4

Hypervascular HCC in cirrhosis

Figure 2-2-5

Hepatocellular Carcinoma Small HCC in Cirrhosis [Figure 2-2-4]

Hepatocellular Carcinoma Capsule and Macroscopic Fat [Figure 2-2-5]

HCC with capsule and macroscopic fat

Hepatocellular Carcinoma - Mosaic Pattern

Figure 2-2-6

Hepatocellular Carcinoma Mosaic Pattern with Capsular Enhancement [Figure 2-2-6] Hepatocellular Carcinoma Mosaic Pattern Hepatocellular Carcinoma Fibrosis

Hepatocellular Carcinoma Multifocal with Portal Vein Invasion Hepatocellular Carcinoma Solitary and Portal Vein Invasion

Hepatocellular Carcinoma Hepatic Vein/IVC Invasion [Figure 2-2-7]

Figure 2-2-7

Gastrointestinal Radiology

HCC with IVC invasion on MDCT

277

HCC with a mosaic pattern and capsular enhancement

Malignant Hepatic Neoplasms

Hepatocellular Carcinoma - MR Imaging in Cirrhosis

Figure 2-2-8

Hepatocellular Carcinoma - Noncirrhotic Liver • • • • •

Large, solitary masses Heterogeneous Capsule Fat (10%) Calcification (25%)

Hepatocellular Carcinoma - Noncirrhotic Liver Fibrolamellar Carcinoma [Figure 2-2-8] •

•

Variant of HCC ➢ Bands of fibrous lamellae ➢ Tumor cells have "oncocytic" cytoplasm Young patients ➢ Mean age, 23 years ➢ No cirrhosis ➢ AFP usually normal

Fibrolamellar Carcinoma - Gross Pathology •

• •

Central scar ➢ Radiating septa ➢ Calcification Lobulated contour Bile staining

Histology and gross pathology of fibrolamellar carcinoma

Fibrolamellar Carcinoma - CT Features[Figures 2-2-9 and 2-2-10] • •

•

Lobulated, well defined margins Heterogeneous mass ➢ Arterial phase enhancement Central scar ➢ Hypodense in all phases of enhancement ➢ Calcification in 40%

Figure 2-2-9

Fibrolamellar Carcinoma MR Features • •

•

Lobulated margins Heterogeneous signal mass ➢ Dark T1 ➢ Bright T2 Hypointense central scar ➢ Dark T1 ➢ Dark T2 ➢ No enhancement

Fibrolamellar carcinoma

Fibrolamellar Carcinoma

How can I differentiate FLC from FNH? •

• •

Figure 2-2-10

Tumor heterogeneous in FLC ➢ Homogeneous in FNH Scar nonenhancing in FLC ➢ Delayed enhancement in FNH Scar dark T2 signal in FLC ➢ Scar bright T2 in FNH

Fibrolamellar carcinoma Malignant Hepatic Neoplasms

278

Gastrointestinal Radiology

Intrahepatic Cholangiocarcinoma (ICC) •

• •

Adenocarcinoma arising from intrahepatic bile ducts ➢ 10% of bile duct adenocarcinomas Synonyms ➢ Peripheral cholangiocarcinoma, cholangiocellular carcinoma, intrahepatic bile duct carcinoma Geographic incidence variation ➢ 10 times more common in Japan compared to U.S.

Figure 2-2-11

Intrahepatic Cholangiocarcinoma - Etiology •

Majority of cases ➢ Unknown etiology ➢ Noncirrhotic liver

Intrahepatic Cholangiocarcinoma - Etiologic Associations •

• • • •

Chronic cholestatic disease ➢ Primary sclerosing cholangitis ➢ Primary biliary cirrhosis ➢ Caroli disease/congenital hepatic fibrosis Chronic biliary inflammation ➢ Recurrent pyogenic cholangitis ➢ Parasitic infection ➢ Hepatolithiasis Hepatitis B and C ETOH abuse Radiation

Intrahepatic cholangiocarcinoma

Figure 2-2-12

Intrahepatic Cholangiocarcinoma Pathologic Features [Figure 2-2-11] •

• • • •

Morphology ➢ Solitary ➢ Multifocal ➢ Diffuse Satellite nodules Marked fibrosis No capsule Rare ➢ Hemorrhage and necrosis ➢ Calcification

Intrahepatic cholangiocarcinoma showing capsular contraction and biliary dilatation peripheral to the mass

Figure 2-2-13

Intrahepatic Cholangiocarcinoma CT and MR Features •

• •

• •

Irregular borders ➢ Infiltrative Enhancement pattern ➢ Due to fibrosis/hypovascularity ➢ Delayed peripheral to central Intrahepatic cholangiocarcinoma on MR showing central to peripheral enhancement on gadolinium enhanced T1-weighted images. Biliary dilatation peripheral to the The gross photograph shows tumor characteristic fibrosis within the tumor Capsular contraction Vascular invasion

Intrahepatic Cholangiocarcinoma [Figures 2-2-12 and 2-2-13]

Gastrointestinal Radiology

279

Malignant Hepatic Neoplasms

How can I differentiate ICC from HCC? •

•

Difficult ➢ HCC has variable morphology ➢ HCC occurs more commonly ➢ HCC associated with cirrhosis and hepatitis ➢ But, HCC may occur in normal livers Ultimately ➢ Biopsy is needed for diagnosis

How can I differentiate ICC from HCC? •

• •

•

Enhancement ➢ Delayed, peripheral to central favors ICC ➢ Rapid filling favors HCC ➢ Marked heterogeneity (mosaic) favors HCC Tumor margins ➢ Lobulated, irregular favors ICC ➢ Capsule favors HCC Capsular contraction ➢ More common in ICC Biliary dilatation peripheral to the tumor ➢ More common in ICC

Figure 2-2-14

Angiosarcoma • • •

Malignant neoplasm of endothelial cells Rare ➢ But, most common hepatic sarcoma Etiologic associations ➢ Vinyl chloride ➢ Arsenical compounds ➢ Radiation therapy ➢ Anabolic steroids

Angiosarcoma on CT and MR showing central hemorrhage that is fluid attenuation on CT and high signal on T1-and T2-weighted MR

Angiosarcoma • •

More common in men, 3:1 Clinical presentation ➢ Variable ➢ Hemoperitoneum ➢ Metastasis in 60%, spleen, lung

Figure 2-2-15

Angiosarcoma - Imaging Features • • •

•

Solitary or multifocal Evidence of hemorrhage Enhancement ➢ Peripheral or heterogeneous Metastatic disease ➢ Spleen and lung

Angiosarcoma [Figure 2- 2-14]

Epithelioid Hemangioendothelioma [Figure 2- 2-15]

•

•

Rare malignancy of endothelial origin ➢ Contains dense fibrous stroma Imaging ➢ Multifocal, lesions coalesce over time ➢ Peripheral enhancement ➢ Central fibrous stroma ➢ Retracted liver capsule ➢ May calcify

Malignant Hepatic Neoplasms

Epithelioid hemangioendothelioma showing multifocality and capsular contraction 280

Gastrointestinal Radiology

Approach to the incidentally discovered liver mass? •

• • •

•

•

•

Does the mass meet the criteria for a benign, nonsurgical lesion? ➢ Bile duct cyst ➢ Hemangioma ➢ FNH Are there equivocal features of hemangioma or FNH on CT and/or MR? ➢ Consider scintigraphy Is there clinical history that will suggest the etiology? ➢ History of primary malignancy ➢ History of chronic liver disease ➢ History or exogenous estrogens Are there features that suggest HCC? ➢ Capsule ➢ Fat ➢ Vascular invasion ➢ Mosaic pattern Are there features that suggest hepatocellular adenoma? ➢ Clinical/demographic history ➢ Capsule ➢ Fat ➢ Diffuse low attenuation ➢ Hemorrhage Are there features that suggest cholangiocarcinoma? ➢ No capsule ➢ Ill-defined margins ➢ Biliary dilatation ➢ Capsular contraction If the answer is NO to all the above, and the finding is a small, focal area of arterial enhancment on MDCT, the possibilities are: ➢ Hemangioma ➢ FNH ➢ Small adenoma ➢ Small HCC ➢ Hypervascular met ➢ AVM ➢ THAD

Summary Hepatocellular Carcinoma • • •

Most common primary hepatic malignancy Strong association with chronic liver disease Variable imaging features ➢ Rapid enhancement ➢ Capsule ➢ Mosaic pattern ➢ Focal fat ➢ Vascular invasion

Summary Fibrolamellar Carcinoma • • • •

Variant of HCC Young patients Otherwise normal liver Key features ➢ Lobular tumor ➢ Central scar ➢ Heterogeneous mass

Summary Intrahepatic Cholangiocarcinoma • • •

Arise from bile duct epithelium Uncommon Key features ➢ Delayed central enhancement

Gastrointestinal Radiology

281

Malignant Hepatic Neoplasms

➢ Biliary dilatation peripheral to tumor ➢ Capsular contraction

Summary Angiosarcoma • •

Rare Key features ➢ Evidence of hemorrhage ➢ Splenic metastasis at presentation

References

Hepatocellular Carcinoma 1. Winter TC, 3rd, Takayasu K, Muramatsu Y, et al. Early advanced hepatocellular carcinoma: evaluation of CT and MR appearance with pathologic correlation. Radiology 1994; 192:379-387. 2. Takayasu K, Furukawa H, Wakao F, et al. CT diagnosis of early hepatocellular carcinoma: sensitivity, findings, and CT-pathologic correlation. AJR Am J Roentgenol 1995; 164:885-890. 3. Baron RL, Oliver JH, 3rd, Dodd GD, 3rd, Nalesnik M, Holbert BL, Carr B. Hepatocellular carcinoma: evaluation with biphasic, contrast-enhanced, helical CT. Radiology 1996; 199:505-511. 4. Kelekis NL, Semelka RC, Worawattanakul S, et al. Hepatocellular carcinoma in North America: a multiinstitutional study of appearance on T1-weighted, T2-weighted, and serial gadolinium-enhanced gradientecho images. AJR Am J Roentgenol 1998; 170:1005-1013. 5. Loyer EM, Chin H, DuBrow RA, David CL, Eftekhari F, Charnsangavej C. Hepatocellular carcinoma and intrahepatic peripheral cholangiocarcinoma: enhancement patterns with quadruple phase helical CT--a comparative study. Radiology 1999; 212:866-875. 6. Winston CB, Schwartz LH, Fong Y, Blumgart LH, Panicek DM. Hepatocellular carcinoma: MR imaging findings in cirrhotic livers and noncirrhotic livers. Radiology 1999; 210:75-79. 7. Ward J, Guthrie JA, Scott DJ, et al. Hepatocellular carcinoma in the cirrhotic liver: double-contrast MR imaging for diagnosis. Radiology 2000; 216:154-162. 8. Murakami T, Kim T, Takamura M, et al. Hypervascular hepatocellular carcinoma: detection with double arterial phase multi-detector row helical CT. Radiology 2001; 218:763-767. 9. Brancatelli G, Federle MP, Grazioli L, Carr BI. Hepatocellular carcinoma in noncirrhotic liver: CT, clinical, and pathologic findings in 39 U.S. residents. Radiology 2002; 222:89-94. 10. Iannaccone R, Laghi A, Catalano C, et al. Hepatocellular carcinoma: role of unenhanced and delayed phase multidetector row helical CT in patients with cirrhosis. Radiology 2005; 234:460-467.

Fibrolamellar Carcinoma 1. Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 1999; 213:352-361. 2. McLarney JK, Rucker PT, Bender GN, Goodman ZD, Kashitani N, Ros PR. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. RadioGraphics 1999; 19:453-471. 3. Soyer P, Roche A, Levesque M, Legmann P. CT of fibrolamellar hepatocellular carcinoma. J Comput Assist Tomogr 1991; 15:533-538. 4. Titelbaum DS, Hatabu H, Schiebler ML, Kressel HY, Burke DR, Saul SH. Fibrolamellar hepatocellular carcinoma: MR appearance. J Comput Assist Tomogr 1988; 12:588-591. 5. Titelbaum DS, Burke DR, Meranze SG, Saul SH. Fibrolamellar hepatocellular carcinoma: pitfalls in nonoperative diagnosis. Radiology 1988; 167:25-30. 6. Blachar A, Federle MP, Ferris JV, et al. Radiologists' performance in the diagnosis of liver tumors with central scars by using specific CT criteria. Radiology 2002; 223:532-539.

Intrahepatic Cholangiocarcinoma 1. Choi BI, Park JH, Kim YI, et al. Peripheral cholangiocarcinoma and clonorchiasis: CT findings. Radiology 1988; 169:149-153. 2. Tani K, Kubota Y, Yamaguchi T, et al. MR imaging of peripheral cholangiocarcinoma. J Comput Assist Tomogr 1991; 15:975-978. 3. Kim TK, Choi BI, Han JK, Jang HJ, Cho SG, Han MC. Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings. Radiology 1997; 204:539-543. 4. Loyer EM, Chin H, DuBrow RA, David CL, Eftekhari F, Charnsangavej C. Hepatocellular carcinoma and intrahepatic peripheral cholangiocarcinoma: enhancement patterns with quadruple phase helical CT--a comparative study. Radiology 1999; 212:866-875. 5. Zhang Y, Uchida M, Abe T, Nishimura H, Hayabuchi N, Nakashima Y. Intrahepatic peripheral cholangiocarcinoma: comparison of dynamic CT and dynamic MRI. J Comput Assist Tomogr 1999; 23:670-677. Malignant Hepatic Neoplasms

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6.

7. 8.

Vitellas KM, Keogan MT, Freed KS, et al. Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. RadioGraphics 2000; 20:959-975; quiz 1108-1109, 1112. Han JK, Choi BI, Kim AY, et al. Cholangiocarcinoma: pictorial essay of CT and cholangiographic findings. RadioGraphics 2002; 22:173-187. Levy AD. Malignant liver tumors. Clin Liver Dis 2002; 6:147-164.

Angiosarcoma 1. Peterson MS, Baron RL, Rankin SC. Hepatic angiosarcoma: findings on multiphasic contrast-enhanced helical CT do not mimic hepatic hemangioma. AJR Am J Roentgenol 2000; 175:165-170. 2. Koyama T, Fletcher JG, Johnson CD, Kuo MS, Notohara K, Burgart LJ. Primary hepatic angiosarcoma: findings at CT and MR imaging. Radiology 2002; 222:667-673. 3. Levy AD. Malignant liver tumors. Clin Liver Dis 2002; 6:147-164. 4. Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the Spleen: Imaging Characteristics in 12 Patients. Radiology 2005. In Press Epithelioid Hemangioendothelioma 1. Ishak KG, Sesterhenn IA, Goodman ZD, Rabin L, Stromeyer FW. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol 1984; 15:839-852. 2. Miller WJ, Dodd GD, Federle MP, Baron RL. Epithelioid hemangioendothelioma of the liver: imaging findings with pathologic correlation [see comments]. AJR Am J Roentgenol 1992; 159:53-57. 3. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer 1999; 85:562-582. 4. Mermuys K, Vanhoenacker PK, Roskams T, D'Haenens P, Van Hoe L. Epithelioid hemangioendothelioma of the liver: radiologic-pathologic correlation. Abdom Imaging 2004; 29:221-223.

Gastrointestinal Radiology

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Malignant Hepatic Neoplasms

Hepatic Infections

Angela D. Levy, COL, MC, USA Hepatic Infections • • • • • •

Pyogenic Abscess Amebic Abscess Echinococcal Infections Schistosomiasis Clonorchiasis Infections in the Immunocompromised host ➢ Candidasis ➢ Pneumocystis

Pyogenic Hepatic Abscess • •

Polymicrobial infections Variable clinical presentation ➢ Septicemia, pain, fever, indolent symptoms ➢ Tender hepatomegaly

Pyogenic Hepatic Abscess: Pathogenesis •

• • • • •

Biliary ➢ MOST COMMON ETIOLOGY ➢ Cholangitis, biliary obstruction ➢ Multiple and bilateral Portal vein ➢ Pylephlebitis ➢ Solitary, 65% right lobe Hepatic artery Direct extension Traumatic-blunt or penetrating trauma Necrotic tumor

Pyogenic Hepatic Abscess • •

Mortality rate <10% Effectively treated with percutaneous drainage ➢ 8% failure rate ➢ 8% recurrence rate

Pyogenic Hepatic Abscess: Sonography •

• •

•

Variable echogenicity ➢ Anechoic (50%) ➢ Hyperechoic (25%) ➢ Hypoechoic (25%) Ill-defined margins Internal character ➢ Irregular wall ➢ Septations ➢ Fluid-fluid levels ➢ Debris ➢ Reverberation artifact if gas is present Posterior acoustic enhancement

Hepatic Infection

284

Gastrointestinal Radiology

Pyogenic Hepatic Abscess: CT •

•

•

Figure 2-3-1

Singe best imaging method ➢ Sensitivity 97% ➢ Intravenous contrast essential Hypoattenuating ➢ 0 to 45 H.U. Helpful CT features ➢ Rim-enhancement ➢ Transition zone ➢ Cluster sign ➢ Gas (<20%) ➢ Air/fluid or debris/fluid level ❖ Suspect GI communication

Pyogenic hepatic abscess shows cluster sign and transition zone

Pyogenic Hepatic Abscess CT

Pyogenic Hepatic Abscess: Cluster Sign

Pyogenic Hepatic Abscess: Cluster Sign/ Transition zone [Figure 2-3-1]

Pyogenic Hepatic Abscess: Intrahepatic Gas

Pyogenic Hepatic Abscess: Imaging Guided Drainage • •

•

Unilocular and liquefied Multilocular or multiple ➢ Multiple catheters Multiple, small (<1 cm) ➢ Aspiration for diagnosis ➢ Treatment: antibiotics or aspiration + antibiotics

Amebic Liver Abscess • •

Most common extra-intestinal manifestation of amebiasis ➢ 3%-7% of patients with amebic infection ➢ Fever, RUQ pain Route of spread ➢ Portal venous (most common) ➢ Lymphatic ➢ Direct extension from colon

Amebic Abscess: Sonography •

• •

Round or oval shape ➢ Absent wall echoes ➢ Homogenous low level internal echoes Location ➢ Near or touching the liver capsule ➢ 85% solitary ➢ 72% right lobe Enhanced through transmission

Figure 2-3-2

Amebic Abscess [Figure 2-3-2] Amebic Abscess: CT •

• •

Enhancing wall (3-15 mm) ➢ Round ➢ Smooth or irregular ➢ Peripheral zone of edema Low attenuation/complex fluid ➢ Septations ➢ Fluid/debris level Extrahepatic extension

Gastrointestinal Radiology

Amebic abscess

285

Hepatic Infection

Amebic Abscess

Figure 2-3-3

Amebic vs. Pyogenic Abscess • •

Cannot reliably differentiate by imaging Patients with amebic abscess ➢ More likely to have hepatomegaly and diarrhea ➢ History of recent travel or inhabitant of high prevalence areas ➢ Serologic tests positive in >90%

Amebic Abscess: Therapy • • •

Medical therapy Percutaneous biopsy of abscess wall ➢ If serology does not confirm diagnosis and clinical suspicion is high Percutaneous drainage if ➢ Large, >5 cm abscess ➢ Left lobe ➢ Biliary communication ➢ Pregnancy ➢ Perforation ➢ Poor response to drug therapy

Worldwide distribution of E. granulosus

Figure 2-3-4

Amebic Abscess / Pyogenic Abscess

Echinococcus: E. granulosus and E. multilocularis •

•

Worldwide distribution of E. multilocularis Nomenclature ➢ Hydatidosis is the infection by the larval tapeworm of the genus Echinococcus Endemic worldwide ➢ Humans accidental host ➢ Infection usually acquired during childhood

E. granulosus [Figure 2-3-3]

Figure 2-3-5

E. multilocularis [Figure 2-3-4] Echinococcus E. granulosus and E. multilocularis •

•

Symptoms occur during adulthood ➢ Cyst enlargement ➢ Erosion of cyst into peritoneal or pleural cavity ➢ Development of biliary communication Serology confirms diagnosis ➢ Positive >80% of cases

Echinococcus [Figure 2-3-5]

Echinococcus: E. granulosus

Echinococcus: E. multilocularis

Hepatic Infection

Echinococcus lifecycle

286

Gastrointestinal Radiology

E. granulosus: Imaging Features •

•

Figure 2-3-6

Unilocular or multilocular cyst ➢ Calcification in cyst wall ➢ Internal debris (hydatid sand) Complex cyst ➢ Internal daughter cysts ➢ Undulating membrane (water lily sign) ➢ Fibrous and avascular walls and membranes ❖ Low MR signal ❖ No enhancement

Daughter cysts of E. granulosus

E. granulosus

Figure 2-3-7

E. granulosus: Daughter cysts [Figure 2-3-6]

E. granulosus: Water Lily Sign [Figures 2-3-7 and 2-3-8] E. granulosus: Complications and Treatment •

•

Cyst rupture ➢ Anaphylaxis ➢ Biliary tract, peritoneal cavity ➢ Pleural, pericardial cavity Treatment ➢ Surgical excision ➢ Laparoscopic excision ➢ Percutaneous drainage + sclerosing scolicidal agents

Laminated membranes and water lily sign of E. granulosus

Figure 2-3-8

E. multilocularis: Pathologic Features • • •

Alveolar hydatid disease Propagation by external budding Invade surrounding tissue ➢ Infiltrative mass ➢ No limiting host tissue ➢ Resembles neoplasm

E. multilocularis: Imaging Features •

•

Ultrasound ➢ Echogenic ➢ Single or multiple ➢ Ill-defined walls ➢ Partially calcified CT ➢ Geographic ➢ Infiltrating lesions ➢ Amorphous calcification

Water lily sign of E. granulosus

Figure 2-3-9

E. multilocularis [Figure 2-3-9]

E. multilocularis Gastrointestinal Radiology

287

Hepatic Infection

Schistosomasis (Bilharziasis) •

• •

Figure 2-3-10

Trematode (fluke) ➢ S. Japonicum, S. mansoni, S. hematobium Humans are definitive host ➢ Mature in the portal venules Migrate to deposit eggs ➢ Intestine (S. japonicum, S. mansoni) ➢ Bladder (S. hematobium)

Schistosomasis: S. japonicum Schistosomasis: S. mansoni

Schistosomasis: S. hematobium

Lifecycle of Schistosomiasis

Schistosomasis [Figure 2-3-10]

Figure 2-3-11

Schistosomasis [Figure 2-3-11] • •

• •

Granulomatous inflammation Fibrosis ➢ Symmers' fibrosis ➢ Turtle back liver Progressive portal vein occlusion Presinusoidal portal hypertension

Schistosomasis: Imaging Features • •

S. japonicum ➢ Hepatic calcification ➢ “Turtle back” configuration S. mansoni ➢ Low attenuation, rounded foci ➢ Low attenuation, linear branching bands

Symmers' fibrosis

Schistosomiasis japonicum [Figure 2-3-12]

Figure 2-3-12

Biliary Parasites •

Parasites that invade bile ducts ➢ Trematodes ❖ Clonorchis sinensis ❖ Fasciola gigantica, Fasciola hepatica ❖ Opisthorchis viverrini ❖ Opisthorchis felineus ➢ Nematodes ❖ Ascariasis lumbricoides ➢ Cestodes ❖ Taenia saginata

Hepatic Infection

Schistosomiasis japonicum on CT

288

Gastrointestinal Radiology

Clonorchis sinensis [Figures 2-3-13 and 2-3-14] •

•

Figure 2-3-13

Peripheral intrahepatic bile ducts ➢ Dilatation of small intrahepatic ducts ➢ Periductal fibrosis Complications ➢ Cholangitis ➢ Cholangiohepatitis ➢ Liver abscess ➢ Cholangiocarcinoma

Figure 2-3-14

Lifecycle of Clonorchis sinensis

Fasciola Hepatica

Cholangiogram shows a filling defect, peripheral intrahepatic strictures, and dilatation due to infestation of Clonorchis sinensis

Hepatic Infections in the Immunocompromised Host • • • •

Candidiasis Pneumocystis Carinii Herpes Simplex Virus Liver Abscess ➢ Pyogenic ➢ Multiorganism

Disseminated Candidiasis • •

• •

Synonym: hepatosplenic candidiasis Pathogenesis ➢ Prolonged neutropenia ➢ Mucosal damage to the GI tract ➢ Local invasion of candida with entry into the hepatosplenic circulation Clinical manifestations ➢ Neutropenic with fever ➢ Return of neutrophil count Organ Involvement ➢ Spleen 94%, liver 75%, kidney 69%

Hepatosplenic Candidiasis: Pathology • • • •

Necrosis with minimal inflammation Microabscesses with severe inflammation Collagen formation/fibrosis Granuloma formation

Gastrointestinal Radiology

289

Hepatic Infection

Hepatosplenic Candidiasis: Sonography [Figures 2-3-15] •

• •

•

Type 1 lesion ➢ “wheel-within-a-wheel” Type 2 lesion ➢ “bull’s-eye” Type 3 lesion-most common ➢ hypoechoic nodule Type 4 lesion ➢ hyperechoic nodule

Figure 2-3-15 I

III

II

IV Figure 2-3-16

Hepatosplenic Candidiasis: CT Features [Figure 2-3-16] • • •

Concentric rings Hypodense nodules Punctate calcification

• • • •

Low T1, high T2 Fat-suppressed T2 improves detection Gd-FLASH most sensitive Splenic gamna-gandy bodies false positive T1

Hepatosplenic Candidiasis: MR Features

Hepatic Infection

Hepatic candidiasis on MDCT

290

Gastrointestinal Radiology

Hepatosplenic Candidiasis: Imaging Management • •

• • •

High index of suspicion Imaging during neutropenia is often negative ➢ Follow up studies if clinical suspicion high and prophylactic therapy contraindicated Prophylactic therapy Biopsy Lesions change morphology with healing

Pneumocystis jiroveci • • •

Previously classified as Pneumocystis carinii Now considered a fungus Opportunistic infection ➢ AIDS ➢ Organ transplant recipients

Pneumocystis jiroveci (carinii): Imaging Features Figure 2-3-17] •

•

Sonography ➢ Nonshadowing hyperechoic nodules ➢ Shadowing echogenic clumps of calcification CT scan ➢ Hypodense nodules with progressive calcification ➢ Renal and lymph node calcification

Summary: Pyogenic Abscess • • •

Transition zone Cluster sign Percutaneous drainage

•

Cannot reliably distinguish from pyogenic abscess on imaging Percutaneous biopsy if necessary Viable organisms in wall Drainage if necessary

Figure 2-3-17

Summary: Amebic Abscess • • •

Summary: Echinococcus • •

E. granulosus ➢ Daughter cysts ➢ Water-lily sign ➢ Rim-like calcification E. multilocularis ➢ Infiltrating mass ➢ Calcification

Sonogram and CT of disseminated pneumocystis

Summary: Immunocompromised Hosts •

•

Candidiasis ➢ Neutropenics ➢ Imaging negative during neutropenia ➢ Imaging positive during WBC rebound Pneumocystis carinii ➢ Hyperechoic nodules ➢ +/- shadowing ➢ Hypodense on CT with progressive calcification ➢ Renal and lymph node calcification

Gastrointestinal Radiology

291

Hepatic Infection

References

Pyogenic and Amebic Hepatic Abscess 1. Halvorsen RA, Korobkin M, Foster WL, Silverman PM, Thompson WM. The variable CT appearance of hepatic abscesses. AJR Am J Roentgenol 1984; 142:941-946. 2. Mathieu D, Vasile N, Fagniez PL, Segui S, Grably D, Larde D. Dynamic CT features of hepatic abscesses. Radiology 1985; 154:749-752. 3. Jeffrey RB, Jr., Tolentino CS, Chang FC, Federle MP. CT of small pyogenic hepatic abscesses: the cluster sign. AJR Am J Roentgenol 1988; 151:487-489. 4. Radin DR, Ralls PW, Colletti PM, Halls JM. CT of amebic liver abscess. AJR Am J Roentgenol 1988; 150:12971301. 5. Juimo AG, Gervez F, Angwafo FF. Extraintestinal amebiasis. Radiology 1992; 182:181-183. 6. Gabata T, Kadoya M, Matsui O, et al. Dynamic CT of hepatic abscesses: significance of transient segmental enhancement. AJR Am J Roentgenol 2001; 176:675-679. 7. Mortele KJ, Segatto E, Ros PR. The infected liver: radiologic-pathologic correlation. Radiographics 2004; 24:937955. 8. Yang DM, Kim HN, Kang JH, Seo TS, Park CH, Kim HS. Complications of pyogenic hepatic abscess: computed tomography and clinical features. J Comput Assist Tomogr 2004; 28:311-317. Hydatid Disease (Echinococcus) 1. de Diego Choliz J, Lecumberri Olaverri FJ, Franquet Casas T, Ostiz Zubieta S. Computed tomography in hepatic echinococcosis. AJR Am J Roentgenol 1982; 139:699-702. 2. Pandolfo I, Blandino G, Scribano E, Longo M, Certo A, Chirico G. CT findings in hepatic involvement by Echinococcus granulosus. J Comput Assist Tomogr 1984; 8:839-845. 3. Claudon M, Bessieres M, Regent D, et al. Alveolar echinococcosis of the liver: MR findings. J Comput Assist Tomogr 1990; 14:608-614. 4. Acunas B, Rozanes I, Acunas G, Celik L, Alper A, Gokmen E. Hydatid cyst of the liver: identification of detached cyst lining on CT scans obtained after cyst puncture. AJR Am J Roentgenol 1991; 156:751-752. 5. Taourel P, Marty-Ane B, Charasset S, Mattei M, Devred P, Bruel JM. Hydatid cyst of the liver: comparison of CT and MRI. J Comput Assist Tomogr 1993; 17:80-85. 6. Ustunsoz B, Akhan O, Kamiloglu MA, Somuncu I, Ugurel MS, Cetiner S. Percutaneous treatment of hydatid cysts of the liver: long-term results. AJR Am J Roentgenol 1999; 172:91-96. Schistosomiasis 1. Araki T, Hayakawa K, Okada J, Hayashi S, Uchiyama G, Yamada K. Hepatic schistosomiasis japonica identified by CT. Radiology 1985; 157:757-760. 2. Fataar S, Bassiony H, Satyanath S, et al. CT of hepatic schistosomiasis mansoni. AJR Am J Roentgenol 1985; 145:63-66. 3. Monzawa S, Uchiyama G, Ohtomo K, Araki T. Schistosomiasis japonica of the liver: contrast-enhanced CT findings in 113 patients. AJR Am J Roentgenol 1993; 161:323-327. 4. Lee RC, Chiang JH, Chou YH, et al. Intestinal schistosomiasis japonica: CT-pathologic correlation. Radiology 1994; 193:539-542. 5. Willemsen UF, Pfluger T, Zoller WG, Kueffer G, Hahn K. MRI of hepatic schistosomiasis mansoni. J Comput Assist Tomogr 1995; 19:811-813. 6. Cheung H, Lai YM, Loke TK, et al. The imaging diagnosis of hepatic schistosomiasis japonicum sequelae. Clin Radiol 1996; 51:51-55. 7. Mortele KJ, Ros PR. Imaging of diffuse liver disease. Semin Liver Dis 2001; 21:195-212.

Hepatosplenic Candidiasis 1. Ho B, Cooperberg PL, Li DK, Mack L, Naiman SC, Grossman L. Ultrasonography and computed tomography of hepatic candidiasis in immunosuppressed patients. J Ultrasound Med 1982; 1:157-159. 2. Shirkhoda A. CT findings in hepatosplenic and renal candidiasis. J Comput Assist Tomogr 1987; 11:795-798. 3. Pastakia B, Shawker TH, Thaler M, O'Leary T, Pizzo PA. Hepatosplenic candidiasis: wheels within wheels. Radiology 1988; 166:417-421. 4. Thaler M, Pastakia B, Shawker TH, O'Leary T, Pizzo PA. Hepatic candidiasis in cancer patients: the evolving picture of the syndrome. Ann Intern Med 1988; 108:88-100. 5. Gorg C, Weide R, Schwerk WB, Koppler H, Havemann K. Ultrasound evaluation of hepatic and splenic microabscesses in the immunocompromised patient: sonographic patterns, differential diagnosis, and follow-up. J Clin Ultrasound 1994; 22:525-529. 6. Rudolph J, Rodenwaldt J, Ruhnke M, Hamm B, Kopka L. Unusual enhancement pattern of liver lesions in hepatosplenic candidiasis. Acta Radiol 2004; 45:499-503. Hepatic Infection

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Gastrointestinal Radiology

Imaging of Chronic Liver Disease Angela D. Levy, COL, MC, USA Chronic Liver Disease: Objectives • • • •

Cirrhosis Steatosis and steatohepatitis Budd-Chiari Disorders of Iron Deposition ➢ Hemosiderosis ➢ Hemochromatosis

Cirrhosis: Definition •

Endpoint of chronic liver disease

• • • • •

Hepatocyte injury and loss Fibrosis Nodule formation Architectural reorganization Nodules ➢ Micronodular (<3mm) ➢ Macronodular (>3mm) ➢ Mixed Liver volume ➢ Early, hepatomegaly from inflammation ➢ Late, small liver from fibrosis

Cirrhosis: Pathology

•

Cirrhosis: Segmental Alterations in Volume • •

•

•

Common feature Not fully understood ➢ Alteration in blood flow Atrophy ➢ Segments IV, VI, VIII Hypertrophy ➢ Segments I, II, III

Cirrhosis: Imaging • •

Cannot assess severity Role of imaging ➢ Assess disease complications ➢ Evaluation of portal hypertension ➢ HCC surveillance

Cirrhosis: Sonography •

• • •

Fibrosis ➢ Increased parenchymal echogenicity ➢ Decreased penetration of the ultrasound beam ➢ Poor visualization of hepatic vasculature ➢ Loss of triphasic hepatic vein doppler ➢ Increased pulsatility of portal vein doppler Nodules Volume redistribution Portal hypertension

Gastrointestinal Radiology

293

Imaging of Chronic Liver Disease

Cirrhosis: CT •

•

•

Figure 2-4-1

Morphologic changes ➢ Volume redistribution ➢ Nodules Fibrosis ➢ Prominent porta and fissures ➢ Focal confluent fibrosis ➢ Decreased parenchymal enhancement Mesenteric changes ➢ Lymphadenopathy Cirrhosis with fibrosis, altered enhancement and nodules on CT ➢ Increased mesenteric attenuation

Cirrhosis: Volume redistribution and nodules Cirrhosis: Volume redistribution

Figure 2-4-2

Cirrhosis: Fibrosis, altered enhancement, nodules [Figure 2-4-1] Cirrhosis: Mesenteric Changes, Adenopathy [Figure 2-4-2] Cirrhosis: Nodules •

•

•

Chronic hepatitis B cirrhosis with nodules, mesenteric changes, and adenopathy

Regenerative nodule ➢ Benign ➢ Proliferation of hepatocytes ➢ Precursor to dysplastic nodule and HCC Dysplastic nodule ➢ Premalignant Hepatocellular carcinoma

Figure 2-4-4

Cirrhosis: Regenerative Nodule • • • •

Benign proliferation of hepatocytes Hemosiderin deposition ➢ "Siderotic nodule" ➢ Noncontrast scans helpful for detection CT ➢ Isodense with and without contrast ➢ Hyperdense on noncontrast (siderotic nodule) MR ➢ Dark T1, T2, gradient echo ➢ Bright T1 (rare), Dark T2 ➢ Best seen on GRE and T2 images

Cirrhosis: Regenerative Nodules on CT [Figures 2-4-3 and 2-4-4]

Figure 2-4-3

Cirrhosis with high attenuation regenerating nodules (siderotic nodules) on noncontrast CT

Imaging of Chronic Liver Disease

294

Cirrhosis with regenerating nodules on CT

Gastrointestinal Radiology

Cirrhosis: Regenerative Nodules and HCC on MR Cirrhosis: Regenerative Nodules on MR [Figure 2-4-5]

Figure 2-4-5

Cirrhosis: Dysplastic Nodule •

• • •

Premalignant nodule ➢ Nodule with histologic evidence of dysplasia Very common ➢ Most undetectable on CT and MR ➢ Rarely seen on preoperative imaging Imaging appearance variable Detection of malignant transformation depends upon evidence of angiogenesis ➢ Arterial enhancment ➢ Nodule in a nodule ➢ MR bright T1 with central dark signal ➢ MR dark T2 with central high signal

Cirrhosis: Dysplastic Nodule on MR

Cirrhosis: Hepatocellular Carcinoma [Figure 2-4-6] •

• •

Incidence in cirrhosis ➢ 20% of hepatitis B and C cirrhosis ➢ 10% of alcoholic cirrhosis CT and MR equally accurate ➢ 70%-75% of patients ➢ 35%-40% of lesions Late arterial phase imaging is key ➢ Maximum contrast volume ➢ 4 to 5 ml/sec injection rate

Cirrhosis with regenerating nodules on T2 MR and gross photography

Figure 2-4-6

Cirrhosis: HCC Detection •

False positives ➢ Transient hepatic attenuation (intensity) difference (THAD/THID) ➢ Focal confluent fibrosis (look for associated atrophy) ➢ Enhancing regenerative nodule ➢ Flash filling hemangioma ➢ Early enhancing pseudolesions (arterioportal shunting)

Cirrhosis: Transient Hepatic Attenuation (Intensity) Difference •

•

Causes ➢ Portal vein obstruction ➢ Hepatic venous outflow obstruction ➢ Adjacent benign or malignant mass ➢ Arterioportal shunting ➢ Aberrant venous drainage Imaging features ➢ Typical locations ❖ Subcapsular ❖ Adjacent to falciform ligament ➢ No mass effect ➢ Straight margins ➢ Wedge shape

Gastrointestinal Radiology

HCC in cirrhosis

295

Imaging of Chronic Liver Disease

Cirrhosis: Focal Confluent Fibrosis •

• •

Massive areas of fibrosis ➢ Present in up to 30% of cirrhotic livers Typical location ➢ Anterior segment right lobe ➢ Medial segment left lobe Imaging ➢ Focal mass ➢ Wedge shape, radiating from porta hepatis ➢ Capsular retraction ➢ Low density on noncontrast CT ➢ Isodense with contrast or irregular enhancement ➢ MR: low signal T1, high signal T2

Figure 2-4-7

Cirrhosis: Hemangioma

Primary Biliary Cirrhosis • •

•

Primary biliary cirrhosis

Chronic cholestasis Unknown etiology ➢ Probably immune mediated Middle-aged women ➢ Median age 50 ➢ Female to male ratio 9:1

Primary Biliary Cirrhosis: CT [Figure 2-4-7] • • • •

•

Global or segmental atrophy Nodules Fibrosis ➢ Lace-like pattern ➢ Segmental ➢ Focal confluent Portal hypertension ➢ Often present before morphologic changes At risk for HCC

Fatty Liver Diseases •

•

Steatosis ➢ Alcohol-associated ➢ Nonalcoholic fatty liver disease (NAFLD) Steatohepatitis ➢ Alcoholic steatohepatitis ➢ Nonalcoholic steatohepatitis (NASH)

Steatosis •

• •

Nomenclature ➢ Fatty infiltration, fatty change, nonalcoholic fatty liver disease (NAFLD) Very common Pathogenesis ➢ Abnormal fatty acid metabolism ➢ Insulin/glucagon imbalance ➢ Shift to lipogenesis

Imaging of Chronic Liver Disease

296

Gastrointestinal Radiology

Steatosis: Etiology

• •

ETOH Obesity, diabetes Malnutrition Parenteral nutrition Hepatitis, hepatotoxins, chemotherapy, hyperlipidemia, drugs Malabsorption syndromes Idiopathic

• • • •

Asymptomatic Mild RUQ pain Mild hepatomegaly and/or tenderness on exam Mild transaminase elevation

•

Diffuse ➢ Echogenic parenchyma ➢ Poor visualization hepatic vasculature ➢ Absorption of sound Focal ➢ Focal fat ➢ Focal sparing

• • • • •

Figure 2-4-8

Sonography of diffuse fatty infiltration

Steatosis and Steatohepatitis: Clinical Features

Figure 2-4-9

Steatosis: Sonography

•

Diffuse Steatosis: Sonography [Figure 2-4-8]

Focal Steatosis: Sonography

Focal Fatty Sparing: Sonography

Focal fatty infiltration

Steatosis: CT • •

•

Normal liver noncontrast CT ➢ 30 to 60 HU ➢ 8 to 10 HU > spleen Fatty liver noncontrast CT ➢ 10 HU < spleen Fatty liver contrast CT ➢ 25 HU < spleen

Steatosis in Celiac Disease

Nonalcoholic Steatohepatitis (NASH): CT NASH with Cirrhosis: CT

Focal Steatosis: CT [Figure 2-4-9] •

• •

Features of focal fat ➢ No mass effect ➢ Straight line margin ➢ No contour abnormality Often transient Common locations ➢ Falciform ligament ➢ Subcapsular ➢ Adjacent to porta hepatis ➢ Adjacent to gallbladder fossa

Gastrointestinal Radiology

297

Imaging of Chronic Liver Disease

Focal Steatosis: Common Locations

Figure 2-4-10

Focal Steatosis vs. Focal Sparing

Focal Sparing [Figure 2-4-10]

Focal Steatosis: Transient Steatosis: MR • •

Conventional spin echo typically insensitive to fat deposition Chemical shift imaging ➢ Fat and water signal additive in-phase ➢ Fat signal subtracted from water signal out-of-phase

Steatosis: 1.5T MR [Figure 2-4-11]

Focal fatty sparing

Figure 2-4-11

Steatosis: MR [Figure 2-4-12]

Multifocal Steatosis: Pseudometastatic Disease Budd-Chiari Syndrome • • •

Hepatic venous outflow obstruction Primary ➢ Membranous (web) obstruction of hepatic veins Secondary ➢ Hypercoaguable states, infections, neoplasms, trauma

Budd-Chiari Syndrome: Clinical • •

Acute fulminant disease ➢ Total obstruction ➢ Rare Subacute/Chronic ➢ Vague illness, 6 months duration ➢ Ascites ➢ Triad of hepatomegaly, ascites, pain

Figure 2-4-12

Budd-Chiari Syndrome: Pathophysiology • • • •

Sinusoidal dilatation Increase sinusoidal pressure Centrolobular hepatocyte necrosis Centrolobular fibrosis ➢ Lobular collapse ➢ Nodular regeneration

Budd-Chiari Syndrome: Pathology •

•

1.5 T MR fat and water proton signal intensity

Focal fat on in-phase images and out-of-phase images

Acute ➢ Hepatomegaly ➢ Sinusoidal dilatation ➢ Hemorrhagic necrosis Chronic ➢ Fibrosis ➢ Cirrhosis

Imaging of Chronic Liver Disease

298

Gastrointestinal Radiology

Budd Chiari Syndrome: Imaging •

•

Figure 2-4-13

Vascular changes ➢ Hepatic vein stenosis ➢ Intravascular thrombus ➢ Web-like stenosis or narrowed IVC ➢ Intrahepatic collaterals Parenchymal changes ➢ Nonvisible hepatic veins ➢ Cirrhosis ➢ Nodular regenerative hyperplasia

Hepatic Venous Waveforms [Figure 2-4-13] Budd-Chiari Syndrome: Enhancement Patterns •

•

Noncontrast ➢ Heterogeneous hypodensity ❖ Hepatic parenchymal congestion ➢ Hyperdense thrombi Contrast enhancement ➢ Patchy enhancement ➢ Normal central hepatic, left lobe, and caudate lobe enhancement ➢ Late peripheral enhancement

Normal hepatic vein (upper image) and Budd Chiari (lower image)

Figure 2-4-14

Budd-Chiari Syndrome [Figure 2-4-14]

Budd Chiari Syndrome [Figure 2-4-15]

Budd Chiari Syndrome: MR [Figure 2-4-16]

• • •

Narrowed veins Intraluminal thrombus Collaterals

Budd Chiari

Figure 2-4-15

Figure 2-4-16

Budd Chiari

Budd-Chiari Gastrointestinal Radiology

299

Imaging of Chronic Liver Disease

Budd Chiari from IVC Web

Budd Chiari and Nodular Regenerative Hyperplasia Disorders of Iron Deposition •

Functional Classification ➢ Hemosiderosis ❖ Iron accumulation in the reticuloendothelial system ❖ Iron in the liver with no organ damage ➢ Hemochromatosis ❖ Iron in hepatocytes with eventual fibrosis and cirrhosis ❖ Two types Hereditary hemochromatosis Secondary hemochromatosis ❖ High risk for HCC

Hemosiderosis in Sickle Cell Anemia [Figure 2-4-17] Hemochromatosis • •

Hereditary hemochromatosis ➢ Increased intestinal absorption of iron ➢ Iron predominantly within hepatocytes ➢ Highest incidence of cirrhosis and HCC (14%) Secondary hemochromatosis ➢ Multiple transfusions ➢ Iron predominantly in the reticuloendothelial system

Hereditary Hemochromatosis (HHC): Clinical Features

• • • • •

Hyperpigmentation Diabetes mellitus (bronze diabetes) Hepatomegaly Chondrocalcinosis/osteoarthritis Cardiomyopathy

Hereditary Hemochromatosis (HHC): Pathology

Figure 2-4-17

Hemosiderosis

Hemochromatosis: Increased CT Attenuation (75-135 HU)

Figure 2-4-18

Hereditary Hemochromatosis Increased Hepatic CT Attenuation •

Differential Diagnosis ➢ Iron deposition ➢ Glycogen storage disease ➢ Amiodarone ➢ Wilson’s disease ➢ Chronic arsenic poisoning

Hereditary Hemochromatosis

Hemochromatosis: MR •

•

T2*-gradient echo imaging is most sensitive ➢ Quantitate with liver:muscle ratio Decrease signal on T2-weighted images ➢ Hereditary = iron in liver and pancreas ➢ Secondary = iron in liver and spleen

Hereditary Hemochromatosis [Figure 2-4-18]

Imaging of Chronic Liver Disease

300

Gastrointestinal Radiology

Secondary Hemochromatosis Summary: Cirrhosis

•

Endpoint of chronic liver disease Nodules ➢ Regenerative ➢ Dysplastic ➢ HCC HCC false positives

• •

Steatosis Steatohepatitis

• • •

No mass effect Straight line margins Typical Locations ➢ Subcapsular ➢ Falciform ligament Chemical shift MR ➢ Signal loss on out-of-phase images

• •

Summary: Fatty Infiltration Summary: Focal Steatosis

•

Summary: Budd-Chiari Syndrome • • •

•

Hepatic venous outflow obstruction Sonography CT enhancement ➢ Early central ➢ Late peripheral MR

Summary: Disorders of Iron Overload • •

Hemosiderosis Hemochromatosis ➢ Hereditary ➢ Secondary

References

Cirrhosis 1. Baron RL, Peterson MS. From the RSNA refresher courses: screening the cirrhotic liver for hepatocellular carcinoma with CT and MR imaging: opportunities and pitfalls. Radiographics 2001; 21 Spec No:S117-132. 2. Brancatelli G, Baron RL, Peterson MS, Marsh W. Helical CT screening for hepatocellular carcinoma in patients with cirrhosis: frequency and causes of false-positive interpretation. AJR Am J Roentgenol 2003; 180(4):1007-1014. 3. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, Federle MP. Spectrum of imaging findings of the liver in end-stage cirrhosis: Part II, focal abnormalities. AJR Am J Roentgenol 1999; 173(5):1185-1192. 4. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, Federle MP. Spectrum of imaging findings of the liver in end-stage cirrhosis: part I, gross morphology and diffuse abnormalities. AJR Am J Roentgenol 1999; 173(4):1031-1036. 5. Hussain HK, Syed I, Nghiem HV, et al. T2-weighted MR imaging in the assessment of cirrhotic liver. Radiology 2004; 230(3):637-644. 6. Ohtomo K, Baron RL, Dodd GD, 3rd, et al. Confluent hepatic fibrosis in advanced cirrhosis: appearance at CT. Radiology 1993; 188(1):31-35. 7. Ohtomo K, Itai Y, Ohtomo Y, Shiga J, Iio M. Regenerating nodules of liver cirrhosis: MR imaging with pathologic correlation. AJR Am J Roentgenol 1990; 154(3):505-507. 8. Shimizu A, Ito K, Koike S, Fujita T, Shimizu K, Matsunaga N. Cirrhosis or chronic hepatitis: evaluation of small (
Gastrointestinal Radiology

301

Imaging of Chronic Liver Disease

Steatosis/Steatohepatitis 1. Yoshikawa J, Matsui O, Takashima T, et al. Focal fatty change of the liver adjacent to the falciform ligament: CT and sonographic findings in five surgically confirmed cases. AJR Am J Roentgenol 1987; 149:491-494. 2. Jain KA, McGahan JP. Spectrum of CT and sonographic appearance of fatty infiltration of the liver. Clin Imaging 1993; 17:162-168. 3. Siegelman ES. MR imaging of diffuse liver disease. Hepatic fat and iron. Magn Reson Imaging Clin N Am 1997; 5:347-365. 4. Jacobs JE, Birnbaum BA, Shapiro MA, et al. Diagnostic criteria for fatty infiltration of the liver on contrast-enhanced helical CT. AJR Am J Roentgenol 1998; 171:659-664. 5. Kroncke TJ, Taupitz M, Kivelitz D, et al. Multifocal nodular fatty infiltration of the liver mimicking metastatic disease on CT: imaging findings and diagnosis using MR imaging. Eur Radiol 2000; 10:1095-1100. 6. Brunt EM. Nonalcoholic steatohepatitis: definition and pathology. Semin Liver Dis 2001; 21:3-16. 7. Siegelman ES, Rosen MA. Imaging of hepatic steatosis. Semin Liver Dis 2001; 21:71-80. 8. Kemper J, Jung G, Poll LW, Jonkmanns C, Luthen R, Moedder U. CT and MRI findings of multifocal hepatic steatosis mimicking malignancy. Abdom Imaging 2002; 27:708-710. Budd-Chiari Syndrome 1. Noone TC, Semelka RC, Siegelman ES, et al. Budd-Chiari syndrome: spectrum of appearances of acute, subacute, and chronic disease with magnetic resonance imaging. J Magn Reson Imaging 2000; 11:44-50. 4. Brancatelli G, Federle MP, Grazioli L, Golfieri R, Lencioni R. Large regenerative nodules in Budd-Chiari syndrome and other vascular disorders of the liver: CT and MR imaging findings with clinicopathologic correlation. AJR Am J Roentgenol 2002; 178:877-883. 5. Maetani Y, Itoh K, Egawa H, et al. Benign hepatic nodules in Budd-Chiari syndrome: radiologic-pathologic correlation with emphasis on the central scar. AJR Am J Roentgenol 2002; 178:869-875. Disorders of Iron Deposition 1. Bonkovsky HL: Disorders of iron overload. In Bloomer JR, Goodman ZD, Ishak KG (eds): Clinical and pathologoical correlations in liver disease: approaching the next millennium. Washington, DC: Armed Forces Institute of Pathology, 1998 2. Gandon Y: Iron, liver, and MRI. http://www.radio.univ-rennes1.fr/Sources/EN/Hemo.html, 2001 3. Siegelman ES, Mitchell DG, Semelka RC: Abdominal iron deposition: metabolism, MR findings, and clinical importance. Radiology 199:13, 1996

Imaging of Chronic Liver Disease

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Gastrointestinal Radiology

Benign Biliary Disease

Angela D. Levy, COL, MC, USA Objectives •

•

Congenital Disorders ➢ Caroli disease ➢ Choledochal cyst ➢ Polycystic Liver Disease Inflammatory Disorders ➢ Primary sclerosing cholangitis ➢ AIDS-related cholangiopathy ➢ Recurrent Pyogenic Cholangitis ➢ Acute Pyogenic Cholangitis

Differential Diagnosis • • • • •

Obstructive biliary dilatation Caroli disease Choledochal cyst Polycystic liver disease Cholangitis ➢ RPC, Pyogenic

Figure 2-5-1

41-year-old male with history of renal stones and diagnosis of medullary sponge kidney presents with abdominal pain, sepsis, elevated LFTs [Figure 2-5-1] Obstructive Biliary Dilatation

• • •

Tubular dilatation Diffuse dilatation proximal to the obstruction Abrupt termination at level of obstruction

• • •

Caroli disease Choledochal cyst Polycystic liver disease

• • •

Autosomal recessive Secondary to ductal plate malformation (DPM) Associated with renal disorders ➢ ARPCKD, ADPCKD ➢ Medullary sponge kidney ➢ Medullary cystic disease

Congenital Disorders

Saccular and fusiform biliary dilatation in Caroli disease

Caroli Disease

Ductal Plate •

Embryologic precursor of intrahepatic bile ducts

• •

Abnormal development of intrahepatic bile ducts Lack of ductal plate remodeling ➢ Persistence of embryonic structures (DPM) ➢ Segmental dilatation ➢ Destructive inflammation/fibrosis Spectrum of diseases ➢ Small interlobular ducts: Congenital hepatic fibrosis (CHF) ➢ Large intrahepatic ducts: Caroli disease ➢ All ducts: Caroli syndrome (CHF and Caroli disease)

Ductal Plate Malformation

•

Gastrointestinal Radiology

303

Benign Biliary Disease

Intrahepatic Duct Embryology: Ductal Plate [Figures 2-5-2 and 2-5-3]

Figure 2-5-2

Figure 2-5-3

Fusion and absorption of ductal plate cells Normal ductal plate development of the intrahepatic bile ducts

Single layer of ductal Double layer of ductal plate cells plate cells

Caroli Disease: Clinical Features • •

•

Presentation at any age (mean age 37 years) Pain, fever, jaundice ➢ Recurrent cholangitis ➢ Stone formation Liver failure ➢ Fibrosis

Caroli Disease: Complications

• • • • •

Recurrent biliary stones Recurrent cholangitis Hepatic abscess Fibrosis/cirrhosis Cholangiocarcinoma (7%)

•

Intrahepatic duct dilatation ➢ Distribution: segmental (83%) or diffuse (17%) ➢ Shape: saccular (76%) or fusiform (24%) ➢ Central dot sign Extrahepatic duct dilatation ➢ 53% of cases ➢ Secondary to cholangitis and stone passage Cirrhosis

Caroli Disease: Radiologic Features

• •

Normal ductal plate remodeling results in intercommunicating intrahepatic bile ducts surrounding a normal portal tract. Ductal plate malformation results in biliary duct ectasia and fibrosis surrounding the portal tract

Figure 2-5-4

Caroli Disease: “Central Dot Sign” [Figure 2-5-4]

Caroli disease showing saccular biliary dilatation and the central dot sign Benign Biliary Disease

304

Gastrointestinal Radiology

Caroli Syndrome Cirrhosis, Portal Hypertension [Figure 2-5-5]

Figure 2-5-5

Caroli Disease Cholangiography Caroli Disease: Segmental

Caroli Disease: Hepatic Abscess

Caroli Disease: Intrahepatic Lithiasis

Caroli Disease: Intraductal Cholangiocarcinoma Caroli Disease: Cholangiocarcinoma

•

Enhancing intraductal masses Focal strictures ➢ Irregular margins ➢ Shoulders ➢ Irregular tapering Infiltrating masses

• •

Congenital dilatation of the bile ducts Association with anomalous pancreatico-biliary junction (APBJ)

•

Presentation at any age (mean, 17 years) ➢ F>M Clinical presentation ➢ Pain, jaundice, palpable mass Complications ➢ Stones ➢ Cholangitis ➢ Malignancy

• •

Choledochal Cyst

Choledochal Cyst: Clinical Features • •

Choledochal Cyst: Etiology [Figures 2-5-6 and 2-5-7] • •

Caroli disease in two different patients showing diffuse fusiform and saccular dilatation

Figure 2-5-6

Normal pancreaticobiliary junction showing union of the ducts in the sphincter complex, which is located in the duodenal wall. There may be a common channel (ampulla) or not

Normal Pancreaticobiliary Junction ➢ Sphincter complex encircles distal CBD and PD ➢ 80% to 90% have a common channel (4-5 mm Anomalous Junction (APBJ) ➢ Union of CBD and PD outside of duodenum and sphincter complex ➢ Reflux of pancreatic enzymes into CBD)

Figure 2-5-7

Choledochal Cyst: Pathologic Features • • •

Extrahepatic duct dilatation Mural thickening Normal epithelium

Anomalous pancreaticobiliary junction showing union of the common bile duct and pancreatic duct proximal to the duodenal wall and sphincter complex Gastrointestinal Radiology

305

Benign Biliary Disease

Choledochal Cyst: Todani Classification [Figure 2-5-8] Todani Type I

Figure 2-5-8

Todani Type II: Diverticulum Todani Type III: Choledochocele

Tubulovillous Adenoma

Todani Type IV [Figure 2-5-9]

Todani Type V: Caroli Disease Is Todani Type V Caroli Disease? •

•

Choledochal cyst ➢ Congenital, not inherited ➢ Extrahepatic bile duct dilatation with varying degrees Todani classification of choledochal cysts of proximal dilatation ➢ Surgical therapy with biliary reconstruction Caroli disease Figure ➢ Congenital, inherited ➢ Intrahepatic +/- extrahepatic dilatation ➢ Liver biopsy shows DPM ➢ Medical therapy (surgery for complications)

2-5-9

Malignancy and Choledochal Cyst • •

Adenocarcinoma most common Locations ➢ Within the choledochal cyst ➢ Gallbladder ➢ Biliary tract

Malignancy and Choledochal Cyst Polycystic Liver Disease •

•

Todani Type IV choledochal cyst showing central Two forms intrahepatic and extrahepatic duct dilatation on CT. ➢ Isolated to the liver The cholangiogram shows an anomalous ➢ Occurring with ADPCKD pancreaticobiliary junction as well as the extent of Bile duct cysts duct dilatation ➢ Secondary to von Meyenberg complexes ➢ Von Meyenberg complex is DPM at the lowest level

Polycystic Liver Disease - Radiologic Features •

• •

Multiple liver cysts ➢ May have internal hemorrhage ➢ Rim-like calcification Normal bile ducts Associated feature of ADPCKD ➢ Renal cysts ➢ Pancreatic cysts ➢ Thyroid cysts ➢ Seminal vesicle cysts in males

Benign Biliary Disease

306

Gastrointestinal Radiology

Polycystic Liver Disease

Figure 2-5-10

Polycystic Liver Disease in ADPCKD [Figure 2-5-10] Polycystic Liver Disease without ADPCKD Polycystic Liver Disease •

Bile ducts ➢ Displaced, but normal ➢ Rare, mucosal irregularity

41-year-old male with history of renal stones and diagnosis of medullary sponge kidney presents with abdominal pain, sepsis, elevated LFT’s Differential Diagnosis • • • • • ➢

Obstructive biliary dilatation Caroli disease Choledochal cyst Polycystic liver disease Cholangitis RPC, Pyogenic

Polycystic liver disease occurring in ADPCKD

Figure 2-5-11

Caroli Disease

Summary: Congenital Disorders •

•

Exclude obstructive dilatation Congenital disorders ➢ Caroli disease ❖ Intrahepatic ➢ Choledochal cyst ❖ Extrahepatic ➢ Polycystic liver disease ❖ Noncommunicating cysts

Primary sclerosing cholangitis

40-year-old woman with elevated LFT’s

Figure 2-5-12

[Figure 2-5-11]

Differential Diagnosis

•

Cholangitis ➢ Primary sclerosing ➢ AIDS-related ➢ Recurrent pyogenic ➢ Acute pyogenic cholangitis Neoplasm

• • • •

Primary sclerosing cholangitis AIDS-related cholangiopathy Recurrent pyogenic cholangitis Acute pyogenic cholangitis

• • • •

Cholestatic liver disease Unknown etiology Fibrosing inflammation Diagnosis ➢ Liver biopsy ➢ Cholangiogram

•

Inflammatory Disorders

Primary Sclerosing Cholangitis [Figure 2-5-12]

Gastrointestinal Radiology

Illustration showing disease distribution, mural thickening, and inflammatory changes of primary sclerosing cholangitis 307

Benign Biliary Disease

PSC - Imaging • •

•

•

Thickened duct wall ➢ Asymmetric or circumferential ➢ 2 to 5 mm Hepatic parenchymal changes ➢ Cirrhosis ➢ Periportal fibrosis ➢ Discontinuous duct dilatation ➢ Portal hypertension Distribution ➢ Intrahepatic 100% ➢ Extrahepatic 70% ➢ Cystic duct 30% ➢ Pancreatic duct (rare) Other features ➢ Gallbladder disease (40%) ➢ Ductal stones (8%) ➢ Adenopathy ➢ Neoplasm

Figure 2-5-13

CT of primary sclerosing cholangitis showing discontinuous bile duct dilatation, mural thickening of the common hepatic duct, and hepatoduodenal ligament adenopathy

Figure 2-5-14

PSC: Sonographic Features PSC: CT Features [Figure 2-5-13] PSC: Cholangiography • • • •

Beading Pruned-tree Mural irregularity Diverticula

[Figure 2-5-14]

MRCP

PSC: Cholangiocarcinoma •

• •

Stricture (90%) ➢ Long strictures (>1cm) ➢ Completely obstructing strictures ➢ Associated mass Multicentric (10%) Polypoid mass

ERCP of primary sclerosing cholangitis showing beading and pruning of the intrahepatic bile ducts. The extrahepatic bile duct shows mural irregularity with focal stricture formation

Figure 2-5-15

AIDS Cholangiopathy [Figure 2-5-15] •

• •

Group of disorders ➢ Sclerosing cholangitis ➢ Papillary stenosis ➢ Acalculous cholecystitis Opportunistic infection ➢ Cryptosporidium ➢ Cytomegalovirus Declining incidence ➢ HAART therapy

AIDS Cholangiopathy •

Cholangiographic features ➢ Beading ➢ Pruning ➢ Mural irregularity ➢ Filling defects (granulation tissue) ➢ Papillary stenosis (papillitis) ➢ No EHD stenosis or diverticula

Benign Biliary Disease

Illustration of AIDS cholangiopathy showing disease distribution 308

Gastrointestinal Radiology

AIDS Cholangiopathy [Figure 2-5-16]

Figure 2-5-16

AIDS Cholangiopathy •

Sonographic features ➢ Gallbladder wall thickening ❖ Acalculous cholecystitis ➢ Bile duct wall thickening ➢ Hyperechoic nodule in distal CBD (papillitis)

AIDS Cholangiopathy Acalculous cholecystitis [Figure 2-5-17]

Recurrent Pyogenic Cholangitis (RPC) [Figure 2-5-18] •

• •

Clinical syndrome ➢ Pigmented stones ➢ Recurrent infection Unknown etiology ➢ Biliary parasites ➢ Malnutrition ➢ Portal bacteremia Complications ➢ Biliary cirrhosis ➢ Cholangiocarcinoma

RPC: Imaging • • • •

AIDS cholangiopathy with papillary stenosis

Figure 2-5-17

[Figure 2-5-19]

Stones, sludge Duct dilatation Left lobe predominant Parenchymal changes ➢ Atrophy ➢ Fatty change ➢ Altered enhancement ➢ Abscess

Acalculous cholecystitis in AIDS

RPC

Figure 2-5-18

Acute Pyogenic Cholangitis [Figure 2-5-20] • • •

Almost always post-obstructive Polymicrobial Etiology ➢ Stones ➢ Anastomotic stricture ➢ Papillary stenosis ➢ Carcinoma ➢ Underlying biliary disease

Acute Pyogenic Cholangitis •

Imaging Features ➢ Duct dilatation ➢ Obstructive lesion ➢ Echogenic bile ➢ Mural irregularity ➢ Hepatic abscess

Gastrointestinal Radiology

Illustration of recurrent pyogenic cholangitis showing typical disease distribution

309

Benign Biliary Disease

Figure 2-5-19

Recurrent pyogenic cholangitis with intrahepatic lithiasis and duct dilatation

Acute Pyogenic Cholangitis with Microabscesses

Figure 2-5-20

40-year-old woman with elevated LFT’s Differential Diagnosis •

•

Cholangitis ➢ Primary sclerosing ➢ AIDS-related ➢ Recurrent pyogenic ➢ Acute pyogenic cholangitis Neoplasm

Primary Sclerosing Cholangitis Summary •

• • •

PSC ➢ Fibrosis AIDS cholangiopathy ➢ Papillary stenosis ➢ Acalculous cholecystitis RPC ➢ Stones ➢ Focal dilatation Pyogenic cholangitis ➢ Obstruction

Illustration showing typical manifestations of acute pyogenic cholangitis

References

Caroli Disease 1. Choi BI, Yeon KM, Kim SH, et al: Caroli disease: central dot sign in CT. Radiology 174:161, 1990 2. Desmet VJ: Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities. Mayo Clinic Proceedings 73:80, 1998 3. Krause D, Cercueil JP, Dranssart M, et al: MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr 26:541, 2002 4. Levy AD, Rohrmann CA, Jr., Murakata LA, et al: Caroli's disease: radiologic spectrum with pathologic correlation. AJR 179:1053, 2002 5. Marchal GJ, Desmet VJ, Proesmans WC, et al: Caroli disease: high-frequency US and pathologic findings. Radiology 158:507, 1986 Benign Biliary Disease

310

Gastrointestinal Radiology

6. 7. 8.

Miller WJ, Sechtin AG, Campbell WL, et al: Imaging findings in Caroli's disease. AJR 165:333, 1995 Pavone P, Laghi A, Catalano C, et al: Caroli's disease: evaluation with MR cholangiopancreatography (MRCP). Abdom Imaging 21:117, 1996 Pavone P, Laghi A, Catalano C, et al: Caroli's disease: evaluation with MR cholangiography. AJR 166:216, 1996

Choledochal Cyst 1. Babbitt DP, Starshak RJ, Clemett AR: Choledochal cyst: a concept of etiology. AJR 119:57, 1973 2. Govil S, Justus A, Korah I, et al: Choledochal cysts: evaluation with MR cholangiography. Abdom Imaging 23:616, 1998 3. Levy AD, Rohrmann CA, Jr.: Biliary cystic disease. Curr Probl Diagn Radiol 32:233, 2003 4. Liu CL, Fan ST, Lo CM, et al: Choledochal cysts in adults. Arch Surg 137:465, 2002 5. O'Neill JA, Jr.: Choledochal cyst. Curr Probl Surg 29:361, 1992 6. Savader SJ, Benenati JF, Venbrux AC, et al: Choledochal cysts: classification and cholangiographic appearance. AJR 156:327, 1991 7. Savader SJ, Venbrux AC, Benenati JF, et al: Choledochal cysts: role of noninvasive imaging, percutaneous transhepatic cholangiography, and percutaneous biliary drainage in diagnosis and treatment. J Vasc Interv Radiol 2:379, 1991 8. Todani T, Watanabe Y, Fujii T, et al: Anomalous arrangement of the pancreatobiliary ductal system in patients with a choledochal cyst. Am J Surg 147:672, 1984 9. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263, 1977 10. Wearn FG, Wiot JF: Choledochocele: not a form of choledochal cyst. J Can Assoc Radiol 33:110, 1982 Polycystic Liver Disease 1. Dranssart M, Cognet F, Mousson C, et al: MR cholangiography in the evaluation of hepatic and biliary abnormalities in autosomal dominant polycystic kidney disease: study of 93 patients. J Comput Assist Tomogr 26:237, 2002 2. Grunfeld JP, Albouze G, Jungers P, et al: Liver changes and complications in adult polycystic kidney disease. Adv Nephrol Necker Hosp 14:1, 1985 3. Gupta S, Seith A, Dhiman RK, et al: CT of liver cysts in patients with autosomal dominant polycystic kidney disease. Acta Radiol 40:444, 1999 4. Itai Y, Ebihara R, Eguchi N, et al: Hepatobiliary cysts in patients with autosomal dominant polycystic kidney disease: prevalence and CT findings. AJR 164:339, 1995 5. Pirson Y, Lannoy N, Peters D, et al: Isolated polycystic liver disease as a distinct genetic disease, unlinked to polycystic kidney disease 1 and polycystic kidney disease 2. Hepatology 23:249, 1996 6. Segal AJ, Spataro RF: Computed tomography of adult polycystic disease. J Comput Assist Tomogr 6:777, 1982

Primary Sclerosing Cholangitis 1. Ament AE, Haaga JR, Wiedenmann SD, et al: Primary sclerosing cholangitis: CT findings. J Comput Assist Tomogr 7:795, 1983 2. Brandt DJ, MacCarty RL, Charboneau JW, et al: Gallbladder disease in patients with primary sclerosing cholangitis. AJR Am J Roentgenol 150:571, 1988 3. Campbell WL, Ferris JV, Holbert BL, et al: Biliary tract carcinoma complicating primary sclerosing cholangitis: evaluation with CT, cholangiography, US, and MR imaging. Radiology 207:41, 1998 4. Campbell WL, Peterson MS, Federle MP, et al: Using CT and cholangiography to diagnose biliary tract carcinoma complicating primary sclerosing cholangitis. AJR Am J Roentgenol 177:1095, 2001 5. Dodd GD, 3rd, Baron RL, Oliver JH, 3rd, et al: End-stage primary sclerosing cholangitis: CT findings of hepatic morphology in 36 patients. Radiology 211:357, 1999 6. Fulcher AS, Turner MA, Franklin KJ, et al: Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study. Radiology 215:71, 2000 7. Gulliver DJ, Baker ME, Putnam W, et al: Bile duct diverticula and webs: nonspecific cholangiographic features of primary sclerosing cholangitis. AJR Am J Roentgenol 157:281, 1991 8. Ito K, Mitchell DG, Outwater EK, et al: Primary sclerosing cholangitis: MR imaging features. AJR Am J Roentgenol 172:1527, 1999

Gastrointestinal Radiology

311

Benign Biliary Disease

9.

10. 11.

12. 13. 14. 15. 16.

Lumsden AB, Alspaugh JP: Cholangiocarcinoma complicating primary sclerosing cholangitis: cholangiographic appearances. Radiology 158:856, 1986 MacCarty RL, LaRusso NF, Wiesner RH, et al: Primary sclerosing cholangitis: findings on cholangiography and pancreatography. Radiology 149:39, 1983 Majoie CB, Smits NJ, Phoa SS, et al: Primary sclerosing cholangitis: sonographic findings. Abdom Imaging 20:109, 1995 May GR, Bender CE, LaRusso NF, et al: Nonoperative dilatation of dominant strictures in primary sclerosing cholangitis. AJR Am J Roentgenol 145:1061, 1985 Olsson R, Danielsson A, Jarnerot G, et al: Prevalence of primary sclerosing cholangitis in patients with ulcerative colitis. Gastroenterology 100:1319, 1991 Teefey SA, Baron RL, Rohrmann CA, et al: Sclerosing cholangitis: CT findings. Radiology 169:635, 1988 Teefey SA, Baron RL, Schulte SJ, et al: Patterns of intrahepatic bile duct dilatation at CT: correlation with obstructive disease processes. Radiology 182:139, 1992 Williams SM, Harned RK: Hepatobiliary complications of inflammatory bowel disease. Radiol Clin North Am 25:175, 1987

AIDS Cholangiopathy 1. Chen XM, LaRusso NF: Cryptosporidiosis and the pathogenesis of AIDS-cholangiopathy. Semin Liver Dis 22:277, 2002 2. Collins CD, Forbes A, Harcourt-Webster JN, et al: Radiological and pathological features of AIDS-related polypoid cholangitis. Clin Radiol 48:307, 1993 3. Da Silva F, Boudghene F, Lecomte I, et al: Sonography in AIDS-related cholangitis: prevalence and cause of an echogenic nodule in the distal end of the common bile duct. AJR Am J Roentgenol 160:1205, 1993 4. Defalque D, Menu Y, Girard PM, et al: Sonographic diagnosis of cholangitis in AIDS patients. Gastrointest Radiol 14:143, 1989 5. Dolmatch BL, Laing FC, Ferderle MP, et al: AIDS-related cholangitis: radiographic findings in nine patients. Radiology 163:313, 1987 Recurrent Pyogenic Cholangitis 1. Chan FL, Man SW, Leong LL, et al: Evaluation of recurrent pyogenic cholangitis with CT: analysis of 50 patients. Radiology 170:165, 1989 2. Federle MP, Cello JP, Laing FC, et al: Recurrent pyogenic cholangitis in Asian immigrants. Use of ultrasonography, computed tomography, and cholangiography. Radiology 143:151, 1982 3. Jeyarajah DR: Recurrent Pyogenic Cholangitis. Curr Treat Options Gastroenterol 7:91, 2004 4. Kim MJ, Cha SW, Mitchell DG, et al: MR imaging findings in recurrent pyogenic cholangitis. AJR Am J Roentgenol 173:1545, 1999 5. Okuno WT, Whitman GJ, Chew FS: Recurrent pyogenic cholangiohepatitis. AJR Am J Roentgenol 167:484, 1996 6. Park MS, Yu JS, Kim KW, et al: Recurrent pyogenic cholangitis: comparison between MR cholangiography and direct cholangiography. Radiology 220:677, 2001

Benign Biliary Disease

312

Gastrointestinal Radiology

Biliary Neoplasms

Angela D. Levy, COL, MC, USA Objectives •

•

Biliary adenocarcinoma ➢ Intrahepatic cholangiocarcinoma ➢ Intraductal cholangiocarcinoma ➢ Hilar (Klatskin) cholangiocarcinoma ➢ Extrahepatic duct adenocarcinoma Differential diagnosis ➢ Benign strictures ➢ Other neoplasms

Biliary Adenocarcinoma •

• •

Incidence in U.S. ➢ ~2000 to 2500 cases per year Incidence worldwide ➢ Up to 10 times greater in Asian countries More common in men ➢ 2:1

Biliary Adenocarcinoma •

High risk groups ➢ Autoimmune diseases ❖ PSC, ulcerative colitis, primary biliary cirrhosis ➢ Congenital anatomic anomalies ❖ Caroli, choledochal cyst, anomalous pancreaticobiliary junction ❖ Abnormal tumor suppressor genes, FAP, NF1 ➢ Infection ❖ Biliary parasites, recurrent pyogenic cholangitis

Biliary Adenocarcinoma •

Clinical presentation ➢ Jaundice ➢ Pain ➢ Fever if secondary cholangitis

Biliary Adenocarcinoma • • •

Moderately to well differentiated Desmoplastic stroma Infiltrative margins ➢ NO CAPSULE

Biliary Adenocarcinoma • • •

Dismal 5-year survival < 20% resectable at diagnosis Curative resection ➢ Tumor free margins ➢ “No touch” technique

Biliary Adenocarcinoma •

Intrahepatic Cholangiocarcinoma

•

Intraductal Cholangiocarcinoma

•

Hilar Cholangiocarcinoma

Biliary Adenocarcinoma Biliary Adenocarcinoma Gastrointestinal Radiology

313

Biliary Neoplasms

Biliary Adenocarcinoma •

Extrahepatic bile duct adenocarcinoma

• • • • • • •

Solitary, large mass No capsule Dense fibrous stroma No necrosis or hemorrhage Multifocal mass Satellite lesions Intrahepatic metastasis

• • •

Delayed enhancement Peripheral biliary dilatation Capsular contraction

•

Differential diagnosis ➢ Metastasis ➢ HCC ➢ Gallbladder adenocarcinoma ➢ Rare, sarcoma Identifying key features of ICC ➢ Evidence of fibrous stroma ➢ Contrast enhancement pattern ➢ Capsular contraction

Figure 2-6-1

Intrahepatic Cholangiocarcinoma: Pathologic Features

Intrahepatic Cholangiocarcinoma [Figure 2-6-1] Intrahepatic Cholangiocarcinoma

•

Intraductal Cholangiocarcinoma [Figure 2-6-2]

Intrahepatic cholangiocarcinoma with delayed enhancement and capsular contraction

• • •

Rare Intrabiliary mass Biliary diliatation peripheral to the mass

• • •

Tumors arising at or near the confluence of the right and left hepatic ducts Most common site of biliary adenocarcioma Aggressive biologic behavior

• • •

Imaging features Anatomic location Pathologic features

•

Imaging features ➢ Duct dilatation ➢ Ill-defined mass ➢ Lobar atrophy ➢ Vascular invasion

Hilar Cholangiocarcinoma: Klatskin Tumor

Hilar Cholangiocarcinoma: Klatskin Tumor

Figure 2-6-2

Hilar Cholangiocarcinoma: “Klatskin Tumor”

Hilar Cholangiocarcinoma: Imaging Features [Figure 2-6-3] •

•

•

Dilated ducts ➢ Discontinuous ducts Poorly defined mass ➢ Poor visibility of tumor mass ➢ Minimal tumor enhancement on CT (50% of cases) ➢ More likely to enhance on MR ➢ Parenchymal invasion (segment IV) 30% of cases Lobar or segmental atrophy ➢ Secondary to vascular compromise

Biliary Neoplasms

314

Intraductal cholangiocarcinoma. There is an intrabiliary mass on the CT and corresponding gross photograph Gastrointestinal Radiology

Hilar Cholangiocarcinoma [Figure 2-6-4]

Figure 2-6-3

Hilar Cholangiocarcinoma [Figure 2-6-5] •

•

Cholangiographic features of malignant strictures ➢ Evidence of mass effect ➢ Irregular margins ➢ Irregular or abrupt tapering at obstruction Limitations of MRCP ➢ Spatial resolution ➢ Evaluation of secondary ducts

Hilar Cholangiocarcinoma Role of Preoperative Imaging • •

Determination of resectablility Surgical planning ➢ Bismuth-Corlette classification1 ➢ Define extent of duct involvement

1Bismuth H, Corlette MB. Surg Gynecol Obstet 1975, 140: 170-178.

Hilar Cholangiocarcinoma: Unresectability •

• • • •

Bilateral tumor extension ➢ Into secondary ducts ➢ Into hepatic parenchyma ➢ Hepatic artery or portal vein Occlusion main portal vein N2 nodes Distant mets Medically unfit patients

Classic sonographic appearance of hilar cholangiocarcinoma showing biliary dilatation and an ill-defined hilar mass

Figure 2-6-4

Bismuth-Corlette: Type I [Figures 2-6-6 and 2-6-7]

•

Tumor below confluence

Bismuth-Corlette: Type II [Figure 2-6-8]

•

Tumor at confluence

•

Tumor extends to right or left hepatic ducts Hemiliver resection ➢ Preoperative PV embolism

Hilar cholangiocarcinoma. Ill-defined mass adjacent to bile duct stent and extending into hepatoduodenal ligament

Bismuth-Corlette: Type IIIa and IIIb [Figure 2-6-9] •

Figure 2-6-5

Bismuth-Corlette: Type IIIa Bismuth-Corlette: Type IV [Figures 2-6-10 and 2-6-11]

•

Tumor in bilateral ducts

Gastrointestinal Radiology

Hilar cholangiocarcinoma on MRCP and percutaneous transhepatic cholangiography

315

Biliary Neoplasms

Figure 2-6-6

Figure 2-6-7

Bismuth Corlette Type I hilar cholangiocarcinoma. Tumor is below the confluence of the right and left hepatic ducts

Bismuth Corlette Type I

Figure 2-6-8

Bismuth Corlette Type II hilar cholangiocarcinoma. Tumor involves the confluence

Figure 2-6-10

Figure 2-6-9

Bismuth Colette Type IV hilar cholangiocarcinoma. Tumor involves bilateral intrahepatic ducts

Figure 2-6-11

Bismuth Colette Type IIIa (upper) and IIIb (lower) hilar cholangiocarcinoma. Tumor involves the right (IIIa) or left (IIIb) hepatic duct

Bismuth Corlette Type IV Biliary Neoplasms

316

Gastrointestinal Radiology

EHBD Adenocarcinoma [Figure 2-6-12] •

Figure 2-6-12

Variable morphology ➢ Diffusely infiltrating ➢ Polypoid ➢ Nodular ➢ Constricting (scirrhous)

EHBD Adenocarcinoma: Imaging • •

Biliary obstruction Tumor ➢ Intraluminal mass ➢ Stenosis ➢ Complete obstruction

Papillary Adenocarcinoma [Figure 2-6-13]

EHBD Adenocarcinoma vs. Benign Stricture • •

Malignant ➢ Duct abruptly terminates at stricture Benign ➢ Duct tapers to stricture

Baron, RL. Radiol Clin N Am 1991. 29:1237.

Figure 2-6-13

Morphologic types of extrahepatic duct adenocarcinoma

Papillary adenocarcinoma of the extrahepatic bile duct

Malignant Stricture [Figures 2-6-14 and 2-6-15] EHBD Adenocarcinoma Figure 2-6-14

Figure 2-6-15

Cholangiographic and CT features of a malignant stricture

Malignant stricture Gastrointestinal Radiology

317

Biliary Neoplasms

Benign Stricture [Figures 2-6-16 and 2-6-17]

Figure 2-6-16

Pancreatitis with Stricture Differential Diagnosis •

•

•

Post-inflammatory benign strictures ➢ Pancreatitis ➢ Post radiation or chemotherapy Inflammatory strictures ➢ Primary sclerosing cholangitis ➢ AIDS cholangiopathy ➢ Recurrent pyogenic cholangitis ➢ Biliary parasites Other neoplasms ➢ Gallbladder adenocarcinoma ➢ Pancreatic adenocarcinoma ➢ Metastasis ➢ Granular cell tumor ➢ Biliary papillomatosis

Granular Cell Tumor • • •

•

[Figure 2-6-18]

Benign tumors of Schwann cell origin 90% of patients are females, mean age 34 years Location: ➢ CBD (50%) ➢ Cystic duct (37%) ➢ CHD (11%) ➢ Gallbladder (4%) ➢ Intrahepatic ducts (4%) Infiltrative lesions that produce short annular strictures

Benign stricture

Figure 2-6-17

Figure 2-6-18

Cholangiographic and CT features of a benign stricture

Granular cell tumor Biliary Neoplasms

318

Gastrointestinal Radiology

Biliary Papillomatosis

• • • •

Multiple and recurrent adenomas of the biliary tract Men and women in the 6th and 7th decades Clinical presentation: biliary obstruction, cholangitis Local recurrence and malignant transformation common

•

Biliary Adenocarcinomas ➢ Uncommon Peripheral intrahepatic cholangiocarcinoma ➢ Mass forming tumors ➢ Delayed, patchy enhancement ➢ Look for imaging evidence of fibrosis Intraductal cholangiocarcinoma ➢ Rare ➢ Intraductal masses ➢ Biliary obstruction Hilar cholangiocarcinoma ➢ Most common subtype ➢ Look for discontinuous biliary dilatation ➢ Determination of resectablility Extrahepatic duct adenocarcinoma ➢ Must differentiate from a benign stricture

Summary • • • •

Summary: Benign vs. Malignant •

•

Malignant stricture ➢ Abrupt change Benign stricture ➢ Tapering

Klatskin / PSC

Summary: Granular Cell Tumor •

Benign Neoplasm ➢ Young, women ➢ True mimic for carcinoma

References

Intrahepatic Cholangiocarcinoma 1. Choi BI, Lee JM, Han JK: Imaging of intrahepatic and hilar cholangiocarcinoma. Abdom Imaging 29:548, 2004 2. Ishak KG, Goodman ZD, Stocker JT: Tumors of the Liver and Intrahepatic Bile Ducts. Washington, D.C.: Armed Forces Institute of Pathology under the auspices of Universities Associated for Research and Education in Pathology For sale by the Armed Forces Institute of Pathology, 2001 3. Kim TK, Choi BI, Han JK, et al: Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings. Radiology 204:539, 1997 4. Lim JH: Cholangiocarcinoma: morphologic classification according to growth pattern and imaging findings. AJR Am J Roentgenol 181:819, 2003 5. Tani K, Kubota Y, Yamaguchi T, et al: MR imaging of peripheral cholangiocarcinoma. J Comput Assist Tomogr 15:975, 1991 6. Vilgrain V, Van Beers BE, Flejou JF, et al: Intrahepatic cholangiocarcinoma: MRI and pathologic correlation in 14 patients. J Comput Assist Tomogr 21:59, 1997 7. Worawattanakul S, Semelka RC, Noone TC, et al: Cholangiocarcinoma: spectrum of appearances on MR images using current techniques. Magn Reson Imaging 16:993, 1998 8. Yalcin S: Diagnosis and management of cholangiocarcinomas: a comprehensive review. Hepatogastroenterology 51:43, 2004 9. Zhang Y, Uchida M, Abe T, et al: Intrahepatic peripheral cholangiocarcinoma: comparison of dynamic CT and dynamic MRI. J Comput Assist Tomogr 23:670, 1999

Gastrointestinal Radiology

319

Biliary Neoplasms

Hilar Cholangiocarcinoma 1. Arepally A, Georgiades C, Hofmann LV, et al: Hilar cholangiocarcinoma: staging with intrabiliary MRI. AJR Am J Roentgenol 183:1071, 2004 2. Bold RJ, Goodnight JE, Jr.: Hilar cholangiocarcinoma: surgical and endoscopic approaches. Surg Clin North Am 84:525, 2004 3. Koea J, Holden A, Chau K, et al: Differential diagnosis of stenosing lesions at the hepatic hilus. World J Surg 28:466, 2004 4. Manfredi R, Masselli G, Maresca G, et al: MR imaging and MRCP of hilar cholangiocarcinoma. Abdom Imaging 28:319, 2003 5. Principe A, Ercolani G, Bassi F, et al: Diagnostic dilemmas in biliary strictures mimicking cholangiocarcinoma. Hepatogastroenterology 50:1246, 2003 6. Soyer P, Bluemke DA, Reichle R, et al: Imaging of intrahepatic cholangiocarcinoma: 2. Hilar cholangiocarcinoma. AJR Am J Roentgenol 165:1433, 1995 Extrahepatic Bile Duct Adenocarcinoma 1. Albores-Saavedra J, Henson DE, Klimstra DS: Tumors of the gallbladder and extrahepatic bile ducts, and ampulla of vater. Washington, D.C.: Armed Forces Institute of Pathology under the auspices of Universities Associated for Research and Education in Pathology For sale by the Armed Forces Institute of Pathology, 2000 2. Park MS, Kim TK, Kim KW, et al: Differentiation of extrahepatic bile duct cholangiocarcinoma from benign stricture: findings at MRCP versus ERCP. Radiology 233:234, 2004 3. Stroszczynski C, Hunerbein M: Malignant biliary obstruction: value of imaging findings. Abdom Imaging 30:314, 2005 4. Uchida M, Ishibashi M, Tomita N, et al: Hilar and suprapancreatic cholangiocarcinoma: value of 3D angiography and multiphase fusion images using MDCT. AJR Am J Roentgenol 184:1572, 2005

Biliary Neoplasms

320

Gastrointestinal Radiology

Pancreatic Neoplasms

Angela D. Levy, COL, MC, USA Classification of Pancreatic Tumors •

• • • •

Tumors of the Exocrine Pancreas ➢ Ductal adenocarcinoma ➢ Acinar cell carcinoma ➢ Solid-pseudopapillary neoplasm ➢ Intraductal papillary mucinous neoplasm ➢ Mucinous cystic neoplasm ➢ Microcystic adenoma ➢ Pancreatoblastoma ➢ Mature cystic teratoma Tumors of the Endocrine Pancreas ➢ Islet cell tumors (neuroendocrine tumors) ➢ Small cell carcinoma Non-epithelial tumors ➢ Soft tissue tumors ➢ Lymphoma Secondary Tumors Tumor like lesions ➢ Pancreatitis ➢ Lymphoepithelial cyst ➢ Pseudocyst

Objectives •

• •

• •

Adenocarcinoma ➢ Ductal adenocarcinoma ➢ Mucinous noncystic adenocarcinoma Intraductal papillary mucinous neoplasm Cystic neoplasms ➢ Intraductal papillary mucinous neoplasm ➢ Solid and pseudopapillary epithelial neoplasm ➢ Mucinous cystic neoplasm ➢ Microcystic adenoma Endocrine neoplasms Metastasis

Pancreatic Adenocarcinoma: Epidemiology • •

85% to 90% of pancreatic neoplasms Second most common GI tract malignancy in the U.S. ➢ US: colorectal, pancreas, stomach, liver, esophagus, gallbladder ➢ Worldwide: stomach, colorectal, liver, esophagus, pancreas, gallbladder

Pancreatic Adenocarcinoma: Epidemiology • • •

Death:incidence ratio of .99 ➢ 5-year survival 4%, overall ➢ 5-year survival 17%, early stage More common in men than women 80% of cases occur between 60 to 80 years of age

Pancreatic Adenocarcinoma: Risk Factors • •

Cigarette smoking (2-3 fold relative risk) Hereditary risk factors ➢ Hereditary nonpolyposis colon cancer (HNPCC) ➢ Familial breast cancer (BRCA2) ➢ Familial adenomatous polyposis ➢ Peutz-Jeghers

Gastrointestinal Radiology

321

Pancreatic Neoplasms

➢ ➢ ➢ ➢

Peutz-Jeghers Ataxia telangiectasia Familial atypical multiple mole-melanoma Familial pancreatitis

Figure 2-7-1

Pancreatic Adenocarcinoma: Clinical Features •

•

Symptoms ➢ Pain most common ➢ Unexplained weight loss ➢ Jaundice in 50% tumors in the head of the pancreas ➢ Diabetes in 25% to 50% Distribution ➢ 60% head ➢ 20% body ➢ 10% tail ➢ 5% to 10% entire gland

Pancreatic Adenocarcinoma: Pathology [Figure 2-7-1] •

•

Microscopy ➢ Moderately to well differentiated Pathology of ductal adenocarcinoma of the pancreas ➢ Desmoplastic stromal reaction Gross Pathology ➢ Fibrotic ➢ Infiltration and invasion of adjacent structures ➢ Hemorrhage and necrosis uncommon

Pancreatic Adenocarcinoma: MDCT •

Dual Phase Technique ➢ Rapid bolus, 3 ml/sec ➢ Pancreatic phase, 40 sec after injection ➢ Portal venous phase ➢ Thin reformations with overlap, 1.25 mm

Fletcher JG, Wiersema MJ, Farrell MA, et al. Pancreatic malignancy: value of arterial, pancreatic, and hepatic phase imaging with multi-detector row CT. Radiology 2003; 229(1):81-90.

Pancreatic Adenocarcinoma: MDCT • • •

Volume rendering Maximum intensity projection (MIP) Curved planar reformations ➢ Additional information for local extension ➢ Secondary signs in iso-attenuating carcinomas

Pancreatic Adenocarcinoma: CT Features •

• • • •

Pancreatic and CBD duct dilatation ➢ "Double duct sign” ➢ Abrupt tapering of the CBD Atrophy of the distal gland ➢ Focal soft tissue density in a fatty gland Alterations in morphology ➢ Spherical enlargement of the pancreatic head ➢ Rounded borders of the uncinate process Extension to adjacent organs Vascular encasement

Pancreatic Neoplasms

322

Gastrointestinal Radiology

Double Duct Sign [Figure 2-7-2]

Figure 2-7-2

Spherical Enlargement of the Pancreatic Head

Rounded Borders of the Uncinate Process [Figure 2-7-3] Atrophic Changes in the Distal Pancreas [Figure 2-7-4]

Pancreatic Adenocarcinoma: MR •

•

Double duct sign of ductal adenocarcinoma of the pancreas

Figure 2-7-3

Problem solving ➢ Equivocal CT ➢ Small tumors T1-weighted images ➢ Low signal tumor on unenhanced images ➢ Subtraction images from in and out of phase images

Nonresectability • • •

• •

Ductal adenocarcinoma of the pancreas showing a rounded contour Invasion of adjacent organs, to the uncinate process except duodenum Tumor diameter > 5 cm Encasement or occlusion of vessels Figure 2-7-4 ➢ SMA, SMV, portal vein ➢ Celiac trunk and major branches ➢ +/- isolated focal involvement of PV or SMV ➢ Accuracy of CT 88%-90% ➢ 3D CT angiography Distant nodal metastasis Liver metastasis

Resectable? No, stomach and vascular invasion

Ductal adenocarcinoma of the pancreas showing atrophy of the distal pancreas

Figure 2-7-5

Resectable? No, SMA Encasement Resectable? No, SMA Encasement and Liver Mets [Figure 2-7-5]

Resectable? YES

Nonresectable adenocarcinoma of the pancreas

Mucinous Noncystic Adenocarcinoma (Infiltrating Colloid Carcinoma)[Figure 2-7-6] • • • •

Figure 2-7-6

Rare variant of adenocarcinoma Marked extracellular mucin Signet rings cells Imaging ➢ Large tumors ➢ Well-defined hypoattenuating mass ➢ May have calcification

Mucinous noncystic adenocarcinoma of the pancreas Gastrointestinal Radiology

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Mucinous Noncystic Adenocarcinoma (Infiltrating Colloid Carcinoma) •

Differential diagnosis ➢ Microcystic adenoma ➢ Intraductal papillary mucinous neoplasm ➢ Microcystic (Oligocystic) adenoma ➢ Pancreatic pseudocyst

Figure 2-7-7

Intraductal papillary mucinous neoplasm, diffuse main duct pattern

Intraductal Papillary Mucinous Neoplasm (IPMN) •

• •

Intraductal papillary neoplasm ➢ Produces mucin All have malignant potential ➢ May or may not have an invasive component Synonyms ➢ Intraductal mucin-hypersecreting neoplasm ➢ Ductectatic type of pancreatic ductal carcinoma ➢ Mucinous ductal ectasia ➢ Mucin-producing tumor (or carcinoma) ➢ Mucin-hypersecreting tumor

Intraductal Papillary Mucinous Neoplasm (IPMN) • •

Figure 2-7-8

Most common in men, 7th decade Clinical symptoms similar to chronic pancreatitis ➢ Pain ➢ Malabsorption ➢ Diabetes ➢ Prior episodes of pancreatitis

IPMN: Histopathology • • •

Neoplastic papillary epithelium Mucin production Duct dilatation

•

Duct dilatation ➢ Main duct or side branch ➢ Diffuse or focal (cystic appearing) Pancreatic glandular atrophy Calcification Bulging duodenal papilla

IPMN: Imaging Features • • •

Intraductal papillary mucinous neoplasm, focal main duct pattern

IPMN: Diffuse Involvement of Main Pancreatic Duct

Figure 2-7-9

[Figure 2-7-7]

IPMN: Focal Involvement of Main Pancreatic Duct [Figure 2-7-8]

IPMN: Focal Involvement of Main Pancreatic Duct •

•

Show communication with duct structures ➢ MRCP ➢ ERCP/EUS Show intraductal masses

IPMN: Focal Involvement of a Side Branch Duct [Figure 2-7-9]

Pancreatic Neoplasms

324

Intraductal papillary mucinous neoplasm, focal side branch pattern

Gastrointestinal Radiology

Side Branch IPMN

IPMN: Diffuse Involvement of a Side Branch Duct [Figure 2-7-10]

Figure 2-7-10

Bulging Papilla [Figure 2-7-11]

IPMN: Diagnostic Difficulties •

MR/MRCP ➢ Target imaging for ductal communication

IPMN: MR/MRCP

IPMN: Diagnostic Difficulties • •

•

Must differentiate from chronic pancreatitis Side branch variant may mimic ➢ Pseudocyst ➢ Mucinous cystic neoplasm ➢ Microcystic adenoma ERCP ➢ Definitive imaging technique

Intraductal papillary mucinous neoplasms, diffuse side branch pattern

Solid and Pseudopapillary Tumor (SPT) •

• • •

Neoplasm of young women ➢ Mean age, 24 years Benign or low-grade malignancy Good prognosis Synonyms ➢ Solid-cystic tumor ➢ Papillary cystic tumor ➢ Solid and pseudopapillary epithelial neoplasm

Solid and Pseudopapillary Tumor (SPT) •

Clinical features ➢ Usually incidentally discovered ➢ Abdominal discomfort, pain ➢ Jaundice or duct obstruction is rare

SPT: Pathology Features •

•

Pathologic features ➢ Capsule ➢ Hemorrhage, necrosis, cystic areas ➢ Solid areas ➢ May calcify Histopathology ➢ Highly cellular areas ➢ Pseudopapillary areas ➢ Hemorrhage ➢ Sclerosis

Figure 2-7-11

Bulging papilla in IPMN

SPEN: Imaging Features •

• • •

Circumscribed ➢ Capsule ➢ Early peripheral enhancement Cystic change ➢ Hemorrhage ➢ Fluid-fluid levels Calcification Rare features ➢ Biliary/pancreatic duct dilatation ➢ Adjacent organ invasion

Gastrointestinal Radiology

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SPT [Figures 2-7-12 and 2-7-13]

Figure 2-7-12

SPT: Diagnostic Difficulties • • •

•

Older patient age Male patient May mimic other cystic lesions ➢ Pancreatic pseudocyst ➢ Mucinous cystic neoplasm ➢ Oligocystic adenoma Supportive CT and MR features for diagnosis ➢ Evidence of capsule ➢ Evidence of blood products

Mucinous Cystic Neoplasm (MCN) •

• •

Mucin-secreting cystic neoplasm ➢ Mucinous cystadenoma ➢ Mucinous cystadenocarcinoma Most common in women ➢ 80% occur in women ➢ Mean age 49 years Less common in men ➢ Older age, mean 70 years

Solid and pseudopapillary tumor

Figure 2-7-13

Mucinous Cystic Neoplasm (MCN) •

• •

Clinical presentation ➢ Variable ➢ Dependent upon tumor size Jaundice and CBD obstruction are rare All have malignant potential ➢ Mucinous cystadenoma

MCN: Histopathology • • • • •

Columnar cell lining May have ovarian-type stroma Mucin Hemorrhage Calcification

•

Majority in tail of pancreas ➢ 70% to 95% Large size at diagnosis ➢ Mean diameter, 6 to 10 cm Multilocular cysts ➢ Septations ➢ Solid mural nodules ➢ Occasional calcifications ➢ Unilocular, rare

MR showing early capsular enhancement in SPT

MCN: Gross Pathology • •

MCN: Imaging •

• •

•

Well-circumscribed cystic mass ➢ Cannot differentiate benign from malignant Enhancement ➢ Cyst wall, septations, mural nodules Calcifications ➢ Cyst wall, septations, mural nodules Cyst fluid ➢ Variable CT attenuation/MR signal intensity ➢ Mucin ➢ Hemorrhage ➢ Proteinaceous fluid

Pancreatic Neoplasms

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Gastrointestinal Radiology

Mucinous Cystic Neoplasm [Figure 2-7-14]

Figure 2-7-14

MCN: Diagnostic Difficulties • • • •

Small lesions Lesions in the head of the pancreas Lesions without septations and mural nodules Differential diagnosis ➢ Pseudocyst ➢ Oligocystic adenoma ➢ Solid and pseudopapillary tumor ➢ Rare, congenital cysts

Microcystic Adenoma • •

•

Benign Synonyms ➢ Serous cystadenoma ➢ Glycogen-rich cystadenoma Variants ➢ Oligocystic adenoma

Mucinous cystic neoplasm

Microcystic Adenoma •

•

Most common in women ➢ 70% in women ➢ Mean age, 66 years Variable clinical presentation ➢ Incidental ➢ Pain, nausea, vomiting, weight loss ➢ Jaundice is unusual

Figure 2-7-15

Microcystic Adenoma: Gross Pathology •

Small cysts arranged around a central scar ➢ “Honeycomb” or “sponge” appearance ➢ Thin fibrous septae ➢ Central stellate scar ➢ Occasional hemorrhage

Microcystic Adenoma: Histopathology •

Histology ➢ Small cysts ➢ Cuboidal cells ➢ High glycogen content

Microcystic Adenoma: Pathology

Microcystic Adenoma: Imaging features [Figures 2-7-15 and 2-7-16]

•

• •

Sharp, circumscribed margins ➢ Lobular margins Honeycomb appearance ➢ Multiple small cysts Central scar ➢ +/- calcification

Microcystic adenoma

Figure 2-7-16

Microcystic adenoma Gastrointestinal Radiology

327

Pancreatic Neoplasms

Oligocystic Adenoma [Figure 2-7-17] • • • •

Figure 2-7-17

Uncommon variant of microcystic adenoma Few, very large cysts ? association with von Hippel Lindau syndrome Synonyms ➢ Macrocystic serous cystadenoma ➢ Serous oligocystic adenoma

Microcystic Adenoma: Diagnostic Difficulties •

•

Oligocystic variant ➢ Differential diagnosis ❖ Mucinous cystic neoplasm ❖ Pseudocyst Small lesions ➢ Difficult to identify central scar/septations

Oligocystic adenoma

Islet Cell Tumors (Neuroendocrine Tumors) • • •

• •

Tumors of the pancreatic islets Occur in all age groups Variable biologic behavior ➢ Benign or malignant Clinical evidence of hormone production ➢ 65% to 85% Clinically silent ➢ 15% to 35%

Figure 2-7-18

Insulinoma [Figure 2-7-18] •

Somatostatin receptor scintigraphy ➢ Positive in only 60-70% of cases

Zollinger-Ellison Syndrome - Pancreatic Gastrinoma •

Somatostatin receptor scintigraphy ➢ Sensitivity for primary tumor, 58%-75% ➢ Sensitivity for metastatic disease, 100%

Insulinoma

Glucagonoma (DM-Dermatitis Syndrome) MEN I Syndrome (Wermer’s Syndrome) •

•

3P’s ➢ Pituitary ➢ Pancreas ➢ Parathyroid ➢ Other: thymus, thyroid, adrenal gland, GI tract Autosomal dominant ➢ Long arm chromosome 11

Clinically-Silent Islet Cell Tumors [Figures • •

•

Occur at any age Typically large at time of diagnosis ➢ Range, 6 to 20 cm Nonspecific imaging appearance ➢ Necrosis and cystic degeneration common ➢ 25% calcify ➢ Liver metastases are common

Pancreatic Neoplasms

2-7-19 and 2-7-20]

328

Gastrointestinal Radiology

Metastatic Disease •

• •

Figure 2-7-19

Mets to pancreas ➢ Lung, breast ➢ Melanoma ➢ Renal cell ➢ Lymphoma-adjacent nodal disease Mimic primary pancreatic neoplasms ➢ Ductal adenocarcinoma ➢ Islet cell Biliary obstruction in 30%

Clinically-silent islet cell tumor

Summary: Adenocarcinoma • • •

Most common pancreatic neoplasm MDCT ➢ Dual phase ➢ Thin reformations ➢ Overlapping images RESECTABLILITY ➢ Vascular encasement ➢ Adjacent organ invasion ➢ Liver mets

Figure 2-7-20

Summary: IMPN • • •

High index of suspicion Main duct or side branch Imaging ➢ Duct dilatation ➢ Intraductal masses ➢ Bulging papilla

Summary: Solid Pseudopapillary Tumor • •

Renal cell metastatic to the pancreas

Young women Imaging features ➢ Capsule ➢ Solid and cystic ➢ Hemorrhage

Summary: Mucinous Cystic Neoplasm • • • ➢ ➢ ➢

Mucinous cystadenoma Mucinous cystadenocarcinoma Complex cyst Middle-aged women Tail of the pancreas Septations, nodules, calcification

Summary: Microcystic Adenoma • •

Older women Benign neoplasm ➢ Central scar ➢ Honeycomb pattern of cysts ➢ Lobulated margins

Gastrointestinal Radiology

329

Pancreatic Neoplasms

References

Adenocarcinoma 1. Vargas R, Nino-Murcia M, Trueblood W, Jeffrey RB, Jr. MDCT in Pancreatic adenocarcinoma: prediction of vascular invasion and resectability using a multiphasic technique with curved planar reformations. AJR Am J Roentgenol 2004; 182:419-425. 2. Bronstein YL, Loyer EM, Kaur H, et al. Detection of small pancreatic tumors with multiphasic helical CT. AJR Am J Roentgenol 2004; 182:619-623. 3. Roche CJ, Hughes ML, Garvey CJ, et al. CT and pathologic assessment of prospective nodal staging in patients with ductal adenocarcinoma of the head of the pancreas. AJR Am J Roentgenol 2003; 180:475-480. 4. Fletcher JG, Wiersema MJ, Farrell MA, et al. Pancreatic malignancy: value of arterial, pancreatic, and hepatic phase imaging with multi-detector row CT. Radiology 2003; 229:81-90. 5. Prokesch RW, Chow LC, Beaulieu CF, et al. Local staging of pancreatic carcinoma with multi-detector row CT: use of curved planar reformations initial experience. Radiology 2002; 225:759-765. 6. Prokesch RW, Chow LC, Beaulieu CF, Bammer R, Jeffrey RB, Jr. Isoattenuating pancreatic adenocarcinoma at multi-detector row CT: secondary signs. Radiology 2002; 224:764-768. 7. Imbriaco M, Megibow AJ, Camera L, et al. Dual-phase versus single-phase helical CT to detect and assess resectability of pancreatic carcinoma. AJR Am J Roentgenol 2002; 178:1473-1479. 8. Horton KM, Fishman EK. Adenocarcinoma of the pancreas: CT imaging. Radiol Clin North Am 2002; 40:12631272. 9. McNulty NJ, Francis IR, Platt JF, Cohan RH, Korobkin M, Gebremariam A. Multi--detector row helical CT of the pancreas: effect of contrast-enhanced multiphasic imaging on enhancement of the pancreas, peripancreatic vasculature, and pancreatic adenocarcinoma. Radiology 2001; 220:97-102. 10. Tabuchi T, Itoh K, Ohshio G, et al. Tumor staging of pancreatic adenocarcinoma using early- and late-phase helical CT. AJR Am J Roentgenol 1999; 173:375-380. 11. O'Malley ME, Boland GW, Wood BJ, Fernandez-del Castillo C, Warshaw AL, Mueller PR. Adenocarcinoma of the head of the pancreas: determination of surgical unresectability with thin-section pancreatic-phase helical CT. AJR Am J Roentgenol 1999; 173:1513-1518. 12. Hough TJ, Raptopoulos V, Siewert B, Matthews JB. Teardrop superior mesenteric vein: CT sign for unresectable carcinoma of the pancreas. AJR Am J Roentgenol 1999; 173:1509-1512. 13. Keogan MT, Tyler D, Clark L, et al. Diagnosis of pancreatic carcinoma: role of FDG PET. AJR Am J Roentgenol 1998; 171:1565-1570. 14. Zeman RK, Cooper C, Zeiberg AS, et al. TNM staging of pancreatic carcinoma using helical CT. AJR Am J Roentgenol 1997; 169:459-464. 15. Raptopoulos V, Steer ML, Sheiman RG, Vrachliotis TG, Gougoutas CA, Movson JS. The use of helical CT and CT angiography to predict vascular involvement from pancreatic cancer: correlation with findings at surgery. AJR Am J Roentgenol 1997; 168:971-977. 16. Lu DS, Reber HA, Krasny RM, Kadell BM, Sayre J. Local staging of pancreatic cancer: criteria for unresectability of major vessels as revealed by pancreatic-phase, thin-section helical CT. AJR Am J Roentgenol 1997; 168:14391443. 17. Lu DS, Vedantham S, Krasny RM, Kadell B, Berger WL, Reber HA. Two-phase helical CT for pancreatic tumors: pancreatic versus hepatic phase enhancement of tumor, pancreas, and vascular structures. Radiology 1996; 199:697-701. 18. Megibow AJ, Zhou XH, Rotterdam H, et al. Pancreatic adenocarcinoma: CT versus MR imaging in the evaluation of resectability--report of the Radiology Diagnostic Oncology Group. Radiology 1995; 195:327-332. 19. Megibow AJ. Pancreatic adenocarcinoma: designing the examination to evaluate the clinical questions. Radiology 1992; 183:297-303.

Intraductal Papillary Mucinous Neoplasm 1. Cellier C, Cuillerier E, Palazzo L, et al: Intraductal papillary and mucinous tumors of the pancreas: accuracy of preoperative computed tomography, endoscopic retrograde pancreatography and endoscopic ultrasonography, and long-term outcome in a large surgical series. Gastrointest Endosc 47:42, 1998 2. Fukukura Y, Fujiyoshi F, Sasaki M, et al: Intraductal papillary mucinous tumors of the pancreas: thin-section helical CT findings. AJR Am J Roentgenol 174:441, 2000 3. Itai Y, Kokubo T, Atomi Y, et al: Mucin-hypersecreting carcinoma of the pancreas. Radiology 165:51, 1987 4. Lim JH, Lee G, Oh YL: Radiologic spectrum of intraductal papillary mucinous tumor of the pancreas. Radiographics 21:323, 2001 5. Prasad SR, Sahani D, Nasser S, et al: Intraductal papillary mucinous tumors of the pancreas. Abdom Imaging 28:357, 2003 6. Procacci C, Graziani R, Bicego E, et al: Intraductal mucin-producing tumors of the pancreas: imaging findings. Radiology 198:249, 1996 Pancreatic Neoplasms

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7. 8.

Procacci C, Megibow AJ, Carbognin G, et al: Intraductal papillary mucinous tumor of the pancreas: a pictorial essay. Radiographics 19:1447, 1999 Taouli B, Vilgrain V, Vullierme MP, et al: Intraductal papillary mucinous tumors of the pancreas: helical CT with histopathologic correlation. Radiology 217:757, 2000

Mucinous Cystic Neoplasm 1. Buetow PC, Rao P, Thompson LD: From the Archives of the AFIP. Mucinous cystic neoplasms of the pancreas: radiologic-pathologic correlation. Radiographics 18:433, 1998 2. Procacci C, Carbognin G, Accordini S, et al: CT features of malignant mucinous cystic tumors of the pancreas. Eur Radiol 11:1626, 2001 3. Thompson LD, Becker RC, Przygodzki RM, et al: Mucinous cystic neoplasm (mucinous cystadenocarcinoma of low-grade malignant potential) of the pancreas: a clinicopathologic study of 130 cases. Am J Surg Pathol 23:1, 1999

Solid and Pseudopapillary Tumor 1. Buetow PC, Buck JL, Pantongrag-Brown L, et al: Solid and papillary epithelial neoplasm of the pancreas: imaging-pathologic correlation on 56 cases. Radiology 199:707, 1996 2. Cantisani V, Mortele KJ, Levy AD, et al: MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. AJR Am J Roentgenol 181:395, 2003 3. Coleman KM, Doherty MC, Bigler SA: Solid-pseudopapillary tumor of the pancreas. Radiographics 23:1644, 2003 4. Friedman AC, Lichtenstein JE, Fishman EK, et al: Solid and papillary epithelial neoplasm of the pancreas. Radiology 154:333, 1985 Microcystic Adenoma 1. Buck JL, Hayes WS: From the Archives of the AFIP. Microcystic adenoma of the pancreas. Radiographics 10:313, 1990 2. Healy JC, Davies SE, Reznek RH: CT of microcystic (serous) pancreatic adenoma. J Comput Assist Tomogr 18:146, 1994 3. Hough DM, Stephens DH, Johnson CD, et al: Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings. AJR Am J Roentgenol 162:1091, 1994 4. Itai Y, Ohhashi K, Furui S, et al: Microcystic adenoma of the pancreas: spectrum of computed tomographic findings. J Comput Assist Tomogr 12:797, 1988 5. Khurana B, Mortele KJ, Glickman J, et al: Macrocystic serous adenoma of the pancreas: radiologic-pathologic correlation. AJR Am J Roentgenol 181:119, 2003 6. Minami M, Itai Y, Ohtomo K, et al: Cystic neoplasms of the pancreas: comparison of MR imaging with CT. Radiology 171:53, 1989 7. Yeh HC, Stancato-Pasik A, Shapiro RS: Microcystic features at US: a nonspecific sign for microcystic adenomas of the pancreas. Radiographics 21:1455, 2001

Islet Cell Tumors 1. Buetow PC, Miller DL, Parrino TV, et al: Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization. Radiographics 17:453, 1997 2. Buetow PC, Parrino TV, Buck JL, et al: Islet cell tumors of the pancreas: pathologic-imaging correlation among size, necrosis and cysts, calcification, malignant behavior, and functional status. AJR Am J Roentgenol 165:1175, 1995 3. Ichikawa T, Peterson MS, Federle MP, et al: Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection. Radiology 216:163, 2000 4. Semelka RC, Cumming MJ, Shoenut JP, et al: Islet cell tumors: comparison of dynamic contrast-enhanced CT and MR imaging with dynamic gadolinium enhancement and fat suppression. Radiology 186:799, 1993 5. Sheth S, Hruban RK, Fishman EK: Helical CT of islet cell tumors of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol 179:725, 2002 6. Stafford Johnson DB, Francis IR, Eckhauser FE, et al: Dual-phase helical CT of nonfunctioning islet cell tumors. J Comput Assist Tomogr 22:59, 1998 7. Van Hoe L, Gryspeerdt S, Marchal G, et al: Helical CT for the preoperative localization of islet cell tumors of the pancreas: value of arterial and parenchymal phase images. AJR Am J Roentgenol 165:1437, 1995

Metastases 1. Klein KA, Stephens DH, Welch TJ: CT characteristics of metastatic disease of the pancreas. Radiographics 18:369, 1998 2. Ng CS, Loyer EM, Iyer RB, et al: Metastases to the pancreas from renal cell carcinoma: findings on three-phase contrast-enhanced helical CT. AJR Am J Roentgenol 172:1555, 1999 Gastrointestinal Radiology

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Gastric Malignancies

Angela D. Levy, COL, MC, USA Gastric Malignancies • • • • • •

Adenocarcinoma Lymphoma Gastrointestinal Stromal Tumors Carcinoid Kaposi Sarcoma Metastases

•

Fourth most common cancer worldwide1 ➢ Lung, breast, colorectum, stomach, liver

Gastric Adenocarcinoma

1Steward BW and Kleihues P (eds). World Cancer Report. IARC Press. Lyon 2003.

Gastric Adenocarcinoma: Geographic Variation •

•

High incidence areas ➢ China, Japan, South America, Eastern Europe Low incidence areas ➢ North America and Northern Africa

Gastric Adenocarcinoma: Clinical • •

• •

Peak incidence 50 to 70 years of age More common in men ➢ 2:1 Early disease ➢ Asymptomatic Advanced disease ➢ Epigastric pain, bloating, nausea ➢ Early satiety, anorexia, vomiting ➢ Upper GI bleeding

Figure 2-8-1

Gastric Adenocarcinoma: Etiology [Figure 2-8-1] •

• •

Atrophic Gastritis ➢ Helicobacter pylori (80% of cases) ➢ Pernicious Anemia ➢ Partial Gastrectomy Adenomatous Polyps ➢ Polyposis syndromes HNPCC-Hereditary Nonpolyposis Colon Cancer Syndromes (Lynch Syndromes)

Gastritis

Normal

Dysplasia

Atrophic Gastritis

Intramucosal Carcinoma

Intestinal Metaplasia

Invasive Carcinoma

Pathogenesis of gastric adenocarcinoma

Gastric Malignancies

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Gastrointestinal Radiology

WHO Classification of Gastric Adenocarcinoma • • •

Signet Ring [Figure 2-8-2] Papillary [Figure 2-8-3] Mucinous [Figure 2-8-4]

Figure 2-8-3

Figure 2-8-2

Signet ring cell adenocarcinoma produces "linitis plastica"

Figure 2-8-4

Papillary adenocarcinoma produces intraluminal polypoid masses

Early Gastric Carcinoma • •

Japanese Research Society for Gastric Carcinoma Carcinoma limited to the mucosa and submucosa, irrespective of nodal metastases

Mucinous adenocarcinoma produces tumor calcifications

Advanced Gastric Carcinoma [Figure 2-8-5] • • •

Borrmann Classification Most common gastric cancer in the U.S. Tumor penetrating the muscularis propria

•

Morphology ➢ Polypoid ➢ Ulcerating ➢ Infiltrating ➢ Scirrhous

Figure 2-8-5

Advanced Gastric Carcinoma

Gastric Carcinoma: Polypoid [Figure 2-8-6]

Figure 2-8-6

Advanced gastric carcinoma (Borrmann) classification

Polypoid gastric adenocarcinoma Gastrointestinal Radiology

333

Gastric Malignancies

Figure 2-8-7

Ulcerated - Bulk of tumor mass has been replaced by ulceration

Ulcerated Carcinoma Lesser Curvature

Carmen Meniscus Sign [Figure 2-8-7]

Ulcerated gastric adenocarcinoma showing the Carmen meniscus sign

Gastric Carcinoma: Ulcerated

Gastric Carcinoma: Infiltrating [Figure 2-8-8]

Scirrhous Carcinoma [Figure 2-8-9] •

•

Infiltrating tumors with desmoplasia ➢ Signet ring cell carcinomas Radiologic Features ➢ Irregular narrowing ➢ Decreased gastric capacity ➢ Rigidity, “linitis plastica” ➢ Most common in the antrum ➢ Rarely-nodular,distorted folds when the tumor is proximal

Figure 2-8-8

Carcinoma of the Cardia • •

• • • •

One-third of all gastric carcinomas in the U.S. Compared to other gastric carcinomas: ➢ Male predominance, 6:1 ➢ 40% associated with hiatal hernia ➢ Atrophic gastritis and signet ring cell types are uncommon ➢ Association with smoking and alcohol Early lesions are difficult to detect Difficult to differentiate from Barrett’s adenocarcinoma Pseudoachalasia Pitfalls on CT: ➢ GE junction pseudomass ➢ Hiatal hernias

Infiltrating gastric adenocarcinoma

Figure 2-8-9

Scirrhous carcinoma

Figure 2-8-10

Normal Gastric Cardia

Carcinoma of the Cardia [Figure 2-8-10]

Carcinoma of the cardia Gastric Malignancies

334

Gastrointestinal Radiology

Staging •

•

Figure 2-8-11

Endoscopic Ultrasound ➢ Depth of tumor invasion ❖ T stage accuracy 85% ➢ Perigastric nodes ❖ Sensitivity 55%-80% CT ➢ Presence and extent of extragastric spread ➢ Peritoneal ➢ Lymph nodes ➢ Distant metastasis

Staging - Extragastric Spread [Figure 2-8-11 and 2-8-12] •

•

Anatomic Pathways ➢ Lesser omentum ➢ Greater omentum ➢ Transverse mesocolon ➢ Lesser sac ➢ Lower esophagus CT features ➢ Soft tissue stranding ➢ Soft tissue nodules

Routes of extragastric spread of carcinoma

Figure 2-8-12

Staging - Lymphatic Spread

Staging - Krukenberg Metastasis

Staging - Adjacent Organ Invasion • • •

Contiguous tumor Loss of fat planes Focal enlargement of the adjacent organ

Direct Extension and Adjacent Organ Invasion Gastric Lymphoma • • • •

Increasing incidence Up to 10% of gastric malignancies Most common site of extranodal lymphoma Most common site of GI lymphomas

•

Low grade ➢ Dyspepsia, nausea, vomiting High grade: ➢ Bleeding, pain, early satiety, weight loss

Gastric Lymphoma: Clinical •

Mucosa-Associated Lymphoid Tissue (MALT) • •

•

Gastric adenocarcinoma extending into Organized lymphoid tissue located in mucosal sites the greater omentum and lesser sac Native MALT ➢ Intraepithelial lymphocytes ➢ Plasma cells, B and T lymphocytes in the lamina propria ➢ Mesenteric lymph nodes ➢ Peyer’s patches Acquired MALT ➢ Arises as a result of antigenic stimulation (H. pylori infection) ➢ Accumulates before the development of B cell lymphomas

Gastrointestinal Radiology

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Gastric Malignancies

Primary Gastric Lymphoma •

•

MALT lymphoma ➢ Arises from acquired MALT ➢ H. pylori is invariably present ➢ Good clinical prognosis High grade B cell lymphoma ➢ Probably arises from MALT lymphoma

Normal mucosa

Figure 2-8-13

Primary Gastric Lymphoma: Etiology and Pathogenesis Figure 2-8-13] •

H. pylori infection

H. pylori infection

H. pylori gastritis

formation of acquired MALT

low-grade MALT lymphoma

high-grade B-cell lymphoma

Evolution of gastric lymphoma

Gastric Malignancies

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Gastrointestinal Radiology

Low Grade Malt Lymphoma [Figure 2-8-14] •

•

Figure 2-8-14

Clinical ➢ Dyspepsia, nausea, vomiting ➢ Good prognosis Imaging ➢ Nodules ➢ Ulcers ➢ Erosions ➢ Thick rugal folds

Low Grade Malt Lymphoma: CT Features • •

Homogeneous mural thickening Perigastric adenopathy

•

Clinical ➢ Bleeding, pain, ➢ Early satiety, weight loss Imaging ➢ Mural thickening ➢ Adenopathy

High Grade B Cell Lymphoma •

High Grade B Cell Lymphoma:Typical CT Features [Figure 2-8-15]

• •

Homogeneous mural thickening Perigastric adenopathy

•

Cavitation

• • •

Heterogeneous attenuation Enhancement Low attenuation necrosis

•

Wall thickening1 ➢ Tends to be greater (mean, 4 cm) than that of adenocarcinoma ➢ Tends to be homogeneous attenuation Ulceration Polypoid masses Regional adenopathy

High Grade B Cell Lymphoma:Typical CT Features High Grade B Cell Lymphoma: Atypical CT Features

Low grade MALT lymphoma

Figure 2-8-15

CT features of Gastric Lymphoma

• • •

1Buy J, Moss A. AJR 138:859-865, 1982

CT features differentiating Gastric Adenocarcinoma and Lymphoma1 • • • • •

Gastric wall thickening in lymphoma (mean 4 cm) is typically more impressive than adenocarcinoma (mean 1.8 cm) Wall thickening is more homogeneous in lymphoma Perigastric fat is more likely to be preserved in lymphoma Regional adenopathy is common in both Adenopathy in lymphoma tends to be bulky and may extend below the level of the renal veins

High grade B cell lymphoma

1Buy J, Moss A. AJR 138:859-865, 1982

Fork FT, Haglund U, Hogstrom H. Endoscopy 17:5-7, 1985

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Gastric Lymphoma

Mesenchymal Neoplasm of the Stomach • • • • • • • • • • •

Gastrointestinal Stromal Tumor Leiomyoma Leiomyosarcoma Schwannoma Neurofibroma Ganglioneuroma Paraganglioma Granular cell tumor Lipoma, liposarcoma Fibrous lesions Tumors of blood vessels

• • •

Most common mesenchymal neoplasm of the GI tract Arise from the muscularis propria Cellular origin ➢ Primitive "stem cell” like cell ➢ Interstitial cell of Cajal (gut pacemaker cell)

Gastrointestinal Stromal Tumors (GIST)

GIST: Clinical Features • • •

Uncommon tumors Prevalence in the U.S. ➢ 5000 to 6000 new cases per year1 Increased incidence ➢ Neurofibromatosis (NF-1) ➢ KIT germline mutations

1Fletcher CD, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 2002. 33:459-465

GIST: Clinical Features • • • •

Median age 50-60 years 60% occur in the stomach Benign and malignant Defined by KIT expression ➢ Initial diagnosis ➢ Therapy (Gleevac)

What is KIT?

• • • • •

KIT-tyrosine kinase growth factor KIT-tyrosine kinase growth factor receptor CD117 binds to KIT receptors Normally expressed ➢ Hematopoietic stem cells ➢ Germ cells ➢ Interstitial cell of Cajal (gut pacemaker cell) KIT-inhibitor therapy ➢ STI-571, Imatinib [Gleevac]

What Happened to Leiomyomas and Leiomyosarcomas? • •

Very rare Except, ➢ Leiomyomas are the most common benign tumor of the ESOPHAGUS ➢ Leiomyosarcomas of the RETROPERITONEUM

GIST •

Most common sites ➢ Stomach ➢ Small bowel

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•

Figure 2-8-16

➢ Anorectum Unusual sites ➢ Esophagus ➢ Colon ➢ Mesentery/omentum

GIST • •

Spindle Cell GIST Epithelioid GIST

GIST: CD117 (KIT) Positive GIST [Figure 2-8-16] •

Mural Origin ➢ Mural mass ➢ Exophytic mass ➢ Mural and exophytic

GIST: Mural Origin [Figure 2-8-17] Figure 2-8-17

Imaging and morphologic patterns of gastrointestinal stromal tumors: intramural polypoid mass, exophytic mass, or intramural and exophytic mass

Gastrointestinal stromal tumor Gastrointestinal Radiology

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GIST: Internal Hemorrhage and Necrosis

Figure 2-8-18

[Figure 2-8-18]

GIST: Cyst Formation

Gastrointestinal stromal tumor

GIST: Cavity Formation GIST: Calcification

GIST: Metastatic Spread • • •

Direct invasion Peritoneal Hematogenous ➢ Liver

Management of Metastatic and Recurrent Disease •

•

Kit-inhibitor therapy ➢ Gleevac ➢ Clinical trials of other kit-inhibitors Imaging features of treated metastasis ➢ Cystic transformation ➢ Pet important to determine residual functional tumor

Figure 2-8-19

Imaging of Recurrent Disease

Choi H, Charnsangavej C, de Castro Faria S, et al. CT evaluation of the response of gastrointestinal stromal tumors after imatinib mesylate treatment: a quantitative analysis correlated with FDG PET findings. AJR Am J Roentgenol 2004; 183:1619-1628.

Differential Diagnosis Gastric GIST vs. Adenocarcinoma [Figures 2- 7-19 and 2-7-20]

Gastric Carcinoid •

• •

Type I: autoimmune chronic atrophic gastritis ➢ Hypergastrinemia ➢ Multiple, small ➢ Benign biologic behavior Type II: MEN I and Zollinger Ellison syndrome ➢ Hypergastrinemia ➢ Multiple, small ➢ Benign biologic behavior Type III: sporadic ➢ Single ➢ Aggressive biologic behavior

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Carcinoid: Imaging Features • • • • •

Figure 2-8-20

Submucosal mass Central ulceration-”bull’s eye” Pedunculated polypoid lesions, rarely Large ulcerative masses Thick, rugal folds if hypergastrinemia is present

Carcinoid: “Bull′′s Eye Lesion”

Carcinoid: Pedunculated Polyps Kaposi Sarcoma [Figure 2-8-21] • • • •

AIDS patients Cutaneous KS usually Stomach, duodenum, and small bowel most common GI locations Radiologic features ➢ Submucosal masses ➢ “Bull’s-eye”appearance ➢ Polypoid masses ➢ Infiltrating variant, rare

Metastases • •

Melanoma, breast, lung Radiologic features ➢ Ulcerating masses ➢ Polyps ➢ Infiltrating ➢ “Linitis Plastica”

GIST vs. lymphoma

Figure 2-8-21

Summary: Adenocarcinoma • • •

•

H. pylori Chronic atrophic gastritis Primary tumor morphology ➢ Polypoid ➢ Ulcerating ➢ Infiltrating ➢ Schirrous CT: extragastric spread

Summary: Lymphoma • • •

H. pylori Low grade MALT to high grade B cell Compared to adenocarcinoma ➢ Greater wall thickening ➢ Bulky, more extensive adenopathy

Summary: GIST • •

• •

Most common mesenchymal neoplasm KIT reactivity ➢ Diagnosis ➢ Gleevac therapy Classic mural masses on barium May have extensive extragastric growth Bull’s eye lesions from Kaposi Sarcoma. Endoscopy shows hemorrhagic masses

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Summary: Bull’s Eye Lesions • •

• • • •

Carcinoid Metastasis ➢ (Breast, Lung, Melanoma) Kaposi’s Sarcoma Lymphoma Adenocarcinoma Ectopic Pancreas

References

Gastric Carcinoma 1. Balthazar EJ, Siegel SE, Megibow AJ, et al: CT in patients with scirrhous carcinoma of the GI tract: imaging findings and value for tumor detection and staging. AJR 165:839, 1995 2. Gore RM: Gastric cancer. Clinical and pathologic features. Radiol Clin North Am 35:295, 1997 3. Gore RM, Levine MS, Ghahremani GG, et al: Gastric cancer. Radiologic diagnosis. Radiol Clin North Am 35:311, 1997 4. Levine MS, Kong V, Rubesin SE, et al: Scirrhous carcinoma of the stomach: radiologic and endoscopic diagnosis. Radiology 175:151, 1990 5. Longmire WP, Jr.: A current view of gastric cancer in the US. Ann Surg 218:579, 1993 6. Miller FH, Kochman ML, Talamonti MS, et al: Gastric cancer. Radiologic staging. Radiol Clin North Am 35:331, 1997 7. Morales TG: Adenocarcinoma of the gastric cardia. Dig Dis 15:346, 1997 8. Parsonnet J: Helicobacter pylori and gastric cancer. Gastroenterol Clin North Am 22:89, 1993 9. Parsonnet J, Friedman GD, Vandersteen DP, et al: Helicobacter pylori infection and the risk of gastric carcinoma. N Engl J Med 325:1127, 1991 10. Sipponen P, Marshall BJ: Gastritis and gastric cancer. Western countries. Gastroenterol Clin North Am 29:579, 2000

Gastric Lymphoma 1. An SK, Han JK, Kim YH, et al: Gastric mucosa-associated lymphoid tissue lymphoma: spectrum of findings at double-contrast gastrointestinal examination with pathologic correlation. Radiographics 21:1491, 2001 2. Buy JN, Moss AA: Computed tomography of gastric lymphoma. AJR 138:859, 1982 3. Choi D, Lim HK, Lee SJ, et al: Gastric mucosa-associated lymphoid tissue lymphoma: helical CT findings and pathologic correlation. AJR 178:1117, 2002 4. Jaffe ES, Harris NL, Stein H, et al (eds): World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues), Lyon: IARC Press, 2001 5. Kim YH, Lim HK, Han JK, et al: Low-grade gastric mucosa-associated lymphoid tissue lymphoma: correlation of radiographic and pathologic findings. Radiology 212:241, 1999 6. Levine MS, Elmas N, Furth EE, et al: Helicobacter pylori and gastric MALT lymphoma. AJR Am J Roentgenol 166:85, 1996 7. Levine MS, Rubesin SE, Pantongrag-Brown L, et al: Non-Hodgkin's lymphoma of the gastrointestinal tract: radiographic findings. AJR Am J Roentgenol 168:165, 1997 8. Megibow AJ, Balthazar EJ, Naidich DP, et al: Computed tomography of gastrointestinal lymphoma. AJR 141:541, 1983 9. Parsonnet J, Hansen S, Rodriguez L, et al: Helicobacter pylori infection and gastric lymphoma. N Engl J Med 330:1267, 1994 10. Wotherspoon AC, Doglioni C, de Boni M, et al: Antibiotic treatment for low-grade gastric MALT lymphoma. Lancet 343:1503, 1994 11. Yoo CC, Levine MS, Furth EE, et al: Gastric mucosa-associated lymphoid tissue lymphoma: radiographic findings in six patients. Radiology 208:239, 1998 Gastrointestinal Stromal Tumor (GIST) 1. Burkill GJ, Badran M, Al-Muderis O, et al: Malignant gastrointestinal stromal tumor: distribution, imaging features, and pattern of metastatic spread. Radiology 226:527, 2003 2. Chen MY, Bechtold RE, Savage PD: Cystic changes in hepatic metastases from gastrointestinal stromal tumors (GISTs) treated with Gleevec (imatinib mesylate). AJR 179:1059, 2002 3. Dematteo RP, Heinrich MC, El-Rifai WM, et al: Clinical management of gastrointestinal stromal tumors: before and after STI-571. Hum Pathol 33:466, 2002 4. Fletcher CD: Clinicopathologic correlations in gastrointestinal stromal tumors. Hum Pathol 33:455, 2002

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Fletcher CD, Berman JJ, Corless C, et al: Diagnosis of gastrointestinal stromal tumors: A consensus approach. Hum Pathol 33:459, 2002 6. Levy AD, Remotti HE, Thompson WM, et al: From the Archives of the AFIP: Gastrointestinal Stromal Tumors: Radiologic Features with Pathologic Correlation. RadioGraphics 23:283, 2003 7. Miettinen M, El-Rifai W, Sobin LH, et al: Evaluation of malignancy and prognosis of gastrointestinal stromal tumors: a review. Hum Pathol 33:478, 2002 8. Miettinen M, Lasota J: Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 438:1, 2001 9. Nishida T, Kumano S, Sugiura T, et al: Multidetector CT of high-risk patients with occult gastrointestinal stromal tumors. AJR Am J Roentgenol 180:185, 2003 10. Sharp RM, Ansel HJ, Keel SB: Best cases from the AFIP: gastrointestinal stromal tumor. Armed Forces Institute of Pathology. RadioGraphics 21:1557, 2001 5.

Gastric Carcinoid 1. Balthazar EJ, Megibow A, Bryk D, et al: Gastric carcinoid tumors: radiographic features in eight cases. AJR Am J Roentgenol 139:1123, 1982 2. Berger MW, Stephens DH: Gastric carcinoid tumors associated with chronic hypergastrinemia in a patient with Zollinger-Ellison syndrome. Radiology 201:371, 1996 3. Binstock AJ, Johnson CD, Stephens DH, et al: Carcinoid tumors of the stomach: a clinical and radiographic study. AJR 176:947, 2001 4. Borch K, Renvall H, Kullman E, et al: Gastric carcinoid associated with the syndrome of hypergastrinemic atrophic gastritis. A prospective analysis of 11 cases. Am J Surg Pathol 11:435, 1987 5. Ho AC, Horton KM, Fishman EK: Gastric carcinoid tumors as a consequence of chronic hypergastrinemia: spiral CT findings. Clin Imaging 24:200, 2000

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Abdominal Non Hodgkin Lymphoma Angela D. Levy, COL, MC, USA Objectives • •

•

Definition Patterns of disease ➢ NHL Adenopathy ➢ Gastrointestinal Lymphoma Immunodeficiency-related lymphomas ➢ Post-transplantation Lymphoproliferative Disorder (PTLD) ➢ AIDS-related Lymphomas

Lymphoid Neoplams • •

•

2001 WHO classification of Hematological Malignancies Three major categories ➢ B cell, T and NK (natural killer) cell, Hodgkin lymphoma NHL ➢ Large group of diverse diseases ➢ Indolent, aggressive, and very aggressive

Non-Hodgkin Lymphoma (NHL) •

• • • •

4% of all cancers ➢ 5th most common cancer ➢ 5th leading cause of cancer death 4 times more common than Hodgkin lymphoma Male to female ratio: 1.3 to 1 Median age 55 years Third most common cancer mortality in children under age 15

Non-Hodgkin Lymphoma •

•

Rising incidence ➢ True increase in incidence ➢ Improved identification and understanding ➢ HIV infection ➢ Organ transplants Immunodeficiency increases risk ➢ Wiskott-Aldrich syndrome ➢ Ataxia telangiectasia ➢ Long-term immunosuppressive therapy

Role of Imaging in Newly Diagnosed NHL • •

Clinical Staging: ➢ Ann Arbor Staging Classification ➢ Tumor bulk has important prognostic significance in intermediate and high grade NHL Identification of nodal and extranodal sites ➢ Mesenteric adenopathy ➢ GI tract ➢ Liver ➢ Spleen

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Role of Imaging in Management of NHL • •

•

Figure 2-9-1

CT and PET complimentary CT imaging ➢ Define anatomy ➢ Response to therapy Limitations of CT ➢ Decrease in node size is not a reliable indicator of response ➢ Cannot differentiate residual tumor vs. fibrosis vs. necrosis

Well-defined mesenteric nodes in NHL Raanani P, Shasha Y, Perry C, et al. Is CT scan still necessary for staging in Hodgkin and non-Hodgkin lymphoma patients in the PET/CT era? Ann Oncol 2005.

PET/CT in Management of NHL •

•

Initial staging ➢ Equivalent or better to CT alone Prognosis ➢ Persistent uptake after chemotherapy predicts treatment failure/early recurrence

Figure 2-9-2

Kumar R et al. Utility of fluorodeoxyglucosePET imaging in the management of patients with Hodgkin’s and non-Hodgkin’s lymphomas. RCNA 2004. 42:1083-1100

Sandwich sign of the mesentery in NHL

NHL: Abdomimal Adenopathy • • •

• •

Mesentery Retroperitoneum CT Patterns ➢ Discrete rounded nodes ➢ Confluent nodes ➢ Ill-defined masses ➢ Mesenteric caking ➢ Stellate mesentery CT attenuation at diagnosis ➢ Homogeneous in most cases ➢ Heterogeneous in cases with aggressive histology CT attenuation during treatment ➢ Heterogeneous from necrosis ➢ Calcification may occur

Figure 2-9-3

Confluent retroperitoneal nodes in NHL

NHL: Discrete Nodes [Figure 2-9-1] NHL: Sandwich Sign [Figure 2-9-2]

Figure 2-9-4

NHL: Confluent Nodes [Figure 2-9-3] NHL: Confluent Nodes in AIDSAssociated NHL [Figure 2-9-4]

NHL: Mesenteric Caking in AIDSAssociated NHL Confluent nodes in AIDS-associated NHL. There is extensive necrosis and heterogeneous enhancement

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Differential Diagnosis: Mesenteric Masses • • • • • • •

• • •

Lymphoma Metastasis Carcinoid Castleman disease Mesenteric fibromatosis Gastrointestinal Stromal Tumor (GIST) Granulomatous disease ➢ Tuberculosis ➢ Histoplasmosis ➢ Sarcoid Sprue Whipple disease Inflammatory Pseudotumor

Castleman Disease •

•

Hyperenhancing masses ➢ Homogeneous or heterogeneous May calcify

Mesenteric Fibromatosis (Desmoid Tumor) •

• •

Homogeneous attenuation ➢ Soft tissue or low attenuation myxoid stroma Heterogeneous attenuation ➢ Foci of low attenuation myxoid stroma May infiltrate adjacent bowel

Carcinoid • •

Primary lesion in bowel Mesenteric metastasis ➢ Spiculations/tethering of mesentery ➢ May calcifiy

Gastrointestinal Stromal Tumor (GIST) • •

Mesenteric masses of GIST ➢ Primary to small bowel or mesentery ➢ Metastatic disease Heterogeneous attenuation ➢ Low attenuation hemorrhage/necrosis

Sarcoid • •

Small, discrete nodes Retrocural nodes atypical in sarcoid

•

Lymphoma that presents with GI disease and no other major site of involvement Most common extranodal site of NHL ➢ 4.4% of all lymphomas ➢ 25% of all extranodal lymphomas Almost exclusively NHL Stomach is the most common site in US and Western Europe Small bowel is the most common site in the Mediterranean, Northern Africa, Middle East B-cell lymphomas ➢ MALT lymphomas ❖ Immunoproliferative small intestinal disease, “alpha-heavy chain disease” ➢ Mantle cell lymphoma (multiple lymphomatous polyposis) ➢ Burkitt and Burkitt-type lymphoma ➢ Nodal equivalents (diffuse large B-cell lymphomas, follicular, etc)

Gastrointestinal Lymphoma • • • • •

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•

T-cell lymphoma ➢ Enteropathy-type T-cell lymphoma (ETTL)

NHL: Small Intestine • • •

• •

Approximately 25% of all primary small bowel malignancies Male predominance, mean age 60 years Clinical presentation: ➢ Weight loss, pain, bleeding ➢ Intussusception, obstruction, perforation Ileum most common location and duodenum least common Multiple lesions in 10 to 25% of cases

Figure 2-9-5

NHL Small Bowel: Radiologic Patterns •

• • •

Mural infiltration ➢ Fold thickening ➢ Circumferential wall thickening ➢ Luminal dilatation Polypoid nodules ➢ Solitary ➢ Multiple (lymphomatous polyposis) Cavities Mesenteric disease

NHL Small Intestine: Tumor Morphology [Figure 2-9-5] NHL Small Intestine: Mural Infiltration [Figures 2-9-6 to 2-9-8]

Figure 2-9-7

Lymphoma histology shows tumor extension from mucosa to serosa

Figure 2-9-6

Ileal lymphoma showing mural infiltration, ulceration, and nodularity

Ileal lymphoma extending into small bowel mesentery Gastrointestinal Radiology

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NHL Small Intestine: Cavitary Mass [Figure 2-9-9]

Figure 2-9-8

Figure 2-9-9

Cavitary mass

NHL Small Intestine: Adjacent Mesenteric Disease [Figure 2-9-10]

Figure 2-9-10

Mesenteric mass engulfing small intestine

Burkitt Lymphoma • • •

• •

High grade B-cell lymphoma More common in males Endemic ➢ African Burkitt, related to EBV ➢ Head and neck disease Sporadic ➢ Western countries, not related to EBV ➢ ileocecal region of children Clinical presentation ➢ Intestinal obstruction ➢ Intussusception

Mural infiltration with luminal dilatation

Mantle Cell Lymphoma [Figure 2-9-11]

Mantle Cell Lymphoma (Multiple Lymphomatous Polyposis) • • •

•

Histologically resembles the mantle zone of the lymph follicle Median age 65, male predominance Clinical presentation ➢ Abdominal pain and bloody stools Imaging ➢ Multiple polyps, 0.5 to 2.0 cm ➢ Solitary polyp ➢ Most common in the ileocecal region

Figure 2-9-11

Polypoid masses of mantle cell lymphoma Abdominal Non-Hodgkin Lymphoma

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Enteropathy-Type T-cell Lymphoma (ETTL) [Figure 2-9-12] •

• • •

Associated with celiac disease (Sprue) ➢ Sixth to seventh decade of life Most common site jejunum Gross pathology ➢ Ulcerated plaques ➢ Strictures Poor prognosis

NHL Small Intestine: Differential Diagnosis • • • • • • • •

Adenocarcinoma GIST Carcinoid Metastases Crohn disease Tuberculosis Mesenteric fibromatosis Causes of fold thickening ➢ Sprue ➢ Hemorrhage ➢ Edema ➢ Ischemia

Figure 2-9-12

Enteropathy type T-cell lymphoma

Figure 2-9-13

Malignant Melanoma Metastases Gastrointestinal Stromal Tumor

Jejunal Adenocarcinoma

Tuberculosis / Lymphoma

Post-transplantation Lymphoproliferative Disorder (PTLD) • • • •

Spectrum of benign and malignant disorders Variable incidence ➢ 1% renal transplants ➢ 10% combined heart/lung ➢ 10% of patients on cyclosporine and OKT3 Association with EBV infection Lung, GI tract

PTLD [Figures 2- 9-13 and 2-9-14] •

•

Pathologic Features ➢ Driven by Epstein-Barr Virus infection ➢ Diffuse polyclonal expansion Pathogenesis of post-transplantation ➢ Reduced T-cell control lymphoproliferative disorder ➢ Malignant transformation Clinical ➢ May respond to reducing immunosuppression, anti-virals, surgery

Figure 2-9-14

AIDS-Related Lymphoma • • • •

Second most common neoplasm in HIV infection AIDS defining illness Incidence is 4% to 10% in the AIDS population Three categories ➢ Systemic (nodal and/or extranodal) ➢ Primary CNS

Gastrointestinal Radiology

Colonic lymphoma in a patient with a renal transplant 349

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•

➢ Body cavity-based (primary effusion) lymphomas Major histologic subtypes ➢ Burkitt lymphoma ➢ Burkitt-like lymphoma ➢ Large cell lymphoma ➢ Large cell immunoblastic lymphoma

AIDS-Related Lymphoma • • •

•

25% have GI tract disease Higher incidence of mesenteric disease than non-AIDS lymphomas Aggressive histology and biologic behavior ➢ Atypical radiologic features ➢ Hemorrhage ➢ Necrosis Unique subtypes ➢ Body cavity-based lymphoma (Kaposi’s sarcoma-associated herpes virus (KSHV)) ➢ Anorectal lymphoma

Figure 2-9-15

Primary Peritoneal Lymphoma [Figure 2-9-15]

Colonic Lymphoma

Anorectal Lymphoma [Figure 2-9-16] Summary • •

• •

Primary peritoneal lymphoma in AIDS Spectrum of Adenopathy ➢ GI lymphomas are predominantly NHL ➢ Unique subtypes involve the bowel ➢ Various patterns: infiltrating masses, luminal dilatation, polyps, cavitary masses, mesenteric masses AIDS-related ➢ Aggressive behavior ➢ Unusual sites, unusual manifestations PTLD Figure ➢ Colon, liver

Patterns of Adenopathy

2-9-16

Patterns of Small Bowel Disease 1-Mural Infiltration 2-Polyps

Anorectal lymphoma in AIDS

3-Cavitary Masses

4-Mesenteric Masses

AIDS-Related Lymphomas

Post Transplantation Lymphoproliferative Disorder (PTLD)

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References

Ann Arbor Staging of Gastrointestinal Lymphomas Stage IE: Confined to the wall of the stomach or bowel Stage II1E: Regional lymph nodes contiguous to primary site Regional lymph nodes not contiguous to primary site Stage II2E: Stage III: Lymph nodes on both sides of the diaphragm, spleen (IIIS), or both (IIIE&S) Stage IV: Bone marrow or other non-hematolymphoid organ World Health Organization Classification of B-Lymphoid Neoplasms Precursor B-cell Neoplasms Precursor B-lymphoblastic leukemia/lymphoma Mature (peripheral) B-cell Neoplasms B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma B-cell prolymphocytic leukemia Lymphoplasmacytic lymphoma Splenic marginal zone B-cell lymphoma Hairy cell leukemia Plasma cell myeloma/plasmacytoma Extranodal marginal zone B-cell lymphoma of MALT type Nodal marginal zone B-cell lymphoma Follicular lymphoma Mantle-cell lymphoma Diffuse large B-cell lymphoma Burkitt lymphoma

World Health Organization Classification of T- and NK-Lymphoid Neoplasms Precursor T-cell Neoplasms Precursor T-lymphoblastic lymphoma/leukemia Mature (peripheral) T-cell Neoplasms T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia Aggressive NK-cell leukemia Adult t-cell lymphoma/leukemia Extranodal NK/T-cell lymphoma Enteropathy-type T-cell lymphoma Hepatosplenic gamma-delta T-cell lymphoma Subcutaneous panniculitis-like T-cell lymphoma Mycosis fungoides/Sezary syndrome Peripheral T-cell lymphoma, not otherwise characterized Angioimmunoblastic T-cell lymphoma Anaplastic large-cell lymphoma

Lymphoma Classification 1. Harris NL, Jaffe ES, Diebold J, et al: The World Health Organization classification of neoplasms of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting--Airlie House, Virginia, November, 1997. Hematol J 1:53, 2000 2. Jaffe ES, Harris NL, Stein H, et al (eds): World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues), Lyon: IARC Press, 2001 Imaging of non Hodgkin lymphoma 1. Byun JH, Ha HK, Kim AY, et al: CT Findings in Peripheral T-Cell Lymphoma Involving the Gastrointestinal Tract. Radiology 227:59, 2003 2. Choi D, Lim HK, Lee SJ, et al: Gastric mucosa-associated lymphoid tissue lymphoma: helical CT findings and pathologic correlation. AJR 178:1117, 2002 3. Crump M, Gospodarowicz M, Shepherd FA: Lymphoma of the gastrointestinal tract. Semin Oncol 26:324, 1999 4. Gossios K, Katsimbri P, Tsianos E: CT features of gastric lymphoma. Eur Radiol 10:425, 2000 5. Isaacson PG: Gastrointestinal lymphoma. Hum Pathol 25:1020, 1994 6. Isaacson PG: Gastrointestinal lymphomas of T- and B-cell types. Mod Pathol 12:151, 1999 7. Isaacson PG: Intestinal lymphoma and enteropathy. J Pathol 177:111, 1995

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8. 9.

10. 11.

12. 13. 14. 15. 16.

Isaacson PG: Mucosa-associated lymphoid tissue lymphoma. Semin Hematol 36:139, 1999 Isaacson PG, MacLennan KA, Subbuswamy SG: Multiple lymphomatous polyposis of the gastrointestinal tract. Histopathology 8:641, 1984 Kessar P, Norton A, Rohatiner AZ, et al: CT appearances of mucosa-associated lymphoid tissue (MALT) lymphoma. Eur Radiol 9:693, 1999 Levine MS, Elmas N, Furth EE, et al: Helicobacter pylori and gastric MALT lymphoma. AJR Am J Roentgenol 166:85, 1996 Levine MS, Rubesin SE, Pantongrag-Brown L, et al: Non-Hodgkin's lymphoma of the gastrointestinal tract: radiographic findings. AJR Am J Roentgenol 168:165, 1997 Megibow AJ, Balthazar EJ, Naidich DP, et al: Computed tomography of gastrointestinal lymphoma. AJR 141:541, 1983 Park MS, Kim KW, Yu JS, et al: Radiographic findings of primary B-cell lymphoma of the stomach: low-grade versus high-grade malignancy in relation to the mucosa-associated lymphoid tissue concept. AJR 179:1297, 2002 Rodallec M, Guermazi A, Brice P, et al: Imaging of MALT lymphomas. Eur Radiol 12:348, 2002 Sheth S, Horton KM, Garland MR, et al: Mesenteric Neoplasms: CT Appearances of Primary and Secondary Tumors and Differential Diagnosis. Radiographics 23:457, 2003

AIDS-related lymphomas 1. Albin J, Lewis E, Eftekhari F, et al: Computed tomography of rectal and perirectal disease in AIDS patients. Gastrointest Radiol 12:67, 1987 2. Brar HS, Gottesman L, Surawicz C: Anorectal pathology in AIDS. Gastrointest Endosc Clin N Am 8:913, 1998 3. Burkes RL, Meyer PR, Gill PS, et al: Rectal lymphoma in homosexual men. Arch Intern Med 146:913, 1986 4. Ferrozzi F, Tognini G, Mulonzia NW, et al: Primary effusion lymphomas in AIDS: CT findings in two cases. Eur Radiol 11:623, 2001 5. Gottlieb CA, Meiri E, Maeda KM: Rectal non-Hodgkin's lymphoma: a clinicopathologic study and review. Henry Ford Hosp Med J 38:255, 1990 6. Ioachimm HL, Antonescu C, Giancotti F, et al: EBV-associated anorectal lymphomas in patients with acquired immune deficiency syndrome. Am J Surg Pathol 21:997, 1997? 7. Munn S: Imaging HIV/AIDS. Burkitt's lymphoma. AIDS Patient Care STDS 16:395, 2002 Post-transplantation lymphoproliferative disorder 1. Meador TL, Krebs TL, Cheong JJ, et al: Imaging features of posttransplantation lymphoproliferative disorder in pancreas transplant recipients. AJR 174:121, 2000 2. Pickhardt PJ, Siegel MJ: Abdominal manifestations of posttransplantation lymphoproliferative disorder. AJR Am J Roentgenol 171:1007, 1998 3. Pickhardt PJ, Siegel MJ: Posttransplantation lymphoproliferative disorder of the abdomen: CT evaluation in 51 patients. Radiology 213:73, 1999 4. Pickhardt PJ, Siegel MJ, Hayashi RJ, et al: Posttransplantation lymphoproliferative disorder in children: clinical, histopathologic, and imaging features. Radiology 217:16, 2000 5. Tubman DE, Frick MP, Hanto DW: Lymphoma after organ transplantation: radiologic manifestations in the central nervous system, thorax, and abdomen. Radiology 149:625, 1983 6. Vrachliotis TG, Vaswani KK, Davies EA, et al: CT findings in posttransplantation lymphoproliferative disorder of renal transplants. AJR Am J Roentgenol 175:183, 2000 7. Wu L, Rappaport DC, Hanbidge A, et al: Lymphoproliferative disorders after liver transplantation: imaging features. Abdom Imaging 26:200, 2001

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Small Intestinal Neoplasms Angela D. Levy, COL, MC, USA

Small Intestinal Neoplasms: Introduction •

• • • •

• •

Small bowel neoplasms ➢ Uncommon ➢ Spectrum from hamartoma to benign to malignant ➢ Majority of malignant small bowel tumors are metastatic ➢ Primary small bowel tumors account for 1% to 2% of GI malignancies Most common benign small bowel tumors ➢ Adenoma ➢ GIST (may be benign or malignant) ➢ Lipoma Location of benign tumors ➢ Least common in duodenum ➢ Most common in ileum Primary malignant small bowel tumors ➢ Strong associations with chronic inflammation: sprue, Crohn disease ➢ Association with precursor conditions: FAP, MEN, NF1, Peutz-Jegher Trend in distribution of malignant tumors is relative to histology ➢ Adenocarcinoma most common in duodenum ➢ Carcinoid most common in ileum ➢ Lymphoma most common in ileum ➢ GISTs, even distribution throughout small bowel Clinical features ➢ Many occult and asymtomatic until advanced disease ➢ Symptoms dependent upon location and tumor morphology ➢ Symptoms are similar in benign, malignant, primary or secondary Diagnostic approach ➢ Differential diagnosis based upon location, morphology, and associated conditions

Small Intestinal Neoplasms: Objectives •

•

Case based approach ➢ Tumors of proximal duodenum ➢ Periampullary tumors ➢ Polypoid jejunal/ileal tumors ➢ Annular tumors of jejunum and ileum ➢ Tumors associated with adjacent disease in the mesentery ➢ Tumors associated with NF1 Case based approach ➢ Brunner gland lesions ➢ Adenomas ➢ Adenocarcinomas ➢ Carcinoid ➢ GISTs ➢ Metastatic disease

38-year-old man with recent onset of abdominal pain

Figure 2-10-1

[Figure 2-10-1]

Brunner gland hamartoma Gastrointestinal Radiology

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Differential Diagnosis: Proximal Duodenal Polyp •

•

Nonneoplastic ➢ Brunner gland hamartoma ➢ Heterotopia ➢ Prolapsed antral mucosa ➢ Peutz-Jegher hamartoma Neoplastic ➢ Adenoma ➢ Adenocarcinoma ➢ GIST ➢ Carcinoid ➢ Prolapsed gastric neoplasm

Brunner Gland Hamartoma [Figure 2-10-1] •

• • • •

Solitary hamartoma ➢ Brunner glands, muscular, and fatty elements ➢ Heterotopic pancreatic acini and ducts Synonym: Brunner gland adenoma Most common in duodenal bulb Clinical ➢ Peak incidence, 4th to 6th decade ➢ Asymptomatic or rarely, obstruction or bleeding Treatment ➢ Resection

Brunner Gland Hamartoma •

•

•

Imaging features ➢ Solitary ➢ Sharply circumscribed polyp ➢ Proximal duodenum Homogenous CT attenuation ➢ Composed of mostly glandular elements Heterogeneous CT attenuation ➢ Abundant fat, smooth muscle, and cystic change

Brunner Gland Hyperplasia • •

•

Smaller, multifocal version of Brunner hamartoma Clinical associations ➢ Duodenal ulcers ➢ Gastric hypersecretory states Treatment ➢ None

Figure 2-10-2

Brunner Gland Hyperplasia [Figure 2-10-2] •

Differential diagnosis ➢ Brunner gland hyperplasia ➢ Lymphoid hyperplasia ➢ Duodenitis ➢ Adenomas in FAP ➢ Hamartomas in Peutz-Jegher ➢ Heterotopia

Brunner gland hyperplasia

Small Intestinal Neoplasms

354

Gastrointestinal Radiology

68-year-old woman with recurrent pancreatitis [Figure 2-10-3]

Differential Diagnosis Periampullary Duodenal Mass •

•

Figure 2-10-3

Nonneoplastic ➢ Choledochocele ➢ Duplication cyst ➢ Peutz-Jegher hamartoma Neoplastic ➢ Adenoma/adenocarcinoma ➢ Carcinoid/NF1 ➢ GIST Periampullary tubulovillous adenoma ➢ Metastatic disease ➢ Adjacent pancreatic or ampullary adenocarcioma

Figure 2-10-4

Tubulovillous Adenoma Adenoma •

• •

Benign intraepithelial neoplasm composed of dysplastic cells ➢ Tubular, villous, or tubulovillous histology ➢ May progress to adenocarcinoma Locations ➢ 80% are periampullary Increased incidence ➢ Familial adenomatous polyposis, FAP ➢ Hereditary nonpolyposis colon carcinoma, HNPCC

Periampullary Adenocarcinoma •

Imaging features ➢ Biliary obstruction ➢ Duodenal mural thickening or polypoid mass ➢ May extend into adjacent pancreas and/ampulla

Periampullary adenocarcinoma

Figure 2-10-5

Adenocarcinoma Duodenum: Ampullary/Periampullary [Figures 2-10-4 and 2-10-5]

• • •

May arise from periampullary duodenal mucosa May arise from ampulla May be mixed location ➢ Origin not clear

Small Bowel Adenocarcinoma •

•

More common in proximal small intestine1 ➢ 55% periampullary/ampullary ➢ 10% duodenum ➢ 25% jejunum ➢ 10% ileum Association with colonic adenocarcinoma ➢ APC gene ➢ Mismatch repair gene

Ampullary adenocarcinoma

1Riddel RH, Petras RE, Williams GT, Sobin LH. Atlas of Tumor Pathology:Tumors of the Intestines. AFIP 2003

Gastrointestinal Radiology

355

Small Intestinal Neoplasms

Small Bowel Adenocarcinoma •

•

Most patients between 50 and 60 years ➢ Mean age, 55 years Predisposing conditions ➢ Inherited syndromes: FAP, HNPCC, Peutz-Jegher, NF1 ➢ Chronic inflammation: sprue, Crohn disease, ileostomies, ileal pouches, bypassed bowel

Differential Diagnosis: Small Bowel, Intussuscepting Mass •

•

Benign ➢ Adenoma ➢ Peutz Jegher polyp ➢ Lipoma ➢ Uncommon ❖ Neurofibroma ❖ Schwannoma ❖ Inflammatory fibroid polyp ❖ Heterotopia Malignant ➢ Metastasis ➢ Adenocarcinoma ➢ Lymphoma ➢ GIST ➢ Carcinoid

Figure 2-10-6

Jejunal Adenocarcinoma: Annular [Figure 2-10-6]

•

CT features ➢ Focal, annular mural thickening ➢ Spiculated or irregular margins ➢ +/- mesenteric adenopathy

Adenocarcinoma of the jejunum with annular morphology

Figure 2-10-7

Ileal Adenocarcinoma: Annular and Infiltrating [Figure 2-10-7]

Differential Diagnosis: Jejunal or Ileal Stricture •

•

Neoplastic ➢ Adenocarcinoma ➢ Carcinoid ➢ Lymphoma ➢ Metastasis Nonneoplastic ➢ Crohn disease ➢ Celiac disease ➢ NSAID (tend to be web-like) ➢ Ischemia ➢ Tuberculosis ➢ Heterotopia ➢ Radiation

Adenocarcinoma of the ileum with annular and infiltrating morphology

Figure 2-10-8

Ileal Adenocarcinoma: Cavitary Mass [Figure 2-10-8] • •

Unusual presentation for adenocarcinoma More aggressive histology ➢ Poorly differentiated ➢ Endocrine features mixed with adenocarcinoma

Small Intestinal Neoplasms

Adenocarcinoma of the ileum manifesting as a cavitary mass

356

Gastrointestinal Radiology

Differential Diagnosis: Jejunal or Ileal Cavitary Mass • •

• •

Figure 2-10-9

Metastatic disease Lymphoma ➢ Look for homogenous attenuation tumor GIST Adenocarcinoma

50-year-old man with abdominal pain and diarrhea [Figures 2-10-9 and 2-10-10]

•

CT findings ➢ Fixed segment of ileum ➢ Mural thickening ➢ Adjacent spiculated mesenteric mass

Carcinoid • • •

•

Well-differentiated endocrine neoplasms All have malignant potential Classification ➢ Foregut, stomach and proximal duodenum ➢ Midgut (60% to 80%), distal duodenum, jejunum, ileum, appendix, ascending colon, proximal transverse colon ➢ Hindgut, distal transverse colon, descending colon, rectum Spectrum of clinical/imaging features ➢ Population and type of endocrine cell changes throughout the bowel ➢ Variety of hormones produced ➢ Biologic behavior ranges from benign to malignant

•

Most common in first and second portion ➢ Low-grade malignancies ➢ Gastrin or somatostatin production most common ➢ Periampullary tumors = somatostatin producing and NF1 association Associations ➢ Zollinger-Ellison syndrome ➢ Multiple endocrine neoplasia (MEN 1) ➢ Neurofibromatosis type 1 (NF1)

Duodenal Carcinoid [Figure 2-10-11] •

Figure 2-10-10

Carcinoid

Duodenal Carcinoid •

Carcinoid

Figure 2-10-11

Imaging features ➢ Solitary or multifocal polyps ➢ Intramural mass

Jejunal and Ileal Carcinoid • •

Aggressive biologic behavior Serotonin production ➢ Desmoplasia ➢ Kinking of bowel ➢ Spiculation of mesentery ➢ Ischemia from "elastic vascular sclerosis” ➢ May have carcinoid syndrome with liver mets

Gastrointestinal Radiology

357

Duodenal carcinoid

Small Intestinal Neoplasms

Jejunal and Ileal Carcinoid: Imaging Features •

• •

Figure 2-10-12

Discrete mass in wall of bowel ➢ Mural mass ➢ Polypoid mass ➢ Multiple masses, less common Extensive wall abnormalities ➢ Luminal narrowing ➢ Thick, spiculated folds Local nodal mesenteric metastasis often most prominent feature ➢ Spiculated, fibrotic mass adjacent to bowel ➢ Sunburst pattern of vessels on angiogram ➢ May calcify

Carcinoid Ileum [Figure 2-10-12] Carcinoid Ileum • • • • •

”Elastic vascular sclerosis” Sunburst pattern of mesenteric vessels Multifocal nodules Kinking of bowel Rigid segment of bowel

Ileal carcinoid

Somatostatin Receptor Scintigraphy: Octreotide Scans (111In-Pentetreotide) [Figure 2-10-13] Carcinoid Syndrome

• • •

•

•

10% of patients with carcinoids Most common with ileal carcinoids Hepatic metastasis are usually present ➢ Serotonin and metabolites in systemic circulation Classic syndrome ➢ Paroxysms of sweating, flushing, cyanosis, wheezing, abdominal colic, right-sided heart failure, diarrhea ➢ Symptoms precipitated by ETOH intake, stress, exercise Carcinoid heart disease ➢ Right sided valvular dysfunction ➢ Congestive heart failure

Metastatic carcinoid shown on CT and 111In-pentetreotide scintigraphy

Carcinoid: Differential Diagnosis • • • •

Figure 2-10-13

Figure 2-10-14

Metastatic disease Lymphoma Granulomatous infection Sclerosing mesenteritis

77-year-old asymptomatic man [Figure 2-10-14]

Gastrointestinal stromal tumor Small Intestinal Neoplasms

358

Gastrointestinal Radiology

Differential Diagnosis: Small Bowel Polypoid Mass •

•

Figure 2-10-15

Benign ➢ Adenoma ➢ Peutz Jegher polyp ➢ Inflammatory fibroid polyp ➢ Inflammatory pseudotumor Malignant ➢ Metastatic disease ➢ Adenocarcinoma ➢ Lymphoma ➢ GIST ➢ Carcinoid

Gastrointestinal stromal tumor

Gastrointestinal Stromal Tumor (GIST) • • •

Most common mesenchymal neoplasm Small bowel second most common site Variable biologic behavior

Figure 2-10-16

GIST: Small Bowel, polypoid GIST: Small Bowel, mural [Figure 2-10-15]

GIST: Small Bowel, polypoid and exophytic

GIST: Small Bowel, exophytic [Figure 2-10-16]

Gastrointestinal stromal tumor

GIST: Small Bowel, cavitary

52-year-old man with NF-1 complains of abdominal pain Gastrointestinal Neoplasms in NF-1

• •

• • • •

Neurofibroma Carcinoid ➢ Duodenal ➢ Somatostatinoma Gastrointestinal stromal tumors ➢ Small intestine, multiple Ganglioneuroma Leiomyoma, leiomyosarcoma Adenocarcinoma

Metastatic Disease • • • •

• •

Most common site for metastasis in GI tract Metastasis are more common than primary malignancies in the small bowel Widespread metastatic disease usually present Hematogenous spread ➢ Melanoma ➢ Lung ➢ Breast ➢ Kidney Intraperitoneal, direct extension, lymphatic spread ➢ Tumors of GI origin ➢ Ovarian and endometrial carcinoma Imaging patterns ➢ Identical to primary neoplasms ➢ Polyps, mural masses, annular strictures, cavitary lesions, association with mesenteric nodal masses

Gastrointestinal Radiology

359

Small Intestinal Neoplasms

Metastatic Disease: Renal Cell Carcinoma [Figure 2-10-17] Metastatic Disease: Melanoma [Figure 2-10-18]

Figure 2-10-17

Summary: Brunner Gland Lesions •

•

Brunner gland hamartoma ➢ Solitary mass ➢ Proximal duodenum Brunner gland hyperplasia ➢ Multiple nodules ➢ Proximal duodenum

Renal cell carcinoma metastatic to the small bowel

Summary: Adenoma • • •

Uncommon Most periampullary Association ➢ FAP ➢ HNPCC

Figure 2-10-18

Summary: Adenocarcinoma • •

Periampullary location most common Morphology ➢ Polypoid ➢ Annular ➢ Infiltrating ➢ Cavitary

Summary: Carcinoid • •

•

Melanoma metastatic to the small bowel

Endocrine neoplasms Midgut most common ➢ Serotonin production ➢ Octreotide scintigraphy Key imaging features ➢ Mural wall thickening ➢ Fixation of bowel ➢ Mesenteric mass ➢ Mesenteric retraction

Summary: GIST •

•

Most common mesenchymal neoplasm ➢ KIT positive Mural masses ➢ Intraluminal polyp ➢ Exophytic component ➢ Hemorrhage ➢ Cyst formation ➢ Cavitation

Summary: Metastatic Disease •

Most common malignancy in the small bowel

Small Intestinal Neoplasms

360

Gastrointestinal Radiology

Colorectal Carcinoma

Angela D. Levy, COL, MC, USA Colorectal Carcinoma: Objectives • • •

•

Epidemiology/pathogenesis Screening Detection ➢ Preoperative assessment Staging ➢ Rectal carcinoma

Colorectal Carcinoma •

Third most frequent cancer in the U.S.1 ➢ ~150,000 new cases per year ➢ 11% of cancers in men and women ➢ 10% of cancer deaths

1Jemal A et al. CA Cancer J Clin 2005; 55:10-30

Colorectal Carcinoma: Risk Factors • • • •

Lifetime risk 6% Incidence increases after age 50 Familial risk ➢ 2 to 4 fold increase risk with a single first degree relative ➢ 3 to 6 fold increase risk with two first degree relatives Increased risk ➢ Familial adenomatous polyposis syndrome (FAP) ➢ Hereditary nonpolyposis colon cancer (HNPCC) ➢ Inflammatory bowel disease

Colorectal Carcinoma: Pathogenesis •

•

Adenoma-Carcinoma Sequence ➢ Slow evolution to cancer, average 10 years ➢ Adenoma detection and removal = cure Exception to adenoma-carcinoma sequence ➢ Carcinomas in inflammatory bowel disease ➢ Hereditary nonpolyposis colon cancer (HNPCC)

Adenoma-Carcinoma Sequence [Figures 2-11-1] Colorectal Carcinoma: Role of Radiology •

•

• •

Screening ➢ ACBE ➢ CT colonography Detection ➢ Symptomatic patients Preoperative screening ➢ Primary disease complications ➢ Preoperative staging Recurrent disease

Figure 2-11-1

Adenoma to carcinoma sequence progressive from normal mucosa, unicryptal adenoma, polypoid adenoma, dysplasia, high-grade dysplasia, carcinoma in-situ, to invasive carcinoma Gastrointestinal Radiology

361

Colorectal Carcinoma

Colorectal Carcinoma: American Cancer Society Screening Recommendations •

Average risk adults begin screening at age 50 ➢ Annual fecal occult blood (FOBT) or fecal immunochemical test (FIT) ➢ Sigmoidoscopy every 5 years ➢ Annual FOBT or FIT + Sigmoidoscopy every 5 years ➢ Colonoscopy every 10 years ➢ DCBE every 5 years

Colorectal Carcinoma: American College of Gastroenterology Polyp guidelines1 • •

Colonoscopy every 3 years, high risk for metachronous adenomas ➢ >2, >1cm, villous histology or high-grade dysplasia Colonoscopy every 5 years, low risk for metachronous adenomas ➢ 1-2 tubular adenomas, no family history

1Bond JH. Am J Gastroenterology 2000. 95(11): 3053-3063

Colorectal Carcinoma: Screening •

•

Air contrast barium enema ➢ Accuracy 90% for polyps >1 cm ➢ Pitfalls ❖ Anatomic difficulties (overlapping segments) ❖ Diverticular disease ❖ Perceptive errors Colonoscopy ➢ Accuracy 90% ➢ Invasive, requiring sedation ➢ Perforation rate .1% to .5% ➢ Pitfalls ❖ Failure to reach cecum ❖ Blind spots

Colorectal Carcinoma: Screening •

Virtual colonography ➢ Sensitivity 73% to 93% for >10mm polyps ➢ Prone and supine imaging improves sensitivity ➢ Difficult lesions ❖ Poor bowel preparation ❖ Flat adenomas ❖ Adenomas on folds ❖ Adenomas seen in only one position

Colorectal Polyps: Histologic Spectrum •

• • • •

Hyperplastic ➢ Most common ➢ Usually <5 mm, descending colon and rectum ➢ NOT neoplastic Adenoma ➢ Tubular, 75% are <1 cm, most pedunculated ➢ Villous, 60% are > 2 cm, most sessile ➢ Mixed Juvenile Peutz-Jeghers Inflammatory/post-inflammatory

Tubular Adenoma Villous Adenoma

Colorectal Carcinoma

362

Gastrointestinal Radiology

Adenoma •

• •

Figure 2-11-2

Size ➢ < 5 mm, benign ➢ 5 mm to 1 cm, 1% are carcinoma ➢ 1 - 2 cm, 10% are carcinoma ➢ > 2 cm, 30% to 50% are carcinoma Synchronous adenomas ➢ 40% to 50% Recurrence ➢ 20% to 60% recurrence rate ➢ Majority recur within 2 years

Adenoma: Barium Features • •

•

Filling defect in barium pool Protrusion into the lumen ➢ “Innies not Outies” ➢ Bowler hat sign ➢ Sessile or pedunculated Carpet lesions ➢ Sessile lesions ➢ Bubbly or nodular contour ➢ Villous change

Bowler Hat Sign [Figure 2-11-2] Virtual Colonography Villous Adenoma • • •

•

Higher rate of malignancy Recurrence rate 9.3% Three types ➢ Flat, carpet-like ➢ Sessile, lobulated ➢ Pedunculated Histology ➢ Nonbranching finger-like fronds

Sessile adenomatous polyp showing the Bowler Hat sign

Villous Adenoma: Pathology [Figure 2-11-3]

Figure 2-11-3

Villous adenoma of the cecum showing a bubbly, carpet-like appearance Gastrointestinal Radiology

363

Colorectal Carcinoma

Villous Adenoma: CT Features [Figure 2-11-4] •

•

Soft tissue mass ➢ Sessile ➢ Eccentric ➢ Stalk ➢ Expands lumen Irregular luminal margin ➢ Low attenuation luminal margin ➢ High mucin content

Colonic Adenocarcinoma

Colorectal Carcinoma: Distribution

Figure 2-11-4

Villous adenoma of the rectum showing low attenuation along the luminal margin [Figure 2-11-5]

Colorectal Carcinoma: Clinical Presentation • •

• • • • •

Minimal or absent symptoms in up to 12% of patients Bleeding ➢ Initial complaint in 50% Weight loss, malaise Pain Change in bowel habits Right vs. left sided lesions Symptoms from complications ➢ Obstruction, ischemia, perforation, peritonitis, fistula

Figure 2-11-5

Colorectal Carcinoma: Morphologic Patterns • •

• •

Polypoid ➢ Intraluminal masses ➢ Bulky, fungating masses in cecum and ascending colon Infiltrating/annular constricting ➢ Transverse, descending, and sigmoid colon ➢ Encircle the bowel ➢ “Apple core” ➢ Diffuse infiltration (linitis plastica) uncommon Ulcerating ➢ Deeply invade colonic wall ➢ Edge of tumor slightly elevated above normal mucosa Flat plaques ➢ Carcinomas from flat adenomas ➢ Carcinomas in inflammatory bowel disease

Distribution of colorectal carcinoma

Colorectal Carcinoma: Computed Tomography •

• • • •

Primary Tumor ➢ Discrete mass ➢ Mural thickening Extension beyond the bowel ➢ Irregular outer margin ➢ Soft-tissue stranding in pericolonic fat Adjacent organ/muscle invasion ➢ Loss of fat planes ➢ Tumor mass in adjacent organ or muscle Liver metastasis Lymph node metastasis

Colorectal Carcinoma

364

Gastrointestinal Radiology

Polypoid Adenocarcinoma

Figure 2-11-6

[Figure 2-11-6]

Annular Adenocarcinoma [Figure 2-11-7]

Infiltrating Adenocarcinoma [Figure 2-11-8]

Pericolonic Extension and Adenopathy Adjacent Organ Invasion: Contiguous Soft Tissue Attenuation [Figure 2-11-9]

Polypoid adenocarcinoma of the cecum showing pericolonic extension

Figure 2-11-7

Adjacent Organ Invasion Coloduodenal Fistula Multiple Carcinomas •

•

•

Synchronous carcinomas ➢ Diagnosed within 6 months of each other ➢ Incidence 1.5% to 12% ➢ Most are >5 cm away from each other Metachronous carcinomas ➢ Incidence 0.6% to 9.1% ➢ Time interval to second lesion discovery ❖ 64% within 5 years ❖ 45% within 3 years ❖ 20% within 1 year 8% to 20% of patients with colorectal carcinomas have malignancies in other organs

Annular adenocarcinoma of the distal transverse colon

Figure 2-11-8

Infiltrating adenocarcinoma of the sigmoid colon with pericolonic extension and pericolonic adenopathy

Figure 2-11-9

Synchronous Carcinomas [Figure 2-11-10]

Figure 2-11-10

Rectal adenocarcinoma (T4) showing contiguous soft tissue attenuation into the pelvic side walls consistent with adjacent organ invasion

Synchronous adenocarcinomas of the hepatic flexure and descending colon Gastrointestinal Radiology

365

Colorectal Carcinoma

Colonic Adenocarcinoma in Inflammatory Bowel Disease: [Figure 2-11-11]

•

• •

Ulcerative colitis ➢ Highest incidence Crohn disease ➢ Large and small intestinal adenocarcinoma Features of carcinoma in IBD ➢ Typically do not arise in pre-existing adenomas ➢ Arise in flat mucosa ➢ Carcinomas may be long and flat

Figure 2-11-11

Adenocarcinoma in Ulcerative Colitis Colorectal Carcinoma: Complications •

• •

CT of Obstructing Colon Carcinomas • • • •

Adenocarcinoma in ulcerative colitis

Bleeding ➢ Occult ➢ Chronic anemia ➢ Massive bleeding, unusual Obstruction ➢ Occlusion of the colonic lumen ➢ Colocolic intussusception Perforation ➢ Abscess ➢ Fistula ➢ Differential diagnosis, diverticulitis IV contrast Identify obstructing lesion ➢ Infiltration of adjacent fat ➢ Adjacent organ invasion Evaluate bowel integrity ➢ Obstructive colitis (1% to 7%) ➢ Ischemic changes ➢ Pneumatosis Stage ➢ Local extension ➢ Lymph node mets ➢ Liver mets

CT of Obstructing Colon Carcinomas

Figure 2-11-12

Colonic ischemia in an obstructing carcinoma of the descending colon

Figure 2-11-13

Perforated adenocarcinoma of the transverse colon with abscess formation

Ischemia in Obstructive Cancers [Figure 2-11-12]

Carcinoma with Perforation and Abscess [Figure 2-11-13]

Colorectal Carcinoma:Role of Preoperative Imaging •

•

Tumors proximal to the rectum are staged surgically ➢ Preoperatively image patients with clinical evidence of advanced disease Preoperative imaging rectal tumors ➢ EUS and CT ➢ MR

Colorectal Carcinoma

366

Gastrointestinal Radiology

Colorectal Carcinoma: Preoperative CT •

• •

Local tumor extension ➢ Adjacent organ invasion Liver metastasis ➢ Early rim enhancement, followed by hyperdensity ➢ Hypodense in the portal venous phase ➢ Isodense in the equilibrium phase Lymphatic Spread

Rectal Adenocarcinoma: Preoperative EUS •

•

Endoscopic Ultrasound (EUS)* ➢ 360 degree probe ➢ Normal 5-layer rectal wall ➢ T stage accuracy 69% to 97% ➢ Nodal accuracy 70% to 80% EUS limitations ➢ EUS best at early stage tumors ➢ Limited assessment because of location or bulk occurs ➢ May overstage (fibrosis vs. tumor vs. inflammation) ➢ Intraobserver variability

Wolfman NT, Ott DJ. Endoscopic Ultrasonography. Semin Roentgenol 1996. 31(2): 154-161. Beets-Tan RGH, Beets GL. Rectal cancer: review with emphasis on MR imaging. Radiology 2004. 232: 335-346

Rectal Adenocarcinoma: Preoperative MR • •

Endoluminal MR ➢ Equal accuracy for early stage tumors to EUS ➢ T stage accuracy, 71% to 91% Phased array MR ➢ High spatial resolution ➢ Large field of view ➢ Limitations differentiating T2 and T3 lesions

Colorectal Carcinoma: Role of PET • •

•

No role in screening/diagnosis Preoperative staging ➢ Highly sensitive for liver mets ➢ Not sensitive for T staging and nodal mets Detection of recurrent disease ➢ Following liver met resection/treatment ➢ Scar vs. recurrent tumor at resection margin

Hustinx R. PET imaging in assessing gastrointestinal tumors. RCNA 2004. 112 (6) 1123-1139.

Rectal Adenocarcinoma: Management •

• •

High T1 or T2 lesion ➢ Lesions 5 to 6 cm above dentate line or at peritoneal reflection ➢ Primary resection and anastomosis (LAR) Low T1 or T2 lesion ➢ APR (Miles procedure), LAR, coloanal anastomosis with J-pouch, local or transanal excision, total mesorectal excision, posterior proctotomy T3 or T4 ➢ Downstage with preoperative neoadjuvant chemoradiation ➢ APR and post operative XRT, adjuvant chemotherapy

Gastrointestinal Radiology

367

Colorectal Carcinoma

TNM Staging [Figure 2-11-14] •

• •

Figure 2-11-14

T-Primary tumor ➢ T1 invades submucosa ➢ T2 invades muscularis propria ➢ T3 through muscularis propria or into nonperitonealized pericolic fat ➢ T4 perforates visceral peritoneum or directly invades adjacent organs or structures N-Regional nodes M-Distant metastasis

EUS Layer 1: Hyperechoic superficial mucosa

EUS Layer 2: Hypoechoic deep mucosa EUS Layer 3: Hyperechoic submucosa EUS Layer 4: Hypoechoic muscularis propria

TNM Staging for colorectal carcinoma

EUS Layer 5: Hyperechoic perirectal fat [Figure 2-11-15] T1 N0 M0

Figure 2-11-15

T2 N0 M0

T3 N2 M0 [Figure 2-11-16] T3 N10 M0 T3 N8 M1 T4

T4: Extension to pelvic side wall T4: Extension to labia

Normal anatomy of the colon wall with endoscopic ultrasound

Figure 2-11-16

T3N2M0 rectal adenocarcinoma

Colorectal Carcinoma

368

Gastrointestinal Radiology

Colorectal Carcinoma: Lymphatic Spread [Figure 2-11-17]

Figure 2-11-17

Pericolonic nodes ➢ Paracolic ➢ Epiploic Mesenteric Nodes ➢ Intermediate nodes Principal nodes ➢ SMA ➢ IMA

•

• •

Pericolonic Nodes Intermediate Principal

Rectal Adenocarcinoma: Lymphatic Drainage [Figures 2-11-18 and 2-1119]

• •

•

Pararectal nodes Internal iliac nodes ➢ Tumors above dentate line Inguinal nodes ➢ Tumors below dentate line

Distribution of lymphatic spread for colon carcinoma

Figure 2-11-18

Distribution of lymphatic spread for rectal carcinoma

Figure 2-11-19

Inguinal lymph nodes in a rectal adenocarcinoma that extended below the dental line Gastrointestinal Radiology

369

Colorectal Carcinoma

Summary: Adenoma • • •

40% - 50% synchronous 20% - 60% recur BE features ➢ Filling defect ➢ Bowler hat ➢ Sessile ➢ Pedunculated

Summary: Villous Adenoma • • • •

Carpet lesions Bubbly appearance Expand lumen Low attenuation on luminal surface

•

Morphology ➢ Polypoid ➢ Infiltrating/annular ➢ Ulcerating ➢ Flat plaques Synchronous carcinomas CT ➢ Local extent ➢ Adjacent organ invasion

Summary: Primary Tumor

• •

Summary: Complications •

• •

Bleeding ➢ Usually chronic blood loss ➢ Massive GI bleed, unusual Obstruction ➢ CT ➢ Identify lesion and bowel wall integrity Perforation ➢ Abscess ➢ Fistula ➢ Differential diagnosis inflammatory disorders

Summary: Role of Imaging •

Preoperative CT ➢ Local tumor extent ➢ Liver metastasis ➢ Lymphatic spread

Summary: Rectal Adenocarcinoma •

•

Preoperative staging ➢ EUS and CT, MR T3 lesions ➢ Through muscularis propria ➢ Spiculated outer margin on CT ➢ Perirectal adenopathy

Colorectal Carcinoma

370

Gastrointestinal Radiology

References:

Virtual Colonography 1. Fidler JL, Johnson CD, MacCarty RL, et al: Detection of flat lesions in the colon with CT colonography. Abdom Imaging 27:292, 2002 2. Fletcher JG, Johnson CD, MacCarty RL, et al: CT colonography: potential pitfalls and problem-solving techniques. AJR Am J Roentgenol 172:1271, 1999 3. Fletcher JG, Johnson CD, Welch TJ, et al: Optimization of CT colonography technique: prospective trial in 180 patients. Radiology 216:704, 20 4. Gluecker TM, Fletcher JG, Welch TJ, et al: Characterization of Lesions Missed on Interpretation of CT Colonography Using a 2D Search Method. AJR Am J Roentgenol 182:881, 2004 5. Gluecker TM, Johnson CD, Harmsen WS, et al: Colorectal cancer screening with CT colonography, colonoscopy, and double-contrast barium enema examination: prospective assessment of patient perceptions and preferences. Radiology 227:378, 2003 6. Johnson CD, Ahlquist DA: Computed tomography colonography (virtual colonoscopy): a new method for colorectal screening. Gut 44:301, 1999 7. Johnson CD, Harmsen WS, Wilson LA, et al: Prospective blinded evaluation of computed tomographic colonography for screen detection of colorectal polyps. Gastroenterology 125:311, 2003 8. Johnson CD, Toledano AY, Herman BA, et al: Computerized tomographic colonography: performance evaluation in a retrospective multicenter setting. Gastroenterology 125:688, 2003 9. Macari M: Virtual colonoscopy: clinical results. Semin Ultrasound CT MR 22:432, 2001 10. Pescatore P, Glucker T, Delarive J, et al: Diagnostic accuracy and interobserver agreement of CT colonography (virtual colonoscopy). Gut 47:126, 2000 11. Pickhardt PJ: Three-dimensional endoluminal CT colonography (virtual colonoscopy): comparison of three commercially available systems. AJR Am J Roentgenol 181:1599, 2003 12. Pickhardt PJ, Choi JR, Hwang I, et al: Computed tomographic virtual colonoscopy to screen for colorectal neoplasia in asymptomatic adults. N Engl J Med 349:2191, 2003 13. Royster AP, Fenlon HM, Clarke PD, et al: CT colonoscopy of colorectal neoplasms: two-dimensional and threedimensional virtual-reality techniques with colonoscopic correlation. AJR Am J Roentgenol 169:1237, 1997 14. Spinzi G, Belloni G, Martegani A, et al: Computed tomographic colonography and conventional colonoscopy for colon diseases: a prospective, blinded study. Am J Gastroenterol 96:394, 2001 15. Taylor SA, Halligan S, Bartram CI: CT colonography: methods, pathology and pitfalls. Clin Radiol 58:179, 2003 16. Taylor SA, Halligan S, Bartram CI, et al: Multi-detector row CT colonography: effect of collimation, pitch, and orientation on polyp detection in a human colectomy specimen. Radiology 229:109, 2003 17. Taylor SA, Halligan S, Goh V, et al: Optimizing bowel preparation for multidetector row CT colonography: effect of Citramag and Picolax. Clin Radiol 58:723, 2003 18. Taylor SA, Halligan S, Goh V, et al: Optimizing colonic distention for multi-detector row CT colonography: effect of hyoscine butylbromide and rectal balloon catheter. Radiology 229:99, 2003

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Colorectal Carcinoma

Mesenteric Masses and Cysts Angela D. Levy, COL, MC, USA

Mesenteric Masses and Cysts: Objectives •

•

Definitions ➢ Review mesenteric anatomy Case based approach to differential diagnosis ➢ Mesenteric and omental cysts ➢ Mesothelioma ➢ Mesenteric Fibromatosis ➢ Sclerosing mesenteritis ➢ Inflammatory myofibroblastic pseudotumor ➢ Extrapleural solitary fibrous tumor

Figure 2-12-1

MesentericAnatomy: Definitions •

•

Mesentery ➢ Double fold of peritoneum ➢ Connects an organ to the abdominal wall Omentum ➢ Specialized mesentery extending from stomach to an adjacent organ

Anatomy Mesentery [Figure 2-12-1]

• • • •

Transverse mesocolon Small bowel mesentery Sigmoid mesentery Mesoappendix

Anatomy: Omentum [Figure 2-12-2]

•

•

Greater omentum ➢ Gastrocolic ligament ➢ Gastrosplenic ligament ➢ Gastrophrenic ligament Lesser omentum ➢ Gastrohepatic ligament ➢ Hepatoduodenal ligament

Normal posterior attachments of the mesentery in sagittal and AP planes

Figure 2-12-2

Mesenteric and Omental Cyst • •

Descriptive term 5 histologic subtypes ➢ Defined by internal lining

Mesenteric and Omental Cyst •

• •

• •

Lymphangioma ➢ Endothelial lining Enteric duplication cyst ➢ Enteric lining with muscular wall Enteric cyst ➢ Enteric lining with a fibrous wall Mesothelial cyst ➢ Mesothelial lining Nonpancreatic pseudocyst ➢ No lining

Mesenteric Masses and Cysts

Normal anatomy of greater and lesser omentum 372

Gastrointestinal Radiology

35-year-old woman with increasing abdominal girth [Figure 2-12-3] Differential Diagnosis: Cystic Mesenteric Mass •

•

•

•

Mesenteric cyst ➢ Lymphangioma ➢ Enteric duplication cyst ➢ Enteric cyst ➢ Mesothelial cyst ➢ Nonpancreatic pseudocyst Cystic neoplasm ➢ Teratoma ➢ Cystic malignant mesothelioma ➢ Benign multicystic mesothelioma ➢ Cystic soft tissue primary ➢ Pseudomyxoma peritonei Complex ascites ➢ Infectious, neoplastic Pseudocyst ➢ Internal hemorrhage, abscess

Figure 2-12-3

Lymphangioma

Lymphangioma • • • •

Figure 2-12-4

Benign Vascular origin Affect all ages Many anatomic sites ➢ 95% neck, axilla ➢ 5% mesentery ➢ Lymphangiomatosis

Abdominal Lymphangioma: Pathology • • •

Lymphangioma of the greater omentum

Interconnecting cysts Endothelial lining Dilated lymphatic spaces ➢ Proteinaceous fluid ➢ Chyle, low attenuation ➢ Hemorrhage

Abdominal Lymphangioma: Imaging Features [Figures 2-12-4 and 2-12-5] • •

• •

• •

Mesenteric, omental or retroperitoneal location Complex cyst ➢ Multilocular ➢ Enhancing septations ➢ Internal debris Closely associated with small bowel Figure 2-12-5 Lack features of free fluid ➢ Mass effect ➢ Septations ➢ No fluid in dependent spaces peritoneum Infiltration/insinuation ➢ Within mesentery and bowel Complications ➢ Small bowel obstruction ➢ Volvulus Mesenteric lymphangioma showing low attenuation ➢ Infection and insinuating growth

Gastrointestinal Radiology

373

Mesenteric Masses and Cysts

71-year-old woman with abdominal pain [Figure 2-12-6]

Figure 2-12-6

Differential Diagnosis •

• •

•

Pancreatic cystic neoplasm ➢ Mucinous cystic neoplasm ➢ Oligocystic adenoma Pancreatic pseudocyst Mesenteric cyst ➢ Lymphangioma ➢ Enteric duplication cyst ➢ Enteric cyst ➢ Mesothelial cyst ➢ Pancreatic pseudocyst Cystic mesenteric neoplasm ➢ Metastatic disease ➢ Cystic mesothelioma

Enteric duplication cyst

Enteric Duplication Cyst

Enteric Cyst and Mesothelial Cyst •

• • •

Enteric cyst ➢ Variant of enteric duplication, does not contain muscular wall Mesothelial cyst ➢ Rare ➢ Fusion failure of visceral/parietal peritoneum Nonspecific imaging features Similar appearance compared to enteric duplication cyst

Figure 2-12-7

Nonpancreatic Pseudocyst [Figure 2-12-7] • • •

Old hematoma, abscess No histologic lining Imaging ➢ Thick walled ➢ Internal debris

55-year-old man, former shipyard worker, with worsening abdominal pain [Figure 2-12-8]

Nonpancreatic pseudocyst

Differential Diagnosis • •

•

Metastatic disease Primary neoplasms ➢ Diffuse malignant mesothelioma ➢ Serous papillary carcinoma ➢ Intra-abdominal desmoplastic round cell tumor ➢ Leiomyomatosis peritonealis disseminata Diffuse Infection ➢ Tuberculosis ➢ Histoplasmosis

Figure 2-12-8

Diffuse Malignant Mesothelioma • • •

Diffuse malignant mesothelioma

Malignancy of mesothelial origin Association with asbestos Variants ➢ Diffuse peritoneal malignant mesothelioma ➢ Cystic malignant mesothelioma

Mesenteric Masses and Cysts

374

Gastrointestinal Radiology

Diffuse Malignant Mesothelioma •

•

Gross Pathology ➢ Nodules, masses, caking ➢ Bowel encasement ➢ Thick, nodular peritoneum ➢ Ascites Histopathologic variants ➢ Desmoplastic ➢ Lymphohistiocytoid ➢ Small cell ➢ Papillary

Diffuse Peritoneal Malignant Mesothelioma •

Imaging features ➢ Peritoneal soft tissue nodules ➢ Omental and mesenteric masses, nodules ➢ Ascites ➢ Bowel wall thickening ➢ Fixation of small bowel

Diffuse Peritoneal Malignant Mesothelioma: Peritoneal, Omental Nodules and Masses Diffuse Malignant Mesothelioma: Small Bowel Fixation

Figure 2-12-9

Cystic Malignant Mesothelioma [Figure 2-12-9]

Benign Multicystic Mesothelioma [Figure 2-12-10]

•

• • • •

•

Cystic malignant mesothelioma

Rare ➢ Arises from pelvic peritoneum Unrelated to asbestos Unrelated to malignant mesothelioma Synonym ➢ Multilocular peritoneal inclusion cyst Most common in women ➢ Mean age, 37 years Clinical symptoms ➢ Chronic pelvic pain

Figure 2-12-10

Benign Multicystic Mesothelioma •

Imaging features ➢ Multicystic pelvic mass ➢ Enhancing septa ➢ Peritoneal surfaces of uterus, bladder ➢ May extend into upper abdomen

Benign multicystic mesothelioma

Benign Multicystic Mesothelioma: Differential Diagnosis •

• • •

Metastasis ➢ Mucinous adenocarcinoma ➢ Serous papillary carcinoma of ovary Cystic malignant mesothelioma Primary serous papillary carcinoma of peritoneum Infection with complex ascites ➢ Tuberculosis

Gastrointestinal Radiology

375

Mesenteric Masses and Cysts

31-year-old man complained of abdominal fullness and early satiety. Physical exam revealed a palpable mass

Figure 2-12-11

[Figure 2-12-11]

Differential Diagnosis: Solid Mesenteric Mass •

•

Malignant ➢ Soft tissue sarcoma ➢ Lymphoma ➢ Gastrointestinal stromal tumor ➢ Metastatic disease Benign ➢ Mesenteric fibromatosis ➢ Sclerosing mesenteritis ➢ Inflammatory pseudotumor

Mesenteric fibromatosis

Figure 2-12-12

Mesenteric Fibromatosis: (Intraabdominal Fibromatosis or Abdominal Desmoid) •

• • •

Classified as a deep fibromatosis ➢ Mesenteric, pelvic, retroperitoneal ➢ Abdominal wall ➢ Extraabdominal Benign proliferative process Mesenteric fibromatosis with low CT attenuation, located in the ➢ Locally aggressive greater omentum ➢ Recurs following excision ➢ Does not metastasize Mesenteric fibromatosis ➢ No gender predilection ➢ Most cases sporadic ➢ 13% associated with FAP Abdominal fibromatosis ➢ Most common in young women, 20-30 years of age

Mesenteric Fibromatosis: Pathologic Features •

•

Gross pathology ➢ Well-defined or infiltrative margins Histology ➢ “Melting insinuating” and “tentacular growth” ➢ Microscopic tumor infiltration into bowel ➢ Collagenous and/or myxoid stroma

Figure 2-12-13

Mesenteric Fibromatosis: Imaging •

•

Homogeneous ➢ Collagenous stroma ➢ Myxoid stroma (low attenuation CT/high signal T2 MR) Heterogeneous ➢ Bands of myxoid stroma “whorls”

Mesenteric Fibromatosis: Homogeneous Attenuation

Mesenteric fibromatosis

Mesenteric Fibromatosis: Low CT Attenuation Myxoid Stroma [Figure 2-12-12]

Mesenteric Fibromatosis : High T2 Signal [Figure 2-12-13]

Mesenteric Masses and Cysts

376

Gastrointestinal Radiology

Mesenteric Fibromatosis: MR Enhancement Mesenteric Fibromatosis in FAP Mesenteric Fibromatosis •

Complications ➢ Small bowel obstruction ➢ Fistula formation ➢ Perforation ➢ GI bleeding

Mesenteric Fibromatosis: Infiltrates small bowel wall

Mesenteric Fibromatosis in FAP •

•

[Figure 2-12-14]

Figure 2-12-14

Mesenteric fibromatosis in a patient with FAP. The myxoid stroma creates a “whorled” pattern in this example

Almost always post operative ➢ Occurs at operative sites ➢ Usually within 4 years of surgery Unusual manifestations ➢ Multiplicity ➢ May occur with abdominal wall fibromatosis ➢ Diffuse form may involve mesentery, pelvis, and retroperitoneum

Mesenteric, Pelvic, and Retroperitoneal: Fibromatosis in FAP Differential Diagnosis: Solid Mesenteric Mass •

•

Malignant ➢ Soft tissue sarcoma ➢ Lymphoma ➢ Gastrointestinal stromal tumor ➢ Metastatic disease Benign ➢ Mesenteric fibromatosis ➢ Sclerosing mesenteritis ➢ Inflammatory pseudotumor

Metastatic Disease: Metastatic Lung Carcinoma

Soft Tissue Sarcoma: Synovial Sarcoma Lymphoma

Gastrointestinal Stromal Tumor: Small Bowel Primary

Gastrointestinal Stromal Tumor: Primary to the Mesentery Gastrointestinal Stromal Tumor: Retroperitoneal Mesenteric Fibromatosis: Management •

• •

Controversial ➢ Wide excision, antiestrogens, chemotherapy, radiation therapy ➢ Complications and recurrence common Sporadic cases ➢ Surgery often curative FAP ➢ Higher recurrence rate ➢ Higher morbidity ➢ Nonsurgical therapy more commonly used

Gastrointestinal Radiology

377

Mesenteric Masses and Cysts

Mesenteric Fibromatosis: Postoperative Recurrence Sclerosing Mesenteritis • • • •

Rare Idiopathic, nonneoplastic Chronic inflammation Synonyms represent histologic spectrum ➢ Mesenteric panniculitis ➢ Fibrosing mesenteritis ➢ Mesenteric lipodystrophy

Sclerosing Mesenteritis: Clinical Features • • •

Twice as common in men Mean age, 60 years Symptoms ➢ Pain ➢ Palpable mass ➢ Bowel complications ➢ Incidental

Figure 2-12-15

Sclerosing Mesenteritis: Pathologic Features •

•

Pathologic spectrum ➢ Loose myxomatous to dense sclerosis Histologic features ➢ Sclerosing fibrosis ➢ Fat necrosis ➢ Lipid-laden macrophages ➢ Chronic inflammation ➢ Focal calcification

Sclerosing mesenteritis

Sclerosing Mesenteritis: Imaging Features [Figure 2-12-15] •

•

Mesenteric mass ➢ Mixed fat and soft tissue ➢ Radiating fibrosis ➢ Fat-ring sign ➢ Calcifications ➢ Cystic appearance Small Bowel ➢ Kinking or fixation ➢ Small bowel obstruction

Sclerosing Mesenteritis

Figure 2-12-16

Sclerosing mesenteritis with the “fat ring” sign on CT

[Figure 2-12-16]

•

“Fat-ring Sign”

Sclerosing Mesenteritis [Figure 2-12-17]

Sclerosing Mesenteritis: Differential Diagnosis •

• •

Carcinoid metastasis ➢ Look for primary ➢ Somatostatin scintigraphy Metastatic disease Mesenteric fibromatosis

Figure 2-12-17

Sclerosing mesenteritis with low CT attenuation from loose myxomatous stroma shown on the accompanying histology image Mesenteric Masses and Cysts

378

Gastrointestinal Radiology

Sclerosing Mesenteritis: Somatostatin Receptor Scintigraphy Sclerosing Mesenteritis: Management • • •

•

Biopsy may establish diagnosis Many cases self-limiting Asymptomatic or mild symptoms ➢ Observation Symptomatic ➢ Immunosuppresive therapy ➢ Surgical resection

Inflammatory Pseudotumor (Inflammatory Myofibroblastic Tumors) • •

•

Chronic inflammation Unclear pathogenesis ➢ Sequelae occult infection ➢ Minor trauma ➢ Post surgical Variable nomenclature ➢ Inflammatory myofibroblastic pseudotumor ➢ Plasma cell granuloma ➢ Plasma cell pseudotumor ➢ Inflammatory fibrosarcoma

Inflammatory Pseudotumor: Clinical Features • • •

Most common in pediatrics and young adults May occur in may anatomic locations Symptoms ➢ Fever ➢ Malaise ➢ Weight loss ➢ Pain

Inflammatory Pseudotumor [Figure 2-12-18]

Figure 2-12-18

Extrapleural Solitary Fibrous Tumor • • •

Rare neoplasms Submesothelial origin Most commonly pleural origin

• • •

Few case reports Variable clinical presentation Natural history unknown ➢ Pleural lesions may show aggression ➢ Long-term follow up

“Solitary Fibrous Tumor of the Peritoneum” Inflammatory myofibroblastic pseudotumor

Extrapleural Solitary Fibrous Tumor Summary • •

Mesenteric cysts Solid mesenteric masses

• • •

Lymphangioma Most common Imaging ➢ Multilocular ➢ Enhancing septa ➢ Insinuating growth

Summary: Mesenteric Cyst

Gastrointestinal Radiology

379

Mesenteric Masses and Cysts

Summary: Mesenteric Cyst • • • •

Enteric duplication cyst Enteric cyst Histologic differentiation Identical imaging

• •

Mesothelial cyst Nonspecific imaging appearance

• • • •

Nonpancreatic pseudocyst No histologic lining Old trauma/abscess Imaging ➢ Thick wall ➢ Internal debris

Summary: Mesenteric Cyst Summary: Mesenteric Cyst

Summary: Mesothelioma •

•

Diffuse malignant mesothelioma ➢ Asbestos ➢ Nodules, masses ➢ Bowel encasement ➢ Bowel fixation Cystic malignant mesothelioma ➢ Variant of DMM ➢ Cystic masses ➢ Ascites

Summary: Benign Multicystic Mesothelioma •

Controversial ➢ AKA peritoneal inclusion cyst ➢ Unrelated to DMM ➢ Unrelated to asbestos ➢ Pelvic peritoneum ➢ Multicystic mass

Summary: Benign Fibrous Lesions • • • •

Mesenteric fibromatosis Sclerosing mesenteritis Inflammatory myofibroblastic pseudotumor Solitary fibrous tumor of peritoneum

•

Benign ➢ Locally aggressive ➢ Association with FAP Imaging ➢ Homogeneous ➢ Heterogeneous Myxoid stroma ➢ Low CT attenuation ➢ High T2 signal ➢ “whorled” pattern

Summary: Mesenteric Fibromatosis • •

Summary: Sclerosing Mesenteritis • •

•

Idiopathic inflammation Imaging ➢ Mixed attenuation ➢ Bowel retraction ➢ May calcify Conservative treatment

Mesenteric Masses and Cysts

380

Gastrointestinal Radiology

Summary: Inflammatory Pseudotumor • •

Inflammatory/fibrotic infiltrate Nonspecific imaging

•

Few case reports

Summary: Extrapleural Solitary Fibrous Tumor References

Lymphangioma 1. Kempson RL, Fletcher CDM, Evans HL, Hendrickson MR, Sibley RK. Tumors of the soft tissues: atlas of tumor pathology, third series, fascicle 30. Washington, DC: Armed Forces Institute of Pathology; 2001 2. Levy AD, Cantisani V, Miettinen M. Abdominal Lymphangiomas: Imaging Features with Pathologic Correlation. AJR 2004. 182: 1485-1491 3. Ros PR, Olmsted WW, Moser RP, Jr., Dachman AH, Hjermstad BH, Sobin LH. Mesenteric and omental cysts: histologic classification with imaging correlation. Radiology 1987;164:327-332

Mesenteric Fibromatosis 1. Burke AP, Sobin LH, Shekitka KM, Federspiel BH, Helwig EB. Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups. Am J Surg Pathol 1990; 14(4):335-341. 2. Levy AD, Rimola J, Mehrotra AK, Sobin LH. Benign Fibrous Tumors and Tumor-like Lesions of the Mesentery: Radiologic Pathologic Correlation. RadioGraphics 2006; 26: 245- 264 3. Magid D, Fishman EK, Jones B, Hoover HC, Feinstein R, Siegelman SS. Desmoid tumors in Gardner syndrome: use of computed tomography. AJR Am J Roentgenol 1984; 142(6):1141-1145.

Sclerosing Mesenteritis 1. Emory TS, Monihan JM, Carr NJ, Sobin LH. Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity? Am J Surg Pathol 1997; 21(4):392-398. 2. Sabate JM, Torrubia S, Maideu J, Franquet T, Monill JM, Perez C. Sclerosing mesenteritis: imaging findings in 17 patients. AJR Am J Roentgenol 1999; 172(3):625-629. 3. Valls C. Fat-ring sign in sclerosing mesenteritis. AJR Am J Roentgenol 2000; 174(1):259-260.

Gastrointestinal Radiology

381

Mesenteric Masses and Cysts

Idiopathic Inflammatory Bowel Disease Angela D. Levy, COL, MC, USA

Idiopathic Inflammatory Bowel Disease: Objectives • •

Ulcerative colitis (UC) Crohn disease

•

Etiology unknown ➢ ? Genetic basis ➢ ? Immune related ➢ ? Infectious agent Incidence of IBD ➢ UC is more common than Crohn

Idiopathic Inflammatory Bowel Disease: General Features

•

IBD: Epidemiologic Comparison

•

Ulcerative colitis • ➢ 35-100 cases/100,000 ➢ Age range, 15-25 years, second peak, 50 to 80 years ➢ Urban dwellers ➢ Developed countries ➢ Whites, 2 to 5 times risk ➢ Jewish, 2 to 4 times risk ➢ Family history, 30 to 100 times risk

Crohn disease ➢ 10-70 cases/100,000 ➢ Age range, 15-25 years, second peak, 50-80 years ➢ Urban dwellers ➢ Whites ➢ Jewish (8 fold increase) ➢ Family history, 12 to 15 times risk

Ulcerative colitis • ➢ Diarrhea ➢ Obstruction rare ➢ Rectal bleeding usually ➢ Abdominal pain, predefecatory urgency ➢ Chronic, low grade illness in most ➢ Acute, fulminating in 15%

Crohn disease ➢ Diarrhea ➢ Obstruction common ➢ Rectal bleeding, less common ➢ Abdominal pain, post prandial, colicky ➢ Abdominal mass ➢ Vomiting ➢ Perianal disease ➢ Alternating attacks and remissions

IBD: Comparison of Clinical Features

•

IBD: Comparison of Disease Distribution

•

Ulcerative colitis • ➢ Colon to anus, rarely TI ➢ Continuous disease or ➢ Ulcerative proctitis that may extend proximally

Idiopathic Inflammatory Bowel Disease

Crohn disease ➢ Entire GI tract, mouth to anus ➢ Asymmetric, skip lesions ➢ May extend beyond bowel

382

Gastrointestinal Radiology

IBD: Pathologic Features

•

Ulcerative colitis ➢ Mucosal and submucosal inflammation ➢ Minimal mural edema

•

Crohn disease ➢ Transmural inflammation ➢ Marked mural edema

IBD: Gross Pathologic Features [Figure 2-13-1]

•

Ulcerative colitis ➢ Fine ulceration ➢ Granular mucosa ➢ Hyperemic

•

Ulcerative colitis ➢ Shallow ulcers, granularity ➢ Loss of haustra

•

Crohn disease ➢ Linear ulceration ➢ Nodules

•

Crohn disease ➢ Aphthous ulcer ➢ Linear, serpiginous ulcers ➢ Wide-based ulcers ➢ Cobblestones ➢ Fissures, fistulas, abscesses ➢ Strictures ➢ Pseudopolyps

IBD: Gross Pathologic Features

•

IBD: Histologic Features

•

Ulcerative colitis ➢ Diffuse mucosal ulceration ➢ Crypt abscesses ➢ Inflammatory infiltrate ➢ Pseudopolyps

Crohn disease ➢ Linear ulcers ➢ Cobblestones ➢ Marked mural thickening

IBD: Histologic Features of Active Disease

•

Ulcerative colitis ➢ Crypt abscesses, lymphoplasmacytic lamina propria infiltrate

IBD: Histologic Features

•

Ulcerative colitis ➢ Crypt destruction ➢ Crypt abscess ➢ Hemorrhage

IBD: Histologic Features

•

•

•

Ulcerative colitis • ➢ Atrophic, distorted mucosa ➢ Thick muscularis mucosa ➢ Fat within submucosa

Crohn disease ➢ Ulcers, fissures, transmural lymphoid aggregates, granulomas

Figure 2-13-1

Crohn disease ➢ Aphthous ulcer ➢ Lymphoid aggregates Crohn disease ➢ Fissures ➢ Transmural lymphocytes

Gross pathologic specimens show ulcerative colitis with a hemorrhagic ulcerated mucosa and mural thinning. Crohn disease has marked mural thickening, mucosal cobblestones, and proliferation of adjacent mesenteric fat Gastrointestinal Radiology

383

Idiopathic Inflammatory Bowel Disease

Ulcerative Colitis: Imaging Features Acute Changes [Figure 2-13-2]

Figure 2-13-2

• • • • • •

Mucosal granularity Mucosal stippling Collar button ulcers Haustral thickening or loss Inflammatory polyps Confluent, contiguous, circumferential disease

• • • • • •

Haustra loss Luminal narrowing Loss of rectal valves Widened presacral space Backwash ileitis Post-inflammatory pseudopolyps

•

Early, acute disease ➢ Low diagnostic sensitivity ➢ Often normal Complementary to endoscopy to assess for complications ➢ Toxic megacolon ➢ Pneumatosis ➢ Perforation

Ulcerative Colitis: Imaging Features Chronic Changes

Ulcerative Colitis: Role of CT •

Gore RM et al. AJR 1996: 167:3-15.

Ulcerative Colitis: CT Features [Figures 2-13-3 and 2-13-4] •

•

Severe, acute disease ➢ Mural thinning ➢ Pneumatosis ➢ Perforation Subacute and chronic disease ➢ Mural thickening ➢ Luminal narrowing ➢ Proliferation of perirectal fat ➢ Assessment/detection of carcinoma

Figure 2-13-4

Acute ulcerative colitis with fine mucosal ulceration producing granularity and stippling on barium evaluation

Figure 2-13-3

Gore RM et al. AJR 1996: 167:3-15.

Chronic ulcerative colitis with mural stratification (target sign), submucosal fat in the rectum, and an increase in the perirectal fat Idiopathic Inflammatory Bowel Disease

Acute ulcerative colitis with mild mural thickening and pericolonic hyperemia on CT 384

Gastrointestinal Radiology

Ulcerative Colitis •

Mural stratification and luminal narrowing in chronic UC ➢ Thickening of muscularis mucosa ➢ Edema and fat in submucosa

Ulcerative Colitis: Perirectal Fat Proliferation Toxic Megacolon and IBD •

• • • •

Clinical features ➢ Fever, tachycardia, hypotension Incidence ➢ 5% to 10% of UC ➢ 2% to 4% Crohn disease ➢ May be initial manifestation of IBD Other causes ➢ Pseudomembranous colitis ➢ Ischemia ➢ Infection Pathology ➢ Transmural inflammation ➢ Loss of normal tissue cohesion ➢ Thinned wall and areas of denuded mucosa Imaging ➢ Abdominal radiograph establishes diagnosis ➢ Marked colonic dilatation, 6 to 15 cm ➢ Transverse colon most often involved ➢ Nodular mucosa ➢ Loss of haustra ➢ Intraluminal fluid

Figure 2-13-5

Toxic Megacolon [Figure 2-13-5] •

Ulcerative colitis ➢ Colonic distension ➢ Pseudopolyps

Ulcerative Colitis Differential CT Features •

CT features suggesting UC over Crohn disease ➢ Mural stratification, 61% UC vs. 8% Crohn ➢ Mural thickness less in UC compared to Crohn ➢ Outer colonic contour, smooth in UC and irregular in Crohn

Gore RM et al. AJR 1996: 167:3-15

Crohn Disease Early Imaging Features •

• • • • •

Distribution ➢ Asymmetric ➢ Segmental ➢ Skip lesions Lymphoid hyperplasia Ulceration ➢ Aphthous ulcerations ➢ Linear ulcers ➢ Deep ulcerations (fissuring) Cobblestoning Mural thickening Inflammatory pseudopolyps

Gastrointestinal Radiology

Ulcerative colitis with toxic megacolon

385

Idiopathic Inflammatory Bowel Disease

Crohn Disease: Aphthous Ulcers [Figure 2-13-6]

Figure 2-13-6

Crohn Disease: Linear Ulceration and Nodules Crohn Disease •

Rose thorn ulcers

Crohn Disease: Cobblestoning [Figure 2-13-7] Crohn Disease [Figure 2-13-8] Figure 2-13-7

Figure 2-13-8

Crohn disease with aphthous ulcers

Crohn disease with cobblestoning

Crohn disease with terminal ileal nodularity and ulceration Idiopathic Inflammatory Bowel Disease

386

Gastrointestinal Radiology

Crohn Disease: Acute/Early CT Features • •

Figure 2-13-9

Mural thickening ➢ 1 to 2 cm Mural stratification ➢ Mural enhancement ➢ Target or double halo sign

Crohn Disease • •

Mesenteric hyperemia Target sign ➢ Acute inflammation

Crohn Disease • • •

Linear ulceration Mural thickening Inflammatory polyps

•

Mural thickening ➢ Homogeneous, nonenhancing = fibrosis or stricture ➢ Enhancing wall = reversible inflammatory disease Mesenteric changes ➢ Fibrofatty proliferation ➢ Lymphadenopathy ➢ Hypervascularity ➢ Inflammatory stranding ➢ Phelgmon/abscess

Crohn Disease: Subacute to Chronic CT Features •

Crohn Disease •

•

Homogeneous mural thickening ➢ Nonenhancing = irreversible fibrosis Loss of mural stratification

Crohn Disease

Crohn disease with distal ileal inflammation (target sign), fibrofatty proliferation of the small bowel mesentery, and mesenteric lymphadenopathy

Crohn Disease [Figure 2-13-9] •

•

Fibrofatty proliferation ➢ “creeping fat” Mesenteric lymphadenopathy, 3 to 8 mm

Crohn Disease [Figure 2-13-10] •

Prominent, dilated vasa recta ➢ “comb sign”

Figure 2-13-10

Crohn disease with prominent and engorged vasa recta (comb sign) Gastrointestinal Radiology

387

Idiopathic Inflammatory Bowel Disease

Crohn Disease: Chronic Features • • • • •

Fissures, fistulas, and sinus tracts Haustral loss Strictures Sacculations Post-inflammatory pseudopolyps

Figure 2-13-11

Crohn Disease: Strictures

Sacculations and Strictures [Figure 2-13-11]

Crohn Disease: Stricture Crohn Disease •

Complications ➢ Sinus tracts ➢ Fistula ➢ Abscess ➢ Carcinoma

Crohn Disease Intramural Fistula

Crohn Disease Fistulae [Figure 2-13-12]

Crohn Disease: Abscess [Figure 2-13-13]

•

• •

Chronic Crohn disease with structuring and sacculations

Secondary to deep penetrating ulcers ➢ Sinus tracts ➢ Fistulas ➢ Perforation 15% to 20% of patients Most frequently associated with small bowel or ileocolic disease

Figure 2-13-12

Crohn Disease: Perirectal Sinus Tract

Figure 2-13-13

Crohn disease with a ileal-ileal fistula

Crohn disease with a psoas abscess Idiopathic Inflammatory Bowel Disease

388

Gastrointestinal Radiology

Crohn Disease: Extraintestinal Complications •

• • •

Hepatobiliary ➢ Hepatic steatosis, 20% to 50% ➢ Cholelithiasis, 30% to 50% ➢ PSC, 1% to 4% ➢ Hepatic abscess Pancreatic ➢ Pancreatitis Musculoskeletal ➢ Arthritis ➢ Sacroileitis-spondylitis GU tract ➢ Nephrolithiasis, 2% to 10%

Neoplasia in IBD •

• •

Adenocarcinoma ➢ Ulcerative colitis, highest incidence ➢ Crohn disease, small and large intestine Lymphoma ➢ Increased incidence in Crohn disease Features of carcinoma in IBD ➢ Typically do not arise in pre-existing adenomas ➢ Arise in flat mucosa ➢ Carcinomas may be long and flat ➢ May arise in bypassed segments of bowel

Adenocarcinoma in Ulcerative Colitis Adenocarcinoma in Crohn Disease

Additional Imaging Modalities: Sonography UC vs. Crohn Disease

•

Ulcerative colitis ➢ Hypoechoic wall ➢ Mural stratification ➢ Loss of haustra ➢ Loss of peristalsis

•

Crohn disease ➢ Hypoechoic wall ➢ Loss of mural stratification ➢ Loss of haustration ➢ Diminished compressibility ➢ Absent peristalsis ➢ Increased blood flow

Gore RM, Laufer I, Berlin, JW. Ulcerative and granulomatous colitis: idiopathic inflammatory bowel disease. In: Gore RM, Levine MS (eds), Textbook of Gastrointestinal Radiology. 2nd ed. Sarrazin J, Wilson SR. Manifestations of crohn disease at US. RadioGraphics 1996. 16: 499-520.

Additional Imaging Modalities: MR Enterography of Crohn Disease • •

Evolving technique Assessment of active disease ➢ Mural thickening > 4mm ➢ Mural enhancement ➢ Increase in mesenteric vascularity

Koh DM et al. MR imaging evaluation of the activity of crohn’s disease. AJR 2001: 177(6) 1325-1332.

Gastrointestinal Radiology

389

Idiopathic Inflammatory Bowel Disease

Establishing Diagnosis: UC vs. Crohn • • • •

Clinical course Disease distribution Endoscopy findings Biopsy

•

Mural thickening ➢ Greatest with Crohn disease Submucosal fat ➢ More commonly seen in UC Mesenteric fibrofatty proliferation ➢ Crohn disease ➢ But, perirectal fat may increase in UC Sinus tracts, fistulas, abscess ➢ Crohn disease

UC vs. Crohn Disease: CT Features • • •

Differential Diagnosis of IBD • • • • • •

Infectious colitis Ischemic colitis Radiation enteropathy and colitis Behçet disease Graft vs. host disease Diverticular disease

•

UC vs. Crohn ➢ Similar demographics ➢ UC, contiguous colonic disease ➢ Crohn, entire GI tract with skip areas ➢ UC, mucosal and submucosal disease ➢ Crohn, transmural disease with extension into the mesentery

Summary

Idiopathic Inflammatory Bowel Disease

390

Gastrointestinal Radiology

Approach to Inflammatory Disease of the Colon Angela D. Levy, COL, MC, USA Objectives • •

General approach Differential diagnosis of idiopathic IBD ➢ Pseudomembranous colitis ➢ Neutropenic colitis (typhlitis) ➢ Ischemic colitis ➢ Diverticulitis ➢ Infectious colitis

General Approach •

• • •

Disease location ➢ Small vs. large bowel ➢ Focal vs. multifocal vs. diffuse ➢ Ascites Degree and pattern of mural thickening ➢ How much mural thickening? ➢ Mural enhancement? ➢ Fat attenuation in the submucosa? Associated mesenteric disease ➢ Inflammation ➢ Phelgmon/abscess ➢ Mesenteric fat proliferation Clinical history

Helpful Features for Differential Diagnosis •

•

•

Location of disease ➢ Small bowel involvement ➢ Diffuse vs. focal vs. multifocal Degree of mural thickening ➢ Marked mural thickening favors PMC Clinical history ➢ Antibiotics (PMC) ➢ Radiation therapy ➢ Neutropenia ➢ Travel history

Colitis Differential Diagnosis

•

Right-sided disease ➢ Campylobacter ➢ Yersinia ➢ Salmonella ➢ Typhlitis ➢ Crohn disease ➢ TB ➢ Amebiasis ➢ Histoplasmosis

Gastrointestinal Radiology

•

Diffuse disease ➢ Ischemia ➢ PMC ➢ E. Coli ➢ Shigella, campylobacter, salmonella, amebiasis ➢ CMV ➢ Inflammatory bowel disease ➢ Behçet syndrome ➢ Graft vs. host disease (GVDH) ➢ Radiation

391

Inflammatory Disease of the Colon

41-year-old man who developed diarrhea one month after hospitalization for pneumonia [Figure 2-14-1]

Figure 2-14-1

Pseudomembranous Colitis •

Features suggesting PMC ➢ Diffuse colonic involvement ➢ Marked low attenuation mural thickening ➢ Accordion sign ➢ Ascites

Pseudomembranous Colitis • •

Onset following antibiotic therapy ➢ Clostridium difficile toxin Clinical features ➢ Symptoms within days or weeks following antibiotic therapy ➢ Copious watery diarrhea ➢ Abdominal pain ➢ Fever ➢ Leukocytosis

Pseudomembranous Colitis • • • •

Epithelial necrosis Inflammatory infiltrate Crypt eruption Pseudomembranes

•

Mural thickening ➢ Low attenuation ➢ Accordion sign ➢ Target sign Intraluminal plaques or nodules Pericolonic inflammation Ascites Complications ➢ Luminal dilatation, toxic megacolon ➢ Perforation

Pseudomembranous colitis

Figure 2-14-2

Pseudomembranous Colitis: CT Features

• • • •

Pseudomembranous Colitis: Thumbprinting = Mural Thickening

Pseudomembranous Colitis: Low Attenuation Wall with Accordion Sign

Pseudomembranous Colitis: Target Sign [Figure 2-14-2] • •

PMC typically has the greatest amount of mural thickening of the compared to other colitis Presence of ascites favors acute colitis over IBD ➢ PMC, infectious, ischemia

Pseudomembranous colitis

65-year-old woman abdominal pain during chemotherapy for leukemia [Figure 2-14-3] Neutropenic Colitis (Typhlitis) •

•

Suggestive features ➢ Clinical history ➢ Right-sided involvement Clinical features ➢ Children and adults ➢ Neutrophil counts <500 to 1000 cells/mm3 ➢ Fever, diarrhea, pain, abdominal distension

Inflammatory Disease of the Colon

392

Gastrointestinal Radiology

• •

Figure 2-14-3

Pathogenesis ➢ Cecal stasis ➢ Cytotoxic mucosal injury ➢ Bacterial invasion CT features ➢ Predominant right-sided disease ➢ Mural thickening, low attenuation ➢ Pericolonic inflammation ➢ Ascites ➢ Pneumatosis

70-year-old man with acute abdominal pain and bloody diarrhea [Figure 2-14-4] Ischemic Colitis •

•

•

Most common in elderly ➢ Underlying atherosclerosis, diabetes, hypertension ➢ Low flow states ➢ Occlusive disease ➢ Complicates infectious colitis, especially CMV Ischemic Injury ➢ Acute, fulminant ✧ Transmural necrosis and perforation ➢ Transient, reversible ✧ Confined to mucosa and submucosa ➢ Chronic ✧ Submucosal fibrosis Locations ➢ Diffuse ➢ SMA and IMA watershed vunerable ✧ Normal communication through Arc of Riolan (marginal artery of Drummond) ✧ Absent in 5% ✧ Splenic flexure and rectosigmoid

Neutropenic colitis

Figure 2-14-4

Ischemic Colitis • •

Denuded mucosa Pseudomembranes, Hemorrhage

• •

Mucosal ulceration Mural thickening ➢ Low attenuation ➢ Target sign Luminal dilatation Pericolonic inflammation Ascites Chronic changes ➢ Fibrosis, stricture

Ischemic Colitis- Imaging Features

• • • •

Ischemic Colitis- Acute Figure 2-14-5

[Figure 2-14-4 and 2-14-5]

Ischemic colitis

Ischemic colitis Gastrointestinal Radiology

393

Inflammatory Disease of the Colon

Ischemic Enteritis - Pathophysiology •

Figure 2-14-6

Blood supply reduced by >50% ➢ Arterial occlusive ➢ Venous occlusive ➢ Nonocclusive (low flow states)

Ischemic Enteritis • • • •

•

• • • •

Segmental Diffuse Segmental, necrotic, ulcerated mucosa Mural thickening ➢ Stack of coins Thick wall, engorged mesentery ➢ Target sign [Figure 2-14-6] Thin wall, infiltrated mesentery ➢ Infiltrated mesentery [Figure 2-14-7] Mucosal cast, intramural fistula Mucosal ulceration, pneumatosis Pneumatosis, mesenteric venous gas

Figure 2-14-7

Ischemic enteritis due to SMV thrombosis

Figure 2-14-8

. Ischemic enteritis

Ischemic Colitis or Enteritis [Figure 2-14-8] •

Chronic

•

Ileus ➢ Dilated bowel ➢ Gasless abdomen ➢ Unchanging bowel Mural thickening ➢ Stack of coins ➢ Target sign Mucosal ulceration ➢ Ulceration ➢ Intramural fistulas ➢ Loss of folds Mucosal breakdown ➢ Intraluminal mucosal cast ➢ Pneumatosis ➢ Mesenteric or portal venous gas ➢ Intraperitoneal air

Imaging Findings Suggestive of Ischemia

• • •

Inflammatory Disease of the Colon

394

Chronic ischemic colitis in the watershed region of the colon

Gastrointestinal Radiology

50-year-old woman with abdominal pain and fever [Figure 2-14-9] •

Diverticulitis - Hepatic Flexure

•

Most common disease of the colon Diverticulosis increases with age ➢ 33% to 50% of people over 50 ➢ 50% of people over 80

Figure 2-14-9

Diverticular Disease •

Acute diverticulitis

Diverticular Disease Etiology of Pulsion Diverticula •

•

Pressure gradient ➢ Between lumen and serosa ➢ Compartmentalized by haustra ➢ Highest in sigmoid Weakness in bowel wall ➢ Intramural vasa recta penetrate wall ➢ Between taenia mesocolica and taenia libera ➢ Between taenia mesocolica and taenia omentalis

Diverticular Disease - Pathologic Features •

•

False diverticula ➢ Mucosa and submucosa only ➢ 0.5 to 1.0 cm Myochosis ➢ Thickening of circular muscle ➢ Shortening of taenia ➢ Narrowing of the lumen ➢ Results in corrugated appearance

Diverticular Disease: CT Features • •

Mural thickening Diverticular outpouchings

• • •

Diverticulitis Hemorrhage Giant sigmoid diverticulum

•

Most common complication ➢ 10% to 20% of patients with diverticulosis Pathogenesis ➢ Stagnation of fecal material ➢ Inflammatory erosion of the mucosa ➢ Perforation ✧ Intramural abscess ✧ Extramural abscess

Diverticular Disease: Complications Diverticulitis •

Diverticular Disease: Complications •

Diverticulitis ➢ Luminal obstruction ➢ Infection

Gastrointestinal Radiology

395

Inflammatory Disease of the Colon

Diverticulitis: CT Features •

•

• • •

Figure 2-14-10

Diverticula ➢ May have hyperdense fecal material Inflammatory changes ➢ Pericolonic stranding ➢ Pericolonic phlegmon ➢ Intramural abscess ➢ Pericolonic abscess Circumferential mural thickening ➢ Usually < 1 cm ➢ Rarely exceeds 2-3 cm Tethered lumen ➢ Saw-tooth configuration ➢ Due to muscular spasm Pneumoperitoneum, abscess

Diverticulitis: Hyperdense Fecal Material

Diverticulitis: Intramural Fistula - Pneumatosis [Figure 2-14-10]

Diverticulitis: Complications • • • •

• • •

Hemorrhage 25% Muscular hypertrophy and obstruction 25% Pericolic abscess 20% Free perforation 18% ➢ Debilitated patients ➢ Corticosteroid therapy Vesicocolic fistula 8% Small bowel obstruction Pyelophlebitis and liver abscess

Acute diverticulitis with intramural fistula and pneumatosis

Diverticulitis: Perforation

Diverticulitis: Pericolic Abscess

Diverticulitis: Colovesical Fistula Differential Diagnosis • •

• •

Colon carcinoma Colitis ➢ Infectious ➢ Ischemic ➢ Crohn disease Foreign body perforation Epiploic appendagitis

Diverticulitis vs. Carcinoma •

• •

•

Wall thickening ➢ Mild circumferential thickening in diverticulitis (4 to 5 mm) ➢ Carcinoma usually > 2 cm Zone of transition ➢ Abrupt change in lumen caliber favors carcinoma ➢ Lobulated soft-tissue favors carcinoma ➢ Tethered (saw-tooth) lumen favors diverticulitis Inflammatory changes ➢ Favors diverticulitis Regional adenopathy ➢ Favors carcinoma

Inflammatory Disease of the Colon

396

Gastrointestinal Radiology

Diverticulitis vs. Carcinoma

Figure 2-14-11

Diverticulitis: CT Pitfalls •

Differential diagnosis of colon cancer ➢ Problematic in 10% of cases ➢ If immediate surgery not performed: mucosal evaluation (endoscopy or BE) to exclude cancer

Diverticulitis: CT and Surgical Management •

• • •

Antibiotic therapy ➢ Mild Diverticulitis CT guided drainage ➢ Focal abscess Immediate Surgery ➢ Free perforation with peritonitis ➢ Severe hemorrhage Elective surgical resection ➢ Following successful abscess drainage ➢ Recurrent diverticulitis ➢ Persistent pain ➢ Bleeding

Diverticular Hemorrhage • •

•

Most common cause of adult rectal bleeding Site of hemorrhage ➢ Single diverticulum ➢ Right side of colon in 2/3 of cases Rupture of the vasa recta

Giant sigmoid diverticulum

Diverticular Hemorrhage •

Clinical features ➢ Elderly patients ➢ Sudden onset ➢ Stops spontaneously in 80% ➢ Rebleeding in 25%

Cecal Diverticulitis •

•

Congenital or acquired diverticula ➢ Congenital are true diverticula ➢ Acquired are most common CT features ➢ Pericolonic inflammation ➢ Mural thickening ➢ Demonstration of diverticula ➢ Intramural or pericolonic abscess ➢ Normal appendix

Giant Sigmoid Diverticulum [Figure 2-14-11] • •

•

Rare Etiology ➢ Subserosal perforation and inflammation ➢ Air trapping ➢ Ball-valve mechanism Clinical Features ➢ Chronic pain ➢ Palpable mass

Gastrointestinal Radiology

397

Inflammatory Disease of the Colon

Giant Sigmoid Diverticulum •

Figure 2-14-12

CT features ➢ Mesenteric side of sigmoid colon ➢ Usually 7 cm or greater ➢ Thin wall ➢ Unilocular

30-year-old man with diarrhea and pain following vacation in Mexico [Figure 2-14-12] Campylobacter Colitis ➢ Infectious colitis ✧ Distribution ✧ Clinical

Summary •

General approach ➢ Location of disease ➢ Degree of mural thickening ➢ Ascites ➢ Mesenteric disease ➢ Clinical history

Summary - Pseudomembranous Colitis •

• • • •

History of antibiotics ➢ Clostridium difficile Mural thickening Accordion sign Pericolonic inflammation Ascites

Campylobacter colitis

Summary - Neutropenic Colitis/Typhlitis • • •

Chemotherapy ➢ Low neutrophil counts Bacterial invasion Predominant right-sided disease ➢ Mural thickening ➢ Pneumatosis ➢ Ascites

Summary - Ischemic Colitis •

• •

Elderly patients ➢ Diffuse ➢ Watershed regions Acute ➢ Mucosal ulceration ➢ Mural thickening Chronic ➢ Mural thickening ➢ Stenosis

Summary - Diverticulitis • •

•

Focal disease Diverticula ➢ Air filled ➢ Hyperdense Adjacent inflammation

Inflammatory Disease of the Colon

398

Gastrointestinal Radiology

Summary - Diverticulitis vs. Carcinoma •

•

Favor Diverticulitis ➢ Lesser mural thickening ➢ Gradual zone of transition ➢ Tethered lumen ➢ Pericolonic inflammation Favor carcinoma ➢ Greater mural thickening ➢ Lobular mural thickening ➢ Sharp zone of transition ➢ Pericolonic adenopathy

Summary •

• •

Infectious colitis ➢ Clinical history Right-sided colitis ➢ Campylobacter, yersinia, salmonella ➢ TB ➢ Amebiasis Diffuse colitis ➢ PMC ➢ E. Coli ➢ Shigella, campylobacter, salmonella ➢ Amebiasis ➢ CMV

Gastrointestinal Radiology

399

Inflammatory Disease of the Colon

Gastrointestinal Seminar 1: Abdominal Gas Angela D. Levy, COL, MC, USA

Case 1: 45 year old man with chronic pancreatitis and acute onset of lower abdominal pain, distension, and constipation

Marked Cecal Dilatation •

Cecal volvulus

Differential diagnosis ➢ Cecal volvulus ➢ Cecal bascule ➢ Pseudoobstruction (Ogilvie syndrome)

Cecal Volvulus •

• •

Volvulus is an axial twist of at least 90 degrees ➢ Abnormal fixation to posterior parietal peritoneum ➢ Freely mobile cecum Mechanical obstruction Radiographic features ➢ Cecal dilatation ➢ Beak on contrast enema ➢ Whirl on CT

Cecal volvulus Seminar 1: Abdominal Gas

400

Gastrointestinal Radiology

Cecal Bascule •

Anterior-cephalad fold ➢ May cause obstruction Volvulus:Bascule = 10:1

• •

Colonic pseudo-obstruction Marked cecal dilatation

•

Ogilvie Syndrome Sigmoid Volvulus

Transverse Colon Volvulus Cecal bascule

Sigmoid volvulus

Gastrointestinal Radiology

401

Seminar 1: Abdominal Gas

Case 2: 85 year old woman with abdominal pain, fever, and shock

Intestinal ischemia with infarction and hepatic portal venous gas

Intestinal Ischemia with Infarction and Hepatic Portal Venous Gas Hepatic Portal Venous Gas • • •

Branching radiolucencies extending to within two cm of the hepatic capsule Must differentiate from pneumobilia Differential Diagnosis: ➢ Bowel Necrosis (75%) ➢ IBD (10%) ➢ Abscess ➢ Obstruction ➢ Ulcer

Pneumobilia

Pneumobilia

Portal venous gas

Intestinal Infarction with Portal Venous Gas Portal venous gas

Seminar 1: Abdominal Gas

402

Gastrointestinal Radiology

Case 3: 60 year old man with progressive dyspepsia and acute, severe upper abdominal pain Pneumoperitoneum Gastric Ulcer Perforation Pneumoperitoneum Signs on the Supine Abdominal Film • • • • • •

Diaphragmatic Slips (Leaping Dolphins) Central Diaphragm (Cupola) Morison’s Pouch (Doge’s Cap) Lesser Sac Falciform Ligament Fissure of Ligamentum Teres

“Leaping Dolphin” Cupola Sign

“Doge’s Cap”

Pneumoperitoneum from gastric ulcer perforation

Case 4: 67 year old man with severe chest pain after vomiting Boerhaave’s Syndrome • •

• •

Dr. Hermann Boerhaave Emetogenic rupture ➢ Distal esophagus or ➢ Gastric cardia Left posterolateral region ➢ 1.5 - 4 cm tear ➢ Reduced muscle fibers ➢ Entrance of nerves, vessels Radiographic features ➢ Mediastinal gas ➢ LLL infiltrate, atelectasis ➢ Left effusion

Causes of Esophageal Rupture •

• •

Spontaneous ➢ Boerhaave ➢ Mallory-Weiss Iatrogenic ➢ Endoscopic ➢ Dilation ➢ Tube placement Other ➢ Caustic Ingestion ➢ Trauma ➢ Inflammatory ➢ Neoplastic

Boerhaave syndrome Gastrointestinal Radiology

403

Seminar 1: Abdominal Gas

Case 5: 50 year old man with upper abdominal pain, epigastric fullness, and constipation Cecal Herniation through the Foramen of Winslow Foramen of Winslow

Foramen Of Winslow Hernia • •

•

•

•

8% of internal hernias Involved bowel ➢ Small intestine 70% ➢ Cecum 25% Cecal herniation ➢ Persistence of ascending mesocolon ➢ Mobility ➢ Alterations in intraabdominal pressure Radiographic features Cecal herniation through the foramen of Winslow ➢ Circumscribed gas collection LUQ ➢ Medial and posterior to stomach ➢ Stomach displaced left and anterior ➢ Small bowel obstruction Differential diagnosis ➢ Gas in lesser sac from abscess or perforation

Cecal herniation through the foramen of Winslow

Seminar 1: Abdominal Gas

404

Gastrointestinal Radiology

Gastrointestinal Seminar 2: Nonneoplastic Disease of the Stomach Angela D. Levy, COL, MC, USA

Case 1: 65-year-old woman presents with retching and the production of little vomitus. The ER physician cannot pass a NG tube into her stomach

Gastric Volvulus •

•

Abnormal rotation of the stomach ➢ Rare ➢ Children and adults ➢ Most are secondary to fixation defect Associated anomalies ➢ Diaphragmatic defects ➢ Malrotation ➢ Wandering spleen ➢ Asplenia

Mesenteroaxial volvulus

Gastric Volvulus •

•

Classic Clinical presentation ➢ Severe epigastric pain ➢ Violent retching with production of little vomitus ➢ Inability to pass NG tube into stomach Outcomes ➢ Recurrent ➢ Resolve spontaneously ➢ Resolve with NG placement ➢ Complete obstruction

Gastrointestinal Radiology

405

Seminar 2: Nonneoplastic Disease of the Stomach

•

• • •

Organoaxial ➢ Rotation about a line extending from cardia to pylorus Mesenteroaxial ➢ Rotation about a line connecting middle of lesser curvature to middle of greater curvature Mixed types occur 30% associated with hiatal hernia

Gastric Volvulus - Radiologic Features • •

• •

Double air-fluid level Inversion of stomach ➢ Greater curve above lesser curve Positioning of cardia and pylorus at the same level Downward pointing pylorus and duodenum

Organoaxial Volvulus

Mechanism for organoaxial volvulus

Mesenteroaxial Volvulus

Mechanism for mesenteroaxial volvulus

Seminar 2: Nonneoplastic Disease of the Stomach

406

Gastrointestinal Radiology

Case 2: 22-year-old woman developed epigastric pain when she was dieting in preparation for her wedding Gastric Bezoar • •

• •

Accumulated ingested material Trichobezoar ➢ Hair Phytobezoar ➢ Vegetable matter Pathophysiology ➢ Altered gastric motility ➢ Altered gastric anatomy ➢ Trichotillomania

Gastrointestinal Radiology

Gastric Bezoar

407

Seminar 2: Nonneoplastic Disease of the Stomach

Case 3: 8-year-old girl with recurrent emesis and diarrhea Imaging findings • • • • •

Thick gastric folds Thick duodenal wall Liver metastasis Large, enhancing pancreatic mass Positive pentetreotide scan

Zollinger-Ellison Syndrome: Pancreatic Gastrinoma Zollinger-Ellison Syndrome • •

• •

Affects all ages, peak 3rd to 5th decade Gastrin-secreting neuroendocrine tumor (gastrinoma) ➢ Pancreas (75%) ➢ Duodenum (15%) ➢ Liver, ovary, lymph nodes ➢ 60% malignant Clinical Features ➢ One or more benign peptic ulcers ➢ Diarrhea from hypergastrinemia (30%) ➢ Elevated gastrin levels May occur in MEN I syndrome

Pancreatic gastrinoma producing Zollinger Ellison syndrome

Zollinger-Ellison Syndrome •

•

Radiologic features ➢ Multiple ulcers ➢ Increased gastric secretions ➢ Thick gastric folds Preoperative localization of gastrinoma ➢ CT ➢ MR ➢ Somatostatin receptor scintigraphy

47-year-old man with recurrent PUD

Ectopic Gastrinoma in Retroperitoneal Lymph Node Thick Gastric Wall? Gastric Air-Fluid Sign*

*Hammerman AM, Mirowitz SA, Susman N. Gastrointestinal Radiology 14:109112. 1989

Differential Diagnosis: Thick Gastric Folds •

• • •

Hypertrophic Gastropathy ➢ Menetrier disease ➢ Zollinger-Ellison syndrome Gastritis Neoplasm ➢ Adenocarcinoma ➢ Lymphoma ➢ Metastasis Miscellaneous ➢ Amyloid ➢ Eosinophilic gastritis ➢ Adjacent inflammation

Seminar 2: Nonneoplastic Disease of the Stomach

408

Gastrointestinal Radiology

Case 4: 70-year-old man presents epigastric pain and pedal edema

Menetrier Disease

Menetrier Disease : Adult Form • •

• • • •

Most common in men, 50 to 70 years Symptoms ➢ Epigastric pain ➢ Vomiting ➢ Weight loss ➢ Peripheral edema Hypoalbuminemia and hypochlorhydria Diffuse enlargement of gastric folds ➢ Proximal stomach Mucus hypersecretion Irreversible

Menetrier Disease : Pediatric Form • • •

• • •

Associated with CMV infection Allergic or autoimmune reaction Symptoms ➢ Periorbital and facial edema ➢ Vomiting ➢ Pain Self-limited Spontaneous resolution and reversal of protein loss Antrum more commonly involved

Menetrier Disease : Pathology •

Pathology ➢ Foveolar hyperplasia, glandular atrophy, cysts ➢ Enlarged folds (1-3 cm) resembling cerebral convolutions ➢ H. pylori?

Menetrier Disease : Radiology •

• • •

Thick folds ➢ Nonuniform ➢ Tortuous Spiculation of greater curvature Antral sparing Flocculation of contrast

Gastrointestinal Radiology

409

Seminar 2: Nonneoplastic Disease of the Stomach

Case 5: UGI images from two different patients that complained of epigastric pain. Both patients had a history of diarrhea Case 5A: Radiologic Findings • • • • •

Narrowed antrum Multiple filling defects Nodularity Ulceration? Effaced/nodular duodenal bulb

•

Multiple ulcers ➢ Aphthous ulcers Nodularity

Case 5B

Case 5A

Case 5B: Radiologic Findings •

Crohn disease

Case 5 : Differential Diagnosis •

• •

Gastritis ➢ H. pylori ➢ Radiation ➢ Caustic ingestion Neoplasm ➢ Adenocarcinoma ➢ Lymphoma ➢ Mets Granulomatous disease ➢ Crohn ➢ Sarcoid ➢ Amyloid ➢ TB

Gastric Crohn Disease • • •

Histologically present in up to 33% of patients with Crohn disease 20% of patients with ileo-colic disease have abnormal UGI1 Antrum and duodenum most often affected

1Levine MS. Crohn’s disease of the upper gastrointestinal tract. RCNA 1987

Gastric Crohn Disease •

• • •

Ulcers ➢ Aphthous lesions ➢ One or more large ulcers Nodules ➢ Focal nodules ➢ Cobblestone mucosa Abnormal gastric motility Fibrosis ➢ Tubular antrum (ram’s horn sign or shofar sign) ➢ Obliteration of the pylorus (pseudo-Billroth I sign)

Seminar 2: Nonneoplastic Disease of the Stomach

410

Gastrointestinal Radiology

Gastrointestinal Seminar 3: Pancreatic Duct Angela D. Levy, COL, MC, USA

18 days

Normal Anatomy •

•

Minor Papilla ➢ Accessory PD ➢ Duct of Santorini Major Papilla ➢ Main PD ➢ Duct of Wirsung

Normal Pancreatic Embryology

5 weeks

Normal pancreatic and biliary duct anatomy

22 days

5 weeks

Anatomic variants of the pancreatic duct

Normal pancreatic duct by ERCP and MRCP Gastrointestinal Radiology

411

Seminar 3: Pancreatic duct

Case 1: 25-year-old woman with a long history of nausea, vomiting, and abdominal distension Annular Pancreas • • •

Bilobed ventral pancreatic bud Buds migrate in opposite directions Duodenal obstruction

Proposed mechanism for annular pancreas

Annular pancreas

Seminar 3: Pancreatic duct

412

Gastrointestinal Radiology

Case 2: 17-year-old female with abdominal pain and elevated LFT’s

Pancreatic divisum with focal chronic pancreatitis

Pancreatic Divisum and Chronic Pancreatitis Pancreatic Divisum • • • • •

Incomplete fusion of dorsal and ventral pancreas Body and tail drain through the duct of Santorini, minor papilla Incidence ➢ 4 to 11% (autopsy) ➢ 3 to 4% (ERCP) Most asymptomatic 12-24% develop idiopathic recurrent pancreatitis Choledocholithiasis in a patient with pancreatic divisum by MRCP

Gastrointestinal Radiology

413

Seminar 3: Pancreatic duct

Case 3: Two Different Patients with the same Disease

Chronic Pancreatitis

Chronic Pancreatitis: Ductal Features •

• • •

•

Ectasia ➢ Loss of normal tapering Contour irregularity Side branches ➢ Clubbing ➢ Stenosis ➢ Opacification of cavities Stenoses or occlusion ➢ “Chain of lakes” Intraductal calculi

Chronic Pancreatitis

Chronic pancreatitis by MRCP

Seminar 3: Pancreatic duct

414

Gastrointestinal Radiology

Case 4: 50-year-old man with abdominal pain

Intraductal papillary mucinous neoplasm

Intraductal Papillary Mucinous Neoplasm IPMN: Imaging •

• • •

Duct dilatation ➢ Focal or diffuse ➢ Main duct or side branch Intraductal masses Bulging duodenal papilla Glandular atrophy

Main Duct IMPN

Side Branch IPMN Bulging Papilla

Gastrointestinal Radiology

415

Seminar 3: Pancreatic duct

Case 5: 45-year-old man with chest pain and elevation of serum amylase

Mediastinal pseudocyst from acute pancreatitis

Mediastinal Pseudocyst

Fluid Collections and Pancreatitis •

50% of patients ➢ Rupture of pancreatic duct ➢ Exudation of fluid from gland surface

Seminar 3: Pancreatic duct

416

Gastrointestinal Radiology

Gastrointestinal Seminar 4: Hepatic Imaging Angela D. Levy, COL, MC, USA Case 1: 50-year-old woman with vague abdominal discomfort Differential Diagnosis: Complex Hepatic Cyst •

•

Nonneoplastic ➢ Echinococcal cyst ➢ Simple cyst with hemorrhage/infection ➢ Post-traumatic cyst ➢ Abscess ➢ Ciliated hepatic foregut cyst Neoplastic ➢ Biliary cystadenoma ➢ Biliary cystadenocarcinoma ➢ Cystic metastasis ➢ Peliosis ➢ Teratoma

Biliary cystadenoma

Biliary Cystadenoma Simple Cyst

Echinococcus granulosus

Pyogenic Hepatic Abscess

Gastrointestinal Radiology

417

Seminar 4: Hepatic Imaging

Case 2: 10-year-old girl with right upper quadrant pain

Focal nodular hyperplasia

Differential Diagnosis: Hepatic Mass with a Scar • • • • •

Focal nodular hyperplasia Fibrolamellar carcinoma Hepatocellular carcinoma Hepatocellular adenoma Hemangioma

Hepatic Mass with a Scar

Focal Nodular Hyperplasia Fibrolamellar Carcinoma

Hepatocellular Carcinoma (HCC)

Hepatocellular Adenoma (HCA) with Fibrosis

Hemangioma

Hemangioma: Tagged RBC Scan

Seminar 4: Hepatic Imaging

418

Gastrointestinal Radiology

Case 3: 54-year-old man with right upper quadrant pain and jaundice

Intrahepatic cholangiocarcinoma

Differential Diagnosis: Rim-like Enhancement • • • • • •

Hemangioma Metastatic disease Hepatocellular carcinoma Intrahepatic cholangiocarcinoma Angiosarcoma Epithelioid hemangioendothelioma

Intrahepatic Cholangiocarcinoma Hemangioma

Epithelioid Hemangioendothelioma

Gastrointestinal Radiology

419

Seminar 4: Hepatic Imaging

Case 4: 50-year-old male with vague abdominal pain

Hepatocellular carcinoma

Differential Diagnosis: Liver Mass with Fat

• • • • • •

Hepatocellular carcinoma Angiomyolipoma Myelolipoma Hepatocellular adenoma Metastasis ➢ Liposarcoma VERY RARE, Teratoma

Hepatocellular Carcinoma

Hepatocellular Adenoma: Focal Fat and Capsule Hepatocellular Adenoma: Diffuse Hypodensity

Angiomyolipoma Myelolipoma

Hepatic Teratoma

Seminar 4: Hepatic Imaging

420

Gastrointestinal Radiology

Case 5: 26-year-old woman with RUQ pain and mild elevation of serum AST

Fibrolamellar carcinoma

Differential Diagnosis: Calcified Liver Mass •

• •

Nonneoplastic ➢ Hematoma ➢ Simple cyst ➢ Parasitic infection ➢ Healed infection Benign neoplasm ➢ Hemangioma ➢ Teratoma Malignant neoplasm ➢ Fibrolamellar carcinoma ➢ Epithelioid hemangioendothelioma ➢ Hepatoblastoma (kids)

Fibrolamellar Carcinoma

Colon Adenocarcinoma Metastases Echinococcus multilocularis

Gastrointestinal Radiology

421

Seminar 4: Hepatic Imaging

Gastrointestinal Seminar 5: Complications of Meckel Diverticulum Angela D. Levy, COL, MC, USA Cases 1-5 • • •

All patients have the same disease The underlying disease is a congenital anomaly Each presents with a different manifestation

• • • • • •

Most common anomaly of the GI tract 2% - 3% of the population M=F Symptoms more common in males 60% of patients present before age 10 Omphalomesenteric duct anomaly ➢ Improper closure and absorption

Omphalomesenteric (Vitelline) Duct Anomalies

Meckel Diverticulum

Omphalomesenteric (Vitelline) Duct • •

Embryonic connection between yolk sac and midgut 10th week of embryogenesis ➢ Midgut returns to abdomen ➢ Duct is a thin fibrous band connecting midgut to umbilicus ➢ Disintegrate ➢ Absorption

Umbilico-ileal fistula

Umbilical sinus

Umbilical cyst

Persistent fibrous cord

Meckel diverticula

Meckel diverticula with a fibrous attachment to the umbilicus

Meckel diverticula supported by a mesentery

Seminar 5: Meckel Diverticulum

422

Gastrointestinal Radiology

Omphalomesenteric (Vitelline) Duct Anomalies • • • • •

Umbilico-ileal fistula Umbilical sinus Umbilical cyst Persistent fibrous cord Meckel diverticulum ➢ With a fibrous cord ➢ With a portion of mesentery

Meckel Diverticulum: Pathology •

• •

Antimesenteric side of distal ileum ➢ Within 100 cm of ileocecal valve True diverticulum ➢ Composed of all layers of the small bowel wall Heterotopic tissue ➢ 50% of resected diverticula ➢ Gastric most common (23% - 50%) ➢ Pancreas (5% to 16%) ➢ Rare, Brunner glands, colonic, biliary

Meckel Diverticulum: Heterotopic Gastric Mucosa

Meckel Diverticulum: Heterotopic Pancreatic Mucosa

Gastrointestinal Radiology

423

Seminar 5: Meckel Diverticulum

Case 1: 22-year-old man with fever and guaiac positive stools

Meckel diverticulitis located in the midline because of persistent attachment to the umbilicus

Differential Diagnosis • • • • •

Inflammatory bowel disease Urachal remnant Colonic Diverticulitis Meckel diverticulitis Idiopathic ileal diverticula

Meckel Diverticulitis

Case 2: 22-year-old man with chronic abdominal pain and anemia

Hemorrhagic Meckel diverticulum

Differential Diagnosis • • • •

Neoplasm Ulcer Vascular ectasia Meckel diverticulum

Hemorrhagic Meckel Diverticulum

Angiographic Features of Meckel Diverticulum •

• •

Vitellointestinal artery ➢ Arises from a distal ileal branch of the SMA Tubular shaped angiographic blush Intraluminal contrast if brisk bleeding

Hemorrhage in Meckel Diverticulum • •

Most frequent complication Tc99-pertechnetate ➢ Localizes in ectopic gastric mucosa ➢ Modality of choice in pediatric population ➢ Sensitivity 85%, specificity 95% in kids ➢ Sensitivity 63%, specificity 2% in adults

Seminar 5: Meckel Diverticulum

424

Gastrointestinal Radiology

Case 3: 61-year-old woman with intermittent abdominal pain

Inverted Meckel diverticulum

Differential Diagnosis • •

Lipoma Inverted Meckel diverticula

Inverted Meckel Diverticulum

Inverted Meckel's Diverticulum with Intussusception Illustration of an inverted Meckel diverticulum

Case 4: 57-year-old man with abdominal pain and fever

Meckel diverticulitis with perforation and a stone

Meckel Diverticulitis with Perforation and a Stone Meckel Diverticulitis with a Stone Meckel Diverticulitis: Etiology •

• •

Luminal obstruction ➢ Enterolith ➢ Foreign body ➢ Edema of orifice Peptic ulceration Torsion

Gastrointestinal Radiology

425

Seminar 5: Meckel Diverticulum

Meckel Diverticulitis •

•

Differential diagnosis ➢ Appendicitis ➢ Inflammatory bowel disease ➢ Idiopathic ileal diverticula Helpful CT features ➢ Blind-ending pouch ➢ Mural contrast enhancement ➢ Connection to ileum ➢ Midline location ➢ Associated SBO

Case 5: 40-year-old man with pain and vomiting

Small bowel obstruction from Meckel diverticulitis

Inflamed Meckel with Small Bowel Obstruction

Small Bowel Obstruction due to Meckel Diverticula • •

Second most common complication of Meckel Etiology ➢ Inversion with intussusception ➢ Diverticulitis ➢ Volvulus from attachment to umbilicus ➢ Congenital mesodiverticular bands ➢ Foreign body impaction ➢ Inclusion of Meckel in a hernia (hernia of Littre) ➢ Neoplasm ➢ Inclusion of Meckel in a true knot

Summary: Complications of Meckel Diverticula

• • •

Hemorrhage Obstruction Diverticulitis Inversion ➢ Intussusception Stones Torsion Neoplasm

•

High index of suspicion

• • • •

Summary: Complications of Meckel Diverticula

Seminar 5: Meckel Diverticulum

426

Gastrointestinal Radiology

Gastrointestinal Seminar 6: Beyond Appendicitis

Angela D. Levy, LTC (P), MC, USA

Case 1: 23-year-old man with a 1-day history of left lower quadrant pain and bilious emesis Imaging Features • • • •

Reversal of SMA and SMV Swirling vessels about SMA Absent colon right side of abdomen Inflammatory process in LLQ ➢ Inflamed tubular structure ➢ Mesenteric inflammation

Differential Diagnosis • •

Malrotation Inflammation ➢ Diverticulitis ➢ Meckel diverticulum ➢ Inflammatory bowel disease ➢ Appendicitis

Malrotation with Left-sided Appendicitis

Malrotation with left-sided appendicitis

Various locations of the cecum and appendix within the abdomen Gastrointestinal Radiology

427

Seminar 6: Beyond Appendicitis

Case 2: 47-year-old man complains of fever and right lower quadrant pain

Appendiceal mucinous cystadenoma causing appendicitis

Appendiceal Mucinous Cystadenoma Causing Appendicitis Appendiceal Neoplasms •

•

Uncommon ➢ <0.4% of intestinal tumors Histologic subtypes ➢ Carcinoid ➢ Mucinous cystadenoma/cystadenocarcinoma ➢ Adencarcinoma ➢ Non-mucin producing adenocarcinoma

Appendiceal Carcinoid •

• •

Most common location for GI tract carcinoid ➢ 45% of gastrointestinal carcinoids Most common appendiceal tumor ➢ 50% to 85% of appendiceal tumors Majority benign clinical course ➢ 70% to 90% discovered incidentally ➢ >95% of appendiceal carcinoids have benign biologic behavior

Mucinous Cystadenoma/Cystadenocarcinoma • •

• •

Mucin producing epithelial neoplasm ➢ M=F ➢ 27 to 77 years of age Presentation ➢ Right lower quadrant pain, nausea, vomiting, abdominal swelling Complications ➢ Bowel obstruction, torsion, perforation, intussusception, appendicitis 20% with a synchronous colonic adenocarcinoma

Mucinous Cystadenoma/Cystadenocarcinoma •

•

Radiologic Findings ➢ RLQ mass on plain film ➢ Rim-like calcification ➢ Mass effect medial cecal wall ➢ Nonfilled appendix on BE Cross-sectional imaging ➢ Fluid-filled, complex mass on CT or US ➢ Mass bulges into cecal lumen ➢ Short T1 and long T2 signal on MR ➢ May be the lead point for intussusception

Mucinous Cystadenoma

Seminar 6: Beyond Appendicitis

Mucinous cystadenoma of the appendix 428

Gastrointestinal Radiology

Appendiceal Adenocarcinoma • • •

Non mucin producing Less common than mucinous tumors Radiologically resembles colonic adenocarcinoma ➢ Focal soft tissue mass ➢ Soft tissue mural infiltration

Neoplastic vs. Nonneoplastic Appendicitis •

Appendiceal Adenocarcinoma

CT findings suggestive of neoplasm ➢ Focal soft tissue mass ➢ Cystic dilatation of the appendix ➢ Nonspecific inflammatory changes may be seen in neoplasms of the appendix ➢ 95% sensitivity for neoplasm if you combine morphologic changes with a diameter > 15 mm1

1Pickhardt PJ, Levy AD, Rohrmann CA, Kende AI. Primary Neoplasms of the Appendix Manifesting as Acute Appendicitis: CT Findings with Pathologic Correlation. Radiology 2002. 224 (3): 775-781

Gastrointestinal Radiology

429

Seminar 6: Beyond Appendicitis

Case 3: 65-year-old man with acute RLQ pain

Epiploic appendagitis

Epiploic Appendagitis •

•

Appendix epiploica ➢ Torsion ➢ Infarction ➢ Ischemia Clinical course ➢ Self limited ➢ Spontaneous resolution

Epiploic Appendagitis: Imaging Features •

Pericolonic fatty mass ➢ Peripheral inflammatory change ➢ Central high attenuation from vascular thrombosis ➢ May have mass effect on adjacent bowel

Epiploic Appendagitis

Case 4: 17-year-old man with RLQ pain and poor appetite

Mesenteric adenitis

Mesenteric Adenitis •

• • •

Inflammation of ileocecal nodes ➢ Coexistent inflammation of the TI and cecum may be present Children, young adults Self limited Diagnosis of exclusion

Seminar 6: Beyond Appendicitis

430

Gastrointestinal Radiology

Case 5: 64-year-old man with RLQ pain that progressed to involve the entire abdomen, fever, and vomiting

Cecal adenocarcinoma resulting in perforation, appendicitis, and small bowel obstruction

Cecal Adenocarcinoma

Acute Appendicitis: Pathogenesis •

Luminal obstruction followed by infection ➢ Stones ➢ Food, mucus, adhesions ➢ Mucosal edema, lymphoid hyperplasia ➢ Parasites ➢ Tumors ➢ Endometriosis

Cecal Adenocarcinoma •

• • •

Mural thickening ➢ Eccentric or asymmetric Intraluminal mass ➢ Often near appendiceal orifice if patient presents with appendicitis Pericolic lymph nodes Peritoneal implants, distant mets

Non Hodgkin Lymphoma

Case 6: 42-year-old woman with RLQ pain and peritoneal signs on physical exam

Omental Infarction •

•

Omental Infarction

Omental torsion ➢ Most commonly site free edge of the right lateral omentum CT features ➢ Focal inflammation of omental fat ➢ Normal appendix, colon, terminal ileum ➢ Fatty mass with concentric or swirling lines

Gastrointestinal Radiology

431

Seminar 6: Beyond Appendicitis

Gastrointestinal Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder Angela D. Levy, LTC (P), MC, USA

Case 1: 45-year-old woman with RUQ pain

Adenomyomatous hyperplasia

Sonographic Findings • •

Gallbladder wall thickening Reverberation artifact

•

Infection/inflammation ➢ Acute cholecystitis ➢ Chronic cholecystitis ➢ Xanthogranulomatous cholecystitis Edema ➢ Cardiac, liver, renal failure ➢ Hepatitis Neoplasm ➢ Primary or secondary Tumor-like lesions ➢ Adenomyomatous hyperplasia

Differential Diagnosis: Gallbladder Wall Thickening

• •

•

Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder

432

Gastrointestinal Radiology

Differential Diagnosis: Reverberation (“comet-tail”) Artifact • •

Gas in gallbladder wall Adenomyomatous hyperplasia

• •

Gallbladder wall thickening “String of pearls”

• •

Gallbladder wall thickening “String of pearls”

•

Common ➢ 9% cholecystectomy specimens ➢ More common in women than men Gallstones frequently present Three variants ➢ Diffuse ➢ Segmental ➢ Localized (fundic adenomyoma)

Coronal T2 MR Findings

Adenomyomatous Hyperplasia Adenomyomatous Hyperplasia • •

Fundal Adenomyomatous Hyperplasia: "Adenomyoma"

Gastrointestinal Radiology

433

Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder

Case 2: 47-year-old man complains of fever and right lower quadrant pain

CT Findings

Xanthogranulomatous cholecystitis

• • • • •

Gallstone Gallbladder wall thickening Hypodense nodules in gallbladder wall Ill-defined hepatic margin Inflammatory change

•

Cholecystitis ➢ Acute ➢ Chronic ➢ Xanthogranulomatous Neoplasm

Differential Diagnosis

•

Xanthogranulomatous Cholecystitis • •

• •

Aggressive inflammatory process Pathophysiology ➢ Intermittent cystic duct obstruction ➢ Bile enters gallbladder wall Clinical presentation ➢ RUQ pain, fever, tenderness Surgical treatment ➢ Reported association with gallbladder carcinoma ➢ Difficult to preoperative distinguish from carcinoma ➢ Involvement of adjacent organs

Xanthogranulomatous Cholecystitis: Pathology Xanthogranulomatous Cholecystitis: Imaging • •

• • • •

Wall thickening Mural nodules or bands ➢ Hypoechoic on sonography ➢ Low-attenuation on CT Stones Pericholecystic fluid Adjacent invasion Lymphadenopathy

Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder

434

Gastrointestinal Radiology

Case 3: 75-year-old woman with RUQ pain and weight loss MR Findings • •

Intraluminal gallbladder mass Hepatoduodenal ligament mass

• • •

Gallbladder carcinoma Bile duct carcinoma Metastatic disease

•

Sixth most common GI tract malignancy ➢ Worldwide: stomach, colorectal, liver, esophagus, pancreas, gallbladder ➢ US: colorectal, pancreas, stomach, liver, esophagus, gallbladder More common in women (3:1) ➢ Mean age 72 years

Differential Diagnosis

Gallbladder Carcinoma

•

Gallbladder Carcinoma: Pathology •

•

Epithelial malignancies (98%) ➢ Adenocarcinoma (90%), squamous cell, adenosquamous, small cell carcinoma Other (2%) ➢ Sarcomas, lymphomas, carcinoid, metastases

Gallbladder Carcinoma •

Gallbladder adenocarcinoma

Imaging patterns ➢ Intraluminal polypoid mass (15% to 25%) ➢ Focal or diffuse wall thickening (20% to 30%) ➢ Mass replacing the gallbladder (40% to 65%)

Adenocarcinoma: Diffuse Wall Thickening

Papillary Adenocarcinoma : Polypoid Mass

Adenocarcinoma: Mass Replacing the Gallbladder Fossa Gallbladder Adenocarcinoma: Direct Extension to Liver Gallbladder Adenocarcinoma: Direct Extension to Hepatoduodenal Ligament

Gastrointestinal Radiology

435

Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder

Case 4: 30-year-old man with RUQ pain

Metastatic melanoma

Differential Diagnosis: Polypoid Gallbladder Mass • • • • •

Cholesterol polyp Gallbladder adenoma Adenomyomatous hyperplasia Carcinoma Metastatic disease

Metastatic Melanoma

Management of Gallbladder Polyps •

• •

•

Size < 5 mm ➢ Do nothing Size 5 to 10 mm ➢ Follow Size >10 mm ➢ Remove Features suggesting malignancy ➢ Adjacent gallbladder wall thickening ➢ Abnormal gallbladder/liver interface ➢ Abnormal liver parenchyma ➢ Hepatoduodenal ligament adenopathy

Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder

436

Gastrointestinal Radiology

Case 5: 35-year-old woman with RUQ pain

Sonographic Findings

Cholesterol polyp

• •

Small, nonshadowing echogenic mass Adherent to gallbladder wall

•

Common ➢ 50% of polypoid lesions in the gallbladder More common in women ➢ 3:1

Cholesterol Polyp •

Cholesterol Polyp • •

Lipid laden macrophages Normal gallbladder epithelium

• •

Most <10 mm Small lesions ➢ Echogenic nodules Larger lesions ➢ Hypoechoic ➢ Internal echogenic foci

Cholesterol Polyp: Sonography •

Summary •

•

Look for features to suggest a specific process tumor-like process ➢ Ring-down artifact, pearl necklace sign for adenomyomatous hyperplasia ➢ Focal mural nodules for XGC in the right clinical setting Look for features to suggest a malignancy ➢ Gallbladder wall thickening in association with a polypoid mass ➢ Abnormal gallbladder/liver interface ➢ Abnormal liver parenchyma ➢ Hepatoduodenal ligament mass or adenopathy

Gastrointestinal Radiology

437

Seminar 7: Tumors and Tumor-like Lesions of the Gallbladder

Cholelithiasis and Cholecystitis Robert K. Zeman, MD Outline/Objectives • • • •

Detection of cholelithiasis Gravel versus sludge Acute cholecystitis Complications of acute cholecystitis ➢ Gangrenous cholecystitis ➢ Emphysematous cholecystitis ➢ Empyema of the gallbladder ➢ Gallbladder perforation ➢ Choledocholithiasis

Premise • •

The radiologist plays a central role in identifying the cause of the patient’s symptoms and… Detecting complications of cholecystitis (inflammatory and neoplastic) that will dictate the therapeutic approach

Cholelithiasis • • • •

30 million American adults harbor stones Should “silent” stones be treated? 22% of patients with stones are symptomatic (Sirmione study) In symptomatic patients, 50% chance of colic in 1 year; 1–2% cumulative risk of acute cholecystitis.

Cholelithiasis • •

For symptomatic stones, recommend elective laparoscopic cholecystectomy For acute cholecystitis: ➢ Delayed surgery allows for better vizualization of surgical field ➢ Early surgery means less adhesions

US of Cholelithiasis [Figure 2-22-1] •

3 common appearances ➢ Solitary stone ➢ Gravel ➢ Double-arc (WES)

Figure 2-22-1

Solitary Gallstone Gravel

Double-Arc Sign (WES) How Sensitive is US? •

Remember the neck of the gallbladder

• •

Gravel represents small, discrete calculi Sludge is viscous, lithogenic bile

Sludge vs Gravel?

Cholelithiasis and Cholecystitis

Solitary stone

438

Gravel

Double-arc (WES)

Gastrointestinal Radiology

Tumefactive Sludge [Figure 2-22-2] • •

Figure 2-22-2

Baseline After walking

Is there any role for the OCG? [Figure 2-22-3]

• •

No stones on OCG See 5mm stone on US

• • •

Uncomplicated vs complicated Treatment options if complications (do imaging findings influence operative approach?) Cholescintigraphy vs US

Acute Cholecystitis

•

Cholescintigraphy vs. Ultrasound • • • • •

Tumefactive sludge mimics GB mass-but changes shape with change in patient position

Both equally sensitive and specific Emergency availability is key Ultrasound screens for more non-biliary diseases If biliary obstruction present, scintigraphy does not identify cause despite high sensitivity; US may see cause Scintigraphy great problem solver; can add EF when confusing symptoms

Figure 2-22-3

Cholescintigraphy In AC • •

The only reliable indicator of acute cholecystitis is non-visualization of the gallbladder – remember the lateral High sensitivity, moderate specificity

Cholescintigraphy: Positive study for acute cholecystitis

Potential Causes of False-Positive Scintigraphy For Acute Cholecystitis • • • • • • • • • • • •

Lack of adequate fasting Chronic cholecystitis Failure to obtain delayed views Pancreatitis Hyperalimentation Biliary obstruction Prolonged fasting Intercurrent illness Alcoholism Overly conservative pathologic criteria of Acute Cholecystitis Trauma Gallbladder Neoplasm

Missed stones often “hide” in the gallbladder neck

Is There Anything That Can Reduce False-Positives? Pharmacologic Enhancement of Cholescintigraphy

• • • •

Can dramatically reduce false-positives* Two approaches – CCK versus morphine (in setting of suspected AC) CCK to “pre-empty” GB Morphine (.04 mg/kg) given if GB fails to visualize by 30–50 minutes

*Kim et al, AJR 147:1177,1986

Gastrointestinal Radiology

439

Cholelithiasis and Cholecystitis

Use of Morphine Reduces False-Positives [Figure 2-22-4] • •

35 minutes (pre-MS) 55 minutes (post-MS)

•

Most useful signs ➢ Cholelithiasis ➢ Intramural sonolucency ➢ Sonographic Murphy Sign* ➢ GB wall hyperemia** Secondary signs ➢ Pericholecystic fluid ➢ GB distention ➢ Sludge ➢ Gas in the gallbladder wall or lumen ➢ Pericholecystic abscess

Figure 2-22-4

Sonographic Findings Associated With Acute Cholecystitis

•

35 minutes (pre-MS)

Normal scan after morphine 55 minutes (post-MS)

*Laing et al, Radiology 140:449, 1981 **Uggowitzer AJR 168:707, 1997

Intramural Sonolucency • • •

Described in 11 patients as first specific sign for acute cholecystitis* “Consists of a hypo-reflective or sonolucent band, continuous or interrupted, within the hyper-reflective gallbladder wall” Focal lucency or concentric rings (striate) most suggestive of inflammation**

Figure 2-22-5

*Marchal et al, Radiology 133:429, 1979 **Cohen et al, Radiology 164:31, 1987

Acute Cholecystitis-Striate GB Walls [Figure 2-22-5]

Acute Cholecystitis-Focal Lucency Lucency in GB Wall is not always Edema •

Gallbladder varices in portal hypertension

Which one has Varices?

Gallbladder Wall Thickening • • • • • • • • • • • •

Failure to fast Acute cholecystitis Chronic cholecystitis Hypoalbuminemia Hepatitis Ascites Varices AIDS Carcinoma Cholesterolosis Mononucleosis

Figure 2-22-6

Beware of “sliver” of edema in some of these entities

Gallbladder Wall Thickening [Figure 2-22-6]

• •

Multiple patients

Hepatitis Ascites

Cholelithiasis and Cholecystitis

Gallbladder wall thickening has many causes and is not as specific as a striate wall 440

Gastrointestinal Radiology

Hyperemia of Acute Cholecystitis

Complications/Severity of Acute Cholecystitis Options for Treatment of Acute Cholecystitis • • • •

PCCL OC LC Temporize

•

Identify findings that make temporizing ill-advised or that would potentially result in open cholecystectomy: ➢ Extreme striations ➢ Gangrenous cholecystitis ➢ Emphysematous cholecystitis ➢ Perforation ➢ Biliary obstruction / Mirizzi syndrome ➢ Incidental findings that preclude LC

Role of the Radiologist in Acute Cholecystitis

Incidental Finding •

Hemangioma next to GB neck

• • •

Not always Clostridal infection Implies severe inflammation Sonography-may see desquamated mucosa/membranes Scintigraphy-increased pericholecystic activity* due to: ➢ delayed excretion from perihepatitis ➢ hyperemia with increased tracer delivery

Figure 2-22-7

Gangrenous Cholecystitis

•

*Smith et al, Radiology 156:197, 1985

Two different patients show a band of tracer where the liver abuts the inflamed gallbladder (arrow)

Gangrenous Cholecystitis-Rim Sign [Figure 2-22-7] •

Two different patients

•

Sloughed membranes

• • • • • •

Elderly patients, 20–30% are diabetic Male predominance 1/3 infected with Clostridia welchii Perforation 5 times as common as for non-emphysematous cholecystitis “Dirty” shadowing and echogenic GB wall on sonography is suggestive Don’t forget plain film – differential diagnosis of RUQ air

• •

US-echogenic foci=gas KUB

Gangrenous Cholecystitis Emphysematous Cholecystitis

Emphysematous Cholecystitis

Figure 2-22-8

Emphysematous Cholecystitis [Figure 2-22-8]

• •

US-ring-down KUB

US-ring-down (reverb) Gastrointestinal Radiology

441

KUB Cholelithiasis and Cholecystitis

Not all GB Walls with Ring-Down Contain Gas •

Diagnosis?

•

Adenomyomatosis

• •

Gas confined to GB wall Gas in lumen, hepatic ducts

•

Infection in obstructed, inflamed gallbladder 25% incidence-rises to 80% if untreated after 7 days* Results in marked GB distention in 38% of patients** US, CT-Nonspecific. See distention, bile/debris level, “snow storm”

Not all GB Walls with Ring-Down Contain Gas Emphysematous Cholecystitis-CT Gallbladder Empyema • • •

Figure 2-22-9

[Figure 2-22-9]

*Goldman et al, Gastro 11:318, 1948 **Fry et al, Am J Surg 141:366, 1981

Perforation of the Gallbladder • • • • •

Seen both in the context of chronic cholecystitis (eg., gallstone ileus) and AC In older literature, occurs in 3–15% of patients with acute cholecystitis Patient feels transiently better and then develops peritoneal signs Cholescintigraphy – extravasated activity – maybe Sonography, CT-pericholecystic collection, non-specific

GB Perforation in AC [Figure 2-22-10] GB Perforation

Choledocholithiasis • •

• •

May occur as primary duct stone (usually pigment), secondary to gallstones, or following cholecystectomy Most small stones will pass spontaneously. The duct caliber and dynamics may rapidly change CT and US are approx. 70-80% sensitive for detection of choledocholithiasis If you suspect CBD stones…options are MRCP, ERCP, intraop cholangiogram

When to Perform MRCP

•

Jaundice Dilated ducts on US Delayed egress on IDA Anatomic finding that suggests process that may result in altered duct anatomy or make laparoscopic cholecystectomy risky Post-cholecystectomy complications

• •

T2 wt TSE or FSE, thin sections or “slab” Extra sections as needed

• • • •

Figure 2-22-10

Contracted GB

MRCP

Cholelithiasis and Cholecystitis

442

Gastrointestinal Radiology

RUQ Pain (789.01) and Fever: Approach • • • • •

“R/O Acute calculous cholecystitis”...IDA, US “R/O Stones”...US “R/O Acute acalculous cholecystitis”...? “R/O a reason to operate”... if suspect biliary do US, if suspect acute abdomen/GI disease do CT Remember MRCP

1. 2.

Zeman RK, Garra BS. Gallbladder Imaging: The State-of-the-art. Gastroent Clin N. Am 2:127, 1991. Garra BS, Davros WJ, Lack EE, Horii SC, Zeman RK: Visibility of gallstone fragments at ultrasound and fluoroscopy. Implications for monitoring of gallstone lithotripsy. Radiology 174:343, 1990. Mathieson JR, So CB, Malone DE, Becker CD, Burhenne HJ: Accuracy of sonography for determining the number and size of gallbladder stones before and after lithotripsy. AJR 153:977, 1989. duPlessis DJ, Jersky J. Management of acute cholecystitis. Surg Clin North Am 53:1071, 1973. Halasz NA. Counterfeit cholecystitis: A common diagnostic dilemma. Am J Surg 130:189, 1975. Zeman RK, Burrell MI, Cahow CE, Caride V. Diagnostic utility of cholescintigraphy and ultrasonography in acute cholecystitis. Am J Surg 141:446, 1981. Weissmann HS, Badia J, Sugarman LA et al. Spectrum of 99m Tc-IDA cholescintigraphic patterns in acute cholecystitis. Radiology 138:167, 1981. Eikman EA, Cameron JL, Colman M et al. A test for patency of the cystic duct in acute cholecystitis. Ann Int Med 82:318, 1975. Fonseca C, Greenberg D, Rosenthall L et al. Assessment of the utility of gallbladder imaging with 99m Tc-IDA. Clin Nucl Med 3:437, 1978. Freitas JE. Cholescintigraphy in acute and chronic cholecystitis. Semin Nucl Med 12:18, 1982. Shuman WP, Gibbs P, Rudd TG et al. PIDIDA scintigraphy for cholecystitis: False positives in alcoholism and total parenteral nutrition. AJR 138:1, 1982. Kalff V, Froelich JW, Lloyd R et al. Predictive value of an abnormal hepatobiliary scan in patients with severe intercurrent illness. Radiology 146:191, 1983. Laing FE, Federle MP, Jeffrey RB et al. Ultrasonic evaluation of patients with acute right upper quadrant pain. Radiology 140:449, 1981. Ralls PW, Colletti PM, Lapin SA et al. Real-time sonography in suspected acute cholecystitis: Prospective evaluation of primary and secondary signs. Radiology 155:767, 1985. Cohan RH, Mahony BS, Bowie JD, Cooper C, Baker ME, Illescas FF: Striated intramural gallbladder lucencies on US studies. Predictors of acute cholecystitis. Radiology 164:31–35, 1987. Teefey SA, Baron RL, Bigler SA: Sonography of the gallbladder. Significance of striated (layered) thickening of the gallbladder wall. AJR 156:945, 1991. Shaler WJ, Leopold GR, Scheible FW: Sonography of the thickened gallbladder wall. A nonspecific finding. AJR 136:337, 1981. West MS, Garra BS, Horii SC, Zeman RK et al. Gallbladder varices: Imaging findings in patients with portal hypertension. Radiology 179:179, 1991. Weissmann HS, Berkowitz D, Fox MS et al. The role of technetium-99m iminodiacetic acid (IDA) cholescintigraphy in acute acalculous cholecystitis. Radiology 146:177, 1983. Shuman WP, Rogers JV, Rudd TG et al. Low sensitivity of sonography and cholescintigraphy in acalculous cholecystitis. Radiology 142:531, 1984. Swayne LC. Acute calculous cholecystitis: Sensitivity in detection using technetium-99m iminodiacetic acid cholescintigraphy. Radiology 160:33, 1986. Mirvis SE, Vainright JR, Nelson AW, et al. The diagnosis of acute acalculous cholecystitis: A comparison of sonography, scintigraphy, and CT. AJR 147:171, 1986. Jeffrey RB, Laing FC, Wong W, Callen PW. Gangrenous cholecystitis: Diagnosis by ultrasound. Radiology 156:797, 1985. Wales LR. Desquamated gallbladder mucosa: Unusual sign of cholecystitis. AJR 139:810, 1982. Smith R, Rosen J, Gallo LN, Alderson PO. Pericholecystic hepatic activity in cholescintigraphy. Radiology 156:797, 1985. Siskind B, Hawkins M, Cinti D, Zeman RK, Burrell MI. Perforation of the gallbladder: Radiologic-pathologic correlation. J Clin Gastroenterol 9:670–78, 1987. Clemett AR, Lowman RM. The roentgen features of the Mirizzi syndrome. AJR 94:480, 1965. Weltman D, Zeman RK. Imaging of acute diseases of the gallbladder and bile ducts. Radiological Clinics of North America 32:933-950, 1994. Fulcher AS, Turner MA, Capps GW. Technical Advances and Clinical Applications. RAdiographics 19:25-41, 1999.

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17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29.

Gastrointestinal Radiology

443

Cholelithiasis and Cholecystitis

Inflammatory Diseases of the Esophagus Marc S. Levine, MD

Figure 2-23-1

Technique •

•

Double-contrast: ➢ Upright ➢ Right lateral cardia Single-contrast: ➢ Separate swallows ➢ Prone esophagus

Reflux Esophagitis • •

Most common inflam condition Purpose of Ba study not simply to show HH/GER but to R/O morphologic sequelae of GERD

Pathogenesis •

•

Frequency of GER ➢ Decreased LES tone ➢ Mult trans LES relaxations Duration of GER ➢ Abnormal motility ➢ (scleroderma)

Pathogenesis •

•

Acidity of refluxate ➢ ZES (increased acid) ➢ Billroth II (bile or panc) Resistance of mucosa ➢ Age ➢ Debilitation

Clinical Findings • • • •

Heartburn and regurg Epigastric or RUQ pain Upper GI bleeding Dysphagia (peptic stx)

• •

Occur independently Spont GER at fluoro: ➢ 30–60% in esophagitis ➢ 40–50% in volunteers

Reflux esophagitis with granular mucosa

Figure 2-23-2

Hiatal Hernia and GER

Reflux Esophagitis: Radiographic Findings [Figures 2-23-1 and 2-23-2]

• • • • •

Abnormal motility Granularity Thickened folds Inflammatory EG polyp Ulceration

• • • • •

Radiating folds Deformity of wall Peptic stricture Sacculations Transverse folds

Peptic Scarring: Radiographic Findings

Inflammatory Disease of the Esophagus

Reflux esophagitis with ulceration 444

Gastrointestinal Radiology

Radiologic Dx of Esophagitis Gr 1 13% 22% 53%

Gr 2 90% 83% 93%

• • •

Koehler Ott Creteur

• •

Variant of peptic stx Episodic dysphagia (meat) ➢ (Steakhouse syndrome) Symm ringlike constriction Vertical height 2–4 mm Usually sx if < 13 mm diam Best seen on prone views

Schatzki Ring • • • •

Gr 3 100% 95% 100%

Barrett’s Esophagus • •

Prog columnar metaplasia from GER and esophagitis Prevalence: ➢ 10% with esophagitis ➢ 40% with peptic stx

Clinical Findings • • • •

Men > Women, W > B Reflux sx, dysphagia 40% asymptomatic Tx of GER may not cause Barrett’s to regress

• •

Projections or islands of columnar epith separated by squam epith Foveolar epith > 3 cm above LES or intestinal metaplasia with goblet cells

• • •

Risk of adenocarcinoma Dysplasia-Ca sequence Endoscopic surveillance

• •

Classic: High stx or ulcer or reticular pattern Common: GER, hiatal hernia, reflux esophagitis, or peptic stricture

• •

200 pts with reflux sx Classified at high, mod, or low risk for Barrett’s

Histologic Findings

Premalignant Condition

Figure 2-23-3

Radiographic Findings [Figure 2-23-3] Dx of Barrett’s by D/C Tech AJR 150:97–102, 1988

Classification of Risk • • •

High: High stx or ulcer or reticular pattern Mod: Distal stx or reflux esophagitis Low: None of above

• • •

Risk High Moderate Low

• • •

Less sensitive than endoscopy Most false negative exams in mild disease Vast majority do not have Barrett’s esophagus

Radiologic vs Endoscopic Dx 10 73 117

Endo 9 (90%) 12 (16%) 1 (1%)

Barrett esophagus with mid esophageal stricture and reticular pattern

Radiologic Diagnosis

Gastrointestinal Radiology

445

Inflammatory Disease of the Esophagus

Reflux Symptoms

Figure 2-23-4

Candida Esophagitis • • •

Most common type Immunocompromised (75%) Local esophageal stasis (25%) ➢ (achalasia, scleroderma)

Clinical Findings • • •

Dysphagia/odynophagia OP Candidiasis (50%) Marked clinical response to antifungal agents (ketoconazole)

•

Mucosal Plaques (90%) ➢ Linear ➢ Etched in white “Shaggy” esophagus (AIDS) ➢ Plaques and membranes ➢ Superimposed ulcers

Radiographic Findings [Figures 2-23-4 and 2-23-5] •

Candida esophagitis with plaques

Figure 2-23-5

Herpes Esophagitis

• • • •

2nd most common type Herpes simplex virus type 1 Immunocompromised Viral Cx or Bx (intranuclear inclusions in cells adjacent to ulcers)

• • •

Dysphagia/odynophagia Oropharyngeal herpes Marked clinical response to antiviral agents (acyclovir)

• •

Discrete ulcers in upper and midesophagus Ulcers and plaques (mimics Candida)

• • • • • •

Young men (15–30 y/o) Sexual partners with OP herpes Flu-like prodrome (3–10 days) Severe odynophagia Multiple tiny ulcers Sx resolve in 3–14 days

Clinical Findings

Radiographic Findings [Figure 2-23-6]

Herpes Esophagitis in Healthy Pts

Inflammatory Disease of the Esophagus

Candida esophagitis with shaggy esophagus 446

Gastrointestinal Radiology

CMV Esophagitis • • • •

Pts with AIDS Odynophagia Viral Cx or Bx (intranuclear inclusions in cells at ulcer base) Tx with ganciclovir (endo for confirmation)

• • •

Nodular mucosa Small ulcers (mimics herpes) Giant ulcers

Figure 2-23-6

Radiographic Findings [Figures 2-23-7] Figure 2-23-7

Figure 2-23-8

Herpes esophagitis with tiny ulcers

HIV esophagitis with giant ulcer

CMV esophagitis with giant ulcer

HIV Esophagitis • • • • • •

Odynophagia and giant ulcers Palatal ulcers Maculopapular rash Recent seroconversion Dx of exclusion (No CMV) Treatment with steroids

•

•

Giant ulcers ➢ (mimics CMV) Satellite ulcers

• • •

Cause HIV CMV Both

Radiographic Findings [Figure 2-23-8] Giant Ulcers in 21 HIV + Pts No Pts 16 3 2

% 76 14 10

Radiology 194:447–451, 1995

Giant Ulcers in 21 HIV + Pts • • •

All had AIDS (CD4 ct < 200) Avg time from serodetect 2 yrs Only 1 pt had palatal ulcers or rash

Gastrointestinal Radiology

447

Inflammatory Disease of the Esophagus

Conclusions • • •

Most giant ulcers in HIV+ Pts caused by HIV not CMV Impossible to diff by clin or rad criteria Endoscopy for definitive Dx

•

Contact esophagitis (doxycycline, tetracycline, KCl, quinidine, NSAIDs, alendronate) Aortic arch or lt main bronchus Superficial ulcers Severe odynophagia but rapid clinical improvement after withdrawal of offending agent

Figure 2-23-9

Drug-Induced Esophagitis [Figure 2-23-9] • • •

Radiation Esophagitis • • •

2–5000 rad: self-limited esophagitis (1 – 2 weeks) 5000 or more rad: stx, progressive dysphagia (4–8 months) Adriamycin potentiates XRT (only 500 rad)

•

Acute ➢ Ulceration ➢ Granular mucosa ➢ Decreased distensibility Chronic ➢ Abnormal motility ➢ Strictures

Radiographic Findings

•

Caustic Esophagitis • • •

Strong acids or alkali (liquid lye) Three phases of injury: ➢ Acute necrosis ➢ Ulceration and granulation ➢ Cicatrization Chest pain, odynophagia, hematemesis, shock

Tetracycline-induced esophagitis with three ulcers

Figure 2-23-10

Radiographic Findings •

•

Figure 2-23-11

Acute ➢ Abnormal motility ➢ Ulceration ➢ Perforation Chronic ➢ Strictures ➢ (1–3 months)

Esophageal Intramural Pseudodiverticulosis • • • • •

Dilated excretory ducts Ductal obstruction Candida, diabetes, alcohol High strictures classic Peptic stx more common

Radiographic Findings [Figures 2-23-10 and 2-23-11]

• • •

Flask-shaped outpouchings “Floating” outside wall Associated strictures ➢ (especially peptic stx)

Inflammatory Disease of the Esophagus

Diffuse esophageal intramural pseudodiverticulosis with high stricture 448

Localized esophageal intramural pseudodiverticulosis with peptic stricture Gastrointestinal Radiology

Esophageal Varices

Location Distal Mid

Cause Portal HTN SVC obst

• •

Uphill Downhill

• • •

Cause Reflux Candida Glycogenic acanthosis

Finding Granularity Plaques Nodules/plaques

• • •

Cause Candida Sup spr Ca Barrett’s

Finding Plaques Coalesce nodules Reticular pattern

• • • • •

Cause Reflux Herpes Drugs CMV HIV

Finding Distal Small, mid Small, mid Giant Giant

• • •

Esophagitis Varices Varicoid Ca

• • • •

Barrett’s esophagus Mediastinal irradiation Caustic ingestion Primary or metastatic tumor

• • •

Peptic stricture Lower esoph ring Barrett’s Ca

Diffuse Nodules/Plaques

Localized Nodules/Plaques

Ulcers

Thickened Folds High Strictures

Distal Strictures

Gastrointestinal Radiology

449

Inflammatory Disease of the Esophagus

Tumors of the Esophagus Marc S. Levine, MD Mucosal Lesions • • •

Squamous papilloma Adenoma Glycogenic acanthosis

• • •

Coral-like excrescence Fibrovascular core Hyperplastic squamous epithelium

• • •

Usually asymptomatic Malignant degeneration rare Multiple papillomas (papillomatosis)

• • • •

Small, sessile polyp Lobulated mass Bubbly appearance Diff Dx – early Ca

• • • •

Accum of cytoplasmic glycogen White nodules/plaques Rarely causes esophageal sx No risk of malignant degeneration

• • • •

Round nodules/plaques 1–5 mm in diameter Predominantly midesophagus DDx – Candidiasis

• • • • •

Fibrovascular polyp Leiomyoma Granular cell tumor Duplication cyst Idiopathic varix

• • • • •

Most common benign tumor Bands of smooth muscle 60% DT, 30% MT, 10% PT Up to 20 cm in diameter Patterns – submucosal, exophytic, intraluminal, circumferential

• • • •

Most pts asx Dysphagia GI bleed rare Enucleation

•

CXR – soft tissue mass, Ca++ rare

Squamous Papilloma: Pathologic Findings Clinical Findings

Figure 2-24-1

Radiographic Findings

Glycogenic Acanthosis

Radiographic Findings [Figure 2-24-1]

Intramural Lesions

Leiomyoma: Pathologic Findings

Glycogen acanthosis with nodules

Clinical Findings

Radiographic Findings [Figure 2-24-2] Tumors of the Esophagus

450

Gastrointestinal Radiology

• • •

Ba – submucosal mass CT – soft tissue mass DDx – fibroma, hemangioma, granular cell tumor, duplication cyst

• • • • •

Annular lesion Giant intraluminal mass Gastric involvement Multiple lesions Leiomyomatosis

• • • • •

Proliferation of smooth m. Children/adolescents Long-standing dysphagia Familial – autosomal dominant Alport’s syndrome (nephritis, deafness, ocular lesions)

• • • •

Ba – tapered narrowing of distal esophagus (1° achalasia?) Length > achalasia Symmetric fundal defects CT – thickened wall (2° achalasia?)

• • • • •

Benign intraluminal tumor Fibrous/adipose/vascular tissue with nl squam epith Hamartoma/fibroma/lipoma/fibrolipoma/angiolipoma All classified as FVPs Malig degen rare

• • • • •

Arises in cervical esophagus Loose submucosal conn tiss Dragged inf by peristalsis Occas prolapses into fundus Pedicle in cervical esophagus

• • • •

Dysphagia Resp sx – inspiratory stridor, choking, wheezing Regurgitation of fleshy mass Asphyxia/sudden death

•

CXR ➢ Rt sup med mass ➢ Retrotracheal bowing Ba ➢ Smooth, expansile intraluminal mass ➢ Var size & location ➢ Lobulation common ➢ Prox pedicle rare DDx Air bubble, achalasia, malignant tumor

Figure 2-24-2

Unusual Findings

Leiomyomatosis

Esophageal leiomyoma in profile

Radiographic Findings

Figure 2-24-3

Fibrovascular Polyp: Pathologic Findings

Pathologic Findings

CXR with right superior mediastinal mass caused by fibrovascular polyp

Clinical Findings

Figure 2-24-4

Radiographic Findings [Figures 2-24-3 to 2-24-5] •

•

Radiographic Findings [Figures 2-24-5 and 2-24-6]

Path

CT

Gastrointestinal Radiology

Figure 2-24-5

Fibrovascular polyp with fat attenuation on CT 451

Fibrovascular polyp on barium study (same patient as Figure 2-22-3) Tumors of the Esophagus

• • •

Adipose Mixed Fibrous

Figure 2-24-6

Lipid density [1] Heterogeneous Soft tissue density

[1] High-signal intensity on T1MR / High echo on endoscopic U/S

Duplication Cyst • • • • •

Abnl embryo development Sequest from prim foregut Ciliated columnar epith Most pts asymptomatic Occas bleeding/infection

• • • •

CXR: Mediastinal mass Ba: Submucosal mass CT: Homogen low atten MR: High-signal on T2

• • • • • • • • •

Squamous cell carcinoma Adenocarcinoma Spindle cell carcinoma Small cell carcinoma Leiomyosarcoma Kaposi’s sarcoma Malignant melanoma Lymphoma Metastases

• • • •

Epidemiological Factors Tobacco and alcohol Geographic variations (China, Iran, S Africa) Low molybdenum in soil (accum of nitrosamines)

• • • • • •

Achalasia Lye strictures Head and neck tumors Celiac disease Plummer-Vinson Tylosis

• • •

Early: mucosa or submucosa without lymph node mets Superficial: mucosa or submucosa with or without lymph node mets Small: < 3.5 cm regardless of depth of invasion or lymph node mets

• • •

Direct extension – trachea, bronchi, lungs, pericard, aorta, diaphragms Lymphatic spread – nodes in med, neck, upper abdomen (paracardiac, lesser curv, celiac) Hematogenous – lungs, adrenals, liver

• • • • •

Dysphagia and wt loss Odynophagia (if ulcerated) Chest pain (poor sign) Paroxysmal coughing (if TEF) 5-year survival < 10%

Radiographic Findings

Fibrovascular polyp with geographic areas of fat and soft tissue attenuation on CT

Malignant Tumors

Squamous Cell Carcinoma:

Predisposing Factors

Definitions

Routes of Spread

Clinical Findings

Tumors of the Esophagus

452

Gastrointestinal Radiology

Early Squamous Cell Carcinoma: Radiographic Findings [Figure 2-24-7]

• • • •

Small, sessile polyp Plaquelike (central ulcer) Focal irregularity in wall Superficial spreading

•

Plain Film ➢ Widened med ➢ Ant tracheal bowing ➢ Thick RT stripe ➢ A/F level in esoph Barium ➢ Infiltrating ➢ Polypoid ➢ Ulcerative ➢ Varicoid

Figure 2-24-7

Adv Squamous Cell Carcinoma [Figures 2-24-8 and 2-24-9]

•

Squamous Cell Carcinoma: Staging • • •

CT: Sens limited by adenopathy (mets in nl-sized nodes) MRI: Comparable to CT US: Depth of invasion & lymph node mets

• • • • • • • • • •

Arises in Barrett’s mucosa Dysplasia-Ca sequence (low-grade, high-grade, ca-in-situ, invasive) Comprises 20–50% of esoph Ca’s Predominantly in distal esophagus Often invades proximal stomach Prevalence 10% in Barrett’s esoph 30–40X greater risk than gen pop Dysphagia and weight loss Same prognosis as squamous Ca Endoscopic surveillance

Superficial spreading carcinoma with focal nodularity

Adenocarcinoma

Figure 2-28-9

Figure 2-28-8

Primary ulcerative carcinoma with giant meniscoid ulcer surrounded by rind of tumor Gastrointestinal Radiology

Varicoid carcinoma with large submucosal defects in lower esophagus 453

Tumors of the Esophagus

Radiographic Findings [Figures 2-24-10 and 2-24-11] • •

Early: sessile polyp, plaque, sup spreading, stricture Adv: infiltrating, polypoid, ulcerative, varicoid (often invades cardia)

Figure 2-24-10

Figure 2-24-11

Adenocarcinoma in Barrett esophagus invading gastric cardia Early adenocarcinoma in Barrett’s esophagus with plaque-like lesions

Figure 2-24-12

Spindle Cell Carcinoma • • • •

Carcinomatous and sarcomatous elements Spindle cell metaplasia Dysphagia and weight loss Same prognosis as squamous Ca

• • •

Ba-polypoid intraluminal mass expanding lumen without obstruction CT-expansile esophageal mass DDx-malignant melanoma

Radiographic Findings [Figure 2-24-12]

Spindle cell carcinoma seen as polypoid mass expanding esophagus without causing obstruction Tumors of the Esophagus

454

Gastrointestinal Radiology

Radiology of Peptic Ulcer Disease Marc S. Levine, MD

Hypertrophic Gastritis [Figures 2-25-1 and 2-25-2] • • • •

Glandular hyperplasia Increased acid secretion Thickened folds Diff Dx: ➢ Menetrier’s ➢ Lymphoma

Figure 2-25-1

Figure 2-25-2

Erosive gastritis with varioloform erosions in antrum

Antral Gastritis [Figure 2-25-1] • • • •

Thickened antral folds Longitudinal or Transverse Crenulation of lessercurvature Hypertrophic antral-pyloric fold

• • • • • •

PUD NSAIDs Alcohol Stress Trauma Crohn’s

• • •

Finding Erosions Max damage Healing

• • • •

Spastic bulb Thickened folds Nodules Erosions

Antral gastritis with hypertrophied antralpyloric fold on lesser curvature of distal antrum

Figure 2-25-3

Causes of Erosive Gastritis [Figure 2-25-3]

Acute Aspirin Ingestion (2–8 tabs/day in nl pts)

Duodenitis

Gastrointestinal Radiology

Time Span 8–24 hrs 1–3 days 3–7 days

NSAID-induced erosive gastritis with linear erosions clustered in gastric body near greater curvature 455

Peptic Ulcer Disease

Gastric Ulcers • • • •

Shape Size Location Morphology

Figure 2-25-4

Figure 2-25-5

Benign posterior wall gastric ulcer

Anterior wall gastric ulcer seen as ring shadow on double contrast view

Figure 2-25-6

Figure 2-25-7

Ulcer fills with barium on prone compression

Giant NSAID greater curvature ulcer

Multiple Gastric Ulcers • • •

2–30% of pts with GUs Association with aspirin in 80% Each ulcer evaluated separately

• •

More than 95% GUs Dx in North America are benign 6–16% of benign-app GUs on S/C studies are malignant Is endo always necessary?

Figure 2-25-8

Upper GI vs Endoscopy •

Benign Gastric Ulcers [Figures 2-25-4 to 2-25-8-] •

•

En Face ➢ Round or ovoid crater ➢ Smooth mound of edema ➢ Symmetric radiating folds In Profile ➢ Projection outside lumen ➢ Hampton’s line or ulcer mound or collar

Peptic Ulcer Disease

NSAID-induced greater curvature ulcer with gastrocolic fistula

456

Gastrointestinal Radiology

Malignant Gastric Ulcers [Figure 2-25-9] •

•

Figure 2-25-9

En Face ➢ Irregular crater in mass ➢ Loss of areae gastricae ➢ Nodularity, clubbing, or fusion of radiating folds In Profile ➢ Projection of crater inside lumen within mass ➢ Acute angles of mass

Equivocal Ulcers • • • • •

Irregularity of ulcer shape Asymmetry of mass effect Nodularity, irregularity, or clubbing of radiating folds Enlarged areae gastricae Location on greater curve

• • •

Rad Dx Benign Equiv Malig

Radiologic Dx of Gastric Ulcers No Pts 191 69 72

AJR 141:331–333, 1983

Endo 164 63 68

Radiologic Dx of Gastric Ulcers

• • •

Rad Dx Benign Equiv Malig

No Pts 68 37 3

AJR 164:9–13, 1987

Endo 24 33 3

Malignant lesser curvature ulcer with clubbed folds abutting ulcer on prone compression

Final Dx All ben 56 ben / 7 malig 2 ben / 66 malig Final Dx All ben All ben All malig

Gastric Ulcer Investigation

Advantages of Upper GI over Endoscopy • • •

Shorter procedure time Negligible risk Lower cost

• • • • •

D/C upper GI Endoscopy procedure Pathology Hospital Total

Cost $218 $540 $180 $102 $822

• • •

UGI + endo UGI + sel endo Diff in Cost

$1 billion $490 million $510 million

Upper GI vs Endoscopy

Cost of Evaluating 1 Million GUs in United States

Gastrointestinal Radiology

457

Peptic Ulcer Disease

Ulcer Healing

Figure 2-25-10

• • •

Change in size and shape Avg pd for healing 8 wks Ulcer scar in 90%

• • •

Central pit or depression Radiating folds Retraction of adjacent wall

• • • • •

90% < 1 cm in size 50% on anterior wall 85% with deformed bulb 5% linear 15% multiple

• • •

Greater than 2 cm in size Higher frequency of complix (bleeding, obst, perforation) Fixed configuration at fluoro

• • • • •

5% of all duodenal ulcers Medial wall of prox descending duodenum above papilla Indentation of lateral wall Notoriously difficult to Dx Can result in development of ring stricture

Ulcer Scar

Duodenal Ulcers [Figure 2-25-10] Duodenal bulbar ulcer

Giant Duodenal Ulcers

Figure 2-25-11

Postbulbar Duodenal Ulcers [Figure 2-25-11]

Investigation of Duodenal Ulcers

Post-bulbar duodenal ulcer with ulcer niche in proximal descending duodenum

H. Pylori • • •

Gram-negative bacillus Increases with age (50% of pop > age 60) Eradicated by antibiotics and antisecretory agents

• • •

Thickened gastric folds (predom antrum and body) Polypoid gastritis with thickened, lobulated folds Enlarged areae gastricae

• • •

Increased risk of gastric Ca Less than 1% develop Ca Not enough evidence to treat all pts with H.pylori

Figure 2-25-12

Radiographic Findings [Figure 2-25-12]

Association with Gastric Carcinoma

Peptic Ulcer Disease

Polypoid H. pylori gastritis with markedly thickened, lobulated folds in gastric body 458

Gastrointestinal Radiology

H. Pylori & Gastric Lymphoma • • • •

Stomach devoid of lymphoid tissue Development of lymph follicles with H. pylori (MALT) Low-grade MALT lymphoma (MALTOMA) Characteristic pathologic features

• • • •

Regress with antibiotics in 70-80% Precursor of high-grade lymphoma 50-72% of all gastric lymphomas More common than prev recognized

• •

Nodularity of mucosa (rounded 2-7 mm nodules) Diff Dx: ➢ Focal gastritis ➢ Intest metaplasia ➢ Enlarged areae gastricae

Gastric MALT Lymphoma

Figure 2-25-13

Radiographic Findings [Figure 2-25-13]

Risk of Ulcers

Prevalence Gastric ulcer 60-80% Duodenal ulcer 95-100%

Gastric MALT lymphoma with confluent nodules in gastric body

Detection of H.Pylori • • •

Endoscopic bx Urea breath test Serum Ab test

Gastrointestinal Radiology

459

Peptic Ulcer Disease

Pancreatitis: Imaging Has Made a Difference Bruce P. Brown, MD

“Is this heaven?” “No. It’s the anterior pararenal space.” Normal Pancreas

Acute Pancreatitis • • • • •

(Marseilles 1985) Sudden onset abdominal pain Increased pancreatic enzymes, blood, urine Pancreatic edema, fat and gland necrosis, hemorrhage Variable involvement of regional or remote tissues (Atlanta 1992)

• • • •

• •

(Marseilles 1985) Recurrent or persistent abdominal pain +/- increased enzymes Irreversible morphologic change in pancreas ➢ Fibrosis ➢ Acinar destruction ➢ Calcification Diffuse, Focal Loss of function

• • • • • • • • • • • • • •

Biliary stones (45%) Alcohol (35%) Idiopathic (10–15%) Hypercalcemia Hypertriglyceridemia Drugs Post ercp Hereditary Trauma Infection Vasculitis Pancreatic cancer Pancreas divisum Sludge?

•

Alcohol ➢ Alters duct permeability -> protein precipitation in ductules Gallstones ➢ Common channel of bile and pancreatic ducts -> bile reflux into pancreatic duct

Chronic Pancreatitis

Acute Pancreatitis: Who Gets It?

Acute Pancreatitis: Pathophysiology •

Acute Pancreatitis: A Cascade of Events

Pancreatitis

460

Gastrointestinal Radiology

Acute Pancreatitis: Good and Bad •

•

Interstitial ➢ Edema ➢ Architecture preserved ➢ No hemorrhage Hemorrhagic ➢ Tissue necrosis, pancreas, fat ➢ Hemorrhage ➢ Vascular thrombosis & inflammation

Acute Pancreatitis: Clinical Dx • • •

•

Abdominal pain->back Nausea, vomiting DDx ➢ Perforated ulcer, bowel ischemia, cholecystitis Labs ➢ Hyperamylasemia ➢ Elevated lipase, more specific for pancreatitis ➢ Degree of enzyme elevation: no correlation w. severity

Acute Pancreatitis: Complications •

•

•

Early ( 2-3 days ) multi-organ failure ➢ Cardiovascular, pulmonary, renal ➢ Phospholipase A2, elastase, tumor necrosis factor, cytokines, IL-1,2,6, trypsinogen activated peptide (TAP) Intermediate ( 2-5 weeks ) ➢ Infection, pseudocyst, GI, biliary Late ( months – years ) ➢ Vascular, pseudoaneurysm

Balthzar 2002 Radiol Clin

Acute Pancreatitis: Plain Films •

•

Chest film ➢ Pleural effusion 43% w. severe pancreatitis ➢ ARDS ➢ Pulmonary infarction Duodenum or colon distention ➢ Sentinal loop = focal dilation ➢ Colon cutoff = gas-filled colon -> abrupt cutoff at splenic flexure

Acute Pancreatitis: GI Contrast • • • • •

No primary role; may screw up CT Perigastritis, duodenitis, colitis -> Thick folds Mucosa intact Mass effect from pancreatic fluid Fundal varices from splenic vein thrombosis

• • • • •

Of limited use in Dx Is pancreatitis associated w gallstones? Fluid collections? Vascular complications? Intervention

Acute Pancreatitis: Ultrasound

Gastrointestinal Radiology

461

Pancreatitis

Acute Pancreatitis: MRI •

•

Advantages ➢ Gadolinium easy on kidneys ➢ Able to view biliary tract Sick Patients ➢ Motion artifacts ➢ Difficult to monitor ➢ Specialized equipment ➢ Intervention difficult

Figure 2-26-1

Acute Pancreatitis: CT • • • • •

Best overall modality Global view Prognosis & followup Understand widespread nature of pancreatitis Routes for intervention

• •

Confusion of terms Acute pancreatitis ➢ Mild = minimal organ involvement, uncomplicated recovery w. supportive Rx ➢ Severe = organ failure or complications eg. pseudocyst, necrosis, infected necrosis, abscess

Large, well-encapsulated pseudocyst adjacent to pancreatic tail

Acute Pancreatitis: Terminology Atlanta Symposium 1992

Acute Pancreatitis: Acute Fluid Collections • • • • • • •

Extravasated pancreatic fluid Anterior pararenal space, lesser sac Not loculated No capsule 40% patients w. acute pancreatitis 50% resolve spontaneously May develop into pseudocyst

• •

Loculated collect. of panc. enzymes Non-epithelialized wall of fibrous or granulation tissue 4-6 wks to develop Arise from acute fluid collect. (30-50%) 50% resolve spontaneously > 5 cm less likely to resolve

Figure 2-26-2

Acute Pancreatitis: Pseudocyst [Figure 2-26-1] • • • •

Pseudocyst: Complications [Figure 2-26-2] • • • •

Infection Bile duct or GI obstruction Perforation -> adjacent organs Vascular ➢ Venous stenosis,occlusion ➢ Gastric varices ➢ Pseudoaneurysm ➢ Hemorrhage

(top)Large gastric varices produced by splenic vein thrombosis from pancreatic pseudocyst adjacent to splenic hilum. (bottom) Pseudocyst projecting from the pancreatic tail to the splenic hilum with no visualization of hilar splenic vein Pancreatitis

462

Gastrointestinal Radiology

Acute Pancreatitis : Necrosis

•

Non-enhancing pancreas or peripancreatic tiss. – old “phlegmon” Non-viable tissue Poor prognosis Type determines Rx ➢ Sterile-trial of med Rx ➢ Infected-debridement ➢ If infected, mortality 15-50% Needle aspiration to Dx

• • • •

Circumscribed collection of pus Develops after several weeks Needle aspiration Percutaneous drainage

• •

Central location affords several routes for spreading disease Anterior pararenal space ➢ Pancreas ➢ Duodenum ➢ Colon Bare area = reflection of post parietal peritoneum to form the transverse mesocolon Root of the small bowel mesentery contiguous w. transverse mesocolon Tail = intraperitoneal -> splenorenal ligament Posterior to the lesser sac

• • • •

Acute Pancreatitis: Abscess

Acute Pancreatitis: Location

• • • •

Barium Left Anterior Pararenal Space-o-Gram [Figure 2-26-3]

Figure 2-26-3

(top left) Extensive necrosis in the anterior pararenal space on both sides with air anterior to the left kidney mistaken for air in the colon. (bottom left). Delayed views showing contrast anterior to the left kidney. Thought to be in the colon. (right) Barium upper gi contrast study showing erosion of the pancreatic inflammation into the small bowel with barium contrast leaking throughout the entire left anterior pararenal space

Acute Pancreatitis: Good and Bad •

•

Interstitial (Good) 80-90% ➢ < 10% organ failure ➢ 1-3% mortality Hemorrhagic (Bad) 10-20% ➢ Necrosis ➢ 50-60% organ failure ➢ 15-20% mortality

Banks. Gastro Endoscopy 2002

Gastrointestinal Radiology

463

Pancreatitis

Acute Pancreatitis: Can we predict trouble? • • • • •

75% acute pancreatitis resolve w/o complications 5–20% mortality Can′t biopsy Who is really sick? Clinical Predictors ➢ Ranson Criteria Sens = 57–85%; Spec = 68–85% ➢ APACHE II = 77% pos. pred. value on admit; 88% after 48 hrs

Clinical Assessment of Pancreatitis Severity • •

•

RANSON Non-Gallstone pancreatitis ➢ Admission (any 3) ❖ > 55yrs old; WBC > 16K, ❖ Blood sugar >200 ❖ LDH >350 ; AST > 250 ➢ At 48 hrs (any 3), ❖ Hct decr >10 ❖ Rise in BUN > 5 ❖ Calcium < 8 ❖ PO2 < 60 ❖ Fluid deficit > 6L ❖ Base deficit > 4 APACHE II ➢ Vitals signs ➢ PO2 ➢ pH ➢ Electrolytes ➢ Creatinine ➢ HCT; WBC ➢ Glasgow coma score

Can Imaging Alone Predict Trouble? Yes •

CT grading (Balthazar 1985;1990) ➢ A = Normal ➢ B = Focal or diffuse enlargement ➢ C = Peripancreatic inflammation ➢ D = Single fluid collection outside gland ➢ E = 2 or more fluid collections or gas in or near panc. ➢ 83 PTS ❖ All A’s discharged w/o complications within 2 wks ❖ A or B -> no abscess ❖ D or E -> 5 of 6 deaths; 89% of abscesses

Imaging Predicts Trouble. Can we refine this further? • •

Problem: After classifying patients as high-risk, fluid collections resolved in 54% Pancreatic necrosis ➢ Poor gland enhancement correlates w. degree of necrosis at surgery (Kivisaari GI Radiol 1984) ➢ Gland necrosis correlates with development of complications (Balthazar Radiol 1990) ❖ No necrosis = no mortality; 6% morbidity ❖ Necrosis = 23% mortality; 82% morbidity

Pancreatitis

464

Gastrointestinal Radiology

Acute Pancreatitis: Can we predict trouble? [Figure 2-26-4] • •

• •

CT Severity Index (Balthazar Radiol: 1990) CT anatomic changes ➢ A = 0, B = 1, C =2, D = 3, E = 4 Gland necrosis < 30% = 2, 30-50% = 4, > 50 = 6 ➢ 0-1 = no mortality or complications ➢ 2 = no mortality; 4% complications ➢ 7-10 = 17% mortality; 92% complications

Figure 2-26-4

Acute Pancreatitis: The Power of CT • • • • •

Suspected pancreatitis – Dx in doubt Severe pancreatitis suspected of complications Pancreatitis w/o improvement in 72 hrs of med. Rx Improving pancreatitis that deteriorates Severe pancreatitis w. initial scan D-E; CTSI 3–10 – follow-up may detect asymptomatic complications

Chronic Pancreatitis •

Definition (Marseilles 1985) ➢ Recurrent or persistent abdominal pain ➢ May or may not see increase enzymes ➢ Irreversible morphological change in pancreas ❖ Fibrosis ❖ Acinar destruction ❖ Calcification, duct /parenchyma ➢ Focal, segmental, diffuse ➢ Progressive loss of exocrine/endocrine function

Chronic Pancreatitis •

•

Who Gets It? ➢ Chronic alcohol abuse (60–70%) ➢ Idiopathic (30%) ➢ Biliary tract disease ➢ Hereditary ➢ Hyperlipidemia ➢ Hyperparathyroid ➢ Pancreas divisum Clinical ➢ Recurrent abdominal pain (95%), ➢ Pancreatic insufficiency , ❖ Malabsorption, ❖ Diabetes, ➢ Amylase/Lipase levels +/– abnormal

Balthazar Classification of severity of acute pancreatitis

(top). Mild Pancreatitis: CTSI = 0-1. Small amount of peripancreatic stranding. No fluid collections. Entire gland enhances. (bottom). Severe Pancreatitis: CTSI = 8-9. Pancreas outlines are obliterated with necrosis. No enhancement with contrast

Chronic Pancreatitis: Pathophysiology •

Poorly understood ➢ Etoh increases ductal secretion -> ➢ Precipitation of protein plugs -> ➢ Calcification ❖ Chain of lakes / dilated duct ➢ Inflammatory infiltrate + fibrosis

Chronic Pancreatitis: Plain Films •

Pancreas Ca++ (75-90%) ➢ Most common in Etoh pancreatitis, ➢ Ductal or parenchymal ➢ May be focal ➢ Increase w. progression pancreatic dysfunction ➢ Also w. hereditary pancreatitis, cystic fibrosis

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Chronic Pancreatitis: Barium [Figure 2-26-5] • •

Inflammation/scar -> perigastritis Not primary disease of bowel

Figure 2-26-5

(left) Severe distortion of the gastric contours on double-contrast barium study from chronic pancreatitis with inflammatory changes and scar in the perigastric tissues, so-called "perigastritis." There is no primary gastric disease. (right) CT appearance of the same patient showing changes of chronic pancreatitis with parenchymal calcifications and gland atrophy

Chronic Pancreatitis: Ultrasound

Heterogenous echotexture ➢ Hyperechoic foci = Ca++/ fibrosis, ➢ Bile &/or pancreatic duct dilation ➢ 40% focal mass DDx = cancer ➢ Complications ❖ Pseudocyst portal / splenic vein thrombosis ➢ Endoscopic ultrasound? ❖ 98% sensitivity / 90% specificity?

Chronic Pancreatitis: Endoscopic Ultrasound • •

Difficult to establish a gold standard esp. for mild to moderate disease Few studies with histology ➢ Sens = ?87%; Spec = ?64%

Chronic Pancreatitis: CT • • • • • •

Not as useful as in acute pancreatitis Gland enlargement (30%) Mass (30%) ? Cancer Atrophy (15%) Sens. 50-90%: Spec. 55-85% Acute + chronic w. exacerbation of disease

•

Parenchymal enhancement ➢ T1 fat-supressed, pre & post Gd dynamic ➢ Decreased signal/Delay in peak vs. controls ➢ Sens = 79%; Spec = 75% ➢ Better than morphologic changes alone MRCP- ductal anatomy ➢ Highly T2 weighted, single breath-hold sequences ➢ 85-90% agreement w. ERP for duct caliber ➢ Limited ability to dx early chronic pancreatitis ➢ Functional exam w secretin not conclusive

Chronic Pancreatitis: MRI

•

Remer EM Radiol. Clin. of N. Am.2002

Chronic Pancreatitis: ERCP •

Cambridge Classification of chronic pancreatitis ➢ Mild = 3 side branches dilated; main duct 2–4 mm ➢ Moderate = small cysts, irregular duct ➢ Severe = any of above + ❖ Cyst >10mm, intraductal filling defect, calculi, main duct obstruction, severe irregularity

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Chronic Pancreatitis: Pancreas Divisum • • • • •

Dorsal & ventral ducts fail to fuse (5%) Minor papilla (Santorini) atretic Most of pancreas drained through atretic minor papilla Pancreatitis? Dx = ERCP

Pancreatitis Requests We Have Known and Loved

Pancreatitis: “Febrile. Please aspirate fluid.” What then? • • •

•

You are in charge of thinking ahead. Modality? Route? ➢ Transgastric? Not for diagnosis only What is the plan? ➢ Pus -> tube ➢ Indeterminate -> Gram stain +/- tube ➢ Clear fluid -> Gram stain, culture ➢ Solid stuff -> no flow -> saline -> culture. BX?

Pancreatic Pseudocyst

Pancreatic Fluid Collection: “I am happy to help, but what is the indication for drainage? My staff wants it” • • •

Indication for access to evolving fluid collection or necrosis decided on full evaluation of clinical, lab, and imaging Percutaneous drain useless if won′t flow through tube ➢ No tube for necrosis or hematoma ➢ Aspiration to dx infected necrosis Uninfected collections and small pseudocysts may resolve on their own

References 1.

Topazian M, Gorelick GS. Acute Pancreatitis. In: Yamada T, Textbook of Gastroenterology, Third Edition, Volume 2. Lippincott Williams and Wilkins, 1999, 2121-2150. 2. Owyang C. Chronic Pancreatitis. In: Yamada T, Textbook of Gastroenterology, Third Edition, Volume 2, Lippincott Williams and Wilkins, 1999, 2151-2177. 3. Banks PA. Epidemiology, natural history, and predictors of disease outcome in acute and chronic pancreatitis. Gastrointestinal Endoscopy 2002; 56 (6) S226-S230 4. Meyers MA. Dynamic Radiology of the Abdomen Normal and Pathologic Anatomy, Fifth Edition.Springer, New York 2000. 5. Balthazar EJ. Staging of Acute Pancreatitis. Radiol. Clin. of N. Am. 2002;40:6, 11991209. 6. Balthazar EJ. Complications of Acute Pancreatitis. Radiol. Clin. of N. Am 2002; 40:6, 1211-1227. 7. Remer EM, Baker ME. Imaging of Chronic Pancreatitis. Radiol. Clin of N. Am 2002; 40:6, 1229-1242. 8. Fulcher AS, Turner MA. MR Cholangiopancreatography. Radiol. Clin of N. Am 2002: 40:6, 1363-1376. 9. Strate T, Knoefel WT, Yekebas E, Isbicki JR. Chronic Pancreatitis: etiology, pathogenesis, diagnosis, and treatment. Int. J Colorectal Dis. 2003; 18: 97-106,. 10. Chatizicostas C, Roussomoustakaki M, et al. Balthazar Computed Tomography Severity Index Is Superior to Ranson Criteria and APACHE II and III Scoring Systems in Predicting Acute Pancreatitis Outcome. J. Clin. Gastroenterol. 2003; 36: 3, 253-260. 11. Wiersema MJ, Hawes RH, et al. Prospective evaluation of endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography in patients with chronic abdominal pain of suspected pancreatic origin. Endoscopy 1993;25:555-564.

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Gastrointestinal Bleeding In The Age of the Endoscope. What Does a Radiologist Have To Contribute? Bruce P. Brown, MD

GI Bleeding: Demographics • • • • •

Older Male Use alcohol, tobacco Aspirin, non-steroidal anti-inflammatory Anticoagulants

Peura et al, Am.J.Gastro. 1997

Gastrointestinal Bleeding: Presentation • • •

Hematemesis – Bloody vomitus, red, coffee grounds; indicates upper GI bleeding Melena – Black, tarry stools; usually indicates upper GI bleeding Hematochezia – Red blood per rectum; lower GI bleed, large-volume upper GI bleed (> 1000 cc)

Acute GI Bleeding: Demographics •

•

• • • • • •

Upper GI 76% ➢ Duodenal & gastric ulcers >50%, Lower GI 24% ➢ Diverticular 30-50% 79% Anemia 31% Hypovolemia 59% Transfused 45% Endoscopic Rx 7% Surgery 2% Death

Peura et al, Am.J.Gastro. 1997

Gastrointestinal Bleeding: How Bad Is It? •

Hypovolemia - 30% of GI bleeders ➢ 5 L (10 Units) = normal volume ➢ Hct poor measure of acute bleeding ➢ 20% blood loss -> 10 mmHg drop BP w. standing ➢ 40% blood loss = Shock = resting supine tachycardia, hypotension, pallor, agitation ➢ Massive GI bleed = > 6 units transfusion needed in 24 hours

Acute Gastrointestinal Bleeding: Diagnosis is NOT the first priority •

Resuscitation ➢ Two BIG lines 18 gauge ➢ Fluids immediately ➢ Blood when available; 6 u typed & crossed ➢ ICU

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Gastrointestinal Bleeding: Where Is It? •

•

Upper GI ➢ Proximal to ligament of Treitz, ➢ Usually melena ➢ NG tube – 16% negative even w. UGI bleed Lower GI ➢ Distal to the ligament of Treitz ➢ Usually hematochezia

Upper GI Bleeding: Causes •

Diagnosis ➢ Duodenal Ulcer ➢ Gastric Erosions ➢ Gastric Ulcer ➢ Varices ➢ Mallory-Weiss tear ➢ Esophagitis ➢ Neoplasm ➢ Other

% of total 24 23 21 10 7 6 3 11

Silverstein et al,Gastro.Endosc. 1981

Lower GI Bleeding: Causes •

Diagnosis ➢ Diverticulosis ➢ Vascular Ectasia ➢ Idiopathic ➢ Neoplasia ➢ Colitis ❖ Radiation ❖ Ischemia ❖ Ulcerative colitis ➢ Other

Reinus et al GI Clin NA 1990

% of total 43 20 12 9 6 2 1 7

GI Bleeding: Endoscopy •

• •

First line procedure in UGI bleed ➢ 90–95% accurate Dx ➢ Useful for prognosis, treatment Performed immediately ➢ Alcoholics, ➢ Large volume loss ➢ Aorto-enteric fistula Performed more “electively” ➢ Young, no evidence of hypovolemia

Nuclear Scintigraphy • • •

•

Most sensitive non-invasive test Detects bleeding rates 0.1ml/min Two techniques ➢ Tc 99m sulfur colloid ➢ Tc 99m labeled red blood cells Used to ➢ Delineate obscure sources – small bowel, intermittent bleeding ➢ Enhance the efficacy of angiography

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Angiography • • •

•

Usually preceded by RBC study Detects 0.5 ml/min Upper GI bleeding ➢ When endoscopy inconclusive ➢ Anticipation of transcatheter intervention Lower GI bleeding ➢ Procedure of choice?

Upper GI Bleeding: Peptic Ulcer Disease • • •

•

Gastric, duodenal, stomal ulcers = 50% UGI bleeding Etiology: Non-steroidals, H. Pylori Anatomic risk factors ➢ High lesser curve ➢ Posterior-inferior duodenal bulb ➢ Giant gastric (>3 cm) & duodenal (>2 cm) Endoscopic risk factors

Risk of Rebleeding: Endoscopy •

Peptic ulcer disease rebleeding ➢ Clean fibrin base ➢ Flat spot ➢ Adherent clot ➢ Visible vessel ➢ Spurting vessel

5% 10% 22% 43% 90%

Laine NEJM; 717; 1994. UCLA-CURE studies.

Gastritis • • •

Hemorrhage, erythema, erosions Erosion = superficial break in mucosa w. punctate bleeding, fibrin base Causes ➢ Non-steroidals -> antral erosions, ulcer ❖ bleeding usually not severe, resolve w. D/C ➢ Alcohol ingestion ❖ Direct toxin? ->erythema

Gastritis •

• •

Portal hypertension ➢ Diffuse or patchy erythema, punctate bleeding, vascular ectasia Requires reduction of portal hypertension Stress Erosions ➢ ICU patients ➢ One or more bleeding erosions ❖ Bleeding may be severe

Acute Hemorrhagic Gastritis Esophageal Varices • • • •

• • • •

50% cirrhotics develop esoph. varices. 1/3 of these bleed Portal v. pressure >12 mmHg. above Hep.v At risk to bleed ➢ Large size ➢ Located near GE Junct. ➢ Vascular ectasia on the varices Rapid bleeding Emergent endoscopy 50% of cirrhotics w. bleed = non-variceal Poor prognosis ➢ 30–50% mortality for first bleed ➢ 2/3 die within one year

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Esophageal Varices: Rx • •

•

•

•

•

Vasopressin ( somatostatin/octreotide ) ➢ 50% effective Sclerotherapy – 85–95% effective ➢ Probably improves survival; complications Band ligation ➢ As effective as sclero Rx; few complications Balloon tamponade ➢ 70–90% effective ➢ 30–50% rebleed after balloon down, ➢ 10–30% severe complications TIPS (Transjugular Intrahepatic Portosystemic Shunt) ➢ Expandable stent – hepatic to portal v. ➢ 95% technically successful ➢ As effective as sclero Rx ➢ 10–15% complications ➢ 10–25% encephalopathy ➢ 30–50% stenosis at 1 year Surgical porta-caval shunts ➢ 50–80% mortality for emergency shunt ➢ Elective shunts for endoscopic Rx failures

Gastric Varices Without Esophageal Varices Mallory-Weiss Tear • • • • •

5–10% GI bleeds Hx of retching; 40% no retching Non-penetrating linear tear(s) near GEJ ➢ 25% multiple lesions; 75% have o. pathol. 90% resolve spontaneously Rx ->endo.oversewing

Gut Hemangioma • • • •

Rare Described in young and old Esophagus, stomach, sm. bowel, colon Classification ➢ Capillary – collection of thin-walled vess. ➢ Cavernous – large, dilated channels w. thrombosis -> Ca++ ❖ Tendency to bleed ➢ Angiomatosis – large area of hemangioma

Gut hemangioma •

• •

Cavernous hemangioma ➢ Phleboliths on plain film ➢ UGI = Submucosal mass CT ➢ Thick wall ➢ Early enhancement – network of vessels & sinuses thickening the wall ➢ Late enhancement – confluent sinus fill-in Endoscopy ➢ Soft, submucosal mass or thickened folds, blue-red discoloration

Small Bowel Bleeding: Tough to Dx • • • • •

3–5% GI bleeds occur in small bowel (2nd portion duod. to ileocec. valve) Bleeding is intermittent Most common causes are vascular Inaccessible Anatomy variable

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Small Bowel Bleeding: Causes •

• •

• •

Vascular lesions ➢ Angiodysplasia, hemangioma, AVM, vasculitis Small bowel tumors ➢ Leiomyoma/sarcoma, adenoma/carcinoma, lymphoma, mets Ulcers ➢ Crohn’s, Meckel’s diverticulum, ZE syndrome Diverticula Aortoenteric fistula

Small Bowel Bleeding: How Well Does Imaging Do? •

• •

•

Small bowel series vs enteroclysis ➢ 71% lesions missed on small bowel series [1] Small bowel series for occult bleeding ➢ 5% yield for bleeding site [2] Enteroclysis ➢ 10 % yield for bleeding site [3] Enteroscopy ➢ Cumbersome, not generally available

[1] Maglinte, Radiol 144:737; 1982 [2] Rabe, Radiol. 140:47; 1981 [3] Rex, Gastro 58;89; 1997

Small Bowel Bleeding Nuclear Scintigraphy • • •

•

Most sensitive non-invasive test Detects bleeding rates of 0.1 ml/min Two techniques ➢ Tc 99m sulfur colloid ➢ Tc 99m labeled RBC′s Used to ➢ Delineate obscure sources – small bowel, intermittent bleeding ➢ Enhance the efficacy of angiography

Technetium 99m Labeled RBC′′s • •

New in vitro process (Ultratag) >95% eff. Continuous dynamic imaging ➢ Large FOV camera over abdomen ➢ 60 images q 15 min ➢ Stored for dynamic playback to detect labeled RBC’s outside normal blood pool

Technetium 99m Labeled Red Blood Cells •

• •

Disadvantages ➢ Origin of bleed unclear on delayed scans ➢ Vascular organs may interfere w. detection ➢ Loss of tag can produce false +/Advantages ➢ Detects intermittent bleeding Labeled RBC′s ➢ Sensitivity = 85–95%; Specificity = 70–85%, ➢ Method of choice

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Meckel’s Diverticulum

•

Most common congenital GI tract anom. Vitelline duct fails to resorb True diverticulum – ➢ 2% of population ➢ 2 x more common in males ➢ 2 cm long ( 1–10 cm), ➢ 2 feet from ileocecal valve 50% ectopic gastric or pancreatic mucosa 25–40% symptomatic Complications ➢ Bleeding – usually in kids <5 yr, ➢ Intussusception – kids & adults ➢ Volvulus, diverticulitis, perforation Bleeding – ulceration of gastric mucosa

• • • • •

Erosion of aorta into 3rd portion of duod, Dacron graft, atheroma, mycotic aneurysm “Herald bleed” stops spontaneously followed by exsanguinating bleed High index of suspicion Preemptive surgery

• • • • • • •

Ingestible capsule 7 hour recording 2 images per second Localizing surface antennae View in “real-time” Contraindicated w. obstruction 22% Capsule vs. 3% barium-positive in 52 pts w. occult gi bleed

• • •

• • •

Aortoenteric Fistula

Pill Endoscopy

Hara AK, Radiol 2004, 230: 260-265)

Lower GI Bleeding: Causes •

Diagnosis ➢ Diverticulosis ➢ Vascular Ectasia ➢ Idiopathic ➢ Neoplasia ➢ Colitis ❖ Radiation ❖ Ischemia ❖ Ulcerative colitis ➢ Other

Reinus et al GI Clin NA 1990

% of total 43 20 12 9 6 2 1 7

Colonic Diverticulosis •

Colon Diverticula = herniations of mucosa and submucosa through muscular layers at site of penetration of vasa recta through bowel wall.

Colonic Diverticular Bleeding • • • • • • • •

35–50% prevalence of diverticula 15% pts. tics bleed 5% massively The major cause of lower GI bleed 75% of tics in left colon 70% of bleeding tics in right colon [1] 80% resolve spontaneously Not asst′s w. diverticulitis

[1] Cassarella, NEJM 286:450;1972 Gastrointestinal Radiology

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Colonic Diverticular Bleeding: RX • •

•

Colonoscopic vasoconstrictor injection, heater probe, laser – select patients Angiography ➢ Selective catheterization ❖ Vasopressin 50-90% success ❖ Embolo Rx – Gelfoam, coils Surgery

Angiodysplasia • •

• • • •

• • •

20–40% acute LGI bleeding Vascular ectasia ➢ 2/3 in pts >70 yrs old ➢ Aortic valve disease ❖ Von Willebrand factor depletion? < 5mm vascular tufts Cecum & right colon Bleeding ➢ Not massive, intermittent ➢ Stop spontaneously, 85% bleed again Pathogenesis ➢ Increased tension in cecal wall ➢ Repeated, intermittent obstruction of submucosal veins -> dilation & tortuosity ➢ Develop small A-V malformation Colonoscopy 80–90% sensitive Angiography ➢ early tangle of vessels ➢ early filling & slow emptying dilated veins Treatment ➢ Abnormal vessels – poor response to vasoconstrictors; may temporize ➢ Endoscopic electrocoagulation ➢ Embolo Rx ➢ Diffuse disease – estrogen-progesterone ➢ Surgery

References 1. 2. 3. 4. 5. 6. 7.

Peura DA, Lanza FL, et al. The American College of Gastroenterology Bleeding Registry: Preliminary Findings. Am J Gastroenterol. 1997, Jun: 92(6): 924-8. Reinus JF, Brandt LJ. Upper and lower gastrointestinal bleeding in the elderly. Gastroenterology Clinics of North America. 1990 Jun; 19(2): 293-318. Mitros FA, Atlas of Gastrointestinal Pathology. Gower Medical Publishing. Elta GH, Approach to the patient with gross gastrointestinal bleeding. In Textbook of Gastroenterology, Lippincott, Williams and Wilkins. Philadelphia, 1999, Yamada T, et al eds. Fritscher-Ravens A, Swain CP. The wireless capsule: new light in the darkness. Dig. Dis. 2002;20(2): 127-33. Hara, AK et al. Small bowel: preliminary comparison of capsule endoscopy with barium study and CT Radiology 2004, 230: 260-265. Hara AK. Capsule endoscopy: the end of the barium small bowel examination?Abdom Imaging, 2005, Jan.

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Small Bowel Obstruction Francis J. Scholz, MD

Figure 2-28-1

Small Bowel Obstruction

• • • •

“Impaired passage of contents thru SB.” Partial vs Complete (“High Grade”) Intermittent vs Continuous Mechanical vs Paralytic (“Ileus”)

•

Review ➢ Mechanical ➢ Classic Acute “Complete” SBO ❖ Simple SBO ❖ Closed Loop Obstruction (CLO) - Urgent Emergency ! ➢ Classic Appearances ❖ Intermittent “Chronic” SBO ❖ Partial SBO ➢ Motility ❖ Common Ileus ❖ Unusual dysmotilities

SBO

SBO •

Motility ➢ Paralytic Ileus ➢ Scleroderma “Collagen Vasc Disease” ➢ Sprue, MAB diseases ➢ Radiation enteritis, earliest stage ➢ Hypothyroidism, metabolic ➢ Chronic Intestinal Pseudo-obstruction ➢ DYSMOTILITY is a FUNCTIONAL Obstruction ! ➢ Slow passage acts / looks obstructive

Acute mechanical SB obstruction showing uniformly distended bowel

Figure 2-28-2

Chronic vs Acute SBO: Concept to help analyze SB in CT, KUB, SB Series •

Distention vs Dilatation: 2 variables ➢ Dilatation: SB diameter larger than expected ❖ A few loops or entire SB ❖ May or may not be Distended ➢ Distention: SB uniform appearance of maximum possible diameter ❖ Like a sausage shaped balloon inflated to its capacity ❖ Appears tensely filled, to capacity

Simple SBO • •

A tapered distension meandering back toward Treitz. A single transition point

•

Dilated but not distended

• •

Distention vs Dilatation Distended, not (XS) Dilated: ➢ Acute, initial SBO Dilated, not Distended: ➢ Chronic, intermittent SBO or ➢ DYSMOTILITIES !

Chronic Intermittent SBO

Chronic vs Acute SBO [Figures 2-28-1 to 2-28-3] •

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Scleroderma SB shows dilated loops with segments whose diameter are greater than last case of acute SBO. Loops are not uniformly distended with segments that are partly collapsed

Small Bowell Obstruction

•

Figure 2-28-3

Dilated and Distended: ➢ Acute, recurrent SBO

Chronic Idiopathic Intestinal Pseudo-obstruction “CIIPO” • •

•

No cause (readily) apparent. Myopathic forms: ➢ More common ➢ Dilated, ➢ Atonic ➢ Conceptually like: Scleroderma Neuropathic forms: ➢ Spastic, ➢ Non propulsive peristalsis ➢ Pulsion divertics of SB ➢ Conceptually like: Diffuse Esophageal Spasm

Bloating, Obstruction • • • •

Prior Colectomy for constipation with Ileo-rectal anastomosis Idiopathic Intestinal Pseudoobstruction Myopathic type

• •

Mitochondrial Neuro Gastro Intestinal Encephalopathy MNGIE Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy, Intestinal Pseudo-obstruction. P-O-L-IP Rarenth power (73 cases up to 2005) Familial, Autosomal Recessive

[Figure 2-28-4]

MNGIE = POLIP • •

Blondon H, et al Digestive smooth muscle mitochondrial myopathy in pts with mitochondrial-neuro-gastro-intestinal encephalomyopathy (MNGIE), 3 cases & review of literature; Gastroenterologie 2005 Aug. SB appears both dramatically dilated and uniformly distended.(double VOL 29 - N 8-9,p. 773 - 778 arrow). This suggests acute Simon et al, Polyneuropathy, Ophthalmoplegia, obstruction in a patient with chronic Leukoencephalopathy, Pseudoobstruction: POLIP Syndrome; Ann recurring obstruction from Crohns Neurol 1990;28:349-360 Disease (arrow)

Figure 2-28-4

48 Hr films shows barium in proximal SB. Dysmotility diseases may produce massive dilatation. Segments that do not propel act like mechanical obstruction. Diseases affecting nerves and muscles of the bowel are multiple, infrequent, and require extensive work-up. A history of prior partial colon resection, SMA syndrome requiring duodeno-jejunostomy, volvulus of colon in a young pt, or recurrent obstructions without apparent cause should raise suspicion Small Bowell Obstruction

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Radiology of “POLIP” “MNGIE” [Figure 2-28-5] • •

• • • • • •

Slow GI Transit Non-propulsive SB hypermotility ➢ LIKE DES Corkscrew esophagus SB Tics from segmental spasm Malabsorption pattern: wet, moulage, delay MRI: White matter changes, high signal on T2 Transition Point: “Single Discordance” Tapering: Normal @ Treitz to max obstruction SB Meanders to max allowed by mesentery

Figure 2-28-5

CT: Acute SBO [Figure 2-28-6] •

•

Early (ER) CT: ➢ Reduce mortality & morbidity ➢ Cost effective Diagnose SBO ➢ Grade Severity ❖ Simple ❖ Closed Loop ❖ Strangulating ❖ Dead Bowel ➢ Etiology

Segmental non coordinated SB contractions of the small bowel may produce PULSION DIVERTICULA (T) and marked delay in transit, findings both evident on a 24 hr follow up film with contrast still in stomach and proximal SB. This “neuropathic intestinal pseudo-obstruction pattern” may be seen in other rare diseases of disordered mitochondrial activity altering peristaltic neuromuscular bowel coordination

CT: Acute SBO • •

Holy Grail = Transition Point Define Lesion: ❖ Tumor, hernia ➢ No “Lesion” = “Adhesion” ➢ Study: ❖ Colon -? Collapsed SBO Fluid filled: Ileus, MAB ❖ Ileocecal Valve ❖ Duodenal Crossing ❖ Mesenteric Vessels

Figure 2-28-6

Adhesion • •

•

MAJOR CAUSE SBO: Benign Adhesions ➢ Surgical ➢ Inflammatory -itis ➢ Radiation ➢ Endometriosis ➢ Ischemia Neoplastic Adhesions ➢ (Carcinoid) Transition point without discernable mass or hernia indicates adhesion

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Small Bowell Obstruction

Tethered Adhesion [Figure 2-28-7]

Figure 2-28-7

Bezoar [Figure 2-28-8] •

•

Rarely sole cause SBO: ➢ THINK: motility dis. ❖ Radiation, scleroderma ➢ Nl person: Fiber binge More often part of SBO: ➢ Fibrous Food impaction just above minor “lesion”. (adhesion) “SB Feces sign” ➢ POINTS TO OBSTRUCTION

Mayo-Smith WW, Wittenberg, et al. CT SB faeces sign: description & clinical significance. Clin Radiol. 1995 Nov;50(11):765-7

Abdominal Hernias •

• •

•

Tenting or sharp angulation of a loop is suspicious for adhesion or entrapment of its mesentery

By Location ➢ External vs Internal ➢ Inguinal, Femoral, Sciatic, Hiatal, Spigelian, etc By Type ➢ Complete vs Partial (Richter) By Content ➢ Littre (pre-existing tic), Amyand (appendix) By Severity ➢ Reducible ➢ Non-Reducible or “Incarcerated” ➢ Ischemic or “Strangulated” Figure 2-28-9 ➢ Infarcted

Figure 2-28-8

Obturator Hernia [Figure 2-28-9]

• • • • • • •

Elderly F 10 to 1 M R >> L Assoc w recent wt loss Not palpable SBO in Obturator Pectineal Space Howship Romberg Sign ➢ Pain medial thigh +/50% Hannington Kiff ➢ Absent thigh adductor reflex

Ijiri R, et al Oburator H: usefulness of CT in DX.: Surg.1996 Feb;119(2):137-40.

SB “Bezoars” are useful to point to a site of obstruction. They are composed of residual fibrous material that begins fermentation. Carrots, mushrooms, and other fibrous foods may cause transient symptomatic obstruction in normal patients when the bolus reaches the ileocecal valve. Poor peristaltic tone may play a role in pts with dysmotility disorders

Obturator Hernias are unusual hernias difficult to detect by simple physical examination. Special maneuvers are needed. Tiny barely visible hernias may produce significant obstruction Small Bowell Obstruction

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Spigelian Hernia SBO [Figure 2-28-10] •

Note hernia under extern obl muscle

Figure 2-28-10

Spigelian Hernias may not be evident on physical examination. Classic location is either lower quadrant lateral to rectus muscle. The hernia sac protrudes through a normal weak point where the transversalis muscle fascia changes from posterior to anterior attachment to the rectus sheath. Often the hernia stays under the external oblique muscle

Figure 2-28-11

Incarcerated & Strangulated Parastomal Hernia [Figure 2-28-11]

•

• •

•

SBO upstream ➢ Efferent Limb collapsed ➢ Neck squeezed CLO ➢ Distended Hernia Loop Strangulation ➢ Hernia Sac Fluid Incarceration ➢ Compressed Abd Wall

Chronic incarceration, or nonreducibility, may cause inward bowing of the anterior abdominal wall. Incarceration often is associated with intermittent obstruction and predisposed to vascular compromise Gastrointestinal Radiology

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Small Bowell Obstruction

Gallstone Ileus [Figure 2-28-12]

Figure 2-28-12

Zalcman M, et al Helical CT signs in Dx of intestinal ischemia in SBO. AJR 2000 Dec;175(6):1601-7

Value of Combining 2 signs for Dx of Ischemia in SBO

Sign Combination

Mural Thickening + Mesen Fluid Mural thickening + Mesen Vasc Congest Mural Thickening + Ascites Mesenteric Fluid + Mesen Vasc Congest Mesenteric Fluid + Ascites Mesenteric Vasc Congestion + Ascites

Sens% Spec % 29.2

99.2

25

94.2

29.2 50

66.7 41.7

93.3 94.2 94.2 94.2

Zalcman et al Helical CT Signs in Dx of Intestinal Ischemia in SBO; AJR 2000;175:1601-1607

SB Series: Value of Tangent

Value of Tangent…with Valvalsa

? ISCHEMIA Gallstone Ileus with compromised bowel. Note fistula to duodenum, air in hepatic bile duct, and stone in distal SB. Top right image shows a target sign loop (left of diamond) with white internal mucosa and (right of diamond) adjacent loop which has less mucosal enhancement . The lower right arrow points to the longitudinal equivalent of the target sign. Acute high grade unrelieved obstruction causes edema from compression of mucosal venous drainage. Although above findings are of obstructive edema, simple SBO may lead to bowel infarction

Parastomal Hernias: Tangent with Valsalva Mesenteric Volvulus •

•

Assoc w ➢ Malrotation ❖ Left Colon ❖ Right SB ❖ “Weak” Treitz ➢ Internal Hernia ➢ External Hernia ➢ Post operative Short / bunched mesentery

Closed Loop Obstruction •

• • • •

Lumen occluded at 2 adj. sites ➢ Adhesion, ➢ Hernia - Internal, External ➢ Tumor, ➢ Volvulus Obstructed loop fills w fluid, Distends, elongates Base narrows, loop twists Venous & Art Occlusion - Infarct may result or may be chronic intermittent

Maglinte, Herlinger, Nolan Rad of CLO: 25 confirmed cases. Radiology. 1991 May;179(2):383-7 (Chronic)

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CT Closed Loop Obstruction (CLO) [Figure 2-28-13] •

• •

•

Closed Loop “knot” ➢ Clustering of SB loops ➢ Blocked at two ends ➢ Very distended Mesentery: ➢ Bunching of engorged vessels SBO above CLO ➢ Less distended Ascites

Figure 2-28-13

Single Discordance of Simple SBO Double Discordance of CLO - SBO

SB CLO Ischemic & Hemorrhagic [Figure 2-28-14] •

• •

Bowel wall changes ➢ Thickening, ➢ High attenuation I-, ➢ Target sign, Abnormalities in attached mesentery, Absence of findings of ischemia or infarction in CLO does not rule out strangulation

Balthazar et al Closed-loop & strangulating intestinal obstruction: CT signs. Radiology. 1992 Dec

CLO SBO Problems in Dx • •

•

• • •

Knot may be elongated Mesenteric engorgement ➢ Knot angle on slice may obscure Obst loops prox to CLO, and decomp loops distal may mingle with CLO Ascites may mask mes changes “BOW TIE” CLO SBO High Suspicion on all SBOs: ➢ Diameter CLO big & uniform ➢ Criss Crossing: Vessels / SB ➢ Thick walls ➢ No oral contrast gets in

It is critical to diagnose Closed Loop SB Obstruction. With early CLO SBO, fluid will fill the lumen of the closed loop. Then the fluid heavy loop will spin slightly around the vessels. This compromises venous outflow engorging the wall. Then the elevated hydrostatic pressure forces more fluid into the lumen distending it to the maximum. Additional serum, plasma,and eventually blood may ooze into the wall. This increases the weight of the loop and it tends to twist further. This progressive twisting begins compromising arterial inflow. Rapid bowel infarction may then occur. This may all happen before the attending radiologist comes in the next morning

Figure 2-28-14

A non-contrast CT shows high density in the wall of distended loops of SB indicating intramural hemorrhage is occuring

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CLO SBO in Camouflage [Figure 2-28-15]

Figure 2-28-15

CLO SBO Dead Bowel

Criss-Crossing Vessels / Bowel [Figure 2-28-16]

Bow Tie CLO SBO [Figure 2-28-17] Gastric Bypass [Figure 2-28-18] Gastric Bypass Simple Obstruction

Gastric Bypass Closed Loop Obstruction

CLO: Stomach to Roux [Figures 2-28-19 and 2-28-20]

Sandrasegaran K, Maglinte DD, et al CT of acute bilio-pancreatic limb obst. AJR 2006 Jan;186(1):104-9 Scheirey C, Scholz F, et al. Radiology Lap Roux-Y Gastric Bypass: Conceptualization and Precise Interpretation Radiographics in press Sept 2006

CLO SBO may be obscured by location of the “knot”, by small or large size of knot, by the angle of the “knot” relative to the slice, by ascites obscuring the mesenteric bunching, and by intertwining of other loops of decompressed distal SB and simply-obstructed proximal SB among the loops of the CLO SBO complex

Figure 2-28-16

Note that on sequential sections there is an abrupt crisscrossing of bowel and vessels. This finding is suggestive of entrapment of mesentery by adhesions or internal hernia

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Obst post Roux

Figure 2-28-17

Paraduodenal Hernia • • •

• •

50% IH = Paraduodenal Mortality pre-CT era (20%) Clinically: ➢ Asymptomatic, ➢ Pain, ➢ SBO, Left 3X > R; M > F SB entrapment = ➢ Congenital anomaly

J Comp. Assist. Tomo. 10:542, 1986

Figure 2-28-18

Bilio Pancreatic Limb

Alimentary Limb

The “Bow Tie” CLO SBO occurs when two or more loops are involved in the closed loop. There will be the simple SBO above the first closed loop. There will be two or more CLOs with each having uniform but differing degrees of distension. CLO 1 in the upper abdomen is shorter in length, more distended, and shows thin perfusing walls. CLO2 involves a more distal longer limb with thicker walls

Common Limb to Cecum Gastric bypass for morbid obesity offers many opportunities for SBO and one unique situation for CLO SBO

Figure 2-28-19

Closed Loop SBO obstruction may occur in Gastric Bypass patients when the bypassed Bilio-Pancreatic Limb obstructs at the Roux Y anastomosis. The Stomach has been stabled shut so a closed loop obstruction is created. While there is not the risk of a twisting of vessels, this obstruction puts high pressure on the closed gastric stump staple line

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Ascending Meso-colic H [Figure 2-28-21] • • • • • •

Figure 2-28-20

“Right PDH” Ascending Mesocolic H Absent lig. Treitz Normal Cecum Transverse colon not displaced caudally CT: Ascending Colon vessels anterior to SB loops

Desc Meso-colon H • • • • • • •

“Left PDH” Descending mesocolic H Ligament of Treitz OK Cecum OK Stomach displaced to right Neck contains IMVein & Left Colic Art. displaced anteriorly by hernia CT: IMV ant to SB loops

Desc Meso-colon Hernia [Figure 2-28-22]

• • • • •

Treatment with percutaneous draininage will allow elective surgery if the obstruction does not resolve

1. Ligament of Treitz OK 2. IMV in front of SB 3. SMA, V’s into hernia 4. Bunched SB possible If you want to diagnose a Left PDH, look for the IMV

Figure 2-28-21

Figure 2-28-22

Right and left meso-colic hernias, or “PDHs”, can be defined by the relationship of the right and left colic vessels to the proximal small bowel. Normally they pass behind the upper SB. Note the anterior course of the right colic vessels anterior to the SB (arrows) in a right “PDH”). Note the IMV ( (large pointers) in its normal position behind the SB on the left

A Left PDH or Ascending Meso-colic hernia with the Inferior Mesenteric Vein coursing anterior to the entrapped proximal small bowel Small Bowell Obstruction

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Gastrointestinal Radiology

Truth about “PDH”s [Figure 2-28-23] • •

PDH: Bad name. “Retro-mesocolic congenital hernia” ➢ Behind ascending, transverse, or descending mesocolon (and colon). ➢ Space usually obliterated as embryo ➢ Mesocolon fuses to parietal peritoneum. ➢ Rotation and fixation anomaly ❖ SB does not get out of the way ❖ SB prevents fusion

Figure 2-28-23

Foramen of Winslow • •

“Epiploic Foramen” SB above antrum

•

SBO ➢ Potential in Cecal Volvulus ➢ SB follows IC Valve

ascending

Cecal Volvulus into lesser sac Pre op DX: Infarcting Internal Hernia • •

Assoc w prior bowel surg ( Clips ) See ➢ Mesenteric: ❖ Bunching ❖ Engorgement ❖ Twisting ➢ Criss-crossed vessels

transverse

SB Obstruction • • • •

Common disease. Mechanical: Acute, Chronic, Internal, External Dysmotilities Critical ➢ Diagnose ➢ Stage ➢ Establish etiology

SBO •

The Paraduodenal Hernia will remain in our literature and our Board Examinations even though they are not hernias but errors of rotation in which the SB is trapped behind the returning meso-colon. Because there is a wide arc of returning colon, from the ascending, the transverse,and the descending colon, there will be variation in radiographic appearance depending on where the SB is trapped. Above one can see ascending, transverse, and descending retro-meso-colic entrapment

Reviewed ➢ Mechanical ❖ Classic Acute “Complete” SBO Simple SBO Closed Loop Obstruction (CLO) Urgent Emergency !!!!!!!! ❖ Classic Appearances Intermittent “Chronic” SBO Partial SBO ➢ Paralytic ❖ Common ❖ Unusual

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descending

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References 1. 2. 3. 4. 5. 6. 7. 8. 9.

10. 11. 12. 13. 14.

Balthazar et al Closed-loop & strangulating intestinal obstruction: CT signs. Radiology. 1992 Dec Blondon H, et al Digestive smooth muscle mitochondrial myopathy in pts with mitochondrial-neuro-gastrointestinal encephalomyopathy (MNGIE), 3 cases & review of literature. Gastroenterologie 2005 Aug. 29N, 89:773-778 Frager D, Medwid SW, et al . CT of SBO: Value in Establishing Diagnosis and Determining Degree and Cause.AJR 1994;162: 37-41. Ljiri R et al. Oburator H: usefulness of CT in DX.:Surg. 1996 Feb; 119(2): 137-40 Luedke, Scholz. Larsen CT of Spigelian H. Comp Med Imag Graph. 198812(2):123-9. Maglinte, Herlinger, Nolan. Rad of CLO: 25 confirmed cases. Radiology. 1991 May;179(2):383-7 (Chronic) Mayo-Smith WW, Wittenberg, et al. CT SB faeces sign: description & clinical significance. Clin Radiol. 1995 Nov;50(11):765-7. Megibow A, Balthazar E, Cho K, et al. Bowel Obstruction: evaluation with CT. Rad 1991;180:313-318. Passas V, Karavias D, Grilias D, Birbas A. Computed tomography of left paraduodenal hernia. J Comput Assist Tomogr. 1986 May-Jun;10(3):542-3. Sandrasegaran K, Maglinte DD, et al CT of acute bilio-pancreatic limb obst. AJR 2006 Jan;186(1):104-9 Scheirey C, Scholz F, et al. Radiology Lap Roux-Y Gastric Bypass: Conceptualization and Precise Interpretation Radiographics in press Sept 2006. Simon et al, Polyneuropathy, Ophthalmoplegia, Leukoencephalopathy, Pseudoobstruction: POLIP Syndrome; Ann Neurol 1990;28:349-360 Zalcman et al Helical CT Signs in Dx of Intestinal Ischemia in SBO; AJR 2000;175:1601-1607 Zalcman M, et al Helical CT signs in Dx of intestinal ischemia in SBO. AJR 2000 Dec;175(6):1601-7.

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Acute Mesenteric Ischemia Francis J. Scholz, MD Bowel Ischemia • • •

Small Bowel or “Mesenteric” ischemia ➢ SMA distribution: SB and Right Colon ”Colonic Ischemia” - a different disease ➢ Watershed: Sigmoid, Splenic ESD: never: unless ➢ Surgery ➢ Radiation ➢ Vasculitis

The Radiology of Mesenteric Ischemia • • •

1. Review classifications & pathophysiology 2. Rad Findings: Ischemia & Infarction 3. Clues to Etiology, emphasis on CT

Acute Mesenteric Ischemia 3 categories

➢ Arterial Occlusive ❖ Without Reperfusion ❖ With Reperfusion ➢ Venous Occlusive ➢ Non-Occlusive Arterial - Low Flow

Pathophysiology of Ischemia • • • •

•

Initial Damage to Endothelial cells of pre-capillary arteriole and capillaries. Blood vessels leak fluid, then cells Mucosa & submucosa ➢ Most sensitive, high metabolism ➢ Edema, hemorrhage, & slough Muscularis propia ➢ Initial spasm ➢ Then atonia ➢ Then perforation / death (healing with stricture) Serosa ➢ Petechiae, Ascites ➢ With healing may see adhesions

Ischemia •

Rad Findings, Symptoms & Prognosis depend on: ➢ Duration ❖ Momentary to Permanent ➢ Degree ❖ 1%-100% ➢ Extent ❖ % of SB

Fast Ischemia •

Cell, Tissue, Organ & Organism death -24-48 H ➢ Eg. Embolus to SMA ➢ Eg. Hypotension: Profound & prolonged

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Slow or Minimal Ischemia • •

Cellular & localized tissue death → Organ dysfunction ➢ Eg Radiation Enteritis ➢ Eg Scleroderma ➢ Eg Arteriosclerotic Abdominal Angina

Ischemia • • •

Chronic - recurrent - slow Acute - sudden - fast Often both ➢ Chronic for months then Acute

Wet vs Dry Ischemia • •

Wet: Ischemia w arterial inflow p insult (“reperfusion”) ➢ SEE: Thickest wall, bleeding into wall, ascites. ➢ Eg: Venous occlusion, Transient hypotension, fleeting, partial embolism Dry: Ischemia w no arterial inflow. (“non-reperfused”) ➢ SEE: “Thinner” or normal wall, no / min ascites. ➢ Eg. Complete proximal SMA embolus, sudden thrombosis.

Chou C, CT Manifestations of Bowel Ischemia. AJR2002;178-87 Chou C, CT of SB ischemia. Abd Imaging 2004; 29:18-22

Wet vs Dry Ischemia : Personal experience • • •

Two extremes: Prune vs Plum Wet: Classic ➢ Radiologists overcall on CT ➢ Surgeons undercall at Surgery Dry: Puzzling SBO ➢ Radiologists undercall - miss completely ➢ Surgeons baffled by our stupidity (SECRET: Study Mesenteric Vessels I+)

Most Specific Single Finding of Acute Mesenteric Ischemia [Figure 2-29-1]

• • •

No CT perfusion of bowel wall 100% specific, 30%-50% sensitive Diagnosis depends on ➢ History ➢ Summation of findings ❖ Wall thickening ❖ Mesenteric Edema ❖ Ascites

Figure 2-29-1

Absence of wall opacification is the most specific sign of wall ischemia. It is not sensitive in all cases. Because of the length of the SB, small segments that are not perfusing may not be evident. In this image one segment is perfusing while another loop is not

Acute Mesenteric Ischemia • • •

WBC Elevated Lactic Acid History ➢ Suggestive History: ❖ Pain in excess of Physical Exam ➢ Risk Factors

High Risk Patients (Boley, Clark) • • • • • • •

Pt > 50 yrs with: Valvular or Atherosclerotic Ht Dis Longstanding CHF Arrythymia Hypovolemia or hypotension Dig or diuretic Rx Recent MI

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Gastrointestinal Radiology

• •

Also: AAA w or wo repair Also: Any Abd, Cardiac, Thoracic Surg

•

Tendency to: ➢ Thinner Wall ➢ Absent “Target” ➢ No intramural blood ➢ Ascites min /absent ➢ No perfusion Beware: “Ileus or SBO” in Sick Pt at high risk

Figure 2-29-2

Dry Infarct [Figure 2-29-2]

•

Dry Infarct, Dead Bowel - CLO SBO

Spectrum: Ischemia to Infarction [Figure 2-29-3] • • •

• • • • • • • • • • •

Gasless abdomen Ileus Thick Folds ➢ Target - CT, US ➢ Stack of Coins - Films Loss of Folds in Unchanging Thick-walled Loop Focal ulcer Shaggy gas pattern Collar button ulcers Intramural fistulas Intralum mucosal cast Mesenteric or portal vein gas Intraperitoneal air Stricture Pseudodiverticulum Many findings possible in same pt

Four sequential images (A-D) of a patient with infarction WITHOUT REPERFUSION. Thin walls without target sign or intramural blood. No ascitic fluid. Good example of a dry infarct with minimal or absent inflow of arterial blood. Dry infarcts may be due to a large central SMA embolus or smaller embolic or thrombi with lack of adequate collaterals to allow inflow. This form of ischemia and infarction is less common and is hard to diagnose unless mesenteric vessel contrast is studied carefully Usually there is REPERFUSION or extensive inflow via collaterals producing edema or hemorrhage into the bowel wall and ascitic fluid

Remember the Law of Burps & Farts [Figure 2-29-4]

•

Air rises & thins normal walls (Also note engorged mesentery)

Figure 2-29-4

Figure 2-29-3

With reperfusion ischemia the wall thickens with fluid and blood from leaking capillaries. Stack of coin appearance is due to fluid and blood in the valvulae conniventes Gastrointestinal Radiology

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Image shows normal thin wall effaced by air (arrows) while arrowheads show thick wall not effaced by air. A loop with a target sign is also noted (curved arrow). The slightest amount of SB (or colon) air will rise and thin wall of normal bowel. Walls thickened by blood or tumor will not thin out. Understanding this concept allows for easy detection of abnormal bowel

Mesenteric Ischemia

The Law of Bowel Gas ll [Figure 2-29-5] • •

Figure 2-29-5

Air in lumen coalesces into one bubble.(unless trapped in pneumatosis, blood, stool, or bezoar). Note dense blood layering

Regular Stack [Figure 2-29-6] • •

•

Blood / Edema in Wall Suggests: ➢ Acute ➢ Recent ➢ Severe Not specific for ischemia

Irregular Stack [Figure 2-29-7] • •

Blood / Edema in wall Suggests: ➢ Chronicity ➢ Recurrence ➢ Fibrosis

Images show non-coalescent air bubble (small arrow) suggesting either pneumatosis or air trapped in viscous blood. There is a low density / high density fluid-fluid level (large arrow) indicating bleeding into lumen

Loss of Folds [Figure 2-29-8]

Figure 2-29-7

Figure 2-29-6

An irregular stack of coins suggests re-bleeding and chronicity with fibrosis

Figure 2-29-8

A regular stack of coins with relatively uniform appearance of folds is suggestive of recent bleeding into wall. A stack of coins appearance can be due to blood or fluid. It is a longitudinal image of cross sectional “target sign”. Pts with coagulopathies or with leaking capillaries due to vasculitis will have a similar appearance

Mesenteric Ischemia

Loss of folds in an unchanging loop is an ominous sign for infarction. It indicates extreme wall thickening and loss of peristalsis 490

Gastrointestinal Radiology

Thick Wall & Ulcers [Figure 2-29-9]

Figure 2-29-9

Ischemic Pneumatosis [Figure 2-29-10]

Intramural and Intravenous Air [Figure 2-29-

11]

V Air & Lack of Wall Perfusion [Figure 2-29-12]

CT Equivalent to stack of coins with arrows pointing to collections of contrast in the wall suggesting loss of mucosal integrity

Figure 2-29-11

Figure 2-29-10

Image A: Intramural and intravenous air (arrows), evidence of a degree of bowel infarction. The degree of infarction is difficult to determine and may not correlate with amount of air. A small focus of wall infarction may allow a large amount of air to enter veins. Once air enters a vein it may travel long distances. When SB obstruction is present air will be under pressure and large amounts may enter veins through even tiny foci of loss of mucosal integrity. With even the severest mesenteric ischemia and infarction, the unaffected stomach muscles will continue to peristalse fluid and air into the SB creating SB distension

Two spot films(images A & B) from a water soluble contrast ileostomy enema shows multiple ulcerations filling with contrast or with air. The CT scan slice (image C) shows pneumatosis

Figure 2-29-12

Look carefully for AIR in VESSELS USE lung windows in Ischemic and in SBO cases to look for venous air. Use narrow windows to find subtle wall density changes indicating either blood or lack of perfusion Gastrointestinal Radiology

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Mesenteric Ischemia

Extreme Perfusion Variations [Figure 2-29-13]

Intrahepatic Portal Venous Air [Figure 2-29-14] •

Splanchnic air will go everywhere splanchnic. It doesn’t stay near its origin

Figure 2-29-13

Figure 2-29-14

Loops show EXTREME variations in wall perfusion from absent to HYPERperfusion. This pt with infarction and perforation from vasculitis. Pt is being treated for hypotension and has dense enhancement from “shock bowel” in unaffected loops not involved with vasculitis

Air is seen in portal branches and within veins in the otherwise normal stomach. (Arrow) Air in portal system may distribute anywhere within portal circulation by gravity. In prone position, air in intra-splenic veins would likely be seen

Figure 2-29-15

Sloughed Mucosa / Serosa [Figure 2-29-15] Mucosal Cast [Figure 2-29-16] •

Inside Serosa

• • • •

SBO makes isch / Infarxn look worse We overestimate Infarction Edema greater Air dissects great distances under pressure: ➢ Neck crepitus reported Pneumatosis not = Infarxn

SBO Pearl [Figure 2-29-17]

•

Figure 2-29-16

The mucosa (long arrows) is seen sloughed in lumen outlined by contrast reaching to smooth as yet intact serosa (short arrows) With infarction and diffuse loss of mucosal integrity, there will be slough of mucosa. The serosa is more resistant to ischemia (tough as hot dog or sausage skins) and will be last to perforate

Two CT images, A & B, show shaggy irregular intramural air, a sign of a mucosal cast, a sloughing of mucosa

Mesenteric Ischemia

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Gastrointestinal Radiology

Pneumatosis Intestinalis (PI) • •

•

•

Figure 2-29-17

Venous gas … not allow prediction of transmural infarction, … observed with only partial … wall damage Outcome ..pneumatosis… depends … on underlying disease ➢ Wiesner W, et al . PI and portomesenteric venous gas in intestinal ischemia: correlation of CT with severity of ischemia and clinical outcome. Am J Roentgenol. 2001 Dec;177(6):1319-23. CT Dx of PI: Lactic Acid > 2.0 mmol/L at Dx assoc with > 80 % mortality ➢ Hawn MT, et al Serum lactic acid determines outcomes of CT Dx of pneumatosis of GI tract. Am Surg. 2004 Jan;70(1):1923; AIR GOES EVERYWHERE…LOOKS WORSE THAN IT IS

Ischemia Mimic: J Tube Jejunitis •

•

•

•

Rad Findings: ➢ Jejunal Edema ➢ Ascites ➢ Portal & SMV Gas ➢ Pneumatosis ➢ SB Necrosis Clinical: ➢ RARE, 4 / 1460 pts - .021% ➢ J Tube Abd or ENT surg ➢ Post op feeding day 1 ➢ Day 5 bloating, N & V ➢ Day 7 hypotension - death. ➢ “They suddenly crump” CT Findings ➢ Portal Venous Gas ➢ SMV Gas ➢ Pneumatosis ➢ Penrose, J Tubes OP: ➢ Normal Bowel, pink, no resection

Two slices from a pt show a shaggy irregular mucosal cast (image A) indicating mucosal slough (long arrow). Other loops of bowel have extensive pneumatosis (image B). The length of bowel involved with pneumatosis may greatly exceed amount of bowel that is infarcted because air travels long distance in veins and in areolar submucosal tissue

Rare… but recognize early •

Carucci LR, Levine MS, Rubesin SE, Laufer I, Assad S, Herlinger H. Evaluation of pts with jejunostomy tubes: imaging findings. Radiology. 2002 Apr;223(1):241-7

J Tube Jejunitis •

•

•

•

•

•

•

•

Schunn CD, Daly JM. SB necrosis associated w post-op jejunal tube feeding. J Am Coll Surg. 1995 Apr;180(4):410-6. Lawlor DK, et al SB necrosis assoc w jejunal tube feeding. Can J Surg. 1998 Dec;41(6):459-62. Rai J, et al SB necrosis in assoc w jejunostomy tube feedings. Am Surg. 1996 Dec;62(12):1050-4. Munshi IA, et al.SB necrosis assoc with early post-op jejunal tube feeding in a trauma pt.J Trauma. 2000 Jul;49(1):163-5. Schloerb PR, et al. Bowel necrosis caused by water in jejunal feeding. J Parenter Enteral Nutr. 2004 Jan-Feb;28(1):27-9. Brenner DW, Schellhammer PF. Mortality assoc w feeding catheter jejunostomy after radical cystectomy. Urology. 1987 Oct;30(4):337-40. Gaddy MC, et al . SB ischemia: consequence of feeding jejunostomy? South Med J. 1986 Feb;79(2):180-2. Jorba R, et al. SB necrosis in assoc w early post-op enteral feeding after pancreatic resection. Surgery. 2000 Jul;128(1):111-2.

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“Luxury” Reperfusion Hyperemia [Figure 2-29-18] •

Figure 2-29-18

Vessels lose auto-regulation ➢ Tiniest vessels in mucosa ❖ Dead, clotted, ❖ Mucosa non-perfused ➢ Bigger vessels ❖ Lose muscular tone ❖ Hyperemic bowel musculature ❖ Shunt to veins

Reperfusion Bleeding [Figure 2-29-19]

Ischemia may cause strictures [Figure 2-29-20]

Figure 2-29-19

Luxury Reperfusion with gray mucosa and hyperemic muscularis and mesentery

Intra-luminal bleeding may be obscured or overlooked if pt is given opaque contrast material. This pt had intraluminal bleeding from diffuse vasculitis of Degos Syndrome initially assumed to be ingested contrast. The pt did not drink opaque material! Always look carefully at the density of ascites. High density ascites indicates bleeding into peritoneal cavity

Figure 2-29-20

A SB spot film (A) and a CT slice (B) show a tapered stricture (arrows) and a long tubular stricture (arrowheads). With recovery from a severe ischemic insult, healing with permanent stricturing can occur Mesenteric Ischemia

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Gastrointestinal Radiology

Etiologies of Ischemia

• •

Arterial Occlusive ➢ Emboli 40%-50% ➢ Atherosclerosis - Thrombosis 10%-20% ➢ Mechanical ❖ Closed Loop Obstruction ❖ Volvulus, ❖ Incarceration ❖ Avulsion ➢ Large and Small Vessel Vasculitides Venous Occlusive Arterial Non-Occlusive

• • • •

Atrial Fibrillation Valvular heart disease Sharp cut off Filling defect

•

Figure 2-29-21

Embolus [Figure 2-29-21]

Lateral aortogram shows a filling defect (arrow) in SMA, a sign of embolus. AP injection of SMA in another pt. shows long filling defect of embolic clot (two arrows)

Embolism [Figure 2-29-22]

Figure 2-29-22

White SMA (short arrows) Then Gray SMA (long arrows) ANGIO shows cutoff and clot. Dead bowel at surgery within 8 hrs of symptoms. Pt died 16 hrs after symptoms Gastrointestinal Radiology

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Mesenteric Ischemia

Figure 2-29-23

Figure 2-29-24

Straddle embolus vs. mural thrombus extending into SMA Reperfusion by collaterals distally Slow filling of an irregular faint SMA (arrow) usually indicative of atherosclerotic thrombotic disease

Figure 2-29-25

Figure 2-29-26

Non-opacification of the SMA proximally with contrast in SMA distally indicating distal perfusion by collaterals, confirmed with a coronal reconstruction

A small segment of distal SB shows stack of coins appearance (arrows) on UGI series ( Image A). An angiogram (Image B) shows tapered narrowing of small peripheral branch of SMA (arrow). A careful SB series or enteroclysis may be needed to diagnose short segment disease caused by segmental dis. in peripheral SMA branches

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Gastrointestinal Radiology

“Straddler”: Embolus Vs Clot [Figure 2-29-23]

Figure 2-29-27

Thrombosis [Figures 2-29-24 and 2-29-25] • • • • • •

Absent segment Slow filling distal vessel Large collaterals Reconstitution Vascular calcification Irregular lumen

•

Thrombosis vs. Embolus appearance can be similar, Hx AF or Ht Valve important

SMA flow thru GD collaterals

Peripheral focal lesion [Figure 2-29-26] Ulcerating Plaque [Figure 2-29-27] Dysplasia [Figure 2-29-28]

A SB series (Image A) shows a stack of coins appearance (within circle). An angiogram (Image B) shows a bulging segment of SMA (arrow), an ulcerating plaque showering cholesterol emboli. (This could also be a mycotic aneurysm if patient were septic.)

Becker Duodenal necrosis as presenting manifestation of polyarteritis nodosa. Clin Rheumatol. 2002 Aug;21(4):314-6 Chronic Radiation Enteritis

Degos Disease • •

• • • • •

“Malignant atrophic papulosis” Porcelain-white, atrophic papules ➢ Peripheral erythema ➢ Telangiectases. Small vessel thromboses M3 X F, all ages Skin presentation May rarely remain dermal When systemic, 2-4 yr prognosis

Coskun B Benign Cutaneous Degos' Disease: case report and review of literature. J Dermatol. 2004 Aug;31(8):666-70

Degos GI Path • • •

GI - Any portion, but SB predominant 60% Degos: GI perfs lead to death Sub-serosal white or yellow plaque, transmural bowel inflam., ulcers, hemorrhage, infarction

Figure 2-29-28

Etiologies of Ischemia •

Arterial Occlusive

• •

Atherosclerosis

➢ Embolus Venous Occlusive 5%-10% ➢ Proximal Obstruction ➢ Distal Disease Arterial Non-Occlusive ➢

Etiology SMV Thrombosis • • • • • • •

Idiopathic 20% Recent Surgery, esp Colon Hypercoagulable States ➢ Protein S, C defic, polycythemia, hematological Cirrhosis Portal Vein Thrombosis Pancreatic Inflamm / Neoplasm Pelvic Infectious Processes

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An SMA angiogram (Image A) shows an irregular abrupt tapered narrowing of SMA branch vessel (arrowhead), consistent with dysplasia. There is also subtle corrugation of main trunk and small branches within circle. There is a clip (arrow) where right renal artery, resected previously for renal artery dysplasia, originated Mesenteric Ischemia

Symptoms SMV Thrombosis [Figure 2-29-29] • • • • • •

Figure 2-29-29

Duration of symptoms ➢ 9.1 days, range 1-42 d Pain 84%, N & V 56%, Fever & Chills 56% Diarrhea 23%, Blood in Stools 23% Ischemia 21% ➢ Bowel Wall Thickening ➢ Mesenteric Congestion Mortality in 7%, (rapidly falling in MDCT era) (MDCT: Incr detection & Rx)

Warshauer DM, Lee JKT, Mauro MA, White GC; Superior Mesenteric Vein Thrombosis w Radiologically Occult Cause: Retrospective Study of 43 Cases; AJR2001;177:837-841 Images A-C: CT images show thrombus (arrows) in Portal Vein. Images D-F Images of mid SB Infarction [Figure 2-29-30] abdomen show engorged mesenteric SB leaves • Lymphoma of SB Mesentery and thickening of SB loops (arrows) consistent • Nodes Compress Veins with ischemia. While wall and mesenteric • Engorged Mesentery thickening may mimic Closed Loop SBO, absence • Infarction / Slough of bunching, lack of bowel distension, and preservation of transit distinguish the two.In Etiologies of Ischemia absence of ongoing thrombotic coagulopathy, • Arterial Occlusive venous thromboses often eventually resolve with ➢ Atherosclerosis or without therapy ➢ Embolus • Venous Occlusive Figure 2-29-30 • Arterial Non-Occlusive ➢ Low Flow States ❖ Shock ❖ Steals ❖ Arterial Vasospasm ❖ SBO

Figure 2-29-31

Aortogram shows lush perfusion and opacification of even small peripheral branches of upper abdomen vessels with minimal mesenteric vasculature apparent. There is no IMA visible. The main SMA trunk stops abruptly (arrowhead). An embolus is possible but absence of visible filling defect in SMA and extensive vascular disease makes thrombosis more likely Mesenteric Ischemia

Images A-C show bulky nodes in the SB mesentery (straight arrow). The mesentery is engorged (arrowhead) indicating compression of mesenteric vessels. Shaggy irregular intramural air (curved arrows) indicates infarction with mucosal slough. Compression of SB veins may lead to venous engorgement and bowel infarction 498

Gastrointestinal Radiology

Abdominal Angina, a Clinical Syndrome • • • • •

Figure 2-29-32

1. Pain following eating 2. Weight loss 3. Diarrhea, rapid transit “Classic” ➢ Occlusion of 2 of 3: Celiac, SMA, IMA ➢ May be 1 vessel occlusion, part others ➢ May be absent with full 3 vessel occlusion Vasculitis, Radiation, Median Arcuate Ligament Syndrome, Steal Syndromes, CA Pancreas

Vasculopath “Blood Thievery”[Figure 2-29-31] • • •

Lush vasculature in upper abdomen Abrupt SMA end. Paucity lower abdomen

Vasculopath Collateral Steal from SMA [Figure 2-29-32]

• •

See steal from SMA to SMA, IMA and beyond Reflex Arterial Vasospasm in SMA

Three images from SMA injection (A-C) show abrupt termination (arrow) and collateral flow filling Abdominal Angina: Median Arcuate an enlarged marginal arcade vessel (arrowheads) Ligament and filling of IMA branches (curved arrow). [Figure 2-29-33] Chronic steal syndromes in vasculopaths have • Median Arcuate Ligament of diaphragm variations in amount stolen depending on varying • Compression / fibrosis of Celiac Artery (occ SMA too) demand. Walking may deplete visceral flow and • Collateral Steal from SMA produce Reflex Arterial Vasopasm and abdominal angina

Figure 2-29-33

Figure 2-29-34

Image A in a pt with chronic intermittent abdominal pain shows a stack of coins appearance to the jejunum. Image B and a detail from it, image C, shows a short segment narrowing of Celiac Axis with a normal SMA just caudal to it. The appearance allows diagnosis of median arcuate ligament syndrome. The median arcuate ligament of diaphragm may compress Celiac Axis. This forces a physiologic steal from SMA which may be asymptomatic when bowel is at rest. Following eating, classic abdominal angina may occur because steal creates a functional mesenteric ischemia

Expiration / Inspiration

Median Arcuate Ligament Collateral Steal from SMA [Figure 2-29-34] •

•

Large collaterals ➢ SMA -> Celiac Reflex Mesenteric Vasoconstriction

Gastrointestinal Radiology

499

The AP projection during an SMA injection shows collateral filling of Celiac vessels and reflex mesenteric vasoconstriction of mid and distal branches of SMA, placing them at risk of thrombosis. Celiac artery occlusion may be cause of mesenteric angina, ischemia, or infarction due to collateral steal

Mesenteric Ischemia

Reflex Mesenteric Vasoconstriction

Figure 2-29-35

Shock Bowel •

• • • • • • •

Dense persistent enhancement: ➢ Bowel Wall, solid organs Delayed pyelogram Small aorta, IVC, Spleen Ascites Variable Distension - Wall Thickness Periportal extravasation of fluid Major trauma with ➢ Resuscitation ➢ Volume repletion 2° to Reflex vasoconstriction

Mirvis SE, et al Diffuse SB ischemia in hypotensive adults after blunt trauma (shock bowel): CT findings & clinical significance. AJR Am J Roentgenol. 1994 Dec;163(6):1375-9

Shock Bowel •

•

May develop: ➢ Ischemia ➢ Infarction SB = Low Flow State usually caught in time

CT images (A-F) show wall thickening with fluid density in pt with angioedema, a process where capillaries leak serum. It may be due to allergies to food, drugs, or other exogenous allergens. A hereditary form occurs without specific causation. Angiotensin Converting Enzyme inhibitor drugs may produce this finding alone or in association with glottic or generalized edema. It may be dose related or seen with certain ACE inhibitors. Those with bowel angioedema from ACE inhibitors may present with a rad and clin picture suggesting mesenteric ischemia. Pts on hypertensive or cardiac medications should be questioned about antihypertensive medication to exclude this as an etiology. Cessation of offending ACE inhibitor may provide relief and rad return to normal within 24 to 48 hrs

Ischemia Mimic: Angioedema [Figure 2-29-35]

• • • • •

Enhancement of mucosa Submucosa edema Fluid in lumen Ascites Etio ➢ Allergic reaction, ➢ Hereditary, ➢ ACE inhibitors.

DeBacker AI, et al; CT of Angioedema of the Small Bowel, AJR 2001; 176: 649-52 3 cases: 3 different etiologies 1 case report of 1 pt 1 NEJM Images in Clinical Medicine

Ischemia Mimic: Angioedema Serum Leak from Capillaries •

ACE Inhibitors ➢ Accupril ➢ Aceon ➢ Altace ➢ Capoten ➢ Captopril ➢ Lisinopril ➢ Lotensin ➢ Mavik ➢ Monopril ➢ Prinivil ➢ Univasc ➢ Vasotec ➢ Zestril

Mesenteric Ischemia

500

Gastrointestinal Radiology

Ischemia Mimic: ACE I A E • • • •

• •

7 Female 1 Male Ascites > Bowel Change Preserved Transit ACE I stopped: ➢ < 24 Hr resolution Bowel changes mild enough to wait / watch, avoid surgery!! Stiff Arcs in 6 of 8 pts

Figure 2-29-36

Lahey experience

Ischemia Mimic: Stiff Arc Sign • •

• •

Serum leaks from capillaries Intact ➢ Arteries ➢ Capillaries ➢ Veins ➢ Blood Flow ➢ Oxygenation Wall stiffest per degree of thickness Long Arcs of erect SB possible

Ischemia Mimic: Edema Post Obstructive 9 Days Earlier: SBO & LBO Diverticulitis

Ischemia Mimic: Obstructive & Post Obst Edema [Figure 2-29-36]

• • • •

Drop in high IL pressure Local Arteriolar Hypertension & Increase Vasc Permeability = Tissue Edema

• •

Some CT features overlap: target sign, hemoperitoneum Intramural Hemorrhage: ➢ Short segment < 15 cm ➢ Wall thicker = or > 1 cm Ischemia: Long > 30 cm ➢ Wall less thick < 1 cm 15 -30 cm overlap

Ischemia Mimic: Hemorrhage vs Ischemia [Figure 2-29-37]

• • •

Note thumbprinting of colon and stack of coins appearance of SB. Chronic high intra-luminal pressure will affect the hemodynamics of perfusion. Submucosal edema will occur with severe obstruction. When obstruction is relieved, the edema and altered perfusion dynamics may persist, and the edema may become more prominent immediately after relief of obstruction

Figure 2-29-37

Macari M, et al Intestinal ischemia versus intramural hemorrhage: CT evaluation. AJR. 2003 Jan;180(1):177-84

Ischemia Mimic: ITP • • • • • •

•

Immune Thrombocytopenic Purpura Bowel Hemorrhage Note: post splenectomy Ascites 2 groups: Age 2-4, Adult Causes ➢ Idiopathic ➢ Drug Induced ➢ SLE ➢ Infection ➢ Pregnancy Rx ➢ Immune Suppression ➢ Splenectomy

Gastrointestinal Radiology

A regular stack of coins with relatively uniform appearance of folds is suggestive of recent bleeding into wall. A stack of coins appearance can be due to blood or fluid. Pts with coagulopathies or with leaking capillaries due to vasculitis will have a similar appearance

501

Mesenteric Ischemia

Figure 2-29-38

Purpuras are a group of diseases that weep small amounts of blood from many tiny vessels. Henoch Schonlein is transient, often recurring immune mediated vasculitis, usually affecting children, can be seen in adults. Usually palpably raised itchy red lesions (Arrows Image A) are present and allow a diagnosis. Petechiae (arrows in image B of ileal endoscopy) and purpuric lesions also occur in bowel. Abd involvement is seen in 50-75% of pts who present with dramatic colicky abd pain and bleeding, which may be massive in 1-2% of patients. Bleeding into wall of bowel thickens it (Image C arrows) and gives a stack of coins appearance (Image D arrows). This intramural bleeding may cause obstruction, GI bleeding, infarction, perforation, or intussusception in distal SB. While no effective therapy, pts must be monitored for complications until attack subsides

Figure 2-29-39

The descriptive term “target sign” has been applied to a loop of bowel with distinct demarcation of circular layers of the wall. It is seen whenever fluid of some type, or fat, or air enter submucosal space between mucosa - muscularis mucosa and muscularis propria-serosa. Image A innermost gray is fluid in lumen. Then there is a white line representing contrast opacified mucosa and muscularis mucosa. Then there is a gray circle which represents fluid in submucosa. The outermost white ring represents contrast opacified muscularis propria and serosa Image B, the innermost white dot is ingested opaque contrast material. Then a less opaque ring represents mucosa and mucosal muscle. There is a low density ring which is negative in Hounsfield units indicating fat. The outermost layer is muscularis propria-serosa. Fat prominence is seen in pts with Crohns disease and for unknown reasons may be seen in occasional normal pts in the distal Ileum, possibly due to prior infectious gastroenteritis Mesenteric Ischemia

502

Gastrointestinal Radiology

Ischemia Mimic: Henoch Schonlein Purpura [Figure 2-29-38]

Target sign [Figure 2-29-39] • • • • • • • • •

Blood, Serum, Plasma, Interstitial Fluid, Fat, Air Ischemia Vasculitis Intramural Hemorrhage Crohns: edema (or fat) Angioedema Portal Hypertension NSAIDs Enteritis ANY ENTERITIS ➢ Chemo, Rad, Infect. etc

Bowel Damage Pathways •

Loss of Barrier Integrity ➢ Vascular Barrier ❖ Leak of serum, plasma, cells ❖ Edema ❖ Ischemia ❖ Loss of mucosal barrier ➢ Mucosal Barrier ❖ Inflow of excluded molecules ❖ Edema ❖ Loss of vascular barrier ❖ Vascular compromise ❖ Ischemia

Ischemia CT Mimics •

Vascular or Mucosal Barrier Interruption ➢ Ischemia ➢ Vasculitides HSP ➢ Coagulopathies ❖ Bleeders - Purpuras, anticoags ❖ Clotters - P Vera, S,C defic ➢ Angioedema - ACE inhibitors ➢ Regional Inflammation - tic appy itis ➢ Crohns ➢ Infectious Enteritis ➢ Neutropenic Enterocolitis

Mesenteric Ischemia •

• • • •

Diagnosis ➢ Imperative in Acute & Chronic Ischemias ➢ Now earlier Dx by CT - study vessels ➢ Think of it in every abd pain CT. Physiological understanding is critical Remember Steals Surgeons undercall some, be brave, stay bold We undercall some, explain plums & prunes

References

General References 1. Becker Duodenal necrosis as presenting manifestation of polyarteritis nodosa. Clin Rheumatol. 2002 Aug; 21(4):314-6. 2. Carucci LR, Levine MS, Rubesin SE, Laufer I, Assad S, Herlinger H. Evaluation of pts with jejunostomy tubes: imaging findings. Radiology. 2002 Apr; 223(1): 241-7. 3. Coskun B. Benign Cutaneous Degos' Disease: case report and review of literature. J Dermatol. 2004 Aug;31(8):666-70 Gastrointestinal Radiology

503

Mesenteric Ischemia

4. 5. 6.

DeBacker AI, et al; CT of Angioedema of the Small Bowel, AJR 2001; 176: 649-52 Mirvis SE, et al Diffuse SB ischemia in hypotensive adults after blunt trauma (shock bowel): CT findings & clinical significance. AJR Am J Roentgenol. 1994 Dec; 163(6):1375-9. Warshauer DM, Lee JKT, Mauro MA, White GC; Superior Mesenteric Vein Thrombosis w Radiologically Occult Cause: Retrospective Study of 43 Cases; AJR 2001;177:837-841

Wet vs Dry Ischemia 1. Chou C, CT Manifestations of Bowel Ischemia. AJR2002;178-87 2. Chou C, CT of SB ischemia. Abd Imaging 2004; 29:18-22

Pneumatosis Intestinalis (PI) 1. Hawn MT, et al Serum lactic acid determines outcomes of CT Dx of pneumatosis of GI tract. Am Surg. 2004 Jan;70(1):19-23; 2. Wiesner W, et al . PI and portomesenteric venous gas in intestinal ischemia: correlation of CT with severity of ischemia and clinical outcome. Am J Roentgenol. 2001 Dec;177(6):1319-23.

J Tube Jejunitis 1. Brenner DW, Schellhammer PF. Mortality assoc w feeding catheter jejunostomy after radical cystectomy. Urology. 1987 Oct;30(4):337-40. 2. Gaddy MC et al. SB ischemia: consequence of feeding jejunostomy? South Med J. 1986 Feb; 79(2):180-2. 3. Jorba R, et al. SB necrosis in assoc w early post-op enteral feeding after pancreatic resection. Surgery. 2000 Jul;128(1):111-2. 4. Lawlor DK, et al SB necrosis assoc w jejunal tube feeding. Can J Surg. 1998 Dec; 41(6):459-62. 5. Munshi IA, et al.SB necrosis assoc with early post-op jejunal tube feeding in a trauma pt.J Trauma. 2000 Jul; 49(1):163-5. 6. Rai J, et al SB necrosis in assoc w jejunostomy tube feedings. Am Surg. 1996 Dec; 62(12):1050-4. 7. Schloerb PR, et al. Bowel necrosis caused by water in jejunal feeding. J Parenter Enteral Nutr. 2004 JanFeb;28(1):27-9. 8. Schunn CD, Daly JM. SB necrosis associated w post-op jejunal tube feeding. J Am Coll Surg. 1995 Apr; 180(4):410-6.

Mesenteric Ischemia

504

Gastrointestinal Radiology

Malabsorption

Francis J. Scholz, MD

The Radiology of Malabsorption (MAB) •

Review ➢ Celiac Disease “Sprue” in detail ➢ “MAB Pattern” Barium & CT ➢ Other Diseases of MAB ➢ CT Detection of MAB

Images from a Virtual Colonoscopy 75 F Asymptomatic Celiac Disease •

Vessel cloaking nodes, fluid in pelvic SB loops

• • • • • • •

Diarrhea 85% Weight loss 57% Abd distress 29% Edema 29% Bone pain 19% Tetany 10% Failure to grow, hematuria, foot drop, hypovolemic shock, each 2%

1991: Sprue Presentations

Trier J, Celiac Sprue NEJM 1991

2005: Sprue Presentations •

•

50% of adult pts present w Fe Defic Anemia ➢ Farrell RJ, Kelly CP. Celiac Sprue.N Engl J Med. 2002 Jan 17;346(3):1808 “Occult GI Bleeding (FOBT+)…detected in half of pts with Sprue” ➢ Fine KD, Prevalence of occult GI bleeding in Celiac Sprue, NEJM 1996 334:1163-7

Back & Leg Pain • • • •

-> Primary Care MD -> Neurologist - back pelvis films -> Radiologist - “Osteomalacia” -> ➢ GI series -> Gastroenterologist: Biopsy: Sprue

The Physiologist’s MAB • • •

Maldigestion (no enzymes, no mixing) ➢ Biliary - panc insuff, ZE, bacterial overgrowth, SB diverticulosis “Luminal” Cellular MAB (Columnar Cell uptake failure) ➢ Sprue, ischemia, villous tip infiltration Malassimilation (Columnar Cell exit failure) ➢ lymphangiectasia, abetalipoproteinemia, mesenteric diseases “Mesenteric”

The Radiologist’s MAB: “Malabsorption Pattern” •

• • •

Dilution from XS intraluminal fluid ➢ Acute & Chronic Diseases Dilatation Delay “MP” → to enteric fluid overload, greater chronicity: > Dilatation, Delay

Gastrointestinal Radiology

505

Malabsorption

“Malabsorption Pattern” (MP) •

• • • •

Figure 2-30-1

“MP” = Dilution + Dilatation + Delay. ➢ Due to chronic enteric fluid overload Historically, radiologic MAB Pattern = Sprue Sprue is king of MAB pattern BUT Other diseases can cause MAB pattern Not all Sprue pts have “MAB pattern”

Sprue: Gold Standard Dx • •

• •

SB Biopsy Antiendomysial antibody (“EMA”) ➢ IgA Ab to extracellular reticular fibers ➢ 90% sensitive, 98% specific Tissue Transglutaminase antibody (tTGab) ➢ 86% sensitive; 84% specific AntiGliadin IGA antibody ➢ 76% sensitive, 79% specific

Image shows two jejunal biopsies, of similar magnification, contrasting Celiac Sprue at top with a normal biopsy at bottom. With Celiac Sprue there is loss of normal fingerlike villi (arrows) seen below and Crypt Hyperplasia (compare thickness of double arrowheads)

Johnston SD, et al A comparison of antibodies to tissue transglutaminase with conventional serological tests in the diagnosis of coeliac disease. Eur J Gastroenterol Hepatol. 2003 Sep;15(9):1001-4.

Figure 2-30-2

Villous Atrophy & Crypt Hyperplasia Normal Villi & Crypts [Figure 2-30-1] Entero-Enteric Circulation

• • • •

Crypts secrete fluid into lumen Villi absorb fluid + nutrients from lumen Nutrients into portal veins, lymphatics Crypts recycle fluid back into lumen

• • • •

Mucosal Villous Atrophy + Crypt hypertrophy → Chronic Fluid Overload → Dilatation (SB Muscle exhausted- “CGF”) → Delay in Transit –> ➢ Increases Malabsorption ❖ Bacterial overgrowth ❖ Other Degradations

Sprue – Pathophysiologic Sequence

Sprue 1° Rad findings l [Figure 2-30-2] • • • • • •

Dilution WET !! Dilatation WIDE !! Delay in transit WAY LATE !! Segmentation Folds: normal –> nodular –> flat MALABSORPTION PATTERN

Jejunal Peristalsis “Feathery Fishtails” Ileal Peristalsis “Esophageal”

Figure 2-30-3

The “Malabsorption Pattern” is characterized by Dilution evident with watery low density of barium (arrow) caused by fluid mixing with it, Dilatation evident by wide diameter (double arrow head) and Delay evident by a 7 hr marker without any barium reaching colon

[Figure 2-30-3]

The MAB pattern results in part from loss of normal peristalsis. Long arrow in A is normal feathery or fish-tail appearance of jejunal peristalsis. Short arrows in B show normal contractile pattern of Ileum with parallel folds in tapered segments mimicking esophageal contraction Malabsorption

506

Gastrointestinal Radiology

Look at the difference in “tone”: diameter and peristalsis [Figure 2-30-4]

Sprue 1° Rad Findings ll •

•

Proximal SB mucosal villous atrophy ➢ reversal of jejuno-ileal fold pattern ➢ toothpaste jejunum (moulage, < 4 folds/inch) ➢ “jejunization” of ileum (increased ileal folds) ➢ flattened bald duodenal mucosa ➢ foamy mucosal pattern “mosaic” Intussusceptions, momentary + non-obstructing (loss of wall thickness AND tone allow for loops to slide in and out.)

Toothpaste – Reversal [Figure 2-30-5 Foamy, Thick, Bald [Figure 2-30-6] •

The Jejunum looks like Ileum, the Ileum looks like Jejunum, the Duodenum looks like hell

Figure 2-30-4

The MAB pattern is evident in A with dilution giving gray watery barium ( short arrow) and dilated loops(double arrowheads) with minimal peristaltic events. The bowel looks baggy, flabby, like chronically stretched tube socks. Contrast A with a normal SB film in B. Numerous peristaltic events (arrows) are apparent and there is a state of tonic contraction allowing for visualization of the mucosal detail

Figure 2-30-5

Figure 2-30-6

Image A shows a smooth fold-free segment of the jejunum, called “toothpaste” or “moulage” caused by atrophy of mucosal villi and thickening of the wall by crypt hyperplasia. Image B shows a bald jejunum in the LUQ and a feathery abundant fold pattern in the ileum RLQ. Chronic increase in the nutrient mix presented to the ileum because of lack of jejunal absorption cause compensatory hypertrophy of ileal mucosa, hence “Reversal of Fold Pattern”

Image A shows a nodular lacy mucosal pattern (arrow) in the duodenal bulb. This is due to atrophy of the mucosa allowing the normal submucosal glands to become apparent

Gastrointestinal Radiology

507

Malabsorption

Mosaic Pattern [Figure 2-30-7]

Figure 2-30-7

Mucosal Atrophy [Figure 2-30-8] • • •

Fissures Pits Acid burns thinned mucosa

Acid Burns, Ulcerates, Strictures [Figure 2-30-9] Ulcer [Figure 2-30-10] •

“Occult GI Bleeding (FOBT+)…detected in half of pts with Sprue”

Fine KD, Prevalence of occult GI bleeding in celiac sprue, NEJM 1996 334:1163-7

Jejunal Webs [Figure 2-30-11]

Mucosal and fold atrophy may create a lacy granular or “mosaic” pattern in jejunum seen only with double contrast or mucosal detail compression images

Figure 2-30-8

Figure 2-30-9

Areas of narrowing ( arrows) may be seen in the proximal jejunum due to inflammation or scarring Mucosal atrophy leaves the wall susceptible to inflammation with cracks, fissures (arrows A) , ulcers, and pitting from chronic inflammation (Arrows B)

Figure 2-30-10

Figure 2-30-11

Ulcerations may heal with stricture formation

Recurrent ulceration and healing may lead to multiple short segment web-like strictures (arrows) Malabsorption

508

Gastrointestinal Radiology

Classic MAB Pattern [Figure 2-30-12] Intussusception [Figure 2-30-13]

Figure 2-30-12

CT in Sprue •

• • • • •

SB ➢ Fluid filled pelvic SB ➢ Dilated, Non distended SB - BAGGY ➢ Dilution if O = Iodine ➢ Flocculation if O = BA ➢ Intussusceptions ➢ Fragmentation Colon ➢ Big, Gassy, Wet ➢ Foamy Feces (Stool Whip) Nodes, incr number Loss of body fat Small Spleen Fatty Liver

Classic Appearance of SB Intussusception [Figure 2-30-14]

Classic MAB pattern. Dilution is evident with watery appearing barium (curved arrows). Dilatation is seen (double arrowheads). Puddles of isolated barium evident (red dots) which, if large, are called “segmentation”, if small, “flocculation”. Fluid and poor peristaltic activity in MAB causes segmentation and flocculation. Image A (Arrowhead): A worm or threadlike collection of barium is caused by a small amount of barium settling out in a fluid filled length of bowel that has not had enough peristalsis to keep the barium and water mixed

Figure 2-30-13 Figure 2-30-14

Intussusceptions are usually transient with dilated flaccid thin walled loops of bowel sliding easily into, and out of, each other. Because the muscle is weak and stretched out in Celiac Sprue, it cannot pull the intussusception deeper. With normal bowel grabbing a lead point, peristaltic muscle contraction pulls it further and tighter until it is wedged

Gastrointestinal Radiology

CT of Lower abdomen shows classic appearance of SB intussusception. The classic dotted crescent of fat(arrow) represents mesenteric fat and mesenteric vessels pulled in with the intussuscepting loop 509

Malabsorption

Dilated non-distented SB, Fluid filled distal SB loops, colon Slow transit of contrast, Fluid filling Colon [Figures 2-30-15 and 2-30-16]

Figure 2-30-15

Figure 2-30-16

Image A shows fluid and gas in the right and left colon (arrows). Image B show fluid filled mildly dilated loops of SB in pelvis (arrow). Both show minimal body fat. The fluid absorbing ability of colon is preserved in Celiac Sprue. With severe chronic malabsorption, the volume of fluid delivered to colon may overwhelm its ability to dry out wet foamy stool. The colon in severe cases will be fluid filled and pt will have prominent diarrhea

Large amount of stool (arrow), lack of body fat in subcutaneous tissues (curved arrow) and in peritoneal cavity, and dilated flaccid appearing SB loops are consistent with MAB. Sprue should be suggested if the history is appropriate

Figure 2-30-17

Reactive Lymphadenopathy [Figure 2-30-17]

Nutritional Collapse •

• • •

Hypoproteinemia ➢ Hypoalbulminemia ➢ Ascites Vitamin deficiencies ➢ K (Coagulation defects) Iron Deficiency Anemia ➢ Jejunum absorbs Fe ➢ Slowwww bleeding Electolyte Disturbances ➢ Tetany ➢ Seizures

Multiple small and moderate sized lymph nodes are seen in the SB mesentery (arrows) surrounding mesenteric vessels. They are reactive lymph nodes chronically stimulated by the low grade SB autoimmune inflammatory process. They are notable for number but rarely for size

Sprue = “Immune Disease” •

•

Lymphatic activity ➢ Reactive mesenteric nodes ➢ Para-aortic LNs ➢ Large cavitating nodes = poor prognosis ➢ Peripheral lymphadenopathy ➢ Splenic atrophy & clinical hyposplenism Antibody Tests define “Autoimmunity”

“Genetic Disease” •

Unique histo-compatability complex in 80% of sprue HLA B8 , DR3 ➢ (vs 20% of “normal” population) ➢ Increased prevalence of Sprue in families ➢ 10% latent Sprue in 1st order relatives

Malabsorption

510

Gastrointestinal Radiology

“Allergic Disease” • • •

Wheat, rye, barley Alpha-gliadin component of Gluten “Grass Allergy”

• • • • •

Genetic susceptibility ?Viral exposure → immune memory Gluten + endothelium → antigen Lymphocytes flood villous tips Antibodies destroy villi

• • • •

Genetic “Dose” + Gluten “Dose” + Time + Other Factors

Sprue: Immune Sequence

Determinants of Severity

Adapted from Marsh, Gastroenterology 1992: 102:330-54

Marsh Biopsy Categories • • • • •

0. Normal (Latent) 1. Intraepithelial lymphocytes increased 2. Infiltration with lymphocytes 3. Early villous atropy 4. Severe villous atrophy and crypt hyperplasia

Marsh M N, Gluten, major histocompatibility complex, and SB. A molecular and immunobiologic approach to spectrum of gluten sensitivity ('celiac sprue') Gastroenterology 1992 Jan;102(1):330-54)

Sprue Associated Auto-immunities

•

Skin ➢ Dermatitis Herpetiformis Pancreas ➢ Autoimmune Pancreatitis, Macroamylasemia Kidney ➢ IGA mesangial glomuleronephritis Insulin dependant diabetes ➢ 3 X increase incidence of Sprue Hair ➢ Alopecia areata MULTIPLE emerging associated auto-immunities

• • •

Pruritic papulovesicular lesions IG A deposits - dermal-epidermal junction Goes away with gluten restriction

• • • •

General Population Superfamily DR 3 / 3 1° Relative of Celiac Trisomy 21, Turners, Williams Synd Type 1 Diabetic Autoimmune Thyroid 1° Relative of Diabetic IG A Deficiency

•

• •

•

•

Dermatitis Herpetiformis CD in High Risk Patients

• • • •

Gastrointestinal Radiology

0.4%-2% 1% 33 % 4%-10% 5%-10% 4% 3% 2% 2%

511

Malabsorption

Nodules !! In Celiac Sprue ?? [Figure 2-30-18]

Figure 2-30-18

Healing Sprue • • •

Folds slowly return, first Diameter slowly shrinks May take 3-4 years

•

Occult Marsh 1 ➢ Nl SB exam, Asympt, ➢ bx +, labs + ➢ Intraepithelial lymphocytes ➢ 1° relatives of sympt pts: 5%-15 % Nodular Marsh 2 ➢ Sandy Nodules, irritable Classic MAB pattern Marsh 3, 4 ➢ Wet, wide, way late Non-responsive ➢ Diet errors, misdiagnosis ➢ Recalcitrant 5%-20% - Bact Overgr; Panc Insuffic ➢ Lymphoma

Sprue

• •

•

Recalcitrant Sprue • • • • • •

Responsive to initial Rx 8+yrs Loss of responsiveness Smoldering symptoms Thick folds “Ulcerative Jejunitis” High incidence Lymphoma

• •

Misty Mesentery Perivascular Cloaking

• • • • • •

Loss of response to gluten restriction. Rising Ig A, Sepsis. Increasing lymphadenopathy. Thickened bowel loops. Mesenteric Perivascular Cloaking ??? 1 yr lymphoma survival - 31 %, 5 yr - 11% ➢ Survival improves every yr, better CT Dx, Rx 3.4% malignancy in CD (Lymphoma, CA Esophagus, other)

“Recalcitrant” “Relapsing” Sprue

One phase of Sprue is an early transient phase in which lymphoid infiltration occurs. This will produce prominent or even nodular folds in SB, not typical appearance of Sprue. The stage is not often radiographed and it may be a fleeting phase of this disease

Lymphoma in Sprue

•

Figure 2-30-19

Think Physiologically [Figures 2-30-19 and 2-30-20]

• • •

Maldigestion (no enzymes, no mixing) ➢ Biliary - panc insuff, ZE, bacterial overgrowth, SB diverticulosis “Luminal” Cellular MAB (columnar C uptake failure) ➢ Sprue, ischemia, villous tip infiltration Malassimilation (columnar cell exit failure) ➢ Lymphangiectasia, abetalipoproteinemia, mesenteric diseases “Mesenteric”

Malabsorption

A patient with bloating, cramps, gas, indigestion, occasional diarrhea for yrs. CT Scan shows a big gassy colon, fluid filled loops of pelvic small bowel, and baggy proximal SB. The diagnosis was made because of these findings and because of obvious findings in upper slices evident in the first two images 512

Gastrointestinal Radiology

Luminal (Lumenal) [Figure 2-30-21] •

Figure 2-30-20

Pancreatic Insufficiency

College Freshman, Wt Loss, Diarrhea [Figure 2-30-22]

• • •

Fluid Colon Gas Minimal Fat

•

Exocrine Pancreatic Insufficiency ➢ 50% outgrow in adolescence Neutropenia Chronic Infections Myeloid Leukemia Metaphyseal Chondrodysplasia ➢ Dwarfism

Luminal MAB: Shwachman Diamond Syndrome [Figure 2-30-23] • • • •

Figure 2-30-21

Pancreatic calcifications. Pancreatic insufficiency can cause malabsorption. The patient may have no other symptoms other than abdominal bloating, discomfort, and diarrrhea

Figure 2-30-23

The pancreas is absent in this pt with MAB due to pancreatic insufficiency. Only fat is seen (arrows) where pancreas should be

Figure 2-30-22

CT of upper abdomen shows no glandular tissue with fat in the pancreatic bed (arrows). Pt had been diagnosed with Shwachman Diamond previously and had stopped replacement therapy at the start of college

A patient with diarrhea shows fluid filled loops of SB (arrow) in the pelvis indicating MAB and slow transit Gastrointestinal Radiology

513

Malabsorption

Luminal MAB: ZE Syndrome [Figures 2-30-24 and 2-30-25] •

•

MAB X-Ray pattern 2° to ➢ Increased gastric fluid ➢ Decreased pH ➢ > Enzyme non-activation ➢ > Poor digestion ➢ > Hypermotility ➢ > Edema/hyperemic folds Clinical MAB variable

Figure 2-30-24

Figure 2-30-25

Patients with ZE will have radiographic features of MAB. SB series shows dilated loops (double headed arrow) with dilution and with some fragmentation (curved arrows). The proximal SB and stomach shows fold thickening due to edema of folds( arrows) from large volumes of acid reaching the proximal SB

See dilution, dilatation from ZE syndrome

ZE • • •

Excess fluid Thick folds Hyperemic mucosa

•

MAB pattern 2° to ➢ Vagotomy ➢ Loss of pylorus –> bolus into SB ➢ > Poorly mixed food ➢ > Lack of acid digestion ➢ > Poor enzyme synchronization Clinical MAB not common 1yr p Surg ➢ Absorption occurs distally ➢ Pt changes eating patterns

Figure 2-30-26

Luminal MAB: Gastric Surgery [Figure 2-30-26]

•

Cellular Villous Dysfunction • •

•

•

Sprue Cong / Acq Enzyme Deficiencies ➢ Sugar splitting enzymes (Lactase) Bacterial / Viral Toxins ➢ Crypts Hypersecrete ➢ Capillaries Leak ➢ Enterocytes Malfunction ➢ Lymphatic Congestion Cellular Poisons - Drugs - Chemo - Rad Rx

Malabsorption

With a Bilroth L, II, gastrojejunostomy, or other surgery to increase gastric emptying, the proximal SB will become dilated due to surges of fluid and poor synchronization of the digestive process. Absorption equilibrates and occurs in distal SB and pts usually become asymptomatic as they modify their dietary habits 514

Gastrointestinal Radiology

Malabsorption from Bacterial Overgrowth [Figure 2-30-27] • •

Motility Diseases, eg Scleroderma, Chronic Narcotics SB Diverticulosis

Figure 2-30-27

Weight loss and diarrhea [Figure 2-30-28]

Wt Loss, episodic Diarrhea 30 F, MD, Wife of MD, 2 m in US from India for training [Figure 2-30-29] Bacterial infections affect absorption in a number of ways. Invasive bacteria may stun or destroy absorptive endothelial cells, may impair small capillary or lymphatic vessel drainage, compete for nutrients, or degrade critical enzymes MAB pattern is apparent with dilated loops (double arrow in A) and diluted barium (arrows A & B). Transit delay evident image B at 285 minutes. Numerous diverticula (red dots) shelter bacteria from peristaltic cleansing, allowing them to multiply to such a degree that they degrade or utilize nutrients and enzymes. Note how more diverticula are apparent in image B, diverticula may be difficult to assess, hiding amid dilated loops. Pts with numerous large SB diverticula may develop Megaloblastic anemia from B12 and folate deficiency

Figure 2-30-28

Weight loss and diarrhea. There are dozens of large SB diverticula not apparent. Easily overlooked unless you very very very carefully scroll. The MAB pattern with baggy SB loops, fluid levels, foamy feces should make you think hard and look for subtle causes of MAB

Figure 2-30-29

Marcha MAB Tropical Gastrointestinal Radiology

515

Malabsorption

SB Fluid - Dilution with FLOCCULATION !! [Figure 2-30-30] Tropical Sprue • • • •

• •

Figure 2-30-30

Villous and Crypt Atrophy Malabsorption Glossitis, wt loss, diarrhea, skin changes Folate & B12 deficiency prominent ➢ Rx folate, B12 improves partly Antibiotic Rx cures Relapses common in tropics

Westergaard H.Tropical Sprue Curr Treat Options Gastroenterol. 2004 Feb;7(1):7-11. Haghighi P, Wolf PL. Tropical Sprue and subclinical enteropathy.. Crit Rev Clin Lab Sci. 1997 Aug; 34(4): 313-41.

Scleroderma • •

[Figure 2-30-31]

Scleroderma always Dilated Delayed Dry Scleroderma WET = BACTERIAL OVERGROWTH

Marcha MAB Tropical Fluid

Giardiasis - Campylobacter [Figure 2-30-32]

Figure 2-30-31

Chemotherapy Enteritis

MAB: 2° Villus Blockage by • • • • • •

Lymph cells (immune disease, lymphoma, Crohn Disease) PMNs (infections) Eosinophils (eosinophilic gastroenteritis) Mast cells (mastocytosis) Macrophages (Whipples) Amyloid (amyloidosis)

“Giardia” on CT Req [Figure 2-30-33]

In image A, typical changes of Scleroderma involving SB are apparent with dilated loops, hidebound appearance and pseudo-sacculations. Scleroderma creates a motility disturbance without affecting absorption, creating a dry pattern without dilution. However, with severe Scleroderma and dysmotility, patients may have episodes of bacterial overgrowth which then produce MAB as seen in the pt in image B with dilution apparent

Villus Dysfunction • • • • • • •

Engorged Veins & Lymphatics Blocked Arteries (ischemia) Paraplegic with diarrhea MAB pattern Villous atrophy on Bx No response to gluten restricted diet Physical Exam -> NO LEG PULSES

Figure 2-30-32

Arterial Insufficiency [Figure 2-30-34] Lymphangiectasia [Figure 2-30-35] Malabsorption • • •

Radiologists may still be first on scene in pts with Sprue & MAB diseases MAB major radiographic pattern Fluid is the hallmark of MAB pattern ➢ Increased production +/or ➢ Decreased absorption

Image A shows MAB associated with Giardiasis. Image B is a patient with Mab from Campylobacter Malabsorption

516

Gastrointestinal Radiology

Figure 2-30-33

Figure 2-30-34

The oral contrast type determines the appearance of the fluid filled loops of pelvic SB. If barium is given, flocculation may be seen. If water soluble oral contrast is given, dilution may be seen. Acute and subacute infectious diseases may cause SB fluid to increase but may not be chronic enough to cause distention and delay. This patient has dilute water soluble oral contrast in the colon. Clips from a lymph node biopsy are present

Figure 2-30-35

Arterial insufficiency may cause dysfunction of all cells of the small bowel creating dysmotility and malabsorption. Note dilatation (double arrow) and dilution (curved arrow). Usually these bowel symptoms are accompanied by abdominal pain leading to a correct diagnosis. This paraplegic patient had an element of sensory denervation which caused the diagnosis of aortic thrombosis (image B arrow) to be missed initially

Lymphangiectasia may cause degrees of clinical and radiographic MAB due to engorged lymphatics and mucosal edema which will impede absorption. Usually folds remain prominent Gastrointestinal Radiology

517

Malabsorption

Sprue is King of Rad MAB •

•

•

•

Nodular Phase ➢ Rarely seen, ? short phase MAB Phase ➢ Commonest for Radiology, Classic Recalcitrant ➢ Dietary indiscretions, edema, nodes, MALIG Bacterial Overgrowth, Pancreatic Insuffic Lymphoma ➢ Nodes, weight loss, loss of gluten response

References 1. 2. 3. 4. 5. 6. 7.

8.

9.

10. 11.

12. 13. 14.

Farrell RJ, Kelly CP. Celiac Sprue. N Engl J Med. 2002 Jan 17;346(3):180-8 Fine KD, Prevalence of occult GI bleeding in Celiac Sprue. NEJM. 1996 334: 1163-7 Haghighi P, Wolf PL. Tropical Sprue and subclinical enteropathy. Crit Rev Clin Lab Sci. 1997 Aug; 34(4): 313-41. Johnston SD et al. A comparison of antibodies to tissue transglutaminase with conventional serological tests in the diagnosis of coeliac disease. Eur J Gastroenterol Hepatol. 2003 Sep; 15 (9): 1001-4. Jones B, Bayless TM, Hamilton SR, Yardley JH. "Bubbly" duodenal bulb in celiac disease: radiologic-pathologic correlation. Am J Roentgenol. 1984 Jan; 142(1): 119-22 Lomoschitz F et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol. 2003 Apr;13(4):890-6. Lomoschitz F et al. Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features. Eur Radiol. 2003 Apr;13(4):890-6.* Marsh MN. Gluten, major histocompatibility complex, and small intestine. A molecular and immunobiologic approach to spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology 1992 Jan;102(1):330-54)* Marsh M N, Gluten, major histocompatibility complex, and the small intestine. A molecular and immunobiologic approach to the spectrum of gluten sensitivity ('celiac sprue'). Gastroenterology 1992 Jan;102(1):330-54) Rubesin SE, Herlinger H, Furth EE." Bubbly" duodenal bulb in clinically unsuspected or refractory adult celiac disease. Abdom Imaging. 1998 Jul-Aug; 23 (4): 449-52. Schweiger GD, Murray JA. Postbulbar duodenal ulceration and stenosis associated with celiac disease. Abdom Imaging. 1998 Jul-Aug; 23(4):347-9. Tomei E et al. CT of SB in adult celiac disease: jejunoileal fold pattern reversal. Eur Radiol. 2000; 10(1):119-22.* Trier J. Celiac Sprue. NEJM. 1991 Westergaard H. Tropical Sprue. Curr Treat Options Gastroenterol. 2004 Feb; 7(1): 7-11.

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Things that go bump in the bowel: Familial Polyposis and Other Such Francis J. Scholz, MD 54 y/o M, Vomiting • • • • • •

2003: Vomiting 2-3 hrs p meals Liquid diet wks 30 lb wt loss - 4 mos Mother deceased, colon CA. 1996 Proctocolectomy, end-ileostomy 2001 Laparotomy, gastrotomy: 3 big gastric polyps removed

2 yrs earlier Endoscopy • • •

Figure 2-31-1

Many gastric polyps, Large obstructing duodenal polyp(s) Gastrectomy: ➢ Stomach: inflamm, mucosal papillary hyperplasia with atypia. Duodenum : Hyperplastic mucosa, foci suggestive of adenomatous change

FAP •

Usually: ➢ Dx known clinically ~75% ➢ Colonoscopic Dx, not BE, not CT ➢ Not Dx challenge except: ❖ ~25% de novo mutation ❖ Professor’s quiz ➢ Differential important: ➢ More things look like FAP than are FAP

Only a small percentage of Colorectal Cancers are related to FAP

Figure 2-31-2

Colorectal Cancer CRC Perspective [Figure 2-31-1]

•

150,000 new CR CA / yr,

only ~ 1% = FAP

•

Onset of polyps ➢ Age 10 -- 15% ➢ Age 20 -- 75% ➢ Age 30 -- 90% Risk of CA nearly 100% by age 40 yrs

FAP = Colonic Polyposis [Figure 2-31-2]

•

FAP Genotypes & Phenotypes •

• •

VARIABILITY IN: ➢ Age onset ➢ Polyp #: 100 - 1000s ➢ Bone, Eye, Skin, Brain, Thyroid, Desmoid APC is BIG gene, 300+ variable mutations ➢ “Gardner’s Syndrome” ➢ “Turcotte”, etc Early non-colonic changes may help early Dx

Gastrointestinal Radiology

There are many components possible in the patient with Familial Adenomatous Polyposis

519

Familial Polyposis

Uncountable

Figure 2-31-3

Barely Countable

Countable, Pedunculated Variable size [Figure 2-31-3]

1,000s of Small Early Polyps

FAP Colon Polyps = Adenomas No matter what size 100s of Bigger “Older” Polyps

1,000s of Small Polyps

FAP UGI Polyps [Figure 2-31-4] • • • •

Fundal gland hyperplastic polyps 100s of Bigger “Older” Polyps Adenomatous change in hyperplastic polyps in 100% of FAP pts. eventually. 5%-8% develop duodenal or ampullary CA, stomach, SB LIFETIME ENDO SURVEILL

Fundal gland hyperplastic polyps “Fundal” refers to normal location of nl type of gland FAP UGI • • • •

Figure 2-31-4

Start Flat Fundal first If you look you will find them. Follow colon polyps, occ precede.

Fundal gland hyperplastic polyps

Duodenal: Hyperplastic vs Adenoma vs CA ? •

Polyp number, size, and age of appearance vary in FAP patients

Lifetime Surveillance Needed

Figure 2-31-5

While they occur first and most prominently in the gastric fundus, the term Fundal Gland refers to the histologic characteristics of the polyp. “Fundal Gland” hyperplastic polyps in FAP can occur throughout the stomach and duodenum

While they start as hyperplastic polyps, they may undergo adenomatous change and then may undergo malignant degeneration. We cannot differentiate the three types of polyps: Hyperplastic, Adenoma, Adenocarcinoma. Lifetime endoscopy is required Familial Polyposis

[Figure 2-31-5]

520

Gastrointestinal Radiology

FAP Duodenal AdenoCa [Figure 2-31-6]

Figure 2-31-6

FAP Ampullary Tumor (Clue: Stoma) [Figure 2-31-7]

Figure 2-31-7

Obvious Duodenal Adenocarcinoma

Figure 2-31-8

Ampullary tumor and stoma: a link worthy of being an Aunt Minnie

FAP Duo - Ampulla [Figure 2-31-8] • • • • •

1. Cystic Glandular Hyperplasia 2. Adenomas 3. Carcinoma Jaundice, abn LFTs Pancreatitis

FAP - 33 yr p Colectomy [Figure 2-31-9]

Relative Risk: Upper GI CA in FAP Site No of Carcinoma Duodenum 7 Ampulla 4 Gastric 2 Non-duodenal 1 F/u 1391 pts

Rel Risk 331 124 2.4 12.7 18,679 pt - yrs

Offerhaus GJA, et al. Gastroenterology 1992; 102:1980

Osteomas [Figure 2-31-10] • • •

Sinuses Mandible Anywhere

•

Note absent colon gas

Ampullary Polyp and absent colon. Another linkup creating an almost Aunt Minnie

Figure 2-31-9

Osteomas “Benign Bone Islands” Mandibular Osteomas

“Exostotic Osteomas” [Figure 2-31-11] Diffuse Cortical Thickening

Cortical Endo & Exos [Figure 2-31-12]

Gastrointestinal Radiology

Ileostomy and SB tumor. With history of remote total colectomy, another almost Aunt Minnie. Without history, a great differential 521

Familial Polyposis

Figure 2-31-10

Figure 2-31-11

Osteomas growing out from the angle of the mandible, a favorite place for these rare manifestations to occur in FAP

Figure 2-31-12

Osteomas may be “innie” or “outie” osteomas

Figure 2-31-13 Osteomas of paranasal sinuses

FAP Dental ~80% [Figure 2-31-13] • • • • • •

Fused roots 1st & 2nd molars Missing Impacted Supernumerary Long tapered roots post teeth Mal-erupted

Carl, W. Herrera, L. Dental and bone abnormalities in patients with familial polyposis coli; Semin Surg Oncol 73-83, 1987

“CHRPE”: Congenital Hypertrophy of Retinal Pigment Epithelium • • •

Evident in subset of FAP Screening marker for subset of certain families ~Earliest clinical marker

• •

Sebaceous Cysts Pigment Changes

• • • •

Rare benign “tumors”, Never metastasize, invade locally, FAP associated, occ spontaneous In FAP pts:

Dental anomalies, mild, moderate, or severe, occur in FAP

FAP Skin

Desmoids

Familial Polyposis

522

Gastrointestinal Radiology

Figure 2-31-14

➢ 50% abd wall (surg excision) ➢ 50% intra-abdom, ❖ 85%-100% are mesenteric: -SBO or ischemia, -Hydronephrosis. ❖ Rx: NSAID in comb. w tamoxifen

Knudsen AL, Bulow S. Desmoid tumour in familial adenomatous polyposis. A review of literature. Fam Cancer. 2001;1(2):113-21

Desmoid Radiology [Figure 2-31-14] •

• •

1. Desmoid “Tumor” ➢ Geometric: muscle or intraperitoneum 2. Mesenteric Fibromatosis ➢ Infiltration: mesentery, retroperitoneum Both can occur in same pt

Desmoid

NOTE: Colectomy, Hazy Dense Mesentery

Desmoid tumors may be geometric or infiltrative

NON FAP DESMOID: TUMORAL & INFILTRATIVE with Ischemia Infiltrative / Tumoral

FAP: Causes of death

Cause Desmoid tumor Periampullary cancer Trauma/accident Perioperative death Rectal cancer Other

DCR 1990, 33:639

Number 11 (31%) 8 (22%) 3 ( 8%) 3 ( 8%) 3 ( 8%) 8 (22%)

Yrs after Colectomy 7 23 5 11 13 11

Mean age 35 49 31 37 42 50

Figure 2-31-15

Peritoneal Inclusion Cysts: “PIC” [Figure 2-31-15] • •

• • • •

F, usually premenopausal Prior proctocolectomy ➢ (Endometriosis, PID) Large pelvic cyst(s), multilocular Ovary trapped in pelvic loculation. Cysts lined by mesothelial cells. May see ovary on wall

Peritoneal Inclusion Cyst [Figure 2-31-16] •

• • •

Entrapped Ovary Syndrome ➢ Ovary secretes more fluid than can be absorbed by peritoneal locule. Mimic Ovarian Cystadenoma or CA Hx important. Rx Cycle suppression

PIC [Figure 2-31-17] FAP Mimics • •

Numerically, more things resemble FAP than the cases of FAP that we see. More common entities mimic classic polyposis syndromes

Gastrointestinal Radiology

523

Peritoneal Inclusion Cysts are a complication produced by pelvic peritoneal adhesions in premenopausal women. Familial Polyposis

Classification of Hereditary GI Polyposis Syndromes (Gene) • •

Figure 2-31-16

Familial adenomatous polyposes ➢ Adenomatous polyposis coli (APC) incl: Gardner, Turcot, Attenuated (APC) Hamartomatous polyposes ➢ Peutz Jeghers syndrome 1/8 Less common ➢ Familial juvenile polyposis rarer still ➢ Cowden’s disease ➢ Intestinal ganglioneuromatosis ➢ Ruvalcaba-Myrhe-Smith syndrome ➢ Tuberous sclerosis

Classification of Hereditary GI Polyposis Syndromes (Gene) • •

Familial adenomatous polyposes ➢ Born Adenomas -> Malignant transformation Hamartomatous polyposes HPs ➢ Born Hamartomas of varying types ➢ Some undergo epithelial atypia from “overwork” ➢ Some adenomas may develop, at risk CA ➢ Bowel CA can occur in all HPs ✧ More than general population ✧ Far less than FAP

Non-Hereditary GI Polyposes

• • • • •

Inflammatory and post inflammatory ➢ CUC, Crohns, Infectious colitides Lymphoid ➢ Reactive nodular lymphoid hyperplasia ➢ Lymphoma Pneumatosis cystoides intestinalis Lipomatosis Angiomatosis Leiomyomatosis Cronkhite-Canada syndrome

•

3 SB Hamartomas

•

•

Peritoneal Inclusions Cyst (s) progressively enlarge unless cycle suppression therapy is instituted. Reoccurrence may occur after lysis of adhesions

Figure 2-31-17

Peutz Jeghers [Figure 2-31-18] Figure 2-31-18

Ovary is evident in the wall of the PIC. A cystic lesion with septations and a mural nodule may simulate ovarian carcinoma Peutz Jeghers with three large polyps in SB. Polyps may be large, medium, small, endoscopically visible, or so teeny weeny to be visible only by microscopic biopsy. All sizes may be encountered in the same patient Familial Polyposis

524

Gastrointestinal Radiology

Peutz Jeghers [Figure 2-31-19] •

Episodic Pain due to intussusceptions

•

One 5 X 8 mm, many 1-2 mm polyps

Figure 2-31-19

Peutz Jeghers

Peutz-Jeghers [Figure 2-31-20]

Cowden’s Syndrome [Figure 2-31-21] • • • • • •

Autosomal Dominant - rare “Multiple Hamartoma Synd.” Age onset: 1st to 3rd decade Hamartomatous GI polyps, Non GI manifestations. CA, Breast~ 50% of F ➢ Thyroid ➢ Colon

Peutz-Jeghers pts may have painful episodes due to intussusception requiring surgery

Cowden’s Syndrome • • •

Papillomas / fibromas mucosa, tongue, (cobblestone appearance) Acral keratosis Tricholemoma of face Also: angiomas, lipomas, skin vitiligo

• • •

The polyps are juvenile hamartomas Syndrome can be adult presentation. Anywhere

•

Figure 2-31-20

Juvenile Polyposis Syndrome [Figure 2-31-22]

Peutz-Jeghers hamartomatous polyps in the stomach and duodenum

Figure 2-31-21

Figure 2-31-22

Cowdens Syndrome

Gastric Juvenile Polyposis refers to the type of polyp defined by microscopy, not the age of presentation

Gastrointestinal Radiology

525

Familial Polyposis

SB Juvenile Polyps [Figure 2-31-23]

Figure 2-31-23

Ruvalcaba-Myhre-Smith • • • • •

“Bannayan-Riley-Ruvalcaba syndrome (BRRS)” “Riley - Smith syndrome” “Bannayan - Zonana syndrome” “R M S” is best “Macrocephaly hamartoma papilledema syn”

RMS [Figure 2-31-24] RMS Syndrome

• • • •

Rare2, autosomal dominant ? (80% male) Men more common Ileal & Colonic polyps in 45% Other: ➢ Colonic tumors ➢ GI tumor/polyp/hemangioma ➢ Lobulated tongue (including hamartomas) ➢ Skin ❖ Abnormal genital pigmentation ❖ Acanthosis nigricans ❖ Cafe au lait spots ❖ Nevi or lentigines ❖ Lipomata ❖ Capillary hemangioma ❖ Cavernous hemangioma ❖ Other tumors of skin ❖ Supernumerary nipples ➢ Bones ❖ Delayed bone age ❖ Asymmetric limbs ❖ Joint laxity ❖ Muscle weakness/myopathy ➢ Brain ❖ Tumors/cysts ❖ Intra-cranial calcification ❖ Vascular malformations of brain ❖ Macrocephaly ❖ Papilledema ➢ Eye ❖ Anterior chamber abnormalities, ❖ Visible nerve fibers on cornea ❖ Palpebral fissures slant down ➢ High birth wt (> 90th %centile)

Large Juvenile Polyps in the SB

Figure 2-31-24

Ruvalcabre Myhre Smith in SB and Colon

Figure 2-31-25

Cronkite-Canada syndrome [Figure 2-31-25] • • • • •

Hyperpigmentation Alopecia Glossitis Dystrophic nails GI manifestations ➢ Harmatomatous polyps ➢ Exocrine pancreatic insufficiency) ➢ Diarrhoea ❖ Pancreatic insufficiency ❖ Protein losing enteropathy, low protein

Familial Polyposis

Cronkhite-Canada Syndrome

526

Gastrointestinal Radiology

CCS [Figure 2-31-26]

Figure 2-31-26

Lymphoma

[Figure s 2-31-27 and 2-31-28]

• •

Nodularity one form of Lymphoma. Remember L is a “PoLyposis+

Nodular Lymphoid Hyperplasia Colon [Figure 2-31-29] •

Can be: ➢ Related to GI infection ➢ Assoc w hypogammaglobulinem ➢ Assoc w Giardia ➢ Diff to histo diff from Lymphoma ➢ TI most frequent ➢ Anywhere in GI tract

Cronkhite Canada Syndrome

Figure 2-31-27

Figure 2-31-28

Lymphoma probably produces diffuse polyposis of the colon, and other organs, more frequently than does any one of the more famous Polyp Syndromes. Remember Lymphoma !!!!! In your differential for polyposis cases Diffuse Colonic Polyposis. One of the most frequent causes of diffuse polyposis is not due to a syndrome

Nodular Lymphoid Hyperplasia Small Bowel [Figure 2-31-30] •

Figure 2-31-29

Figure 2-31-30

Radiology ➢ Uniform, 1-4 mm ➢ May be umbilicated ➢ Diffuse ➢ Regional ➢ Clustered -”patches”

Nodular Lymphoid Hyperplasia Colon •

Radiology ➢ Uniform, 1-4 mm ➢ May be umbilicated ➢ Diffuse ➢ Regional ➢ Clustered

Gastrointestinal Radiology

NLH of SB 527

Nodular Lymphoid Hyperplasia may mimic a polyposis syndrome or Lymphoma Familial Polyposis

Mastocytosis [Figure 2-31-31] • • • •

Hyperplastic Polyps CUC [Figure 2-31-32]

Filiform Polyposis [Figure 2-31-33] • •

Figure 2-31-31

Secret: Small nodules differential: Think: White Blood Cell Differential: ➢ PMN ➢ Lymph ➢ Eo ➢ Mast ➢ Macroph

Crohns CUC

CUC

Figure 2-31-32

NLH of SB Nodules in the SB are usually caused by cellular infiltrates. To ease your differential brain pain, think of a WBC smear and all the types of WBCs seen. So nodularity in the SB from ordinary PMNs caused by infections, acute or chronic eg Whipples Lymph cells: Lymphoma, Nodular Lymphoid Hyperplasia, Lymph Cell Granulomas of Crohns or TBC, Immunoproliferative Small Intestinal Disease (IPSID) Eosinophils: Eosinophilic Gastroenteritis Mast Cells: Mastocystosis Macrophages: Chronic infections: IPSID, Tropical Sprue, Amyloid, Whipples

Figure 2-31-33

Polyps in CUC may be due to islands of residual mucosa persisting after slough of diseased mucosa or may due to inflammatory polyps developing as the mucosa attempts regeneration. Note ahaustral colon

Giant Hyperplastic Polyposis [Figure 2-31-34]

•

Seen in ➢ CUC ➢ Crohns ➢ Infect. Colitis

Hyperplastic polyps often have random shapes including wormlike or “filiform” polyps

Figure 2-31-34

Hyperplastic polyps may be massive. Imagine polyps so long, so numerous that they fill the colon like hamburger stuffing the colon like a sausage. Any colitis that sloughs the mucosa may create localized of diffuse inflammatory polyposis. Each polyp weeps serum creating hypoproteinemia. Pts may have inactive colitis and present with CHF, peripheral edema, or anasarca Familial Polyposis

528

Gastrointestinal Radiology

All Granulomatous Disease may give bumps !! •

Figure 2-31-35

Granulomatous change in ….

Crohn’s Stomach & Colon

Crohns Stomach [Figure 2-31-35]

Hyperplastic Polyps [Figure 2-31-36] • • • • • •

Benign Assoc w Atrophic Gastritis Atrophic Gastritis assoc w Gastric CA HP - AG - CA !! ? Assoc w Acid Suppression therapy: “Purple Pill Polyps”

Crohns of the Stomach!!

Declich P, et.al. Fundic gland polyps under omeprazole treatment. Am J Clin Pathol. 1999

Carcinoid Hyperplasia [Figure 2-31-37] •

• •

IN: ➢ Atrophic Gastritis ➢ Zollinger-Ellison 2° to: ➢ Absent Acid ➢ Acid Suppression ➢ Elevated Gastrin Cause: ➢ Carcinoid gland stimulation

Figure 2-31-36

Solcia E et al Morphology & pathogenesis of endocrine hyperplasias, precarcinoid lesions, & carcinoids arising in chronic atrophic gastritis. Scand J Gastroenterol Suppl. 1991;180:146-59

Figure 2-31-37

“Hyperplastic polyps” are the most common polyp of stomach. 1 to 2 cm size, PLUS multiple, PLUS mucosal, PLUS fundal and body location, PLUS approximately equal in size add up to allow statistical call of benign hyperplastic polypsl They are in themselves truly benign but are associated with degrees of atrophic gastritis which has risk for developing cancer. I call these the “purple pill” polyps because of suggested association with Acid Suppressive therapies. In your lifetime the argument will be settled

Carcinoid Glandular Hyperplasia

Gastrointestinal Radiology

529

Familial Polyposis

Pneumatosis Cystoides Coli [Figure 2-31-38]

Figure 2-31-38

Familial Polyposis • • •

Protean manifestations Multiple mimics Worthy of further study: ➢ For itself ➢ As starting point for Bumps of the Bowel

Pneumatosis Cystoides Coli may fool the unwary

References 1. 2. 3.

Carl, W. Herrera, L. Dental and bone abnormalities in patients with familial polyposis coli; Semin Surg Oncol 73-83, 1987 Knudsen AL, Bulow S. Desmoid tumour in familial adenomatous polyposis. A review of literature. Fam Cancer. 2001;1(2):113-21. Offerhaus GJA et al. Gastroenterology 1992;102:1980

Familial Polyposis

530

Gastrointestinal Radiology

The Spleen

Deborah J. Rubens, MD Embryology • •

•

Formed from the mesenchymal cells between the layers of the dorsal mesentery, which lies between the stomach and pancreas Rotates to the left pulling the mesentery with it and forming the lesser sac between the stomach and pancreas. Left side of the dorsal mesentery fuses with the parietal peritoneum covering the left kidney and adrenal to form Gerota’s fascia. This fusion brings the splenic vessels and the pancreas into the retroperitoneum. Hilum of the spleen is retroperitoneal, while most of the spleen is intraperitoneal, with a bare area similar to that of the liver, along posterior surface adjacent to the left kidney

Anatomy • • •

Crescent shaped, convex toward the diaphragm and concave medially, located in the LUQ Bounded by ribs, stomach, left kidney and splenic flexure of the colon. Splenic hilum contains splenic vessels and tail of pancreas (retroperitoneal) ➢ Splenic artery-tortuous, often containing aneursyms ➢ Splenic vein-straight. Confluence with SMV forms the portal vein. Splenic vein often enlarges with splenomegaly. Upper normal is 1.5cm

Important Connections •

• •

Splenorenal ligament (retroperitoneal) ➢ Connects the splenic hilum to the left kidney ➢ Contains the pancreatic tail, and splenic artery and vein Gastrosplenic ligament (peritoneal) ➢ Peritoneal fusion of the lesser and greater sacs, connecting the splenic hilum to the stomach Phrenicocolic ligament (peritoneal) ➢ Connects the lower pole of the spleen to the splenic flexure of the colon and to the diaphragm

Histopathology • • •

Stroma supports functional red and white pulp White pulp: the functional cells of the spleen; lymphocytes, plasma cells and macrophages. Red pulp: surrounds white pulp and is comprised of arteries and sinuses filled with blood. Also contains chords which slowly filter the blood, removing aging cells

Splenic Function •

• • •

Adult: filtration of aged rbc’s, sequesters platelets, removes foreign particles with macrophages. Childhood: adult functions plus production of lymphocytes and monocytes. Fetal hematopoesis Maintains immunity against bacterial pathogens (streptococcus D)

Normal Size • •

•

Long axis from diaphragm to inferior pole Usual size (US and CT and MRI) length 12cm, width 7cm and thickness 34cm. (you can allow greater length if the spleen is thinner) Spleen may be horizontal or longitudinal in orientation

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Normal Appearance (CT) [Figure 2-32-1] • • •

Figure 2-32-1

Normally less dense than liver by 15 HU precontrast, if greater than liver, then liver is fatty, or spleen contains iron. Normal is 40-60 HU. Arterial phase imaging (30 sec p/injection) ➢ Heterogenous enhancement with a serpentine, zebra-like pattern. Portal phase imaging (70 sec p/injection) ➢ Homogenous enhancement of 100-150 HU, generally 25 HU greater than the liver. If more than that, then liver is fatty

Normal Spleen. Normal arterial phase (left) and portal venous phase (right) CT images of the Measures 12 cm cranial-caudal spleen. Note the striped pattern on the arterial Normally does not extend beyond the left kidney phase at 30 seconds post injection has become Homogeneous mid-level echoes, slightly greater than uniform by the 70 second delay portal scan liver and equal to or greater than left kidney. Image in longitudinal and transverse planes, often easier to see in expiration

Normal Spleen (US) [Figure 2-32-2] • • • •

Vos PM, Mathieson JR, Cooperberg PL, The Spleen: in Diagnostic Ultrasound; Rumack CM, Wilson SR, and Charbonneau JW eds. .Elsevier Mosby 2005, St Louis, pp 147-170

Figure 2-32-2

Figure 2-32-3

Normal US. Longitudinal image between the ribs (left) and transverse image (right) shows the uniform medium level echoes of the spleen with the concave hilum containing the anechoic splenic vessels in the pancreatic tail

Normal signal intensity of the spleen on T1 (top left) T2 fat suppressed (lower left), Early arterial T1 Gadolinium enhanced (top right) and portal phase Gadolinium T1 enhanced (lower right) MRI Normal MR Appearance [Figure 2-32-3] scans. The early arterial phase in MRI shows • T1-low signal intensity, similar to muscle, lower than heterogeneous enhancement which becomes liver. uniform in the portal phase • T2 - high signal intensity, greater than liver. • Gradient echo: in and out of phase Figure 2-32-4 • Post contrast appearance: Arciform with serpiginous bands of low signal intensity separating larger regions of intense enhancement, similar to that seen on CT

Normal Variants • •

The spleen is formed from multiple cell aggregates which coalesce. This gives rise to: ➢ Accessory spleens ➢ Clefts ➢ Splenosis ➢ Splenic rests

Accessory Spleens [Figure 2-32-4] • •

30%-40% incidence in normal population and 10% of patients have >1 focus Frequently (75%) in splenic hilum

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Normal accessory spleen Left: normal spleen in left upper quadrant Right: Isoattenuating 2cm enhancing nodule lateral to the left kidney and adjacent to the splenic flexure is a normal accessory spleen

Gastrointestinal Radiology

• • •

Figure 2-32-5

Remainder usually adjacent to splenic poles or along splenic artery or in pancreatic tail Size ranges from microscopic to 2-3cm. May hypertrophy following splenectomy up to 5cm. Importance is not to mistake for other soft tissue pathology, usually lymphadenopathy

Accessory Spleen: Appearance [Figure 2-32-5] • •

Isoattenating to spleen on CT, isointense on MRI, and isoechoic on US. Ultimate diagnostic test is Tc99mSC nuclear medicine scan (macrophages take up radiopharmaceutical, differentiating accessory spleen from lymphatic tissue)

Spleen Cleft [Figure 2-32-6] • • • •

These are the residual spaces between partially fused lobules. These are sharp and well defined. They may be as deep as 2-3cm Most commonly they occur along the lateral margin of the spleen and on the superior diaphragmatic portion. They may mimic splenic lacerations

Splenosis [Figures 2-32-7 and 2-32-8] • • • • •

Enlarged spleen with accessory spleen (arrows) on US (top left), CT (lower left) and MRI T1 weighted ( right top), T2 weighted ( right middle) and Gadolinium enhanced (right lower) images. The image characteristics of the accessory spleen match those of the adjacent prinicipal splenic tissue on all imaging modalities

Residual splenic tissue following splenectomy. Fairly large (up to 5cm Figure 2-32-6 diameter) multiple nodules may occur, but typically small, multiple and enhancing. Location: most frequently in the LUQ, but may be anywhere in the abdomen along the peritoneal surfaces and/or mesentery. May involve the diapharagm and pleura DDX: endometriosis, mesothelioma Dx with Tc99mSC or RBC study Splenic clefts. Left: fine shallow clefts are noted at the superior and posterior margins of the spleen adjacent to the diaphragm. Note the lack of any fluid, and the sharp margins. The superior cleft extends from the medial to the lateral surface. Center: a deeper cleft in the posterior spleen, adjacent to the diaphragm. Right: An unmistakable cleft at the junction of 2 lobules, not a splenic tumor. Note the normal enhancement similar to the rest of the spleen

Figure 2-32-7

Splenosis. Left: multiple splenules in the left upper quadrant after splenectomy. Middle CT show splenules adjacent to the gallbladder and in the hepatorenal fossa (right) as well as in the left splenic and renal fossae Gastrointestinal Radiology

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Splenic Gonadal Fusion • • • • • •

Splenic tissue contained within epididymis, spermatic cord, or testis Male: female ratio is 17:1 Mimics tumor Believed to arise from adhesion between the gonadal primordial tissue and the spleen prior to gonadal descent. A fibrous band between gonad and spleen contains additional splenules in 50% and is associated with other congenital anomalies (cardiac or limb defects, hernias, undescended testes, micrognathia) Unconnected gonadal rests not associated with other anomalies

Figure 2-32-8

Warshaer DM. Spleen; in Computed Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th Edition, Lippincott Williams and Wilkins, Philadelphia, 2006: 973-1006

Splenosis.Top left: 2 cm enhancing round mass anterior to the aortic arch. Lower left: Round soft Polysplenia tissue mass posterior to the left atrium. Top right: • Bilateral left-sidedness with multiple other organ Bowel fills the left upper quadrant in the splenic system manifestations fossa. Lower right: Technesium 99m sulfur colloid ➢ ie 2 left lungs or left sided azygous or interrupted study shows the normal liver, and enhancing IVC, biliary atresia, absent gallbladder, GI nodules in the chest at the level of the aortic arch malrotation and the heart ➢ Associated with cardiac abnormalities including VSD, ASD, right sided arch, partial anomalous pulmonary venous return (PAPVR), transposition of the great vessels

Asplenia •

•

Bilateral right-sidedness ➢ 2 right lungs in 2/3 ➢ Midline liver ➢ More complex cardiac anomalies including single AV valve, pulmonary stenosis or atresia, TAPVR, transposition of the great vessels, ASD, single ventricle ➢ Mortality is as high as 80% in first year. Impaired immune response due to asplenia ➢ May present with serious bacterial infections

“Wandering Spleen” • • • •

Long mesentery if dorsal mesentery fails to fuse with the posterior peritoneum. Diagnosis made by US, CT or MR showing classic splenic tissue in abnormal location May torse and lead to acute or chronic abdominal pain. Lack of enhancement is present in complete infarction. Chronic torsion may lead to hypersplenism, splenomegaly, or gastric varices

Enlarged Spleen •

•

Moderately large ➢ Portal hypertension most common (check for cirrhosis and collaterals) ➢ Anemia ➢ Infection ➢ AIDS Very large (17 cm or more) ➢ Leukemia or lymphoma ➢ Infectious mononucleosis ➢ Myelofibrosis ➢ Portal hypertension

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Splenomegaly [Figure 2-32-9]

Figure 2-32-9

Splenomegaly with Spontaneous Rupture

81 yo female with polycythemia presented acutely with abdominal pain. Note massively enlarged spleen with anterior disruptions and hemoperitoneum. Splenomegaly. Right: transverse US shows an enlarged spleen anterior to the kidney. Center and right: massive splenomegaly Benign Focal Lesions in a patient with portal hypertension • Cysts • Hemangiomas Figure 2-32-10 • Granulomas • Abscesses • Infarcts • Trauma •

Simple Splenic Cysts [Figure 2-32-10]

•

Most commonly post traumatic in origin and lack an epithelial lining, thus are really pseudocysts. Appearance: ➢ US: anechoic with imperceptible wall and posterior acoustic enhancement. ➢ MR: low on T1, bright on T2 ➢ CT: water attenuation without enhancement Splenic cysts may rarely contain debris (cholesterol crystals, post trauma) No enhancement on CT or MR after contrast

• •

True congenital lesions discovered incidentally Often with trabeculations or septations and occasional peripheral calcification

• •

•

Epidermoid Cysts Other Cysts

Splenic Cyst by CT (left) is often calcified post trauma. Post-traumatic splenic cyst in a different patient. Sagittal ultrasound (right) shows a largely anechoic mass with some near field echoes but a sharp back wall, and posterior acoustic enhancement. This one also has some rim calcifications

Figure 2-32-11

Echinococcal cysts ➢ Extensive wall calcification • Pancreatic pseudocysts may arise within the spleen. Check for accompanying features of pancreatitis. Pseudocysts may contain debris or hemorrhage • Abscesses may mimic cysts by US, but on CT or MR should have an enhancing rim. On US they may contain gas or debris, differentiating them from Echinococcal cyst. Left: CT shows a heterogenous simple cysts cystic and solid lesion replacing the splenic tissue. The lesion contains central soft tissue and a Echinococcal Cyst [Figure 2-32-11] spoke-wheel appearance of multiple cysts. • 38-year-old Russian woman with persistent left flank Right: Gross pathology shows multiple cysts in an pain encapsulated mass within the spleen •

Pancreatic Pseudocyst [Figure 2-32-12]

Figure 2-32-12

36 yo female with 3 days of LUQ and flank pain. Cysts occupy the spleen (left and center) and the gastric wall and supcapsular regions of the spleen (right). Surgery revealed multiple pseudocysts. The pseudocyst in the gastric wall is a clue to the origin of the cysts Gastrointestinal Radiology

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Hemangioma [Figures 2-32-13 and 2-32-14] • • • •

Figure 2-32-13

Most common benign splenic neoplasm Echogenic on ultrasound Low signal on T1 and high on T2 (MRI) Low attenuation with early phase enhancement on MR or CT, often lacking the nodules and centripetal fill-in seen with liver hemangiomas, especially if <2cm

Other Benign Lesions • • •

Lymphangiomas-cystic with septations, may be septated and/or calcified Hamartomas-normal splenic tissue, predominantly red pulp, single or multiple, variable size (1-15cm)-slow and prolonged enhancement noted. Littoral Cell angioma-vascular tumor unique to spleen, multiple lesions of low attenuation on CT .2-9cm in size

Malignant Lesions

Sagittal US shows an echogenic well circumscribed mass on this assymptomatic patient presenting for renal ultrasound

Figure 2-32-14

• • •

Lymphoma Metastases Angiosarcoma

• •

Most common splenic malignancy Rarely as isolated lesion, usually as part of diffuse disease Low grade lymphomas usually diffuse enlargement Splenic hemangiomas. Left: early phase dynamic CT shows Hodgkins and higher grade NHL cause markedly enhancing smooth round nodules. Right: Delayed CT discrete low attenuation/echogenicity image at the same level shows delayed washout, characteristic nodules. of a benign lesion. On CT splenic hemangiomas often show Accuracy of CT prediction of splenic diffuse bright enhancement, more than the puddling peripheral involvement ranges from 30%-70%. enhancement seen in hepatic hemangiomas Marked splenomegaly the best predictor of involvement. FDG PET reported 98%-100% accuracy in predicting splenic lymphoma

Lymphoma • • •

Warshauer, D. Spleen: Computed Body Tomography with MRI Correlation. Lee JKT, Sagel SS, Stanley RJ, Heiken JP eds. Lippincott, Williams and Wilkins. Philadelphia, PA. 2006 pp 973-1006

Lymphoma • • • • •

Often presenting with splenic enlargement, LUQ pain or fever, weight loss, malaise. US: nodules are hypoechoic CT or MR, nodules usually not seen without contrast, but can be low signal on T2w MRI. Post contrast (CT or MR) are less intense than normal spleen but fill in quickly (2 min). Look for adjacent adenopathy

Figure 2-32-15

Lymphoma [Figure 2-32-15] •

A 53 yr old female with hepatitis C,splenomegaly and thrombocytopenia is evaluated for portal hypertension

The Spleen

Focal Non-Hodgkins lymphoma. A 53 yo female with hepatitis C, splenomegaly and thrombocytopenia is evaluated for portal hypertension. Left US shows a well circumscribed hypoechoic lesion. On Doppler imaging (center) it is vascularized. On CT (right ) there are multiple ill defined low attenuation lesions which are non-specific, and could be tumor, infarct or infection 536

Gastrointestinal Radiology

Lymphoma [Figures 2-32-16 and 2-32-17]

Figure 2-32-16

Hodgkins Lymphoma •

A 35 yr old man with past left seminoma presents with new lymphadenopathy and focal splenic lesions. Lymph node biopsy yielded Hodgkins lymphoma

Metastases

Common at autopsy difficult to image except on early arterial phase contrast imaging (CT or MR) Diffuse Non-Hodgkins lymphoma. 22 yo male with • Sources include islet cell tumors, melanoma, breast fever and LUQ mass. Left: Longitudinal US shows carcinoma and lung carcinoma massive splenomegaly with multiple diffuse hypoechoic lesions. Right: US guided biopsy of a Angiosarcoma [Figure 2-32-18] focal lesion returned non-Hodgkins lymphoma. • Most common primary nonlymphomatous splenic The patient died within 2 weeks of splenic rupture malignancy and hemorrhage • Single or multifocal • Aggressive growth with hemorrhage and necrosis. • Very vascular and enhance intensely with contrast in arterial phase Figure 2-32-17 •

Figure 2-32-18

Initial CT (left) shows isolated hypoechoic lesion of Non Hodgkins Lymphoma. One year later (right) the lesion has nearly resolved

44-year-old woman with abdominal pain, nausea, vomiting, chills. Patient underwent US, CT, US biopsy, laparoscopic biopsy with hemorrhage; exploratory laparotomy to rebiopsy, and evacuate hemoperitoneum, continued bleeding and dies 2 days later. CT (left) shows a low density lesion with peripheral nodular enhancement and multiple mixed attenuation partially enhancing lesions in the liver as well as ascites. Ultrasound (right) shows heterogenous echogenic lesions in the liver, consistent with highly vascular lesions with multiple interfaces

Figure 2-32-19

Infection [Figure 2-32-19] •

• •

Bacterial ➢ Aerobic from GI tract, sepsis ➢ TB Viral ➢ CMV, mononucleosis Fungal ➢ Candida

Gastrointestinal Radiology

40yo male with AIDS presented with marked splenomegaly. CT with contrast showed inumerable tiny low attenuation lesions. US (upper right) shows a 20 cm spleen with multiple tiny hypoechoic lesions. Core biopsy (lower right) with 18 g needle revealed granulomas, eventually proven to be TB

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5 Years Later [Figure 2-32-20]

Figure 2-32-20

Crohns Disease [Figure 2-32-21] •

27 yo male with Crohns and ileal perforation presents 3 weeks postoperatively (ileocecectomy) with LUQ pain and fever. What is the cause of the splenic lesion?

Abscess Evolution • •

2 weeks later 6 weeks later

• • • • •

What caused the abscess? How did it get to the spleen? Dx: Diverticulitis extending along the phrenicocolic ligament What else do you see? Splenic artery aneurysm

Abscess

TB in the spleen. Left: original US Right: repeat US 5 years later shows persistant splenomegaly, now with inumerable calcifications. Patient was treated successfully and was assymptomatic

Figure 2-32-21

Sarcoidosis

Splenic involvement common on biopsy (24%-59%) • Most patients asymptomatic • May show splenomegaly, or diffuse hypoattenuating nodules on CT and MR Splenic abscess. 27 yo male with Crohn’s disease presents 3 which lack peripheral enhancement weeks status post ileal and cecal resection with LUQ pain and • Associated abdominal fever. Left CT shows portal vein thrombus. Center CT shows lymphadenopathy common splenic vein thrombosis. Right CT shows typical rosette shaped abscess, in this case from septic thrombophlebitis from the GI Trauma tract • Spleen most frequently affected organ in blunt abdominal trauma. • Highly associated with left lower rib fractures. • Four appearances: ➢ Lacerations (check for splenic hilar involvement) are decreased attenuation on contrast enhanced CT-perisplenic blood or clot often more apparent than the laceration. ➢ Intrasplenic hematoma (contusion) may be low attenuation or contain higher attenuation clot ➢ Subcapsular hematoma: non-enhancing fluid with crescentic compression of underlying splenic tissue. ➢ Infarcts-non-enhancing wedge shaped areas extending to the capsule. • Severe trauma shatters the spleen into fragments. • Active bleeding is identified as focal extravascular enhancement similar in intensity to the aorta •

Trauma

• Clinically important injury is accompanied by hemoperitoneum Active bleeding identified as area of contrast enhancement with arterial intensity. Surgical intervention is based on clinical stability/hypotension, lacerations involving the hilum, and presence of pseudoaneurysms or arteriovenous fistulae Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma; Computed Body Tomography with MRI Correlation, 4th ed, Lippincott, Williams and Wilkins, Philadelphia 2006, pp1440-1429

Trauma •

Massive splenic trauma with fragmentation of the spleen, active arterial extravasation of contrast and hemoperitoneum

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Splenic Trauma? •

23 yo snowboarder with injury. Initial arterial phase images (left) show multiple possible contusions, without perisplenic hematoma. Portal venous phase images on repeat exam (right) show a normal spleen

Trauma [Figures • • •

Figure 2-32-22

2-32-22 and 2-32-23]

Lesion usually < 1cm Signal void on all pulse sequences Susceptibility artifact on GE images seen as blooming artifact

Splenic Angiosarcoma • • • •

Exceedingly rare Most common nonlymphoid primary malignant tumor of the spleen More common in patients with thorotrast exposure Splenomegaly with well defined nodules or diffuse involvement

Delayed Splenic Rupture • • •

Not predicted by degree of splenic injury Actual incidence unknown, but not uncommon. May occur days to weeks after initial injury

Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma. Computed Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th Edition, Lippincott Williams and Wilkins, Philadelphia, 2006: 1417-1480

Trauma. Initial CT shows tiny low attenuation lesion (top left ) without fluid, and subtle low attenuation lesions (lower right) in an otherwise normal spleen (lower left and upper right

Figure 2-32-23

Sequelae of Trauma •

•

Injuries (lacerations, infarcts and hematomas) may take months to a year to heal and may leave scars, deformed splenic margins, or splenic pseudocysts. Uncommon injuries requiring intervention include pseudoaneurysms or arteriovenous fistulae

Blunt Splenic Trauma •

Initial CT for blunt trauma shows a bright area of extravasation superiorly (left) and multiple lacerations more caudally (right). The patient was discharged

Splenic Trauma

Trauma. Eleven days later patient presents with diffuse abdominal pain. Top left CT shows medial contusion and perisplenic blood and ascites around the liver. Lower left and upper right show additional lesions. Lower right CT in the pelvis shows marked hemoperitoneum (high attenuation ascites)

Vascular Abnormalities •

• • • •

Portal hypertension ➢ Enlarged splenic vein and/or collaterals Splenic vein thrombosis ➢ Infection (Crohn’s, diverticulitis) ➢ Pancreatitis Splenic artery thrombosis (embolic) Splenic artery aneurysms Pseudoaneurysms ((trauma, pancreatitis)

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Portal Hypertension • •

•

Splenomegaly-often up to 20cm c-c Collaterals ➢ Located at the spenic hilum and most commonly directed into the renal vein (a spontaneous splenorenal shunt) ➢ Venous flow in the spleen is never reversed even if it is reversed in the splenic vein. Gamma gandy bodies

Portal Hypertension •

Figure 2-32-24

Gamma Gandy Bodies. Spin echo T1 (top left) and T2 weighted series (center), show signal voids at the iron foci. Gradient echo T1 images (Right) show the blooming effect of iron due to magnetic susceptibility (pathognomonic)

53 yo woman with cirrhosis and portal hypertension has massively enlarged liver on ultrasound and LUQ collaterals (US and CT)

Gamma Gandy Bodies • • • •

Focal iron deposition Common in patients with cirrhosis and portal hypertension due to microhemorrhages Generally mm in size (less than 1cm) Signal void on all MR images with pathognomonic blooming on gradient echo images due to susceptibility artifact

Gamma Gandy Bodies [Figure 2-32-24] •

Spin echo T1, T2, show signal voids at the iron foci. Gradient echo T1 images show blooming effect of iron due to magnetic susceptibility (pathognomonic)

Figure 2-32-25

10 year old with sickle cell disease. Nn-contrast CT (left) shows high attenuation spleen, greater than the liver.(normally should be less dense than liver pre contrast). MR T1 weighted (center) and T2 weighted (right) show very low signal intensity in the spleen, which is normally equal to muscle on T1 and much brighter than liver on T2. Note also the marked splenomegaly and the gallstones (T2)

Hemosiderosis [Figure 2-32-25]

• •

Hematoma ➢ Sucapsular ➢ Intrasplenic ➢ Perisplenic Look for sharp margin b/w spleen and peritoneal fluid Fluid will accumulate in dependent areas

• • •

Common due to arterial emboli Common in lymphomatous spleens which outgrow their blood supply Autoinfarction with sickle cell disease

• • •

Focal wedge-shaped peripheral lesions Invisible on US Decreased attenuation on contrast enhanced CT and low signal on T1 weighted post-Gd enhanced MRI May rupture with peripsplenic hematoma

•

Infarcts

Infarct Appearance

•

Splenic Infarcts [Figure 2-32-26] •

53 yo man with MDS presents with increasing LUQ pain. Initial US (left) shows large heterogeneous avascular cystic lesion in an enlarged spleen. 2 weeks later the lesion has retracted slightly. The patient underwent splenectomy which showed hemorrhagic infarcts

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Infarcts [Figure 2-32-27] •

Patient with sarcoidosis and multiple infarcts.

•

Several months later the splenic contour is diminished and the areas of infarction still lack enhancement

Figure 2-32-26

Healing Infarcts

What Is Your DX? • •

Dx: Aortic valve vegetation with splenic infarcts 80 yo with abdominal pain, “R/O aortic dissection or aneurrysm”

12 Hours Later Conclusion • • • •

•

The spleen is easily evaluated on cross-sectional imaging modalities.MRI is most sensitive for iron and for small lesions such as diffuse Candidiasis. Anatomic variants (splenules, clefts) are common and should not be mistaken for pathologic processes. Splenomegaly is non-specific, but usually related to hematologic abnormalities or portal hypertension. Infection is acquired by hematogenous or direct spread (splenic vein, colon, pancreas) Splenic infarction is often the sequelae to other disease processes and should encourage you to search harder to make the diagnosis

Round, hemoorhagic splenic infarcts. Top left US shows a large heterogenous mass with through transmission. The mass is avascular (upper right). Lower left Ultrasound 2 weeks later shows less fluid in the lesion with smaller diameter, indicating some clot retraction. Pathology specimen (lower right) shows 2 infarcts, the larger one corresponding to the US images

Figure 2-32-27

Classical infarcts going from superior spleen (left) to hilum (center) to inferior spleen (right) show varying shapes of infarcts which all extend to the periphery of the spleen and lack enhancement

References 1. 2. 3. 4. 5.

Vos PM, Mathieson JR, Cooperberg PL. The Spleen In: Diagnostic Ultrasound; Rumack CM, Wilson SR, and Charbonneau JW eds.Elsevier Mosby 2005, St Louis, pp 147-170 Kelekis NL, Burdeny DA, and Semelka RC. Spleen In: MRI of the Abdomen and Pelvis. Semlka RC, Ascher SM and ReinholdC, eds. 1997, Wiley-Liss New York, NY. Pp 239-256 Warshaer DM. Spleen; In: Computed Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th Edition, Lippincott Williams and Wilkins, Philadelphia, 2006: 973-1006 Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma; Computed Body Tomography with MRI Correlation, 4th ed, Lippincott, Williams and Wilkins, Philadelphia 2006, pp1440-1429. Molina PL, Quinn MT, Bouchard EW, Lee JKT. Computed Tomography of Thoracoabdominal Trauma. Computed Body Tomography with MRI Correlation, Lee JK, Sagel SS, Stanley RJ and Heiken JP, eds. 4th Edition, Lippincott Williams and Wilkins, Philadelphia, 2006: 1417-1480

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Portal Venous Doppler Deborah J. Rubens, MD

WHY DO WE USE DOPPLER? • • •

To identify vascular from non-vascular structures To find vessels invisible on gray scale To make diagnoses based on arterial or venous spectral or color flow patterns

• • • •

3MHz transducer-small footprint often preferred for intercostal approaches Doppler gain as high as possible without image or spectral noise Wall filter as low as possible to avoid false diagnosis of thrombosis Scale (PRF) as low as possible to localize vessels quickly with color, then sample and angle correct for spectral Doppler

SCANNING PARAMETERS

WHICH DOPPLER TO USE? •

• •

Spectral Doppler most sensitive to flow ➢ Inefficient for quick overview of flow direction, requires precise gate placement, suspended respiration. ➢ Use wide gate and search for small hepatic arteries not seen with color or power, and turn off color. Color Doppler good for flow direction (portal and hepatic veins) ➢ Quickly localizes hepatic arteries for spectral sampling. ➢ Aliasing identifies areas of stenosis (HA’s, TIPS) Power Doppler has best flow sensitivity ➢ Limited by motion (flash) artifact ➢ Best used in TIPS (which often have poor Doppler angles) and in portal vessels with slow flow

COLOR DOPPLER ALIASING IN TIPS •

Color Doppler shows direction of flow and mean velocity. Spectral Doppler shows angle-corrected true velocity

POWER vs. COLOR DOPPLER

• •

Partial thrombosis suggested. Spectral Doppler implies PV flow. Note HA visualized on power Doppler as well as intrahepatic vessels

•

Monotonous continuous waveform normally directed into the liver (hepatopedal) Color doppler should fill out the entire vessel to exclude portal vein thrombosis (may be anechoic) Spectral doppler velocities are typically low, 15-40cm/sec and may even be bidirectional due to swirling flow in a large slow-flowing vein. Portal vein flow reverses (out of the liver, hepatofugal) in portal hypertension, and in patent, well functioning porto-systemic shunts

THE NORMAL PORTAL VEIN

•

• •

PORTAL HYPERTENSION •

• •

Most commonly caused by cirrhosis, but may also be caused by diffuse metastatic disease and by venous outflow obstruction. Color flow Doppler and spectral doppler are used in the evaluation of portal hypertension to detect portal vein thrombosis, the presence of collaterals and to assess bypass shunts (portocaval, splenorenal, mesocaval, and TIPS) for patency. A positive diagnosis can be made by reversed (hepatofugal) flow on Doppler or by presence of porto-systemic collaterals

eMedicine - Portal Hypertension : Article by Ali Nawaz Khan, MBBS ... Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005, Mosby, Inc. pp 77-146 Gastrointestinal Radiology

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Seminar: Portal Venous Doppler

CASE 1: 55 YO WOMAN WITH ABNORMAL LFT’S •

A transverse image of the right lobe of the liver shows 2 equal-sized vessels adjacent to the gallbladder

CASE 1 What is your diagnosis? •

DX: Portal hypertension with hepatofugal flow

CASE 1: 55 year old woman with abnormal liver function tests. (below) A transverse image of the right lobe of the liver shows 2 equal sized vessels to the right of the gallbladder CASE 1: (below) Spectral display of the hepatic artery (left) and portal vein (right). What is your diagnosis?

PORTAL HYPERTENSION WITH HEPATOFUGAL FLOW •

•

•

US offers information re: flow direction; into or out of the liver, which is unavailable on CT. Flow reversal out of the liver is a late, but diagnostic sign of advanced portal hypertension. Quick clues are the opposing colors and opposing spectral flow directions of the hepatic artery and portal vein, which should normally both flow in the same direction

CASE 2 •

Patient with AML and rising liver function tests. Baseline examination

•

Dx: Veno-occlusive disease with reversed portal flow

CASE 2: 3 days later. What has happened and what is your diagnosis?

CASE 2: Three days later the patient returns with abnormal liver function tests (below) What has happened and what is your diagnosis?

CASE 2: Patient with Acute myelogenous leukemia and rising liver function tests. Baseline examination shows normal portal Doppler waveform (right)

Seminar: Portal Venous Doppler

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VENO-OCCLUSIVE DISEASE WITH REVERSED PORTAL FLOW • • •

Hepatic veno-occlusive disease involves hepatic venules in bone marrow transplant patients. Major veins appear normal – the diagnosis is confirmed by liver biopsy. Rapid development of portal vein hepatofugal flow has been reported with veno-occlusive disease* and when present is diagnostic

*Brown BP, Abu-Yousef M, Farner R, La Brecque D, Gingrick R. AJR 1990;154:721-4

CASE 3 :Explain the spectral tracings in the right and left lobes •

Dx: Liver metastases with localized left-sided portal hypertension

•

While portal hypertension is most commonly the sequelae of cirrhosis, it can be caused by any process which obstructs the sinusoids including Venoocclusive disease, Budd Chiari syndrome, and metastases. Focal flow reversal should prompt a search for localized disease, including analysis of the gray scale images

ATYPICAL PORTAL HYPERTENSION

•

PORTOSYSTEMIC COLLATERALS •

•

•

CASE 3: 61 year old female with rising liver function tests and jaundice. Prior Doppler ultrasound was normal. Left: Doppler tracing of the right HA and PV. Center: Gray scale transverse image of the left lobe Right: Spectral Doppler tracing of the left HA and PV. Explain the difference between the left and right lobe. Diagnosis: Metastatic breast carcinoma with localized left portal hypertension

These are diagnostic of portal hypertension Frequent locations include the gastroesophageal junction, paraumbilical vein in the falciform ligament, splenorenal and gastrorenal in the left upper quadrant, intestinal veins in the retroperitoneum, and hemorrhoidal veins in the pelvis. Visualization of the paraumbilical vein is specific for the diagnosis of portal hypertension. Visualization of collaterals requires slow flow settings, and a good sonographic window, unobscured by bowel gas

CASE 4: 31 year old male with ascites

CASE 4: Can you account for the alternate flow directions in the left and right portal veins? What is your diagnosis? •

Portal hypertension with a patent umbilical collateral vein

CASE 4: Can you account for the alternate flow directions in the left and right portal veins? What is your diagnosis?

CASE 4: 31 year old male with ascites

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PORTAL HYPERTENSION WITH COLLATERALS • • • •

Multiple collateral pathways carry portal blood around the liver into the systemic circulation The most common is the coronary vein, however this is not specific for portal hypertension Another common pathway is the patent umbilical vein, seen in up to 20% of patients and 100% specific for portal hypertension. A patent umbilical vein collateral will preserve forward (hepatopetal) flow in the left portal vein while the flow in the right portal vein is reversed (hepatofugal).

eMedicine - Portal Hypertension : Article by Ali Nawaz Khan, MBBS ...

CASE 5: What is happening in the splenic vein? CASE 5: What is your diagnosis now? • Portal hypertension with spontaneous splenorenal shunt formation

CASE 5: Portal hypertension with spontaneous splenorenal shunt formation seen on sequential CT images and corresponding transverse US image (bottom right)

CASE 6: A 30 yr old woman presents with acute epigastric pain. What is the diagnosis and possible etiology? •

Case 5: A 68 year old woman presents in liver failure. Top: Midline transverse flow in the splenic vein is toward the transducer (toward the spleen). Bottom: Flow in the splenic vein is away from the spleen at the hilum

Dx: Splenic and portal vein thrombosis, patient taking birth control pills

Portal Vein Thrombosis

Bland thrombus is a frequent sequelae to slow flow and portal hypertension. Other etiologies include hypercoagulability, pancreatitis, pyelophlebitis from diverticulitis or Crohn’s disease or cholangitis, and portocaval shunts. Thrombus may be partial or complete, involving main or branch vessels. • Early thrombus is hypoechoic, and may require color Doppler to detect. • Older thrombus is hyperechoic and recanalizes and/or forms collaterals, which have typical portal spectral waveforms. CASE 6: A 30 yr old woman presents with acute epigastric • Tumor thrombus (HCCA) contains pain. What is the diagnosis and possible etiology? hepatic arterial waveforms and may be Left: US shows enlarged anechoic portal vein with normal color biopsied for staging.* flow in the IVC posteriorly. Right: Transverse image of noncontrast CT corresponds to US on the left. *Dodd GD, Memel DS, Baron RL, Eichner L, Dx: Splenic and portal vein thrombosis in a patient taking birth Santaguida LA. AJR 1995;165:573-577 control pills •

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Gastrointestinal Radiology

CASE 7: A 64 year old man is being considered for liver transplant. •

Dx: Cavernous transformation of the portal vein

•

This refers to numerous collateral vessels at the porta due to acute or longstanding thrombosis. (Cavernous transformation may occur as soon as a week after occlusion) The absence of an adequate portal vein for anastamosis precludes successful liver transplantation

CAVERNOUS TRANSFORMATION OF THE PORTAL VEIN

•

CASE 7: A 64 year old man is being considered for liver transplant

De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K. Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR 165, 1151-1155,

CASE 8: Is this normal or abnormal flow? •

Right and left portal vein flow reversal in normally functioning TIPS

PORTO-SYSTEMIC SHUNTS • • •

These are used to decompress portal hypertension to control bleeding Color Doppler is useful to assess shunt patency, providing an adequate acoustic window is present (may be limited in mesocaval and splenorenal shunts) Intrahepatic portal venous flow should be hepatofugal if the shunt is working properly

PORTOSYSTEMIC SHUNTS: TIPS • •

•

•

CASE 8: TIPS shunt. Is the portal vein flow normal or abnormal?

These are the most common portosystemic shunt now used. The entrance is percutaneous from the jugular vein into the right hepatic vein through the liver to the main portal vein. Spectral Doppler TIPS peak velocity is obtained in the proximal (portal venous end), mid and distal (hepatic end) shunt. Absent flow indicates shunt occlusion Color flow in the intrahepatic portal veins should be toward the TIPS, and the involved hepatic vein flow should be toward the IVC

TIPS – DOPPLER SURVEILLANCE • • •

Monitoring is recommended post procedure, then q3 months and as clinically indicated Normal velocity is between 90 and 190cm/s in the mid and distal shunt.* (although the original lower limit of normal was reported to be 50-60 cm/sec,** this has been unreliable in the proximal shunt due to portal mixing) A change in peak stent velocity by >50cm/sec over time, reversed flow in the draining hepatic vein, and focal shunt stenosis are also useful ***

*Kanterman RY, Darcy MD, Middleton WD, Sterling KM, Teefey SA, Pilgram TK. AJR 1997;168:467-472. **Foshager MC, Ferral H, Nazarian GK, Castaneda-Zuniga WR, Letournea JG. AJR 1995;165:1-7. ***Dodd GD, Zajko AB, Orons PD, Martin MS, Eichner LS, Santaguida LA. AJR 1995;164:1119-1124.

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PROXIMAL TIPS SHUNT VELOCITY •

Doppler signal from the proximal end of the TIPS shunt. Flow velocity increases as the cursor is moved from the main portal vein (less than 40 cm/sec) into the shunt (69 cm/sec)

NORMAL TIPS

ABNORMAL TIPS •

28mm gradient between TIPS and right atrium due to tight stenosis at the hepatic venous end which was balloon angioplastied. Note monophasic portal wave form

Proximal TIPS Shunt Velocity Doppler signal from the proximal end Increased shunt velocity at the distal end of the shunt indicated by of the TIPS shunt. Note the increase color Doppler aliasing in flow velocity as the cursor is Spectral Doppler signal demonstrates the focal distal shunt moved from the main portal vein into stenosis, velocity is 256.7 cm/sec the shunt

TIPS Shunt Stenosis • •

Abnormal US, Normal Angiogram •

US abnormal by strict velocity criteria, but note normal pulsatility

TIPS Monitoring: Low Velocities •

Subsequent venogram showed severe stenosis throughout the stent, which was replaced with an intrastent following balloon angioplasty

CONCLUSION • • •

Portal venous Doppler US is an effective way to assess the patency and function of the hepatic portal TIPS Shunt Stenosis. (Right) Increased shunt system. velocity at the distal end of the shunt indicated by It permits rapid identification of portal flow direction color Doppler aliasing. (Left)Spectral Doppler and can identify collaterals, specific to portal signal demonstrates the focal distal shunt hypertension. stenosis, velocity is 256.7 cm/sec It is particularly useful to serially monitor TIPS shunt function

eMedicine - Portal Hypertension : Article by Ali Nawaz Khan, MBBS ... Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005, Mosby, Inc. pp 77-146. References 1. 2. 3. 4. 5. 6. 7. 8.

Brown BP, Abu-Yousef M, Farner R, La Brecque D, Gingrick R. AJR 1990; 154:721-4. De Gaetano AM, Lafortune M, Patriquin H, De Franco A, Aubin B, Paradis K. Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR 165, 1151-1155, Dodd GD, Memel DS, Baron RL, Eichner L, Santaguida LA. AJR 1995;165:573-577. Dodd GD, Zajko AB, Orons PD, Martin MS, Eichner LS, Santaguida LA. AJR 1995; 164:1119-1124. eMedicine - Portal Hypertensions: Article by Ali Nawaz Khan, MBBS. http://www.emedicine.com/radio/topic571.htm Foshager MC, Ferral H, Nazarian GK, Castaneda-Zuniga WR, Letournea JG. AJR 1995;165:1-7. Kanterman RY, Darcy MD, Middleton WD, Sterling KM, Teefey SA, Pilgram TK. AJR 1997;168:467-472. Wilson SR, Withers CE. The Liver in Diagnostic Ultrasound, Rumack, Wilson and Charboneau eds.2005, Mosby, Inc. pp 77-146.

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Gastrointestinal Radiology

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Genitourinary Radiology

Genitourinary Radiology

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Imaging of Uterine Disorders Paula J. Woodward, MD

Figure 3-1-1

Overview • • • • •

Normal Anatomy Imaging Techniques Congenital Anomalies Benign Lesions Malignancies

• • •

Fundus Corpus Cervix

•

Serosa ➢ peritoneal reflection Myometrium ➢ involuntary smooth muscle Endometrium ➢ stratum basalis ➢ stratum functionalis

Uterus

Uterine Corpus • •

Cervix [Figure 3-1-1] • • •

Internal os Endocervical canal ➢ columnar epithelium ➢ plicae palmatae ➢ surrounded by fibrous stroma and muscular layer External os ➢ squamocolumnar junction

Uterine Ligaments • • • • •

Normal cervix

Figure 3-1-2

Broad ligaments ➢ double sheet of peritoneum Cardinal ligaments Uterosacral ligaments Uterovesical ligaments Round ligaments

Blood Supply [Figure 3-1-2] •

•

Uterine artery ➢ branch of internal iliac ➢ passes superficial to the ureter ➢ enters myometrium at internal os Ovarian artery ➢ branch of the aorta ➢ anastomosis with uterine artery

Dual blood supply to uterus

Imaging Techniques • • • •

Ultrasound Hysterosalpingography Sonohysterography MRI

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Ultrasound •

•

Figure 3-1-3

Transabdominal ➢ full baldder ➢ 2.5–5.0 MHz transducer Transvaginal ➢ empty bladder ➢ 5.0–7.5 MHz transducer

Myometrium [Figure 3-1-3] • • •

Homogeneous intermediate echogenicity Can sometimes see hypoechoic inner and outer layers Blood supply ➢ uterine ➢ arcuate ➢ radial ➢ spiral (endometrium)

Endometrium [Figures 3-1-4 and 3-1-5] •

• •

•

Early proliferative phase ➢ thin echogenic line Late proliferative phase ➢ hypoechoic thickening, 4–8mm Secretory phase ➢ hyperechoic thickening, 7–14mm Menstrual phase ➢ thin broken echogenic line

The uterine arteries give rise to the arcuate arteries located in outer third of myometrium. Radial arteries course through the myometrium and terminate as spiral arteries in the endometrium

Figure 3-1-4

Figure 3-1-5a

Before ovulation (days 1 – 14), the ovary is in the follicular phase and the endometrium is in the proliferative phase. After ovulation (days 14-28) the ovary is in the luteal phase and the endometrium is in the secretory phase

Endometrium, proliferative phase

Figure 3-1-5b

Endometrium, secretory phase

Hysterosalpingography: HSG • • • • •

First ten days of menstrual cycle Active PID contraindication Radiation dose 75–750 mrad Only visualizes internal contour Primary use tubal patency

Imaging of Uterine Disorders

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Genitourinary Radiology

Pelvic MRI • •

Phased array coils Fast T2WI images

• •

Pelvic Coil Coronal localizer – FMSPGR ➢ Always include kidneys FSE T2 – sagittal, axial, coronal, oblique ➢ TR 4,000–5,000 ➢ TE 90–130 ➢ ETL 16 ➢ FOV 20–24 cm ➢ Thickness 4–5 mm, 1 mm gap ➢ Matrix 256x256 ➢ 2–4 NEX T1 SE ➢ Axial ➢ TR 300–500 ➢ TE min T1 Fat Sat with Gd

Figure 3-1-6

Pelvic Protocol •

• •

Normal Uterus: Sagittal T2WI and HSG view

Figure 3-1-7

Uterus • •

T1 – uniform intermediate signal T2 – zonal anatomy ➢ Endometrium – high signal ➢ Junctional zone – low signal ➢ Myometrium – intermediate signal

Normal Cervix: sagittal and donut view

Figure 3-1-8

Normal Uterus [Figure 3-1-6]

Normal Cervix [Figure 3-1-7]

Embryology

[Figures 3-1-8 and 3-1-9]

Figure 3-1-9

Embryologic paramesonephric ducts. Metanephrosis (kidney) with concurrent development

Uterus forms from fused paramesonephric ducts. Distal vagina forms from urogenital sinus Genitourinary Radiology

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Imaging of Uterine Disorders

Mullerian Duct Anomalies [Figure 3-1-10] • Class • I • II • III • IV • V • VI

Figure 3-1-10

Description Agenesis or hypoplasia Unicornuate Didelphys Bicornuate Septate VIDES-related

Unicornuate / Didelphys [Figure 3-1-11]

• • • •

Low rate of pregnancy loss Limited surgical options Unicornuate – highest rate of renal agenesis Didelphys – 75% have vaginal septum

Bicornuate

[Figures 3-1-12 and 3-1-13]

• • •

Partial fusion of ducts Concave external contour Bicollis or unicollis

Classification of Mullerian duct anomalies

Figure 3-1-12

Figure 3-1-11

Unicornuate and didelphys

Figure 3-1-13

Bicornuate bicollis (2 cervices) and bicornuate unicollis (1 cervix)

Bicornuate, unicollis Imaging of Uterine Disorders

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Genitourinary Radiology

Septate

[Figures 3-1-14 and 3-1-15]

• • • •

Most common uterine malformation Highest spontaneous abortion rate Septum may be complete or partial Septum may be fibrous or composed of myometrium

Figure 3-1-14

Figure 3-1-15

Complete and partial septum

Septate uterus with a complete fibrous septum through the cervix

Bicornuate vs. Septate [Figure 3-1-16]

Angle between horns External morphology Complications Treatment

Arcuate Uterus

Bicornuate

Septate

>90º <90º concave normal abnormall lie increased spontaneous premature labor abortion rate metroplasty hysteroscopic resection

[Figure 3-1-17]

Figure 3-1-16

Figure 3-1-17

At least 1 cm of remaining myometrium should be present for hysteroscopic resection

Arcuate uterus is a normal variant. 1 1.5 cm indentation Genitourinary Radiology

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Imaging of Uterine Disorders

Diethylstilbestrol: DES – Related

Figure 3-1-18

[Figure 3-1-18]

• • • •

1–1.5 million female progeny exposed 50% have a uterine anomaly Associated with clear cell carcinoma of the vagina No associated urinary tract abnormality

• • • • •

Form a continuum Renal anomalies in 25% Obstructions are common – risk for endometriosis and adenomyosis Septate has highest spontaneous abortion rate DES exposure risk factor for clear cell carcinoma of the vagina

• •

Leiomyomas Adenomyosis

• • •

Benign smooth muscle tumor Dysmenorrhea, hypermenorrhea, fertility problems 25% of premenopausal women

Mullerian Duct Anomalies

Benign Uterine Masses Leiomyoma

DES exposure

Leiomyoma [Figure 3-1-19]

• • • •

Submucosal, intramural, subserosal Well circumscribed US – generally hypoechoic MRI – low signal on T1 and T2 unless they have undergone degeneration

Figure 3-1-19

US, MRI, and hysteroscopic image of a submucosal fibroid

Leiomyoma Degeneration [Figure 3-1-20] • • • • •

Hyaline Myxomatous Cystic Hemorrhagic (carneous) Sarcomatous

Figure 3-1-20

Cystic and hemorrhagic degenerated fibroid Imaging of Uterine Disorders

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Genitourinary Radiology

Indications for MRI • • •

Figure 3-1-21

Pre-myomectomy Rapidly growing fibroid When US is confusing

Adenomyosis

[Figures 3-1-21 and 3-1-22]

• • •

Heterotopic implants of endometrium within the myometrium Dysmenorrhea, hypermenorrhea 25% of hysterectomy specimens

Adenomyosis [Figure 3-1-23] • •

May be diffuse or focal (adenomyoma) MRI ➢ junctional zone > 1 cm ➢ low signal on T1 and T2 ➢ punctate areas of high signal ➢ irregular borders

Diffuse adenomyosis

Adenomyosis •

Figure 3-1-22

Ultrasound ➢ Enlarged heterogeneous uterus ➢ Focal form often confused for fibroids

Endometrial Thickness • •

•

< 15 mm in premenopausal patient (secretory phase) ≤ 8mm in asymptomatic post-menopausal patient (if on hormones scan after withdrawl bleeding) ≤ 4mm postmenopausal and bleeding

Abnormal Uterine Bleeding • • • • •

Polyps Submucosal fibroids Hyperplasia Carcinoma Atrophy ➢ most common cause of post-menopausal bleeding

Sonohysterography

Adenomyosis

Figure 3-1-23

[Figure 3-1-24]

Figure 3-1-24

Focal adenomyosis (adenomyoma)

Sonohysterography. Saline is infused while scanning

Genitourinary Radiology

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Imaging of Uterine Disorders

Endometrial Polyps [Figure 3-1-25]

• • • •

Focal overgrowth of endometrial tissue Pedunculated or sessile 20% are multiple May be cystic

Figure 3-1-25

Endometrial polyp

Submucosal fibroid

Figure 3-1-26

Endometrial Hyperplasia [Figure 3-1-26] •

•

Increased estrogen stimulation ➢ hormone replacement (unopposed estrogen) ➢ tamoxifen ➢ anovulatory cycles, polycystic ovarian disease ➢ obesity ➢ estrogen producing tumors (granulosa cell, thecoma) Risk factor for carcinoma

Tamoxifen [Figure 3-1-27] • • •

Has an antiestrogen effect on the breast but weak estrogen effect on the uterus Increased risk of endometrial carcinoma, hyperplasia, and polyps Cystic changes often present

Endometrial hyperplasia

Postmenopausal Bleeding • •

Figure 3-1-27

≤ 4mm – atrophy > 4mm – sonohysterogram ➢ diffuse thickening – random bx or D&C ➢ focal thickening – hysteroscopy

Endometrial Carcinoma • •

Most common GYN malignancy- 33,000 cases/year Risk factors: ➢ unopposed estrogens, tamoxifen ➢ nulliparous ➢ diabetes ➢ obesity

Cystic endometrium from tamoxifen Imaging of Uterine Disorders

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Endometrial Carcinoma •

•

Histology ➢ adenocarcinoma (80%–90%) ➢ adenosquamous ➢ papillary serous ** ➢ clear cell carcinoma ** Grade ➢ I – well differentiated ➢ II – moderately well differentiated ➢ III – poorly differentiated

Endometrial Carcinoma: FIGO – Staging Stage 0 Ia Ib Ic II III IVa IVb

Description Carcinoma in situ Limited to endometrium Less than 1/2 myometrium Greater than 1/2 myometrium Invades cervix but not beyond uterus Beyond uterus but not outside pelvis Outside true pelvis / bladder / bowel Distant metastases

MRI Findings

Figure 3-1-28

• • • •

Intermediate signal mass Expands endometrial cavity Enhances less than myometrium Not for screening

• • • • •

Disruption of junctional zone Depth of myometrial invasion Extension into cervix Extension beyond uterus Adenopathy

• • • •

Histology Tumor grade Depth of myometrial invasion Lymph node involvement

• • • •

14,000 cases/year, 4,900 deaths/year Begins at squamocolumnar junction 90% are squamous cell Association with papilloma virus, herpes, and HIV

MRI Staging [Figure 3-1-28]

Prognostic Factors

Cervical Carcinoma

Endometrial carcinoma extending to serosa

Cervical Carcinoma Stage 0 I II IIa IIb III IVa IVb

Description Carcinoma in situ Confined to cervix Invades beyond cervix but not to pelvic sidewall or lower third of vagina No parametrial invasion Parametrial invasion Extension to pelvic sidewall / lower third of vagina / causes hydronephrosis Invasion into bladder / rectum Distant metastases

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Staging •

•

Clinical ➢ errors in 32% for stage IB (greater than 5mm deep and 7mm wide) ➢ 62% for II-IV MRI ➢ 93% accuracy for tumor size within 5mm ➢ Staging accuracy 87%- 92%

Prognosis • • • •

Tumor size Depth of invasion Parametrial extension Lymph node involvement

• •

Intermediate signal T2WI Check list ➢ tumor size ➢ depth of stromal invasion ➢ parametrial invasion ➢ hydronephrosis ➢ lymphadenopathy

Figure 3-1-29

MRI

Cervical Carcinoma Stage I [Figure 3-1-29]

Cervical Carcinoma Stage II [Figure 3-1-30]

Stage I cervical carcinoma. The tumor remains confined to the cervix

Figure 3-1-30

Stage IIB cervical carcinoma with obvious parametrial invasion References 1. 2. 3. 4. 5. 6. 7. 8.

Bazot M, Cortez A, Darai E, et al. Ultrasonography compared with magnetic resonance imaging for the diagnosis of adenomyosis: correlation with histopathology. Hum Reprod 2001; 16:2427-2433. Davis PC, O'Neill MJ, Yoder IC, Lee SI, Mueller PR. Sonohysterographic findings of endometrial and subendometrial conditions. Radiographics 2002; 22:803-816. Nicolet V, Carignan L, Bourdon F, Prosmanne O. MR imaging of cervical carcinoma: a practical staging approach. Radiographics 2000; 20:1539-1549. Reinhold C, Khalili I. Postmenopausal bleeding: value of imaging. Radiol Clin North Am 2002; 40:527-562. Reinhold C, Tafazoli F, Mehio A, et al. Uterine adenomyosis: endovaginal US and MR imaging features with histopathologic correlation. Radiographics 1999; 19 Spec No:S147-160. Scheidler J, Heuck AF. Imaging of cancer of the cervix. Radiol Clin North Am 2002; 40:577-590, vii. Troiano RN, McCarthy SM. Mullerian duct anomalies: imaging and clinical issues. Radiology 2004; 233:19-34. Ueda H, Togashi K, Konishi I, et al. Unusual appearances of uterine leiomyomas: MR imaging findings and their histopathologic backgrounds. Radiographics 1999; 19 Spec No:S131-145.

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Approach to Renal Masses Paula J. Woodward, MD

Approach to Renal Masses: 4 Questions • • • •

Cyst vs Solid Infiltrative vs Expansile Fatty vs Soft Tissue Solitary vs Multiple

• •

Expansile – “Ball” Infiltrative – “Bean”

• •

Spherical, exophytic, frequently encapsulated DDx ➢ Malignant – adenocarcinoma, metastases, lymphoma ➢ Benign – cyst, angiomyolipoma, oncocytoma, etc.

Tumor Growth: Ball vs. Bean Expansile Renal Mass

Infiltrative Renal Masses • •

Invades parenchyma, preserves renal contour, poorly marginated DDx ➢ Malignant – transitional cell, squamous cell, lymphoma, atypical adenocarcinoma ➢ Benign – pyelonephritis, XGP, TB

Intravenous Urography • • •

Only good for expansile masses Misses 1/3 of masses <3cm All lesions require further work up ➢ US, CT, MRI

Ultrasound •

•

Cyst ➢ anechoic ➢ acoustic enhancement ➢ sharp posterior wall RCCA ➢ can be hypo, iso, or hyperechoic

Computed Tomography • • • •

94% sensitive for lesions 3 cm or less 90%-95% accuracy in staging Pre and post contrast of lesion Scan in both corticomedullary and nephrographic phase

Genitourinary Radiology

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Renal Neoplasms

Volume Averaging – All Tissues in the Slice Volume are Averaged

Figure 3-2-1

[Figures 3-2-1 to 3-2-3]

Phases of Excretion · · ·

Corticomedullary phase 25 –80 sec Nephrographic phase 90–120 sec Excretory phase 3 –5 min ➢ Varies with injection rate, cardiac output, and renal function

Corticomedullary Phase (CMP) · · ·

Cortex and medulla > 100 HU difference Best for metastases and vascular invasion Pitfalls: Can miss hyperdense cortical masses and hypodense medullary masses, pseudotumor in the IVC

Volume averaging

Figure 3-2-2

Nephrographic Phase · ·

Renal lesions are best seen in the nephrographic phase 1.1 ---> 2.4 more masses detected

Venous extension

Contiguous vs. overlapping reconstruction

Figure 3-2-3

[Figure 3-2-4]

Excretory Phase · ·

Decreasing density of nephrograms Worsening streak artifact especially with non-ionic contrast

Excretory Phase · · ·

Decreasing density of nephrograms Worsening streak artifact especially with non-ionic contrast New role in CT urography Affect of volume averaging on an AML

Figure 3-2-4

Surgical approach is based on extent of venous invasion

Enhancement · · ·

< 10 HU no enhancement > 15 HU enhancement 10–15 HU gray zone

Renal Neoplasms

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De-enhancement ·

Decrease 15 HU at 15 minutes

CT Technique for Renal Mass Characterization · · · · ·

Scan kidneys both pre and post contrast Slice thickness of 5–7mm Scan during corticomedullary and nephrograhic phase Perform overlapping reconstruction if the lesion is small Delayed scans

Magnetic Resonance Imaging · · ·

Equivalent to CT in accuracy with Gd Calcification difficult to detect Excellent for vascular invasion

Magnetic Resonance Imaging ·

·

Cysts ➢ low-signal T1WI ➢ high-signal T2WI ➢ no enhancement Solid ➢ 15% enhancement with Gd

Calculating % Enhancement ·

(Post SI – Pre SI) / Pre SI x 100 = % enhancement

Malignant Neoplasms · ·

· ·

Adenocarcinoma Uroepithelial tumors ➢ Transitional cell ➢ Squamous cell Lymphoma Metastases

Renal Cell Carcinoma · · · · ·

25,000 –30,000 new cases 12,000 deaths per year Peak incidence – sixth and seventh decade M:F (2–3:1) Bilateral 2%, multicentric 15%

Renal Cell Carcinoma ·

Expansile cortical mass ➢ 90% originate from proximal convoluted tubule

Renal Cell Carcinoma · ·

Slow growing low grade malignancies may be encapsulated Can have areas of necrosis, cyst formation, hemorrhage, or calcification

Histology ·

· ·

Clear cell – 70%–80% ➢ Deletion on chromosome 3p ➢ Lipid rich Papillary – 10%–15% ➢ Slower growing, less vascular, calcification more common, often encapsulated, better prognosis Other – chromophobe, sarcomatoid, medullary, etc.

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Renal Neoplasms

Renal Cell Carcinoma - Risk factors · ·

·

Figure 3-2-5

Dialysis [Figure 3-2-5] von Hipple Lindau [Figure 3-2-6] ➢ often multiple RCCAs ➢ multiple renal cysts ➢ affects other abdominal organs Tuberous sclerosis (much less common)

Renal Cell Carcinoma: Presentation · · · ·

“Classic triad” – hematuria, flank pain, mass < 50% Paraneoplastic – hypertension, erythrocytosis, hypercalcemia Other – fever, weight loss, anemia, varicocele 30% present with metastases Cystic disease of dialysis with RCCA

Calcification · · · ·

20%–30% of RCCA 1%–2% of benign cysts Rim calcification – 80% benign Central calcification – 87% malignant

Figure 3-2-6

“Cystic” Changes ·

15–25% of RCCA ➢ Necrosis 75% ➢ Cystic 25% – often papillary histology ❖ Mural nodule or septations [Figure 3-2-7] ❖ Malignant cell lining (VHL)

Benign Lesions ·

·

Simple Cyst ➢ Water density ➢ Thin (1-2 mm) wall ➢ No enhancement Minimally Complicated Cyst ➢ High density cysts (protein or blood) ➢ Thin septations ➢ Thin curvilinear calcifications

VHL with multiple RCCA

Figure 3-2-7

Surgical Lesions · · · · ·

Enhancing lesions Nodularity Thick wall (>2mm) Thick septations Irregular or central calcifications ➢ Less important

Spontaneous Renal Hemorrhage · · · · · · ·

RCAA (men) AML (women) Infarction Infection AV malformation Vasculitis Glomerulonephritis

Cystic RCCA

Infiltrating Renal Cell · · · ·

7% of adenocarcinomas Arise from the renal medulla – difficult to differentiate from invasive uroepithelial tumor Medullary carcinoma, collecting duct carcinoma, sarcomatoid neoplasms Poor prognosis

Renal Neoplasms

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Genitourinary Radiology

Medullary Carcinoma [Figure 3-2-8] · · ·

Figure 3-2-8

Young Black male with sickle cell trait From epithelium of papilla or distal collecting duct Survival < 4 mos.

Robson Staging [Figure 3-2-9] · · · · · · ·

I – Confined to kidney II – Within Gerota’s fascia III A – Renal vein or IVC invasion III B – Lymph nodes III C – Vascular invasion plus nodes IV A – Direct organ invasion IV B – Distant metastases

Medullary carcinoma with infiltrative pattern

TNM Staging · · · · · · · · · · · · · ·

·

T1 – < 7cm T2 – > 7 cm T3a – local invasion not beyond Gerota’s fascia T3b – venous invasion below diaphragm T3c – venous invasion above diaphragm T4 – extension beyond Gerota’s fascia N0 – no regional lymph nodes N1 – metastasis in a single regional lymph node N2 – metastasis in more than one regional lymph node M0 – no distant metastasis M1 – distant metastasis Stage I ➢ T1,N0,M0 Stage II ➢ T2,N0,M0 Stage III ➢ T1,N1,M0 ➢ T2,N1,M0 ➢ T3a,N1,M0 ➢ T3b,N0,M0 ➢ T3b,N1,M0 ➢ T3c,N0,M0 ➢ T3c,N1,M0 Stage IV ➢ T4,N0,M0 ➢ T4,N1,M0 ➢ Any T,N2,M0 ➢ Any T,any N,M1

Figure 3-2-9

Stage 1

Stage II with invasion into Gerota’s fascia

Figure 3-2-10

American Joint Commitee on Cancer

Nephron-sparing Surgery · · · · ·

Margins of at least 5 mm normal tissue < 4cm Away from renal hilum (polar, cortical) Survival rates comparable to radical nephrectomy RF ablation non-surgical alternative

Stage II vs. Stage I [Figure 3-2-10]

· ·

Nodule in perinephric space most specific but present < 50% Perinephric stranding unreliable Stage II with nodule in perirenal space

Genitourinary Radiology

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Renal Neoplasms

Stage III [Figure 3-2-11] Figure 3-2-11

Stage III

Stage III Renal Carcinoma: Imaging [Figure 3-2-12]

Figure 3-2-12

Vascular invasion extending to the right atrium Renal Neoplasms

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Genitourinary Radiology

Stage IV [Figure 3-2-13]

Figure 3-2-13

Stage IV

Stage IV renal carcinoma: Imaging Figure 3-2-14

[Figure 3-2-14]

Renal Cell Carcinoma: Metastases · · · · · ·

Lung Bone Liver Nodes Brain Adrenal

69% 43% 34% 22% 5% 4%

Abdominal CT Checklist · · · · ·

Renal vein, IVC Regional lymph nodes Adrenal glands Contralateral kidney Review lung and bone windows

Stage IV with invasion into the descending colon

Uroepithelial Neoplasms · · · ·

5%–10% of all urinary tract malignancies Transitional cell 85%– 95% Squamous cell carcinoma 5%–10% Rare – adenocarcinoma, sarcoma, metastases

Transitional Cell Carcinoma · · ·

50–70 yo Males > females (3:1) Risk factors ➢ Smoking ➢ Aniline dyes ➢ Benzene ➢ Analgesic nephropathy (phenacetin) ➢ Balkan nephropathy

Transitonal Cell Carcinoma · · · ·

Hematuria 75% Multicentric 30%–50% Bilateral 10% Incidence by location: ➢ Bladder 92% ➢ Pelvis 6% ➢ Ureter 2%

Genitourinary Radiology

567

Renal Neoplasms

Transitional Cell Carcinoma [Figure 3-2-15]

· · · ·

Figure 3-2-15

Small, hypovascular masses Majority papillary with endophytic growth Renal invasion in 25% Imaging ➢ Retrogrades, IVP with compression ➢ CT urography ➢ US and conventional CT poor

Squamous Cell Carcinoma [Figure 3-2-16]

· · · ·

Squamous metaplasia from chronic irritation Associated with stones (>50%) Aggressive behavior, commonly infiltrative Survival < 1 yr

Renal Lymphoma

Invasive transitional cell carcinoma presenting as a renal mass

[Figures 3-2-17 to 3-2-19]

· · · · · · · · · ·

Common in widespread disease Primary lymphoma very rare Hematogenous spread or direct invasion 50-70% bilateral Homogeneous Multiple masses 50% Infiltrating hilar mass 25% Perirenal 10% Renal enlargement 10% Solitary mass 5%

Figure 3-2-16

Figure 3-2-17

Sqaumous cell carcinoma with a staghorn calculus and large soft tissue mass. SCCA often has overlapping features with XGP

Figure 3-2-18

Renal lymphatics are located around the capsule and renal hilum

Figure 3-2-19 Renal lymphoma with homogeneous expansile and infiltrative masses

Perirenal lymphoma with capsular invasion Renal Neoplasms

568

Genitourinary Radiology

Metastases · · ·

Figure 3-2-20

7%–20% of autopsy cases Generally asymptomatic Primaries ➢ Lung ➢ Breast ➢ GI (esp colon) ➢ Melanoma

Metastates [Figure 3-2-20] ·

· ·

Spread ➢ Hematogenous – usually cortical ➢ Lymphatic – perirenal ➢ Direct invasion Expansile or infiltrative pattern Solitary or multiple masses

Metastases with adenopathy

Figure 3-2-21

Benign Renal Neoplasms ·

·

Cystic ➢ Multilocular cystic nephroma Solid ➢ Parenchymal ❖ Oncocytoma ❖ Juxtaglomerular tumor ❖ Leiomyoma (capsuloma) ➢ Mesenchymal ❖ Angiomyolipoma

Multilocular Cystic Nephroma [Figure 3-2-21]

· · · ·

Bimodal age distribution ➢ < 2 yo (M:F, 3:1) ➢ > 40 yo (F:M, 9:1) Multiple well-defined cysts with enhancing septa, no hemorrhage Can herniate into renal pelvis DDx: cystic renal cell carcinoma, MCDK complicated benign cyst, abscess

Oncocytoma [Figure 3-2-22]

· · ·

Oncocyte – Greek “swollen cell” Large epithelial cells with granular eosinophilic cytoplasm (abundant mitochondria) Found in kidney, salivary glands, thyroid, parathyroid, and pancreas

Oncocytoma · · · · ·

Usually asymptomatic Large – 7 cm avg. at detection Older males Solid exophytic enhancing mass Can not distinguish from RCCA

Multilocular cystic nephroma with herniation into the renal pelvis

Figure 3-2-22

Oncocytoma ·

·

Helpful features – all non-specific ➢ Central scar ➢ “Spoke wheel” angio appearance ➢ Necrosis, hemorrhage, calcification rare ➢ No adenopathy or metastases Gross – tan/brown tumor with pale central scar Oncocytoma with a central scar

Genitourinary Radiology

569

Renal Neoplasms

Juxtaglomerular Cell Tumor · · · ·

Figure 3-2-23

Renin producing tumor Rare cause of hypertension, may also have headache and muscle weakness Young adults (F:M, 2:1) Hypovascular mass – imaging non-specific

Leiomyomas [Figure 3-2-23] · · ·

Small solid masses on renal surface (capsuloma) Usually incidental finding Imaging non-specific ➢ Low signal on T2-weighted MRI is suggestive

Angiomyolipoma: AML · · ·

·

Renal hamartoma with blood vessels, smooth muscle, and fat Prevalence 0.3%–3% 80% sporadic ➢ Females 30–50 yo ➢ Usually solitary 20% tuberous sclerosis

Angiomyolipoma: Imaging · ·

· ·

Ultrasound non-specific [Figure 3-2-24] AML ➢ Shadowing ➢ Markedly hyperechoic RCCA ➢ Hypoechoic rim ➢ Cystic spaces Must prove with CT or MRI

Low-signal, non-enhancing leiomyoma (capsuloma)

Figure 3-2-24

Angiomyolipoma: Imaging ·

· ·

CT ➢ HU < –10 will detect 85% of AMLs ➢ No calcifications ➢ Vascular phase imaging can detect aneurysms MRI ➢ fat bright on T1 and T2 ➢ fat saturation sequence Angiography ➢ Tortuous, abnormal vessels with small aneurysms ➢ Embolization

AML with shadowing

Tuberous Sclerosis [Figure 3-2-25] · · ·

Autosomal dominant Clinical triad – seizures, adenoma sebaceum, mental retardation Multiple hamartomatous lesions including: retinal hamartoma, cortical tubers, subependymal nodules, ungual fibroma, angiofibroma, pulmonary lymphangiomyomatosis, cardiac rhabdomyoma

Figure 3-2-25

Tuberous Sclerosis [Figure 3-2-25]

·

Renal involvement ➢ Approx 3/4 will have AML ❖ 75% multiple ❖ 50% bilateral ➢ Cysts can also be seen especially in children ➢ 1%–2% develop RCCA

Renal Neoplasms

Tuberous sclerosis with large bilateral AMLs

570

Genitourinary Radiology

Angiomyolipoma: Presentation

Figure 3-2-26

[Figure 3-2-26]

· · · · ·

Incidental finding ➢ Usually < 4 cm Hemorrhage ➢ Usually > 4cm ➢ May be spontaneous or minor trauma Bleeding may be life-threatening in up to 25% of cases Vessels thick walled with decreased elastin Predisposition for aneurysm formation

RCCA with Fat [Figure 3-2-27]

· · ·

Osseous metaplasia – calcification Lipid necrosis – large necrotic masses Engulfed perirenal or sinus fat – large masses, irregular invasive appearance

Angiomyolipoma with perinephric hemorrhage

Figure 3-2-27

RCCA with osseous metaplasia

Genitourinary Radiology

571

Renal Neoplasms

Helpful Tips ·

· · · · · ·

No enhancement (etc.) ➢ Benign cyst Fat ➢ AML Multiple AMLs ➢ Tuberous sclerosis Infiltrative + expansile ➢ Lymphoma Herniation into renal pelvis + female ➢ Multilocular cystic nephroma Cysts + solid masses ➢ VHL or dialysis Central scar + no adenopathy or vein invasion ➢ Oncocytoma

References 1.

Catalano C, Fraioli F, Laghi A, et al. High-resolution multidetector CT in the preoperative evaluation of patients with renal cell carcinoma. AJR Am J Roentgenol 2003; 180:1271-1277. 2. Choyke PL, Glenn GM, Walther MM, Zbar B, Linehan WM. Hereditary renal cancers. Radiology 2003; 226:3346. 3. Khan A, Thomas N, Costello B, et al. Renal medullary carcinoma: sonographic, computed tomography, magnetic resonance and angiographic findings. Eur J Radiol 2000; 35:1-7. 4. Rendon RA, Stanietzky N, Panzarella T, et al. The natural history of small renal masses. J Urol 2000; 164:11431147. 5. Sheth S, Scatarige JC, Horton KM, Corl FM, Fishman EK. Current concepts in the diagnosis and management of renal cell carcinoma: role of multidetector ct and three-dimensional CT. Radiographics 2001; 21 Spec No:S237254. 6. Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologicpathologic correlation. Radiographics 1995; 15:653-669. 7. Israel GM, Bosniak MA, Slywotzky CM, Rosen RJ. CT differentiation of large exophytic renal angiomyolipomas and perirenal liposarcomas. AJR Am J Roentgenol 2002; 179:769-773. 8. Urban BA, Fishman EK. Renal lymphoma: CT patterns with emphasis on helical CT. Radiographics 2000; 20:197212. 9. Wong-You-Cheong JJ, Wagner BJ, Davis CJ, Jr. Transitional cell carcinoma of the urinary tract: radiologicpathologic correlation. Radiographics 1998; 18:123-142; quiz 148. 10. Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angiomyolipoma: relationships between tumor size, aneurysm formation, and rupture. Radiology 2002; 225:78-82.

Renal Neoplasms

572

Genitourinary Radiology

Urinary Tract Trauma Paula J. Woodward, MD GU Trauma • •

Evaluate lower tract before upper tract if both may be injured Males always perform retrograde urethrogram before foley is inserted if there is blood at the meatus or pubic rami fx/diastasis

Figure 3-3-1

Male Urethra [Figure 3-3-1] •

•

Posterior ➢ Prostatic ➢ Membranous Anterior ➢ Bulbous ➢ Penile

Retrograde Urethrogram: RUG [Figure 3-3-2]

• • • • •

Inflate pediatric foley (3–5cc) in fossa navicularis 50 cc of 30%–60% contrast Inject 20–30 cc and take film while continuing to inject Oblique if possible or gently move penis laterally May perform pericatheter RUG if foley is in place

Figure 3-3-2

Normal urethral anatomy

Figure 3-3-3

Intact urethra

Urethral Trauma [Figures 3-3-3 to 3-3-8]

•

Posterior – pelvic fractures I – stretch II – rupture above UGD ➢ retropubic extravasation III – rupture above and below UGD ➢ perineal/scrotal extravasation IV – bladder neck and urethra

Type 1 - Stretch injury

Figure 3-3-4

Figure 3-3-5

Type II – rupture above UGD Genitourinary Radiology

573

Urinary Tract Trauma

Figure 3-3-7

Figure 3-3-6

Type III – rupture above and below UGD

Type V – Anterior urethral trauma [Figures 3-3-9 and 3-3-10] • •

•

Bony injury uncommon Partial/complete ➢ Corpora/venous extravasation Associated scrotal trauma

Figure 3-3-9

Figure 3-3-8

Figure 3-3-10 Type IV – Bladder neck and urethra

Type V – Anterior urethral trauma (straddle injury)

Complications • •

Strictures Fistulas

• • •

Blunt or penetrating 5%–10% of pubic rami fx Pelvic fractures in 80% of ruptures ➢ 83% of extraperitoneal ➢ 62% of intraperitoneal

Bladder Trauma

Bladder Trauma Evaluation •

•

Standard cystogram ➢ 300–500cc incomplete filling may miss leak ➢ 15%–30% I concentration ➢ AP, obliques ➢ post drainage important for small leaks CT ➢ clamp foley ➢ delayed images, post drain

Urinary Tract Trauma

574

Genitourinary Radiology

Extraperitoneal Bladder Rupture [Figures 3-3-11 and 3-3-12]

• • •

60% focal extravasation, “flame-shaped” conservative therapy

Figure 3-3-11

Figure 3-3-12

Intraperitoneal Bladder Rupture [Figures 3-3-13 and 3-3-14]

• • •

40% free flowing extravasation, outlines intraperitoneal organs surgical therapy

Bladder Trauma Evaluation [Figure 3-3-15 and 3-3-16]

•

CT cystogram ➢ perform routine CT ➢ drain bladder ➢ refill with 2%–3% I solution (300 cc) ➢ scan full and post drain

Figure 3-3-15

Extaperitoneal bladder rupture

Figure 3-3-13

Figure 3-3-14

Intraperitoneal bladder rupture

Figure 3-3-16

Intraperitoneal bladder rupture on CT cystogram

Ureteral Injury • • •

Least common site of injury (<3%) Penetrating trauma – anywhere UPJ disruption

Genitourinary Radiology

Extraperitoneal bladder rupture on CT cystogram

575

Urinary Tract Trauma

Renal Injuries

[Figures 3-3-17 to 3-3-21]

•

•

Category I - Minor (85%) ➢ contusion ➢ intrarenal hematoma ➢ small subcapsular/perirenal hematoma ➢ segmental infarction ➢ superficial laceration Conservative management

Figure 3-3-17

Figure 3-3-18

Figure 3-3-19

Hematoma

Figure 3-3-20

Subcapsular hematoma with delayed nephrogram

Figure 3-3-21

Renal Injuries [Figures 3-3-22 and 3-3-23] •

•

Subcapsular hematoma has an abrupt start and stop point and deforms renal parenchyma

Category II - Serious (10%) ➢ deep lacerations ➢ laceration through the collecting system ➢ large perinephric/subcapsular hematoma Conservative management vs. surgery

Figure 3-3-22

Laceration breaks through renal capsule and causes a perinephric hematoma

Figure 3-3-23

Segmental infarction

Laceration into the collecting system Urinary Tract Trauma

Laceration into the collecting system with urine leak 576

Genitourinary Radiology

Renal Injuries [Figures 3-3-24 to 3-3-30] •

•

Category III - Catastrophic ➢ fractured/shattered kidney ➢ renal artery occlusion/avulsion ➢ renal vein occlusion/avulsion ➢ UPJ avulsion Often surgical treatment

Figure 3-3-24

Figure 3-3-25

Figure 3-3-26

Shattered kidney

Vascular avulsion

Figure 3-3-29

Vascular avulsion with contrast extravasation

Figure 3-3-27 Figure 3-3-28 Acute arterial thrombosis with subsequent development of a rim sign

Figure 3-3-30

Renal artery thrombosis. Non-functioning normal sized kidney

Rim sign: collateral circulation forming after thrombosis

Renal vein thrombosis. Kidney will be enlarged Genitourinary Radiology

577

Urinary Tract Trauma

UPJ disruption [Figures 3-3-31 and 3-3-32] • • • •

Deceleration injury 3:1, children:adults 3:1, R:L Stent, nephrostomy, surgery

Figure 3-3-31

Figure 3-3-32

UPJ disruption. Leak obvious on delayed films

UPJ disruption

Conclusion • • • •

If there is blood at the meatus, perform urethrogram first A normal bladder on CT does not rule out a leak Post-drainage films are key for small leaks Don’t forget delayed images

References 1.

Ali M, Safriel Y, Sclafani SJ, Schulze R. CT signs of urethral injury. Radiographics 2003; 23:951-963; discussion 963-956. 2. Blankenship B, Earls JP, Talner LB. Renal vein thrombosis after vascular pedicle injury[clin conference]. AJR Am J Roentgenol 1997; 168:1574. 3. Fishman EK, Horton KM. CT evaluation of bladder trauma: a critical look. Acad Radiol 2000; 7:309-310. 4. Goldman SM, Sandler CM, Corriere JN, Jr., McGuire EJ. Blunt urethral trauma: a unified, anatomical mechanical classification. J Urol 1997; 157:85-89. 5. Herschorn S, Radomski SB, Shoskes DA, Mahoney J, Hirshberg E, Klotz L. Evaluation and treatment of blunt renal trauma. J Urol 1991; 146:274-276; discussion 276-277. 6. Kamel IR, Berkowitz JF. Assessment of the cortical rim sign in posttraumatic renal infarction. J Comput Assist Tomogr 1996; 20:803-806. 7. Kawashima A, Sandler CM, Corriere JN, Jr., Rodgers BM, Goldman SM. Ureteropelvic junction injuries secondary to blunt abdominal trauma. Radiology 1997; 205:487-492. 8. Kawashima A, Sandler CM, Corl FM, et al. Imaging of renal trauma: a comprehensive review. Radiographics 2001; 21:557-574. 9. McAndrew JD, Corriere JN, Jr. Radiographic evaluation of renal trauma: evaluation of 1103 consecutive patients. Br J Urol 1994; 73:352-354. 10. Nunez D, Jr., Becerra JL, Fuentes D, Pagson S. Traumatic occlusion of the renal artery: helical CT diagnosis. AJR Am J Roentgenol 1996; 167:777-780. 11. Roberts JL. CT of abdominal and pelvic trauma. Semin Ultrasound CT MR 1996; 17:142-169.

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Retroperitoneum

Paula J. Woodward, MD

Figure 3-4-1

Retroperitoneum •

•

• •

Non-neoplastic ➢ Fluid collections ❖ Pancreatic, urinoma, hematoma, abscess ➢ Retroperitoneal fibrosis ➢ Extramedullary hematopoiesis Lymphadenopathy ➢ Inflammatory/infectious ➢ Castleman disease ➢ Lymphoma ➢ Metastatic adenopathy Organs ➢ Pancreas, colon, duodenum ➢ Kidneys, adrenal, ureters Primary ( > 100 benign and malignant tumors) ➢ Neurogenic ❖ Nerve sheath, ganglioneuroma, ganglioneuroblastoma, neuroblastoma ❖ Paraganglioma ➢ Mesenchymal ❖ Lipoma/sarcoma, leiomyoma/sarcoma, malignant fibrous histiocytoma (MFH), lymphangioma, hemangioma, hemangiopericytoma, angiosarcoma ➢ Germ cell ❖ Teratoma (benign and malignant)

Retroperitoneal spaces

Figure 3-4-2

Anatomy: 3 spaces [Figure 3-4-1] • • •

Anterior pararenal Perirenal Posterior pararenal

• • •

Colon (ascending and descending) Pancreas Duodenum (2nd and 3rd portions)

• • •

Kidneys Adrenal glands Upper portion of ureters

• •

No solid organs Fat, connective tissue, nerves

• •

Parietal peritoneum separates peritoneal space from APRS Anterior renal fascia separates APRS from perirenal space (Gerota’s fascia) Posterior renal fascia separates PPRS from perirenal space (Zuckerkandl’s fascia) Lateral conal fascia demarcates the lateral Sagittal view of the extent of the APRS retroperitoneum. All 3 Separates the APRS from the PPRS compartments All spaces communicate inferiorly communicate inferiorly

Updated view of the perirenal space with complex fascial boundaries

Anterior Pararenal Space (the GI space)

Figure 3-4-3

Perirenal Space (the GU space) [Figure 3-4-2] Posterior Pararenal Space (the nothing space) Borders [Figure 3-4-3] • • • •

Genitourinary Radiology

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Retroperitoneum

Retroperitoneal Fibrosis • • • •

Microscopically: collagen, fibroblasts, and inflammatory cells Typical distribution: below kidneys to bifurcation 40% may have atypical distribution 8%–10% malignant ➢ Desmoplastic reaction to infiltrating metastases ➢ Breast, lung, colon, prostate, cervix ➢ Prognosis poor (3-6 months)

Figure 3-4-4

Retroperitoneal Fibrosis: Etiology

• • • • • • • • •

2/3 idiopathic (Ormand’s Disease) Methysergide toxicity Aortic aneurysm Surgery Hemorrhage Inflammatory bowel disease Collagen vascular disease Radiation/surgery Fibrosing conditions elsewhere

•

IVP, retrogrades ➢ Medial deviation of ureters ➢ Hydronephrosis

Retroperitoneal Fibrosis [Figure 3-4-4] Retroperitoneal Fibrosis [Figure 3-4-5] •

•

CT ➢ Wispy plaque-like deposits to confluent masses ➢ Enhancement variable ➢ Aorta is encased but not deviated MR ➢ Fibrotic phase ❖ Low on T1 and T2 ❖ No enhancement ➢ Active phase ❖ High on T2 ❖ Enhancement ❖ Can not rule out malignancy ❖ Must biopsy

RPF with medial deviation of the ureters and hydronephrosis

Figure 3-4-5

Treatment • • • •

Stents Steroids Immunosuppression Surgery

•

Nerve sheath ➢ Schwannoma (neurilemmoma), neurofibroma, malignant nerve sheath tumor Ganglionic ➢ Ganglioneuroma, ganglioneuroblastoma, neuroblastoma Paraganglionic ➢ Paraganglioma (pheochromocytoma)

Neurogenic Tumors • •

Neurogenic Tumors • • • • •

Paraspinal masses Mass often elongated and well-defined RPF with low signal on T2WI Smooth or mildly lobular Generally benign Rapid growth, increased vascularity, poorly circumscribed suggest malignancy

Retroperitoneum

580

Genitourinary Radiology

Neurogenic Tumors • • • •

Low density on CT Low signal on T1WI May be hyperintense on T2WI (myxoid matrix) May have calcifications

• • • •

Often appears as psoas mass Look at neuroforamen May have intraspinal (extradural) extension Multiple consider neurofibromatosis

• •

Form from primitive neural crest cells Sympathoblast ➢ Neuroblastoma ➢ Ganglioneuroblastoma ➢ Ganglioneuroma Pheochromoblast ➢ Paraganglioma

Figure 3-4-6

Nerve sheath tumors [Figure 3-4-6]

Ganglion Cell Tumors [Figure 3-4-7]

•

Nerve sheath tumor presenting as a psoas mass

Figure 3-4-7

Ganglioneuroma • • • • •

Benign More common in mediastinum Generally asymptomatic Elongated low-density masses Maybe be hyperintense on T2WI

• •

About 10% of pheochromocytomas Most (60%–80%) have known catecholamine excess ➢ Hypertension, palpitations, sweating, tremor, diarrhea, nausea More commonly malignant than adrenal pheochromocytomas

Paraganglioma (Extra-adrenal pheochromocytoma)

•

Paraganglioma [Figure 3-4-8] • •

• •

Organs of Zuckerkandl CT non-specific ➢ Enhance avidly ➢ Contrast contraindicated High signal on T2 is suggestive but is not universally seen Uptake on MIBG scan

Figure 3-4-8

Sympathetic chain

Paraganglioma with high signal on T2WI Genitourinary Radiology

581

Retroperitoneum

Leiomyosarcoma [Figure 3-4-9] • • •

More commonly necrotic than other tumors May have intravascular invasion May arise in the wall of the IVC

Figure 3-4-9

Leiomyosarcoma with IVC invasion

MFH / Malignant Fibrous Histiocytoma [Figure 3-4-10] • • •

Generally large masses Necrosis less common T2WI helpful – “Bowl of fruit sign” ➢ Mosaic of low and high signal ❖ Fibrous tissue – low ❖ Myxoid stroma – hyperintense ❖ Soft tissue - intermediate

Lymphangioma • • • • •

Figure 3-4-10

[Figure 3-4-11]

Benign Fluid-filled Uni-multiloculated Insinuates itself around organs Can be huge

Figure 3-4-11

MFH with “bowl of fruit” on T2WI

Lymphangioma Retroperitoneum

582

Genitourinary Radiology

Liposarcoma [Figure 3-4-12] • • • •

Figure 3-4-12

The most common primary retroperitoneal tumor 85% have fat detected by CT or MR Well-differentiated, pleomorphic, myxoid, de-differentiated Poorly differentiated tumors have no detectable fat by imaging studies

Liposarcoma [Figure 3-4-13] • • •

Clinical presentation ➢ Often present late ➢ Weight gain Infiltrative margins Complete surgical excision may be difficult Local recurrence common

• • •

Neurogenic tumors Malignant fibrous histiocytoma Myxoid liposarcoma

•

Teratoma ➢ Mature ➢ Immature Malignant germ cell tumors

•

Hyperintense T2WI: Things with a myxoid matrix

Liposarcoma

Figure 3-4-13

Germ Cell Tumors •

Teratoma •

Most are mature (benign) and are cured by surgery Children (less than 6 months) and young adults (15–25 years) Female:male = 3:1

• • •

Fat (sebum or adipose tissue) Calcification in 90% (may be clump-like) Cystic portion in 75%

• •

Teratoma [Figures 3-4-14 and 3-4-15]

Figure 3-4-14

Myxoid liposarcoma with high signal on T2WI

Figure 3-4-15

Embryo with germ cells in the retroperitoneum Genitourinary Radiology

Retroperitoneal teratoma 583

Retroperitoneum

Malignant germ cell tumors are more common in males and are much more likely to be secondary to a testicular tumor (rather than primary retroperitoneal) Fat containing retroperitoneal masses • • • •

Liposarcoma: large, heterogeneous Teratoma: young patients, calcification, cystic area Myelolipoma: usually arising from adrenal Angiomyolipoma: arises from kidney, but attachment may be hard to find

1. 2. 3.

Engelken JD, Ros PR. Retroperitoneal MR imaging. Magn Reson Imaging Clin N Am 1997; 5:165-178. Granstrom P, Unger E. MR imaging of the retroperitoneum. Magn Reson Imaging Clin N Am 1995; 3:121-142. Kim T, Murakami T, Oi H, et al. CT and MR imaging of abdominal liposarcoma. AJR Am J Roentgenol 1996; 166:829833. Nishimura H, Zhang Y, Ohkuma K, Uchida M, Hayabuchi N, Sun S. MR imaging of soft-tissue masses of the extraperitoneal spaces. Radiographics 2001; 21:1141-1154. Nishino M, Hayakawa K, Minami M, Yamamoto A, Ueda H, Takasu K. Primary retroperitoneal neoplasms: CT and MR imaging findings with anatomic and pathologic diagnostic clues. Radiographics 2003; 23:45-57. Morton A. Meyers. Dynamic Radiology of the Abdomen. Springer-Verlag. A must read book in your residency

References

4. 5. 6.

Retroperitoneum

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Genitourinary Radiology

Radiologic Evaluation of the Scrotum Paula J. Woodward, MD Embryology • • •

Sex is chromosomally determined at fertilization No morphologic differentiation until week 7 (“indifferent stage”) Testis determining factor on short arm of Y chromosome ➢ induces formation of seminiferous tubules

Figure 3-5-1

3 Components of Gonad [Figure 3-5-1 to 3-5-4] •

• •

Germ cells ➢ arise from yolk sac ➢ migrate to genital ridges Mesothelium ➢ primitive sex cord ➢ Sertoli cells Mesenchyme ➢ Interstium ➢ Leydig cells

Migration of germ cells along the hindgut to the genital ridges

Figure 3-5-3

Figure 3-5-2

Normal seminiferous tubules

Figure 3-5-4

Embryologic formation of the testes

• •

Leydig cells secrete testosterone ➢ stimulate mesonephric ducts Sertoli cells secrete mullerian-inhibiting factor

Sex organs at indeterminate stage Genitourinary Radiology

585

Radiologic Evaluation of the Scrotum

Mesonephric (Wolffian) Ducts • • • •

Epididymis Vas deferens Ejaculatory ducts Seminal vesicles

•

Mullerian ➢ appendix testis Wolffian ➢ appendix epididymis

Figure 3-5-5

Embryologic Remnants •

Testis [Figure 3-5-5] • • •

200-300 lobules Seminiferous tubules (300-980 meters) Efferent ductules (15-20) converge at mediastinum

• • •

Form single convoluted tubule in head (600 cm) Tail loosely attached inferiorly Exits as single tube

• • • •

Vas deferns Testicular, deferential, cremasteric arteries Pampiniform plexus Nerves, lymphatics

•

Testes ➢ homogeneous low level echoes ➢ linear echogenic mediastinum testis Epididymis ➢ globus major (head), body, tail ➢ iso- to slightly hyperechoic

Epididymis

Normal testis

Spermatic cord [Figure 3-5-6]

Figure 3-5-6

Ultrasound •

MRI • •

T1WI - homogeneous intermediate signal T2WI - high signal with low signal mediastinum testis and linear septations

MRI [Figure 3-5-7] •

Tumors are low signal masses

• •

Intra-testicular vs. extra-testicular Cyst vs. solid

• •

Germ Cell Neoplasms (95%) Sex cord, Stromal Tumors ➢ Sertoli cell ➢ Leydig cell Lymphoma Metastases Epidermoid Cysts

Spermatic cord

Goals of Ultrasound

Figure 3-5-7

Testicular Neoplasms

• • •

Testicular tumors are low signal on T2WI Radiologic Evaluation of the Scrotum

586

Genitourinary Radiology

Germ Cell Neoplasms • • • • • •

Figure 3-5-8

Seminoma (most common “pure” tumor) Embryonal Cell Carcinoma Yolk Sac Tumor (Endodermal Sinus Tumor) Teratoma Choriocarcinoma MGCT - Mixed Germ Cell Tumor (most common overall)

Seminoma [Figure 3-5-8] • • • • • •

Homogeneous, well-defined May be lobular and multifocal Bilateral 2% Peak age 30-40 years Radiosensitive Good prognosis

•

Embryonal ➢ Rare in pure form ➢ 87% of MGCT Yolk Sac (endodermal sinus tumor) ➢ Most common childhood tumor ➢ 44% of MGCT Teratoma ➢ Mature and immature (always malignant in adults) ➢ Cysts/calcifications common features Choriocarcinoma ➢ Very rare ➢ Dismal prognosis

Non Seminomatous Germ Cell Tumor [Figure 3-5-9] • • •

Seminoma

Figure 3-5-9

Non Seminomatous Germ Cell Tumor • • • • •

MGCT with large amount of teratoma Mixed germ cell tumors much more common than any pure tumor Heterogeneous, ill-defined Peak age 20’s Not radiosensitive Often higher stage and less favorable prognosis than seminoma

Germ Cell Tumors: Modes of Spread •

•

Lymphatic ➢ ipsilateral renal hilum Hematogeous ➢ common with choriocarcinoma ➢ lung, liver, brain

Tumor Markers •

• •

•

Alpha-fetoprotein (AFP) ➢ from fetal liver, GI tract, and yolk sac ➢ elevated in tumors with yolk sac elements Human chorionic gonadotropin (HCG) ➢ produced by syncytiotrophoblasts from developing placenta ➢ elevated in tumors with choriocarcinoma (occasional seminoma) LDH ➢ Non-specific, correlates with bulk of disease Elevated in 80% of non-seminomatous tumors

Genitourinary Radiology

587

Radiologic Evaluation of the Scrotum

Burned-Out Germ Cell Tumor •

Presents with metastases (often extensive) The primary may not contain any active tumor and may be difficult to identify Orchiectomy performed if any suspicious area seen

• • • •

0.6% in general population Present in 40% of germ cell tumors Usually bilateral Consider annual screening

• • • • • •

Prior testicular tumor Cryptorchidism Infertility Family history Intersex syndormes (hermaphrodite) ??? microcalcifications

• • • • •

5% testicular agenesis 65% migratory testis 30% undescended Increased incidence of malignancy Risk is also increased in the contralateral testis

• • • •

Sertoli (sex cord) and Leydig (stromal) 90% benign More common in pediatric age group May be be hormonally active ➢ precocious puberty, gynecomastia ➢ more common with Leydig Sertoli cell tumors may be bilateral and calcified

• •

Figure 3-5-10

Testicular Microlithiasis

Risk Factors for Testicular Carcinoma Bilateral undescended testes

Figure 3-5-11

Cryptorchidism [Figure 3-5-10]

Sex Cord, Stromal Tumors [Figure 3-5-11]

•

Sertoli cell tumor with calcification

Figure 3-5-12

Testicular Lymphoma − Presentation • • • •

Most common testis tumor > 60 yo 5% of testicular neoplasms < 1% of patients with lymphoma Often presents as the site of recurrent disease because of “blood-testis barrier” (Sertoli cells)

Testicular Lymphoma − Imaging [Figure 3-5-12] • • •

Most common bilateral tumor Homogeneous Epididymis and spermatic cord often involved

• • • • • •

Benign ? germ cell tumor Filled with keratin Well-defined Ringed-appearance Can not differentiate from a malignant neoplasm ➢ May do focal resection rather than orchiectomy

Epidermoid Cyst [Figure 3-5-13]

Lymphoma with bilateral testicular masses

Figure 3-5-13

Concentric rings in a epidermoid cyst Radiologic Evaluation of the Scrotum

588

Genitourinary Radiology

Tumors Summary •

• • •

Children ➢ Yolk sac tumor ➢ Sertoli, Leydig cell Younger men (20’s) ➢ Mixed germ cell tumor ➢ Heterogeneous, poorly defined Somewhat older (30s) ➢ Seminoma ➢ Homogeneous Older males (> 60 yo) ➢ Lymphoma ➢ Bilateral ➢ May involve paratesticular structures

Figure 3-5-14

Non-neoplastic Testicular Masses • • • • •

Tubular ectasia Cysts Sarcoidosis Adrenal rests Acute scrotum ➢ infection ➢ infarction ➢ trauma

Tubular ectasia with an intratesticular cyst

Figure 3-5-15

Tubular Ectasia

• • • • •

Dilatation of the rete testis Often bilateral Associated with a spermatocele Tubular US appearance Iso- to hyperintense on T2WI

•

Peripheral ➢ Tunica albuginea cyst Central ➢ Must be careful to differentiate from cystic neoplasm ➢ Can not have any solid component ➢ Often associated with dilated rete testis

Testicular Cysts [Figure 3-5-14] •

Sarcoidosis

[Figure 3-5-15]

• • • •

Multisystem chronic granulomatous disorder 5% will have genital involvement Epididymis more commonly involved More common in Blacks (testicular tumors are rare)

• • • •

Adrenal rests in 7.5-15% of newborns, 1.6% adults Hypertrophy when exposed to elevated ACTH Bilateral, multiple, eccentric Tx – glucocorticoids not orchiectomy

• • • • •

Lymphoma Seminoma (rarely) Metastases Sarcoidosis Adrenal rests

Sarcoidosis with multiple testicular masses

Figure 3-5-16

Adrenal rest hypertrophy secondary to congenital adrenal hyperplasia [Figure 3-5-16]

Bilateral Testicular Masses

Genitourinary Radiology

Developing adrenals. Adrenal tissue may become entrapped within the developing testis 589

Radiologic Evaluation of the Scrotum

Extratesticular Scrotal Masses [FigureS 3-5-17 and 3-5-18] Figure 3-5-17

Figure 3-5-18

Adult scrotum. Processus vaginalis closes to form tunica vaginalis

Developing scrotum. Testicular descent is aided by the processus vaginalis

Hydrocele • •

Fluid between the parietal and visceral layers of the tunica vaginalis Small amount is normal

•

Congenital ➢ Patent processus vaginalis, may have an inguinal hernia Acquired ➢ Infection, infarction, trauma, tumor

Hydrocele •

Figure 3-5-19

Scrotal Calculi • •

• •

Torsion of appendix or inflammatory deposits Repeated microtrauma ➢ bikers Variable size and calcification Mobile

Epididymal Masses

•

Cyst, Spermatocele [Figure 3-5-19] Infection ➢ Bacterial (acute) ➢ TB (chronic) Tumors ➢ Adenomatoid tumor ➢ Papillary cystadenoma (von Hippel-Lindau) ➢ Lymphoma Sarcoidosis

• • •

Benign Most common epididymal tumor Solid, small, well-circumscribed

• • •

Associated with VHL (70%) 40% bilateral Benign

• •

•

Epididymal cyst

Figure 3-5-20

Adenomatoid Tumor [Figure 3-5-20] Papillary Cystadenoma

Radiologic Evaluation of the Scrotum

Adenomatoid tumor 590

Genitourinary Radiology

Epididymal and Testicular Mass •

•

•

Figure 3-5-21

Lymphoma ➢ Testicular involvement greater than epididymis Sarcoidosis [Figure 3-5-21] ➢ Epididymal involvement greater than testis ➢ More common in Blacks Infection ➢ Bacterial (acute) ➢ TB (chronic)

Tuberculosis • •

Epididymis primary site with testis secondarily involved 30% bilateral 50% will have abscess or fistulas

• • •

Trauma Epididymitis/orchitis Torsion

• • •

Bacterial infection from lower urinary tract - chlamydia, gonococcus, E coli US findings - enlarged, hypoechoic, hyperemia, hydrocele, skin thickening 20% have associated orchitis

• • • •

Usually secondary to epididymitis May rarely be focal US findings - enlarged, heterogeneous echogenicity, hyperemia May lead to focal ischemia/infarction

• • •

Diabetics or other immunosuppression Scrotal abscess with necrotizing infection of the perineum Surgical emergency

• •

• •

Gray scale US may be normal early Decreased or absent flow with Doppler ➢ Compare with normal side ➢ Venous compromise occurs first Look for mass in inguinal canal Testis becomes enlarged and hypoechoic with time

• •

< 6 hrs at diagnosis salvage rate 80%-100% 12 hr salvage rate 20%

• • • •

Varicocele Fibrous pseudotumors Polyorchidism Neoplasms ➢ Lipomas ✧ Half of all spermatic cord tumors ➢ Liposarcoma ➢ Rhabdosarcoma, leiomyosarcoma, MFH ➢ Mesothelioma

•

Sarcoidosis with marked epididymal enlargement

Acute Scrotum

Acute Epididymitis Orchitis

Fournier Gangrene Torsion

Torsion

Paratesticular masses

Genitourinary Radiology

591

Radiologic Evaluation of the Scrotum

Varicocele [Figure 3-5-22] • •

•

Figure 3-5-22

> 3mm Idiopathic ➢ incompetent valves ➢ more common on left (bilateral 10%) ✧ longer course ✧ more perpendicular insertion ✧ “nutcracker” effect of left renal vein under SMA Secondary to abdominal mass

Varicocele • • • •

15% of general population 40% of men with infertility ? increased temperature Improved pregnancy rates (35%-50%) with repair even if subclinical

Fibrous Pseudotumor [Figure 3-5-23] • • • •

Hylanized collagen and granulation tissue Attached to tunica albuginea US non-specific MRI low signal intensity

• • •

Abnormal division of genital ridge Often abnormal spermatogenesis Increased risk of torsion

• • • • • •

Lipomas [Figure 3-5-24] Liposarcoma [Figure 3-5-25] Rhabdosarcoma Leiomyosarcoma MFH Mesothelioma

• • •

Most common extratesticular neoplasm Half of all cord tumors Variable by ultrasound ➢ may be homogenously hypoechoic MR with fat suppression helpful

Large varicocele

Figure 3-5-23

Polyorchidism

Paratesticular Neoplasms

Lipoma [Figure 3-5-24]

•

Fibrous pseudotumor. Round lowsignal mass involving the tunica albuginea

Figure 3-5-24

Figure 3-5-25

Liposarcoma

Hypoechoic lipoma Radiologic Evaluation of the Scrotum

592

Genitourinary Radiology

Mesothelioma [Figure 3-5-26] • • • •

Figure 3-5-26

Tunica vaginalis lined with mesothelial cells Much less common then pleural or peritoneal Benign and malignant Often present with hydrocele

Mesothelioma with nodules and hydrocele References 1. 2. 3. 4. 5.

6. 7. 8. 9.

10. 11.

12. 13. 14. 15. 16. 17. 18. 19. 20. 21.

Black JA, Patel A. Sonography of the abnormal extratesticular space. AJR Am J Roentgenol 1996; 167:507-511. Black JA, Patel A. Sonography of the normal extratesticular space. AJR Am J Roentgenol 1996; 167:503-506. Bostwick DG. Spermatic cord and testicular adnexa. In: Bostwick DG, Eble JN, eds. Urologic surgcial pathology. St. Louis: Mosby, 1997; 647-674. Chung JJ, Kim MJ, Lee T, Yoo HS, Lee JT. Sonographic findings in tuberculous epididymitis and epididymoorchitis. J Clin Ultrasound 1997; 25:390-394. Cramer BM, Schlegel EA, Thueroff JW. MR imaging in the differential diagnosis of scrotal and testicular disease. Radiographics 1991; 11:9-21. Doherty FJ. Ultrasound of the nonacute scrotum. Semin Ultrasound CT MR 1991; 12:131-156. Feuer A, Dewire DM, Foley WD. Ultrasonographic characteristics of testicular adenomatoid tumors. J Urol 1996; 155:174-175. Frates MC, Benson CB, DiSalvo DN, Brown DL, Laing FC, Doubilet PM. Solid extratesticular masses evaluated with sonography: pathologic correlation. Radiology 1997; 204:43-46. Geraghty MJ, Lee FT, Jr., Bernsten SA, Gilchrist K, Pozniak MA, Yandow DJ. Sonography of testicular tumors and tumor-like conditions: a radiologic-pathologic correlation. Crit Rev Diagn Imaging 1998; 39:1-63. Grebenc ML, Gorman JD, Sumida FK. Fibrous pseudotumor of the tunica vaginalis testis: imaging appearance. Abdom Imaging 1995; 20:379-380. Heaton ND, Hogan B, Michell M, Thompson P, Yates-Bell AJ. Tuberculous epididymo-orchitis: clinical and ultrasound observations. Br J Urol 1989; 64:305-309. Horstman WG, Middleton WD, Melson GL. Scrotal inflammatory disease: color Doppler US findings. Radiology 1991; 179:55-59. Kassis A. Testicular adenomatoid tumours: clinical and ultrasonographic characteristics. BJU Int 2000; 85:302304. Kim ED, Lipshultz LI. Role of ultrasound in the assessment of male infertility. J Clin Ultrasound 1996; 24:437453. Kutchera WA, Bluth EI, Guice SL. Sonographic findings of a spermatic cord lipoma. Case report and review of the literature. J Ultrasound Med 1987; 6:457-460. Mattrey RF. Magnetic resonance imaging of the scrotum. Semin Ultrasound CT MR 1991; 12:95-108. Ragheb D, Higgins JL, Jr. Ultrasonography of the scrotum: technique, anatomy, and pathologic entities. J Ultrasound Med 2002; 21:171-185. Sudakoff GS, Quiroz F, Karcaaltincaba M, Foley WD. Scrotal ultrasonography with emphasis on the extratesticular space: anatomy, embryology, and pathology. Ultrasound Q 2002; 18:255-273. Tessler FN, Tublin ME, Rifkin MD. Ultrasound assessment of testicular and paratesticular masses. J Clin Ultrasound 1996; 24:423-436. Woodward PJ, Schwab CM, Sesterhenn IA. From the archives of the AFIP: extratesticular scrotal masses: radiologic-pathologic correlation. Radiographics 2003; 23:215-240. Woodward PJ, Sohaey R, O'Donoghue MJ, Green DE. From the Archives of the AFIP: Tumors and Tumorlike Lesions of the Testis: Radiologic-Pathologic Correlation. Radiographics 2002; 22:189-216.

Genitourinary Radiology

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Radiologic Evaluation of the Scrotum

First Trimester Ultrasound Paula J. Woodward, MD Ovarian Period: (Weeks 1–2) • • •

Ovarian follicle matures Ovulation Corpus luteum formation

• • • •

Fertilization Morula (16 cells) Blastocyst Trilaminar embryo

• • •

C-shaped embryo Major organs develop Yolk sac detaches

• •

Fetal growth Amniotic and chorionic membranes approach each other

• • •

Visualized as early as 4 - 4.5 wks (TV) Intradecidual sac sign [Figure 3-6-1] Double decidual sac sign [Figure 3-6-2] ➢ Basalis [DB] ➢ Capsularis [DC] ➢ Parietalis [DP]

Figure 3-6-1

Conceptus Period: (Week 3–5)

Embryonic Period: (Weeks 6–10) Fetal Period: (Weeks 11–12)

Intradecidual sac sign

Gestational Sac

Gestational Sac = Chorionic Sac [Figures 3-6-3 and 3-6-4]

• •

Chorionic laeve = Smooth chorion = Chorionic membrane Chorionic frondosum + Decidua basalis = Placenta

The sac is eccentrically located with respect to the endometrial cavity

Figure 3-6-2 Figure 3-6-3

Double decidual sac sign

Chorionic sac with chorionic frondosum and laeve (smooth chorion) First Trimester Ultrasound

594

Genitourinary Radiology

Figure 3-6-4

Series of 5 illustrations showing normal 1st trimester development with expansion of the amnion and detachment of the yolk sac

a

b

c

d

e

Genitourinary Radiology

595

First Trimester Ultrasound

Yolk sac [Figure 3-6-5 ] •

Visualized at 5 – 5.5 weeks

•

Visualized 6 – 6.5 wks

• • •

Gestational sac Yolk sac Embryo

•

Types of Twinning ➢ Dizygotic (70%) ✧ 2 eggs ➢ Monozygotic (70%) ✧ single egg

Figure 3-6-5

Embryo [Figure 3-6-6 ]

Major First Trimester Landmarks [Figures 3-6-7 and 3-6-8] 4.5 wks 5.5 wks 6.5 wks

Multiple Gestations

Figure 3-6-6

Normal yolk sac at 5.5 wks

Figure 3-6-7

Multiple Gestations •

•

# of chorions equals # of placentas ➢ sharing is bad Normal 6.5 week embryo with ➢ risk for twin/twin “double bleb” or “diamond ring” sign transfusion # of amnions equals # of separate sacs ➢ sharing is really bad ➢ risk for cord accidents

Dizygotic Twins [Figure 3-6-9] •

Dizygotic must be dichorionic (2 placentas) and diamniotic (2 sacs)

8 week embryo surrounded by amnion

Figure 3-6-8

Figure 3-6-9

Normal first trimester US including rhombencephalon and bowel herniation

Dichorionic, diamniotic twins

First Trimester Ultrasound

596

Genitourinary Radiology

Monozygotic Twins • • •

•

1/3 are Dichorionic/Diamniotic [Figure 3-6-10] ➢ cleavage by day 3 2/3 are Monochorionic/Diamniotic [Figures 3-6-11 and 3-6-12] ➢ cleavage day 4-8 Rare (approx 1%) Monochorionic/Monoamniotic [Figure 3-6-13]] ➢ cleavage > 8 days Conjoined twins ➢ cleavage > 14 days

Figure 3-6-10

Figures 3-6-11 and 3-6-12

Dichorionic, diamniotic twins

Figure 3-6-13

Monochorionic, monoamniotic twins

Monochorionic, diamniotic twins

AIUM Guidelines: First Trimester •

• •

• • •

Gestational sac ➢ Location ➢ Mean Sac Diameter (MSD) MSD = (L+W+D)/3 ➢ Yolk sac Embryo ➢ Crown rump length ➢ Cardiac activity Fetal number ➢ Chorionicity/Amnionicity Uterus, adnexa, cul-de-sac Threshold Level - the size at which a finding may be seen Discriminatory Level - the size at which a finding must be seen

Genitourinary Radiology

597

First Trimester Ultrasound

• •

Gestational Sac Yolk Sac

•

Heartbeat

•

Embryo

Threshold Level – MSD 2 mm TV 4 mm TV 8 mm

2 mm (CRL)

Major: Discriminators

Discriminatory Level – MSD

5 mm TV10 mm TA 20 mm TV 18 mm TA 25 mm 5 mm (CRL)

• • •

MSD > 10 mm must have a yolk sac MSD > 18 mm must have an embryo CRL > 5mm must have a heartbeat

• • •

Must be present if embryo is > 5 mm 5–6 weeks 100–110 bpm 8–9 weeks 150–170 bpm

• • • • • • •

25% threatened abortion Embryonic demise Bradycardia Anembryonic pregnancy [Figure 3-6-14] Perigestational hemorrhage [Figure 3-6-15] Abnormal yolk sac Poor growth

•

Major discriminators ➢ MSD > 10 mm without a yolk sac ➢ MSD > 18 mm without a fetal pole Minor discriminators ➢ weak decidual reaction ➢ abnormal shape or location ➢ empty amnion

Cardiac Activity

Abnormal Frist Trimester

Figure 3-6-14

Anembryonic Pregnancy [Figure 3-6-14] •

Yolk Sac • • •

First landmark in gestational sac In the chorionic cavity Abnormal findings: ➢ >6mm ➢ irregular shape ➢ calcifications ➢ multiple yolk sacs

Anembryonic pregnancy with empty amnion

Figure 3-6-15

Growth •

Normal growth rate 1 mm per day

• •

Tubal 95% Unusual locations 5% ➢ Interstitial ➢ Cervix ➢ Ovary ➢ Abdominal 1:50-1:200 live births Risks factors: IUD, prior ectopic, PID, tubal surgery, infertility treatment

Ectopic pregnancy

• •

First Trimester Ultrasound

598

Perigestational hemorrhage Genitourinary Radiology

Ectopic pregnancy: Uterine Findings

Figure 3-6-16

[Figures 3-6-16 and 3-6-17]

• • • •

No gestational sac Thickened endometrium Pseudogestional sac Discriminatory hCG levels ➢ hCG >1,000 IU/L (2nd IS) ➢ hCG >2,000 IU/L (3rd IRP)

Figure 3-6-17

Double decidual sac sign vs. pseudosac

Figure 3-6-18

Ectopic with echogenic ring and blood in the cul-de-sac

Pseudosac

Ectopic Pregnancy: Adnexal Findings [Figure 3-6-18]

• • •

Living extrauterine embryo Echogenic ring +/- yolk sac ➢ “ring of fire” Adnexal mass (clot) Cul-de-sac blood Normal adnexa

• •

1 in 30,000 spontaneous pregnancies 1 in 4,000 assisted pregnancies

• • • • •

Isthmus Rupture later – catastrophic bleeding Eccentric Lack of encircling myometrium Interstitial line sign

• •

Figure 3-6-19

Heterotopic Pregnancy

Interstitial Pregnancy [Figure 3-6-19]

Cornual ectopic with interstitial line sign

Genitourinary Radiology

599

First Trimester Ultrasound

Management of Ectopic Pregnancy •

•

Surgical ➢ Salpingectomy ➢ Salpingostomy Medical ➢ Systemic methotrexate ➢ Intragestational methotrexate ➢ Intragestational KCI

Systemic Methotrexate • • • •

Preserves fallopian tube Non-invasive Outpatient Criteria ➢ Mass < 4cm ➢ No bleeding ➢ hCG <3,000 IU/L (2IS) ➢ No formed fetal parts

Figure 3-6-20

Cutting Edge • • •

Sonoembryology Early diagnosis of major malformations Screen for aneuploidy ➢ nuchal translucency ➢ hypoplastic nasal bone ➢ abnormal flow in ductus venosus

Nuchal Translucency Screen for Trisomy 21 [Figure 3-6-20]

• • • • •

Accredited lab 11-14 weeks >3mm abnormal Risk assessment based on age, NT, serum screen High detection rates (75%-90%)

Increased nuchal translucency in Down syndrome

References 1.

Ackerman TE, Levi CS, Dashefsky SM, Holt SC, Lindsay DJ. Interstitial line: sonographic finding in interstitial (cornual) ectopic pregnancy. Radiology 1993; 189:83-87. 2. Brown DL, Emerson DS, Felker RE, Cartier MS, Smith WC. Diagnosis of early embryonic demise by endovaginal sonography. J Ultrasound Med 1990; 9:631-636. 3. Brown DL, Doubilet PM. Transvaginal sonography for diagnosing ectopic pregnancy: positivity criteria and performance characteristics. J Ultrasound Med 1994; 13:259-266. 4. Dickey RP, Olar TT, Curole DN, Taylor SN, Matulich EM. Relationship of first-trimester subchorionic bleeding detected by color Doppler ultrasound to subchorionic fluid, clinical bleeding, and pregnancy outcome. Obstet Gynecol 1992; 80:415-420. 5. Frates MC, Brown DL, Doubilet PM, Hornstein MD. Tubal rupture in patients with ectopic pregnancy: diagnosis with transvaginal US. Radiology 1994; 191:769-772. 6. Frates MC, Benson CB, Doubilet PM, et al. Cervical ectopic pregnancy: results of conservative treatment. Radiology 1994; 191:773-775. 7. Frates MC, Laing FC. Sonographic evaluation of ectopic pregnancy: an update. AJR Am J Roentgenol 1995; 165:251259. 8. Jarjour L, Kletzky OA. Reliability of transvaginal ultrasound in detecting first trimester pregnancy abnormalities. Fertil Steril 1991; 56:202-207. 9. Jurkovic D, Gruboeck K, Campbell S. Ultrasound features of normal early pregnancy development. Curr Opin Obstet Gynecol 1995; 7:493-504. 10. Nyberg DA, Mack LA, Laing FC, Patten RM. Distinguishing normal from abnormal gestational sac growth in early pregnancy. J Ultrasound Med 1987; 6:23-27. First Trimester Ultrasound

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Genitourinary Radiology

11. Nyberg DA, Filly RA, Laing FC, Mack LA, Zarutskie PW. Ectopic pregnancy. Diagnosis by sonography correlated with quantitative HCG levels. J Ultrasound Med 1987; 6:145-150. 12. Oh JS, Wright G, Coulam CB. Gestational sac diameter in very early pregnancy as a predictor of fetal outcome. Ultrasound Obstet Gynecol 2002; 20:267-269. 13. Rempen A. Diagnosis of viability in early pregnancy with vaginal sonography. J Ultrasound Med 1990; 9:711-716. 14. Sohaey R, Woodward P, Zwiebel WJ. First-trimester ultrasound: the essentials. Semin Ultrasound CT MR 1996; 17:214. 15. van Leeuwen I, Branch DW, Scott JR. First-trimester ultrasonography findings in women with a history of recurrent pregnancy loss. Am J Obstet Gynecol 1993; 168:111-114.

Genitourinary Radiology

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First Trimester Ultrasound

Fetal CNS Malformations Paula J. Woodward, MD AIUM: Fetal Brain [Figure 3-7-1] •

• •

Figure 3-7-1

Ventricular Plane ➢ atrium and choroid BPD Plane ➢ thalami ➢ third ventricle ➢ cavum septi pellucidi Posterior Fossa ➢ cerebellum ➢ cisterna magna ➢ nuchal skin thickeness

Fetal MRI • •

Fast T2WI (SSFSE, HASTE) Safety issues ➢ No known deleterious effects ➢ Do not perform in the first trimester ➢ Do not give gadolinium ➢ Obtain informed consent

Congenital CNS Malformations •

• • •

Dorsal Induction ➢ anencephaly ➢ encephalocele ➢ spina bifida Ventral Induction ➢ holoprosencephaly ➢ Dandy-Walker malformation Neuronal Proliferation ➢ microcephaly ➢ macrocephaly ➢ tumors Migration ➢ agenesis of corpus callosum

3 required images of the fetal brain

There is too much fluid in there • • •

Hydrocephalus Holoprosencephaly Hydranencephaly

• •

Absent cerebral hemispheres Occlusion of ICA -? etiology ➢ infection ➢ vasculitis ➢ emboli Falx Normal facial development

Hydranencephaly

• •

Fetal CNS Malformations

602

Genitourinary Radiology

Holoprosencephaly [Figures 3-7-2 to 3-7-4] •

• • •

Spectrum of arrested development ➢ alobar ➢ semilobar ➢ lobar Absent cavum, absent falx, fused thalami, dorsal sac Midline facial defects ➢ proboscis ➢ cyclopia ➢ midline cleft Trisomy 13

Figure 3-7-3

Figure 3-7-2

Alobar, semilobar lobar holoprosencephaly compared to normal.

Alobar holoprosencephaly with single ventricle

Figure 3-7-4

Hydrocephalus •

Dilated ventricles and enlarged head

•

Dilated ventricles

• • •

Lateral ventricle > 10mm Medial ventricular wall to choroid > 3mm Dangling choroid

• • • •

Aqueductal Stenosis Dandy-Walker Malformation Chiari II Communicating Hydrocephalus

• • •

Block at aqueduct of Sylvius Most common cause of hydrocephalus Male predominance (X-linked form)

Ventriculomegaly Signs

Hydrocephalus: Differential

Semilobar holoprosencephaly with a dorsal sac. Face shows a midline cleft.

Figure 3-7-5

Aqueductal Stenosis [Figures 3-7-5]

Aqueductal stenosis with dilatation of the lateral and third ventricles. Genitourinary Radiology

603

Fetal CNS Malformations

Dandy-Walker Malformation [Figures • • •

Communicating PF cyst Hydrocephalus +/50 have accociated abnormality

3-7-6 and 3-7-7]

Figure 3-7-6

Chiari II [Figures 3-7-8 to 3-7-10] • • • •

Figure 3-7-7

Dandy-Walker Malformation with enlargement of the 4th ventricle and posterior fossa cyst.

Dandy-Walker Malformation

Downward herniation of the 4th ventricle and vermis Myelomeningocele Hydrocephalus Normal “Lemon” and “Banana” sign

Chiari II

Figure 3-7-8

3-7-9

3-7-10

Fetal CNS Malformations

Normal vs. Chiari II 604

Genitourinary Radiology

Communicating Hydrocephalus [Figure 3-7-11] • •

Dilatation of all ventricles and subarachnoid space Etiology ➢ hemorrhage ➢ ? abnormal arachnoid granulations ➢ ? abnormal superior sagittal sinus

Figure 3-7-11

Hydrocephalus: Differential • • • •

Aqueductal Stenosis Dandy-Walker Malformation Chiari II Communicating Hydrocephalus

• • • •

Tear-dropped shaped ventricules (Colpocephaly) Absent cavum septi pellucidi Associated with lipomas and arachnoid cysts Often missed or confused with mild ventriculomegaly

• • • •

Anencephaly Spina Bifida Encephalocele Acrania

• •

Absent cranium and cerebral hemispheres Area cerebrovasculosa

Agenesis of the Corpus Callosum [Figure 3-7-12]

Neural Tube Defects

Communicating Hydrocephalus

Figure 3-7-12

Anencephaly [Figure 3-7-13] Figure 3-7-13

Agenesis of the CC with colpocephaly and arachnoid cyst

Figure 3-7-14

1st trimester anencephaly

Encephalocele [Figure 3-7-14] • • • •

75% occipital Frontal – Southeast Asia Evaluate brain tissue 80% have associated malformations

• • •

30% of trisomy 18 1%–2% of normals 1/500 chance of trisomy 18

Choroid Plexus Cysts

Occipital encephalocele Genitourinary Radiology

605

Fetal CNS Malformations

Trisomy 18 [Figure 3-7-15] • • • •

Overlapping Fingers Cardiac Defects Omphalocele/Diaphragmatic Hernia Choroid plexus cysts

Figure 3-7-15

Trisomy 18 with overlapping fingers References 1.

Chang MC, Russell SA, Callen PW, Filly RA, Goldstein RB. Sonographic detection of inferior vermian agenesis in Dandy-Walker malformations: prognostic implications. Radiology 1994; 193:765-770. 2. Chatzipapas IK, Whitlow BJ, Economides DL. The 'Mickey Mouse' sign and the diagnosis of anencephaly in early pregnancy. Ultrasound Obstet Gynecol 1999; 13:196-199. 3. Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002; 21:12571288. 4. d'Ercole C, Girard N, Cravello L, et al. Prenatal diagnosis of fetal corpus callosum agenesis by ultrasonography and magnetic resonance imaging. Prenat Diagn 1998; 18:247-253. 5. Ghidini A, Strobelt N, Locatelli A, Mariani E, Piccoli MG, Vergani P. Isolated fetal choroid plexus cysts: role of ultrasonography in establishment of the risk of trisomy 18. Am J Obstet Gynecol 2000; 182:972-977. 6. Goldstein RB, LaPidus AS, Filly RA. Fetal cephaloceles: diagnosis with US. Radiology 1991; 180:803-808. 7. Johnson SP, Sebire NJ, Snijders RJ, Tunkel S, Nicolaides KH. Ultrasound screening for anencephaly at 10-14 weeks of gestation. Ultrasound Obstet Gynecol 1997; 9:14-16. 8. Levitsky DB, Mack LA, Nyberg DA, et al. Fetal aqueductal stenosis diagnosed sonographically: how grave is the prognosis? AJR Am J Roentgenol 1995; 164:725-730. 9. McGahan JP, Nyberg DA, Mack LA. Sonography of facial features of alobar and semilobar holoprosencephaly. AJR Am J Roentgenol 1990; 154:143-148. 10. Pilu G, Romero R, Rizzo N, Jeanty P, Bovicelli L, Hobbins JC. Criteria for the prenatal diagnosis of holoprosencephaly. Am J Perinatol 1987; 4:41-49. 11. Ulm B, Ulm MR, Deutinger J, Bernaschek G. Dandy-Walker malformation diagnosed before 21 weeks of gestation: associated malformations and chromosomal abnormalities. Ultrasound Obstet Gynecol 1997; 10:167-170. 12. Vergani P, Ghidini A, Strobelt N, et al. Prognostic indicators in the prenatal diagnosis of agenesis of corpus callosum. Am J Obstet Gynecol 1994; 170:753-758.

Fetal CNS Malformations

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Fetal Body Anomalies Paula J. Woodward, M.D. Neck Masses

Figure 3-8-1

• • • •

Neural Tube Defects Cystic Hygroma Teratoma (Epignathus) Thyroid

• •

Lymphangioma Chromosomal Abnormalities ➢ Turners Syndrome XO ➢ Trisomy 21 (2nd trimester nuchal thickening) Often associated with hydrops

Cystic Hygroma [Figure 3-8-1]

•

Sagittal and transverse images through the fetal neck show a cystic hygroma

Iniencephaly [Figure 3-8-2] • • • • •

Fixed hyperextension of neck (“stargazer” position) Rachischisis Cephalocele Shortened or absent vertebral bodies First trimester ➢ head appears large ➢ CRL less than expected

Figure 3-8-2

Figure 3-8-3

AIUM: Chest [Figures 3-8-4 and 3-8-5] •

•

Four chamber heart ➢ side (stomach and heart both on left) ➢ axis ~35-45° ➢ equal chamber size ➢ excludes 90% of cardiac defect LVOT, RVOT if feasible

First trimester scan of iniencephaly shows a hyperextended head and short body

Figure 3-8-5

Figure 3-8-4

Four-chamber view of normal heart

Genitourinary Radiology

Epignathus (teratoma)

Left ventricular outflow tract and right ventricular outflow tract

607

Fetal Anomalies

Hypoplastic Left Heart [Figure 3-8-6]

Figure 3-8-6

• • • •

Lethal in neonate if untreated ➢ Norwood or transplant Small or invisible LV Hypoplastic aortic arch RA < LA Consider Turner syndrome in female fetuses

• • • •

Congenital Diaphragmatic Hernia Cystic Adenomatoid Malformation Extralobar Sequestration Teratoma

• • • •

90% left-sided through foramen of Bochdalek 50% have other anomalies Pulmonary hypoplasia “Liver-up” poor prognosis

• • • • • •

Lung Hamartoma Types I – III Fetal CCAMs classified as micro- or macro cystic Arterial supply from pulmonary artery May spontaneously regress In utero surgery for hydrops

•

Chest Masses

Congenital Diaphragmatic Hernia [Figure 3-8-7] Hypoplastic left ventricle

Cystic Adenomatoid Malformation [Figure 3-8-8]

Figure 3-8-7

Figure 3-8-8

Congenital diaphragmatic hernia with deviation of the heart

Figure 3-8-9

Sagittal scan of the fetal chest and neonatal CXR showing type II CCAM

Extralobar Sequestration [Figure 3-8-9] • • • •

Non-communicating (sequestered) lung segment Arterial supply from aorta 90% left sided 10% below diaphragm

• • • • •

Stomach Kidneys Bladder UC insertion site Umbilical cord vessel number

AIUM: Abdomen

Amniotic Fluid Balance

Production Fetal/ Embryo plasma volume Uterine Perfusion Metanephros (>10 wks) Lungs Fetal Anomalies

Extralobar sequestration with feeding vessel from the aorta. Removal Intramembranous Transmembranous Swallowing Lungs 608

Genitourinary Radiology

Polyhydramnios

• •

Figure 3-8-10

2/3 idiopathic 1/3 definable cause ➢ macroscomia ➢ GI obstruction ➢ CNS malformation ➢ hydrops

Oligohydramnios • •

Never normal A “DRIP” of fluid ➢ Demise ➢ Renal, also bladder often anhydramnios ➢ IUGR ➢ PROM, post dates

Double bubble, oblique view confirms duodenal atresia

Figure 3-8-11

Fetal GI Tract • •

Atresias Abdominal Wall Detect

• • • •

Esophageal Duodenal Small Bowel Anorectal

• • •

Stomach may be present but small Polyhydramnios after 20 wks IUGR common ➢ ingested fluid important for nutrition

Gastroschisis

Atresias

Esophageal Atresia

Figure 3-8-12

Double Bubble [Figure 3-8-10] •

•

Duodenal Atresia ➢ 30% have trisomy 21 ➢ 50% have other structural abnormalities Ladd’s bands, annular pancreas usually do not present in utero

Jejunal/Ileal Atresia • • •

1/3 have cystic fibrosis 5%-10% may perforate Meconium peritonitis ➢ ascites ➢ calcifications ➢ pseudocyst formation

Omphalocele

Figure 3-8-13

Bowel • • •

Normal bowel herniation at 8 weeks Rotates counterclockwise 270° Returns to abdomen in 12 weeks

• • •

Gastroschisis [Figure 3-8-11] Omphalocele [Figure 3-8-12 and 3-8-13] Limb-body-wall defect Gastroschisis Location Right Membrane No Cord Insertion NL Associated Anomalies No

Abdominal Wall Defects

• • • •

Genitourinary Radiology

Omphalocele Central Yes On sac 50-75% 609

Omphalocele Fetal Anomalies

Limb-Body-Wall Defect (Body Stalk Anomaly) • • • •

Fetus attached to placenta Absent or short umbilical cord Severe (lethal) malformation Scoliosis common

• • • •

Agenesis Renal Cystic Disease Hydronephrosis Masses

• • • •

Autosomal recessive polycystic kidney disease [Figure 3-8-15] Multicystic dysplastic kidneys [Figures 3-8-16 and 3-8-17] Cystic dysplasia due to obstruction Autosomal dominant polycystic kidney disease

•

VACTERL Syndrome ➢ Vertebral, anal atresia, cardiac, TE fistula, renal, limb Inherited Disorders ➢ Meckel-Gruber (renal cystic dysplasia, encephalocele, polydactyly) Chromosomal Abnormalities ➢ Trisomy 13

Figure 3-8-14

Renal Anomalies [Figure 3-8-14]

Renal Cystic Disease

Associations • •

Hydronephrosis • • • • •

UPJ Obstruction UVJ Obstruction Duplications PUV, Urethral Atresia Reflux

•

Renal Pelvis ➢ > 4 mm before 33 weeks ➢ > 7 mm after 33 weeks AP pelvis diam/AP kidney diam >50% Calyceal Dilatation Any degree of dilatation when accompanied by cystic renal changes

a) Normal vs. b) renal agenesis

Figure 3-8-15

Autosomal recessive polycystic kidney disease

Figure 3-8-16

Hydronephrosis [Figure 3-8-18] • • •

Figure 3-8-17

Figure 3-8-18

Bilateral MCDK Fetal Anomalies

Multicystic dysplastic kidney

UPJ obstruction 610

Genitourinary Radiology

Retroperitoneal Masses • • • •

Renal Cystic Disease Renal Tumors ➢ Mesoblastic Nephroma ➢ Wilms Tumor Adrenal ➢ Neuroblastoma ➢ Hemorrhage Extralobar Sequestration

Figure 3-8-19

Cystic Abdominal/Pelvic Collections • • •

Bladder Obstruction Dilated Bowel Cysts ➢ Ovarian ➢ Duplication ➢ Mesenteric ➢ Choledochal ➢ Meconium pseudocyst

Posterior Urethral Valves [Figures 3-8-19 and 3-8-20] • • •

Bladder “funnels” into dilated posterior urethra Oligohydramnios common Renal dysplasia (echogenic cystic kidneys) bad prognostic sign

Posterior urethral valves

Figure 3-8-20

Figure 3-8-21

Severe posterior urethral valves with oligohydramnios

Ovarian Cyst [Figure 3-8-21] • • • •

Most common cyst in 3rd trimester Anywhere in abdomen Complexity suggests torsion or hemorrhage Resolve by 6 mos

• •

Sacrococcygeal teratoma Myelomeningocele

Sacral Mass

Genitourinary Radiology

Ovarian cyst

611

Fetal Anomalies

Sacrococcygeal Teratoma [Figures 3-8-22 and 3-8-23] • •

Solid, cystic, or mixed Location ➢ Type 1: completely external ➢ Type 2: external and internal into pelvis ➢ Type 3: external and internal into abdomen ➢ Type 4: completely internal

Figure 3-8-23

Figure 3-8-22

Sacrococcygeal teratoma Solid sacrococcygeal teratoma with marked growth References 1.

2.

3. 4. 5. 6. 7. 8. 9.

10. 11.

12. 13.

Leung JW, Coakley FV, Hricak H, et al. Prenatal MR imaging of congenital diaphragmatic hernia. AJR Am J Roentgenol 2000; 174:1607-1612. Coleman BG, Adzick NS, Crombleholme TM, et al. Fetal therapy: state of the art. J Ultrasound Med 2002; 21:1257-1288. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998; 179:884-889. Lopoo JB, Goldstein RB, Lipshutz GS, Goldberg JD, Harrison MR, Albanese CT. Fetal pulmonary sequestration: a favorable congenital lung lesion. Obstet Gynecol 1999; 94:567-571. Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133:490-496; discussion 496-497. Nyberg DA, Resta RG, Luthy DA, Hickok DE, Mahony BS, Hirsch JH. Prenatal sonographic findings of Down syndrome: review of 94 cases. Obstet Gynecol 1990; 76:370-377. Corteville JE, Gray DL, Langer JC. Bowel abnormalities in the fetus--correlation of prenatal ultrasonographic findings with outcome. Am J Obstet Gynecol 1996; 175:724-729. Stringer MD, McKenna KM, Goldstein RB, Filly RA, Adzick NS, Harrison MR. Prenatal diagnosis of esophageal atresia. J Pediatr Surg 1995; 30:1258-1263. Meizner I, Levy A, Katz M, Maresh AJ, Glezerman M. Fetal ovarian cysts: prenatal ultrasonographic detection and postnatal evaluation and treatment. Am J Obstet Gynecol 1991; 164:874-878. Muller-Leisse C, Bick U, Paulussen K, et al. Ovarian cysts in the fetus and neonate--changes in sonographic pattern in the follow-up and their management. Pediatr Radiol 1992; 22:395-400. Hutton KA, Thomas DF, Davies BW. Prenatally detected posterior urethral valves: qualitative assessment of second trimester scans and prediction of outcome. J Urol 1997; 158:1022-1025. James CA, Watson AR, Twining P, Rance CH. Antenatally detected urinary tract abnormalities: changing incidence and management. Eur J Pediatr 1998; 157:508-511. Abuhamad AZ, Horton CE, Jr., Horton SH, Evans AT. Renal duplication anomalies in the fetus: clues for prenatal diagnosis. Ultrasound Obstet Gynecol 1996; 7:174-177.

Fetal Anomalies

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Genitourinary Radiology

14. Pryde PG, Bardicef M, Treadwell MC, Klein M, Isada NB, Evans MI. Gastroschisis: can antenatal ultrasound predict infant outcomes? Obstet Gynecol 1994; 84:505-510. 15. Luton D, De Lagausie P, Guibourdenche J, et al. Prognostic factors of prenatally diagnosed gastroschisis. Fetal Diagn Ther 1997; 12:7-14. 16. Getachew MM, Goldstein RB, Edge V, Goldberg JD, Filly RA. Correlation between omphalocele contents and karyotypic abnormalities: sonographic study in 37 cases. AJR Am J Roentgenol 1992; 158:133-136. 17. Salihu HM, Boos R, Schmidt W. Omphalocele and gastrochisis. J Obstet Gynaecol 2002; 22:489-492.

Genitourinary Radiology

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Fetal Anomalies

Cystic Diseases of the Kidney Peter L.Choyke, MD

Cystic Disease of the Kidney • • • • •

Autosomal Dominant Polycystic Kidney Disease (ADPKD) Autosomal Recessive Polycystic Kidney (ARPKD) Tuberous Sclerosis Complex (TS or TSC) Von Hippel-Lindau Disease (VHL) Acquired Cystic Kidney Disease (ACKD)

• • • •

Occurs in 1:1000 Individuals Genetics: Autosomal Dominant ESRD in 50% Risk of Cancer = Not increased

•

PKD 1 (85-95%) ➢ 16p13.3 ➢ Polycystin I ➢ Mean age of ESRD=55y PKD 2 (~5% ) ➢ 4q21–23 ➢ Polycystin II ➢ Mean age of ESRD= 71.5y PKD3? PKD4?

ADPKD

Types of ADPKD

• •

Figure 3-9-1

Illustration of the microvilli on the surface of lumenal cells that are abnormal in ADPKD

Mechanism [Figure 3-9-1]

Clinical Manifestations • • • • • •

Pain Hypertension Infection (Women > Men) Stones Loss of Renal Function Renal Failure

Imaging [Figures 3-9-2 to 3-9-5] Figure 3-9-2

Figure 3-9-3

Montage of ultrasounds at different stages of life. From upper left clockwise: 14 years, mid twenties, mid fifies, mid sixties

Retrograde pyelogram in ADPKD Cystic Diseases of the Kidney

614

Genitourinary Radiology

Figure 3-9-4

Figure 3-9-5

Montage of CT scans of different patients at different stages of their disease MRI of ADPKD

Complications [Figure 3-9-6] •

Acute Infection

• •

Intracranial Aneurysms Cardiovascular Disease: Mitral, Aortic valve, aortic aneurysm Cysts: Hepatic, Pancreatic, Spleen Diverticula: Colon

Figure 3-9-6

Manifestations of ADPKD

• •

Intracranial Aneurysms [Figure 3-9-7] •

•

Gas forming organism requiring percutaneous drainage ICA Figure 3-9-7 ➢ 18–26% of ADPKD ➢ Rupture ~2–11% ➢ 46–61% Mortality Rate ➢ Mean age 39–47 years of rupture Screening (MRA) performed periodically in patients with ADPKD

Extrarenal Cysts • •

Occur in 70–75% of ADPKD Complications (liver): ➢ Pain ➢ Biliary Obstruction ➢ IVC compression

Dissecting aneurysm of the abdominal aorta in ADPKD

Screening • • •

US Screening begins in teenage years ~ 2/3 of affected children will show cysts between 11–20 ~ 95% by age 30

Figure 3-9-8

Localized PKD [Figure 3-9-8] • •

Unilateral, segmental Non Hereditary ➢ Possible “mosaic” form of ADPKD Two cases of unilateral PKD

Genitourinary Radiology

615

Cystic Diseases of the Kidney

Autosomal Recessive Polycystic Kidney Disease (ARPKD) [Figures 3-9-9 and 3-9-10]

• • • • • • • •

Prevalence: Variable 1:6000-50,000 Genetics: Autosomal Recessive ESRD: >50% Cancer: None Unrelated to ADPKD Congenital Hepatic Fibrosis Caroli’s, Biliary Cystic Disease Renal ductal ectasia, abnormal cilia ➢ Enlarged echogenic kidneys ➢ Infantile Form: Renal Failure leading to Oligohydramnios, Pulmonary dysplasia ➢ Childhood form: Portal Hypertension, Caroli’s, Renal enlargement, Late renal failure

Figure 3-9-9

Mechanism: Disease of Microvilli Tuberous Sclerosis • • • • •

Prevalence: 1:10,000 Genetics: Autosomal Dominant** Produces hamartomas throughout the body: ESRD: 15% (cystic/AML bleeding) Risk of Cancer: 1-2% (slight increase) ➢ ** mostly new mutations; not hereditary

Types of TSC •

•

TSC 1 ➢ 9q34 ➢ ~1/3 TSC ➢ “Hamartin” ➢ Assoc with severe MR TSC 2 ➢ 16p 13.3 !! ➢ 2/3 of TSC ➢ “Tuberin” ➢ Assoc with worse renal disease

Pathogenesis •

Tuberous= nodular, Sclerosis= hard ➢ Skin: Adenoma Sebaceum, Angiofibromas ➢ CNS: Tubers, Subependymal nodules, Giant Cell Astrocytoma ➢ Kidneys: Cysts, Angiomyolipomas ➢ Heart: Rhabdomyomas ➢ Lungs: Lymphangiomyomatosis (LAM) ➢ Bone: Islands

Typical appearance of increased echogencity within the kidneys and increased signal on T2W MRI in ARPKD

Figure 3-9-10

Biliary cystic dilation in ARPKD Cystic Diseases of the Kidney

616

Genitourinary Radiology

Cyst Predominant Forms of TSC

Figure 3-9-11

[Figure 3-9-11]

Renal Involvement [Figure 3-9-12]

•

Angiomyolipoma predominant ➢ Mild to severe ➢ Risk of Hemorrhage ➢ Treat conservatively ❖ Partial nephrectomy ❖ Angioembolization ❖ Radiofrequency ablation

Non Fatty Angiomyolipoma [Figure 3-9-13] Renal Manifestations •

Carcinoma of the Kidney ➢ 1–2% of TS patients ➢ Heterogenous solid lesions ➢ Faster growing than AMLs ➢ No screening recommendations

Cystic predominant form of TSC

Figure 3-9-12

Von Hippel Lindau Disease [Figures 3-9-14 to 3-9-17]

• • • • •

Prevalence ~ 1:35,000 to 1:45,000 Genetics: Autosomal Dominant ESRD: < 5% Usually due to nephrectomy Risk of Cancer: 30-40% Target Organs ➢ CNS, Retina --Hemangioblastomas ➢ Kidney--Cysts and Cancers ➢ Pancreas-- Cysts and Neuroendocrine tumors ➢ Adrenal--Pheochromocytomas ➢ Epididymis/ Broad Ligament– Cystadenomas

Renal Manifestations •

• •

Multiple Cysts ➢ Virtually all will have neoplastic clear cell lining Cysts containing tumors Solid (Clear Cell) tumors

Examples of angiogmyolipomas in TSC at differing degress of severity

Management

Risk of Metastases - 3 cm rule - Risk of Renal Failure

Figure 3-9-13

Illustrates features of non fatty angiomyolipomas; hyperdense lesions that enhance uniformly Genitourinary Radiology

617

Cystic Diseases of the Kidney

Figure 3-9-14

Figure 3-9-16

CNS hemangioblastomas in VHL

Figure 3-9-15

Solid islet cell or pancreatic neuroendocrine tumors in VHL

Cystic lesions of the pancreas in VHL

Figure 3-9-17

Radiofrequency Ablation [Figure 3-9-18] Acquired Cystic Kidney Disease • • • •

Rate: up to 100% of dialysis pts Genetics: None ESRD: 100% Risk of Cancer: Increases with duration

• •

Theory 1: Dialysis Toxin Theory 2: Uremic Milieu ➢ Mutations which lead to cysts, adenomas, tumors and metastatic cancers

Pathogenesis

Bilateral pheochromocytomas

Figure 3-9-18

Successful treatment with RFA in patient with VHL Cystic Diseases of the Kidney

618

Genitourinary Radiology

Renal Cancer in ACKD [Figure 3-9-19] •

Figure 3-9-19

10–50 Fold Risk of RCC ➢ Mean dialysis duration 8 yrs ➢ Multifocal & Bilateral ➢ 17% Risk of Metastases

Screening •

“Screening is not routinely justified” ➢ Relatively low risk of cancer ➢ High risk of dying from other causes ➢ Reserve screening for pts with good long term prognosis

Levine E, Abdom Imaging 1995 20:569-71

ACKD-RCC After Transplant •

Transplantation ➢ Cysts Regress ➢ New Tumor Formation Decreases ➢ Increased Risk of Metastases from Existing RCC – Immunosuppression

Take Home Points •

• •

When confronted with a “polycystic kidney” ➢ Pure cysts ? Aneurysms ? ADPKD ➢ Enlarged echogenic kidneys? ARPKD ➢ Cysts and AMLs? Brain, skin? TSC ➢ Cysts and RCCs? VHL ➢ Renal failure on dialysis? ACKD Genetic Testing and Screening: ➢ PKD1, PKD2 ➢ TSC1, TSC2 ➢ VHL Risk of Renal Cancer: ➢ ADPKD None known ➢ ARPKD None known ➢ TSC ~ 1-2% ➢ VHL ~ 35% ➢ ACKD ~ 50% (after 8 years of dialysis)

Examples of tumors in patients on dialysis due to ACKD

References 1. 2.

Witzgall R. New developments in the field of cystic kidney diseases. Curr Mol Med 2005; 5:455-465. Tahvanainen E, Tahvanainen P, Kaariainen H, Hockerstedt K. Polycystic liver and kidney diseases. Ann Med 2005; 37:546-555. 3. Adeva M, El-Youssef M, Rossetti S, et al. Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD). Medicine (Baltimore) 2006; 85:1-21. 4. Okumura M, Bunduki V, Shiang C, Schultz R, Zugaib M. Unusual sonographic features of ARPKD. Prenat Diagn 2006. 5. Choyke PL, Glenn GM, Walther MM, Zbar B, Linehan WM. Hereditary renal cancers. Radiology 2003; 226:3346. 6. Sessa A, Righetti M, Battini G. Autosomal recessive and dominant polycystic kidney diseases. Minerva Urol Nefrol 2004; 56:329-338. 7. Herring JC, Enquist EG, Chernoff A, Linehan WM, Choyke PL, Walther MM. Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience. J Urol 2001; 165:777-781. 8. Seizinger BR, Smith DI, Filling-Katz MR, et al. Genetic flanking markers refine diagnostic criteria and provide insights into the genetics of Von Hippel Lindau disease. Proc Natl Acad Sci U S A 1991; 88:2864-2868. 9. Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology 1995; 194:629-642. 10. Ishikawa I, Saito Y, Asaka M, et al. Twenty-year follow-up of acquired renal cystic disease. Clin Nephrol 2003; 59:153-159. Genitourinary Radiology

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Imaging of Prostate Cancer Peter L.Choyke, MD Prostate Cancer • • •

Diagnosis Staging Image guided Therapy

•

~220,000 new diagnoses per year ➢ ~29,000 cancer deaths (USA) ➢ 2nd most common cause of cancer deaths in males ➢ 21% decrease in cancer deaths in the PSA era

Epidemiology

Only a small fraction of prostate cancers cause death •

We are overdiagnosing and overtreating prostate cancer

•

Gleason Grading System ➢ Two predominant cell types ➢ Add together for score from 2-10

Grading Prostate Cancer Screening and Detection •

Recommendations: ➢ For men > 50 years or > 40 in African Americans or with Family history : ❖ Annual Digital Rectal* ❖ Annual PSA

Prostate Specific Antigen •

Ranges of PSA ➢ 0–4ng/ml Normal (PPV=5%) ➢ 4–10ng/ml Indeterminate (PPV=22%) ➢ > 10 ng/ml Abnormal (PPV=67%)

Figure 3-10-1

Detecting Prostate Cancer • • •

Elevated PSA or Abnormal Rectal Exam Transrectal Ultrasound Guided Biopsies Biopsy Mapping and Grading

•

Zonal Anatomy of Prostate ➢ Peripheral Zone ❖ Glandular ❖ 70% of Cancers ➢ Transitional Zone ❖ Stromal/Glandular ❖ 25% of Cancers, 90% of Hyperplastic nodules ➢ Central Zone

Anatomy

Distribution of Prostate Cancers Prostate Ultrasound [Figure 3-10-1] •

Transrectal Ultrasound-History ➢ The Chair (Watanabe 1968) ➢ Zonal Anatomy (Stamey 1980) ➢ Screening (Lee 1983) ➢ TRUS Guided Biopsy (1985-) ➢ Color Doppler (1995-) ➢ Contrast Enhanced US (2000-)

Imaging of Prostate Cancer

Normal ultrasound of the prostate showing echogenic peripheral zone and ejaculatory duct on sagittal view

620

Genitourinary Radiology

The TRUS Examination [Figure 3-10-2] • • • •

Examine the PZ for nodules Examine the TZ for asymmetry Examine Seminal Vesicles Determine the Volume

•

Prep: ➢ Antibiotics before and after (Cipro) ➢ Enema (Fleets) Core Biopsies with Automatic Cutting Needle ➢ Directed Biopsies at sites of abnormality ➢ Label all specimens; send separately

Figure 3-10-2

TRUS Guided Biopsy •

Doppler power ultrasound can be used to detect prostate cancer vascularity

Tumor Mapping with Biopsy Staging Prostate Cancer Staging (TNM) • • • • • • •

Non palpable Detected by Bx Palpable Extracapsular Fixed, invasive Regional Nodes Distant Mets

• • • • •

A1, 2 B1, 2 C1, 2 D1 D2

•

Local Staging (Extracapsular) [Figure 3-10-3] ➢ Ultrasound ➢ MRI Key Structures: ➢ Neurovascular Bundles ➢ Seminal Vesicles ➢ Apex ➢ Periprostatic Venous Plexus

Treatment

A1, 2 ** B1, 2 C1 C2 D1 D2

T1 a-c T2 a,b T3 a-c T4 Tx, N+, M+

Staging with Imaging •

Figure 3-10-3

T1a, b T1c T2a, b T3a, b, c(sv) T4 Tx, N+ Tx, Nx, M+ Surg/XRT/WW Surg/XRT/WW XRT/WW/Hormonal XRT/WW/Hormonal Hormonal/Chemotherapy

Extracapsular extension on ultrasound

TRUS Staging •

•

Sensitivity ➢ ~40–50% Exceptions: ➢ Seminal Vesicles ➢ Neurovascular Bundle

Figure 3-10-4

Endorectal Coil MRI [Figure 3-10-4] •

Sensitivity: ➢ Early Studies ~85–90% ➢ Multi institutional Trial ~60–70% ❖ Motion ❖ Microscopic Disease ❖ Operator dependent ❖ Observer dependent

Genitourinary Radiology

Extracapsular extension on endorectal coil MRI

621

Imaging of Prostate Cancer

Endorectal Coil MRI •

Figure 3-10-5

Improvements ➢ Dynamic contrast enhancement ➢ MR Spectroscopy

Dynamic Enhanced MRI of the Protaste

MRI and 1H-MRSI- Prostate: Metabolic Interrogation Nodal Staging [Figures 3-10-5 and 3-10-6] •

• •

CT/MRI only ~ 36% sensitive ➢ Size threshold ~8mm + biopsy of nodes ➢ Yield improves for high risk pts ➢ (PSA >20 ng/ml) Prostascint SPECT USPIO (Combidex) MR Lymphography

Staging for Distant Metastases •

Prostascint images demonstrate positive node despite negative CT

Bone Metastases ➢ Bone Scan ❖ Yield increases after PSA >10 ❖ **Superscan** ❖ Quantitation/ Confirmation

Figure 3-10-6

Radioactive Ablation •

Strontium-89 (Metastron) ➢ ~80% response rate ➢ Up to 6 months relief of Examples of nodal metastasis due to prostate cancer. Percutaneous biopsy can be performed to determine if a node is positive bone pain ➢ Samarium and Rhenium

Figure 3-10-7

Image Guided Treatment • •

Brachytherapy Cryotherapy

•

Interstitial Radioactive Seeds (Afterloaded) ➢ Iodine, Iridium, Palladium, Gold Introducers are placed within prostate at regular spacing via: ➢ CT ➢ US ➢ MRI

Brachytherapy [Figure 3-10-7] •

Cryotherapy [Figure 3-10-8] • •

Examples of brachytherapy seeds immediately after seed placement (left) and several years after placement (right)

Liquid Nitrogen instilled via cannulas placed within Prostate under TRUS Not enough Data ➢ High rate of impotence ➢ Steep learning curve

Figure 3-10-8

Cryotherapy of the prostate monitored by ultrasound Imaging of Prostate Cancer

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Take Home Points • • •

Imaging currently plays minor role in prostate cancer detection: ➢ MR spectroscopy, Dynamic MRI may change this Staging depends on PSA/Grade ➢ MRI for local staging ➢ CT/MRI (USPIO) for nodal staging ➢ Bone Scan/CT/MR for distant staging Image Guided therapy is an important trend in treatment

References 1. 2. 3. 4. 5. 6. 7.

8.

9.

10.

11.

12.

13. 14. 15.

Adusumilli S, Pretorius ES. Magnetic resonance imaging of prostate cancer. Semin Urol Oncol 2002; 20:192-210. Campbell T, Blasko J, Crawford ED, et al. Clinical staging of prostate cancer: reproducibility and clarification of issues. Int J Cancer 2001; 96:198-209. el-Gabry EA, Halpern EJ, Strup SE, Gomella LG. Imaging prostate cancer: current and future applications. Oncology (Huntingt) 2001; 15:325-336; discussion 339-342. Engelbrecht MR, Barentsz JO, Jager GJ, et al. Prostate cancer staging using imaging. BJU Int 2000; 86 Suppl 1:123134. Harisinghani MG, Barentsz J, Hahn PF, et al. Noninvasive detection of clinically occult lymph-node metastases in prostate cancer. N Engl J Med 2003; 348:2491-2499. Hocht S, Wiegel T, Bottke D, et al. Computed tomogram prior to prostatectomy. Advantage in defining planning target volumes for postoperative adjuvant radiotherapy in patients with stage C prostate cancer? Strahlenther Onkol 2002; 178:134-138. Hernandez J, Thompson IM. Prostate-specific antigen: a review of the validation of the most commonly used cancer biomarker. Cancer 2004; 101:894-904. Konety BR, Bird VY, Deorah S, Dahmoush L. Comparison of the incidence of latent prostate cancer detected at autopsy before and after the prostate specific antigen era. J Urol 2005; 174:1785-1788; discussion 1788. Karakiewicz PI, Eastham JA, Graefen M, et al. Prognostic impact of positive surgical margins in surgically treated prostate cancer: multi-institutional assessment of 5831 patients. Urology 2005; 66:1245-1250. Kumar R, Zhuang H, Alavi A. PET in the management of urologic malignancies. Radiol Clin North Am 2004; 42:11411153, ix. Mathews D, Oz OK. Positron emission tomography in prostate and renal cell carcinoma. Curr Opin Urol 2002; 12:381385. Ravery V, Boccon-Gibod L. T3 prostate cancer: how reliable is clinical staging? Semin Urol Oncol 1997; 15:202206. Raja J, Ramachandran N, Munneke G, Patel U. Current status of transrectal ultrasound-guided prostate biopsy in the diagnosis of prostate cancer. Clin Radiol 2006; 61:142-153. Sanchez-Chapado M, Angulo JC, Ibarburen C, et al. Comparison of digital rectal examination, transrectal ultrasonography, and multicoil magnetic resonance imaging for preoperative evaluation of prostate cancer. Eur Urol 1997; 32:140-149. Sodee DB, Nelson AD, Faulhaber PF, Maclennan GT, Resnick MI, Bakale G. Update on fused capromab pendetide imaging of prostate cancer. Clin Prostate Cancer 2005; 3:230-238.

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Radiographic Evaluation of Urinary Stone Disease William D. Craig, MD Learning Objectives • • • •

Pathogenesis of renal stone disease Highlight CT as the modality of choice Alternative modalities Reinforce the critical role of Radiology

• •

Limited Rad-Pathology Stone Dz ➢ Major nuisance ➢ Med/Urologic Advances ➢ Previously Debilitating Annual 2-3% incidence White male LTR is 1 in 3-8 ➢ 14% @ 1yr ➢ 35% @ 5yr ➢ 52% @ 10yr Multi Billion $$ Cost

AFIP

• • •

Genetics • • •

Family Hx (3 X) M : F : 3: 1 Recognized D/O ➢ Familial RTA ➢ Mutations in CLCN5 gene

Extrinsic

• • • • •

Climate Water Diet Occupation Stress

• • • • • •

Family Hx Bone/GI Dz Gout Chronic UTI Nephrocalcinosis Stasis

• • • • • •

Composition Ca Oxalate/phosphate Struvite/matrix Uric Acid Cystine Other (incl indinavir)

• • • •

Epithelial cells Urinary Casts RBC’s Homogeneous Nuc---

Predisposing Factors

Stone Makeup

Heterogeneous Nucleation

Radiographic Evaluation of Urinary Stone Disease

Percent of all stones 75 10-15 6 1-2 <5

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Inhibitors • • • • • •

Organic Molecule Magnesium Pyrophosphate Citrate Mucoproteins RNA Fragmets

• •

Glycosaminoglycans Tamm-Horsfall

• •

Free crystals need to grow (2-5 min transit fm glomerulus to nephron) Anatomic Abnl ➢ UPJ ➢ MSK Lack of inhibitors ➢ Light chain proteins

Promoters

Aggregation

•

Formation Product • • •

Figure 3-11-1

Real question? Why don’t we all form stones Kf is 7-11 X Ksp

Calcium Stones [Figure 3-11-1] Hypercalcuria ➢ Idiopathic ➢ Steroid Use Immobilizaion • Hypocitrinuria • Hypomagnesuria

•

Left Ureteral Stone

Calcium Stones •

•

Hyperoxaluria ➢ Crohn disease ➢ Celiac sprue ➢ Pancreatic insufficiency ➢ Small intestinal bypass surgery for obesity CaPhos ➢ PTH or RTA

Figure 3-11-2

Struvite Stone MgNH4 PO4 · 6H2 O Magnesium Ammonium Phos [Figure 3-11-2] • • •

(NH2 )2 --CO ? H2 O + 2NH3 + CO2 Urine pH increases because ammonia hydrolyzes Urease ➢ Proteus ➢ Klebsiella

Struvite • • • •

Low Urine Volumes Infection F to M: 3 to 1 Sx ➢ Malaise ➢ Fever ➢ Wt Loss

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Staghorn Calculus

Radiographic Evaluation of Urinary Stone Disease

Uric Acid Stones •

• •

Hyperuricosuria ➢ Gout 35% if Uric Acid > 700mg/24 hr urine ➢ Newly Dx’ed Gout 1 per 114 with stones ➢ Hereditary (Lesch-Nyhan) ➢ Idiopathic Dehydration Markedly acid urine (< pH of 6.2) ➢ UC is classic example ❖ Ileostomy w/water and bicarb loss

Cystine Stone •

Hereditary Cystinuria ➢ Three Types ➢ Auto Recessive ➢ Abnormal renal tubule transport ➢ Large amounts of cystine are excreted in the urine (10 X normal) ➢ Younger Patients

Presentation •

Autonomic System ➢ Celiac ganglion ➢ Confusion about source ➢ Diaphragm to testicle ➢ GI sx ❖ N/V ❖ Diarrhea ❖ Ileus

Figure 3-11-3

Films • • • •

Visualize Characterize Sens 45% Spec 50%

• • •

Sensitivity: 64-97% Specificity: 92-94% Still 10-15% false negative rate

• • • • • •

1995 Sens of > 90% Spec of nearly 100% Quick No contrast Non-urologic Abnl

• • •

500 mrem 150-350 mrem for full IVP 13 mrem for one image

IVP CT

Distal left ureteral stone demonstrating a Rim Sign

Figure 3-11-4

CT-Radiation 4 by 2.5, 120 KVP, 120mAs,1-1.5 Rim Sign [Figure 3-11-3]

Comet Tail Sign [Figure 3-11-4]

Radiographic Evaluation of Urinary Stone Disease

Calcifications in right pelvis demonstrating the Comet Tail Sign

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Genitourinary Radiology

Secondary Signs Hydronephrosis/ Hydroureter [Figure 3-11-5]

Figure 3-11-5

Secondary Signs Unilateral renal enlargement [Figure 3-11-6] Secondary Signs Perinephric/ureteral edema [Figure 3-11-7]

Secondary Signs Unilateral absence of the white pyramid [Figures 3-11-8 to 3-11-10]

Figure 3-11-6

Asymmetric Right Hydronephrosis

Figure 3-11-7

Asymmetric left renal enlargement

Figure 3-11-8 Left Perinephric stranding

Figure 3-11-9 Normal hyperdense pyramids

Figure 3-11-10

Unilateral absence of right hyperdense pyramids Obstructed left system with lower parenchymal attenuation

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Radiographic Evaluation of Urinary Stone Disease

Conclusions • • • •

If H.U. Discrepancy > 5 Sens = 61% Spec = 100% Accur = 79%

• • • •

Size Number Location Complications

Urologist

Spontaneous Passage Rate of Ureteral Calculi as a Function of Stone Size Stone Size (mm) 1 2 3 4 5 6 7 8 9 10

No of Stones 15 43 23 18 15 18 17 9 3 11

Expectant Treatment

Passage Rate (%) 87 72 83 72 60 72 47 56 33 27

• • • •

Non-infected Two kidneys and normal renal function Small Stones <4mm 90% pass spontaneously

• • • •

Steroids Calcium channel blockers Fluids Pain meds

• • • •

Extracorporeal Shock wave lithotripsy < 2 cm Ca Stones Upper/Mid Pole Calyx

• •

Mechanical extraction Homium Laser

• • •

Percutaneous Nephrolithotomy Not amenable to ESWL or ureteroscopic approaches 1-2 day hospitalization

Figure 3-11-11

Distal Stone Protocol

Non-invasive treatment: ESWL

Invasive treatment: Ureteroscopy

Shadowing stone in central collecting system

Invasive Therapy: PCNL

Figure 3-11-12

Alternative Modalities [Figure 3-11-11]

Quantifying Obstruction: Ultrasound [Figure 3-11-12] Hydronephrosis Examine ureteral jets Potential use of resistive index (>.7)

Radiographic Evaluation of Urinary Stone Disease

Hydronephrotic Kidney 628

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MR [Figure 3-11-13]

Figure 3-11-13

MR Urography shows distal ureteral stone

References

1. Walsh : Campbell's Urology, 8th ed. 2002 W. B. Saunders Company. 2. Tamm EP, Silverman PM, Shuman WP. Evaluation of the patient with flank pain and possible ureteral calculus. Radiology. 2003 Aug;228(2):319-29. Epub 2003 Jun 20. Review. 3. Guest AR, Cohan RH, Korobkin M, Platt JF, Bundschu CC, Francis IR, Gebramarium A, Murray UM. Assessment of the Clinical Utility of the Rim and Comet-Tail Signs in Differentiating Ureteral Stones from Phleboliths AJR 2001;177:1285-1291.

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Radiographic Evaluation of Urinary Stone Disease

Testicular Torsion - Case Based Review Deborah J. Rubens, MD Testicular Torsion • • •

Defined as a twist of the spermatic cord or of the testis itself on its attachments. Degree of ischemia relative to the amount of twisting, beginning with venous compromise, and progressing to arterial occlusion. A 360 degree twist may still have arterial inflow. Torsion most common in puberty (ages 12-18), but also occurs in neonates and adults

Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum. Radiology 2003;227:18-36

Types of Testicular Torsion • •

Extravaginal (Neonatal) Intravaginal ➢ Bell-Clapper deformity

Scrotal Anatomy and Torsion •

Normal Tunica Vaginalis ➢ Inner visceral layer covers the testis and epididymis and cord ➢ Outer parietal layer lines the scrotum except posterolaterally where it fuses with the visceral layer and the scrotal wall to form the bare area. ➢ Hydroceles occur between these 2 layers

Scrotal Anatomy and Torsion •

Bell-Clapper Deformity of the Tunica Vaginalis ➢ Failure of fusion of the visceral and parietal layers to the scrotal wall, so the space completely encircles the epididymis, distal spermatic cord and the testis rather than attaching to the posterolateral aspect of the scrotum to form the normal bare area. ➢ It is bilateral in most cases ➢ 12% incidence found in one autopsy series

Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum. Radiology 2003;227:18-36

Scrotal Anatomy [Figure 3-12-1]

Bell-Clapper Deformity

Tunica Vaginalis

Figure 3-12-1

Sagittal line drawings of bell-clapper (left) and normal (right) testis. Tunica vaginalis is the outermost layer (arrows). Testicular Torsion

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Genitourinary Radiology

Testicular Torsion •

• •

Clinical presentation includes: ➢ Acute onset of scrotal pain ➢ Anorexia, nausea and/or vomiting ➢ Lack of urinary symptoms or fever As many as 35-50% of patients experience gradual onset of pain, similar to epididymitis Pain may be intermittent (detorsion)

Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum. Radiology 2003;227:18-36

Testicular Torsion: Clinical Significance •

•

Time from onset of symptoms to surgery associated with salvage rate:* ➢ 5-6 hours, 80-100% ➢ 6-12 hours 70% ➢ After 12 hours 20% If non salvageable, the necrotic testis is removed to decrease risk of autoimmune reaction to the residual testis

Donohue RE, Utley WL. Urology 1978 11:33

Testicular Torsion: Grayscale Patterns • • • •

Acute torsion with viable testis: normal Acute torsion with infarction: hypoechoic pattern which may be total, or partial in the case of a partial infarct Acute torsion with hemorrhagic infarction: hyperechoic and heterogeneous pattern. Chronic: hypoechoic with small testis

Figure 3-12-2

Middleton WD, Middleton MA, Dierks M, et al. Sonographic prediction of viability in testicular torsion: preliminary observation. J Ultrasound Med. 1997;16:23–27.

Testicular Torsion: Doppler Patterns • • •

Absent arterial and venous flow Increased Resistive Index on affected side (diminished or reversed diastolic flow) Decreased flow velocity difficult to measure due to small vessels/angle correction, but may be subjectively inferred by relative difficulty in finding small low amplitude flow on symptomatic side

Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the scrotum. Radiology 2003;227:18-36 Dogra VS, Sessions A, Mevorach R, Rubens DJ Reversal of diastolic plateau in partial testicular torsion. J Clin Ultrasound 2001; 29:105-108

CASE 1 [Figure 3-12-2] • •

Sudden right sided pain Diagnosis: Acute right testicular torsion with viable testis.

•

Important imaging findings: ➢ Note grayscale symmetry on transverse images ➢ Color Doppler flow is absent in the affected testis, but not in the epididymis ❖ The epididymis has alternate blood supply and may be perfused even if the testis is not

Dx: Acute torsion with viable testis

CASE 1: 21 year old man with sudden right-sided testicular pain Genitourinary Radiology

631

Testicular Torsion

CASE 2 [Figures 3-12-3 and 3-12-4] • • •

A seven year old presents with acute symptoms. Which side is abnormal? Minimal right sided flow Right testis is hypoechoic

Figure 3-12-3

CASE 2: A seven year old presents with acute symptoms. Which side is abnormal?

CASE 2 [Figure 3-12-5] • •

Figure 3-12-4

CASE 2: Minimal right sided flow. Right testis is hypoechoic

Left sided Doppler findings. What is your diagnosis? Chronic left testicular torsion with hemorrhagic non-viable testis

From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion Ultrasound Clin 2006; 1:55-66 with permission.

Chronic Torsion with Hemorrhagic Non-Viable Testis • •

•

In young children the testis may be small and hypoechoic. Flow is often minimal, but note the normal spectral waveform pattern The abnormal side has markedly increased Doppler flow, but it is around the testis, not within it. It is important always to image in 2 planes and to document flow within the actual testis. CASE 2: Left sided Doppler findings in long axis (left) and in Hemorrhage creates additional tissue transverse (right) plane. What is your diagnosis? planes and is hyperechoic and heterogeneous. Hemorrhage is a result of infarction and indicates a non-viable testis

CASE 3 [Figure 3-12-6] • •

Figure 3-12-5

Right sided pain, nausea and vomiting. Is this torsion or epidydimitis? Diagnosis: Torsion with 360 degree twist of the spermatic cord

RT

LT

Figure 3-12-6

CASE 3 Right sided pain, nausea and vomiting in a 15 year old. Is this torsion or epidydimitis?

Testicular Torsion

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Genitourinary Radiology

Torsion with preservation of Doppler flow • • • • •

Torsion is not an all or none phenomenon Venous obstruction occurs first and is indicated by a high-resistance arterial spectral Doppler waveform. Flow may still be present even if the testis is twisted up to 720 degrees. More flow will be detected with power Doppler and probably also with US contrast. THE PRESENCE OF FLOW DOES NOT EXCLUDE TORSION!

Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion Ultrasound Clin 2006; 1:55-66

CASE 4 [Figure 3-12-7] • • •

2 hours of left sided symptoms Can this be torsion? DX: Torsion/detorsion with increased flow post torsion

Figure 3-12-7

From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion Ultrasound Clin 2006; 1:55-66 with permission.

TORSION/DETORSION • • •

History is critical-classically that of intermittent acute and sharp pain with long symptom-free intervals Know which side hurts and if it still hurts during the examination. If scanned immediately after detorsion, the affected testis may show increased blood flow

CASE 5 [Figure 3-12-8] • •

Left sided symptoms progressing over several days. Prior US exam showed an enlarged epididymis with increased flow to the epididymis Dx: Torsion/detorsion with focal infarction

Figure 3-12-8

CASE 4: 2 hours of left sided symptoms. Can this be torsion? From: Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion Ultrasound Clin 2006; 1:55-66 with permission

CASE 5:Left sided symptoms progressing over several days. Prior US exam showed an enlarged epididymis with increased flow to the epididymis

Torsion/detorsion with infarction • • • •

Focal hypoechoic areas with concave margins are typical of infarcts. Focal infarcts when associated with normal or increased flow should alert you to the possibility of intermittent torsion. Frequently the epididymis is enlarged and hyperemic in torsion/detorsion and can be mistaken for epididymitis. Careful surveillance of the testis for focal infarction may lead to the correct diagnosis

Genitourinary Radiology

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Testicular Torsion

CASE 6 [Figure 3-12-9] •

A 2 month old had pain 1 month ago with a normal US exam, Now he has new pain and right scrotal swelling for 18 hours

CASE 6 [Figure 3-12-10] • •

Figure 3-12-9

What is the finding adjacent to the testis. Is this torsion? Dx: Inguinal hernia with obstructed flow to the spermatic cord

Figure 3-12-10

CASE 6: A 2 month old had pain 1 month ago with a normal US exam, Now he has new pain and right scrotal swelling for 18 hours

CASE 6: Note fluid filled structure superior to the right testis (top) with color Doppler flow in the wall (bottom). A hydrocele surrounds the right testis. Is this torsion?

Inguinal Hernia With Obstructed Testicular Perfusion •

Multiple etiologies of altered testicular perfusion may occur, including mass lesions obstructing the spermatic cord. Even in a newborn, flow should be obtainable in the testis

•

4 day old with large left hydrocele. Is there left-sided torsion?

•

Repeat scan 10 hours later

•

CASE 7 [Figure 3-12-11]

Figure 3-12-12

CASE 7 [Figure 3-12-12] Figure 3-12-11

CASE 7: A 4 day old presents with a large left hydrocele. Is there left-sided torsion? Testicular Torsion

634

CASE 7: Repeat scans 10 hours later Right testes (top) Left testes (bottom) Genitourinary Radiology

CASE 7 [Figure 3-12-13] • •

Repeat spectral Doppler exam 10 hours later. What is your diagnosis? Dx: Normal testes

Doppler Technical Considerations • • •

Always use the highest frequency Doppler which will yield a signal without attenuation Initial examination was performed at Doppler frequency of 5MHz, repeat examination at 10 MHz. Always confirm a true arterial spectral waveform. The waveform on the initial examination was only noise, and could have been interpreted as no flow

CASE 8 • • • •

Figure 3-12-13

[Figure 3-12-14]

Acute left sided pain Patient is s/p Lt orchiectomy and s/p Rt Orchiopexy Now with right sided pain. Repeat scan done 2 days later

[Figure 3-12-15]

Dx: Bilateral infarction due to polyarteritis nodosa

Figure 3-12-14

RT

CASE 7: Repeat spectral Doppler exam 10 hours later. What is your diagnosis? Top: Bilateral normal testes with inadequate Doppler on initial examination

LT

Figure 3-12-15

Acute left sided pain No flow in the left testis

Testicular Ischemia

Causes for ischemia other than torsion include: • Vasculitis ➢ Polyarteritis Nodosa ➢ Systemic Lupus Erythematosum • Severe edema from infection ➢ Uncontrolled or unresponsive epididymo-orchitis • Venous thromboses (ie hypercoagulable patients)

CASE 8: 2 days later the patient presents with right sided pain. He is post left orchiectomy and right orchiopexy. Gray scale image (left) is unremarkable. Doppler image (right) shows a noise spectrum. Dx: Bilateral infarction due to polyarteritis nodosa

Imaging Methods

Doppler examination is 86% sensitive, 100% specific and 97% accurate* when using absent flow in the symptomatic side as the single diagnostic criteria. If assymmetric abnormal spectral tracing were also used for diagnosis, sensitivity would improve • In children, power Doppler is more sensitive than color Doppler to detect normal flow, with rates of 97% vs 88% respectively ** *Burks DD, Markey BJ, Burkhard TK, Balsara ZN Haluszka MM, Canning DA. Suspected testicular torsion and ischemia: evaluation with color Doppler sonography. Radiology 1990;175:815-21 ** Barth RA, Shortliffe LD. Normal pediatric testis: comparison of power Doppler and color Doppler US in the detection of blood flow. Radiology 1997;204:389-93 •

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Testicular Torsion

Torsion/Detorsion • • •

Classic history of intermittent symptoms If scanned when asymptomatic or after detorsed, will see increased flow in the affected testis, which may suggest epididymitis Testis may be enlarged, and focal infarcts may or may not be present

Torsion Mimics/Variants • •

•

Infarction may present with pain which mimics torsion Partial infarction may occur from torsion/detorsion, from vasculitis, or from variant arterial anatomy. In some patients, the epidydimal artery, a branch of the testicular artery, supplies the superior and anterior pole of the testis. If this artery is hypoplastic, a small twist may result in focal infarction involving the superior pole Total infarction is more unusual, but should be suspected in patients with underlying vasculitides such as polyarteritis nodosa and systemic lupus

Artery to Epididymis •

In some patients, the epidydimal artery, a branch of the testicular artery, supplies the superior and anterior pole of the testis. If this artery is hypoplastic, a small twist may result in focal infarction

Torsion Take Home Messages • • • •

Torsion may be present despite testicular flow. Diminished or high resistance flow should suggest torsion in the proper setting. A history of intermittent symptoms should suggest detorsion, and corresponding hyperperfusion should not be confused with epididymo-orchitis. Other rare causes of decreased testicular perfusion include vasculitis, and if torsion is not present, appropriate medical therapy should be pursued

References 1. 2.

3. 4. 5. 6. 7.

Barth RA, Shortliffe LD. Normal pediatric testis: comparison of power Doppler and color Doppler US in the detection of blood flow. Radiology 1997;204:389-93. Burks DD, Markey BJ, Burkhard TK, Balsara ZN Haluszka MM, Canning DA. Suspected testicular torsion and ischemia: evaluation with color Doppler sonography. Radiology 1990;175:815-21 Dogra VS, Bhatt S, Rubens, DJ. Sonographic Evaluation of Testicular Torsion. Ultrasound Clin 2006; 1:55-66 with permission. Dogra VS, Gottlieb RH, Oka M, Rubens DJ. Sonography of the Scrotum. Radiology 2003;227:18-36 Dogra VS, Sessions A, Mevorach R, Rubens DJ Reversal of diastolic plateau in partial testicular torsion. J Clin Ultrasound 2001; 29:105-108 Donohue RE, Utley WL. Urology 1978 11:33 Middleton WD, Middleton MA, Dierks M, et al. Sonographic prediction of viability in testicular torsion: preliminary observation. J Ultrasound Med. 1997;16:23–27.

Testicular Torsion

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Imaging of Ovarian Masses Brent J. Wagner, MD Ovarian Masses •

•

Non-neoplastic ➢ physiologic cyst, endometriosis, etc. Neoplastic ➢ epithelial tumors 65% ➢ germ cell tumors 25% ➢ sex-cord stromal tumors 5% ➢ secondary malignancies 5% ➢ gonadoblastoma <1%

Relative Incidence of Ovarian Neoplasms [Figure 3-13-1] Major Ovarian Tumor Types •

•

•

Epithelial ➢ Serous ➢ Mucinous ➢ Endometrioid ➢ Clear Cell ➢ Brenner ➢ (others) Germ Cell ➢ Mature teratoma ➢ Dysgerminoma ➢ Immature teratoma ➢ (others) Sex Cord – Stromal ➢ Fibrothecoma ➢ Granulosa cell ➢ Sertoli-Leydig ➢ (others)

Figure 3-13-1

Relative incidence of ovarian neoplasms

Common Ovarian Epithelial Tumors: Classification • • • • • •

Serous Mucinous Endometrioid Clear Cell Brenner (others, including mixed, undifferentiated, etc.)

• • • • •

65% of ovarian neoplasms 85% of ovarian malignancies 60% of epithelial tumors are benign 35% of epithelial tumors are malignant 5% of epithelial tumors are borderline, low malignant potential

•

major risk factor: “incessant ovulation” (Lancet, 1973) ➢ infertility ➢ celibacy ➢ nulliparity ➢ family history ➢ high socioeconomic status ➢ breast cancer ➢ endometrial cancer ➢ lack of oral contraceptive use

Ovarian Epithelial Tumors

Ovarian Epithelial Tumors

Genitourinary Radiology

637

Imaging of Ovarian Masses

CA-125 • • • • • •

abnormally elevated in 85% of ovarian cancer patients false negative in 50% of Stage I disease false negative in 30% of mucinous tumors false positives occur (especially in pre-menopausal patients) with benign neoplasms, endometriosis, etc. most commonly used to follow known disease for remission and recurrence rarely of use in deciding on surgical vs. non-surgical management at the time of initial presentation of a pelvis mass

Ovarian Epithelial Tumors • •

•

benign low malignant potential (LMP) ➢ “borderline” tumors ➢ based on histologic appearance of primary ➢ may be a heterogeneous group (but histologic features overlap) ➢ 95% five year survival overall• ❖ But the patients who have metastasis or recurrence are clinically similar to patients with (true) ovarian cancer malignant

Figure 3-13-2

Serous cystadenoma (bilateral)

Figure 3-13-3

Epithelial Tumors: Terminology • • •

adenoma or adenocarcinoma add prefix “cyst-” if cystic Serous cystadenoma (LMP) add suffix “-fibroma” if more than 50% fibrous ➢ (e.g. “cystadenofibroma” or “fibrous cystadenocarcinoma”) Figure 3-13-4 ➢ do not confuse with a true fibroma (one of the sex-cord stromal tumors)

Epithelial Ovarian Neoplasms: Serous [Figures 3-13-2 to 3-13-6]

• • • • • • • • •

also known as “papillary” tumors 25% of benign neoplasms 50% of malignant neoplasms 63% benign, 30% malignant, 7% LMP strongest association with CA-125 thin-walled cyst, usually unilocular papillary soft tissue projections often seen psammomatous calcification is more common than with other ovarian neoplasms solid or bilateral tumors suggest malignancy

Figure 3-13-5

Serous cystadenocarcinoma

Imaging of Ovarian Masses

638

Serous cystadenoma (LMP)

Genitourinary Radiology

Epithelial Ovarian Neoplasms: Mucinous • • • • •

mucin-containing cells (cyst content varies) up to 20% of benign ovarian neoplasms 10% of carcinomas 73% benign, 16% malignant, 11% LMP serum marker (CA-125) is less reliable (falsely negative) with mucinous tumors

Figure 3-13-6

Epithelial Ovarian Neoplasms: Mucinous [Figure 3-13-7] • • • • •

thin-walled cyst, usually multilocular often large, may be enormous occasionally, linear calcifications (but calcifications are LESS frequent than with mucinous tumors of colonic origin) solid elements suggest malignancy LMP tumors are associated with pseudomyxoma peritonei ➢ cause / effect relationship is often unclear

Pseudomyxoma peritonei [Figure 3-13-8] • • • • •

usually arises from appendix often difficult to determine whether the process originated from the appendix, ovary, or both (synchronous) prolonged, uncomfortable survival (limited treatment options) low density, “scalloping” seen on CT “Pseudomyxoma peritonei is a poorly understood condition and it is unclear whether its continous production of gelatinous mucin is due to peritoneal implantation of neoplastic mucinous cells or to metaplasia of peritoneal cells into mucinous epithelium, induced by mucin.” [1]

Serous cystadenocarcinoma

Figure 3-13-7

[1] Tropé CG et al. Surgery for borderline tumor of the ovary. Seminars in Surgical Oncology 2000; 19:69–75.

Epithelial Ovarian Neoplasms: Endometrioid • • • • • •

mimics endometrial ca, but is primary to ovary may have malignant stroma (“carcinosarcoma” or “malignant mixed mesodermal tumor” = MMMT) almost all are malignant 10–15% of ovarian cancers 25% of patients have an associated uterine abnormality ➢ endometrial carcinoma (separate primary malignancy) ➢ endometrial hyperplasia 15% of patients have coexistent endometriosis (cause/effect unclear)

Mucinous cystadenoma (LMP?)

Figure 3-13-8

Epithelial Ovarian Tumors: Clear Cell [Figure 3-13-9] •

• •

mimics clear cell cancer of the vagina, but no association with in utero DES exposure 5% of ovarian cancers (all clear cell tumors are malignant) gross appearance is variable: ➢ unilocular cyst with a mural nodule ➢ multilocular Figure 3-13-9 ➢ solid, etc.

Pseudomyxoma peritonei

Clear cell carcinoma Genitourinary Radiology

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Imaging of Ovarian Masses

Ovarian Carcinoma Staging: Local disease (30%) •

•

Stage I ➢ limited to ovary [subtypes] Stage II ➢ extra-ovarian pelvic extension [subtypes]

Figure 3-13-10

Ovarian Carcinoma Staging: Advanced Disease (70%) •

•

Stage III ➢ tumor within the peritoneum (outside the pelvis) [or] retroperitoneal lymph nodes [or] surface of the liver [or] small bowel/omentum (within the pelvis) Stage IV ➢ distant spread ❖ hepatic parenchyma ❖ lung ❖ etc.

Typical Ovarian Cancer Therapy • • •

Surgical: hysterectomy, oophorectomy, appendectomy, omentectomy, removal of peritoneal masses Medical: 6–8 (monthly) cycles of chemotherapy (a platinumbased agent, plus taxol) Second look surgery? (only as part of a structured research protocol)

Ovarian Masses •

•

Non-neoplastic ➢ Physiologic cyst, endometriosis, etc. Neoplastic ➢ Epithelial tumors 65% ➢ Germ cell tumors 25% ➢ Sex-cord stromal tumors 5% ➢ Secondary malignancies 5% ➢ Gonadoblastoma <1%

Ovarian Germ Cell Neoplasms •

most are mature teratomas: ➢ the most common mature teratomas are mature cystic teratomas (commonly referred to as “dermoid cysts”) ➢ less common mature teratomas include carcinoid tumors, struma ovarii, etc.

in the ovary . . . •

all dermoid cysts are mature teratomas (and most, but not all, mature teratomas are dermoid cysts)

Mature Cystic Teratoma [Figures 3-13-10 and 3-13-11] •

•

unilocular cyst ➢ cyst fluid is nearly sonolucent ➢ posterior acoustic enhancement (fluid at body temperature) ➢ images as “fat” (lipid) by CT, MRI – but it is NOT adipose tissue Rokitansky nodule ➢ contains various tissues (cartilage, gastrointestinal epithelium, etc) ➢ echogenic

Mature teratoma

Figure 3-13-11

Malignant transformation of mature teratoma

Imaging of Ovarian Masses

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Genitourinary Radiology

• • • •

Figure 3-13-12

child-bearing years may undergo: ➢ torsion ➢ rupture ➢ malignant transformation (very rare) often discovered as an incidental finding 12% bilateral

Ovarian Malignant Germ Cell Tumors • • • • • •

dysgerminoma (similar to seminoma) embryonal carcinoma endodermal sinus tumor immature teratoma choriocarcinoma mixed germ cell tumor (much less common than testicular mixed GCT)

Malignant Germ Cell Tumors [Figure 3-13-12] •

in general: ➢ younger age group (15–30 years) than epithelial tumors ➢ solid / heterogeneous ➢ highly aggressive ➢ differentiation among the various types is difficult (but immature teratomas are the most likely to have fat, calcification) ➢ may have elevated markers (AFP, HCG)

Sex-cord stromal tumors • •

• •

many are very low grade malignancies generally diagnosed at Stage I (and therefore surgery is often curative) 5–8% of ovarian neoplasms hormonal manifestations include: ➢ estrogenic effects: pseudoprecocious puberty, endometrial stimulation ➢ virilization (less common)

Sex-cord stromal tumors •

• •

fibrothecoma ➢ 50% of all sex-cord stromal tumors ➢ more common than either pure thecoma or pure fibroma granulosa cell tumors ➢ including juvenile variety Sertoli-Leydig ➢ more common than either pure Sertoli or Leydig cell tumors ➢ rare, but the most common virilizing tumor of the ovary

Dysgerminoma

Figure 3-13-13

Fibroma / fibrothecoma

Figure 3-13-14

Hemorrhagic infarction of fibroma

Figure 3-13-15

Fibrothecoma [Figures 3-13-13 and 3-13-14] • • •

thecoma component produces estrogen fibroma component accounts for low signal on T2-weighted MRI sonographically, they tend to be homogeneously hypoechoic but sound-attenuating

Granulosa cell tumors [Figure 3-13-15] • •

“sponge-like” appearance on imaging multicystic lesion with hemorrhage in a patient under 30 suggests juvenile granulosa cell tumor (but these account for only 5% of granulosa cell tumors overall) Granulosa cell tumor

Genitourinary Radiology

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Imaging of Ovarian Masses

Sex-cord stromal tumors • • • • •

•

low signal on T2 suggests fibroma hypoechoic sound-attenuating lesion suggests fibroma diagnosis of this and other sex-cord stromal tumors may be possible if clinical factors are taken into consideration (morphology is generally solid and nonspecific) “. . . difficult to suggest a simple algorithm for evaluation of women with ovarian masses” [1] “[Doppler U/S, CT, and MRI] yielded similar [results] for discrimination between benign disease and cancer . . . Although differentiation of benign from malignant disease is obviously clinically important and these detection rates are higher than those previously reported, they are likely still not high enough for surgery to be avoided in most cases.” [1] “Whatever the modality used, it is hoped that correct staging of advanced disease will lead to appropriate referral to a specialist in gynecologic oncology.” [1]

[1] Kurtz A et al Radiology 1999 Jul;212(1):19–27

Scoring systems for ovarian tumors • • • • • •

wall thickness nodularity septations echogenicity ascites? size?

•

wall irregularities ➢ smooth --> papillary projections wall thickness ➢ thin --> thick (< or > 3 mm) septa ➢ none --> thin--> thick echogenicity ➢ low --> high ascites? size?

Ovarian Masses: Sonographic scoring •

• • •

Doppler sonography* • • •

ideally, should allow more specificity and sensitivity for malignancy based on low resistance flow (high diastolic flow) in malignant neovascularity significant overlap with benign processes, especially in pre-menopausal women

* = controversial

Doppler sonography of ovarian masses* •

•

it should work: ➢ in a large series of patients, the presence of high diastolic flow is predictive of malignancy however, it is of limited usefulness: ➢ specificity is limited, especially in pre-menopausal patients ➢ there is considerable overlap of benign vs. malignant

* = controversial

Imaging of Ovarian Masses

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Genitourinary Radiology

Doppler sonography of ovarian masses* [Figure 3-13-16] •

• • •

Figure 3-13-16

proposed threshold values: ➢ Resistive index (RI) = .45 (or .50) ➢ Pulsatility index (PI) = 1.0 below these values: suggests malignancy corpus luteum may give false positive incomplete sampling may give false negative

* = controversial

Doppler sonography of ovarian masses* • •

do not use the RI or PI values at the exclusion of the sonographic morphology color / amplitude Doppler may be of use in characterizing areas that may be confusing or indeterminate morphologically (for example, clot vs. tissue)

Use of resistive index in assessment of ovarian masses

* = controversial

Is pre-operative staging of ovarian cancer important? Maybe not, because . . . • •

“all” patients go to surgery (cytoreduction) in most centers, staging laparotomies are performed by a gynecologic oncologist

Is prediction of malignancy in a neoplastic mass important? • •

May determine the surgical approach May determine who the surgeon is: ➢ If “probably benign”, general gynecologist ➢ If “probably malignant”, gynecologic oncologist

Is the differentiation of a neoplasm from a non-neoplastic ovarian mass important? •

Yes (and it′s usually accomplished sonography)

•

Acute symptoms? ➢ check pregnancy test ➢ check for fever, elevated white blood cell count ➢ if severe acute pain, consider torsion

Pre-menopausal (ovulating) patient

Pre-menopausal (ovulating) patient •

If sub-acute or mild symptoms: ➢ simple cyst < 30 mm, no follow-up ➢ “hemorrhagic cyst” < 25 mm, no follow-up ➢ simple cyst 30–60 mm, follow-up in 6–10 weeks ➢ “hemorrhagic cyst” 25–60 mm, follow-up in 6–10 weeks ➢ any appearance > 60 mm, consider surgery ➢ any soft tissue component (septation, etc), consider surgery

Post-menopausal patient • •

•

simple cyst <16 mm: ignore simple cyst 16–50 mm: follow-up 4 months ➢ presumed serous inclusion cyst vs. benign neoplasm simple cyst > 50 mm OR any complex lesion: consider surgery

Genitourinary Radiology

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Imaging of Ovarian Masses

References 1. 2. 3.

4. 5. 6. 7. 8. 9.

10. 11.

12. 13. 14.

Gajewski W, Legare RD. Ovarian cancer. Surg Onc Clin N Am 1998; 7:317. Hricak H, Chen M, Coakley FV. Complex adnexal masses: detection and characterization with MR imaging -multivariate analysis. Radiology 2000; 214:39. Jung SE, Lee JM, Rha SE, Byun JY, et al. CT and MR Imaging of Ovarian Tumors with Emphasis on Differential Diagnosis. Radiographics 2002; 22:1305. Kawamoto S, Urban BA, Fishman EK. CT of epithelial ovarian tumors. Radiographics 1999; 19:S85. Kinkel K, Lu Y, Mehdizade A, et al. Indeterminate ovarian mass at US: incremental value of second imaging test for characterization – meta-analysis and Bayesian analysis. Radiology 2005; 236:85-94. Koonings PP, Campbell K, Mishell DJ, Grimes DA. Relative frequency of primary ovarian neoplasms: a 10-year review. Obstet Gynecol 1989; 74:921-926. Kurtz AB, et al. Diagnosis and Staging of Ovarian Cancer: Comparative Values of Doppler and Conventional US, CT, and MR Imaging Correlated with Surgery and Histopathologic Analysis—Report of the Radiology Diagnostic Oncology Group. Radiology 1999; 212:19. Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex-cord stromal and steroid cell tumors of the ovary. RadioGraphics 1998; 18:1523. Patel MD, Feldstein VA, Lipson SD, Chen DC, and Filly RA. Cystic teratomas of the ovary: diagnostic value of sonography. Am J Roentgenol 1998; 171:1061-1065. Siegelman ES, Outwater, EK. Tissue Characterization in the Female Pelvis by Means of MR Imaging. Radiology 1999; 212:5. Sironi S, Messa C, Mangili G, Zangheri B, et al. Integrated FDG PET/CT in Patients with Persistent Ovarian Cancer: Correlation with Histologic Findings. Radiology 2004; 233:433. Tanaka YO, Tsunoda H, Kitagawa Y, Ueno T, et al. Functioning Ovarian Tumors: Direct and Indirect Findings at MR Imaging. RadioGraphics 2004; 24:S147. Wagner BJ, Buck JL, Seidman JD, McCabe KM. Epithelial Neoplasms of the Ovary: Radiologic-Pathologic Correlation. RadioGraphics 1994; 14:1351. Woodward PJ, Hosseinzadeh K, Saenger JS. Radiologic Staging of Ovarian Carcinoma with Pathologic Correlation. RadioGraphics 2004; 24:225.

Imaging of Ovarian Masses

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Adrenal Imaging in Adults Brent J. Wagner, MD Neoplastic • • • • • • •

Adenoma Metastasis Lymphoma Pheochromocytoma Adrenocortical Carcinoma Myelolipoma Hemangioma (rare)

• • • • •

Hemorrhage Inflammation Hyperplasia Cyst Pseudocyst*

Non-neoplastic

*may be secondary to neoplasm (adenoma)

Clinical manifestations of adrenal tumors •

• •

•

Aldosteronism (hypertension, hypokalemia) ➢ 80% due to adenoma (Conn’s syndrome) ➢ 20% due to hyperplasia ➢ < 1% due to adrenal cortical carcinoma Virilization ➢ most due to hyperplasia ➢ 15% due to adenoma ➢ 5% due to carcinoma Cushing’s syndrome (hypertension, obesity, diabetes, etc) may be due to: ➢ exogenous steroids (most common) ➢ pituitary adenoma → ACTH production → bilateral adrenal hyperplasia ➢ non-pituitary tumor → ACTH production → bilateral adrenal hyperplasia ➢ 20% due to adrenal adenoma ➢ 10% due to carcinoma Catecholamine excess (hypertension, tachycardia, flushing, etc.) ➢ pheochromocytoma

Adenoma [Figure 3-14-1] •

(3% of the general population) ➢ Non-hyperfunctional (vast majority) ➢ Hyperfunctional (imaging features are the same as non-hyperfunctional)

Adenoma microscopic pathology • •

clear cells (high lipid content) cords of fibrovascular tissue

• • •

well-circumscribed homogeneous small (usually less than 3 cm)

Figure 3-14-1

Adenoma (typical) macroscopic pathology

Adrenal adenoma Genitourinary Radiology

645

Adrenal Imaging in Adults

Adenoma with degeneration (atypical) [Figure 3-14-2] • • • • •

heterogeneous hemorrhagic cystic / necrotic calcifications (gross and radiologic appearance mimics carcinoma)

Figure 3-14-2

Adenoma radiology

CT findings (NCCT): ➢ small, homogeneous ➢ hypodense due to lipid content (< 18HU?, <15HU?, <10HU?) • CT findings (CECT): ➢ decreased enhancement compared to metastasis, etc. ➢ rapid wash-out of contrast? • Some adenomas are “lipid poor” • A mass that does not satisfy the density requirements for an adenoma may still be an adenoma (biopsy or washout study required?) • A mass that does not decrease in signal on an opposed phase image may still be an adenoma • MR (opposed phase imaging) [Figure 3-14-3] ➢ spleen used as internal reference ➢ visual assessment is generally adequate, although signal intensity ratios of lesion:spleen may be used • Opposed phase MRI operates on the same principle (lipid content) as non-contrast CT, therefore will generally add little to the patient work-up* (i.e. an indeterminate lesion by CT will likely be indeterminate on opposed phase MRI). * controversial •

Degenerating adenoma

Figure 3-14-3

Adrenal Carcinoma [Figure 3-14-4] • • • • • • •

rare heterogeneous large ( mean >10 cm) necrotic percutaneous biopsy unreliable more than 1/3 are calcified half are hyperfunctional (these are generally smaller)

Adrenal adenoma, including opposed phase imaging

Figure 3-14-4

Adrenal Imaging in Adults

Adrenocortical carcinoma with extension to inferior vena cava

646

Genitourinary Radiology

Pheochromocytoma [Figures 3-14-5 and 3-14-6] • • • • • • • • •

almost all are abdominal depends on definition – perhaps all are “adrenal” 90% are adrenal (the remainder are “paragangliomas”) 90% are unilateral 90% are “benign” — benignity established by clinical follow-up elevated catecholamines imaging generally performed for localization, not diagnosis CT: ➢ 3 - 6 cm mass ➢ heterogeneous when large (often cystic) ➢ calcification < 5% MRI: ➢ historically, characterized as very high signal on T2 ➢ not totally specific, but normally needed only for localization MIBG (iodine-131-meta-iodobenzylguanidine): ➢ high sensitivity and specificity ➢ (but generally not needed and availability is limited)

Myelolipoma [Figures 3-14-7 and 3-14-8]

Figure 3-14-5

Pheochromocytoma

• • • • • • • • •

marrow elements: blood precursors and fat benign — no malignant potential small lesions very unlikely to bleed usually incidental findings, but may present with hemorrhage may be diagnosed by needle biopsy (often not needed) rarely extra-adrenal (differential diagnosis — liposarcoma) most are predominantly fat attenuation (CT) or SI (MR) one third have calcification occasionally, associated with hormonal activity

• •

Pheochromocytoma Mass is very large: favor adrenocortical carcinoma Mass is between 2- 6cm in patient with hypertension: hyperfunctioning adenoma vs. pheochromocytoma Mass contains a cystic portion and is less than 6 cm: pheochromocytoma Mass is primarily very high signal on T2: suggests pheochromocytoma Calcification: inflammatory, old hemorrhage, adrenocortical ca [unlikely: mets, pheo] Small, homogeneous, hypodense = adenoma

Figure 3-14-6

Adrenal Mass Evaluation: Helpful features • • •

•

Figure 3-14-7

Figure 3-14-8

Myelolipoma

Genitourinary Radiology

Myelolipoma

647

Adrenal Imaging in Adults

Absolute washout calculation • • •

“percentage of enhancement washout” (HUdyn – HUdelayed) / (HUdynamic – HUpre) if greater than 60%, = adenoma

• •

(HUdyn – HUdelayed) ?(HUdynamic) if > 40%, = adenoma

Relative washout calculation Algorithm • • •

NCCT: ➢ If less than 10 HU, it’s an adenoma [STOP] ➢ If more than 10 HU, proceed to: CECT: (dynamic and 15* minute delay) ❖ If less than 30* HU on delayed scan = adenoma ? ❖ If more than 30 HU on delayed scan, what is washout value * = controversial

Opposed phase MRI (OPMRI) ➢ decrease in signal relative to spleen = adenoma → no further evaluation needed ➢ no decrease → biopsy ➢ indeterminate → consider CT evaluation [will probably need “washout”/delay scans because the lipid content is probably too small to make the lesion sufficiently hypodense]

References 1.

2.

3.

4.

5.

6. 7. 8. 9.

Blake MA, Kalra MK, Sweeney AT, et al. Distinguishing beinign from malignant adrenal masses: multi-detector row CT protocol with 10-minute delay. Radiology 2005; 238:578-85. Blake MA, Slattery JMA, Kalra MK, et al. Adrenal lesions: characterization with fused PET/CT image in patients with proved or suspected malignancy – initial experience. Radiology 2006; 238:970-77. Caoili EM, Korobkin M, Francis IR, et al. Adrenal masses: characterization with combined unenhanced and delayed enhanced CT. Radiology 2002; 222:629-33. Elsayes KM, Narra VR, Leyendecker JR, et al. MRI of adrenal and extraadrenal pheochromocytoma. Am J Roentgenol 2005; 184:860-67. Haider MA, Ghai S, Jhaveri K, Lockwood G. Chemical shift MR imaging of hyperattenuating (>10 HU) adrenal masses: does it still have a role? Radiology 2004; 231:711. Kenney PJ, Wagner BJ, Rao P, Heffess CS. Myelolipoma: CT and pathologic features. Radiology 1998; 208: 8795. Korobkin M. CT characterization of adrenal masses: the time has come. Radiology 2000; 217:629. Mayo-Smith WW. CT characterization of adrenal masses (letter). Radiology 2003; 226:289. Savci G, Yazici Z, Sahin N, et al. Value of chemical shift subtraction MRI in characterization of adrenal masses. Am J Roentgenol 2006; 186:130-53.

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Imaging of the Urinary Bladder and Urethra Brent J. Wagner, MD Outline •

•

Figure 3-15-1

Bladder ➢ filling defects ➢ wall thickening (+/– calcification) ➢ abnormal contour Urethra ➢ anatomy ➢ filling defects ➢ obstructive processes (strictures, valves)

Bladder: Filling defects • • • • • • • •

neoplasm calculus clot fungus ball ureterocele endometriosis schistosomiasis (prostate)

• • • • • •

transitional cell ca (TCC) (urothelial) squamous cell ca adenocarcinoma leiomyoma/sarcoma hemangioma metastasis ➢ invasion ➢ other embryonal rhabdomyosarcoma (child)

Bladder: Types of Neoplasms

•

Bladder Neoplasms: (TCC), urothelial carcinoma [Figure 3-15-1]

• • • • • • • •

males > females 80% over age 50 typically, projects into lumen papilloma = low grade TCC irregular surface, “papillary” occasionally (30% ?) multifocal CT to assess extraluminal extent enhances on early CT scan; filling defect on delayed scan

Urothelial carcinoma

Figure 3-15-2

Bladder neoplasms: Differential features [Figures 3-15-2 and 3-15-3

• • •

TCC = common squamous cell carcinoma = look for associated stones, history of infection (Schistosomiasis?), or chronic indwelling catheter adenocarcinoma = often of urachal origin; look for calcified anterior midline mass with prominent extracystic growth

Urachal Anomalies • • • •

patent urachus umbilical-urachal sinus vesico-urachal diverticulum urachal cyst

Genitourinary Radiology

Urachal carcinoma 649

Imaging of the Urinary Bladder and Urethra

Filling Defects: (may be mobile) •

•

•

clot ➢ often smooth stones ➢ shadowing on U/S, midline on supine radiograph ➢ occasionally radiolucent (or obscured) post-contrast ➢ history of infection (and/or) ➢ evidence for bladder outlet obstruction ❖ trabeculation ❖ hydroureter ❖ prostate impression fungus ball ➢ laminated, gas-containing

Figure 3-15-3

Filling Defects: Miscellaneous: [Figures 3-15-4 and 3-15-5] •

• •

•

ureterocele ➢ smooth prostate ➢ midline, generally smooth endometriosis ➢ can look like anything gastrointestinal inflammation ➢ Crohn’s ➢ diverticulitis

Bladder leiomyoma

Figure 3-15-4

Wall thickening [Figure 3-15-6] •

• • •

I. cystitis and variants ➢ infection ❖ TB* ❖ Schistosomiasis* ❖ malakoplakia ❖ cystitis cystica (lobulated, diffuse) ➢ radiation* ➢ post-cytoxan* II. Neoplasm (TCC) III. Bladder outlet obstruction IV. Inflammation/invasion

Prostate carcinoma

Figure 3-15-5

*may calcify

Malakoplakia • •

•

most common in females with recurrent infection mimics infiltrating carcinoma ➢ cysto/bx to diagnose Michaelis-Gutman bodies

Cystitis cystica et glandularis • • • • •

etiology/significance is controversial (? inflammatory) regenerative / reparative “proliferative cystitis” may result in wall thickening, but typically there are no imaging findings prominent dilated glandular lumina

Endometriosis

Figure 3-15-6

Tuberculosis Imaging of the Urinary Bladder and Urethra

650

Genitourinary Radiology

Cystitis glandularis – is it pre-malignant? • • • •

“Cystitis glandularis is so common that it may be considered a normal feature of the vesical mucosa.” There are 2 types of cystitis glandularis ➢ “typical” ➢ “intestinal” (less common) “Diffuse cystitis glandularis of the intestinal type is termed intestinal metaplasia and usually occurs in chronically irritated bladders such as those of paraplegics or in patients with stones or long term catheterization . . . it is associated with an increased risk of bladder carcinoma.” “It is only the intestinal type of cystitis glandularis that is associated with adenocarcinoma.”

Young RH, Eble JN. Non-neoplastic disorders of the urinary bladder. In: Urologic Surgical Pathology. Mosby 1997. pp 174–5.

Schistosomiasis • • •

calcification in 50% calcification is rare in transitional / urothelial carcinoma Schistosomiasis is a risk factor for squamous cell ca of the bladder

• • •

combination of regional wall thickening and invasion diverticulitis more common than regional enteritis associated findings with regional enteritis ➢ calculi ➢ anterior/right (posterior/left for diverticulitis) progression: ➢ impression/thickening ➢ invasion ➢ fistula

Regional enteritis (Crohn’s) or other gastrointestinal disease

•

Figure 3-15-7

Emphysematous cystitis • • • • •

urinary tract combined with uncontrolled diabetes mellitus gas may be intraluminal* as well as intramural ➢ linear, lucent streaks non-surgical condition treatment: antibiotics and insulin * if gas is only intraluminal, consider fistula

Abnormal contour •

•

smooth narrowing: ➢ pelvic lipomatosis ➢ pelvic hematoma ➢ (irregular narrowing = lymphoma, other mass?) focal outpouching (diverticula): ➢ bladder outlet obstruction ➢ stones/tumors/bleeding ➢ reflux/ureteral obstruction ❖ (especially in children)

Urethra: Anatomy •

•

posterior: ➢ prostatic ➢ membranous anterior: ➢ bulbous ➢ penile

Condyloma acuminata Genitourinary Radiology

651

Imaging of the Urinary Bladder and Urethra

Urethrography: Technique

Figure 3-15-8

• • • •

Clamp vs. catheter Fluoroscopic guidance Hand injection Usually, dilute (30%) contrast

•

urethral carcinoma ➢ most are squamous (if proximal, consider transitional/urothelial carcinoma) ➢ 70% of cases in males are associated with postinflammatory stricture ➢ filling defect or irregular stricturing condyloma acuminata [Figure 3-15-7] ➢ urethral disease in only 5% of pts with external lesions ➢ viral

Urethra: Masses/filling defects

•

Acute and subacute gonococcal urethritis

Figure 3-15-9

Urethra: Strictures [Figure 3-15-8] •

• •

post-inflammatory ➢ especially gonococcal (40% of strictures in the U.S.) post-traumatic ➢ includes iatrogenic may be associated with perineal fistula

Urethra: Obstructive processes [Figure 3-15-9] • •

posterior urethral valve anterior urethral valve ➢ (vs. diverticulum) ➢ acquired or congenital ➢ may obstruct, or develop calculi

Urethral diverticulum (male)

Figure 3-15-10

Urethra: Diverticulum of the female urethra [Figure 3-15-10 •

• •

outpouching of contrast ➢ may require double-balloon technique fluid-filled mass on CT, MR, or sonography associated with carcinoma (usually squamous)

Urethral diverticulum (female)

References

1. Beer A, Saar B, Rummeny EJ. Tumors of the urinary bladder: technique, current use, and perspectives of MR and CT cystography. Abdom Imaging 2003; 28:868. 2. Hahn WY, Israel GM, Lee VS. MRI of female urethral and periurethral disorders. Am J Roentgenol 2004; 182:677-82. 3. Pavlica P, Menchi I, Barozzi L. New imaging of the anterior male urethra. Abdom Imaging 2003; 28:180. Yu J-S, Kim KW, Lee H-J, Lee Y-J, et al. Urachal remnant diseases: spectrum of CT and US findings. RadioGraphics 2001; 21: 451.

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Non-Neoplastic Disorders Of The Ovary And Adnexae And GTD Jade Wong You Cheong, MD Outline • •

Clinical and imaging characteristics of common non neoplastic ovarian and adnexal pathology Role of CT / MR Gestational trophoblastic disease

• • • • • •

Age of patient Symptoms e.g fever, discharge Menstrual status and time in cycle Pregnancy status Previous surgery and medical history Drugs, e.g HRT, ovulation stimulation

• • •

Very common incidental findings occurring during normal ovarian cycle Failure of ovulation or development of fluid in corpus luteum Most regress spontaneously

• • •

Follicular Corpus luteal Theca lutein

• • • • • • •

Failure of mature follicle to rupture or regress Usually 3–8 cm Unilocular simple cyst Well defined thin smooth wall Usually asymptomatic Regress spontaneously (if <5 cm) or may respond to hormonal suppression Clinical or sonographic follow-up in 6–8 weeks

•

Essential Clinical Information

Functional Ovarian Cysts Functional Ovarian Cysts Follicular Cyst [Figure 3-16-1]

Figure 3-16-1

Follicular cyst of left ovary with resolution one month later. a. initial transabdominal ultrasound. b. initial transvaginal ultrasound. Echoes are artefactual. c. Follow up.

Genitourinary Radiology

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Non-Neoplastic Disorders Of The Ovary And Adnexae

Normal Ovaries-MR [Figure 3-16-2]

Figure 3-16-2

MR of normal ovaries. a. T1-w image: left ovary (arrow) is isointense to muscle. On T2-w (b and c) images the right and left ovaries (arrows) contain multiple high signal follicles

Follicular Cyst [Figure 3-16-3]

Figure 3-16-3

Corpus Luteum Cyst [Figure 3-16-4]

•

• • • • •

Persistence of corpus luteum or bleeding into it >3 cm Unilocular Thick vascular wall Wall slightly echogenic CL cyst of pregancy regresses by 16 wk

Corpora Lutea

Symptomatic functional cysts • •

a. Sagittal and b. coronal T2-w MRI showing a right sided follicular cyst (arrow) with surrounding ovarian parenchyma

Internal hemorrhage Rupture ➢ May rupture and bleed into peritoneal cavity with peritoneal signs and hypotension ➢ Or rupture with simple free fluid

Figure 3-16-4

Hemorrhagic Functional Cysts [Figure 3-16-5] • • • • • • • • • •

Hemorrage occurs into existing cyst Acute pain or asymptomatic More common in luteal cysts Imaging spectrum depends on age Rapid change Thin linear fibrin strands “reticular, fish net or lacy” Retracting hyperechoic clot Fluid – debris level Mildly thickened wall Diffuse low level echoes with acoustic enhancement “ground glass” ➢ Rare, usually a feature of endometriosis

Figure 3-16-5

Interval development of hemorrhage into a functional cyst. Note fine lacy linear echoes (arrow)

Non-Neoplastic Disorders Of The Ovary And Adnexae

654

Thick walled unilocular cyst with low resistance arterial flow in wall Genitourinary Radiology

Hemorrhagic Cyst [Figure 3-16-6]

Figure 3-16-7

Figure 3-16-6

Acute left pelvic pain. US and CT of left ovarian hemorrhagic cyst. Note fishnet appearance on US and hematocrit level on CT (arrow)

MR •

Blood products ➢ High on T1 ➢ Usually high on T2

Acute pain from cyst rupture.Note free fluid and crenated cyst (bottom)

Cyst Rupture [Figure 3-16-7]

Cyst Rupture with Hemorrhage [Figure 3-16-8] Theca Lutein Cysts •

•

Gestational trophoblastic disease ➢ Associated with high levels of HCG Ovarian hyperstimulation syndrome ➢ Secondary to infertility drugs ➢ Abdominal pain, distension, nausea, vomiting

Figure 3-16-8

Hyperstimulation Cysts • • • •

Bilateral enlarged ovaries Multiple large cysts May bleed, rupture or torse OHSS associated with ascites, pleural effusion, hemorrhage, DIC

OHSS [Figure 3-16-9] Endometriosis • •

Functioning ectopic endometrium Pelvic peritoneum, ovary, tube

•

Symptoms ➢ Dysmenorrhea ➢ Dyspareunia ➢ Pelvic pain Cyclic pain with menses Associated with infertility ➢ Prevalence 25%

Endometriosis

• •

Genitourinary Radiology

Acute pain and pelvic guarding. Bleeding from left corpus luteum cyst * into peritoneal cavity. U= uterus

655

Non-Neoplastic Disorders Of The Ovary And Adnexae

Figure 3-16-9

Endometrioma - US

Ascites and bilateral enlarged ovaries with multiple cysts

[Figures 3-16-10 and 3-16-11]

• • • • •

Thick walled cystic lesion “Ground glass” homogeneous low level echoes (highly suggestive) Unilocular or multilocular with septations Mural reflectors Rarely fluid-fluid level

Figure 3-16-10

Endometriosis: MR Technique • •

Ground glass appearance of endometriotic cyst. a. Transabdominal Axial T1-w SE *Axial fat-suppressed T1-w SE to and b. transvaginal ultrasound. Note homogeneous internal echoes and posterior acoustic enhancement distinguish fat from blood* Axial/sagittal/coronal T2-w FSE Dynamic enhanced T1-w (optional)

• • • •

Highly accurate, sensitive and specific (90-96%) Thick walled cystic lesion Hyperintense on T1-w Hypointense on T2-w with “shading”

•

Less specific signs ➢ Multiple homogeneous hyperintense lesions on T1-w and T2-w ➢ Low signal hemosiderin ring ➢ Enhancement of cyst wall/peritoneum

• •

Endometrioma: MR [Figure 3-16-12]

Figure 3-16-11

Endometrioma: MR

Endometrioma

Homogeneous endometriomas (*) with mural reflectors (arrows)

Endometrioma with hematosalpinx Rectus Endometriosis

Diffuse Endometriosis • • • •

More common Associated with fibrosis and adhesions Laparoscopy is gold standard allows staging and treatment MR may be useful for inaccessible sites or for evaluation of response to medical treatment

Non-Neoplastic Disorders Of The Ovary And Adnexae

Figure 3-16-12

High T1 signal ovarian endometrioma with no suppression on fat sat. Low signal “shading” on FSE T2 656

Genitourinary Radiology

Endometriosis

Figure 3-16-13

Endometrioma or Hemorrhagic Cyst? • • •

Clinical history Sequential imaging with US MR ➢ Less bright on T1-w ➢ No shading on T2-w ➢ Single

“Rule Out Ovarian Torsion”

Ovarian (adnexal) Torsion [Figure 3-16-13] • • • • • •

3% of gynecologic emergencies Usually premenopausal 20% pregnant 80% associated mass Acute pain, nausea, vomiting Previous self limiting episodes

•

Gray scale non specific, depends on cause ➢ Most suggestive: ipsilateral enlarged hypoechoic ovary (+/peripheral follicles) ➢ Mass e.g. teratoma, functional cyst ➢ Hemorrhagic infarction ➢ Associated thickened fallopian tube

Ovarian Torsion - US

Ovarian Torsion: Enlarged Ovary [Figure 3-16-14] Adnexal Torsion

Figure 3-16-14

Ovarian Torsion - US •

Absence of flow in torsed left ovary (bottom)

Doppler is extremely useful ➢ Absence of arterial and venous flow ➢ High resistance arterial flow ➢ Loss of venous flow ➢ Twisted vascular pedicle ➢ Corkscrew vessels

Ovarian Torsion [Figure 3-16-15] Figure 3-16-15

Asymmetric ovarian sizes in torsion A-CT of enlarged right ovary with hemorrhagic infarction. Paraovarian cyst (arrow) caused the torsion. B-Low signal ovary on T2 with folllicles. C-T1 shows high signal from hemorrhage. D-Post contrast sat sat T1 shows no enhancement in right ovary Genitourinary Radiology

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Non-Neoplastic Disorders Of The Ovary And Adnexae

Torsed Teratoma [Figure 3-16-16]

Figure 3-16-16

Adnexal Torsion: CT/MR • • • • •

Deviation of uterus to affected side Obliteration of fat planes Enlarged displaced ovary Beak sign with congested vessels Lack of enhancement

Torsion of Cystadenoma*

Ovarian Torsion Twisted Pedicle [Figure 3-16-17]

Ovarian torsion caused by para-ovarian cyst

Twisted vascular pedicle on color Doppler of a torsed teratoma

Ovarian Torsion

• •

Diagnostic difficulties ➢ Dual ovarian arterial supply ➢ Incomplete and intermittent torsion ➢ False positives ❖ Technical ❖ Pathologic High index of suspicion if symptomatic ovary enlarged Rescan early

• • •

Tubular fluid filled structure Folding mimics multilocular lesion Sequela of PID, endometriosis, surgery

•

Figure 3-16-17

Hydrosalpinx [Figure 3-16-18] Figure 3-16-18

Twisted pedicle (arrow): intra operative image and CT Dilated thin walled fallopian tube: US and CT

Hydrosalpinx/Pyosalpinx Hydrosalpinx

Non-Neoplastic Disorders Of The Ovary And Adnexae

658

Genitourinary Radiology

Pelvic Inflammatory Disease •

• • • • • •

Figure 3-16-19

Imaging for: ➢ Complications ➢ Failure to respond to first line treatment ➢ Alternative diagnosis US first line CT or MR for difficult / severe cases Thick walled tube Cog wheel Internal echoes Tuboovarian complex or abscess ➢ Complex adnexal mass with pyosalpinx

Acute Salpingitis

Pyosalpinx [Figure 3-16-19]

Tuboovarian Abscess Aspiration Tuboovarian abscesses

Ovarian Vein Thrombophlebitis • •

• • • • • • •

Septic thrombosis in ovarian veins Post partum or post surgery/pelvic inflammatory disease Pain, fever and leucocytosis My be occult Treated with antibiotics and anticoagulants Distended ovarian vein Thrombus Perivenous inflammatory changes Edematous adnexa

a. Coronal and b. longitudinal ultrasound of a dilated thickened tube with internal debris and endosalpingeal fold thickening (arrow). c. longitudinal scan of a thickened dilated tube. d. pathologic specimen showing endosalpingeal folds (arrow)

Figure 3-16-20

Post Partum Thrombophlebitis [Figure 3-16-20]

Peritoneal Inclusion Cysts [Figures 3-16-21 and 3-16-22]

“Benign cystic mesothelioma, multilocular peritoneal cyst” • Loculated peritoneal fluid within adhesions • Previous pelvic surgery/endometrosis/PID • Pre or post menopausal • Treatment ➢ Surgical (30–50% recurrence) ➢ OCP +/– TV US guided aspiration • May mimic a cystic ovarian neoplasm • Septated cystic peritoneal lesion surrounding normal ovary • Ovary suspended by adhesions “spider in a web” • Flow may be present in septations

Figure 3-16-21

Multilocular cystic lesion surrounding normal ovary (o) Genitourinary Radiology

659

MR Venogram. Distended right ovarian vein with intra luminal thrombus (arrow). Uterus (*) is enlarged with central fluid/clot Non-Neoplastic Disorders Of The Ovary And Adnexae

Polycystic Ovary Syndrome • • •

• • • •

Figure 3-16-22

Infertility and hormonal disturbance 3–7% of women “Stein Leventhal Syndrome” ➢ Amenorrhea, Infertility, Hirsutism Absence of mid cycle LH surge Increased LH: FSH Suspended follicular development Androgen production

Polycystic Ovary Syndrome [Figures 3-16-23 and 3-16-24]

• • • •

Enlarged ovaries (>10–12 cm3) Multiple (>10) small (<8–10 mm) peripheral follicles Echogenic stroma Normal ovaries (30%)

Figure 3-16-23 Large peritoneal inclusion cyst status post bilateral renal transplants. The uterus (arrow) is displaced and compressed by a large pocket of fluid which herniates into the perineum

Figure 3-16-24

Hirsutism and amenorrhea. Enlarged ovaries with multiple peripheral follicles

Non-Neoplastic Disorders Of The Ovary And Adnexae

Enlarged ovaries with multiple small follicles 660

Genitourinary Radiology

Paraovarian/Paratubal Cysts • • • •

10–20% of adnexal masses Arise in broad ligament from mesothelial and paramesonephric remnants Any age (3rd–4th decades most common) Complicated by ➢ Hemorrhage ➢ Torsion ➢ Rupture ➢ Neoplasm

Paraovarian Cysts • • • •

Simple unilocular adnexal cyst Separate from ovary Lack of change with time Rarely bilateral or multiple or complex

• • • •

17% of asymptomatic post menopausal women <3 cm, thin walled unilocular cysts Cyclic variation Secondary to remote ovulation with trapping of surface epithelium in ovarian cortex Majority resolve, follow-up sonography

Serous Inclusion Cysts

•

Epithelial Inclusion Cysts

Gestational Trophoblastic Disease • • • •

Heterogenous group of disorders Abnormal proliferation of chorionic tissues Varying propensity to invade and metastasize Elevated beta HCG ➢ Hyperemesis, toxemia, bleeding

Gestational Trophoblastic Disease •

•

Benign ➢ Hydatidiform mole Malignant ➢ Invasive mole ➢ Choriocarcinoma ➢ Placental site trophoblastic disease

Gestational Trophoblastic Disease • •

Chorionic villi of blighted ovum persist Hydropic change in placenta

• • •

Most common (80%) 1 in 1200–2000 pregnancies (US) Risk factors ➢ Extremes of reproductive life ➢ Previous mole ➢ Risk of recurrence 1% after 1 mole ➢ 23% after 2 molar pregnancies

Benign Hydatidiform Mole

Benign Mole

Classic “complete” mole ➢ 80% ➢ 46 XX ➢ Complete molar change ➢ No fetal tissue ➢ Nuclear DNA paternal ➢ 10% malignant change

Genitourinary Radiology

Partial mole Triploid 69 XXY 80% 69 XXX Hydropic placenta 0.5% malignant change 661

Non-Neoplastic Disorders Of The Ovary And Adnexae

Complete Mole - Sonography • • • • • •

Enlarged uterus Echogenic mass in endometrial cavity Small cystic spaces Low impedance flow Theca lutein cysts (20–50%) May mimic incomplete abortion, hydropic placenta

Figure 3-16-25

Transabdominal and transvaginal US of cystic endometrial mass.(left and center) Theca lutein cysts in right ovary (right)

Hydatidiform Mole [Figure 3-16-25]

Complete Hydatidiform Mole [Figure 3-16-26]

Figure 3-16-26

Theca Lutein Cysts Partial Mole •

• •

Triploid fetus ➢ IUGR, anomalies Hydropic placenta Spontaneous abortion

Gestational Trophoblastic Disease •

Thickened endometrium with myometrial hypervascularity (*). Arrows: theca lutein cysts

Management ➢ D&C ➢ Monitoring of beta HCG levels ❖ Exponential drop (near zero by 10–12 weeks) ➢ US to exclude pregnancy ➢ Invasive mole 10% → Chemotherapy ➢ Choriocarcinoma 5% → Chemotherapy

Recurrent Complete Mole Malignant GTD •

•

Invasive mole ➢ Locally invasive, non metastatic, <10% ➢ Vesicular chorionic villi with myometrial invasion Choriocarcinoma ➢ 5%, hematogenous metastases to lungs, brain, liver, etc. ➢ May not necessary follow a gestation ➢ No villous structure

Figure 3-14-28

Choriocarcinoma [Figures 3-14-27 and 3-16-28] Figure 3-16-27

Infiltrating myometrial mass (top) Lung metastases (bottom)

Infiltrating cystic mass in endometrial cavity and myometrium Non-Neoplastic Disorders Of The Ovary And Adnexae

662

Genitourinary Radiology

Summary • • •

Characteristic sonographic features allow diagnosis of most benign adnexal masses MR useful for indeterminate adnexal mass HCG and ultrasound for diagnosis and follow up of GTD

References 1. 2. 3. 4. 5. 6. 7. 8. 9.

10.

11.

12.

13. 14.

15. 16. 17. 18. 19. 20.

Albayram F, Hamper UM. Ovarian and adnexal torsion: spectrum of sonographic findings with pathologic correlation. J Ultrasound Med 2001;20:1083-1089. Bennett GL, Slywotzky CM, Giovanniello G. Gynecologic causes of acute pelvic pain: spectrum of CT findings. Radiographics 2002;22:785-801. Christensen JT, Boldsen JL, Westergaard JG. Functional ovarian cysts in premenopausal and gynecologically healthy women. Contraception 2002;66:153-157. Descargues G, Tinlot-Mauger F, Gravier A, Lemoine JP, Marpeau L. Adnexal torsion: a report on forty-five cases. Eur J Obstet Gynecol Reprod Biol 2001;98:91-96. Green CL, Angtuaco TL, Shah HR, Parmley TH. Gestational trophoblastic disease: a spectrum of radiologic diagnosis. Radiographics 1996;16:1371-1384. Hertzberg BS, Kliewer MA, Paulson EK. Ovarian cyst rupture causing hemoperitoneum: imaging features and the potential for misdiagnosis. Abdom Imaging 1999;24:304-308. Jain KA. Imaging of peritoneal inclusion cysts. AJR Am J Roentgenol 2000;174:1559-1563. Lee EJ, Kwon HC, Joo HJ, Suh JH, Fleischer AC. Diagnosis of ovarian torsion with color Doppler sonography: depiction of twisted vascular pedicle. J Ultrasound Med 1998;17:83-89. Levine D, Gosink BB, Wolf SI, Feldesman MR, Pretorius DH. Simple adnexal cysts: the natural history in postmenopausal women. Radiology 1992;184:653-659. Okai T, Kobayashi K, Ryo E, Kagawa H, Kozuma S, Taketani Y. Transvaginal sonographic appearance of hemorrhagic functional ovarian cysts and their spontaneous regression. Int J Gynaecol Obstet 1994;44:47-52. Pache TD, Wladimiroff JW, Hop WC, Fauser BC. How to discriminate between normal and polycystic ovaries: transvaginal US study. Radiology 1992;183:421-423. Patel MD, Feldstein VA, Chen DC, Lipson SD, Filly RA. Endometriomas: diagnostic performance of US. Radiology 1999;210:739-745. Rha SE, Byun JY, Jung SE, et al. CT and MR imaging features of adnexal torsion. Radiographics 2002;22:283294. Sam JW, Jacobs JE, Birnbaum BA. Spectrum of CT findings in acute pyogenic pelvic inflammatory disease. Radiographics 2002;22:1327-1334. Siegelman ES, Outwater EK. Tissue characterization in the female pelvis by means of MR imaging. Radiology 1999;212:5-18. Sohaey R, Gardner TL, Woodward PJ, Peterson CM. Sonographic diagnosis of peritoneal inclusion cysts. J Ultrasound Med 1995;14:913-917. Sugimura K, Okizuka H, Imaoka I, et al. Pelvic endometriosis: detection and diagnosis with chemical shift MR imaging. Radiology 1993;188:435-438. Togashi K, Nishimura K, Kimura I, et al. Endometrial cysts: diagnosis with MR imaging. Radiology 1991;180:7378. Wagner BJ, Woodward PJ, Dickey GE. From the archives of the AFIP. Gestational trophoblastic disease: radiologic-pathologic correlation. Radiographics 1996;16:131-148. Woodward PJ, Sohaey R, Mezzetti TP, Jr. Endometriosis: radiologic-pathologic correlation. Radiographics 2001;21:193-216; questionnaire 288-194.

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Imaging of Solid Organ Transplants Jade Wong You Cheong, MD Transplantation • • • •

Higher success rates with better anti rejection therapy, patient selection and surgical techniques Rejection remains major cause of graft loss Immunosuppression predisposes to infection and neoplasm Symptoms and signs of infection subtle High index of suspicion

• • •

Renal Pancreas Liver

•

Ultrasound -- primary modality ➢ Parenchymal echotexture ➢ Blood supply ➢ Fluid collections ➢ Specific complications ➢ Guidance for interventional procedures

•

Types of Transplants

Post-transplantation Imaging

Post-transplantation Imaging •

CT ➢ ➢ ➢ ➢ ➢

Collections Infection Surgical Complications Neoplasm Guidance for procedures

Post-transplantation Imaging •

MRI and MRA ➢ Parenchyma ➢ Vascular complications ➢ Masses ➢ Rejection?

Post-transplantation Imaging •

Other ➢ Scintigraphy ➢ Cystography ➢ Cholangiography ➢ Arteriography and intervention

Renal Transplants • • • • •

CRT: Cadaveric renal transplant LRT: Living related renal transplant LNRT: Living non-related renal transplant Dual: “En bloc” pediatric or two adult SPK: Simultaneous pancreas-kidney

Imaging of Solid Organ Transplants

664

Genitourinary Radiology

Renal Transplantation: Surgical Technique • •

• •

Iliac fossa extraperitoneal placement Arterial anastomosis ➢ End to side to external iliac artery Venous anastomosis ➢ End to side to external iliac vein Ureteral anastomosis very variable

Renal Transplants: Sonography • • •

•

Hydronephrosis, echotexture, collections Color Doppler: identify vessels Duplex Doppler of ➢ MRA, MRV ➢ Segmental, interlobar and arcuate arteries ➢ Measure RI – x3 Real time guidance for biopsy

Normal Renal Transplant Resistive Index RI= (PSV–EDV) PSV

Complications • • • • • • • •

Perinephric fluid collections (50%) Rejection – acute and chronic Obstruction (1–10%) Vascular Complications (10%) Acute tubular necrosis (DGF) Cyclosporine toxicity PTLD (1%) Torsion

•

•

Early ➢ Hematomas 9% ➢ Seromas ➢ Urinary leak 18% (1–2 weeks) Later ➢ Abscess 30% ➢ Lymphoceles 43% (4–8 weeks) Aspirate for diagnosis

• • • • •

Appearance depends on age Acute hyperechoic Sub acute mixed Chronic hypoechoic/anechoic Contained or free

Figure 3-17-1

Perinephric Fluid Collections

•

Hematoma [Figure 3-17-1]

Sub acute perinephric hematoma (Left). Acute subcaspular hemorrhage with bleeding vessel post biopsy (Center). Organizing hematoma (Right)

Figure 3-17-2

Hemorrhage [Figure 3-17-2]

Acute perinephric high density hematoma.(Left). Free intraperitoneal hemorrhage (Right) Genitourinary Radiology

665

Imaging of Solid Organ Transplants

Urinoma [Figure 3-17-3]

Figure 3-17-3

Lymphocele [Figure 3-17-4] Abscess

Hydronephrosis • •

Early: edema of UVJ Late: Compression by fluid collections ➢ Denervation (non obstructive) ➢ Full bladder (repeat with empty bladder) ➢ Ureteric ischemia, surgical technique Cystogram showing extra luminal contrast (arrow) from ureteral leak.(Left) (kinks) Renogram showing collection of radioactivity (arrow) inferomedial to ➢ Rejection transplant and non visualization of bladder (Right) ➢ Intraluminal clot or calculi

Ureteral Stricture [Figure 3-17-5] Hydronephrosis • •

Dilatation does not equal obstruction RI not reliable in differentiating dilatation from obstruction

Figure 3-17-4

Figure 3-17-5

Echoes Within Collecting System •

Hemonephrosis Low level echoes Move with patient Hematuria Post biopsy: look for AVF ➢ Urinary infection

➢ ➢ ➢ ➢

Hemonephrosis [Figure 3-17-6]

Figure 3-17-6

Simple fluid collection causing some mass effect on the transplant (Top) Lymphocele (arrow) medial to transplant (Bottom)

Hydonephrosis and hydroureter (arrow) (top) Antegrade pyelogram showing distal ureteral stricture (arrow) (bottom)

a. Hydronephrosis with clot in collecting system. b. Clot in bladder. c. CT showing high density blood in left transplant ureter (arrow) and d. clot in bladder (arrow) as well as Foley catheter Imaging of Solid Organ Transplants

666

Genitourinary Radiology

Candidiasis •

Fungus balls ➢ Highly echogenic, weakly shadowing ➢ Candida in urine

Echoes Within Collecting System •

Calculi or nepohrocalcinosis ➢ Echogenic structures with acoustic shadowing

Nephrocalcinosis

Figure 3-17-7

Gas • •

Emphysematous pyelonephritis Reflux from catheterization

Gas/Stent Rejection • • • • •

Non specific elevation of creatinine Fever, white count, pain over transplant Decreased urine outpout Acute (> 5 days) reversible with treament Chronic (months to years) irreversible

•

Non specific ➢ Enlargement ➢ Increased cortical echogenicity ➢ Decreased echogenicity of central sinus ➢ Loss of corticomedullary differentiation ➢ Prominent pyramids ➢ Thickening of collecting system

Acute Rejection Gray Scale

Edematous kidney with prominent pyramids (arrow), increased cortical echogenicity and thickened urothelium (*)

Figure 3-17-8

Acute Rejection [Figure 3-17-7]

Thick Urothelium [Figure 3-17-8]

Acute Rejection

Circumferentially thickened renal pelvis (arrow) in acute rejection Vascular rejection results in increased resistance with increase in resistive index Correlation highly variable Threshold? 0.7 or 0.9 Figure 3-17-9

[Figure 3-17-9]

• • •

Acute rejection •

BIOPSY - only reliable method to determine cause of renal dysfunction

Chronic Rejection • • • • •

Small allograft Echogenic from fibrosis Fatty replacement Calcification Decreased blood flow

Genitourinary Radiology

High resistance arterial waveforms with reversal of diastolic flow in main renal artery and absence of diastolic flow in interlobar arteries

667

Imaging of Solid Organ Transplants

Vascular Complications •

•

Figure 3-17-10

Early (Surgical emergencies) ➢ Renal vein thrombosis ➢ Renal artery occlusion Later ➢ Renal artery stenosis (10%) ➢ Post biopsy complications (AVF, PSA) ➢ Renal vein stenosis

Renal Vein Thrombosis [Figure 3-17-10] • •

Gray-scale: swollen hypoechoic Doppler: ➢ Absent venous flow ➢ Reversed plateauing of diastole ➢ High resistance

Renal Artery Occlusion • •

•

Gray-scale: swollen kidney Doppler ➢ Absent intrarenal arterial ➢ High resistance, high PSV, no diastolic flow ➢ Spiked preocclusive wave form NB: Severe acute rejection can cause diminished flow

a. Power Doppler with minimal flow.b. Reversed arterial flow in diastole. c. Absent venous flow. Beware of noise

Figure 3-17-11

Renal Artery Stenosis •

• • •

Hypertension, graft dysfunction and bruit Conventional angiography ➢ Reference standard (invasive contrast) ➢ Allows angioplasty Sonography for screening MR Angiography

MRA [Figure 3-17-11]

Sonographic Criteria • • • •

a. MRA of normal transplant renal artery. b. MRA showing diffuse atherosclerosis with a mild stenosis (arrow) of the proximal renal artery. c. MRA of a high grade iliac stenosis (arrow) above a normal transplant renal artery

PSV >2 m/s Velocity gradient >2:1 Post stenotic spectral broadening Pulsus tardus-parvus( prolonged early acceleration, diminished amplitude SAT >0.07s, AI <3 m/s2 , RI <0.56

Main Renal Artery Origin Tardus Parvus

Intrarenal Arteriovenous Fistulae/Pseudoaneurysms • • • •

Secondary to percutaneous biopsy Most clinically insignificant and resolve Treated conservatively if small and asymptomatic Embolized if large or causing ischemia and severe hematuria

Imaging of Solid Organ Transplants

668

Genitourinary Radiology

Intrarenal Arteriovenous Fistulae •

• •

Gray scale ➢ Usually invisible Color Doppler ➢ Flurry/perivascular bleeding ➢ Feeding artery – draining vein if large ➢ Aliasing Duplex ➢ High velocity/low resistance ➢ Arterialized venous flow

Figure 3-17-12

Arteriovenous Fistula [Figure 3-17-12]

Arteriovenous Fistula Embolization [Figure 3-17-13] Pseudoaneurysms [Figure 3-17-14]

•

• •

Gray scale ➢ Simple or complex cyst Doppler ➢ Yin yang swirling disorganized flow ➢ To and fro (neck) May rupture

Renal Vein Stenosis • • •

Perivascular fibrosis Compression by fluid collections Doppler ➢ Aliasing ➢ Velocity increase (x3–4)

a. Color; b. Power Doppler of perivascular thrill. c. duplex of artery and d. of draining arterialized vein

Figure 3-17-13

Pancreas Transplants • • •

SPK: Simultaneous pancreas-kidney PAK: Pancreas after kidney PTA: Pancreas transplant alone

Pancreatic Transplantation: Surgical Technique

•

Endocrine Drainage (Venous) ➢ Systemic (iliac vein) ➢ Portal vein Exocrine Drainage ➢ Bladder ➢ Enteric Arterial supply from common iliac artery

• •

Systemic bladder drainage Portal enteric drainage

• •

Rejection Acute and Chronic Surgical complications ➢ Infection ➢ Anastomotic Leak Vascular thrombosis Arterial / Venous Pancreatitis

•

•

Selective renal arteriography. a. abnormal distal arterial branch with early venous filling (arrow). b. Prompt filling of renal and iliac veins. c. Post embolization, the av fistula is no longer visualized

Figure 3-17-14

SPK

Pancreatic Transplant Complications

• •

Genitourinary Radiology

a. Gray scale; b. color and c. duplex Doppler of pseudoaneurysm. Note to and fro flow in neck of pseudoaneurysm (arrow)

669

Imaging of Solid Organ Transplants

Normal Pancreas Transplant [Figure 3-17-15]

Figure 3-17-15

Pancreas Transplant CT [Figure 3-17-16]

Pancreatic Transplant MR

Pancreas Transplant MRA

Peripancreatic Collections • • • •

2-10% Hematoma, seroma, anastomotic leak, abscess Nonspecific appearance Aspiration needed for diagnosis

Anastomotic Leak with Abscess [Figure 3-17-17]

Pancreatic Transplant Rejection • • • •

40% of graft loss Gray scale, color and duplex of Enlargement and heterogeneity of gland normal pancreas transplant. US Doppler RI – no correlation Arrow on pancreatic duct Diagnosed by percutaneous US guided biopsy

Pancreatic Transplant Vascular Thrombosis • • • • • •

6–10% of graft loss Venous more common than arterial US and MRA most useful Swollen heterogenous gland No flow or enhancement Thrombosed vessels

Figure 3-17-16

Pancreatic Thrombosis [Figure 3-17-18]

Liver Transplantation • • •

Established or fulminant liver failure (hepatitis C, PBC, PSC, alcolhol, cryptogenic cirrhosis, etc.) Cadaveric Living or cadaveric split liver (right lobe)

Liver Transplantation •

Reformatted coronal CT showing pancreas transplant * and vessels (arrows)

Gray scale evaluation includes ➢ Fluid collections ➢ Free fluid (ascites or bile) ➢ Biliary dilatation – choledochojejunostomy or choledocholedochostomy ➢ Parenchyma

Figure 3-17-17

Liver transplantation •

Doppler evaluation includes ➢ MPV, LPV, RPV ➢ CHA, LHA, RHA ➢ HV x 3 ➢ IVC above and below anastomosis Ultrasound (a and b) and CT(c) of a peripancreatic fluid collection containing gas (arrow). P pancreas

Imaging of Solid Organ Transplants

670

Genitourinary Radiology

Complications • • • • • •

Figure 3-17-18

Rejection Vascular thrombosis or stenosis Biliary obstruction or leak Recurrent hepatitis Fatty infiltration Neoplasm

Hepatic Artery Thrombosis [Figure 3-17-19]

MRA • •

Normal Hepatic artery thrombosis

Hepatic Artery Thrombosis [Figure 3-17-20]

Figure 3-17-19

a. Absence of color flow on color Doppler. b. Swollen pancreas transplant. c. Non enhancement of pancreas.following contrast d. Stump of graft artery

Figure 3-17-20

High resistance arterial flow secondary to thrombosis just distal to site of sample. Proximal hepatic artery (arrow) with high resistance pattern of flow

Digital subtraction arteriogram demonstrating hepatic thrombosis. The splenic artery (arrow) is patent.

Percutaneous cholangiogram shows diffuse abnormality of the bile ducts with strictures and filling defects (arrow) resulting from ischemic bile ducts Genitourinary Radiology

671

Imaging of Solid Organ Transplants

Bilomas after Arterial Thrombosis [Figure 3-17-21] Portal Vein Thrombosis [Figure 3-17-22]

Figure 3-17-21

Cavernous Transformation Biliary Tree Collections

Post Transplant Lymphoproliferative Disorder • • •

• •

Related to Epstein Barr virus Any time (mean = 15 months) Spectrum ➢ Polyclonal diffuse B cell proliferation ➢ Malignant monoclonal lymphoma Treatment ➢ Decreased immunosuppression ➢ Antiviral agents ➢ Chemotherapy Radiology ➢ Lymphadenopathy ➢ Solid/hollow visceral involvement ❖ Liver ❖ Lungs ❖ Spleen ❖ Bowel

Post Transplant Lymphoproliferative Disorder [Figure 3-17-23]

PTLD SBO [Figure 3-17-24]

Despite revascularization after hepatic artery thrombosis, multiple bilomas (*) have developed in the liver on CT and MR. Hepatic artery

Figure 3-17-22

Portal vein thrombosis after liver transplant a and b. Coronal reformatted CT showing thrombus (arrow) within the superior mesenteric and portal veins. c. Color Doppler ultrasound showing echoes in the portal vein with lack of color flow. The adjacent hepatic artery is patent

Figure 3-17-24

Figure 3-17-23

Multiple hepatic lesions on CT (a) and enhanced MR (b). Mass in the transplant kidney (c) which was biopsied (d) under ultrasound guidance Soft tissue mass (arrows) causing small bowel obstruction (arrowheads) Imaging of Solid Organ Transplants

672

Genitourinary Radiology

PTLD Post Liver Transplant

Post Transplant Malignancy • • • • • •

Kaposi’s sarcoma Lymphoma Vulva/perineum Lip Skin (squamous) Cervix

Lymphadenopathy

x 400–500 x 20–350 x 100 x 29 x 7–40 x 4–14

Summary • • • • •

Ultrasound with color and duplex Doppler is an ideal first line modality for renal, pancreas and liver transplants Sensitive for vascular complications, fluid collections and hydronephrosis Biopsy needed for diagnosis of rejection CT for infection, fluid collections, procedures, malignancy MR for evaluation of vascular and parenchymal abnormalities

References 1. 2. 3.

4.

5. 6.

7.

8. 9.

10. 11.

12. 13.

14.

Baxter GM. Ultrasound of renal transplantation. Clin Radiol 2001; 56:802-818. Boeve WJ, Kok T, Tegzess AM, et al. Comparison of contrast enhanced MR-angiography-MRI and digital subtraction angiography in the evaluation of pancreas and/or kidney transplantation patients: initial experience. Magn Reson Imaging 2001; 19:595-607. Crossin JD, Muradali D, Wilson SR. US of liver transplants: normal and abnormal. Radiographics 2003; 23:10931114. Dachman AH, Newmark GM, Thistlethwaite JR, Jr., Oto A, Bruce DS, Newell KA. Imaging of pancreatic transplantation using portal venous and enteric exocrine drainage. AJR Am J Roentgenol 1998; 171:157-163. Hohenwalter MD, Skowlund CJ, Erickson SJ, et al. Renal transplant evaluation with MR angiography and MR imaging. Radiographics 2001; 21:1505-1517. Kaushik S, Fulcher AS, Frable WJ, May DA. Posttransplantation lymphoproliferative disorder: osseous and hepatic involvement. AJR Am J Roentgenol 2001; 177:1057-1059. Krebs TL, Daly B, Wong JJ, Chow CC, Bartlett ST. Vascular complications of pancreatic transplantation: MR evaluation. Radiology 1995; 196:793-798. Linkowski GD, Warvariv V, Filly RA, Vincenti F. Sonography in the diagnosis of acute renal allograft rejection and cyclosporine nephrotoxicity. AJR Am J Roentgenol 1987; 148:291-295. Middleton WD, Erickson S, Melson GL. Perivascular color artifact: pathologic significance and appearance on color Doppler US images. Radiology 1989; 171:647-652. Middleton WD, Kellman GM, Melson GL, Madrazo BL. Postbiopsy renal transplant arteriovenous fistulas: color Doppler US characteristics. Radiology 1989; 171:253-257. Sebastia C, Quiroga S, Boye R, Cantarell C, Fernandez-Planas M, Alvarez A. Helical CT in renal transplantation: normal findings and early and late complications. Radiographics 2001; 21:1103-1117. Tobben PJ, Zajko AB, Sumkin JH, et al. Pseudoaneurysms complicating organ transplantation: roles of CT, duplex sonography, and angiography. Radiology 1988; 169:65-70. Vrachliotis TG, Vaswani KK, Davies EA, Elkahammas EA, Bennett WF, Bova JG. CT findings in posttransplantation lymphoproliferative disorder of renal transplants. AJR Am J Roentgenol 2000; 175:183-188. Wong JJ, Krebs TL, Klassen DK, et al. Sonographic evaluation of acute pancreatic transplant rejection: morphologyDoppler analysis versus guided percutaneous biopsy. AJR Am J Roentgenol 1996; 166:803-807.

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The Neglected Nephrogram David S. Hartman, MD Goals • •

Review normal nephrographic physiology Present 6 abnormal patterns with the respective differential diagnosis

Urogram Does Not Equal Nephrogram Nephrogram • • • • • •

Figure 3-18-1

Excretory Urography CT MR Nuclear Medicine Angiography ? Ultrasound

Normal - Nephrographic - Physiology Renal Vascularity • • • •

Normal CT enhancement. Left, enhanced CT in the vascular Most flow is to the cortex (corticomedullary phase); middle, nephrographic phase; right, pyelographic Vasa recta to medulla phase Capsular vessels may supply peripheral nephrons #2,#3 important for rim NG and reverse NG

Normal Nephrographic Pysiology • • •

The main driving force for urine production is filtration pressure (blood pressure) Contrast is filtered. It is not excreted or reabsorbed by the tubules Contrast which gets into the nephron will eventually get to the collecting system

Density of the Nephrogram (3 Factors) • • •

Iodine concentration GFR Transit time

• • • • •

Kidney Blood in Blood out Urine out Nephrons

Figure 3-18-2

Normal Nephrogram requires

Normal Pyelogram [Figures 3-18-1 and 3-18-2]

• • •

Symmetric 3 minute film Delayed side is the diseased side

Normal MR enhancement. Enhanced T1-weighted with fat suppression dynamic scan of the left kidney The Neglected Nephrogram

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6 Nephrographic Patterns • • • • • •

Absent NG Unilateral delayed pyelogram Bilateral persistent NG Rim NG Reverse rim NG Striated NG

•

Diagnosis:Renal agenesis with seminal vesicle cyst

Figure 3-18-3

Pattern #1 Absent [Figure 3-18-3] Pertinent Embryology • •

Ureteral bud comes off of the mesonephric duct Close association of mesonephric duct and ureter

Renal agenesis with ipsilateral seminal vesicle cyst. Left, enhanced CT: there is no left kidney in the left renal fossa which is filled by large bowel. Right, CT at the level of the bladder shows extrinsic impression of the left posterior bladder by a cystic mass

Following nephrectomy the small bowel fills the renal fossa [Figure 3-18-4]

17 Yo, Trauma: Global Infarction [Figure 3-18-5] Nonfunction of the nonobstructed kidney equals vascular occlusion

Figure 3-18-4

No Blood In

20 YO Woman, Rt flank pain, hematuria, facial rash [Figure 3-18-6] No Blood Out

Bowel in the left renal fossa. CT scans of 2 different patients. Left: congenital absence or ectopia of the left kidney with large intestine filling the renal fossa. Right: acquired absence of the kidney (nephrectomy) with small bowel filling the left renal fossa

Figure 3-18-5

Figure 3-18-6

Global infarction. Enhanced CT. There is an absent left nephrogram due to disruption of the left renal artery Right renal vein thrombosis. Left: enhanced CT shows absent nephrogram in the right kidney which is enlarged. Right: IV cavogram shows thrombus in the IVC which had extended into the right renal vein Genitourinary Radiology

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The Neglected Nephrogram

74 Year Old Man, BPH [Figure 3-18-7] • •

There was no dilated right ureter on lower images Right UPJ with post obstructive atrophy

No Urine Out

Figure 3-18-7

22 YO man, Hematuria [Figure 3-18-8] •

Multicystic dysplastic kidney

32 year old woman, RUTI’S [Figure 3-18-9]

Absent Left Nephrogram [Figure 3-18-10]

•

Renal TB

Long standing right UPJ obstruction with post obstructive atrophy. Right, IVP with non visualization of the right kidney. Left, enhanced CT shows a left dilated pelvis and calyces

Figure 3-18-10

Figure 3-18-8

Multicystic dysplastic kidney. Left, KUB with multiple peripheral calcifications in the left renal fossa. Right, IVP shows absent function on the left

Autonephrectmy from renal tuberculosis. Top, KUB shows multiple illdefined calcifications in the left kidney. Bottom, IVP shows no function

No Nephrons • •

Congenital (MCK) Acquired (XGP, TB)

The Neglected Nephrogram

Figure 3-18-9

Xanthogranulomatous pyelonephritis. Upper left, KUB shows a left staghorn caclculus. Upper right, IVP shows left nonfunction. Bottom, CT demonstrates replacement of the left kidney by and inflammatory mass which extends into the perirenal space

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Pseudo-Absent Nephrogram [Figure 3-18-11] •

Figure 3-18-12

Pelvic kidney

Figure 3-18-11

Pelvic kidney. Left, CT through the kidneys shows an absent right kidney with colon in the right renal fossa. Right, lower section shows a nonrotated right pelvic kidney (arrows)

Pattern #1 Absent Nephrogram • • • • •

No kidney No blood in No blood out No urine out No nephrons

•

TCC left UVJ (slow urine out)

Pattern #2 Unilateral Delayed Pyelogram [Figure 3-18-12] Slow Urine Out

10 year old boy hypertension [Figure 3-18-13] Renal Artery Stenosis (Slow Blood In) • • •

Delayed pyelogram Small kidney Hyperdense pyelogram

Slow Blood In

Diagnosis: Compression of Renal Vein by Pancreatic Carcinoma (Slow Blood Out) [Figure 3-18-14] Figure 3-18-13

Figure 3-18-14

Renal vascular hypertension (Takaysu’s). Left, axial CT: the left kidney is small and the pyelogram is delayed. There is soft tissue around the aorta. Right, sagital MR: there is narrowing of the aorta (arrows) Genitourinary Radiology

TCC left UVJ. Upper left, IVP: there is delay of the left pyelogram. Upper right, left kidney: There is hydronephrosis. Lower right, bladder near the UVJ: there is a soft tissue mass (arrow)

Upper, axial CT: There is a mass (M) ventral to the left kidney. The left kidney is in the corticomedullary phase while the right kidney is in the nephrographic phase. Lower, axial CT at the level of the left renal vein. The left renal vein arrow) is compressed by the mass 677

The Neglected Nephrogram

Slow Blood Out

Figure 3-18-15

75 Yo Woman Left Flank Pain [Figure 3-18-15]

• •

Acute pyelonephritis Poor nephron function

Poor Nephron Function

Pattern #2 Delayed Pyelogram (Unilateral) • • • •

Slow urine out (OBST uropathy) Slow blood in (RA stenosis) Slow blood out (RV compress) Poor nephron function (pyelonephritis)

• • •

Slow urine out Slow blood in Slow blood out

•

Obstruction of renal pelvis by blood clot

•

Rarely produces a unilateral hyperdense nephrogram (unless there is tubular or pelvic obstruction with pus)

Acute pyelonephritis. Axial CT: the left kidney has a diminished nephrogram and delayed pyelogram

In Cases with A Delayed Pyelogram There May Be An Ipsilateral Hyperdense Nephrogram

Figure 3-18-16

22 YO man, flank pain, hematuria [Figure 3-18-16] Pyelonephritis

Pattern #3 Persistent Bilateral NG [Figure 3-18-17] •

Diagnosis:Hypotension

Figure 3-18-17

Hyperdense nephrogram from pelvic obstruction by clot. Upper, noncon CT: there is hyperdense blood in the right renal pelvis. Lower, enhanced CT: there is a delayed right pyelogram and a hyperdense right nephrogram

Hypotension. Left, 10 min IVP: there are bilateral persistent nephrograms with delayed pyelograms. Right, 20 min IVP with correction of the hypotension: normal examination

Contrast 3 days ago [Figure 3-18-18] •

Acute tubular necrosis (ATN)

• • •

Tubular damage and obstruction Decrease blood flow “Acute vasomotor nephropathy”

• • •

ATN Bilateral ureteral obsruction Bilateral renal artery stenosis

Figure 3-18-18

ATN

Renogram

The Neglected Nephrogram

Acute tubular necrosis. Axial CT (contrast 3 days ago): There are persistent bilateral nephrograms with delayed pyelograms 678

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Pattern #3 Persistent Bilateral Nephrogram • •

•

Hypotension Intra renal obstruction ➢ ATN ➢ Urate ➢ Protein ➢ Myoglobin Less likely ➢ Bilateral ureteral obstruction ➢ Bilateral renal Artery stenosis ➢ Bilateral renal vein thrombosis

Pattern #4 Rim Nephrogram [Figure 3-18-19 •

21 yo man trauma 10 days ago

Pattern #5 Striated Nephrogram [Figure 3-18-20] 5 Patterns • • • • •

Absent NG Unilateral delayed pyelogram Bilateral persistent NG Rim NG Striated NG

• •

Decreased blood flow to the cortex Continued perfusion to the subcapsular and juxtamedullary cortex Leads to renal failure Late cortical nephrocalcinosis

Global infarction with rim nephrogram. Enhanced CT: The right kidney is normal. The left kidney shows enhancement near the coriticomedullary junction and in the subcapsular area resulting in a “rim” of enhancement. This is the same case as figure 5, 10 days later

Figure 3-18-20

Acute Cortical Necrosis

• •

Figure 3-18-19

#6 Striated Nephrogram [Figure 3-18-21]

• • • • • • • •

ARPCK Acute Pyelo Obstruction RVT Contusion Hypotension Tubular Obst Normal

Figure 3-18-21

Acute cortical necrosis. Enhanced CT scans: There is no cortical enhancement with selective enhancement of the medulla Case courtesy Dr. Parvi Ramchandai University of Pennsylvania

Acute pyelonephritis. The striated nephrogram shows alternating bands of density and lucency Genitourinary Radiology

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The Neglected Nephrogram

6 Patterns • • • • • •

Figure 3-18-22

Absent NG Unilateral delayed pyelogram Bilateral persistent NG Rim NG Reverse rim NG Striated NG

Each Pattern May Be Segmental or Subsegmental [Figure 3-18-22]

• • • •

Tubular obstruction Shock Pyelo Infarct

Segmental abnormal nephrograms, 4 different cases. Upper left: tubular obstruction, upper right: hypotension, lower left: pyelonephritis, lower right: lobar infarction References

1. Davidson AJ, Hartman DS, Choyke PL, Wagner BJ. Davidson’s Radiology of the Kidney & Genitourinary Tract 3rd Edition, W.B. Saunders Philadelphia 1999.

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Problem Renal Masses David S. Hartman, MD Learning Objective •

To use radiological imaging for the characterization and management of the problematic renal mass

Centennial Sounding Board

Personal Refelection on Growth of Diagnostic Imaging

• •

“As we accurately image and inspect the human body with thinner and more detailed sections, we approach the 1–2 mm serial sections of the pathologist, who can find evidence of “disease” in almost every organ and everyone.” “The radiologist of the future will need to understand the implications of their findings and know the natural history of each disease detected.”

Robert J. Stanley, AJR 2000;174:609

Problem Renal Masses • • •

Principles of Neoplasia Small Renal Mass Cystic Renal Mass

• • • •

Carcinoma In Situ (CIS) Angiogenesis Vascular invasion Metastasis

• •

Proliferation Programmed death (Apoptosis)

•

Controlled by genes

•

Results from disequilibrium of proliferation and cell death

• • •

Multi-step process Numerous genetic events Can stop at any point

• • • •

Confined by basement membrane Stops expanding after reaching diffusion limit of the nearest vessel “No” metastatic potential Very, very common

•

•

Most human tumors exist as in situ lesions ➢ 0.2– 2 mm Renal CIS is found in 22% of autopsies

• • •

Ability to recruit host blood supply Penetrate basement membrane May enlarge to become macroscopic

Principles of Neoplasia (4 Arbitrary Steps)

Cell Cycle

Normally Cell Proliferation and Apoptosis are Activated in Parallel Neoplasia

Chromosomal Instability Pathway Carcinoma In Situ

Carcinoma In Situ

Angiogenic Phenotype

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Virtually All Solid Tumors Which are Visible Are Angiogenesis Dependent Vascular Invasion • • •

Tumor shedding and vascular invasion may occur relatively early In animal models, tumors shed 3-6 million cells per Gram per 24 hours Most cells which are shed do not progress to viable metastases

• • •

Very imprecise at knowing which, where and when RCC will metastasize Mets require activation of genes Each metastasis must become angiogenic to grow

Metastasis

Nonangiogenic Metastases May Remain Microscopic and Dormant for Many Years Problem Renal Masses • • •

Principles of neoplasia Small renal mass Cystic renal mass

Figure 3-19-1

Rationale For Management Decisions

Best Way to Treat RCC Excision or Ablation •

Chemotherapy, radiation and immunotherapy are less effective

Critical Feature of RCC

Noncon Enhanced Cyst, AML and solid nonfatty mass in the right kidney. Left, nonenhanced and right, enhanced Renal Tumors <3 cm Uncommonly Have axial CT through the kidney. A is a 1.8 cm cyst. B Detectable Metastases is a 2 cm AML. C is a 1.7 cm nonfatty mass which • The renal tumor doesn’t “know” how large it is most likely represents a renal epithelial tumor with • The larger the renal tumor, the more undifferentiated a low probability of metastasis it may be •

Metastases

•

The more undifferentiated, the greater the liklihood that a metastasis can become angiogenic

Pathology / Radiology

The Small (< 3 cm) Renal Mass • •

1.5 - 3.0 cm A . Cyst B . Cystic C . Solid < 1.5 cm (often TSTC)

The Small Solid Renal Mass [Figure 3-19-1] • •

Fat = AML No Fat A . Renal Epithelial Tumor with low metastatic potential B. Cannot diagnose Adenoma (Oncocytoma)

Management Options • • • • • •

Excise Ablation Follow Biopsy Nephrectomy Ignore

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The Small (< 3 cm) Renal Mass

•

1.5 - 3.0 cm A . cyst B . cystic C . solid < 1.5 cm (often TSTC)

• •

No single algorithm for every case Risks and benefits of any strategy

• • •

Pretest probability Patient’s ability to tolerate uncertainty Your ability to tolerate uncertainty

•

There is no large, prospective, pathologically proven series which indicates correct management

•

Figure 3-19-2

Too Small To Characterize Always Consider

How Should The TSTC Mass Be Managed?

Enhanced CT scans of 2 different patients both of which have a lesion which is too small to characterize. Left, renal cell carcinoma; right, renal cyst

RCC / Cyst [Figure 3-19-2]

Too Small to Characterize (< 1.5 cm) • • •

1. Ignore 2. Follow 3. Get another study

• •

The smaller the lesion, the longer the followup interval If following a lesion, compare oldest comparable study available

How Often Should Small Lesions Be Followed? How Is Doubling Time Calculated?

Figure 3-19-3

http://www.chestx-ray.com/index.html

Small Lesion Considered Aggressive • •

Size >3cm Doubling time faster than 6 months

• • •

1. Ignore 2. Follow 3. Get another study

•

Value of getting a T2 fat sat MR when the CT is equivocal

Too Small to Characterize (< 1.5 cm) T2 Fat Sat: Simple Cyst [Figure 3-19-3]

Get the Referring Doctor Involved

Simple cyst confirmed on MR. Upper CT scans (unenhanced, corticomedullary and pyelographic phases): the mass (arrow) is too small to characterize. Below, T2 fat sat MR: the lesion is homogeneously bright

Get the Patient Involved Problem Renal Masses • • •

Principles of neoplasia Small renal mass Cystic renal mass

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Problem Renal Masses

Teaching Goal

• To allow you to suggest the appropriate management for cystic renal masses: ➢ Ignore ➢ Excise ➢ Follow

Rationale For Management

•

10% of cases of renal carcinoma present as a fluid-filled mass The simple cyst can be confidently diagnosed by ultrasound CT or MR Rarely simple cysts become complicated ➢ Hemorrhage ➢ Infection ➢ Ischemia On gross examination, the complicated cyst may be indistinguishable from cystic renal cell carcinoma Differentiation is based upon histological diagnosis

•

Surgery or Ablation

• • •

•

The most effective treatment for renal cell carcinoma A cyst not simple if it has any of the following

Renal Carcinoma Can Grow As A Fluid Filled Mass • • • • • • •

Calcification Hyperdense/high signal Septations Multiple locules Enhancement Nodularity Thick wall

Although microscopic evaluation is required for precise diagnosis, there are certain radiological findings which are reliable in differentiating complicated cyst from cystic renal cell carcinoma Caveats • •

The portion of the cystic mass which is most worrisome should be used in deciding appropriate management. In cases with discordant imaging findings utilizing different radiological examinations, the lesion should be managed based upon the most aggressive imaging findings

What About Biopsy? •

If there is a strong clinical suspicion that the mass is inflammatory, careful puncture is acceptable. If there is evidence of infection, treat (antibiotics, drainage, etc.) and follow Renal neoplasia very rarely presents with infection

• • •

Ignore Excise Follow

•

Ignore ➢ “Small” amount ➢ Smooth ➢ Septal ➢ Milk of calcium ➢ No enhancement

•

Guidelines

Calcification

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• •

Figure 3-19-4

Excise ➢ Enhancement ➢ Wall thickening ➢ Nodularity Follow ➢ Thick ➢ Nodularity ➢ No enhancement

Hyperdense/High Signal [Figure 3-19-4] •

CT: > 20 HU on unenhanced CT

•

MR: higher signal intensity than water on T1-weighted images

• • •

Blood Protein Colloid

• • • •

Lymphoma RCC (papillary) AML (small amt of fat) Mets

Hyperdense/High Signal [Figure 3-19-5]

Most Hyperdense Masses Are Cystic

Hyperdense Masses May Be Solid [Figure 3-19-6]

Hyperdense renal masses. Unenhanced CT: there are two masses (arrows) which are denser than water. Without contrast, it is impossible to state if they are cystic or solid

Figure 3-19-6

Figure 3-19-5

Renal lymphoma (solid). Unenhanced CT scan shows several hyperdense masses some of which are confluent (arrows)

High signal renal mass. T1-weighted axial MR of the left kidney: the signal of the mass is higher that that of water

Hyperdense/High Signal •

Ignore ➢ Sharp margin ➢ < 3 cm ➢ Not completely intrarenal ➢ Homogeneous or hematocrit ➢ No enhancement ➢ US: cyst or cystic

Significant Enhancement •

CT ➢ <10 H.U.=Beam hardening ➢ 10-15 H.U.=Indeterminate ➢ >15 H.U.=Vascularity

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•

MR ➢ <15% relative enhancement = Benign ➢ >15% relative enhancement = ➢ Surgical

Hyperdense/High Signal •

•

Excise ➢ Poorly defined ➢ Heterogeneous ➢ Enhancement ➢ US: solid Follow ➢ Totally intrarenal ➢ > 3 cm

Septation •

• •

Ignore ➢ Thin (< 1 mm) ➢ Smooth ➢ May calcify ➢ No enhancement Excise ➢ Thick, irregular ➢ Nodular ➢ Enhancement Follow ➢ Only slightly “greater than hairline”

A cyst not simple if it has any of the following • • • • • • •

Calcification Hyperdense/high signal Septations Multiple locules Enhancement Nodularity Thick wall

With More Than 3 or 4 Septa, Multiloculated [Figure 3-19-7] Multiloculated Masses •

Excise ➢ All multiloculated masses

Figure 3-19-7

Three multiloculated masses. Left: ultrasound; middle: enhanced CT; right: T2-weighted axial MR

ML-RCC

Multilocular Cystic Nephroma (MLCN) MLCN • • • •

Female No Blood Pelvic Herniation Usually Benign

ML-RCC

Male Blood Venous Invasion Always Malignant

Wall Thickening, Enhancement, Nodularity: Cannot Tell Benign From Surgery Enhancement •

Excise ➢ All enhancing masses

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Genitourinary Radiology

Nodularity •

•

Figure 3-19-8

Excise ➢ All masses with enhancing nodularity Follow ➢ Very small nonenhancing nodules

Wall Thickening •

Excise ➢ All noninfected masses with wall thickening

“The Current Radiological Approach To Renal Cysts” Morton A. Bosniak, M.D., Radiology January 1986

Bosniak Classification - Ignore, Excise, Follow I Simple cyst II Min complic IIF Probably benign III Benign or malignant IV Clearly malignant

Ignore Ignore Follow Excise Excise

•

Communication is crucial!

•

Calcification that can be ignored [Figure 3-19-8] ➢ Small amt of peripheral Ca++ ➢ Milk of calcium ➢ No enhancement Surgical Calcification [Figure 3-19-9] ➢ Septal ➢ Thick, irregular, nodular ➢ Enhancement Uncomplicated Cyst [Figure 3-19-10]

Calcified cystic renal mass that can be ignored. Top, unenhanced CT: there is a small amount of calcification at the 11 0’clock position as well as milk of calcium at the 6 o’clock position. Bottom, enhanced CT: there is no enhancement of the mass

Examples

• •

Figure 3-19-9

Figure 3-19-10

NON CON

NEPHROGRAPHIC

EXCRETORY

Calcific cystic mass which should be followed. Left, unenhanced CT, middle early phase of enhancement, right, excretory phase of enhancement. The mass is sharply marginated and does not enhanced

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Cystic renal mass which should be excised. Top, unenhanced CT: There is a thin septation which is calcified (horizontal arrow). There is an irregular nodular calcification at the 1 o’clock position. Bottom, enhanced CT shows an area of enhancement (vertical arrow) adjacent to the calcific nodule

Problem Renal Masses

• •

• • •

Figure 3-19-11

Follow-up Calcification [Figure 3-19-11] ➢ Nodular ➢ No enhancement Hyperdense that can be ignored [Figure 3-19-12]

➢ Well defined, homogeneous ➢ < 3 cm ➢ No enhancement Homogeneous with 7 OR 13.5 HU of enhancement [Figure 3-19-13] Surgical Hyperdense [Figure 3-19-14] ➢ Papillary RCC ➢ Solid on US Follow-up Hyperdense [Figure 3-19-15] ➢ Homogenous ➢ No enhancement ➢ Cystic ➢ > 3 cm

NON CON

NEPHROGRAPHIC

EXCRETORY

Higher CT sections show several irregular nodules of calcification. There is no enhancement

Figure 3-19-12

Figure 3-19-13

Hyperdense cyst that can be ignored. Left, unenhanced CT: The mass is hyperdense (34 HU), homogeneous, well-defined and less than 3 cm. Right, enhanced CT: the mass does not enhance (31 HU) and remains homogeneous

Figure 3-19-14

Because the enhancement was equivocal, ultrasound was performed showing a solid mass which was a papillary renal cell carcinoma

Figure 3-19-15

Minimally enhancing hyperdense papillary renal cell carcinoma. Top, unenhanced CT scan: the mass measures 29 HU. Center, enhanced CT scan, nephrographic phase: the mass enhanced 13.5 HU to 42.5 Bottom, enhanced CT, pyelogram: the mass deenhances to 36 HU Problem Renal Masses

Hyperdense cyst which should be followed. Left, unenhanced CT: the 6 cm mass is homogeneous and measures 68 HU. Center, enhanced CT, the mass remains homogeneous and does not enhance. Right, ultrasound: the mass is cystic with a thin septation. Because the mass is greater than 3cm it should be followed 688

Genitourinary Radiology

• •

• • • •

Septations that can be ignored [Figure 3-19-16] ➢ Thin <2mm, no nodularity ➢ No enhancement Figure 3-19-16 ➢ May calcify Surgical Septations [Figure 3-19-17] ➢ Thick > 2mm ➢ Associated nodularity ➢ Associated nodularity ➢ Complicated cyst ➢ Cystic RCC MLCN / MLRCC [Figure 3-19-18] Surgical [Figure 3-19-19] ➢ Enhancing thick wall Septations which can be ignored. Left, ultrasound: the septum Surgical nodularity [Figure 3-19-20] is thin without nodularity. Right, CT: the septum is thin and ➢ Enhancing nodule smooth without apparent enhancement Case 10 [Figure 3-19-21] ➢ Very small nonenhancing nodule

But follow very, very carefully •

Baseline / 1 year later

[Figure 3-19-22]

Figure 3-19-17

Figure 3-19-18

Septations which should be excised. Left, ultrasound: the septation is thick and has associated nodularity (horizontal arrow). Right, CT: the septation is irregular and enhancing Left: complicated cyst. Right: cystic renal cell carcinoma

Figure 3-19-19

2 different multiloculated masses. Top: multilocular cystic nephroma. Bottom: multiloculated renal cell carcinoma Cystic renal cell carcinoma with an enhancing thick wall. Left, unenhanced CT: the thick wall is difficult to appreciate. Right, enhanced CT: the thick enhancing wall is easily visualized

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Problem Renal Masses

Figure 3-19-20

Figure 3-19-21

2 different cases of cystic renal cell carcinoma. Enhancing nodule (arrow) in the wall of each is clearly visualized

Figure 3-19-22

Cystic renal cell carcinoma with a small nodule which was followed. Left, baseline T2 fat sat MR shows a small nodule in the 8 o'clock position (arrow). Right, follow-up T2 fat sat MR one year later shows increased nodularity and thickening

Very small nonenhancing nodule which could be followed. Top, ultrasound: there is a very small nodule (vertical arrow). Bottom, enhanced CT: there is a very small nonenhancing nodule (horizontal arrow)

References 1.

Hartman DS, Choyke PL, Hartman MS.A practical approach to cystic renal masses.RadioGraphics 2004;24: S101S115.

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Genitourinary Seminar 1: MSAFP Paula J. Woodward, MD

All of the following scans were ordered following a routine blood test. • •

What was the test? Was it high or low?

Elevated Maternal Serum Alpha-Fetoprotein (MSAFP) Fetal Alpha-fetoprotein • • • •

Glycoprotein produced by fetal liver, GI tract, and yolk sac Excreted through the urinary tract into the amniotic fluid Peaks at 14–16 wks Small amounts leak into maternal circulation

• • •

Screening in second trimester (16–18 weeks) Elevated if 2.5 MOM (multiples-of-the-median) 10–15% risk of open neural tube defect

Maternal Serum Alpha-fetoprotein (MSAFP) Case 1

Case 2

Elevated MSAFP • • • • • •

Incorrect dates Twins Fetal death Open neural tube defect Abdominal wall defect Subchorionic hemorrhage

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Seminar 1: MSAFP

Case 3

Case 4

Case 5

Placental hemorrhages

Elevated MSAFP • •

Can perform amniocetesis and measure direct AFP and ACE Acetylcholinesterase (ACE) – neural tissue specific

• • •

Inc AFP, inc ACE – ONTD Inc AFP, nl ACE – abdominal wall defect Nl AFP, nl ACE – prior bleed

• •

Trisomy 21,18 Combine with human chorionic gonadotropin (hCG) and estriol (uE3) for increased specificity – triple screen

Elevated MSAFP

Decreased MSAFP

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Genitourinary Seminar 2: Renal Calcifications Paula J. Woodward, MD Renal Calcifications • •

•

Dystrophic calcification Nephrocalcinosis ➢ cortical ➢ medullary Nephrolithiasis

Dystrophic Calcification • •

Calcification of abnormal tissue DDx ➢ tumor ➢ inflammatory mass (TB) ➢ hematoma ➢ cysts

66 yo with hematuria

Renal Tuberculosis • • • •

Hematogenous spread Bacilli lodge in corticomedullary jct. Progress along nephron into pelvo-calyceal system 75% of active TB only in one kidney

Symptoms • • • •

Asymptomatic Frequency Hematuria “Sterile” pyuria Papillary necrosis

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693

Granuloma formation Seminar 2: Renal Calcifications

Radiologic Findings • • •

10% normal Papillary irregularity Papillary necrosis

• • • •

Infundibular stenosis Amputated calyx Parenchymal scarring Tuberculomas

• •

Present in 30–50% Variable appearance ➢ punctate – healed granulomas ➢ amorphous – granulomatous masses ➢ extensive reniform – autonephrectomy (putty kidney) Ureter and bladder may also be involved

Radiologic Findings

Calcifications

•

45 yo woman from Mexico with pyuria

Diffuse renal calcification

Medullary Nephrocalcinosis • • • •

Metastatic calcification – calcification in normal tissue Triangular deposition conforming to pyramids Renal function usually not impaired Often associated with nephrolithiasis

•

Hypercalcemic states ➢ hyperparathyroidism, paraneoplastic, sarcoidosis, milk-alkali syndrome, hyper-vitaminosis D Medullary sponge kidney (renal tubular ectasia) ➢ may be unilateral or focal

Medullary Nephrocalcinosis •

Medullary Nephrocalcinosis •

•

Renal tubular acidosis – Type I (distal) ➢ distal tubule can not secrete hydrogen ion, urine becomes alkaline ➢ symmetric Oxalosis ➢ primary (children) -severe may also see cortical calcification ➢ secondary – distal small bowel resection

Seminar 2: Renal Calcifications

694

Genitourinary Radiology

27 yo man with a history of stone disease

5 yo boy with an inherited disorder

45 yo female with microscopic hematuria and intermittent flank pain

Cortical Nephrocalcinosis • • •

Egg-shell calcification Generally small kidneys Renal function usually impaired

• •

Chronic glomerulonephritis Acute cortical necrosis ➢ pregnancy, sepsis, trauma, nephrotoxins (ethylene glycol) Chronic transplant rejection Alport’s syndrome ➢ nephritis, nerve deafness, hematuria, ocular abnormalities

Cortical Nephrocalcinosis • •

Genitourinary Radiology

695

Seminar 2: Renal Calcifications

33 yo male in an aircraft accident with severe chest and skeletal trauma

Seminar 2: Renal Calcifications

47 yo male admitted for an abdominal abscess. What is the renal disease?

696

Genitourinary Radiology

Radiologic Pathology 2006-2007 - Volume 1 - Index A. Israelii 187 Abdominal Angina 499 Abdominal Wall Defects 609 Abscess (Acute Pancreatitis) 463 Abscess (Crohn Disease) 388 Abscess (tuboovarian) 659 Absent Nephrogram 677 Accessory Spleen 532 ACCP 136 Achalasia 164 Actinomycosis 178 Acute Cholecystitis 439, 441 Acute Epididymitis 591 Acute Interstitial Pneumonia AIP 18 Hammon-Rich 18 Acute Mediastinitis 165 Acute Mesenteric Ischemia 488 Acute Pancreatitis 460 Acute Rejection (Kidney Transplants) 667 Acute Scrotum 591 Adenitis (Mesenteric) 430 Adenocarcinoma (bile ducts) 317 Adenocarcinoma (Gastric) 332 Advanced Gastric Carcinoma 333 Carmen Meniscus Sign 334 Early Gastric Carcinoma 333 WHO Classification 333 Adenocarcinoma (lung) 115 Adenocarcinoma (small intestine) 356 Adenoid Cystic Carcinoma 196 Adenoma (male Breast) 257 Adenoma (Oligocystic) 328 Adenoma (parathyroid) 162 Adenovirus 182 Adhesion (GI) 477 Adnexae (non neoplastic disorders) 653 Adnexal Torsion 658 ADPCKD 306 ADPKD 614 Adrenal Imaging 645 Adrenal Tumors 645 Adenoma 645 Carcinoma 646 Myelolipoma 647 Pheochromocytoma 647 Washout Calculation 648 AIDS Cholangiopathy 308 AIDS-Related Lymphoma (GI) 350 Airways Disease 26 Allergic Bronchopulmonary Aspergillosis 27 Diffuse Panbronchiolitis 27 Langherhans Cell Histiocytosis 27 Lymphangioleiomyomatosis 27 Mosaic density 27 AIUM Guidelines: First Trimester 597 Allergic Bronchopulmonary Aspergillosis 35 Alpha-1 Antitrypsin Deficiency 31 Alpha-fetoprotein (Fetal) 691

Alveolar Filling Pneumonias 178 Amebic Abscess (hepatic) 285 Anembryonic Pregnancy 598 Anencephaly 605 Aneurysm (mediastinum) 164 Angiodysplasia (GI Bleeding) 474 Angiomyolipoma (Kidney) 570 Angiosarcoma (intrahepatic) 280 Angiosarcoma (Spleen) 539 Anorectal Lymphoma 350 Anthrax 189 Appendagitis 430 Appendiceal Neoplasms 428 Appendicitis 427 ARPKD 616 Arteriovenous Fistulae/Pseudoaneurysms (post kidney transplantation) 668 Asbestos 49 Asbestosis and Cigarette Smoking 52 Mesothelioma 50 Pleural Effusion 50 Pleural Plaques 50 Round Atelectasis 51 Rounded atelectasis 49 Ascariasis 188 Ascariasis lumbricoides 189 Aspergillus 77 Air Crescent 77 Halo-Sign 77 Aspiration (Nosocomial Pneumonia and) 186 Asplenia (Ivemark Syndrome) 534 Asthma 33 Atresia 609 Esophageal 609 Jejunal/Ileal 609 Atrial Septal Defect 133 Atypical Adenomatous Hyperplasia 117 B. Anthracis: Anthrax 189 BCG 71 Benign Hepatic Neoplasms 267 Bile Duct (Hepatic) Cyst 272 Biliary Cystadenoma 272 Focal Nodular Hyperplasia 268 Hemangioma 267 Hepatocellular Adenoma 270 Hepatocellular Adenomatosis 271 Lipomatous Tumors 273 Bilharziasis (see Schistosomasis) 288 Biliary Disease (benign) 303 Acute Pyogenic Cholangitis 309 AIDS Cholangiopathy 308 Caroli Disease 304 Choledochal Cyst 305 Polycystic Liver Disease 306 Primary Sclerosing Cholangitis 307 Recurrent Pyogenic Cholangitis 309 Biliary Parasites 288 Clonorchis sinensis 289 I1

Bilomas (liver transplantation) 672 Bladder Neoplasms 649 Blastomycosis 104 Pathology 105 Radiologic Manifestations 105 Bleeding - Gastrointestinal 468 Boerhaave’s Syndrome 403 Bowel Disease (Idiopathic Inflammatory) 382 Bowel Ischemia 487 Brachytherapy (Prostate Cancer) 622 Breast 229 Angiosarcoma 236 Congenital Anomalies 230 Fibroadenoma 231 Fibrosarcoma 236 Invasive Ductal Cancer 233 Invasive Ductal Carcinoma 234 Invasive Lobular Cancer 235 Lobular Neoplasia 232 Mastitis 230 Medullary Carcinoma 234 Normal Anatomy 229 Paget’s Disease 234 Papilloma 232 Phyllodes Tumor 231 Pregnancy Changes 230 Sarcoma 236 Spindle Cell Sarcoma 236 Tubular Carcinoma 235 Breast (Male) 257 Breast (Young Women) 246 Angiosarcoma 254 Benign Lesions 247 Diabetic Mastopathy 251 Fibroadenoma 247 Granular Cell Tumor 249 Granulomatous Mastitis 252 Hamartoma 250 Invasive Ductal Carcinoma 252 Juvenile Hypertrophy 251 Juvenile Papillomatosis 250 Lactating Adenoma 250 Lymphoma 254 Medullary Carcinoma 253 Phyllodes High Grade 254 Phyllodes Tumor 249 Pseudoangiomatous Stromal Hyperplasia (PASH) 251 Sarcoma 253 Secretory Carcinoma 253 Bronchial Adenoma 192 Bronchial Carcinoid 194 Bronchiectasis 190 Bronchiolitis Obliterans 80 Bronchioloalveolar Carcinoma 117 Bronchocentric Granulomatosis 71 Bronchogenic Cyst 159 Brunner Gland 354 Hamartoma 354 Hyperplasia 354 Budd-Chiari Syndrome 298 Burkitt Lymphoma 348 CA-125 638

Calcifications (benign - breast) 225 Fibroadenoma 226 Loa Loa 227 Lobular 226 Secretory 227 Skin 226 Sutural 226 Vascular 227 Cancer (Male Breast) 261 Carcinoid Atypical 193 Typical 192 Duodenal, Jejunal, Ileal 358 Gastric 341 Thymic 154 Syndrome 358 Carcinoma - Adrenal 646 Carcinoma (Male Breast) 262 Invasive Ductal Carcinoma 262 Lymphoma (Male Breast) 263 Metastasis (Male Breast) 262 Papillary Carcinoma 262 Carcinoma (Scirrhous - Stomach) 334 Carcinoma Colon, Rectum (see Colorectal Carcinoma) 361 Carcinosarcoma 197 Cardia (Carcinoma) 334 Carney’s Triad 200 Caroli Disease 304 Castleman Disease 151, 346 Cavitary pneumonia 190 Cavitation 190 Cecal Volvulus 400 Cervix Carcinoma 559 Chest Wall 216 Neoplasms 218 Chiari II 604 Cholangiocarcinoma (intrahepatic) 279 Cholangitis 309 Acute Pyogenic 309 Recurrent Pyogenic 309 Cholecystitis 438, 441 Choledocholithiasis 442 Cholelithiasis 438 Cholescintigraphy 439 Chondroma 201 Chorionic Sac 594 Choroid Plexus Cysts 605 Chronic Idiopathic Intestinal Pseudo-obstruction 476 Chronic Liver Disease 293 Budd-Chiari Syndrome 298 Cirrhosis 293 Hemochromatosis 300 Hemosiderosis 300 Hepatocellular Carcinoma 295 Nonalcoholic Steatohepatitis 297 Primary Biliary Cirrhosis 296 Steatosis 296 Chronic Pancreatitis 460, 465 Chronic Thromboembolic Disease 133 Churg-Strauss Syndrome 67 Cirrhosis 293 CNS Malformations (Fetal) 602 I2

Coccidioidomycosis 106 Coccidioidoma 107 Pathology 106 Radiologic Manifestations 106 Colitis Ischemic 398 Colon (Inflammatory Disease) 391 Cecal Diverticulitis 397 Diverticular Hemorrhage 397 Diverticulitis 395 Diverticulitis vs. Carcinoma 396 Giant Sigmoid Diverticulum 397 Ischemic Colitis 398 Colonic Lymphoma 350 Colonic Polyposis 519 Colorectal Carcinoma 361 Adenoma 363 Complications 366 EUS 368 Inflammatory Bowel Disease 366 Lymphatic Spread 369 Multiple 365 Obstructing 366 Rectal Adenocarcinoma: Lymphatic Drainage 369 Screening 362 Synchronous 365 Villous Adenoma 363 Community-acquired Pneumonia 179 Congenital CNS Malformations 602 Congenital Diaphragmatic Hernia 608 Constrictive Bronchiolitis 36 Cor Pulmonale 133 Corpus Callosum - Agenesis 605 Cowden’s Syndrome 525 Crohn disease 382, 386 Gastric 410 Cronkite-Canada syndrome 526 Cryotherapy (Prostate Cancer) 622 Cryptorchidism 588 Cyst - Bile Duct (Hepatic) 272 Cyst (Breast) 223 Pneumocystography 223 Thickened Skin Pattern (breast) 224 Cyst (Bronchogenic) 159 Cyst (thymic) 161 Cystadenoma (Biliary) 272 Cystic Abdominal/Pelvic Collections 611 Cystic Adenomatoid Malformation 608 Cystic Disease of the Kidney 614 Cystic Fibrosis (P. aeruginosa) 185 Cystic Hygroma 607 Cystic Kidney Disease - Acquired 618 Cystic Lymphangioma (Male Breast) 258 Cystic Nephroma 686 Cystitis 650, 651 Cysts (Congenital - Mediastinum) 160 Cytomegalovirus 78 Cytomegalovirus Pneumonia 78 Dandy-Walker Malformation 604 Daughter cysts (E. granulosus) 287 Delayed Pyelogram 677, 678 Diabetic mastopathy 258

Diffuse Alveolar Damage 79 Diffuse Lung Disease 3 AIP 4 Asbestosis 3 Asthma 3 Bronchiectasis 6 Bronchoalveolar cell carcinoma 4 Constrictive bronchiolitis 3 Cor Pulmonale 11 DAD 4 DIP 4 Edema 6 Emphysema 3 Granuloma 7 Hypersensitivity pneumonitis 3 LAM 3 LCH 4 Löfgren syndrome 12 Lymphoma 4 NSIP 4 Organizing Pneumonia (BOOP) 4 Sarcoidosis 3, 6, 7 Differential Diagnosis 10 Mycetoma 12 Diffuse Panbronchiolitis 36 Diverticulitis 396 Cecal 397 Colovesical Fistula 396 CT 397 Differential Diagnosis 396 Pericolic Abscess 396 Ductal Carcinoma in Situ 238 Biopsy 243 Calcification 240 Classification 239 Ductal Plate 303 Duodenal Carcinoid 357 Duodenitis 455 Echinococcosis 188 Echinococcus granulosus 188, 286 Echinococcus multilocularis 286 Ectopic pregnancy 598 Eisenmenger Physiology 133 Emphysema 27 Emphysematous Cholecystitis 441 Emphysematous Cystitis 651 Encephalocele 605 Endometrial Carcinoma 558 FIGO – Staging 559 Endometrioma 656 Endometriosis 655, 656 Endorectal Coil MRI 622 Enteric Cyst 374 Enteritis - Chronic Radiation 497 Enterography (MR - Crohn Disease) 389 Epidermal Inclusion Cyst (Male Breast) 260 Epidermoid Cyst(Testis) 588 Epididymal Masses 590 Adenomatoid Tumor 590 Lipoma (Paratesticular) 592 Papillary Cystadenoma 590 Polyorchidism 592 I3

Epididymis 586, 636 Epididymitis 591 Epiploic Appendagitis 430 Epithelial Inclusion Cysts 661 Esophageal Rupture (Causes) 403 Esophageal Varices 470 Esophagus - Inflammatory Diseases 444 Barrett’s Esophagus 445 Candida Esophagitis 446 CMV Esophagitis 447 Drug-Induced Esophagitis 448 Herpes Esophagitis 446 HIV Esophagitis 447 Intramural Pseudodiverticulosis 448 Reflux Esophagitis 444 Esophagus - Malignant Tumors 452 Adenocarcinoma 453 Early Squamous Cell Carcinoma 453 Spindle Cell Carcinoma 454 Squamous Cell Carcinoma 452 Esophagus Tumors 450 Duplication Cyst 452 Leiomyoma 450 Leiomyomatosis 451 Squamous Papilloma 450 Extralobar Sequestration 608 Extramedullary hematopoiesis 164 Extrarenal Cysts 615 Fetal Anomalies 607 Fibrolamellar Carcinoma 278 Fibrous Pseudotumor 592 Foramen Of Winslow Hernia 404 Fournier Gangrene 591 Galactocele 223 Gallbladder and Biliary Neoplasms 313 Gallbladder Empyema 442 Gallbladder Wall Thickening 440 Gallstone 438 Ganglion Cell Tumors 581 Ganglioneuroblastoma 158 Ganglioneuroma 158, 581 Gangrenous Cholecystitis 441 Gastric Lymphoma 335 Gastric Malignancies 332 Carcinoid 340 Carcinoma of the Cardia 334 Gastric Adenocarcinoma 332 Gastrointestinal Stromal Tumors (GIST) 338 Kaposi Sarcoma 341 Lymphoma 335 Mesenchymal Neoplasm 338 Metastases 341 Mucosa-Associated Lymphoid Tissue 335 Scirrhous Carcinoma 334 Gastric Ulcer Investigation 457 Gastric Volvulus 405 Bezoar (Gastric) 407 Zollinger-Ellison Syndrome 408 Gastritis 455 Gastrointestinal Bleeding 468 Angiography 470 Endoscopy 469

Gastritis 470 Lower GI 469 Nuclear Scintigraphy 469 Risk of Rebleeding 470 Upper GI 469 Gastrosplenic ligament 531 Germ Cell Neoplasms 155 (Chest) Germ Cell Tumors (Retroperitoneum) 583 Gestation 596 Gestational Sac 594 Gestational Trophoblastic Disease 661 GIST (Gastrointestinal Stromal Tumors) 338 Glucagonoma 328 Goiter (mediastinal) 161 Graft-vs-Host Disease 79 Granular Cell Tumor (Male Breast) 260 Granuloma (lung) 205 Granulomatous Mastitis 261 Gut Hemangioma 471 Gynecomastia 257, 258 H.influenzae 178 H.Pylori 459 Hamartoma 199 Hamartoma (Breast) 222 Hemangioma 268 Hemangioma (Gut) 471 Hemangioma (Mediastinum) 163 Hemochromatosis 300 Hereditary 300 Secondary 301 Hemosiderosis 300 Hepatic Artery Thrombosis (liver transplantation) 671 Hepatic Cyst (Complex) - Differential Diagnosis 417 Hepatic Mass with a Scar - Differential Diagnosis 418 Hepatic Neoplasms 267 Hepatic Portal Venous Gas 402 Hepatocellular Adenomatosis (see Benign Hepatic Neoplasms) 271 Hepatocellular Carcinoma 275 Hereditary GI Polyposis Syndromes 524 Herniations 164 Hiatus Hernia 164 Morgagni 164 Herpes viruses (respiratory) 182 Heterotopic Pregnancy 599 Histoplasmosis 100 Acute Radiology 101 Chronic 102 Disseminated 102 Fibrosing Mediastinitis 104 Histoplasmoma 103 Mediastinal granuloma 103 Pathology 101 Hodgkin Disease - Mediastinum 150 Holoprosencephaly 603 Hydranencephaly 602 Hydrocele 590 Hydrocephalus 603 Hydronephrosis 610 Hydronephrosis (post transplantation) 666 Hydrosalpinx 658 Hypoplastic Left Heart 608 I4

Idiopathic Inflammatory Bowel Disease 382 Immunocompromised Host (Hepatic Infections) 289 Candidiasis 289 Hepatosplenic Candidiasis 290 Pneumocystis jiroveci 291 Infiltrating Colloid Carcinoma 323 Inflammatory Carcinoma (breast) 225 Inflammatory Myofibroblastic Tumors 379 Inflammatory Pseudotumor (lung) 202 Influenzae 178 Influenzae A 181 Inguinal Hernia 634 Iniencephaly 607 Interstitial Pneumonia 182 Interstitial Pneumonias 14 Acute Interstitial Pneumonia (AIP) 14 Cryptogenic Organizing Pneumonia (COP) 14 Desquamative Interstitial Pneumonia (DIP) 14 Idiopathic Pulmonary Fibrosis (IPF) 14 NonSpecific Interstitial Pneumonia (NSIP) 14 Respiratory Bronchiolitis-Interstitial Lung Disease (RBILD) 14 Usual Interstitial Pneumonia (UIP) 14 Interstitial Pregnancy 599 Intracranial Aneurysms 615 Intraductal Papillary Mucinous Neoplasm 324 Intrahepatic Portal Venous Air 492 Intravenous Talcosis 134 Ischemia Mimic (Mesenteric) 500 Jejunal and Ileal Carcinoid 357 Juvenile Laryngeal Papillomatosis 201 Juvenile Polyposis: Syndrome 525 K. pneumoniae 178, 183 Kaposi Sarcoma (Gastric) 341 Kidney Cystic Diseases 614 Neoplasms 561 Transplants 664 Trauma 576 KIT 338 Krukenberg Metastasis 335 L. pneumophila 183 Langerhans Cell Histiocytosis 29 Large Cell Carcinoma 114 Legionella 178 Leiomyoma 258, 261 Leiomyosarcoma (retroperitoneum) 582 Limb-Body-Wall Defect 610 Lipoma 258 Lipoma (breast) 222 Lipomatous Tumors (Liver) Angiomyolipoma 273 Myelolipoma 273 Liposarcoma (breast) 222 Liposarcoma (retroperitoneum) 583 Liver Disease (chronic) 293 Liver Mass with Fat - Differential Diagnosis 420 Liver Neoplasms 267, 275 Liver Transplantation 670 Complications 671 Liver Transplants 664 Lung Cancer 111, 149

Cigarette smoking 111 Clinical Presentation 111 Paraneoplastic Syndromes 111 Lymphangioleiomyomatosis 38 Lymphangioma Mediastinum 162 Lymphangioma Retroperitoneum 582 Mesentery Differential Diagnosis 374 Lymphangitic Carcinomatosis 143 Imaging Features 143 Lymphocele (post kidney transplantation) 666 Lymphoma 150 Lymphoma Burkitt) 348 Lymphoma Primary Gastric 336 Lymphoma Testicular 588 Lymphomatoid Granulomatosis 70 Epstein-Barr Virus 70 M. pneumoniae 180 Malabsorption 505 Malakoplakia 650 Male Breast 257 Cancer 261 Malignant Fibrous Histiocytoma 582 Malignant Germ Cell Neoplasms (Non-Seminomatous) 156 Malignant Hepatic Neoplasms 275 Angiosarcoma 280 Epithelioid Hemangioendothelioma 280 Fibrolamellar Carcinoma 278 Hepatocellular Carcinoma 275 Intrahepatic Cholangiocarcinoma 279 Malignant Neoplasia (Chest)149 Malignant Peripheral Nerve Sheath Tumor 158 Mallory-Weiss Tear 471 MALT 335 Masaoka (Thymoma: Staging) 154 Mastitis 224 Mastitis (Granulomatous - Male Breast) 261 Mature Teratoma 155 Meckel Diverticulum (complications) 422 Diverticulitis 424 Diverticulitis with a Stone 425 Hemorrhage 424 Heterotopic Gastric Mucosa 423 Heterotopic Pancreatic Mucosa 423 Inverted 425 Meckel’s Diverticulum 473 Mediastinal Compartments 148 Fibrosis 135, 151 Goiter 161 Masses 148 Mediastinitis 165 Mediastinum 148 Medullary Carcinoma (Kidney) 565 Menetrier Disease 409 Meningocele (Thoracic) 158 Mesenchymal Neoplasm of the Stomach 338 I5

Mesenteric Adenitis 430 Mesenteric Cyst 372 Mesenteric Fibromatosis (Desmoid Tumor) 346, 376 Mesenteric Ischemia 487 Mesenteric Ischemia - Etiologies 495 Embolus 495 Thrombosis 497 Mesenteric Masses (Differential Diagnosis) 346 Mesenteric Masses and Cysts 372 Benign Multicystic Mesothelioma 375 Diffuse Peritoneal Malignant Mesothelioma 375 Enteric Cyst and Mesothelial Cyst 374 Inflammatory Myofibroblastic Tumors (Inflammatory Pseudotumor) 379 Lymphangioma 373 Nonpancreatic Pseudocyst 374 Sclerosing Mesenteritis 378 Mesothelioma Malignant 215 Mesenteric 374 Benign Multicystic 375 Cystic Malignant 375 Diffuse Malignant 375 Scrotal 593 Metastases (pleural) 216 Metastases (pulmonary) 138 Calcification 142 Cannonball 140 Cavitation 141 Endobronchial 144 Lymphangitic Carcinomatosis 143 Micronodular 141 Parenchymal Nodules 139 Pathogenesis of Hematogenous Metastases 139 Pleural 144 Solitary 142 Tumor Embolism 143 Metastases Breast Young Women 254 Microcystic Adenoma 327 Microscopic Polyangiitis 67 Mole 661 Benign Hydatidiform 661 Choriocarcinoma 662 Complete 662 Partial 662 Morgagni (Herniation) 164 Mounier-Kuhn Syndrome 32 MR Enterography 389 MRSA 186 Mucinous Cystadenoma/Cystadenocarcinoma (Appendiceal) 428 Mucinous Cystic Neoplasm 326 Mucinous Noncystic Adenocarcinoma 324 Mucoepidermoid Carcinoma 196 Multilocular Cystic Nephroma 686 Multiple Lymphomatous Polyposis (Mantle Cell Lymphoma) 348 Myasthenia Gravis (Thymoma and) 152 Mycoplasma 178 Myelolipoma 647 Myofibroblastoma (Male Breast) 257, 260 N. Asteroides 188

Necrotizing Sarcoid Granulomatosis 69 Neoplasms (Germ Cell) 155 Neoplasms (Neurogenic) 157 Nephrocalcinosis 694 Nephrogram 674 Nephroma (Multilocular Cystic) 569 Nerve Sheath Tumor 158 (Chest) Nerve sheath Tumors (Retroperitoneum) 581 Neural Tube Defects 605 Neuroblastoma (Mediastinal) 158 Neuroendocrine Tumors (pancreas) 328 Neurofibroma 157 Neurofibromatosis (NF1) 158 Neurogenic Neoplasms 157 Neurogenic Tumors (Retroperitoneum) 580 NF-1 (GI Neoplasms) 359 Nocardia 178 Nodes (NHL - Gastrointestinal) 345 Nodular Lymphoid Hyperplasia Colon 527 Non Hodgkin Lymphoma (abdominal) 344 Adenopathy 345 AIDS-Related Lymphoma 349 Burkitt Lymphoma 348 Differential Diagnosis 346 Enteropathy-Type T-cell Lymphoma 349 Gastrointestinal Lymphoma 346 Mantle Cell Lymphoma 348 Small Intestine 347 Small Intestine: Differential Diagnosis 349 Non Hodgkin lymphoma - Mediastinum 150 Non-Hereditary GI Polyposes 524 Non-Neoplastic Lymphadenopathy - Mediastinum 151 Non-Seminomatous Malignant Germ Cell Neoplasms 156 Nosocomial Pneumonia 185 Nosocomial Pneumonia and Aspiration 186 Ogilvie Syndrome 401 Oligohydramnios 609 Omental Infarction 431 Oncocytoma 569 Orchitis 591 Organ Transplantation 75 CMV 76 Fungal Infections 77 Graft-vs-Host 76 Pneumocystis carinii 76 Ovarian Cyst 611 Ovarian Cysts 653 Corpus Luteum 654 Follicular 653 Functional 653 Hemorrhagic 654, 655 Hyperstimulation 655 Rupture 655 Theca Lutein 655 Ovarian Neoplasms 637 Epithelial Ovarian Neoplasms: Endometrioid 639 Epithelial Ovarian Neoplasms: Mucinous 639 Epithelial Ovarian Neoplasms: Serous 638 Epithelial Ovarian Tumors: Clear Cell 639 Epithelial Tumors: Classification 637 Epithelial Tumors: Terminology 638 Mature Cystic Teratoma 640 I6

Ovarian Germ Cell Neoplasms 640 Ovarian Malignant Germ Cell Tumors 641 Sex-cord stromal tumors 641 Ovarian Torsion 657 Ovary non neoplastic disorders 653 polycystic 660 Masses 637 Pancoast Tumor 113 Pancreas 412 Annular 412 Divisum 413 Intraductal Papillary Mucinous Neoplasm 415 Pancreatitis, Chronic 414 Pancreas (Neoplasms) 321 Adenocarcinoma 321 Intraductal Papillary Mucinous Neoplasm 324 Islet Cell Tumors 328 Metastatic 329 Microcystic Adenoma 327 Mucinous Cystic Neoplasm 326 Mucinous Noncystic Adenocarcinoma 323 Oligocystic Adenoma 328 Solid and Pseudopapillary Epithelial Neoplasm 325 Pancreas Transplants 664, 669 Complications 669 Rejection 670 Vascular Thrombosis 670 Pancreatic Adenocarcinoma 321 Resectability 323 Pancreatic Duct 411 Pancreatitis 460 Papilloma 201 Papilloma (Male Breast) 257 Papillomatosis (Biliary) 319 Paraganglioma 159 Paraganglioma (Extra-adrenal pheochromocytoma) 581 Paragonimiasis westermani 189 Paraovarian Cysts 661 Parathyroid Adenoma 162 Pelvic Inflammatory Disease 659 Pelvic MRI 553 Peptic Ulcer Disease 455 Peribronchial abscesses 188 Perinephric Fluid Collections 665 Peritoneal Inclusion Cysts 523, 659 Peritoneal Lymphoma (Primary) 350 Persistent Bilateral Nephrogram 679 Peutz Jeghers 525 Pheochromocytoma 647 Pleural Disease 204 Bacterial Pneumonia 208 Empyema 208 Pleural Effusion 208 Pleural Effusion: Asbestos Exposure 210 Pleural Effusion: Subpulmonic 209 Pleural Effusion: Tuberculosis 209 Pleural Fibrosis 211 Pneumothorax 211 Pulmonary Ligament 207 Round Atelectasis 210 Pleural Effusion 213

Malignant 213 Pleural Neoplasms 213 Localized Fibrous Tumor 213 Mesothelioma 215 Pneumatocele 190 Pneumatosis Intestinalis 493 Pneumocystis Jiroveci 78 Pneumocystis Jiroveci Pneumonia 78 Pneumonia 178 Pneumoperitoneum 403 Pneumothorax 211 Polycystic Kidney Disease (Autosomal Recessive) 616 Polycystic Liver Disease 306 Polycystic Ovary Syndrome 660 Polyhydramnios 609 Polymastia 221 Polyposis - Familial 519 Polysplenia 534 Polythelia 221 Post Transplant Lymphoproliferative Disorder 672 Post Transplant Malignancy 673 Posterior Urethral Valves 611 Post-transplantation Imaging 664 Post-transplantation Lymphoproliferative Disorder (GI) 349 Pregnancy 598 Primary Ciliary Dyskinesia 32 Prostate Cancer 620 Grading 620 Screening 620 Prostate Specific Antigen 620 Pseudoaneurysms (kidney transplantation) 669 Pseudocyst (Nonpancreatic) 374 Pseudocyst (Pancreatitis) 462 Pseudogynecomastia 257, 259 Pseudopapillary Epithelial Neoplasm (Solid and) 325 Pulmonary Blastoma 198 Pulmonary Circulation 131 Idiopathic 132 Pulmonary Embolism 82 Arterial Blood Gases 85 Chest X-Ray 84 Clinical Science Probability 85 Combined Pulmonary CTA and Venography 89 Common Radiographic Abnormalities 84 CT Angiography 86, 87 CT Findings 84 Diagnostic Algorithm 89 Pitfalls 88 Small Emboli 86 Ventilation/Perfusion Scanning 85 Pulmonary Gangrene 190 Pulmonary Hypertension 131 Pulmonary Lymphoid Disorders 55 B-Cell Lymphoma 58 Follicular bronchitis 55 Follicular Hyperplasia 55 Lymphoid Interstitial Pneumonia LIP 56 Lymphomatoid Granulomatosis 59 Nodular Lymphoid Hyperplasia 57 Posttransplantation Lymphoproliferative Disease 60 Pseudolymphoma 57 Pulmonary Lymphoid System 54 I7

BALT 54 Bronchus Associated Lymphoid Tissue 54 Pulmonary Thromboendarterectomy 134 Pulmonary Venous Hypertension 135 Pyelogram 674 Pyelonephritis 678 Pyogenic Hepatic Abscess 284 Pyosalpinx 659 Radioactive Ablation (Prostate Cancer) 622 Rejection (kidney transplants) 667 Renal Anomalies 610 Renal Artery (kidney transplantation) 668 Renal Calcifications 693 Dystrophic 693 Medullary 694 Renal Cancer in ACKD 619 Renal Cell Carcinoma 563 Renal Injuries 576 Renal Masses 681 Carcinoma In Situ 681 follow up interval 683 Multilocular Cystic Nephroma 686 Small renal mass 682 Renal Neoplasms 561 Angiomyolipoma 570 Infiltrating Renal Cell 564 Juxtaglomerular Cell Tumor 570 Medullary Carcinoma 565 Metastases 569 Multilocular Cystic Nephroma 569 Oncocytoma 569 Renal Cell Carcinoma 563 Renal Lymphoma 568 Robson Staging 565 Spontaneous Renal Hemorrhage 564 Squamous Cell Carcinoma 568 TNM Staging 565 Transitional Cell Carcinoma 567 Tuberous Sclerosis 570 Uroepithelial Neoplasms 567 Venous extension 562 Renal Transplants 664 Renal Vein Thrombosis (kidney transplantation) 668 Respiratory Bronchiolitis 29 Respiratory Viruses 181 Retroperitoneal Fibrosis 580 Retroperitoneal Masses 611 Retroperitoneal Masses (Fat containing) 584 Retroperitoneum 579 Ruvalcaba-Myhre-Smith 526 S. pneumoniae 178, 179 S.aureus 184 Saber Trachea 28 Sacrococcygeal Teratoma 612 Salpingitis 659 Sandwich Sign (NHL) 345 SARS 182 Scar Carcinoma 116 Schistosomasis (Bilharziasis) 288 Schistosomiasis (Urinary) 651 Schwannoma 157 Sclerosing Cholangitis (primary) 307

Cholangiocarcinoma 308 Sclerosing Mesenteritis 378 Scrotal Anatomy 630 Scrotal Calculi 590 Scrotum 585 Seminoma 156 Sequestration 608 Serous Inclusion Cysts 661 Serum Alpha-Fetoprotein (Elevated Maternal) 691 Severe Acute Respiratory Syndrome 182 Shock Bowel 500 Shwachman - Diamond Syndrome 513 Sigmoid Volvulus 401 Silicosis 46 Adenopathy 47 Alveolar Proteinosis 48 Calcification 47 Conglomeration 48 Scar emphysema 48 Small Bowel Bleeding 472 Small Bowel Lymphoma 347 Small Bowel Obstruction 475 Small Cell Lung Cancer 113 Small Intestinal Benign Neoplasms 353 Adenoma 355 Brunner Gland Hamartoma 354 Brunner Gland Hyperplasia 354 Differential Diagnosis: Duodenal Polyp 354 Periampullary Adenocarcinoma 355 Periampullary Duodenal Mass 355 Tubulovillous Adenoma 355 Small Intestine 347 Adjacent Mesenteric Disease 348 Cavitary Mass 348 Mural Infiltration 347 Small Intestine Malignant Neoplasms Adenocarcinoma 355 Carcinoid 357 Carcinoid Syndrome 358 Differential Diagnosis 356, 359 Metastatic Disease 359 Small Intestine NHL - Differential Diagnosis 349 Smoking Related ILD 16 Macrophages 16 RB 16 Respiratory bronchiolitis 16 Spermatic cord 586 Spleen 531 Splenorenal ligament 531 Splenosis 533 Sprue 505 Squamous Cell Carcinoma 111 Steatosis 296 Stomach Malignancies 332 Stone Urinary 624 Striated Nephrogram 679 Strongyloides stercoralis 189 Strongyloidiasis 188 Swyer James Syndrome 38, 182 Sympathetic Ganglia Tumors 159 Tamoxifen 558 T-cell Lymphoma (Enteropathy-Type T-cell Lymphoma) 349 I8

Teratoma (Mature) 155 Teratoma (Retroperitoneum) 583 Testicular Carcinoma (Risk Factors) 588 Testicular Cysts 589 Testicular Ischemia 635 Testicular Masses (Bilateral) 589 Testicular Neoplasms 586 Germ Cell Neoplasms 587 Non Seminomatous Germ Cell Tumor 587 Seminoma 587 Testicular Torsion 630 Testis 586 Testis (torsion) 632 Thymic Carcinoid 154 Thymic Cyst 161 Thymic Hyperplasia 161 Thymolipoma 154 Thymoma 152 Torsion (Ovary) 657 Torsion (Testicle) 591 Transplants (Solid Organs) 664 Transverse Colon Volvulus 401 Trauma (Urinary Tract) (see Urinary Tract Trauma) 573 Trisomy 18 606 Trisomy 21 600 TRUS 621 Tuberculosis 93 Clinical features 95, 96 Hemoptysis 98 Lymphatic gradient 95 M. tuberculosis 93 Mycetoma 98 Mycobacteria 93 Pathogenesis 94 Radiologic features 95, 96 Rassmussen (pulmonary artery) aneurysm 98 Tuberculoma 97 Tuberculosis (renal) 693 Tuberculosis (scrotal) 591 Tuberous Sclerosis 39, 616 Renal 570 Tubular Ectasia (Testis) 589 Twins 596 Dizygotic 596 Monozygotic 597 Ulcerative Colitis 382, 384 CT Features 384 Imaging Features 384 Toxic Megacolon 385 Ulcers - Duodenal 458 Ulcers - Gastric 456 Benign 456 Equivocal 457 Malignant 457 UPJ disruption 578 Urachal Anomalies 649 Ureteral Injury 575 Urethra 649, 652 Urethral Trauma 573 Urethrography 652 Urinary Bladder 649 Urinary Stone Disease 624

Urinary Tract Trauma 573 Uroepithelial Neoplasms 567 Urothelial carcinoma 649 Usual Interstitial Pneumonia: Histology 15 Fibroblast foci 15 Uterine Disorders 551 Arcuate 555 Bicornuate 554 Diethylstilbestrol: DES – Related 556 Mullerian Duct Anomalies 554 Septate 555 Unicornuate / Didelphys 554 Uterine Masses - Benign 556 Abnormal Uterine Bleeding 557 Adenomyosis 557 Endometrial Hyperplasia 558 Endometrial Polyps 558 Leiomyoma 556 Postmenopausal Bleeding 558 VACTERL Syndrome 610 Varicella Pneumonia 182 Varices (Esophageal) 164 Varicocele 592 Ventilator-associated Pneumonia 186 Viruses - Respiratory 181 Von Hippel Lindau 617 Water Lily Sign (E. granulosus) 287 Wegener’s Granulomatosis 63 halo sign 66 Williams-Campbell 31 Yolk sac 596 Zollinger-Ellison Syndrome 328

I9

Radiologic Pathology Fifth Edition VOLUME 2 Musculoskeletal Radiologic Pathology Correlation

2006 2007

Editors Angela D. Levy, COL, MC, USA Chairman and Registrar Chief, Gastrointestinal Radiology

Ellen M. Chung, LTC, MC, USA Chief, Pediatric Radiology

Jeffrey R. Galvin, MD Chief, Chest Radiology

Kelly K. Koeller, MD Chief, Neuroradiology

Mark D. Murphey, MD Six Week Course Director Chief, Musculoskeletal Radiology

Paula J. Woodward, MD Chief, Genitourinary Radiology

Associate Editor Jean-Claude Kurdziel, MD

Illustrators Aletta A. Frazier, MD Dianne D. Engelby, MAMS, RDMS Heike Blum, MFA

Department of Radiologic Pathology Armed Forces Institute of Pathology Washington DC, USA

American Registry of Pathology Armed Forces Institute of Pathology Washington, DC 20306-6000 _____________________________________ © Copyright 2006 by the American Registry of Pathology. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means: electronic, mechanical, photocopy, recording, or any other information storage and retrieval system without written permission of the publisher. Made in the United States of America _____________________________________ Great care has been taken to guarantee the accuracy of the information contained in this volume. However, neither the American Registry of Pathology, Armed Forces Institute of Pathology, nor the editors and contributors can be held responsible for errors or for any consequences arising from the use of the information contained herein. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as representing the views of the Departments of the Army, Air Force, Navy, or Defense. 987654321 Library of Congress Cataloging-in-publication Data [in process] ISBN 1-933477-00-8

Preface The Armed Forces Institute of Pathology’s Radiologic Pathologic Correlation course presented by the Department of Radiologic Pathology enters its 59th year of educating radiology residents worldwide. For the fifth year, our staff and visiting lecturers have contributed their lecture material and images to compile Radiologic Pathology 2006 – 2007, continuing the tradition of presenting richly illustrated material that teaches the pathologic basis of disease to improve our understanding of the imaging appearance of disease. We hope the efforts of our authors and editors have once again accomplished our goal of bringing the outstanding and unique Radiologic Pathologic Correlation course to your fingertips.

Acknowledgements The annual production of the Radiologic Pathologic Correlation course and syllabus is made possible through the tremendous support, dedication, and selfless service of countless individuals who work in the AFIP and the various institutions and organizations throughout the world that believe in the importance of teaching the principles of disease through radiologic pathologic correlation. The Department of Radiologic Pathology of the Armed Forces Institute of Pathology expresses our deepest appreciation and sincerest gratitude to: - All radiologists and radiology residents who have contributed case material to the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of Pathology, - All pathologists in the AFIP who have donated their time and expertise to radiologic pathologic correlation, - All of our outstanding authors, illustrators, and department staff members who make the course and the syllabus happen effortlessly year after year, - And, to the extraordinary efforts of our production team, headed by JeanClaude Kurdziel, MD, who have tirelessly dedicated the spring and summer of the last five years to the production of this syllabus.

iii

Faculty – VOLUME 2 Musculoskeletal Radiology

Mark E. Schweitzer, MD

Mark D. Murphey, MD

Professor of Radiology and Orthopedic Surgery Chief of Radiology - Hospital for Joint Diseases Director, Musculoskeletal Radiology New York University New York, NY

Chief, Musculoskeletal Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Christopher G. Fielding, COL, DC, USA Department of Oral Maxillofacial Pathology Armed Forces Institute of Pathology Washington, DC

Mark W. Anderson, MD Associate Professor of Radiology and Orthopedic Surgery Division Head, Division of Musculoskeletal Radiology University of Virginia Health System Charlottesville, VA

Donald J. Flemming, CAPT, MC, USN G. Victor Rohrer Professor of Radiology Education Associate Professor of Radiology Penn State Hershey Medical Center Hershey, PA

Mark J. Kransdorf, MD Professor of Radiology Mayo Clinic College of Medicine Rochester, MN and Consultant, Musculoskeletal Radiology Department of Radiology Mayo Clinic Jacksonville, FL

William B. Morrison, MD Associate Professor of Radiology Director, Division of Musculoskeletal and General Diagnostic Radiology Thomas Jefferson University Hospital Philadelphia, PA

Michael Mulligan, MD Associate Professor of Diagnostic Radiology University of Maryland School of Medicine Baltimore, MD

Thomas L. Pope, MD Clinical Professor of Radiology/Orthopedics Medical University of South Carolina Charleston, SC and Former Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Charles S. Resnik, MD Professor of Diagnostic Radiology Director, Section of Musculoskeletal Radiology Director, Residency Program University of Maryland School of Medicine Baltimore, MD

Timothy Sanders, MD Assistant Professor of Radiology Department of Radiology Uniformed Services University of the Health Sciences Bethesda, MD iv

Musculoskeletal Radiology Mark D. Murphey, MD Radiologic Assessment of Joint Replacement and Imaging of Bone Grafts . . . . . . . . . . . . . . . . . . . . . . . . .699 Musculoskeletal Manifestations of Chronic Renal Insufficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .711 Fundamental Concepts of Musculoskeletal Neoplasm: Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .720 Fundamental Concepts of Musculoskeletal Neoplasm: CT and MR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .733 Osteoid Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .743 Cartilaginous Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .757 Fibrous Lesions of the Musculoskeletal System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .771 Alphabet Soup and Cystic Lesions of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .784 Juxtaarticular Soft Tissue Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .792 Musculoskeletal Angiomatous Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .805 Paget Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .812 Musculoskeletal Infection I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .820 Musculoskeletal Infection II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .829 Imaging of Cervical Spine Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .839

Christopher J. Fielding, COL, DC, USA Radiographic Differential Diagnosis of the Jaws . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .849

Mark W. Anderson, MD MRI of the Knee: Part 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .858 MRI of the Knee: Part 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .865 MRI of the Wrist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .872 MRI of the Ankle and Foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .879

Mark J. Kransdorf, MD Osseous Lesions: Unknown Histogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .887 Soft Tissue Lipomatous Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .893 Metabolic Bone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .900 Osteonecrosis and Related Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .906

Donald J. Flemming, CAPT, MC, USN Approach to the Inflammatory Arthropathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .912 MRI of the Rotator Cuff . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .925

Timothy Sanders, MD MR Arthrography of Glenohumeral Instability . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .932 Imaging of Upper Extremity Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .941

Charles S. Resnik, MD Crystal Deposition Diseases and Neuropathic Osteoarthropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .949

Mark Schweitzer, MD / William Morrison, MD MRI of the Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .955

Michael Mulligan, MD Skeletal Metastases, Myeloma, Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .961

Thomas Lee Pope, MD Imaging of Hematologic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .968 Generalized Musculoskeletal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .976 Osseous Musculoskeletal Stress Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .987 Pelvis and Lower Extremity Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .995

Mark D. Murphey, MD Musculoskeletal Seminar I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1005 Musculoskeletal Seminar II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1009 Musculoskeletal Seminar III . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1013 Musculoskeletal Seminar IV . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1018 Musculoskeletal Seminar V . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1025

v

Musculoskeletal Radiology

697

698

Radiologic Assessment of Joint Replacement and Bone Grafts Mark D. Murphey, MD • •

Metal components Ultra-High Molecular Weight Polyethylene

• • •

Cobalt-chromium-molybdenum alloy Cobalt-chromium-tungsten alloy Titanium-aluminum-vanadium alloy

Current Materials For Total Joint Replacement

Metallic Components

• • •

Ultra-High Molecular Weight Polyethylene Component

• • • • • • • •

Figure 4-1-1

Allows articulation of metallic components Lowers friction and prolongs wear Allows some plastic deformity improving joint congruity Radiolucent

Complications of Joint Arthroplasty I. Loosening and/or infection II. Small particle disease (osteolysis) III. Dislocation and abnormal alignment IV. Fractures and nonunion V. Heterotopic bone formation VI. Cement extrusion VII. Polyethylene liner displacement and metal arthropathy

• • • •

Most common complication historically Difficult to differentiate 4–13% hip replacement 7–10% knee arthroplasty

• • • • • • •

Cement-bone lucency or cement metal lucency >2mm Progressive widening of interfaces post-op Component migration Fracture of metal or cement Periosteal reaction Smooth endosteal scalloping with cement lucency Air in soft tissues or joint

Loosening and/or Infection

Aseptic loosening of both acetabular and femoral components of a total hip arthroplasty with bone cement (arrows), and cement-metal lucency (arrowheads) that progresses over several years (right image). Cement fracture (open arrow) and lateral migration of the femoral stem (curved arrow) are also apparent

Radiographic Signs: Loosening-Infection Cemented Arthroplasty [Figure 4-1-1]

Figure 4-1-2

Bone Ingrowth (Porous Coated) Joint Arthroplasty • • • • •

Improved longevity Beads sintered onto metal surface Bone ingrowth into irregular surface (biologic fixation) Technically demanding No motion to promote bone ingrowth

Normal Radiographic Appearance Ingrowth Arthroplasty • •

[Figure 4-1-2]

• • • •

Resorption of medial femoral cortex Thin lucent rim with sclerotic margin about metal (< 2 mm) Endosteal sclerosis Prosthetic subsidence (< 10 mm) Periosteal reaction and cortical thickening Not progress after 9 – 12 months post-op

Musculoskeletal Radiology

Normal appearance of ingrowth hip arthroplasty with superolateral lucency (arrowheads) (<2mm) and surrounding sclerosis and resorption of medial cortex (arrow)

699

Joint Replacement and Bone Grafts

Loosening/Infection Radiographic Findings With Ingrowth Arthroplasty • • • • •

Figure 4-1-3

[Figures 4-1-3 and 4-1-4]

Prominent prosthetic subsidence (>8mm) Bone destruction Component migration or motion Prominent lucent zone about metal (>2mm) Increasing number of displaced beads

•

Infection of Total Joint Arthroplasty The major long-term complication (1%-4%) ➢ 33% first 3 months post-op ➢ 33% 3 months – 12 months post-op ➢ 33% > 12 months post-op • Usually associated loosening • Difficult to differentiate from aseptic loosening

Radiographic Signs Most Suspicious for Infection [Figure 4-1-5] •

Aseptic loosening of femoral component ingrowth hip replacement with prominent lucency, lateral migration of femoral stem (arrow), and subsidence medially (arrowhead)

Extensive bone destruction ➢ 47% sensitive; 96% specific • Air in soft tissue and/or joint • Extensive or aggressive periosteal reaction ➢ 25% sensitive; 92% specific • Wide or irregular lucent zone

Figure 4-1-4

Radionuclide Evaluation of Total Joint Arthroplasty • • • • • •

Bone scintigraphy Gallium scan Indium WBC scan PET (combined with bone scan)

Bone Scintigraphy • • • •

Normal increased activity post-op (6–9 months) Increased activity subsequently suspicious for loosening/infection Nonspecific Overall accuracy 50%-70%

Gallium (Ga-67) Scanning •

In conjunction with bone scan Incongruence with increased gallium uptake vs. bone scan suspicious for infection Not as accurate as bone scan WBC combination

Aseptic loosening of acetabular component ingrowth hip replacement with prominent lucency (arrows) and bead sheading (circles)

Figure 4-1-5

Infection of total knee arthroplasty with early prominent development of lucency and bone destruction (arrows) about both the femoral and tibial components soon following surgery (two months- previous normal post-operative radiographs not shown)

Joint Replacement and Bone Grafts

700

Musculoskeletal Radiology

•

Indium-111 WBC Scanning

Figure 4-1-6

Increased sensitivity (50%-100%) and specificity (45%-100%) for infection of TJR • Increased activity at tip of metal components can be normal for up to 2 years post-op • Used in conjunction with bone and bone marrow scans incongruity with more uptake on WBC scan > 90% accuracy • Sensitivity and the ability to correctly localize infection decreases ➢ Infection becomes more chronic ➢ Anatomic location more central •

Arthrography of Joint Arthroplasty

• •

Purpose ➢ Obtain fluid for culture/sensitivity ➢ Document intra-articular location ➢ Confirm loosening ➢ Detect other causes of pain

Hip Arthrography Technique • • • • •

Preliminary fluoroscopy and radiographs Anterolateral approach to metal at head neck junction 20 gauge spinal needle Aspiration for culture If no aspirate inject saline reaspirate Contrast injection with subtraction technique Radionuclide injection Pre and post exercise radiographs

Digital subtraction arthrogram with contrast in the bone remnant interface of the acetabular component (zone 2-arrowhead) and below the intertrochanteric line (arrows) representing aseptic loosening of both components

Arthrographic Criteria for Loosening/Infection •

[Figure 4-1-6]

Acetabular Component ➢ Contrast in bone – cement or metal-cement interface all zones (90%) ➢ Contrast in bone – cement or metal-cement interface zones I & II or zones II & III (90%) ➢ Contrast in zones I & III with medium or large pseudocapsule bursa (57%) ➢ Rim of contrast >2mm thick any zone (95%) ➢ Lymphatic filling (?) • Femoral Component ➢ Contrast in cement-bone interface distal to intertrochanteric line (98%) ➢ Contrast in bone-metal interface below intertrochanteric line (98%) ➢ Contrast at or below mid component – long stemmed prosthesis (98%) • •

Figure 4-1-7

Knee Arthrography Technique • • • •

Preliminary fluoroscopy and radiographs Lateral patellofemoral or anterior intercondylar approach 20 gauge spinal needle Aspirate for culture If no aspirate inject saline reaspirate Contrast injection-subtraction technique Pre and post exercise radiographs

Arthrography and Bursa • • • •

[Figure 4-1-7]

Greater trochanteric – 50% Supraacetabular – 33% Iliopsoas – 17% Can reduce accuracy of arthrography

II. Small Particle Disease • • • • •

[Figures 4-1-8 and 4-1-9]

Granulomatous pseudotumor/histiocytic reaction/osteolysis Previously unusual late sequelae of arthroplasty Now may be most common cause failure Large lobulated lucencies with cortical thinning Prosthesis loosening

Musculoskeletal Radiology

701

Septic loosening of the femoral component on arthrography of a total hip replacement with synovial nodularity in the joint (arrows) and supraacetabular bursa formation (arrowhead). Contrast extends below intertrochanteric line Joint Replacement and Bone Grafts

Figure 4-1-8

Figure 4-1-9

Small particle disease (osteolysis or granulomatous pseudotumor) as a cause for loosening of the tibial component of a total knee replacement with large mass-like radiolucency/low attenuation in the proximal tibia (arrowheads) with associated fracture (arrow)

Figure 4-1-10 Small particle disease as a cause for loosening of femoral and acetabular components of a total hip replacement with multiple largely intracortical areas of radiolucency (arrowheads)

Dislocation / Abnormal Alignment Normal Alignment •

Acetabular angle: 40 degrees (+ or – 10 degrees) – AP view • Acetabular anteversion ➢ 0–30 degrees – lateral view Knee: • Tibial plateau component parallel to floor • Tibia 5–7 degrees valgus

Abnormal Alignment Predisposing to Subluxation • • • • • • • •

Dislocated noncemented total hip replacement, both femoral (arrowhead) and acetabular components (arrows), with increased inclination of the acetabular component as a predisposition to this complication

[Figure 4-1-10]

Varus position of knee is unacceptable Acetabular angle > 50/55 degrees – AP view Acetabular anteversion < 0 degrees or > 30 degrees lateral view Exceeding extremes of motion Interposed material Greater trochanteric separation Joint effusion Loss of soft tissue support or imbalance (knee)

Figure 4-1-11

IV. Fractures and Nonunion Associated with Arthroplasty • • • •

[Figures 4-1-11 and 4-1-12]

Bone Metal Cement Polyethylene

Greater Trochanteric Nonunion After Total Hip Arthroplasty • • • •

[Figure 4-1-11]

Improves exposure at surgery Osseous union normally 6 – 12 weeks Nonunion results in lack of abductor support Bursitis predisposes to dislocation

Joint Replacement and Bone Grafts

Fracture of greater trochanteric wire mesh on follow up radiograph (right image) with retraction due to the pull of the abductors (arrow) about the total hip replacement.

702

Musculoskeletal Radiology

•

Heterotopic Bone Formation After Total Joint Arthroplasty • • •

Figure 4-1-12

Not infrequent – 3 weeks post-op 2 years to mature Hip: 21%–40%; Knee: 10% anterior to femur Predisposing conditions – Ankylosing Spondylitis, DISH, prior occurrence Treatment – radiation, steroids, diphosphonates, surgery, indocin

Brooker Classification Heterotopic Bone •

• • •

After hip replacement ➢ Class I: Small islands of bone ➢ Class II: Bone projection from acetabulum or femur with >1 cm between osseous surfaces ➢ Class III: <1cm between opposing bridge surfaces ➢ Class IV: Osseous ankylosis bridging joint

Cement Extrusion Usually clinically insignificant Vein or lymphatic Rarely nerve, vascular, bowel or bladder injury

Polyethylene Liner Displacement & Metal Arthropathy • • • • • •

[Figure 4-1-13]

•

Allows metal-metal friction Inflammation and aseptic synovitis Abnormal component position Visualize radiolucent polyethylene Metal – line sign and debris Prevented by early recognition

Ingrowth total hip replacement with fracture (arrow) at the tip of the femoral component transfixed by cerclage wires

Figure 4-1-13

VIII. Silicone Arthroplasty Complications ➢ Fracture ➢ Dislocation ➢ Infection ➢ Silicone arthropathy

Displacement of polyethylene liner on follow-up radiograph of total hip replacement (right image). Note widened medial joint space compared to initial post-op radiograph (left image) “metal-line” sign (arrowhead) and radiolucent rotated polyethylene liner (*)

References Joint Replacement 1. 2. 3. 4.

Bauer TW, Schils J. The pathology of total joint arthroplasty. I. Mechanisms of implant fixation. Skeletal Radiol. 1999 Aug;28(8):423-32. Review. Keogh CF, Munk PL, Gee R, Chan LP, Marchinkow LO. Imaging of the painful hip arthroplasty. AJR Am J Roentgenol. 2003 Jan;180(1):115-20. Manaster BJ. From the RSNA refresher courses. Total hip arthroplasty: radiographic evaluation. Radiographics. 1996 May;16(3):645-60. Review. Oswald SG, Van Nostrand D, Savory CG, Callaghan JJ. Three-phase bone scan and indium white blood cell scintigraphy following porous coated hip arthroplasty: a prospective study of the prosthetic tip. J Nucl Med. 1989 Aug;30(8):1321-31.

Musculoskeletal Radiology

703

Joint Replacement and Bone Grafts

• • •

History and Importance of Bone Graft Procedures

Figure 4-1-14

First performed 1688 Second most frequently transplanted tissue Vital for orthopedic management

Radiologic Assessment: Important for Patient Management • •

Normal bone graft incorporation Abnormal alterations

• • • • •

Radiographs Conventional Tomography Scintigraphy Computed Tomography (CT) Magnetic Resonance Imaging (MRI)

• • • • • • •

Delayed or nonunion Pseudarthrosis Fill osseous defects or cavities Arthrodesis Stabilize spinal segments Bone stock in arthroplasty Restore function in diseased articulations

• • • •

Autograft Allograft (homograft) Xenograft (heterograft) Bone graft substitute

• • •

Cancellous Cortical Combination

• • • • • • •

Onlay Inlay Dowel Muscle pedicle Strut Vascularized Clothespin (H)

Imaging Modalities

Bone Graft Indications

Pictorial representation of onlay (A) and inlay (B and insert) bone graft procedures

Figure 4-1-15

Classification of Bone Graft by Origin

Classification of Bone Graft by Structure

Classification of Bone Graft by Technique [Figure 4-1-14]

Radiographic Evaluation of the Donor Site • • • • • • • • •

[Figures 4-1-15 and 4-1-16]

Iliac Fibula Rib Distal radius Calvarium Femoral head Tibia Greater trochanter Posterior spinal elements

Dowel graft placed across a scaphoid nonunion (arrows) with progressive healing at both the bone grafted site (arrows) and bone graft donor site (arrowheads) on radiographs 3 months apart

Joint Replacement and Bone Grafts

704

Musculoskeletal Radiology

Normal Radiographic Appearance Of Donor Site • • • • •

Wedge or oval defect Irregular margins after surgery Initial increase in ill-defined margins Subsequent marginal sclerosis Complete regeneration

• • • • • •

Pain Failure to recognize (radiologists) Infection Muscle herniation Involvement of sacroiliac joint Fracture

Figure 4-1-16

[Figure 4-1-17]

Donor Site Complications

Normal Bone Graft: Repair/Incorporation • • • •

CT of normal iliac bone graft donor site with outer shell of bone retrieved (arrowheads) and no violation of the SI joint (arrow)

[Figures 4-1-18 and 4-1-19]

Cancellous autograft Cortical autograft Vascularized autograft Allograft

Figure 4-1-17

Figure 4-1-18

Fibula resection for use as bone graft with regeneration over time on three sequential radiographs

1 month post-op

2 months post-op

Figure 4-1-19

4 months post-op 6 months post-op Progressive normal incorporation of cancellous autograft (arrows) about post-traumatic site in the lower leg on radiographs with individual osseous fragments coalescing into a large ossific mass

Musculoskeletal Radiology

Normal incorporation (arrows) of fibular cortical graft replacing a humeral resection for Ewing sarcoma reconstruction. Three sequential radiographs show progressive osseous bridging between fibular graft and native humerus (arrowhead) 705

Joint Replacement and Bone Grafts

•

Important Terminology: Bone Graft Healing

•

Osteoconduction: Tissue ingrowth with prominent vascular and mesenchymal components Osteoinduction: Mesenchymal tissue differentiation into tissue capable of osteogenesis Osteogenesis: Bone formation

• • •

Rib Iliac Fibula

• • • • • •

Intercalary defects Composite defects Mandible reconstruction Tumor resection Conventional failure Congenital pseudarthrosis

•

Vascularized Bone Autograft

Indications: Vascularized Autograft

Normal Autograft Healing: Cancellous

Osseous union 1-1.5 years: allografts successful in this pattern 70-80%

Normal Autograft Healing: Cortical

necrotic

osteoclastic

Joint Replacement and Bone Grafts

706

Musculoskeletal Radiology

Vascularized Bone Autograft Characteristics

Fibula

Rib

Iliac Crest

Bone Length (Max) Shape Structure

22-26 cm Straight Cortico-Cancellous

30 cm Curved Membranous

10 cm Slight Curve Cortico-Cancellous

Vessels Artery

Peroneal

Vein

2 Venae Comitnantes

Vascular Stalk

1.0-7.0 cm

Posterior Intercostal Deep circumflex-iliac or Superficial circumflex iliac 1 Intercostal 1 Vena comitante

3.0-5.0 cm

1.5-5.0 cm

•

Vascularized Bone Autograft Advantages ➢ Graft remains viable ➢ Promotes healing ➢ Participates in osteogenesis ➢ Improved strength • Disadvantages ➢ Microvascular surgery expertise required ➢ Increased time for surgery ➢ Two surgical sites • • •

Insufficient volume Postoperative morbidity risk Inability to mold for function

• • •

Particulate Intercalary Osteoarticular

Autograft Limitations

Allograft

Allograft Healing Depends on the Recognized Immunologic Disparity • • • •

Trauma 15–45 years of age No history neoplasm or infection No steroids or respirator Freeze or freeze-drying

• • •

Osteochondral shell Half-joint Whole joint

Allograft Source and Pretreatment

Osteoarticular Allograft

Musculoskeletal Radiology

707

Joint Replacement and Bone Grafts

Normal Allograft Healing Histology

Radiographic Appearance

Prominent vascular and granulation tissue ingrowth Short initial osteoclastic phase (several Graft resorption with ill-defined and weeks) irregular margins Improved osteoinduction and osteogenesis owing to osteoprogenitor cells improved survival (endosteum and marrow elements) Further healing

Further gradual decrease in graft density and volume (first 1 month) until bone production exceeds resorption Gradual hematopoietic tissue ingrowth w/graft density increasing to normal, loss of margin between native bone and graft w/trabecular continuity and formation of cortex (2-6 months)

Initial increase in strength due to osteoblastic activity

• • •

Low ratio bone: cartilage requires less pretreatment Cartilage immuno-privileged tissue Success depends on osseous component

• • • • •

Infection Nonunion or pseudarthrosis Fracture Graft resorption Joint instability

• • • • •

Persistent tissue swelling Periosteal reaction Progressive bone destruction Lucency about fixation and failure Indium WBC scan may add specificity

Osteoarticular Allograft

Bone Graft Complications

Infection of Bone Graft

Figure 4-1-20

Infection of Bone Graft •

[Figure 4-1-20].

Autograft ➢ Clinical evidence usually present • Vascularized Autograft ➢ 5% • Allograft ➢ 5–13% soft tissue swelling (beyond 6 months post-op) ➢ Increasing bone resorption (beyond 10 weeks post-op) • • • • •

Nonunion and Pseudarthrosis Persistent lucency graft/host junction Sclerosis at margins Rounded osseous margins Fracture or loosening of adjacent fixation Stress views helpful

Joint Replacement and Bone Grafts

Allograft infection with progressive lack of osseous bridging (arrowheads) and ultimately loosening and fracture of the fixation device (arrow) on three sequential radiographs

708

Musculoskeletal Radiology

•

Nonunion and Pseudarthrosis Autograft ➢ Failure to heal by 12 months ➢ 14% in segmental cortical bone grafts • Vascularized Autograft ➢ 7% questionable graft viability ➢ Bone scan – 1 week • Allograft ➢ 11% preventable with adequate osseous contact at host/graft junction ➢ Treated with regrafting and/or fixation change • • •

Fracture Linear lucency through graft Callus Stress views helpful

•

Fracture Autograft ➢ Not infrequent ➢ Often after healing with stress (6–8 months) ➢ More common in longer grafts • Vascularized Autograft ➢ Decreased incidence 3.5% due to improved strength • Allograft ➢ 16.5% most at weak points ➢ Affected by pretreatment method • • • •

Bone Graft Resorption Progressive graft loss Graft decreases in size Graft decreases in density Difficult to distinguish from infection

•

Bone Graft Resorption Autograft ➢ Unopposed osteoclastic activity • Vascularized Autograft ➢ Same as autograft • Allograft ➢ Acute or chronic rejection ➢ Graft replaced by fibrous tissue • • • •

2.9%–5.5 % incidence Causes: articular incongruity; lack of innervation and cartilage viability AVN, neuropathic joint or rejection Can be difficult to distinguish from infection or rejection

• • • • •

Joint narrowing and sclerosis Osteophytes and subchondral cysts Fragmentation with debris Fracture and migration of fixation Weight bearing views helpful

• • • • •

Supply-demand limitations of other graft Calf and ox-bone Treated to prevent rejection Used as spacer prevents soft tissue ingrowth Other graft material in combination

Osteochondral Allograft: Joint Instability

Joint Instability: Radiographic Findings

Xenograft

Musculoskeletal Radiology

709

Joint Replacement and Bone Grafts

• • • •

Hydroxyapatite (Ca10 [PO4] 6 [(OH) 2]) Tricalcium phosphate (Ca3 [Po4] 2) Dense or porous ceramics Osteoconductive but not osteoinductive

• • • • • • • •

Goniopora-cancellous bone Porites-cortical bone Approved human studies 1982 Initially weak mechanically Strength increases after incorporation More dense than native bone Lucent peripheral band obliterated with ingrowth Complications: Fractures, Implant failure, Infection

Bone Graft Substitutes Figure 4-1-21

Porous Ceramics

•

Spinal Bone Graft Assessment Causes of Failed Back Surgery Syndrome (FBSS) ➢ Recurrent disk ➢ Arachnoiditis ➢ Epidural scar ➢ Infection ➢ Facet subluxation ➢ Spinal stenosis ➢ Pseudarthrosis ➢ Spine Bone Autograft: Normal Healing/Incorporation ➢ Cervical - 3-4 months ➢ Lumbar - by 9 months

• •

Cervical – 3–4 months Lumbar – by 9 months

• • •

Cervical and lumbar spine Rib, iliac or fibular graft Initial discrete graft-host junction obliterated

Pseudoarthrosis of posterolateral lumbar spine graft with horizontal radiolucent clefts (arrows) and surrounding sclerosis on conventional tomography

Figure 4-1-22

Spine Bone Autografts: Normal Healing/Incorporation

Anterior Vertebral Interbody Fusion

Radiologic Evaluation: Spine Pseudarthosis •

[Figures 4-1-21 and 4-1-22]

Radiographs ➢ Oblique views best; Radiolucent defect; Motion with bending increase spine curve • Bone Scintigraphy ➢ Wide range specificity and sensitivity Normal uptake <6 months post-op ➢ Abnormal if increased uptake beyond 6 months post-op; Improved with SPECT imaging; Asymptomatic patients may have focal uptake • Conventional Tomography ➢ AP optimal plane (2–5 mm) best reported method – 96% polydirectional best; Limited availability and technically demanding • • • •

Interbody Fusion Complications Nonunion Graft fracture Extrusion of graft fragments Infection

References Bone Graft 1. Murphey MD, Sartoris DJ, Bramble JM: "Radiographic Assessment of Bone Grafts" In: Bone Grafts from Basic Science to Clinical Application, Habal MB, Reddi AH, Editors. Philadelphia: W. B. Saunders, 1992, p. 9-36.

Joint Replacement and Bone Grafts

710

Sagittal T1 (upper image) and T2-weighted (lower image) MR images showing horizontal clefts (arrowheads) in posterolateral lumbar bone graft with high signal on long TR image representing two sites of pseudoarthrosis

Musculoskeletal Radiology

Musculoskeletal Manifestations of Chronic Renal Insufficiency Mark D. Murphey, MD • • •

Primary Secondary Tertiary

• • • • •

Adenoma 80%–90% Hyperplasia 10%–15% Carcinoma 2%–4% Nonparathyroid tumor MEN Syndromes

•

Autonomous gland function

• • • • • • •

Multiple Causes Most common cause glomerulonephritis 0.01% U.S. population 85,000 hemodialysis patients/year 8,000 renal transplantations/year Involves all organ systems Musculoskeletal manifestations common and increasingly recognized

• • • •

Inability of renal excretion of phosphate Resultant hyperphosphatemia Hyperplasia of parathyroid chief cells and increased parathormone (PTH) Also reduced degradation of PTH

• • • •

Development of osteoclasts, osteoblasts, osteocytes Osseous resorption Brown tumors Periosteal reaction

• • • • •

Secondary hyperparathyroidism Osteosclerosis Osteoporosis Osteomalacia Soft tissue and vascular calcification

• • • • • • • •

Caused by osteoclastic activity 10% early stages; 50%–70% long-standing disease Subperiosteal Cortical Subchondral Trabecular Endosteal Subligamentous/Subtendinous

Hyperparathyroidism (HPT)

Primary Hyperparathyroidism: Etiology

Tertiary Hyperparathyroidism: Etiology End-Stage Renal Disease: Secondary Hyperparathyroidism

Secondary Hyperparathyroidism: Etiology

Effects of PTH on Bone

Renal Osteodystrophy

Bone Resorption in Chronic Renal Insufficiency (CRI)

Musculoskeletal Radiology

711

Musculoskeletal Manifestations of Chronic Renal Insufficiency

Subperiosteal Resorption • • •

Figure 4-2-1

[Figures 4-2-1 to 4-2-3]

• •

Initially described by Camp and Ochsner Pathognomonic-hyperparathyroidism Lacelike irregularity of cortical margin progress to scalloping and spiculation Earliest involvement middle phalanges/ terminal tufts-hand Additional sites-upper medial tibia, femur and humerus, ribs, lamina dura

Figure 4-2-2

Subperiosteal resorption involving the middle, proximal and terminal phalanges (arrows) resulting from secondary hyperparathyroidism and renal failure. Similar features are noted on the clinical photograph as well as clubbing

Figure 4-2-3

Figure 4-2-4

Subperiosteal resorption involving the middle and terminal phalanges (solid arrows) resulting from secondary hyperparathyroidism and renal failure. The terminal phalanx also shows band-like acroosteolysis (open arrow) resulting from subperiosteal resorption

Subperiosteal resorption involving the lamina dura (arrows) caused by secondary hyperparathyroidism and renal failure

Cortical Resorption • •

[Figure 4-2-4]

•

Caused by osteoclastic activity within haversian canal Radiographs-intracortical tunneling with increased lucent striations in cortex Nonspecific finding

Intracortical resorption with areas of intracortical tunneling (arrows) resulting from hyperparathyroidism on radiography and matched histologic macrosection showing resorption along preexisting Haversian canals (arrowheads)

Endosteal Resorption • • •

[Figure 4-2-5]

Causes scalloping of endosteum- hands Osteopenia with loss of trabecular sharpness Calvarium – “salt and pepper” appearance with loss of distinction of tables

Figure 4-2-5

Endosteal resorption in the skull on CT causing loss of distinction of inner and outer table cortices (salt and pepper appearance-arrowheads) in secondary hyperparathyroidism Musculoskeletal Manifestations of Chronic Renal Insufficiency

712

Musculoskeletal Radiology

Figure 4-2-6

Figure 4-2-7

Subchondral resorption of the distal clavicle and acromion (arrows and arrowhead) from secondary hyperparathyroidism on radiography

Subchondral resorption of the metacarpal heads (arrows) from secondary hyperparathyroidism on radiography simulating an erosive arthropathy

Figure 4-2-8

Subchondral Resorption • •

[Figures 4-2-6 to 4-2-8]

• • • • • •

Common in appendicular and axial skeleton Often in hands-single DIP joint (4th or 5th) also MCP and PIP joints More recently polyarticular involvement 40% of patients on long-term hemodialysis IP and first CMC joints with symmetry Simulates erosions, often progress, 50% symptomatic Other frequent sites-distal clavicle, AC joint (20%), SI joint, SC joint, symphysis pubis, posterior patella Pathologically-collapsed cortical bone and overlying cartilage Initiate an osteogenic synovitis Accentuated by mechanical stress, joint incongruity and intraarticular debris

Brown tumor (*), diffuse sclerosis and subchondral resorption (arrows) about the SI joints are seen on this CT of the pelvis in a patient with chronic renal failure

Subligamentous/Subtendinous Resorption • • •

[Figure 4-2-9]

•

Patients usually asymptomatic Radiographs-smooth and scalloped or irregular Common sites ➢ Inferior calcaneus ➢ Greater and lesser trochanters ➢ Anterior inferior iliac spine ➢ Humeral greater tuberosity ➢ Ischial tuberosity ➢ Elbow

Figure 4-2-9

Brown Tumors (Osteoclastomas) •

Caused by localized bone replacement by vascularized fibrous tissue May become cystic after necrosis and liquefaction (osteitis fibrosa cystica) higher incidence in primary hyperparathyroidism; 1.5%–1.7% in secondary Subligamentous/subtendinosis resorption in the pelvis on radiography at the ischial tuberosity and anterior inferior iliac spine (arrowheads) resulting from secondary hyperparathyroidism and renal failure

Musculoskeletal Radiology

713

Musculoskeletal Manifestations of Chronic Renal Insufficiency

Brown Tumors: Radiographic Findings • • • • •

Figure 4-2-10

[Figures 4-2-8 and 4-2-10]

• • • • • •

Often solitary; may be multiple Well defined lytic lesions Frequently eccentric or cortical (long bones) May cause scalloping and bone expansion Sites-ribs, pelvis, facial bones and femora, axial skeleton can be involved May heal after treatment with calcification, sclerosis and lesion disappearance

Periosteal New Bone: Formation Caused by osteoblastic activity Prevalence 8%–25% often with severe disease Linear often with radiolucent zone separating it from cortex Can be laminated and chronically thicken cortex Most common-humeri, femora, tibiae, radii, ulnae, metacarpals, metatarsals and phalanges

Osteosclerosis • • • • • • •

[Figure 4-2-11]

• •

Brown tumor of hyperparathyroidism involving the tibia

Cause unknown with pathologic fracture (arrows) on radiography and coronal macrosection. Cyst formation (*) is seen on 9%–34% the macrosection Predilection for axial skeleton “Rugger Jersey” spine Figure 4-2-11 Other sites-pelvis, ribs and clavicles Metaphyses and epiphyses can be involved After renal transplant osteosclerosis may regress but more common to further increase

Osteopenia • • • • • •

Accumulated effect of osteomalacia, bone resorption and osteoporosis Contributory factors-acidosis, poor nutrition, azotemia, steroids, hyperparathyroidism, and reduced vitamin D After renal transplant osteopenia may worsen or bone mineral content may increase Predisposed to fractures (5%–25%): vertebral body, pubic rami and ribs Fracture healing-normal but delayed

Osteomalacia

• •

Decreased active form of vitamin D Renal tissue hydroxylates vitamin D to active form Additional factors-hypocalcemia, inhibitors to calcification in uremia, aluminum toxicity, hepatic dysfunction

Rickets •

Common in children with chronic renal insufficiency Normal vessels that invade zone of provisional calcification fail to develop Result-disorganized cartilage zone columns

Diffuse sclerosis of the cervical spine on radiography in a patient with secondary hyperparathyroidism

Figure 4-2-12

Radiographic Findings in Rickets • •

[Figure 4-2-12]

• • • • • • •

Widened growth plate Epiphyseal irregularity; metaphyseal fraying and cupping Delayed bone age and osteopenia Bowed long bones and scoliosis Concave vertebral endplates Basilar invagination Triradiate pelvis and acetabuli protrusio Rachitic rosary Slipped epiphyses

Musculoskeletal Manifestations of Chronic Renal Insufficiency

Rickets in the distal femur resulting from renal failure on radiography and coronal macrosection with metaphyseal widening and cupping (arrows) caused by growth plate disorganization (arrowheads)

714

Musculoskeletal Radiology

• •

Slipped Epiphyses in CRI Induced Rickets • •

Figure 4-2-13

Not uncommon -10% Proximal femur and humerus, distal femur and radius, metacarpal and metatarsal heads Greatest risk-adolescent boys, uremia > 2 years, treatment close to onset of puberty Usually bilateral in chronic renal insufficiency (CRI) and often asymptomatic initially

Radiographic Findings: SCFE in CRI • • • •

[Figure 4-2-13]

Medial femur subperiosteal resorption Increase epiphyseal plate width Decrease neck-shaft angle Typical findings of slipped capital femoral epiphysis (SCFE)

Radiographic Findings: Osteomalacia in Adults •

Osteopenia with ill defined trabeculae unlike osteoporosis • Looser zones-pseudofractures ➢ Uncommon - 1% CRI patients ➢ Pubic rami, medial femoral neck, scapulae, ribs, long bones, lesser trochanters, ischial tuberosity • •

Rickets of the proximal femora and bilateral slipped capital femoral epiphyses (arrowheads) in a renal failure patient on pelvic radiograph with hips abducted

Figure 4-2-14

Soft Tissue and Vascular Calcification •

Increases calcium - phosphate product > 75mg/dL Contributory factor - local tissue damage and alkalosis (calcium salt precipitation) Common sites - ocular tissue, arteries, subcutaneous, periarticular and visceral

Periarticular Calcification • •

[Figures 4-2-14 and 4-2-15]

• • • • • •

Asymptomatic or pain and joint limitation Prevalence ➢ 7% after 1 year hemodialysis ➢ 55% after more than 4 years hemodialysis Often regresses with treatment Often multifocal and symmetric Dense and cloudlike on radiographs Hydroxyapatite - chalky paste-like material Can extend into tenosynovial tissue and joints Sites - phalangeal joints, wrists, elbows, shoulders, hips, knees, ankles

Figure 4-2-15

Heterogeneous large soft tissue mass (*) about the right shoulder on coronal STIR MR images in a patient with renal failure initially sent to radiology for biopsy of suspected sarcoma. Subsequent radiograph shows subtle calcification and vascular catheters in the central vessels for hemodialysis. Diagnosis of periarticular calcification related to renal failure and no biopsy was necessary Periarticular calcification about the left hip on CT with several calcium fluid levels (arrows) in a patient with renal failure Musculoskeletal Radiology

715

Musculoskeletal Manifestations of Chronic Renal Insufficiency

• • • • •

Arterial Calcification Occur in media and intimal tissue Pipestem appearance radiographically Can make shunts or fistula for hemodialysis difficult Initially - dorsalis pedis artery also leg, hand and forearm Prevalence – 27% < 1 year therapy ➢ 83% with 8 years or more of therapy ➢ Rare in children • Second type - nodular with luminal encroachment, obstruction with ulceration, gangrene and cardiac failure • Calcification in shunt aneurysms • • • • •

Usually not apparent on radiographs May detect on bone scintigraphy (poor looking bone scan) Sites - heart, lungs, stomach, kidneys Prevalence - 79% In myocardial tissue important, can cause conduction defects and death

• • • • • •

Aluminum toxicity Amyloidosis Tendon rupture Crystal deposition Infection Avascular necrosis (AVN)

Visceral Calcification

Musculoskeletal Abnormalities More Common After Treatment

• •

Aluminum Toxicity • • • • •

Prevalence 1%–30% (rare today) Results in osteomalacia previously responsible for most osseous abnormalities in patients on long-term hemodialysis Unknown mechanism Clinically - low PTH, serum aluminum > 100ng/mL Cause - ingestion of aluminum salts in phosphate - binding antacids to control hyperphosphatemia Cannot excrete alumina Toxic effects ➢ Cerebral (Encephalopathy) ➢ Osseous system

Aluminum Toxicity: Radiographic Findings • •

[Figure 4-2-16]

• • • • • • •

Osteomalacia - osteopenia, looser zones, fractures, rickets More than 3 atraumatic fractures (86%) - ribs, vertebrae, hips, pelvis, sternum, clavicles, extremities AVN Lack of osteosclerosis Limited subperiosteal resorption Bone biopsy - histochemical stain for aluminum

Figure 4-2-16

Amyloidosis

•

•

Secondary due to chronic disease B2 – microglobulin Areas of deposition – bone, tenosynovium, intervertebral disk, cartilage, capsule, ligament, muscle Stains with Congo red, characteristic under polarized microscopy and immunoperoxidase methods

Amyloidosis Musculoskeletal Involvement ➢ Carpal tunnel syndrome ➢ Osseous and intraarticular deposition ➢ Destructive spondyloarthropathy

Musculoskeletal Manifestations of Chronic Renal Insufficiency

Osteomalacia due to aluminum toxicity in a renal failure patient with radiograph showing multiple nontraumatic fractures (arrows) and acetabulae protrusio (curved arrows) 716

Musculoskeletal Radiology

• • • • •

Carpal Tunnel Syndrome in CRI

Figure 4-2-17

Long-term hemodialysis prevalence 2%–31 % Equal sex distribution No prevalence for dominant hand Amyloid primary cause of median nerve compression Other causes - venous stasis and edema related to treatment fistula

Osseous and Intraarticular Involvement in CRI •

[Figures 4-2-17 and 4-2-18]

• • • •

Focal lytic areas or less well defined intramedullary lytic lesions Most common - carpus - scaphoid, lunate and capitate - can enlarge Other sites - humeral head, knee (patella) and about hip Endosteal scalloping, fractures, extrinsic erosion from soft tissue mass, neuropathic appearance Intraarticular deposition - common in hip, knee, shoulder-low intensity on T2W images

Amyloidosis in a patient with chronic renal insufficiency causing multiple punched out lytic lesions in the wrist (arrowheads)

Figure 4-2-18

Spontaneous Hemorrhage Associated with CRI • • • • •

In hemodialysis patients likely related to heparin Most frequent in thigh MRI best for evaluation; appearance varies with hemorrhage age

Destructive Spondyloarthropathy • • • • • •

Described 1984 by Kuntz and colleagues Usually in patients on long-term hemodialysis (2–19 years) Prevalence - 15%; symptoms - pain Cervical and lumbar spine Multiple levels >50% of patients Rapid progression 33%; simulates infection Initial postulated etiology - crystal and noncrystal deposition, neuropathic and hyperparathyroidism Amyloid now considered offending agent

Amyloid deposition in the hip joint of a renal failure patient on hemodialysis on coronal T1 and T2-weighted MR images showing low signal intensity material in the joint (*) with extrinsic bone erosion (arrow)

Figure 4-2-19

Destructive Spondyloarthropathy: Radiographic Findings • • • • •

[Figure 4-2-19]

Discovertebral erosions with sclerosis Vertebral body compression Disk space narrowing with Schmorl nodes Lack of osteophytes Facet involvement with subluxation

Destructive spondyloarthropathy at L5-S1 caused by amyloid deposition related to renal failure with disc narrowing and destruction (arrows) on radiograph simulating infectious spondylodiskitis

Musculoskeletal Radiology

717

Musculoskeletal Manifestations of Chronic Renal Insufficiency

Distinction of Infection vs. Destructive Spondyloarthropathy • • • •

[Figure 4-2-20]

•

Clinical symptoms / laboratory evidence lacking Multilevel involvement unusual for bacterial infection T1 Limited uptake on scintigraphy CT - lack of paravertebral soft tissue mass (also MRI) MRI - disc / endplate marrow replaced T1W No prominent increased intensity on T2W

Figure 4-2-20 T2

• • •

Tendon Rupture or Avulsion in CRI Spontaneous; in patients on long- term dialysis Solitary or multiple Tendon sites - quadriceps, patellar, triceps, flexors and extensors of fingers • Cause - PTH excess - increased joint laxity ➢ Tendon calcification ➢ Chronic acidosis • Result - decrease tendon tensile strength and accelerated degeneration

Destructive spondyloarthropathy related to amyloid deposition (same patient as previous radiograph) with marrow and disk replacement remaining predominantly low signal intensity on all pulse sequences (*)

Radiologic Findings in Tendon Rupture in CRI • • •

[Figure 4-2-21]

• • • •

Before rupture - may see subtendonous bone resorption After rupture - focal soft tissue swelling, effusion, subluxation CT, MRI or sonography to evaluate tendon integrity and disruption site

Crystal Deposition Disease in CRI

• • • • • • •

Figure 4-2-21

Calcium hydroxyapatite, CPPD, monosodium urate, calcium oxalate Hemodialysis elbow - olecranon bursitis Calcium hydroxyapatite - EM or X-ray diffraction CPPD - chondrocalcinosis not common - knee, wrist, hip, shoulders and symphysis CPPD arthropathy rare in CRI Gout - infrequent in CRI, radiographic findings same as those in primary podagra except distribution Oxalosis - chondrocalcinosis, calcified joints, disks and periarticular, diffuse osseous sclerosis

Predisposition to Infection in CRI

• • •

Depressed host responsiveness Steroids and immunosuppressive treatment Entry site via arteriovenous fistula for hemodialysis Secondary infection in osteonecrosis

Infection Associated with CRI

•

Spontaneous rupture of the quadriceps Osteomyelitis and septic arthritis tendon (arrows) in a renal failure patient on Bacterial or fungal sagittal T2-weighted MR image Radiographic findings - deep soft tissue swelling, periosteal reaction, bone destruction, and joint space narrowing - same as other situations Unusual syndrome - progressive peripheral ischemic ulcers usually after treatment and secondary infection

• • •

Bone Scintigraphy in CRI Diffuse increased activity “Super bone scan” May use as index of severity Cause of increased activity is combination of vitamin D deficiency and hyperparathyroidism ➢ Increase rate bone turnover and collagen metabolism ➢ Excess immature collagen ➢ High enzyme activity ➢ Increased osseous surface area for binding • Increased focal or diffuse soft tissue uptake

Musculoskeletal Manifestations of Chronic Renal Insufficiency

718

Musculoskeletal Radiology

• •

Avascular Necrosis in CRI • • • • •

Up to 40% of CRI patients after renal transplantation Additional factors - structural weakening, fracture and collapse, excess PTH, graft-host reaction Most common site - femoral head Other sites - humeral head, about knee, talar dome, humeral condyles, cuboid, carpal bones, long bone diaphyses

Avascular Necrosis in CRI Radiologic appearance identical to other causes AVN MRI most sensitive however only 50% show early changes Osseous malignancy can complicate osteonecrosis and general increased malignancy rate after renal transplant

Hyperparathyroidism: Primary vs. Secondary • • • •

Brown Tumors Osteosclerosis Chondrocalcinosis Periostitis

1

2

+++ Rare + Rare

++ +++ Rare +

References 1. 2. 3. 4.

Camacho CR, Talegon Melendez A, Valenzuela A, Gonzalez Guirao MA, Gomez Benitez S, Gil L, palma Alvarez A, Mateos Aguilar J. Radiological findings of amyloid arthropathy in long-term haemodialysis. European Radiology. 1992; 2:305-309. Leone A, Sundaram M, Cerase A, Magnavita N, Tazza L, Marano P.. Destructive spondyloarthropathy of the cervical spine in long-term hemodialyzed patients: a five-year clinical radiological prospective study. Skeletal Radiol. 2001 Aug;30(8):431-41. Murphey MD, Sartoris DJ, Quale JL, Pathria MN, Martin NL. Musculoskeletal manifestations of chronic renal insufficiency. Radiographics. 1993 Mar;13(2):357-79. Slavotinek JP, Coates PT, McDonald SP, Disney AP, Sage MR.. Shoulder appearances at MR imaging in long-term dialysis recipients. Radiology. 2000 Nov;217(2):539-43.

Musculoskeletal Radiology

719

Musculoskeletal Manifestations of Chronic Renal Insufficiency

Fundamental Concepts of Musculoskeletal Neoplasm: Radiographs Mark D. Murphey, MD Tumors are classified by their pattern of differentiation Tumors are graded on their degree of anaplasia Skeletal Components (Derived from Embryonal Mesenchyme) • • • • • • • • • • • •

Bone and cartilage progenitor cells Periosteal cells Hematopoietic cells Lipocytes Nerve and Schwann cells Fibroblasts Osteoclasts and Osteoclast-like cells Endothelial cells Perithelial cells Notochordal cells (rests) Histiocytic cells Epithelial cells (rests)

HISTOGENIC CLASSIFICATION OSTEOID BONE TUMORS BENIGN

MALIGNANT

ENOSTOSIS

OSTEOSARCOMA

OSTEOID OSTEOMA OSTEOMA OSTEOBLASTOMA

HISTOGENIC CLASSIFICATION CARTILAGE BONE TUMORS BENIGN CHONDROBLASTOMA

MALIGNANT CHONDROSARCOMA

CHONDROMYXOID FIBROMA ENCHONDROMA JUXTACORTICAL CHONDROMA OSTEOCHONDROMA

HISTOGENIC CLASSIFICATION MARROW BONE TUMORS BENIGN LIPOMA

MALIGNANT LIPOSARCOMA LYMPHOMA MYELOMA/PLASMACYTOMA

Fundamental Concepts of MSK Neoplasm: Radiographs

720

Musculoskeletal Radiology

HISTOGENIC CLASSIFICATION FIBROUS BONE TUMORS BENIGN

MALIGNANT

DESMOPLASTIC FIBROMA

FIBROSARCOMA

HISTOGENIC CLASSIFICATION HISTIOCYTIC TUMORS BENIGN

MALIGNANT

EOSINOPHILIC GRANULOMA

MALIGNANT FIBROUS HISTIOCYTOMA

HISTOGENIC CLASSIFICATION NOTOCHORD BONE TUMORS BENIGN

MALIGNANT

CHORDOMA (HISTOLOGICALLY BENIGN)

CHORDOMA

HISTOGENIC CLASSIFICATION VASCULAR TUMORS BENIGN

LOW GRADE MALIGNANT

GLOMUS

HEMANGIOENDOTHELIOMA

HEMANGIOMA

HEMANGIOPERICYTOMA

MALIGNANT ANGIOSARCOMA

LYMPHANGIOMA

HISTOGENIC CLASSIFICATION UNKNOWN ORIGIN TUMORS BENIGN

MALIGNANT

GIANT CELL TUMOR

MALIGNANT GIANT CELL TUMOR MALIGNANT FIBROUS HISTIOCYTOMA EWING SARCOMA ADAMANTINOMA

Musculoskeletal Radiology

721

Fundamental Concepts of MSK Neoplasm: Radiographs

Incidence of Bone Tumors •

[Figure 4-3-1]

•

Approximately 1 individual in 75000 develops a primary bone tumor that leads to biopsy About 4000 new cases per year

Figure 4-3-1

Figure 4-3-2

Incidence of Bone Tumors •

[Figures 4-3-2 to 4-3-4]

•

Of biopsied primary bone tumors: malignant tumors are three times more common as benign lesions Metastatic lesions are biopsied about 35 times more frequently than primary tumors

Primary Benign Bone Tumors [Figure 4-3-3]

Primary Malignant Bone Tumors [Figure 4-3-4]

Figure 4-3-3

Figure 4-3-4

5 15 5

Fundamental Concepts of MSK Neoplasm: Radiographs

722

Musculoskeletal Radiology

Important Factors in the Diagnosis of Bone Tumors • • • • • •

[Figures 4-3-5 and 4-3-6]

Patient age and sex Bone involved Location in bone Lesion margin Matrix formation Periosteal reaction

These radiologic characteristics reflect the pathologic process and its biologic activity.

Primary Benign Bone Tumors: Age Distribution by Decade [Figure 4-3-5]

Figure 4-3-5

Primary Malignant Bone Tumors: Age Distribution by Decade [Figure 4-3-6]

Musculoskeletal Radiology

Figure 4-3-6

723

Fundamental Concepts of MSK Neoplasm: Radiographs

“The site frequency, peak age of incidence, and numerical frequency of bone tumor indicate that they are not completely autonomous, but are subject to the laws of field behavior and developmental anatomy of normal bone...” Figure 4-3-7 Johnson L. 1953

Location in Bone: Longitudinal • • •

[Figures 4-3-7 and 4-3-8]

Epiphysis Metaphysis Diaphysis

Figure 4-3-8

Chondroblastoma with lytic lesion in the epiphysis

Ewing sarcoma involving the femoral diaphysis on radiograph, T1-weighted MR and gross specimen

Location in Bone: Axial • • • • •

[Figures 4-3-9 to 4-3-10]

Central Eccentric Cortical Juxtacortical Soft Tissue

Figure 4-3-10

Figure 4-3-9

Fundamental Concepts of MSK Neoplasm: Radiographs

724

Musculoskeletal Radiology

Pattern of Bone Destruction and Lesion Margin •

Figure 4-3-11

[Figure 4-3-11]

Type I: Geographic ➢ A: Well-defined, sclerosis ➢ B: Well-defined, no sclerosis ➢ C: Ill-defined • Type II: Motheaten • Type III: Permeative • Transition Zone

Margin Reflects Biologic Activity Figure 4-3-12

Aggressive versus Nonaggressive Biologic Activity Margin Geographic IA Geographic IB Geographic IC Motheaten Permeative

Growth Rate Slow Slow to Intermediate Intermediate Intermediate Fast

1A Margin • • •

[Figure 4-3-12]

Geographic Well-Defined Sclerosis Geographic 1A

Geographic 1A: Differential Diagnosis

Figure 4-3-13

• • •

[Figure 4-3-13]

Bone cyst Brodie abscess [Figure 4-3-14] Cartilage lesions ➢ Chondroblastoma ➢ Chondromyxoid Fibroma ➢ Enchondroma • Fibroxanthoma • Fibrous Dysplasia

Nonossifying Fibroma /Fibroxanthoma with geographic 1A margin on radiograph, gross specimen and macrosection

Figure 4-3-14

Brodie abscess with geographic 1A margin. Note the channel like extension (arrow) representing a sinus tract inferiorly on the conventional tomogram (right image) Musculoskeletal Radiology

725

Fundamental Concepts of MSK Neoplasm: Radiographs

1B Margin • • •

Figure 4-3-15

[Figure 4-3-15]

Geographic Well-Defined No Sclerosis

• • •

Geographic IB: Differential Diagnosis Giant Cell Tumor [Figure 4-3-16] Bone Cyst Cartilage Lesions ➢ Chondroblastoma ➢ Chondromyxoid Fibroma ➢ Enchondroma • Fibrous Dysplasia • Myeloma/Metastasis

Figure 4-3-16 Geographic 1B

Figure 4-3-17

Giant cell tumor of the distal radius with geographic 1B margin on radiograph and macrosection extending to subchondral bone

1C Margin • •

Geographic Ill-Defined

• • • • • •

Chondrosarcoma Enchondroma (Active) MFH/Fibrosarcoma [Figure 4-3-18] Giant Cell Tumor Osteosarcoma Metastasis/Myeloma

• •

Motheaten [Figure 4-3-19] Permeative[Figures 4-3-20 and 4-3-21]

[Figure 4-3-17] Geographic 1C

Figure 4-3-18

Geographic IC: Differential Diagnosis

Tumor Margin

Fibrosarcoma

Fundamental Concepts of MSK Neoplasm: Radiographs

726

Musculoskeletal Radiology

Figure 4-3-19

Figure 4-3-20

Motheaten

• • • • • • •

Ewing sarcoma Round cell tumors Malignant fibrous histiocytoma/Fibrosarcoma Osteomyelitis Osteosarcoma Langerhans cell histiocytosis (LCH) Metastasis/Myeloma

• • • • • • • •

Ewing sarcoma Round cell tumors Malignant fibrous histiocytoma/Fibrosarcoma Metabolic disorders [Figure 4-3-22] Osteomyelitis (acute) [Figure 4-3-23] Osteosarcoma LCH Myeloma/Metastasis

Figure 4-3-21

Permeative

Motheaten: Differential Diagnosis

Permeative: Differential Diagnosis

Permeative

Figure 4-3-22

Figure 4-3-23

Hyperparathyroidism with variation of the permeative pattern of bone lysis in the cortex on radiograph and macrosection (multiple areas of resorption along Haversion canals – arrows)

Multifocal acute bacterial osteomyelitis with motheaten to permeative destructive pattern of bone lysis on radiographs involving the tibia and femur. Macrosection (right image shows pus on either side of cortex-arrows) Musculoskeletal Radiology

727

Fundamental Concepts of MSK Neoplasm: Radiographs

Lytic Patterns [Figure 4-3-24]

Figure 4-3-24

Invisible Margin [Figure 4-3-25: Lymphoma]

Figure 4-3-25

Lymphoma (same patient) with extensive marrow replacement (*) on T1 weighted MR, not seen on radiograph images.

Fundamental Concepts of MSK Neoplasm: Radiographs

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Musculoskeletal Radiology

•

Changing Margin [Figure 4-3-26] Increased Biologic Activity

Figure 4-3-26

Changing Margin

•

Matrix Formation

•

I. Mineralized ➢ Chondroid - rings, arcs, honeycomb[Figure 4-3-27] ➢ Osteoid - ivory or cloudlike

Osteonecrosis (*) with malignant transformation to malignant fibrous histiocytoma (MFH) showing new lysis (arrow) at periphery on specimen radiograph representing the changing margin

Osteonecrosis (*) with malignant transformation to MFH on CT (same patient as previous radiograph) with new cortical destruction laterally and soft tissue mass (arrow)

Coronally sectioned gross specimen and macrosection showing osteonecrosis (*) and MFH arising at periphery (arrows) (same patient as previous radiograph and CT)

[Figure 4-3-28]

II. Nonmineralized ➢ Fluid ➢ Soft Tissue ➢ Fat

Figure 4-3-27

Figure 4-3-28

Chondrosarcoma of the fibula on specimen radiograph and gross specimen show “ring and arc” matrix mineralization (arrows) Musculoskeletal Radiology

Osteosarcoma of the tibia with dense cloud-like matrix mineralization (arrows) 729

Fundamental Concepts of MSK Neoplasm: Radiographs

Periosteal Reaction: Nonaggressive • • • •

[Figure 4-3-29]

Solid (a) Buttressing (b) Expansion (c) Septation (d)

Figure 4-3-29 (a)

(b)

(c)

(d)

Fundamental Concepts of MSK Neoplasm: Radiographs

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Musculoskeletal Radiology

Periosteal Reaction: Aggressive • • • •

[Figures 4-3-30 and 4-3-31]

Codman triangle (a) Sunburst (b) Hair-On-End (c) Laminated (d)

Figure 4-3-30 (a)

(b)

(c)

(d)

Musculoskeletal Radiology

731

Fundamental Concepts of MSK Neoplasm: Radiographs

Polyostotic vs. Monostotic Holes in Bone • • • • • • • •

Langerhans Cell Histiocytosis (LCH) [Figure 4-3-32] Enchondromatosis Fibrous Dysplasia Hereditary multiple exostoses (HME) Osteomyelitis Paget disease Neurofibromatosis (type 1) Angiomatous lesions

• • •

Metastases Multiple Myeloma [Figure 4-3-33] Hemangioendothelioma

Figure 4-3-31

Polyostotic Lesions: Benign

Polyostotic Lesions: Malignant

Osteosarcoma with aggressive “hairon- end” periosteal reaction (arrows)

Figure 4-3-32

Figure 4-3-33

Langerhans cell histiocytosis with areas of calvarial lysis in the frontal and occipital areas (arrows)

Multiple myeloma on lateral skull radiograph with multiple areas of bone lysis

References 1. 2. 3. 4. 5. 6.

"General Considerations". In: Bone Tumors, ed Dorfman HD, Czerniak B, Mosby: St. Louis 1998. p. 1-33. Ghelman B. Radiology of bone tumors. Orthop Clin North Am. 1989 Jul;20(3):287312. Review. Lodwick GS, Wilson AJ, Farrell C, Virtama P, Dittrich F. Determining growth rates of focal lesions of bone from radiographs. Radiology. 1980 Mar;134(3):577-83. Madewell JE, Ragsdale BD, Sweet DE. Radiologic and pathologic analysis of solitary bone lesions. Part I: internal margins. Radiol Clin North Am. 1981 Dec;19(4):715-48. Ragsdale BD, Madewell JE, Sweet DE. Radiologic and pathologic analysis of solitary bone lesions. Part II: periosteal reactions. Radiol Clin North Am. 1981 Dec;19(4):74983. Sweet DE, Madewell JE, Ragsdale BD. Radiologic and pathologic analysis of solitary bone lesions. Part III: matrix patterns. Radiol Clin North Am. 1981 Dec;19(4):785814.

Fundamental Concepts of MSK Neoplasm: Radiographs

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Fundamental Concepts of Musculoskeletal Neoplasm: CT and MRI Mark D. Murphey, MD • •

Important Features in Evaluation of Musculoskeletal Masses

•

Differential diagnosis Preoperative assessment and staging

Osseous Neoplasm [Figure 4-5-1] •

Differential diagnosis of primary skeletal neoplasms is best determined by radiographs!! But... MRI and/or CT are vital for delineating and staging osseous neoplasms prior to surgery

Figure 4-5-1

Chondrosarcoma (same patient as previous radiograph) on MR due to associated cortical destruction and soft tissue mass (arrows) Enchondroma vs.Chondrosarcoma on radiograph due to chondroid mineralization (arrow)

• •

Soft Tissue Neoplasm •

Radiographs only occasionally helpful CT and more often MRI can be tissue-specific But... MRI and/or CT are again vital for defining extent, staging and preoperative evaluation. Clinical and radiologic characteristic often limit differential diagnosis

Causes of Tissue Specific Diagnosis on CT/MRI in Evaluating Soft Tissue Masses • • • • •

20%–50% cases Contrast resolution, MRI > CT Multiplanar imaging, MRI > CT Location of mass Growth pattern and history

Soft Tissue Masses Diagnosed with Imaging Alone • • • • •

[Figures 4-5-2 and 4-5-3]

Lipomatous lesions Angiomatous lesions Neurogenic tumors Elastofibroma and fibromatosis PVNS and ganglion

Musculoskeletal Radiology

733

Fundamental Concepts MSK Neoplasm: CT and MRI

Figure 4-5-2

T1

T2

Lipoma (coronal T1 and T2-weighted images) isointense to fat on all pulse sequences (*) with single thin septation (arrows)

Figure 4-5-3

Malignant peripheral nerve sheath tumor (arrow) in patient with neurofibromatosis type 1 (note second small subcutaneous neurofibroma- curved arrow)

•

Staging of Musculoskeletal Tumors: Benign (G-O) [Figure 4-5-4] • • •

Stage 1 – Unchanged or healing lesion; well-encapsulated; indolent clinical course Stage 2 – Active growth; symptomatic, remains intracapsular but may be deforming Stage 3 – Aggressive local growth; may penetrate cortex or compartment; higher recurrence rate

Staging of Musculoskeletal Tumors: Malignant [Figure 4-5-5] • •

Stage 1 (G1): Low Grade, well differentiated, few mitoses; tend to recur locally ➢ 1A – Intraosseous / Intracompartmental ➢ 1B – Extraosseous / Extracompartmental Stage 2 (G2) – High Grade, poorly differentiated, many mitoses; high incidence of metastases ➢ 2A – Intraosseous / Intracompartmental ➢ 2B – Extraosseous / Extracompartmental Stage 3 – Metastases; regional or remote (visceral, lymphatic or osseous)

Fundamental Concepts MSK Neoplasm: CT and MRI

734

Musculoskeletal Radiology

Figure 4-5-4 Staging of Musculoskeletal Neoplasm: Histologically Benign (G-O) STAGE

Bone

Soft Tissue

I

II

III

Musculoskeletal Radiology

735

Fundamental Concepts MSK Neoplasm: CT and MRI

Figure 4-5-5 Staging of Musculoskeletal Neoplasm: Histologically Malignant Low grade histo: IA

Low grade histo: IB

Bone

Soft Tissue

High grade histo: IIA

High grade histo: IIB

BONE

BONE

SOFT TISSUE

SOFT TISSUE Fundamental Concepts MSK Neoplasm: CT and MRI

Stage III

736

Musculoskeletal Radiology

American Joint Commission Staging Protocol for Sarcoma of Soft Tissue •

• • •

Histologic grade (G) ➢ G1 well differentiated ➢ G2 moderately well differentiated ➢ G3-4 poorly differentiated, undifferentiated Primary Tumor (T) ➢ T1 tumor 5cm or less in greatest dimension ➢ T2 tumor more than 5cm in greatest dimension Regional lymph nodes (N) ➢ N0 no regional lymph node metastasis ➢ N1 regional lymph node metastasis Distant metastasis (M) ➢ M0 no distant metastasis ➢ M1 distant metastasis

Staging of Musculoskeletal Neoplasm Has Implication on Surgical Treatment • • • • •

Figure 4-5-6

[Figures 4-5-6 and 4-5-7]

Intracapsular excision Marginal excision Wide excision Radical resection Amputation

Figure 4-5-7 LIMB SALVAGE PROCEDURES AMPUTATIONS

Musculoskeletal Radiology

737

Fundamental Concepts MSK Neoplasm: CT and MRI

Important Factors on Imaging for Staging Musculoskeletal Neoplasm • • • • • •

Figure 4-5-8

Intramedullary extent Extent of soft tissue component Lesion matrix Cortical involvement Neurovascular involvement Joint involvement

Intramedullary and Soft Tissue: Extent of Musculoskeletal Neoplasm [Figures 4-5-8 and 4-5-9] •

• • •

MRI superior to CT ➢ Superior contrast resolution ➢ Multiplanar imaging capability Regional Metastases – osseous/lymph node Can be helpful to direct biopsy ➢ Always perform in consultation with orthopod ➢ Done in institution of definitive procedure Give orthopod anatomic landmarks !

Osteosarcoma with spread across physeal plate (arrows) not seen on radiograph

Figure 4-5-9

MRI may overestimate musculoskeletal neoplasm extent because of surrounding edema (reactive zone) Musculoskeletal Neoplasm: Lesion Matrix Evaluation •

•

I. Mineralized – CT > MRI ➢ A. Chondroid – rings and arcs [Figure 4-5-10] ➢ B. Osteoid – cloudlike, ivory-like ➢ C. Other calcification – phlebolith, synovial sarcoma II. Nonmineralized – MRI > CT ➢ A. Fluid, necrosis, hemorrhage [Figure 4-5-11] ➢ B. Fat ➢ C. Soft tissue – nonspecific

T1

T2

Ewing sarcoma following chemotherapy with prominent reactive zone (*) around the low signal intensity pseudocapsule (arrows)

Figure 4-5-10

Figure 4-5-11

Chondrosarcoma with chondroid matrix mineralization not seen on radiographs or MRI

Fundamental Concepts MSK Neoplasm: CT and MRI

Aneurysmal bone cyst with fluid levels on T2-weighted MRI reflecting cystic spaces on gross specimen 738

Musculoskeletal Radiology

Musculoskeletal Neoplasm: Cortical Involvement [Figure 4-5-12] • • • • •

Figure 4-5-12

CT > MRI (my opinion) MRI=CT (literature) CT better spatial resolution Important in differential diagnosis of osseous lesions Important for surgical resection/staging

Musculoskeletal Neoplasm: Neurovascular Involvement • • • • • •

[Figures 4-5-13 and 4-5-14]

• •

Vital information for surgical resection MRI > CT (post-contrast if use CT) Improved contrast resolution Multiplanar MR images often helpful Axial plane usually best Look for intact fat plane Osteoid osteoma (arrow) and lesion was difficult to detect on MRI (right image) compared to CT (left image) ➢ Best on T1W images If fat plane lost cannot exclude involvement Soft tissue mass encase vessels – definite involvement

Figure 4-5-13 Figure 4-5-14

Osteosarcoma with displaced but nonencased neurovascular bundle (arrows)

Musculoskeletal Neoplasm: Ligament and Tendon Involvement • • • • •

Important for surgical reconstruction MRI > CT; best on T2W image Tendons/ligaments low intensity vs. tumor high signal On CT tendon/ligament similar to tumor attenuation Also multiplanar imaging of MR helpful

Figure 4-5-15

Musculoskeletal Neoplasm: Joint Involvement •

Osteosarcoma with encased neurovascular bundle (arrows) with tumor replacing normal fat seen about vessels

[Figure 4-5-15]

• • • •

Dramatically changes surgery from: ➢ Limb salvage; intraarticular resection ➢ Extraarticular limb salvage/amputation MRI superior to CT – multiplanar imaging ➢ Coronal or sagittal plane best Three routes of spread into joint ➢ Through bone/cartilage (transarticular) ➢ Around joint margin (periarticular) ➢ Along ligaments/tendons, or hematogenous Presence of joint effusion – suggestive Absence of joint effusion – excludes

Osteosarcoma invading the knee joint with effusion (arrows) and tumor (*) along ACL (arrowheads) on sagittal T2 MR and gross specimen

Musculoskeletal Radiology

739

Fundamental Concepts MSK Neoplasm: CT and MRI

Overall Delineation of Musculoskeletal Masses: All Features (56 Cases, N=189) • • •

MRI > CT – 60% MRI = CT – 16% CT > MRI – 24%

Intramedullary Extent Soft Tissue Mass Mineralized Matrix Cortical Involvement Neurovascular Involv. Joint Involvement • • •

MRI (N=56) 81% 89% 0% 7% 78% 73%

CT (N=56) 5% 0% 92% 72% 0% 3%

CT Indications Cannot perform MRI Matrix producing neoplasm not adequately evaluated on radiographs Unusual location ➢ Ribs, sternoclavicular region, scapula ➢ Abdominal/chest wall ➢ Fibula

• • • • •

Small size Well marginated Homogeneous signal intensity No neurovascular encasement Enhancement pattern dynamic MRI (late, slow, diffuse/none)

• • • • •

Large size Poor margin definition with edema Heterogeneous signal intensity Neurovascular encasement Enhancement pattern dynamic MRI (early, rapid, peripheral)

• • • • •

Totty – Radiology 1986; 160:135–141 (N=32) Sundaram – MRI 1988; 6:237–248 (N=53) Kransdorf – AJR 1989; 153:541–547 (N=112) Berquist – AJR 1990; 155:1251–1255 (N=95) Crim – Radiology 1992; 185:581–586 (N=83)

Imaging Characteristics Suggesting Benign Soft Tissue Mass

Imaging Characteristics Suggesting Malignant Soft Tissue Mass

Distinction of Benign vs. Malignant Soft Tissue Mass by MRI

Fundamental Concepts MSK Neoplasm: CT and MRI

740

Musculoskeletal Radiology

Soft Tissue Masses Misinterpreted on MRI: Benign vs. Malignant [Figures 4-5-16 to 4-5-18] • • • • • • •

Figure 4-5-16

Diabetic muscle ischemia Hematoma Fibromatosis Reactive lymph node, abscess, bursitis Myositis ossificans Synovial sarcoma Myxoid liposarcoma

General consensus is that in an individual case, MRI is not accurate enough to predict whether a nonspecific solid soft tissue mass is benign or malignant • • •

Musculoskeletal Neoplasm Follow-Up

•

Myositis ossificans with aggressive appearance on coronal T2-weighted MRI

Pre-operative-post therapy Post-operative-recurrence MRI superior to CT

Figure 4-5-17

Post Therapy Imaging • • • • •

Increasing ossification – Osteosarcoma, Ewing sarcoma – Radiographs/CT Change in size and extent – MRI Increasing peritumoral edema Tumor necrosis and hemorrhage ➢ > 90% required for a pathologic good response

Post-Operative Imaging: Radiographs

• • •

Comparison to previous studies Findings of recurrence ➢ New bone destruction ➢ New areas matrix formation

Myositis ossificans with early rim of ossification (arrow) on CT (same patient as previous MRI)

Post-Operative Imaging: MRI/CT: Normal MRI > CT – improved contrast resolution Comparison to baseline study (first 2-3 months) Recognize normal changes ➢ Post-op edema/myositis ➢ Radiation necrosis ➢ Muscle flap ➢ Fluid collections – subfascial, lymphocele/seroma

Figure 4-5-18

Post-Operative Imaging: MRI/CT: Abnormal • •

[Figures 4-5-19 and 4-5-20]

•

New bone destruction/marrow replacement Any recurrent or residual nodular region ➢ Tumor until proven otherwise ➢ Texture sign ➢ Regardless of signal characteristics unless low all sequences representing fibrosis or fluid collection Contrast studies can be helpful

Synovial sarcoma with homogeneous appearance and defined margins suggesting an indolent lesion. Intrinsically the lesion has nonspecific features of a solid mass

Figure 4-5-19

Post operative lymphocoele/seroma (*) in patient with previous MFH resection with homogeneous low (T1) and high (T2) signal intensity as expected for a fluid collection

Musculoskeletal Radiology

741

Fundamental Concepts MSK Neoplasm: CT and MRI

Musculoskeletal Neoplasm: Use of MRI with Gadolinium • •

Figure 4-5-20

[Figure 4-5-21]

• • •

• • •

Increase lesion conspicuity (usually not needed) Tumor, edema, inflammation and fibrosis all enhance Help differentiate cyst/hemorrhage Helpful in post-op cases to show nodular enhancement with recurrence Vanel/Bloem - dynamic subtraction MRI early enhancement of recurrent tumor and response to therapy

Musculoskeletal Masses: Imaging Goals Delineate precise extent of lesion Diagnosis/exclude metastases Give most likely tissue type and differential diagnosis

Recurrent MFH (arrows) adjacent to residual lymphocoele/seroma (*) (same patient as previous MRI but 2 years later). Note the tumor staining on angiogram (far right image-arrowhead) versus vessels draped about fluid collection (curved arrow)

Figure 4-5-21

Myxoid MFH with enhancing peripheral solid nodular tissue (arrows). These enhancing areas represent viable tumor regions and biopsy should be directed toward these regions as they harbor diagnostic tissue and were only detected after contrast administration

References 1. 2. 3. 4. 5. 6.

Berquist TH. Magnetic resonance imaging of musculoskeletal neoplasms. Clin Orthop Relat Res. 1989 Jul;(244):101-18. Review. Sundaram M, McGuire MH. Computed tomography or magnetic resonance for evaluating the solitary tumor or tumor-like lesion of bone? Skeletal Radiol. 1988;17(6):393-401. Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res. 1986 Mar;(204):9-24. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res. 1980 Nov-Dec;(153):106-20. Stacy SG, Mahal RS, Peabody TD. Staging of Bone Tumors: A Review with Illustrative Examples. Am. J. Roentgenol., Apr 2006; 186: 967 - 976. Murphy WA Jr. Imaging bone tumors in the 1990s. Cancer. 1991 Feb 15; 67(4 Suppl):1169-76. Review.

Fundamental Concepts MSK Neoplasm: CT and MRI

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Osteoid Lesions of Bone Mark D. Murphey, MD Figure 4-5-1 • • • • •

Osteoid Lesions of Bone Enostosis and associated conditions Osteoma Osteoid osteoma Osteoblastoma Osteosarcoma

Enostosis (Bone Island): Clinical Characteristics • • • • • • •

Stieda 1905 - “Kompakten Knochenkerne” Usually incidental finding Patient asymptomatic Common lesions-unknown frequency (0.43%–14% rib, pelvis and spine) Rare in children

Enostosis (Bone Island): Histology [Figure 4-5-1] • •

Enostosis histologically with pink cortical bone (*) and irregular thorn-like medullary margins (arrows)

Intramedullary location Composed of normal appearing compact lamellar bone with haversian canals Blends with surrounding trabecular bone creating irregular margin Likely developmental - can be considered a hamartoma

Figure 4-5-2

Enostosis (Bone Island): Radiology • • •

[Figures 4-5-2 and 4-5-3]

• • • • • • • • •

Round to oval (0.2–2.0 cm) osteoblastic area Often epiphyseal or metaphyseal Thorny, radiating spicules at margin but well defined Vast majority (>95%) no need for further radiologic evaluation following radiograph Bone scan (if needed) usually normal (may show Enostosis with thorn-like margins (arrow) in the fifth minimal activity) metacarpal head May slowly increase or decrease in size Differential diagnosis: osteoblastic metastasis, osteoma, osteoid osteoma, low grade osteosarcoma Follow-up 1, 3, 6 and 12 months Figure 4-5-3 Biopsy if increase in size too rapidly ➢ > 25% over 6 months

Giant Bone Island [Figure 4-5-4]

• •

> 2-3 cm in size Most often in pelvis More likely to have increased activity (25%) on bone scan (but usually mild; < ant. iliac crest) Most difficult to differentiate from sclerosing low grade intramedullary osteosarcoma (on histology look for entrapped host lamellar bone) Identical on histology to other bone islands

Enostosis with thorn-like margins (arrow) Musculoskeletal Radiology

743

Osteoid Lesions of Bone

• • •

Possible Diseases Related to Enostosis

Figure 4-5-4

Osteopoikilosis Osteopathia striata Melorheostosis

Osteopoikilosis (Osteopathia Disseminata) Clinical Characteristics • • • •

Autosomal dominant inheritance; asymptomatic Dermatofibrosis lenticularis disseminata Keloid formation May have mild arthralgias (15%-20%)

Osteopoikilosis: Radiology and Pathology • • • • • •

Giant bone island with irregular thorn-like margins (*)

[Figures 4-5-5 and 4-5-6]

Numerous circular or ovoid radiodensities Often symmetric - no increased activity on bone scan Predilection epiphyses and metaphyses Also carpus, tarsus, pelvis May increase or decrease in size Pathology - same as solitary enostosis

Figure 4-5-5

Osteopathia Striata (Voorhoeve Disease): Clinical Characteristics • • • • • •

Osteopoikilosis variant - 1924 Autosomal dominant? Usually asymptomatic or mild arthralgia’s Associated syndromes - Goltz syndrome, cranial sclerosis

Osteopathia Striata: Radiology [Figures 4-5-5 to 4-5-7] • •

• • • •

Linear bands of sclerosis from metaphysis in long bones Fan-like bands of sclerosis in flat bones (iliac) ➢ Could simulate heavy metal poisoning Sometimes associated with osteopoikilosis Bone scan-normal

Mixture of osteopoikilosis (circular areas of sclerosis-arrow) and osteopathia striata (linear areas of sclerosis-arrowhead)

Figure 4-5-6

Melorheostosis: Clinical Data

• •

Described 1922: Leri and Joanny Only 50% evident before age of 20 years Equal sex distribution Often symptomatic - pain, decreased range of motion (ROM), contractures; limb swelling/length discrepancy and bowing Scleroderma like skin lesions over osseous changes

Melorheostosis: Pathology • • •

Figure 4-5-7

Thickened and enlarged cortical bone Haversian canals normal with irregular arrangement Marrow space may show increased cellularity Soft tissue may contain mass of fibrous tissue with or without ossification

Mixture of osteopoikilosis (arrowhead) and osteopathia striata (arrow) showing low signal intensity on MRI

Osteopathia striata with linear bands of sclerosis in the tibia and femur

Osteoid Lesions of Bone

744

Musculoskeletal Radiology

• •

Melorheostosis: Radiology [Figures 4-5-8 to 4-5-10] • • • • •

Distribution - single limb - more common lower extremity One or more bones–sclerotome (skeleton supplied by spinal sensory nerves) pattern Osseous excrescences often exuberant and lobulated along bone surface -“candle wax” Also endosteal involvement may extend into marrow space Can extend into soft tissue with ossification, often periarticular May lead to joint ankylosis Intense activity on bone scan Figure 4-5-10

Figure 4-5-8

Figure 4-5-9

Melorheostosis involving foot with increased uptake on bone scan (same patient as previous two images)

Melorheostosis with mineralized inguinal soft tissue mass (arrowhead)

Figure 4-5-11

Melorheostosis classic “dripping candle wax” appearance (arrow)

Osteoma: Clinical Characteristics • • • • • • • • •

A benign, slow growing tumor, composed of osteoid tissue Found in cranial vault, sinuses, mandible and (rarely) long bones Represent protruding mass of dense periosteal intramembranous bone on surface of host bone Signs/symptoms depend on size/location Sinus lesions may lead to sinusitis, headache, or can grow into cranial vault Orbital lesion may cause exophthalmos, displacement of globe, diplopia 0.42% patients with sinus radiographs

Osteoma: Pathology

• •

Nodules of dense osseous tissue Differences from bone island: ➢ Often a mix of woven & lamellar bone ➢ May/may not contain haversian system ➢ Arises from cortex rather than intramedullary ➢ Do not blend with trabecular bone ➢ Most frequent in the skull In craniofacial area often in spectrum of fibrosseous lesions

Calvarial osteoma (*)

Figure 4-5-12

Osteoma: Radiology [Figure 4-5-11 and 4-5-12] • • •

Sharply defined, homogeneous, bone mass arising from surface of bone Most frequently diagnosed incidentally on radiographs Frontoethmoid sinus region - 75% Sphenoid 1%–4%

Musculoskeletal Radiology

Gross specimen calvarial osteoma (*) on surface of outer table (arrow) but not extending into the diploic space (DS) 745

Osteoid Lesions of Bone

•

Osteoma: Gardner Syndrome

•

Multiple osteomas are almost always associated with Gardner syndrome ➢ Familial autosomal dominant ➢ Intestinal polyposis ➢ Multiple osteomas ➢ Dental lesions ➢ Soft tissue desmoid tumors ➢ Skin lesions ➢ Sebaceous cysts and fibromas

Osteoid Osteoma: History • • • •

Described in 1935 by Jaffe as an osteoblastic tumor composed of osteoid and atypical bone Established by Jaffe as a distinct clinical and pathologic entity Controversy remains as to true nature: inflammatory, traumatic, vascular, viral

Osteoid Osteoma: Clinical Characteristics • • • • • • • • • •

11% of all bone lesions that come to biopsy (3% of primary bone tumors) Spinal lesions commonly present with painful scoliosis, without neurologic dysfunction Intraarticular lesions often present with nonspecific vague joint pain Young patients usually 10–25 years Male : Female approximately 2–3:1 Rare in blacks Pain almost invariably present complaint (1.6% painless - 50% of these in the hand) Pain relief with aspirin/nonsteroidals ➢ Inhibit prostaglandin E2, aggravated by ETOH

Osteoid Osteoma: Skeletal Distribution [Figure 4-5-13]

• • •

Figure 4-5-13

Femur/tibia - 50% - 60% of lesions Most frequent in long bone diaphysis (70% - 80%) Spine - 10% (90% posterior elements; 10% vertebral body) Hand/foot - 10% - 20% - proximal phalanx, metacarpal, scaphoid, navicular, calcaneus Epiphyseal lesions - rare

Osteoid Osteoma: Pathology • • • • •

The “nidus” is the lesion-yellowish to red pea Composed of osteoid and woven bone with interconnected trabeculae Background and rim of highly vascularized fibrous connective tissue Extensive reactive bone may surround the lesion No malignant potential

Osteoid Osteoma: Classification • •

Cortical (70% - 75%): long bone shaft; intense fusiform sclerosis; central nidus Cancellous (25%) intermediate frequency; usually femoral neck, hand/foot; often limited surrounding sclerosis Intraarticular lesions (cortical/cancellous may have limited sclerosis/periosteal reaction and can be distant from nidus Subperiosteal; rare, arises adjacent to bone; usually femoral neck, talar neck, hand, foot; bone may show pressure erosion

Osteoid Lesions of Bone

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Musculoskeletal Radiology

Osteoid Osteoma: Radiology: Cortical Lesions • • • • •

Figure 4-5-14

[Figures 4-5-14 to 4-5-19]

Dense fusiform sclerosis Periosteal bone solid, rarely lamilated Nidus usually central, rarely > 1.5 - 2cm “Hot” on scintigraphy - double density sign Nidus well-defined on CT with a smooth peripheral margin, +/- central mineralization

Figure 4-5-15

CT of osteoid osteoma with central focus of calcification

Humeral osteoid osteoma in the cortex with bone scan showing “double uptake sign”

Figure 4-5-16

Figure 4-5-17

MR imaging of osteoid osteoma with intermediate signal intensity on T1 [left] and T2 [right] -weighting (arrow). The surrounding marrow edema is prominent (*) and can obscure the nidus

• • •

Osteoid osteoma specimen radiograph shows central calcification and entire nidus within the bivalved gross specimen

Osteoid Osteoma: MR Imaging

• • •

Low to intermediate intensity T1-images Intermediate to high intensity T2-images Low intensity all pulse sequences if totally mineralized nidus May detect synovitis/joint effusion/soft tissue edema that can be very prominent/confusing imaging feature Reactive marrow edema may obscure lesion CT more helpful in majority, however, MRI may be very helpful in difficult cases

Figure 4-5-18

Figure 4-5-19

Radiograph and bone scan of spinal osteoid osteoma in facet with a sclerotic pedicle (arrow) and increased uptake on the radionuclide bone scan (arrowhead)

CT of spinal osteoid osteoma in facet with central calcification Musculoskeletal Radiology

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Osteoid Lesions of Bone

Differential Diagnosis of Osseous Lesions with Sequestra-Like Appearance • • • • • • •

Osteomyelitis-pyogenic Subacute osteomyelitis or unusual organism Osteoid osteoma/osteoblastoma Langerhans cell histiocytosis Metastases Fibrosarcoma/MFH Lymphoma

Figure 4-5-20

Cortical Osteoid Osteoma: Differential Diagnosis • • •

Brodie abscess Stress fracture (linear not circular) Langerhans cell histiocytosis

Osteoid Osteoma Radiology: Intraarticular/Cancellous Lesions • • • • • •

Reactive osteosclerosis/periosteal reaction often mild/absent and may be distant from lesion Associated joint effusion/lymphofollicular synovitis Regional osteoporosis-disuse May have associated periostitis May be diffusely “hot” on scintigraphy Subperiosteal lesions present as juxtacortical masses

Subtle intraarticular osteoid osteoma (arrow) with central calcification causing effusion and hip joint widening simulating septic arthritis

Figure 4-5-21

Intraarticular Osteoid Osteoma: Differential Diagnosis • • • • •

[Figures 4-5-20 and 4-5-21]

•

Rheumatoid arthritis, JRA Tuberculous arthritis Nonspecific synovitis Septic arthritis Osteoblastoma (especially spine)

Osteoid Osteoma: Classic Treatment • • •

• • • •

Surgical excision - curative with complete nidus removal - post op biopsy radiographs Dramatic relief of symptoms Recurrence due to incomplete excision can then have multiple nidi Problems ➢ Locating lesion at surgery ➢ Tetracycline and radionuclide labeling

Osteoid Osteoma: Other Treatment Methods

•

Medical- spontaneous healing Percutaneous removal Percutaneous ablation (radiofrequency) Embolization single feeding artery (potentially) ?

Osteoblastoma • • •

Rare benign osteoid producing tumor characterized by osteoid and woven bone production Subtle intraarticular osteoid osteoma Synonyms include: giant osteoid osteoma and osteogenic fibroma (arrow) with central calcification About 1.0% of excised primary osseous tumors causing effusion and hip joint widening Osteosarcoma 20x more common, osteoid osteoma 4x more frequent simulating septic arthritis on CT and MR. The CT shows typical nidus with central calcification (arrow) that is difficult to perceive on the MR

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• • • •

Patients are young, median age 18, 80% are between 10–30 years Males : Females ratio 2–3:1 Pain is most common symptom, less severe than osteoid osteoma Less often at night and may or may not be relieved by aspirin

• • • •

Spine, (40%) equally distributed cervical through sacrum About 30% occur in long bones, most commonly femur and tibia Skull, mandible, maxilla (15%) Also small bones of hand & feet (10%) and pelvis (5%)

Osteoblastoma: Clinical Features Figure 4-5-22

Osteoblastoma: Skeletal Distribution

•

Osteoblastoma: Pathology • • •

Micro: large number of osteoblasts producing trabeculae, osteoid or bone Virtually indistinguishable from osteoid osteoma on high-power histologic examination At times minor microscopic differences from osteoid osteoma but may rely on radiology

Osteoblastoma: Radiology - General

• • •

Described as having 3 radiologic patterns ➢ 1) Similar to but larger than osteoid osteoma (>2cm) Osteoblastoma of C3 spinous process and lamina ➢ 2) Expansile lytic lesion with mineralization ➢ 3) More aggressive appearance – marked bone destruction, scattered calcification and soft tissue mass

Osteoblastoma: Radiology - Specific

• • •

Radiologic features are not always distinctive Lytic lesion with varying bone production Cortex thinned with expanded contour, may be destroyed, and have a soft tissue mass Mineralization may appear like chondroid tissue -“arcs and rings” but no chondroid tissue pathologically May have surrounding edema but less common than osteoid osteoma Up to 16% secondary ABC (Aneurysmal Bone Cyst) component Solid elements often low/intermediate signal T2 MR

• • • • • •

Posterior elements alone most common (>60%) Posterior elements extending into vertebral body (25%) Vertebral body alone (<15%) More likely to contain ossification and soft tissue mass Less likely to elicit sclerosis Scoliosis variably present less characteristic than osteoid osteoma

•

Figure 4-5-23

Osteoblastoma: Radiology – Spine [Figures 4-5-22 and 4-5-23]

Osteoid Osteoma/Osteoblastoma Differential Diagnosis: Spine •

•

Osteoblastoma ➢ Size > 1.5–2.0cm ➢ Growth and soft tissue mass ➢ Matrix - multifocal - noncentral ➢ Scoliosis and classic symptoms absent Pedicle sclerosis - lymphoma, mets, spondylolysis, congenital absence/ hypoplasia posterior elements, malaligned apophyseal joints, unusual infection

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CT of C3 osteoblastoma with extensive mineralization

Osteoid Lesions of Bone

Osteoblastoma Radiology – Long Bones •

Figure 4-5-24

[Figure 4-5-24]

• • • • • • •

Usually eccentric, metaphyseal (25%) or diaphyseal (75%) Intramedullary or cortical, rarely subperiosteal Solid periostitis (50%) May appear as a blister lesion in hand or foot, may also have osteoporosis Correct diagnosis usually not suggested prospectively

Osteoblastoma: Natural History

• • •

The lesion usually grows slowly Treatment is curettage or excision Recurrence rate is 10%–15%

Aggressive “Malignant” Osteoblastoma

Osteoblastoma of proximal femur with calcification on CT

Initially described 1967- Mayer Considerable controversy and definition not clearly established Problems in distinction from osteoblastoma like osteosarcoma and rare reports of osteoblastoma recurring as osteosarcoma

Figure 4-5-25 • • • • •

Aggressive Osteoblastoma: Pathology Similar to conventional osteoblastoma Wider more irregular trabeculae Lace-like osteoid Variable mitoses but no atypical figures Epithelioid osteoblast

Aggressive Osteoblastoma: Clinical and Radiologic Characteristics • • •

[Figure 4-5-25]

• • • •

Older patients average 33 years of age Similar locations Larger more aggressive on radiologic examination with soft tissue mass Local recurrence rate up to 50% Usually no metastases

Aggressive osteoblastoma with large soft tissue mass (arrow) and multilevel involvement

Osteosarcoma: Definitions • •

A mesenchymal malignancy that differentiates to produce osteoid “...If only 1% of a tumor manifests osteoid and/or bone production by malignant cells, it is by general convention an osteosarcoma” “No matter how meager the osseous component” Only true for intraosseous neoplasm

Mirra “Bone Tumors”. Lee & Febiger 1989

Osteosarcoma is the second most common primary malignant bone tumor - 15% of all biopsied primary bone tumors • •

Osteosarcoma (OS): Additional Definitions • •

Primary OS: lesion in absence of a benign precursor lesion or treatment Secondary OS: lesion that has a benign precursor or is metastatic from primary OS Synchronous OS: lesions discovered within 6 months of each other Metachronous OS: lesions discovered more than 6 months apart

• •

Osteoid and/or immature bone production by tumor cells Malignant stromal cells graded on their degree of anaplasia I-IV

Osteosarcoma: Pathology-General

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•

Osteosarcoma: Histologic Pattern

•

Types Osteoblastic (mixed and sclerosing) 82% Fibroblastic (Fibrosarcoma and MFH like) 7% Chondroblastic 5% Telangiectatic 3% Small Cell 1% Other 2% Symptoms usually pain and swelling

• • • • • • •

Intramedullary-high grade 75% Juxtacortical 7%-10% Gnathic 6% Low grade sclerosing 4% - 5% Soft tissue 4% Osteosarcomatosis - multifocal 1% - 2% Intracortical 0.2%

Figure 4-5-26

Primary Osteosarcoma: Classification

• • • •

Intramedullary osteosarcoma with osteoid matrix (arrows) and aggressive bone lysis (arrowheads)

Secondary Osteosarcoma: Classification

• • •

Paget disease (67% - 90%) Figure Radiation induced (6% - 22%) Osteonecrosis Others - fibrous dysplasia, prosthesis, osteogenesis imperfecta, chronic osteomyelitis, retinoblastoma About half of osteosarcoma over age 50 are secondary, 67% over age 60

4-5-27

Osteosarcoma: Primary, High-Grade, Intramedullary • • •

About 75% of all osteosarcomas Most patients are between 15 and 25 years, rare younger than 6 or older than 60 years Male > Females 1.5–2:1 About 70% are in the long bones, more than 50% about the bones of the knee 90% are metaphyseal, 5%–10% diaphyseal

Osteosarcoma Intramedullary: Radiology • • •

[Figures 4-5-26 to 4-5-29]

• • • •

Usually mixed sclerosis and lysis Soft tissue mass (80%) Periosteal reaction (80%) - Codman triangle, lamination, perpendicular (“sunburst”, “hair on end”) Osteoid matrix “fluffy”/ “cloud-like” (90%) Extend across epiphyseal plate (75% - 90%) MRI/CT essential for staging and preoperative planning

Bone scan of intramedullary osteosarcoma of tibia with intense radionuclide uptake. The femur and ankle also show increased uptake (arrowheads) from hypermia and disuse

Figure 4-5-28

Osteosarcoma Telangiectatic • •

Tumor largely composed of cystic cavities containing necrosis and hemorrhage ( > 90%) ABC like – misdiagnosed on radiographs Distribution similar to other intramedullary osteosarcomas ➢ Femur, tibia, humerus ➢ Metaphyseal (90%), diaphyseal (10%)

Coronal STIR MR image and gross specimen of tibial intramedullary osteosarcoma show identical lesion extent (*)

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Osteosarcoma Telangiectatic • •

Figure 4-5-29

[Figures 4-5-30 to 4-5-33]

• • • •

• • • •

Largely osteolytic and expansile Look for small areas of osteoid (58% X-rays, 85% CT) Fluid-fluid levels (CT 48% / MRI 89%) Pathologic fracture (25% - 61%) Donut sign on bone scan (65%) Previously worse prognosis, now may actually be better than other intramedullary osteosarcomas (68% 5 year survival)

Juxtacortical Osteosarcoma Parosteal (65%) Periosteal (25%) High grade surface (10%) Prognosis varies with grade and extent Intramedullary osteosarcoma with skip metastases (*) on coronal STIR MR image and gross specimen with distal primary lesion (arrow) and intervening normal marrow (M)

Figure 4-5-30

Figure 4-5-31

Telangiectatic osteosarcoma of scapula with thick peripheral mineralization (arrow)

Telangiectatic osteosarcoma of scapula with thick enhancing nodular wall containing calcification (arrowheads) and central hemorrhage/necrosis (*) on CT

Figure 4-5-33

Figure 4-5-32

Telangiectatic osteosarcoma of distal femur on sagittal T1weighted post-contrast MR image and gross specimen showing thick nodular enhancement peripherally in viable tumor (arrowheads) correlating to gross specimen with central nonenhancing cystic/necrotic regions (*)

Gross specimen of scapular telangiectatic osteosarcoma with cystic spaces (*) surrounded by solid viable tissue (arrowheads)

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• •

Parosteal Osteosarcoma: Clinical and Pathology • • •

Figure 4-5-34

Arise from outer layer of periosteum Usually a low grade tumor – fibroblastic stroma and streamers of woven bone Patients about a decade older than intramedullary osteosarcoma; F>M Location - femur (65%), humerus (15%), tibia (10%), fibula (3%), forearm (3%) Most common posterior distal femur metaphysis

Parosteal Osteosarcoma: Radiology • •

[Figures 4-5-34 to 4-5-37]

• • • •

Initially an exophytic sclerotic mass Cauliflower-like with lucent cleavage plane between lesion and cortex Radiodense centrally with growth may reattach to cortex and obliterate cleavage plane MRI/CT to evaluate intramedullary invasion important for surgical resection Long term survival 80%–90%

Periosteal Osteosarcoma: Clinical and Pathology • • • •

Parosteal osteosarcoma with specimen radiograph and gross specimen showing surface mass with central dense stalk of attachment to the cortex (*) and lucent cleavage plane (arrows)

Periosteal sarcoma is usually chondroblastic (>90% of tumor) and intermediate grade Arise from inner layer of periosteum More than 85% are in the diaphysis of the femur and tibia; ulna and humerus (10%) Similar age to conventional osteosarcoma and sex distribution (M>F) Better prognosis but 15% rate of metastasis

Figure 4-5-35

Figure 4-5-36

Figure 4-5-.37

Parosteal osteosarcoma of the distal femoral metaphysis with radiograph, CT and gross specimen and specimen radiograph showing surface mass (*), lucent cleavage plane (arrows) and medullary backgrowth (arrowhead)

Parosteal osteosarcoma of the distal femoral metaphysis with radiograph, CT and gross specimen and specimen radiograph showing surface mass (*), lucent cleavage plane (arrows) and medullary backgrowth (arrowhead)

Musculoskeletal Radiology

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Periosteal Osteosarcoma: Radiology •

Figure 4-5-38

[Figure 4-5-38]

• • • •

Saucerized cortex with chondroblastic soft tissue mass on imaging in area of erosion Cortical thickening at margins of erosion (40%) May have Codman triangle Spiculated periosteal reaction Only rarely intramedullary invasion reported

Osteosarcoma: High - Grade Surface •

[Figures 4-5-39 and 4-5-40]

• • •

Histology high-grade similar to a conventional osteosarcoma as is prognosis with same potential for metastasis Sites – femur (45%), humerus (26%), fibula (10%), ulna (6%) Radiologic changes: broad based lesion with osteoid arising on osseous surface Similar appearance to periosteal osteosarcoma but often more aggressive

Periosteal osteosarcoma or radiograph and coronal CT reconstruction with diaphyseal broad based soft tissue mass causing erosion of uderlying thickened cortex and “hair-on-end” periosteal rection

Figure 4-5-39

Figure 4-5-40

High-grade surface osteosarcoma

• • • • •

Gnathic Osteosarcoma [Figure 4-5-41]

• •

About 6% of all osteosarcomas Patients are usually older Lesions are usually lower grade About half are chondroblastic Prognosis is better – don’t tend to metastasize but locally invasion

High-grade surface osteosarcoma (*) on sagittal T1-weighted MR and sagittally sectioned gross specimen. Note the surface location and sparing of the medullary canal (M)

Figure 4-5-41

Osteosarcoma: Low Grade Intramedullary • • •

Described in 1977 by Unni (27 cases) Also called sclerosing osteosarcoma – being recognized with increasing frequency Patients average about a decade older than conventional osteosarcoma Most patients present with pain (85%), or swelling (15%), 5% are incidental findings Four histologic patterns simulating FD (50%), NOF (25%), chondroblastoma (15%), CMF (10%)

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Gnathic osteosarcoma with an aggressive mixed lytic and sclerotic (arrows) lesion destroying the alveolar ridge (arrowhead)

Musculoskeletal Radiology

Osteosarcoma: Low Grade Intramedullary • • • • •

Figure 4-5-42

[Figures 4-5-42 and 4-5-43]

•

Not uncommonly metaepiphyseal Location same as intramedullary conventional Central sclerosis with expansile remodeling Ground glass density and trabeculation within Not as aggressive appearance on radiographs and lack soft tissue mass ➢ ISS 2003 (Skel Rad 2004, 33:373-379) MR/CT all 17 cases had a soft tissue mass Better prognosis with 10% or less metastatic rate

Osteosarcoma: Soft Tissue (Extraskeletal) • • • • • •

Rare – same histology Middle aged to older patients (mean 55 years) Location-deep soft tissues of extremities-thighs and shoulders also retroperitoneum Not uncommonly history of trauma (10%–15%) Relationship to myositis ossificans? Radiology – soft tissue mass with calcification or ossification

Low-grade intramedullary osteosarcoma simulating fibrous dyplasia on radiograph and CT. Note soft tissue mass medially (*)

Osteosarcomatosis: Multifocal Osteosarcoma [Figures 4-5-44 and 4-5-45] • •

• •

Figure 4-5-43

Rare Classified into types in 1969 by Amstutz: ➢ 1-Synchronous, young patients < 18 years of age ➢ 2-Synchronous, adults ➢ 3-Metachronous Type 3 likely represents metastatic disease Types 1 and 2 usually demonstrate a radiologically dominant lesion

Amstutz. Cancer 1969;24:923

Low-grade intramedullary osteosarcoma simulating fibrous dyplasia on radiograph and CT. Note soft tissue mass medially (*)

Figure 4-5-44

Figure 4-5-45

Osteosarcomatosis with multifocal areas of metaohyseal sclerosis (*) with primary dominant sclerotic focus in the tibia ( *) [Figure 4-5-45]

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Osteosarcoma : Intracortical • •

Figure 4-5-46

[Figure 4-5-46]

• •

Very rare, only a handful of cases Almost all in the femur and tibia diaphysis The lesion is dominantly or exclusively intracortical with no (or only minimal) intramedullary involvement Usually lytic/surrounding sclerosis

Intracortical osteosarcoma with matrix mineralization and location within the cortex (arrow) on radiograph and CT

Osteosarcoma: Treatment and Prognosis • • • • •

Complete removal of the primary tumor – limb salvage Preoperative chemotherapy – look for > 90% tumor necrosis – most important predictor of prognosis (90% 5 year survival; 14% <90% necrosis) Post operative chemotherapy Overall 5 year survival 41%–64% 5 year survival 60%-70% no metastases at presentation and surgical resection

References 1. 2. 3. 4. 5. 6.

Greenspan A, Stadalnik RC.. Bone island: scintigraphic findings and their clinical application. Can Assoc Radiol J. 1995 Oct;46(5):368-79. Judkiewicz AM, Murphey MD, Resnik CS, Newberg AH, Temple HT, Smith WS. Advanced imaging of melorheostosis with emphasis on MRI. Skeletal Radiol. 2001 Aug;30(8):447-53. Klein MH, Shankman S. Osteoid osteoma: radiologic and pathologic correlation. Skeletal Radiol. 1992;21(1):23-31. Review. Kroon HM, Schurmans J. Osteoblastoma: clinical and radiologic findings in 98 new cases. Radiology. 1990 Jun;175(3):783-90. Murphey MD, Robbin MR, McRae GA, Flemming DJ, Temple HT, Kransdorf MJ. The many faces of osteosarcoma. Radiographics. 1997 Sep-Oct;17(5):1205-31. Sundaram M, Falbo S, McDonald D, Janney C. Surface osteomas of the appendicular skeleton. AJR Am J Roentgenol. 1996 Dec;167(6):1529-33.

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Cartilaginous Lesions of Bone Mark D. Murphey, MD Figure 4-6-1 • • • • • •

Cartilaginous Lesions

•

Osteochondroma Enchondroma Juxtacortical chondroma Chondromyxoid fibroma (CMF) Chondroblastoma Chondrosarcoma

Osteochondroma

• •

The most common benign neoplasm of bone that leads to biopsy

Osteochondroma: Types

Osteochondromas with marrow (*) and cortical (arrows) continuity on radiography and histology. Note undertubulation on macrosection in patent with hereditary multiple exostoses (HME) with other small lesions identified by hyaline cartilage caps (arrowheads)

Solitary osteocartilaginous exostosis Hereditary multiple exostoses (HME) ➢ Diaphyseal aclasis ➢ Multiple osteochondromas ➢ Osteochondromatosis

Figure 4-6-2 • •

Osteochondroma: Radiographic Subtypes

• • • •

Pedunculated Sessile

Osteochondroma: Clinical Data

• •

• •

•

No sex predilection Young patients - 75% < 20 years old Present as a mass: responsible for symptoms Mechanical, cosmetic, affect on adjacent structures (tendon, muscles, nerve, vessel), fracture Location: femur (30%), tibia (20%), humerus (20%), hand and foot (10%), pelvis (5%), scapula (4%) Symptoms dependent on size/location ➢ Bursa formation Malignant transformation ➢ Solitary < 1% Treatment-Individualized-Resection ➢ Dependent on symptoms/size/location

Sessile and pedunculated osteochondromas with marrow (*) and cortical (arrows) continuity

Figure 4-6-3

Osteochondroma: Pathology • • • • •

Medullary and cortical continuity with underlying bone Hyaline cartilage cap Cartilage cap involutes after growth (skeletal maturity) Only benign skeletal neoplasm associated with radiation Can be induced by implanting epiphyseal tissue Traumatic osteochondroma Pelvic osteochondroma revealing only sclerosis on radiograph with cortical and medullary continuity revealed on CT (arrow) and gross specimen (*)

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• • • •

Osteochondroma: Imaging [Figures 4-6-1 to 4-6-3]

•

•

Figure 4-6-4

Mature bone-cortex and marrow Hyaline cartilage cap-calcification Cortical and marrow continuity with underlying bone Long bones – radiographs to diagnose ➢ Metaphyseal ➢ Grows away from epiphysis ➢ May be associated with failure of tubulation (particularly HME) Flat bones – often need CT/MR to diagnose ➢ Tend to be larger and sessile ➢ More variable appearance

Osteochondroma on CT with marrow and cortical continuity

Figure 4-6-5

Osteochondroma The cartilage cap deserves the most consideration in radiologic interpretation

Osteochondroma: Cartilage Cap •

[Figures 4-6-4 to 4-6-9]

• • • •

•

Radiographs - chondroid calcification ➢ Increasing destruction or change in appearance ➢ Worrisome for malignancy Ultrasound - good for cap and bursae CT - soft tissue with calcification ➢ Can be difficult to distinguish from muscle BS - increased uptake MRI - Intermediate signal T1W images ➢ High signal T2W images Cap thickness - benign vs malignant ➢ Benign < 1.5 cm (0.1- 3.0 cm; ave. 0.6-0.9 cm) ➢ Malignant > 1.5 cm (1.5 -12 cm; ave. 6 cm) ➢ Depends on skeletal maturity

Osteochondroma on radiograph and sagittal T1weighted and axial T2-weighted MR images with thin cartilage cap showing high signal on long TR MR (arrowhead)

Figure 4-6-8

Figure 4-6-6

Gross specimen and macrosection of resected osteochondroma with thin bluish cartilage cap (*) correlating with the imaging (same patient as 4-6-5)

Axial T1-weighted MR images show cortical and marrow ((arrowheads) continuity and thick cartilage cap (*) (15 year old boy; same patient as previous radiographs)

Figure 4-6-7

12 years old

Figure 4-6-9

15 years old Sagittal STIR MR and gross specimen of resected osteochondroma with high signal, thick cartilage cap (15 year old boy with thick cartilage cap simulating malignancy but only represented growth due to young age; same patient as previous radiographs and axial T1-weighted MR)

Lateral ankle radiographs at 3 year interval shows growth of the osteochondroma in this 12 year old boy. The same radiographic appearance would represent malignant transformation in an adult

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Subungual Exostosis: Dupuytren Exostosis • • • • • •

Figure 4-6-11

[Figure 4-6-10]

Osteochondroma variant Females > Males (2:1) Often painful and associated with trauma and infection Great toe (77%-80%); Fingers (10%-14%) Fibrocartilage cap Located away from physis

Figure 4-6-10

Dysplasia epiphysealis hemimelica (Trevor disease) with early genu varus deformity caused by the epiphyseal osteochondroma (arrow)

Subungual exostosis (arrow) with clinical photograph

Figure 4-6-12

Dysplasia Epiphysealis Hemimelica: Trevor Disease • • • • •

[Figures 4-6-11 to 4-6-13]

• • • • •

Male predominance (3:1) Rare Swelling, pain and deformity Usually lower extremity, unilateral 65% multiple bone involvement talus, distal femur, tibia Ankle and knee most common Medial joint 2x lateral Lobular epiphyseal mass Histologically identical to an osteochondroma May produce deformity and secondary osteoarthritis

Figure 4-6-13

Dysplasia epiphysealis hemimelica (Trevor disease) with the epiphyseal osteochondroma (*) arising from the posterior femur on lateral radiograph, and sagittal T2-weighted MR and coronal 3D CT reconstruction

Dysplasia epiphysealis hemimelica (Trevor disease) with the epiphyseal osteochondroma (*) arising from the posterior femur on lateral radiograph, sagittal T2-weighted MR and coronal 3D CT reconstruction (same patient as 4-6-12) Musculoskeletal Radiology

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Hereditary Multiple Exostoses: Clinical Data

• • • •

Figure 4-6-14

[Figures 4-6-14 to 4-6-16]

• • • • •

Male predominance (3:1) Autosomal dominant inheritance Variability in size and number Any portion of the skeleton preformed in cartilage may be involved Present in childhood May be bilaterally symmetric One side may predominate Increased incidence of malignant transformation (10%–20%) Newer literature 3%–5%

Figure 4-6-15

Hereditary multiple exostoses (HME) with associated undertubulation of bone (Erlenmeyer flask deformity)

Figure 4-6-16

HME with typical pelvic and proximal femoral deformity

Enchondroma: The most common tumor encountered in the phalanx • • • •

Enchondroma: Types

•

Solitary enchondroma Multiple enchondromatosis Ollier disease Maffucci syndrome

Enchondroma: Clinical Data

HME with typical pelvic and proximal femoral deformity. Bone

• •

scan shows a left pelvic lesion to reveal more intense 3%–5% all biopsied primary bone lesions; 1% all radionuclide uptake(*) and this area demonstartes a very thick bone tumors hyaline cartilage cap (>3 cm) and soft tissue mass with No sex predilection chondroid mineralization (rings and arcs) on radiograph, CT Peak incidence 3rd decade (10–30 years old) and T1/T2 weighted MR images (arrows) resulting from Hands and feet (40%–65%), long tubular bones malignant transformation to chondrosarcoma. This is also shown on the gross specimen (25%) Phalanges and metacarpals most common locations May be incidental finding or present with pathologic fracture

• • • •

Rests of hyaline cartilage Hyaline cartilage often with myxoid areas Variable amorphous calcification and enchondral ossification May cause expansile remodeling and cortical thinning

• • •

Enchondroma: Pathology

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• • •

Enchondroma: Imaging [Figures 4-6-17 to 4-6-19]

• •

Figure 4-6-17

Geographic lytic lesion IA-IB Central often metaphyseal Expansile remodeling with prominent thinned cortex (short tubular bones only) Chondroid matrix in majority – 17% limited or none (radiographs); all by CT MRI – Lobulated margin ➢ Marked increase intensity T2W images ➢ Calcified chondroid-low intensity

Figure 4-6-18

Enchondroma of the phalanx with typical ring and arc chondroid mineralization and deep endosteal scalloping

Figure 4-6-19

Enchondroma of tibia on coronal T1-weighted (left) and T2-weighted (right) MR images. Note lobular margin and no endosteal scalloping and high signal on long TR image resulting from high water content of nonmineralized hyaline cartilage

• •

Multiple Enchondromatosis: Clinical Data

• • • •

Variable severity May be predominantly unilateral (Ollier disease-1899) May become stable at puberty Increase malignant transformation to chondrosarcoma (5%–50%) Marked skeletal deformity Not hereditary Mild male predilection Presents in childhood

•

Described in 1881 by Maffucci as enchondromatosis with hemangiomas

• •

Enchondroma of the distal femur on radiograph, sagittal T1weighted and T2-weighted MR images and histology. Note lobular margin (arrows), ring and arc mineralization (arrowhead), no endosteal scalloping and high signal on long TR image resulting from high water content of hyaline cartilage

Maffucci Syndrome • • • • • • •

Figure 4-6-20

Maffucci Syndrome: Clinical Data Multiple enchondromas Cavernous hemangiomas Very rare; nonhereditary Mild male predilection Hands and feet greatest involvement Complications of hemangiomas Malignant transformation ➢ Chondrosarcoma (15%–20%) ➢ Vascular sarcoma (3%–5%) ➢ Ovarian malignancy ➢ Glioma and carcinoma Enchondromatosis on radiograph and coronal T2* (GRE) MR image which show diagnostic columns of cartilage extending into metaphysis from epiphyseal plate (arrows)

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Maffucci and Ollier Syndromes: Imaging Characteristics • •

Figure 4-6-21

[Figures 4-6-20 and 4-6-21]

•

• • • • •

• • •

Lesions seen in infancy Lytic columns from epiphyseal plate extending into the metaphysis Later typical enchondromata ➢ Geographic IA-IB margin with expansion Chondroid matrix calcification Growth disturbance and bowing Enchondroma growth slows after plate closure Soft tissue masses with phleboliths Chondrosarcomatous transformation ➢ New bone destruction with ST mass ➢ New periosteal reaction ➢ Disorganized or destroyed matrix calcification

Juxtacortical Chondroma

• •

Arise adjacent to cortex beneath periosteum Metaphyseal Proximal humerus (50%), femur and tibia also hands and feet (25%) < 30 years old, M > F (2:1) Often more cellular than enchondroma

Juxtacortical Chondroma: Imaging • • •

[Figure 4-6-22]

• •

Cortical saucerization (1–3 cm) Variable sclerosis/periosteal reaction Soft tissue mass with chondroid calcification (50%) High intensity T2W MR images Difficult to differentiate chondrosarcoma

Enchondromatosis on bone scan and extensive deformity of the upper extremity on radiograph. Note predominance on one side of the body on bone scan. MR, CT and gross specimen reveal malignant transformation to chondrosarcoma with small associated soft tissue mass laterally (arrows)

Figure 4-6-22

Juxtacortical chondroma with extrinsic erosion or saucerization (arrowheads) of metacarpal

• •

Chondromyxoid Fibroma (CMF): Fibromyxoid Chondroma • •

Rare, least common cartilage tumor Usually lower extremity ➢ 55% around knee, 20%–25% in foot Young adults, 60% < 30 years old Rarely malignant transformation

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•

CMF: Pathology • • • • • •

Figure 4-6-23

Myxoid, fibrous and chondroid tissue in various proportions Myxoid areas-central in lobules Cellular areas peripheral in lobules Foci of calcification 5%–27% Lobulated tumor mass

CMF: Radiology [Figure 4-6-23] • • • •

•

Geographic lytic lesion IA -IC Eccentric metaphyseal location; often cortical (long bone lesions) Expansile remodeling simulate cortical permeation Flat/short tubular bone lesions central Rare matrix mineralization (CT/tomography) MRI – similar to slightly lower intensity than muscle T1W images ➢ Very high signal intensity T2W images

Chondroblastoma: Codman Tumor - History • • • • •

Cartilage containing GCT ➢ Kolodney 1927 Calcifying GCT ➢ Ewing 1928 Epiphyseal chondromatous GCT ➢ Codman 1931

Chondromyxoid fibroma with intracortical location in the tibia and outer margin appearing aggressive on radiograph (arrowhead) but intact on CT (arrow) and low attenuation (*) resulting from high water content hyaline cartilage (note the lack of matrix mineralization)

Figure 4-6-24

Chondroblastoma: Clinical Data

• • •

Uncommon; 1%–2% all bone tumors Male > Female (2:1) Children and young adults; 90% between ages 5 and 25 years

Chondroblastoma: Location Epiphysis/apophysis only 40% Epiphysis and metaphysis 55% Metaphysis only 4%

Epiphyseal/Apophyseal Lesions: Differential Diagnosis • • • • • • • •

Chondroblastoma centered in the epiphysis but extending into the metaphysis, matrix mineralization and periosteal reaction extending into the diaphysis (arrowhead) is also seen

Chondroblastoma GCT Subchondral cyst/intraosseous ganglion Infection Langerhans cell histiocytosis Osteoid osteoma/osteoblastoma Clear cell chondrosarcoma

Figure 4-6-25

Chondroblastoma: Location • • • •

Proximal femur ➢ Head and neck ➢ Trochanter Distal femur Proximal tibia Proximal humerus Hands and feet

23% 16% 7% 20% 17% 17% 10% Chondroblastoma on coronal T1-weighted and T2-weighted MR image with the lesion showing low to intermediate signal intensity on long TR image (arrowheads) and extensive surrounding marrow edema (*) (same patient as previous image)

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•

Chondroblastoma: Histology • • • •

Figure 4-6-26

Chondroblasts - can be confused with chondro/osteosarcoma Multinucleated giant cells Chondroid 1% - 15% ABC component 5% - 15% Initially cellular; later necrosis, fibrosis, maturation

Chondroblastoma: Imaging • • • •

[Figures 4-6-24 to 4-6-28]

• •

•

Geographic lytic lesion IA / IB Eccentric > central; rarely expansile Calcified chondroid matrix 30% - 50% Periosteal reaction 30% - 50% ➢ Adjacent diaphysis/metaphysis CT/MRI-fluid/fluid levels MRI – not typical chondroid characteristics ➢ BEWARE!! - low/intermediate T2W ( 95%) ➢ Extensive surrounding edema ➢ Joint effusion (30% - 50%)

Chondroblastoma of greater trochanter (apophysis = epiphyseal equivalent) on coronal T2-weighted MR image with typical low signal intensity lesion (arrow) and surrounding edema (*)

Figure 4-6-27

Chondroblastoma: Treatment • • •

Curettage and cryosurgery or en bloc resection and bone graft Radiofrequency ablation Local recurrence 5%-10% Malignant chondroblastoma very rare

• • • • • • •

Intramedullary Periosteal/juxtacortical Clear Cell Mesenchymal Myxoid Extraskeletal Dedifferentiated

• • • • •

Enchondroma Osteochondroma Paget Disease Radiation induced Miscellaneous

Chondrosarcoma Types: Primary

Chondrosarcoma Types: Secondary

Figure 4-6-28 Chondroblastoma with ABC component in the patella presenting as a pathologic fracture. Radiograph and sagitally sectioned gross specimen and macrosection show the lytic expansile lesion with fracture, largely composed of cystic areas (*) and small solid component of chondroblastoma inferiorly (arrow)

Chondroblastoma with ABC component in the greater trochanter. Multiple MR image show the lesion largely composed of cystic areas (*) and small solid component of chondrobstoma medially (arrows) Cartilaginous Lesions of Bone

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•

Chondrosarcoma: Pathology • • •

Figure 4-6-29

Malignant tumor of cartilage often with myxoid changes Grades I (30%), II (40%), III (30%) Grade I difficult to differentiate from benign Diagnosis based on histologic and growth features, symptoms and tumor size/location

Intramedullary Chondrosarcoma: Clinical Data • • • • •

Symptoms — pain (95%-99%) and mass (82%) Male > Female (3:2) Average age 40–45 years; metaphysis Location — femur (25%), pelvis (30%), shoulder (15%), ribs/sternum (10%), vertebrae (7%), scapula (5%) 8%-17% all biopsied primary bone tumors

Intramedullary Chondrosarcoma: Imaging •

[Figures 4-6-29 to 4-6-37]

• • • • • • •

Geographic IA -IC to permeative ➢ Often predominantly sclerotic Deep endosteal scalloping Cortical thickening/periosteal reaction Expansile remodeling Soft tissue mass (20% - 76%) Chondroid matrix (78% by X-ray; 94% by CT) CT/conventional tomography if matrix subtle MRI - similar to muscle T1W images ➢ Lobulated high intensity T2W images ➢ Matrix calcification low intensity ➢ Peripheral/Septal contrast enhancement

Low grade chondrosarcoma of the humerus with typical features on multiple imaging modalities. Radiograph shows typical ring and arc mineralization of a chondroid lesion (white arrows) with deep endosteal scallop (black arrow). Bone scan reveals marked increased radionuclide uptake

Figure 4-6-30

Low grade chondrosarcoma of the humerus with typical features on multiple imaging modalities. CT and axial MR images show matrix mineralization on the CT (white arrows) and typical septal and peripheral enhancement of cartilage lesions on the post contrast MR (white arrows) (same patient as previous and next images)

Figure 4-6-31

Figure 4-6-32

Low grade chondrosarcoma of the humerus with typical features on multiple imaging modalities. Coronal T2-weighted MR and coronally sectioned gross specimen reveal marrow replacement (large white arrows) and deep scalloping with early extension into the soft tissues (small white arrows)

Intramedullary chondrosarcoma of femur with chondroid mineralization superiorly (arrow) and deep area of scalloping laterally (arrowhead)

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Figure 4-6-33

Figure 4-6-34

Axial proton density MR images show cortical breakthrough and soft tissue mass (arrowheads) in this femoral intramedullary chondrosarcoma (same patient as previous radiograph) Acetabular intramedullary chondrosarcoma shows subtle bone destruction (arrow) and matrix mineralization in this complex area of anatomy

Figure 4-6-35

Figure 4-6-36

CT of acetabular intramedullary chondrosarcoma shows matrix mineralization (arrowhead) and large associated soft tissue mass (*) (same patient as previous radiograph)

Figure 4-6-37 Coronal T2-weighted MR image of acetabular intramedullary chondrosarcoma shows large high signal intensity mass (*) (same patient as previous CT)

Intramedullary chondrosarcoma of anterior rib on CT with low attenuation mass and matrix mineralization (arrow)

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Juxtacortical/Periosteal: Chondrosarcoma • • •

Figure 4-6-38

[Figures 4-6-38 and 4-6-39]

•

Similar to juxtacortical chondroma Periosteal lesion – cortical erosion Similar in appearance to periosteal OGS (but no hair on end periosteal reaction) Chondroid matrix calcification Larger size than juxtacortical chondroma (>3–4cm) Intramedullary canal spared

• • • • • •

2% of chondrosarcomas Slightly younger age 75%–80% lesions proximal femur or humerus Flat bones involved – 10% Propensity for epiphysis, > 90% Better prognosis

• • • • •

Clear cell chondrocytes Osteoblastoma like osseous metaplasia Areas of conventional chondrosarcoma – 50% Chondroblastoma like areas Osteoclastic giant cells

• •

Clear Cell Chondrosarcoma: Clinical Data

Juxtacortical chondrosarcoma seen on multiple imaging modalities. Radiograph, CT and axial T1-weighted MR show the juxtacortical mass (M) with chondroid matrix mineralization (rings and arcs). There is extrinsic erosion of the fibular cortex (black arrows on radiograph/CT and curved arrow on MR) and low attenuation of the nonmineralized components on CT (*)

Clear Cell Chondrosarcoma: Pathology

Figure 4-6-39

Clear Cell Chondrosarcoma: Imaging • • •

[Figures 4-6-40 and 4-6-41]

• •

Geographic lysis IA to IC Totally lytic (50%); Calcified chondroid matrix 33% Rind of sclerosis (20%) simulates nonaggressive lesion Soft-tissue mass less common – 10% MRI – often high signal T2W images but may have areas of low signal as well

Figure 4-6-40

Juxtacortical chondrosarcoma seen on multiple imaging modalities. Sagittal T2-weighted MR shows high signal intensity of the mass (arrows and black M). The coronally sectioned gross specimen reveals the lobular chondroid growth (C), extrinsic erosion of the cortex (arrows), Normal marrow space (white M) and the periosteal elevation (P) (same patient as figure 4-6-38)

Figure 4-6-41

Clear cell chondrosarcoma of humerus extending to subchondral region simulating a giant cell tumor

Clear cell chondrosarcoma with high signal intensity (unlike giant cell tumor) on coronal STIR MR images Musculoskeletal Radiology

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• • • •

Mesenchymal Chondrosarcoma: Clinical Data

Figure 4-6-42

Less than 10% chondrosarcoma’s Younger age – averages 25 years Males = Females Osseous location – femur (15%), ribs (15%), spine (10%), craniofacial (20%), pelvis (10%)

Extraskeletal Chondrosarcoma: Mesenchymal Type • • • • • • • • • • • •

Young patients 23 – 44 years 20%–30% soft tissue Location – meninges and thigh High grade malignancy Mesenchymal cells with islands of cartilage Large soft tissue masses – may have chondroid matrix calcification Metastases – lymph node, lung

Mesenchymal chondrosarcoma showing very aggressive bone destruction with large soft tissue mass and chondroid matrix mineralization (arrows) on radiograph and CT

Figure 4-6-43

Mesenchymal Chondrosarcoma: Pathology Undifferentiated mesenchymal cells Multifocal islands of malignant cartilage Hemangiopericytoma like areas Aggressive high grade lesions Poor prognosis

Mesenchymal Chondrosarcoma: Imaging •

[Figures 4-6-42 and 4-6-43]

• • • • • • •

Aggressive osseous destruction motheaten to permeative Chondroid matrix calcification less prominent – small foci (60%–70%) Soft tissue mass (near 100%) Masses show lower water content (CT/MR) and enhance diffusely; may see high flow vessels (MR)

Myxoid Chondrosarcoma

Figure 4-6-44

Figure 4-6-45

Rare in bone – 12% chondrosarcomas More aggressive radiologic appearance Worse prognosis Look for myxoid areas ➢ Low attenuation or signal intensity T1W images; may show areas of hemorrhage ➢ Very high signal intensity T2W images ➢ Contrast enhancement — Peripheral / diffuse

Extraskeletal Chondrosarcoma: Myxoid Type (Chordotic) • • • • • • •

Mesenchymal chondrosarcoma on MR images shows marrow replacement (M) and diffuse contrast enhancement as well as small serpentine vessels (arrows). This is not the enhancement pattern of conventional chondrosarcoma (peripheral/septal)

[Figures 4-6-44 and 4-6-45]

Myxoid chondrosarcoma of proximal femur shows bone destruction with intramedullary chondroid mineralization (arrowhead) and large posterior soft tissue mass with marked low attenuation (*)

Middle aged patients – average age 50 years Deep musculature tissue extremities Thigh/Popliteal fossa (70%) Low grade malignancy – may recur late Hemorrhage and myxoid areas can be seen with imaging Typical chondroid regions – radiographs Metastases – lymph node, lung

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Axial T2-weighted MR image reveals marked high signal intensity resulting from very high water content of myxoid chondrosarcoma (same patient as previous image)

Musculoskeletal Radiology

• • •

Dedifferentiated Chondrosarcoma: Clinical Data

•

Figure 4-6-46

Older patients – average 60 years 10%–20% chondrosarcomas Often associated with secondary chondrosarcoma (> 50%) Location – femur (20%), humerus (15%), pelvis (30%), ribs and scapula (12%)

Dedifferentiated Chondrosarcoma: Pathology • • • • •

Low grade chondrosarcoma Small foci higher grade chondrosarcoma Spindle cell component MFH/ fibrosarcoma, osteosarcoma, rhabdomyosarcoma, GCT Collision of two tumors

Dedifferentiated Chondrosarcoma: Imaging •

[Figures 4-6-46 and 4-6-47]

• • •

Dedifferentiated chondrosarcoma with radiographs showing typical chondroid mineralization (rings and arcs-arrows). There is anterior cortical destruction with a small soft tissue mass (arrowheads)

Radiology emulates pathology: beware the dual characteristic ➢ One region chondrosarcoma ➢ Second area aggressive bone destruction Cortical permeation and soft tissue mass (70%) Biopsy of anaplastic region – confusing Dedifferentiated component compared to chondroid component ➢ Different intrinsic characteristics ➢ Different contrast enhancement (diffuse)

Figure 4-6-47

Radiologic Differential of Chondrosarcomatous Lesions •

• •

Aggressive chondroid lesion with soft tissue mass ➢ Higher grade conventional chondrosarcoma ➢ Dedifferentiated chondrosarcoma ➢ Mesenchymal chondrosarcoma Large fluid component bone or soft tissue ➢ Myxoid chondrosarcoma Change in appearance or foci of more aggressive nature ➢ Dedifferentiated chondrosarcoma Dedifferentiated chondrosarcoma with post contrast fat suppressed T1-weighted MR image showing typical peripheral and septal enhancement in the cartilaginous portion of the lesion (arrows) and diffuse enhancement in the dedifferentiated anterior soft tissue component (*) correlating with the sagitally sectioned gross specimen

Low-Grade Chondroid Lesion: Differential Diagnosis • • • • • • • •

Enchondroma Low-grade chondrosarcoma Bone infarct

Bone Infarct: Osteonecrosis [Figure: 4-6-48 and 4-6-49] Ischemic area may undergo mineralization Can have chondroid-like matrix Look for peripheral rim of calcification No cortical thickening Prominent areas of endosteal scalloping or mass exclude osteonecrosis ➢ Except malignant degeneration

Diagnostic Dilemma Long Bone: Enchondroma vs. Chondrosarcoma •

Enchondroma ➢ Common in hand/foot ➢ Rare in axial skeleton ➢ Common in long bones (1.7% distal femur)

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•

Chondrosarcoma ➢ Common in axial skeleton ➢ Common in long bones ➢ Rare in hand/foot

Figure 4-6-48

Enchondroma vs. Low-Grade Chondrosarcoma: Clinical Data • •

• •

Pain (95%–99%) and mass (20%–76%) favor chondrosarcoma Pain in enchondroma – 40% ➢ Often related to activity ➢ Stress microfracture ➢ Vague longer duration Is pain referable to lesion? Radiologic consultation

Enchondroma vs. Low-Grade Chondrosarcoma: Pathology • • • • •

Permeation of chondroid tissue Permeation of cortex Soft tissue mass Fibrous bands separating cartilage Invasion of marrow fat

• • • • • • •

Size < 6–7cm (X-ray); < 5cm (CT/MRI) Bone scan =/< AIC* 79% (70% homogeneous) Majority in diaphysis Endosteal scalloping depth < 2/3 cortex (90% - 95%) No cortical thickening (17%); periosteal reaction (3%) No cortical destruction/soft tissue mass MRI peripheral enhancement?

Long Bone Enchondroma : Imaging

Multiple areas of osteonecrosis with serpentine peripheral calcification (arrows) and simulating chondroid (ring and arc) mineralization

Murphey, Radiographics 98; 18: 1213 * *AIC = Anterior Iliac Crest • • • •

Figure 4-6-49

Long Bone Chondrosarcoma: Imaging

• •

Majority in the metaphysis Size > 6–7cm (X-ray); > 5cm (CT/MRI) Bone scan =/ > AIC* 82% (63% heterogeneous) Endosteal scalloping depth > 2/3 cortex (75% 90%) Cortical thickening (47%); periosteal reaction (51%) MRI peripheral and septal enhancement? CT of osteonecrosis shows peripheral rim of serpentine calcification. Simulation of chondroid mineralization is an artifact of radiographs in looking at a three dimensional structure with a one dimensional image

Murphey, Radiographics 98; 18: 1213 * *AIC = Anterior Iliac Crest References 1. 2. 3. 4. 5. 6.

Bloem JL, Mulder JD. Radiol. 1985;14(1):1-9. Chondroblastoma: a clinical and radiological study of 104 cases. Skeletal Radiol. 1985;14(1):1-9. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation. Radiographics. 2000 Sep-Oct;20(5):1407-34. Review. Murphey MD, Flemming DJ, Boyea SR, Bojescul JA, Sweet DE, Temple HT. Enchondroma versus chondrosarcoma in the appendicular skeleton: differentiating features. Radiographics. 1998 Sep-Oct;18(5):1213-37; quiz 1244-5. Murphey MD, Walker EA, Wilson AJ, Kransdorf MJ, Temple HT, Gannon FH. From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 2003 Sep-Oct;23(5):124578. Review. Robinson P, White LM, Sundaram M, Kandel R, Wunder J, McDonald DJ, Janney C, Bell RS. Periosteal chondroid tumors: radiologic evaluation with pathologic correlation. AJR Am J Roentgenol. 2001 Nov;177(5):1183-8. Wilson AJ, Kyriakos M, Ackerman LV. Chondromyxoid fibroma: radiographic appearance in 38 cases and in a review of the literature. Radiology. 1991 May;179(2):513-8. Review. Erratum in: Radiology 1991 Aug;180(2):586.

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Fibrous Lesions of the Musculoskeletal System Mark D. Murphey, MD Fibrous Lesions · · · · · · ·

Figure 4-7-1

Fibroxanthoma (Nonossifying fibroma) Fibrous dysplasia Osteofibrous dysplasia / Adamantinoma Desmoplastic fibroma Fibromatosis Malignant fibrous fistiocytoma / fibrosarcoma Dermatofibrosarcoma Protuberans (DFSP)

Fibroxanthoma: Other Terms · · · ·

Fibrous cortical defect Nonossifying fibroma (NOF) Fibrous medullary defect Nonosteogenic fibroma

Fibroxanthoma [Figures 4-7-1 and 4-7-2] ·

...If the lesion has attained a fairly large size and has penetrated into and continues to grow in the medullary cavity, it ceases to be a mere fibrous cortical defect and is then known as a nonossifying fibroma (Jaffe 1958)

Fibroxanthoma · · · ·

Very common 20% F, 50% M, older than 2 years of age Children and adolescents; M > F Usually asymptomatic: only 2% of biopsied primary bone tumors Heal spontaneously with average “life span” 29 months

Typical nonossifying fibroma/fibroxanthoma in the tibia (arrow)

Figure 4-7-2

Fibroxanthoma: Pathology · · · · ·

Whorls/bundles of fibrous tissue Variable cellularity Giant cells Foam or xanthoma cells Areas hemorrhage/hemosiderin

Fibroxanthoma: Skeletal Location · · · · ·

Metaphyseal origin can migrate to diaphysis Long tubular bones – 90% Lesions around knee – 55% Tibia – 43%, femur – 38%, fibula – 8% Upper extremity uncommon – 8%, humerus – 5%

Fibroxanthoma: Radiology [Figures 4-7-3 to 4-7-5] · · · · · · · · ·

Eccentric cortically based lesion Longitudinal growth pattern Can extend or primarily involve medullary cavity Lobulated contour Expansile remodeling with trabeculation Cortex may appear permeated focally but no soft tissue mass Usually a rim of sclerosis Bone scan – minimal to mild uptake MR – can be low or high intensity on T2W images

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Nonossifying fibroma/fibroxanthoma with healing by extensive ossification (arrow)

Fibrous Lesions of the Musculoskeletal System

Figure 4-7-3

Figure 4-7-5

Nonossifying fibroma/fibroxanthoma in the distal femur on radiograph, gross specimen and histology

Figure 4-7-4 Nonossifying fibroma/fibroxanthoma in the fibula with medullary location (arrow) as is typical for lesions in this location

Figure 4-7-6

Nonossifying fibroma/fibroxanthoma in the distal femur on radiograph and multiple MR images. Note intracortical location, heterogeneous signal intensity on T2 with areas of high signal and enhancement

Interval growth of nonossifying fibromas/fibroxanthomas and multiple lesions (arrows) between two radiographs

Figure 4-7-7 Fibroxanthoma: Natural History [Figures 4-7-6 and 4-7-7]

· · ·

Often heal with residual sclerosis start from diaphyseal side May persist or grow Pathologic fracture – greater likelihood in lesions > 3 cm and with >50% bone width involved and weight bearing bones

Fractures through three nonossifying fibromas/fibroxanthomas Fibrous Lesions of the Musculoskeletal System

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Musculoskeletal Radiology

Fibroxanthoma: Types and Associations [Figure 4-7-8] · · · ·

Figure 4-7-8

Solitary or multiple Multiple with neurofibromatosis type 1 Multiple with cafe-au-lait spots Jaffe-Campanacci syndrome Oncogenic osteomalacia

Benign Fibrous Histiocytoma [Figure 4-7-9] · · · ·

Use of this terminology controversial Patient often symptomatic Radiographic appearance – larger lesions, more expansion, medullary involvement, older patient Pathology identical to fibroxanthoma

Fibrous Dysplasia: Clinical Characteristics · · · · · · · · ·

Multiple nonossifying fibromas/fibroxanthomas associated with neurofibromatosis 1

Developmental anomaly of bone formation Osteoblasts fail to develop Marrow replaced by fibrosseous tissue Usually diagnosed < age 30 but > age 2 years Males and females equally affected Monostotic (70%–80%) Polyostotic (15%–30%) Cafe-au-lait spots – irregular serrated borders (coast of Maine) 1% of biopsied primary bone tumors

Figure 4-7-9

Fibrous Dysplasia: Monostotic · · · · ·

Smaller sized lesions Often asymptomatic Cafe-au-lait spots less common Distribution – femur (35%–40%), tibia (20%), skull and facial bones (10%–25%), ribs (10%) Uncommon sites – hands and feet, spine, clavicle

Fibrous Dysplasia: Polyostotic · · · · ·

Larger lesions, symptomatic at earlier age 70% present before age 10 – limp, pain, fracture or deformity Cafe-au-lait spots >50% patients Involvement variable – two to >75% skeleton; propensity to involve one side of body more extensively Common sites – skull and facial bone (>50%), long bones, ribs, pelvis

Fibrous Dysplasia Polyostotic: Associations · · · ·

Endocrinopathies: 2%–3% patients McCune Albright syndrome – bone lesions, cutaneous pigmentation, precocious puberty – 20%–50% of females (only 1 in 30 to 40 have complete triad) Others – hyperthyroidism, hyperparathyroidism, acromegaly, diabetes m., Cushing syndrome Soft tissue myxoma – Mazabraud syndrome

Fibrous Dysplasia: Pathologic Characteristics · · · · ·

Benign fibrous histiocytoma in the tibia with prominent sclerotic margin (unusual for GCT). On MR, prominent low signal intensity on both pulse sequences This lesion is identical pathologically to nonossifying fibroma/fibroxanthoma but patients are symptomatic and older age at presentation

Fibrosseous metaplasia Stroma may have cystic or myxoid elements Trabeculae are pure woven bone with “alphabet soup” appearance Occasional osteoblastic rimming and chondroid foci May have ABC component

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Fibrous Dysplasia Radiology: Appendicular Skeleton [Figures 4-7-10 to 4-7-12] · · · · · · ·

Medullary diaphyseal lesions Radiolucent with woven bone in marrow creating “ground glass appearance” Expansile remodeling Usually well defined and may have sclerotic rind (monostotic lesions) May have “multiloculated appearance” caused by subperiosteal reinforcement Areas of sclerosis (most common in skull) Skeletal deformity – fracture, bowing (Shepherd’s Crook), growth disturbance (more common polyostotic disease)

Figure 4-7-10

Figure 4-7-11 Typical "ground glass" appearance in several patents with fibrous dysplasia

Figure 4-7-12

Typical monostotic fibrous dysplasia in intertrochanteric femur with thick rind of sclerosis (arrow)

Fibrous dysplasia of rib on radiograph and macrosection showing elongated involvement (*) of a prominent osseous extent

Fibrous Dysplasia Radiology: Craniofacial Skeleton [Figures 4-7-13 to 4-7-15] · · · ·

Commonly involved – frontal, sphenoid, ethmoid, maxilla, zygoma, parietal, occipital and temporal Often mixed lucency and sclerosis Sclerosis often marked at skull base – can impinge on cranial nerves Calvarium expanded with greater involvement Figure 4-7-14 outer table

Figure 4-7-15

Figure 4-7-13

Fibrous dysplasia of the calvarium with skull base sclerosis (*) and expansion of the occipital outer table (arrow) Fibrous Lesions of the Musculoskeletal System

Coronal CT reconstruction of fibrous dysplasia shows mixed lysis and sclerosis and outer table expansion 774

Fibrous dysplasia on T2-weighted MR with prominent low signal intensity in the frontal bone with expansile remodeling (*) Musculoskeletal Radiology

Fibrous Dysplasia Radiology: Other Studies

Figure 4-7-16

[Figures 4-7-16 and 4-7-17]

· · ·

Bone scan – usually increased activity probably more variable than recognized CT – especially helpful in skull MRI ➢ 20% low intensity T2W images ➢ 20% same as fat T2W images ➢ 60% high intensity T2W images

Figure 4-7-17a

Fibrous dysplasia of the humerus with typical intense uptake of radionuclide bone scan

Figure 4-7-17b

Fibrous dysplasia of femoral diaphysis with nonspecific marrow replacement (*) on coronal T1weighted MR image

Fibrous Dysplasia: Complications [Figure 4-7-18] · · · ·

Malignant transformation – 0.5% Osteosarcoma most frequently but also MFH/fibrosarcoma and chondrosarcoma Both polyostotic and monostotic Prior radiation in 30%

Fibrous dysplasia of femoral diaphysis with nonspecific marrow replacement and high signal intensity on coronal T2-weighted MR image (*) (same patient as previous MR)

Fibrous Dysplasia: Differential Diagnosis · · · · · · · ·

Bone cyst Fibroxanthoma (medullary) Meningioma Osteoblastoma (long bone) Enchondromatosis Paget disease Langerhans cell histiocytosis Neurofibromatosis

Figure 4-7-18

Osteofibrous Dysplasia: Previous Terms · · · ·

Cortical fibrous dysplasia Intracortical fibrous dysplasia Ossifying fibroma – don’t confuse with facial lesion Juvenile adamantinoma

Osteofibrous Dysplasia: Clinical Characteristics · · ·

Unusual lesions – 0.2% of biopsied primary bone tumors Patients <10 years age; rare after 16 years Tibia alone (75% - 80%) or also fibula (12%); fibula only (7%), both tibiae (3%), rarely radius/ulna

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Monostotic fibrous dysplasia of the proximal femur with malignant transformation to MFH on radiograph and gross specimen. Note ground glass appearance distally (*) and more aggressive bone destruction proximal with extension through lesser trochanter proximally (arrows)

Fibrous Lesions of the Musculoskeletal System

Osteofibrous Dysplasia: Pathology · · · ·

Figure 4-7-19

Vascularized fibrous stroma like fibrous dysplasia Prominent osteoblastic rimming No “alphabet soup” of woven bone Can be weakly keratin positive but no epithelial nests

Osteofibrous Dysplasia: Radiology [Figures 4-7-19 and 4-7-20]

· · · · · ·

Lytic lesion anterior cortex mid tibial diaphysis May involve medullary canal No soft tissue mass Expansile remodeling and sclerotic component Causes bowing, fracture, pseudarthrosis and may progress to involve entire tibia Homogeneous intermediate on T1 and high on T2 ➢ Homogeneous mild to moderate enhancement

Adamantinoma: Clinical Characteristics · · · · ·

Typical osteofibrous dysplasia with elongated/multifocal intracortical tibial involvement on radiograph and matched macrosection (*)

Figure 4-7-20

Present with pain/swelling; often history of trauma Don’t confuse with mandibular ameloblastoma Rare low grade malignancy – 0.1% biopsied primary bone tumors Also previously called angioblastoma Male to female ratio 1.3:1, average age 35 years

Adamantinoma: Pathology and Location

· · · ·

Epithelial nests / prominent keratin staining Background of bland fibrous stroma May have foci of Ewing-like areas – worse prognosis Tibia (80% - 85%), tibia and fibula (5%), femur (5%), humerus (4%), ulna (3%), fibula (1%)

Typical osteofibrous dysplasia on MR imaging with elongated intracortical tibial involvement and homogeneous intermediate signa l intensity on T1-weighting and high signal on T2weighting (arrows).

Adamantinoma: Radiology · · · ·

Diaphyseal to metadiaphysis – anterior tibial cortex Mixed lytic and sclerotic May be multifocal with medullary involvement and soft tissue mass Expansile remodeling with cortical thickening

Figure 4-7-21

Adamantinoma: Radiology and Prognosis [Figures 4-7-21 to 4-7-24] ·

· · ·

MRI ➢ Very heterogeneous high intensity T2W ➢ Vascularity with prominent enhancement Locally aggressive 10 year survival: 10% - 65% 15% patients die with metastases

Osteofibrous Dysplasia: Relationship to Adamantinoma ·

· ·

Differentiation - patient age ➢ Multiple recurrence ➢ MRI heterogeneous, intense enhancement Epithelial nests; both can be keratin positive Several cases reported of foci of adamantinoma in osteofibrous dysplasia and progression to adamantinoma Adamantinoma of the tibia on radiograph with mixed lytic and sclerotic lesion centered in the cortex and an elongated lesion

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Figure 4-7-22

Figure 4-7-23

Adamantinoma of the tibia on multiple MR images (same patient as previous radiograph and CT) with elongated lesion centered in the cortex (arrows). There is prominent heterogeneity on STIR

Adamantinoma of the tibia on several CT images (same patient as previous radiograph) with mixed lytic and sclerotic lesion centered in the cortex (arrows)

Desmoplastic Fibroma: Clinical Characteristics · · · · ·

Rare fibrous lesion of bone 0.2% - 0.3% biopsied primary bone neoplasms M=F or slight female predilection 70% between ages 15 and 40 years Desmoid tumor of bone (5–10 times less common than soft tissue lesion)

Figure 4-7-24

Desmoplastic Fibroma: Clinical Characteristics · · · ·

Location – femur, tibia, humerus, radius, mandible, pelvis Metaphyseal – central Pain and swelling (90%) Pathologic fracture (15%)

Desmoplastic Fibroma: Pathology · · · ·

·

Intraoperative photograph of adamantinoma of the tibia with intracortical curretage (same patient as previous radiograph, MR and CT)

Figure 4-7-25

Histology identical to soft tissue desmoid Gross - lobular firm white to gray mass Fibroblasts producing well-formed collagen Nuclear monotony, variable cellularity, rare mitosis Rarely associated with fibrous dysplasia

Desmoplastic Fibroma: Radiology [Figure 4-7-25]

· · · · ·

Lytic lesion with expansile remodeling May have sclerotic margin Internal trabeculae - subperiosteal reinforcement May have more aggressive appearance May be low intensity T2W MR images

Cortical Desmoid [Figures 4-7-26 and 4-7-27 overleaf] · · ·

· · · · · ·

Desmoplastic fibroma of the iliac bone with prominent multilocular appearance caused by internal trabeculation

Avulsive cortical injury (chronic) Posteromedial distal femur metaphysis Stress related at attachment ➢ Adductor magnus ➢ Medial head gastrocnemius Pathology simulates aggressive lesion Children 1st decade (35%) More frequent in boys, often bilateral Surface irregularity/lucency CT – looks like NOF – no soft tissue mass MRI – may see surrounding inflammation

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Figure 4-7-26

Figure 4-7-27

Two patients with cortical desmoids (chronic avulsive injury) of the distal femur (arrows) Sagittal CT reconstruction showing medial head of gastrocnemius muscle extending into cortical desmoid (arrow) and macrosection of reparative tissue (*) in a different patient

Fibromatosis · · ·

Deep – grow rapidly, larger, more aggressive Superficial – slow growing, small, arise from fascia/aponeurosis Can be multifocal 5%-20%

Fibromatosis: Types · · · · · ·

Extra-abdominal desmoid (deep) Aggressive infantile fibromatosis (deep) Juvenile aponeurotic fibroma (sup.) Infantile dermal/digital fibromatosis (sup.) Adult palmar and plantar (sup.) Infantile myofibromatosis (both)

Figure 4-7-28

Fibromatosis: Pathology · · · · ·

Gross - glistening white, variable cellularity Spindle shaped fibrous cells Abundant collagen, can see mitoses Infiltrative growth common No malignant potential

Post-contrast axial T1-weighted MR image showing extraabdominal desmoid (fibromatosis) with an enhancing paraspinal mass (*) and ill-defined margins

Fibromatosis: Radiology · · · ·

·

Soft tissue mass, unusual to calcify Can erode adjacent bone CT-soft tissue mass – may show attenuation greater than muscle MRI ➢ T1W image – low / intermediate signal ➢ T2W image – variable signal ➢ Fascial tail sign ➢ Low signal bands Enhance with contrast

Figure 4-7-29

Axial STIR MR image showing extraabdominal desmoid (fibromatosis) with a soft tissue mass (*) and ill-defined margins and linear extension (fascial tail sign) laterally (arrow). These features are also shown on a gross specimen froma different patient Fibrous Lesions of the Musculoskeletal System

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Fibromatosis: Extraabdominal Desmoid [Figures 4-7-28 and 4-7-29] · · · ·

Painless growing deep mass (25–35 years) Rarely familial Shoulder (20%), chest wall/back (15%), thigh (12%), mesentery (10%), neck (8%), knee (7%), buttock (6%) Intermuscular along fascia/aponeurosis, infiltrative

Figure 4-7-30

Fibromatosis: Aggressive Infantile [Figures 4-7-30 to 4-7-32]

· · · · · ·

Painless soft tissue mass, M>F Discovered first two years of life Intermuscular – head/neck, shoulder, thigh, foot Can erode bone Multifocal (10%-15%) No metastasis but locally aggressive and recur

Fibromatosis: Juvenile Aponeurotic Fibroma · · · · ·

Children/adolescents; M>F Hands (77%), feet (13%) – palms and soles Painless slowly growing mass Calcification and local recurrence (50%) common Attached to tendon/aponeurosis

Aggressive infantile fibromatosis on sagittal T1-weighted MR image with large mass eroding bone (*) which ultimately led to amputation following multiple recurrences.Note low intensity bands (arrowheads)

Figure 4-7-31

Fibromatosis: Infantile Dermal/Digital · · · · ·

Birth to age 2 years; F>M Fingers > toes; dorsum, spare thumb/great toe Bone erosion rare, can have contractures Pathology – intracellular inclusion bodies Recurrence local 60%

Adult Palmar Fibromatosis · · · · ·

Palmar (Dupuytren contracture) 1%–2% population (1/5 people older than 65) M>F (4–5:1); ulnar side, thumb and index finger spared Fibrous nodules - cords (40%–60% bilateral) Other fibromatosis 5%–20% Contractures/recurrence common

Figure 4-7-32

Same patient as previous image showing multicentric involvement with second site (*) in lower calf demonstrating high signal intensity on axial T2-weighted MR image. The patient ultimately required amputation as shown on the gross specimen with the large recurrent mass (*)

Axial T2-weighted MR image in patient with aggressive infantile fibromatosis after radiation shows marked low signal (arrows) resulting from collagenization following successful nonoperative treatment Musculoskeletal Radiology

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Adult Plantar Fibromatosis · · · · · ·

Figure 4-7-33

Ledderhose disease Less common than palmar M>F (2:1); wider age range 55% < age 30 Starts as single nodule middle to medial sole Often leads to early excision, contractures rare Associated with palmar disease 5%–20%

Infantile Myofibromatosis · · · · ·

Discovered at birth or within weeks Solitary form (good prognosis) Multifocal (poor prognosis) – soft tissue, muscle, viscera Bone lesions common but involute Lesions grow in perinatal period Myoblastic and fibroblastic lesion

Osseous MFH with radiograph showing solitary geographic lytic lesion with wide zone of transition (arrows)

Fibromatosis: Treatment and Prognosis · · · · ·

Surgical excision Recurrence common High signal on T2 corresponds to more cellular regions increasing recurrence Re-excision may use radiation therapy ➢ Can follow with MRI Can ultimately require amputation

Figure 4-7-34

Malignant Fibrous Histiocytoma (MFH) and Fibrosarcoma · ·

Osseous Soft tissue (S.T.)

Malignant Fibrous Histiocytoma: Pathology · ·

· · ·

Described 1964 – Stout and coworkers CT of osseous MFH shows no matrix Three cell types present mineralization and cortical penetration ➢ Fibroblastic spindle cells anteriorly with soft tissue mass (*) ➢ Plump histiocytic cell (from marrow monocytes?) (same patient as previous image) ➢ Giant cells benign and malignant Histologic types–storioform (pleomorphic 50%–60%), myxoid (25%), giant cell (10%), inflammatory (10%), angiomatoid (5%) No malignant matrix; diagnosis of exclusion Figure 4-7-35 WHO 2002 – Undifferentiated high grade pleomorphic sarcoma (soft tissue lesions only)

Fibrosarcoma: Pathology · · · ·

Malignant collagen producing spindle cells “Herringbone” pattern – lower grade lesions (I-II) Higher grade (III-IV) lesions – more anaplasia No matrix or malignant giant cells

Osseous MFH and Fibrosarcoma: Clinical Features · · · · ·

Age 40–70 years Pain, swelling and pathologic fracture (30%–50%) Slight male predilection Metaphyseal – around knee (40%–80%), humerus (10%), pelvis (10%) Secondary lesions – Paget disease, osteonecrosis, fibrous dysplasia, chronic osteomyelitis

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Coronal T1-weighted MR image and coronal gross specimen shows distal femoral MFH (same patient as previous two images) not extending to subchondral bone (arrowhead) (as would be seen with giant cell tumor)

Musculoskeletal Radiology

Osseous MFH and Fibrosarcoma: Radiology

Figure 4-7-36

[Figures 4-7-33 to 4-7-38]

· · · ·

Lytic lesions – from geographic IB to motheaten/permeative – reflects tumor grade With geographic IB - IC lesions – GCT like; look for diaphyseal > epiphyseal extension Little periosteal reaction or sclerosis May not show increased intensity on T2W MR images

Soft Tissue MFH: Clinical Features · · · ·

Most common adult S.T. sarcoma Accounting for 15%–30% all S.T. sarcomas Age 50–70 years: M>F 2:1 Location – deep soft tissues – lower extremity (50%), upper extremity (20%), retroperitoneum (15%), head and neck (5%)

Musculoskeletal Soft Tissue Sarcoma Incidence · · · · · · ·

Osseous MFH showing aggressive solitary lytic lesion (arrows) in supraacetabular region with lateral cortical destruction difficult to detect

Figure 4-7-37

Figure 4-7-38

Malignant fibrous histiocytoma and Fibrosarcoma 20%–30% Liposarcoma 16%–19% Rhabdomyosarcoma 10%–19% Nonspecific spindle cell sarcoma 5%–15% Leiomyosarcoma 5%–10% Dermatofibrosarcoma protuberans (DFSP) 5%–10% Synovial sarcoma 5%–10%

Soft Tissue Fibrosarcoma: Clinical Features · · ·

Palpable mass – deep soft tissues Lower extremity – knee and thigh (45%); upper extremity (28%), trunk (17%), head and neck (10%) Age– 30 to 55 years, no sex predilection

Osseous MFH (same patient as previous image) with intraosseous lesion and large associated soft tissue mass (*) but no matrix mineralization

Axial T1-weighted MR image of osseous MFH (same patient as previous two images) shows marrow replacement and associated soft tissue mass (*)

Soft Tissue MFH and Fibrosarcoma: Radiology [Figures 4-7-39 to 4-7-42]

· ·

· · · · ·

Deep soft tissue mass – MRI > CT for evaluating extent prior to surgery MRI ➢ Usually similar to muscle T1W images ➢ High intensity T2W images ➢ May not show increased intensity on T2W images Pseudocapsule – low intensity all sequences Calcification/ossification: 5%–20% MFH May involve underlying bone MFH – hemorrhage – high intensity T1W images Can differentiate myxoid lesions look like fluid with nodular peripheral contrast enhancement

Figure 4-7-39

Soft tissue MFH with mass replacing vastus lateralis muscle (*) on axial T1-weighted MR image [left]; with high signal intensity mass (*) on axial T2-weighted MR image [right] Musculoskeletal Radiology

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Figure 4-7-40

Figure 4-7-41 T1

Sagittal T1-weighted MR images before and after contrast show enhancement of the solid component (arrowheads) of the MFH and nonenhancing hemorrhagic areas (*)

Largely hemorrhagic (*) soft tissue MFH in the anterior thigh on CT with the only solid component adjacent to the anteromedial femur (arrowhead)

Figure 4-7-42

Fibrosarcoma and MFH: Therapy and Prognosis · · ·

T1 GD

Treatment - wide local resection/amputation Local recurrence common (50%) follow up imaging Metastasis (40%) common hematogenous - lung, lymph nodes, liver and bone

Dermatofibrosarcoma Protuberans (DFSP): Clinical Features · · · · ·

6% all soft tissue tissue sarcomas Third to fifth decades of life Reddish brown to bluish superficial nodule May be multiple Most common to affect trunk (50%) ➢ Remainder head/neck, upper/lower extremities

Soft tissue MFH with soft tissue mass (*) causing extrinsic erosion of adjacent femur (arrowheads)

Dermatofibrosarcoma Protuberans (DFSP): Pathology · · ·

Uniform fibroblasts Storiform pattern (may be myxoid) May have areas of higher-grade sarcoma ➢ Usually fibrosarcoma (17% - 27%)

Dermatofibrosarcoma Protuberans (DFSP): Radiologic Characteristics [Figure 4-7-39] · · ·

· ·

Subcutaneous mass - no calcifications Usually centered on skin and protuberant Nonspecific solid intrinsic features CT/MRI ➢ Enhance with contrast, ST attenuation ➢ Intermediate T1; high signal T2 May have hemorrhage Look for linear extension (skin/fascial tail sign) ➢ Satellite nodules

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Figure 4-7-43 T1

DFSP on sagital T1, axial STIR and gross specimen showing protuberant subcutaneous mass (*) involving the skin with linear extensions along the skin surface (arrows)

Dermatofibrosarcoma Protuberans (DFSP): Treatment and Prognosis • •

•

Surgical excision (wide with 3cm margin) Local recurrence 20%-55% (within 3 years) ➢ Higher with head/neck lesions (50%-75%) Metastases ➢ Lungs (5%-6%) ➢ Lymph nodes up to 25% of metastases ➢ Higher incidence with high-grade component (21%)

Fibrous Lesions References 1. 2. 3. 4. 5. 6. 7. 8.

Fitzpatrick KA, Taljanovic MS, Speer DP, Graham AR, Jacobson JA, Barnes GR, Hunter TB. Imaging findings of fibrous dysplasia with histopathologic and intraoperative correlation. AJR Am J Roentgenol. 2004 Jun;182(6):138998. No abstract available. Jee WH, Choe BY, Kang HS, Suh KJ, Suh JS, Ryu KN, Lee YS, Ok IY, Kim JM, Choi KH, Shinn KS. Nonossifying fibroma: characteristics at MR imaging with pathologic correlation. Radiology. 1998 Oct;209(1):197-202. Jee WH, Choi KH, Choe BY, Park JM, Shinn KS. Fibrous dysplasia: MR imaging characteristics with radiopathologic correlation. AJR Am J Roentgenol. 1996 Dec;167(6):1523-7. Murphey MD, Gross TM, Rosenthal HG. Musculoskeletal malignant fibrous histiocytoma: radiologic-pathologic correlation. RadioGraphics 1994; 14:807-826. Ritschl P, Karnel F, Hajek P. Fibrous metaphyseal defects--determination of their origin and natural history using a radiomorphological study. Skeletal Radiol. 1988;17(1):8-15. Robbin MR, Murphey MD, Temple HT, Kransdorf MJ, Choi JJ. Imaging of Musculoskeletal Fibromatosis. RadioGraphics 2001; 21:585-600. Torreggiani WC, Al-Ismail K, Munk PL, Nicolaou S, O'Connell JX, Knowling MA. Dermatofibrosarcoma protuberans: MR imaging features. AJR Am J Roentgenol. 2002 Apr;178(4):989-93. Van der Woude HJ, Hazelbag HM, Bloem JL, Taminiau AH, Hogendoorn PC. MRI of adamantinoma of long bones in correlation with histopathology. AJR Am J Roentgenol. 2004 Dec;183(6):1737-44.

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Alphabet Soup and Cystic Lesions of The Bone Mark D. Murphey, MD • • • • • • •

Alphabet Soup and Cystic Lesions of the Bone

•

Giant cell tumor (GCT) Unicameral bone cyst (UBC) Aneurysmal bone cyst (ABC) Epidermoid inclusion cyst Subchondral cyst Intraosseous ganglion Post-traumatic cyst

Giant Cell Tumor (GCT): Clinical Features • • • • •

•

Approximately 5% of all biopsied primary bone tumors; 18%-23% of benign bone neoplasms Symptoms – pain and swelling often relieved by decreased activity Pathologic fracture 10%–35% Adults – 80% between 20–50 Years Rare in children 1%–3% (<14 years) Sex distribution ➢ F-M ratio 3:2 benign GCT ➢ M-F ratio 3:1 aggressive GCT

Figure 4-8-1

Giant Cell Tumor: Location • • • • • •

Originate metaphyseal side of growth plate and grow to subchondral bone (84%–99%) Long tubular bones 75%–90% About the knee 50%–65%; distal femur 23%-30%; proximal tibia 20%-25% Radius (10%-12%); humerus (4%-8%) Spine – (7%-15%) - vertebral body – sacrum-thoracic-cervicallumbar Pelvis (4%); hands/feet (5%) Multifocal (0.5%–1%)– skull and face (Paget disease), Goltz syndrome

• • • •

Osteoclast like giant cells (90%) Mononuclear spindle cell stromal component Hemorrhage, necrosis and hemosiderin ABC like areas 10%–15%

• • • • • •

GCT/ABC/UBC NOF/CMF/OGS Brown tumor HPT/chondroblastoma Fibrous dysplasia and variants Osteoblastoma Giant cell reparative granuloma

Giant cell tumor (GCT) of the proximal tibia with geographic lytic lesion with a mild rim of partial sclerosis (arrows-unusual in GCT), mild expansile remodeling and increased radionuclide uptake on bone scan (right image)

Figure 4-8-2

Giant Cell Tumor: Pathology

Osseous Lesions Containing Giant Cells

•

Giant Cell Tumor: Radiology [Figures 4-8-1 to 4-8-10] • • •

CT of giant cell tumor shows no mineralized matrix (*) (same patient as previous radiograph)

Solitary eccentric geographic lytic lesion extending into subchondral bone Center of lesion-metaepiphysis Margin IB (80%-85%), IC (10%-20%), IA (1%-2% but up to 20% by CT) No mineralized matrix

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• • • • • • •

Expansile remodeling (47%-60%) with apparent cortical permeation (33% - 50%) ➢ Septations - subperiosteal new bone ➢ Periosteal reaction unusual 10%–30% Radiologic characteristic do not reflect clinical behavior of GCT Bone scan - doughnut sign (57%) Usually a vascular lesion (75%-90%) MRI>CT for evaluation of extent Fluid-fluid levels Low to intermediate intensity usually predominates on T2W images (90%–95%)

Figure 4-8-3

Figure 4-8-4 Giant cell tumor of distal femur shows well defined geographic (1B margin-arrow)) lysis extending to subchondral bone. Bone scan reveals marked increased uptake in femur. Bone scan reveals marked increased uptake in the femoral GCT but also in the adjacent tibia and patella. The increased uptake in the tibia and patella are due to hyperemia and disuse, not tumor involvement

Figure 4-8-5

Axial T1-weighted MR image shows marrow replacement and small anterior soft tissue mass (*) resulting from this benign giant cell tumor (same patient as previous radiograph and bone scan).

Figure 4-8-6

Sagittally sectioned gross specimen and macrosection show identical findings as on the previous images of this benign giant cell tumor including extension to subchondral bone (arrows) and anterior soft tissue component (*)

Figure 4-8-8

Figure 4-8-7

Giant cell tumor of the fibula with marked expansile remodeling of bone

Musculoskeletal Radiology

Coronal T2-weighted MR image shows intermediate to low (*) signal intensity tissue typical of giant cell tumor

785

Giant cell tumor of the patella (sesamoids and apophysis are epiphyseal equivalents for the differential diagnosis of lytic lesions)

Alphabet Soup and Cystic Lesions of Bone

• • • • •

Sacral Lesions: Differential Diagnosis

• • • •

Figure 4-8-9

GCT/ABC Metastasis Myeloma/plasmacytoma Chordoma Neurogenic tumor

Giant Cell Tumor: Treatment and Prognosis

• • • • • • •

Curettage and cryosurgery or en bloc resection and bone graft Local recurrence rate 40%–60% historically Current recurrence rate 2%–25% Recurrence does not correspond to radiologic or microscopic appearance Osseous recurrence - new bone destruction Soft tissue recurrence - mass and may calcify/ossify about periphery May metastasize - 2%-5% (50% benign histology) Malignant GCT 10%–15% (much <5% in our experience) (more common with radiation)

Giant cell tumor of the sacrum with predominantly low to intermediate signal intensity (*) on the axial T2-weighted MR image

Figure 4-8-10

Giant Cell (Reparative) Granuloma

• •

Rare benign lesion described in 1953 by Jaffe Mandible/maxilla and hands/feet Phalanges > metacarpal > metatarsal > carpus > tarsus Women > men (jaw), age to 10 - 50 years May have history trauma

Giant Cell (Reparative) Granuloma: Pathology • • • •

Granuloma - like arrangement of fibroblastic stroma and osteoid on micro Metadiaphyseal lytic lesion Expansile remodeling and trabeculation Recurrence only if incompletely excised

• • • •

Similar to GCT May not extend to subchondral bone (hand) Expansile remodeling and trabeculation May detect small amount of mineralization

Giant cell tumor of the sacrum with predominantly intermediate signal intensity (*) on the sagittal T1-weighted MR image and large associated soft tissue mass (*) correlating identically with the sagittally sectioned gross specimen

Giant Cell (Reparative) Granuloma: Radiology [Figure 4-8-11]

•

Figure 4-8-11

Unicameral Bone Cyst: Simple Bone Cyst A fluid - containing lesion lined by mesothelial (epithelial-like) cells usually arising in metaphysis of long bone adjacent to physis

• • • •

3% of all biopsied primary osseous neoplasms Young patients 85% < 20 years M>F; 2:1 Pathologic fracture 50%

• • •

Clear, straw - colored fluid filled cyst Cyst lining - thin flat epithelial - like cells - mesothelial origin Complicated cysts - hemorrhage, fibro-osseous repair tissue

Simple Bone Cyst: Clinical Features

Simple Bone Cyst: Pathology

Giant cell (reparative) granuloma in the second metacarpal

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•

Simple Bone Cyst: Location and Etiology • • • • • • •

Figure 4-8-12

Under age 20 - humerus (55%–65%), femur (25%–30%), tibia, fibula, radius and ulna rare Over age 20 - iliac bone and calcaneus Cause - lymphatic or venous obstruction vs. synovial origin

Simple Bone Cyst: Radiology [Figures 4-8-12 to 4-8-14]

• •

• • • • •

Geographic IA lesion - originate in central metaphysis (active) Can migrate into the diaphysis (latent) Mild expansile remodeling Not infrequently multilocular Pathologic fracture ➢ ”Fallen fragment” sign (5%) CT/MRI- noncomplicated see simple fluid ➢ Thin wall and septal enhancement beware delayed MR imaging with diffusion CT/MRI- complicated case ➢ Solid components, thick walls, fluid level

Simple Bone Cysts: Treatment and Course

•

Spontaneous regression or heal after fracture Curettage and bone grafting Intralesional steroids (70%–95% effective) Recurrence 20%–40% Extremely rare-malignant transformation

Simple bone cyst with fracture (arrow) and “fallen fragment” sign (curved arrow)

Figure 4-8-13

Aneurysmal Bone Cyst (ABC): Definition “The so called aneurysmal bone cyst is neither a cyst nor a neoplasm; rather it is probably a periosteal to intraosseous arteriovenous malformation not uncommonly seen in association with other well known benign and even malignant lesions.”

Mirra JM. Bone Tumors. Lea & Febiger 1989 • • •

Aneurysmal Bone Cyst: Clinical Features

• • • •

1% of all biopsied primary osseous neoplasms 80% between ages 5 and 20 years Patients present with pain, swelling, and pathologic fracture (10%–20%) May be associated with trauma Slightly more common in women

Aneurysmal Bone Cyst: Secondary Lesion •

Simple bone cyst in the calcaneus (arrow)

Figure 4-8-14

1%–32% of cases Benign lesions - chondroblastoma, CMF, NOF, GCT, fibrous dysplasia, UBC, brown tumor, hemangioma, giant cell reparative granuloma Malignant lesions - hemangioendothelioma, telangiectatic osteosarcoma, chondrosarcoma

Osseous Lesions with Prominent Fluid Levels Differential Diagnosis • • • • • •

Aneurysmal bone cyst (only fluid levels) Giant cell tumor (to bone end,metaphyseal center) Chondroblastoma (epiphyseal center) Osteoblastoma (posterior elements spine) Telangiectatic Osteosarcoma (thick walls, osteoid on CT) Fibrous dysplasia (diaphysis, “ground glass”)

• • •

Gross - “Blood-filled sponge” Cavernous blood filled spaces line by fibrous walls May see chondrosseous tissue indicating repair

Aneurysmal Bone Cyst: Pathology

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Simple bone cyst in the calcaneus on CT with septation (arrowheads) Alphabet Soup and Cystic Lesions of Bone

• • • •

Long tubular bone 70%–80% Spine posterior elements - 15% (thoracic, lumbar, cervical, sacral) Pelvis 5%–10% Hands 10%–15%

• • • • • • • • • •

Only osseous neoplasm named for its radiologic appearance Metaphysis (80%–90%), eccentric medullary geographic lytic lesion Can appear central with expansion Diaphysis (10%–20%), often surface lesions Expansile remodeling uneven in distribution creating one aggressive margin Spine - expansion can lead to neurologic deficits Periosteal membrane intact on CT/MRI Bone scan - peripheral activity (65%) Fluid-fluid levels (CT/MRI)-nonspecific representing sedimentation of blood Angiography-hypovascular lesion with localized areas of increased vascularity

Aneurysmal Bone Cyst: Location

Aneurysmal Bone Cyst: Radiology [Figures 4-8-15 to 4-8-21]

Figure 4-8-16

Figure 4-8-15

Aneurysmal bone cyst (primary) with “donut” sign (increased uptake peripherally and photopenia centrally) on bone scintigraphy (same patient as previous radiographs)

Aneurysmal bone cyst (primary) with more prominent expansile remodeling of bone posteriorly (more aggressive appearancearrow) versus rim of sclerosis in other areas (indolent appearance-arrowheads)

Figure 4-8-18

Figure 4-8-17

T2

Aneurysmal bone cyst (primary) on axial T2-weighted MR image shows fluid levels (arrows) from hemorrhage in all parts of the lesion (same patient as previous bone scan)

Alphabet Soup and Cystic Lesions of Bone

Aneurysmal bone cyst (primary) with sagittal gross specimen showing blood filled spaces (*) lined by thin septae (arrows) (same patient as previous MRI)

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Figure 4-8-19

Figure 4-8-20 T1

T1 GD

Coronal pre and post contrast T1-weighted MR images showing thin enhancing periphery and septae (arrows) typical of a primary aneurysmal bone cyst

T1

T2

Coronal T1-weighted and sagittal T2-weighted MR images of a secondary (chondroblastoma) aneurysmal bone cyst with cystic areas containing fluid levels (arrows) and anterior solid component (*)

Figure 4-8-21

Giant cell tumor with ABC component on various MR pulse sequences with diffuse enhancement and intermediate signal intensity of the solid component (*) and rim enhancement, high signal and fluid level in the cystic component (arrows)

• Rarely spontaneous regression • Curettage, cryosurgery and bone grafting • Recurrence 10%–20% • Radiotherapy • Radiofrequency ablation with methylmethacrylate placement • Embolotherapy* Cory DA et al. AJR 1989; 153:369

Aneurysmal Bone Cyst: Treatment and Prognosis

• •

Aneurysmal Bone Cyst: Solid Variant? •

Recently described (1983); controversial Radiography - similar to other ABC’s but more often aggressive and axial location Histology-fibrous tissue proliferation, osteoid production, osteoclastic giant cells, sinusoids

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• • •

Epidermoid Inclusion Cyst [Figure 4-8-22]

• • • •

Figure 4-8-22

Two types Hand - distal phalanx-traumatic origin Radiographs - punched out lesion with surrounding sclerosis, dorsal cortex often absent Skull-intraosseous-frontal and temporal bone-congenital origin Radiographs - well defined lytic lesion sclerotic margin and can cause expansile remodeling of bone Pathology - stratified squamous epithelium

Subchondral Cyst • • • • • • • •

Other terms - geodes and synovial cyst; no true epithelial or synovial lining Middle to older aged patients Around joints and associated with other arthritic changes Etiology - synovial fluid intrusion vs. osseous contusion Can be large/solitary, articular damage subtle simulating neoplasm (GCT)

Intraosseous Ganglion [Figures 4-8-23 and 4-8-24]

• • • • •

Uncommon lesion; middle aged adults Pain increases with activity Periarticular, eccentric, geographic IA-B lytic lesion Tibia (medial malleolus), femur, about wrist (>65% of lesions) Pathology - same as soft tissue ganglion

Epidermoid inclusion cyst with welldefined terminal phalangeal lytic lesion (arrows)

Figure 4-8-23

Post-Traumatic Cyst [Figure 4-8-25] Occurs as complication of fracture in children Usually forearm - radius/ulna Caused by hemorrhage then fibrosis Radiolucent lesion well defined, may heal or persist

Figure 4-8-24

Intraosseous ganglion in the medial malleolus with geographic lysis (arrows) and thin sclerotic margin (1A)

Figure 4-8-25

Intraosseous ganglion in the subchondral region of the medial malleolus with intermediate signal intensity on T1-weighting and high signal intensity on T2-weighting (*) and septation

Post traumatic cyst in the radius subsequent to a fracture (arrows)

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References 1. 2. 3. 4.

Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol. 1995 Mar;164(3):573-80. Review. Martinez V, Sissons HA. Aneurysmal bone cyst. A review of 123 cases including primary lesions and those secondary to other bone pathology. Cancer. 1988 Jun 1;61(11):2291-304. Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ. From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation. Radiographics. 2001 Sep-Oct; 21(5):1283-309. Review. Parman LM, Murphey MD. Alphabet Soup: Cystic Lesions of Bone. Seminars in Musculoskeletal Radiology 2000; 4(1):89-101.

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Juxtaarticular Soft Tissue Masses Mark D. Murphey, MD •

Soft Tissue Masses In and About Joints • • • • • • •

Figure 4-9-1

Tumor like-tumoral calcinosis, PVNS, ganglion, synovial cyst, myositis ossificans Benign - synovial lipoma, myxoma, synovial chondromatosis/chondroma, nodular fasciitis Malignant-synovial sarcoma, clear cell sarcoma

Tumoral Calcinosis: Clinical Features

• • •

Usually children/young adults Increased incidence in blacks Familial tendency (33% of cases) Large calcified paraarticular mass, hip, shoulder, elbows, and feet Can be associated with CPPD arthropathy, pseudoxanthoma elasticum like syndrome Also skin ulceration, marrow and dental changes Etiology - metabolic (hyperphosphatemia and increased Vitamin D), trauma, idiopathic

Tumoral Calcinosis: Pathology • •

Gross-encapsulated multilocular mass, filled with viscous calcium hydroxyapatite Fibrous septations May have inflammatory elements

Tumoral calcinosis about the shoulder with large calcified periarticular mass (*) and radiolucent septations (arrowheads)

Figure 4-9-2

Tumoral Calcinosis: Radiology • • • • • •

[Figures 4-9-1 to 4-9-6]

• • • • • •

Calcified paraarticular mass Extensor surface Radiolucent septations (“chicken wire”) Extraarticular (bursae); no loss ROM Average 3 lesions/individual* CT/MRI: fluid-fluid levels (liquefied calcium) ➢ More active disease Bone scan-best for detection, and localization MRI – low signal T1W images ➢ Variable low to high signal T2W images Pseudoxanthoma elasticum-skin/vascular calcification, retina angioid streaks CPPD arthropathy Dental abnormalities-root enlargement, intrapulp calcification Marrow involvement - calcific myelitis

Tumoral calcinosis about the knee

Figure 4-9-3

*Martinez, Radiology 1990;174:215

Clinical photograph in patient with tumoral calcinosis about the knee (same patient as previous radiograph) showing cosmetic deformity but no decreased range of motion Juxtaarticular Masses

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Figure 4-9-4

Figure 4-9-5

Tumoral calcinosis about the elbow extensor surface. Contralateral elbow revealed identical findings (not shown)

Tumoral calcinosis about the hip with calcium fluid levels (arrowheads) on CT

Figure 4-9-6

Tumoral calcinosis about the shoulder with large calcified periarticular mass (*) showing peripheral and septal enhancement after contrast (arrowheads)

• • • • •

Scleroderma Other collagen vascular diseases Chronic renal failure (secondary tumoral calcinosis) Milk - Alkali syndrome Synovial sarcoma

• •

Phosphate depletion therapy Surgical excision

• • • •

Proliferative disorder of synovium of joint, tendon or bursa Young adults 3rd and 4th decades Two types diffuse (15%–25%) and localized (75%–85%) Symptoms - pain, swelling, ROM loss

Periarticular Calcification: Differential Diagnosis

Tumoral Calcinosis: Treatment

Pigmented Villonodular Synovitis (PVNS): Clinical Features

• •

P V N S: Pathology •

Etiology unknown - inflammatory/neoplasm/trauma Variable degree of villous/nodular synovial proliferation and pigmentation (hemosiderin) and inflammation components Giant cells, fibrous tissue, xanthoma cells

Musculoskeletal Radiology

793

Juxtaarticular Masses

•

P V N S: Location

Figure 4-9-7

•

Localized form – usually extraarticular ➢ Giant cell tumor tendon sheath (GCT-TS) ➢ Hand (80%), feet, knee (12%) Diffuse form - knee (60%–80%) hip, ankle, shoulder, elbow

• • • •

Second most common mass hand/wrist Lobulated soft tissue mass < 2 cm More common volar surface Osseous erosion uncommon 10%–15%

• • • •

Erosive bone lesions - 50% : hip (93%), shoulder (75%), knee (26%) Geographic IA lytic lesion - extrinsic erosion Joint effusion Arthrography - brownish or chocolate fluid, multinodular filling defects

GCT-TS: Radiology

P V N S - Diffuse Form: Radiology

• • • •

P V N S: Radiology [Figures 4-9-7 to 4-9-11] Bone scan - mild increase activity (statics) Angiography - can show impressive vascularity CT - soft tissue mass, increased attenuation MRI – T1W image – low intensity mass T2W image – variable – usually prominent low intensity regions

Figure 4-9-8

Localized form of PVNS (giant cell tumor of tendon sheath) with volar thumb mass (*) on sagittal T1-weighted MR image

Figure 4-9-9

Same patient as previous MR image with intermediate signal intensity in the giant cell tumor of tendon sheath (*) of the thumb on axial T2-weighted MR

PVNS of the hip (diffuse type) with radiograph showing erosions on both sides of the joint (arrows) and maintained joint space

Figure 4-9-10

PVNS hip (same patient as previous radiograph) with marked low signal intensity tissue on coronal T2-weighted MR image (*)

Juxtaarticular Masses

794

Musculoskeletal Radiology

Figure 4-9-11

Synovial Based Lesion: Differential Diagnosis • • • • • • •

PVNS/Synovial chondromatosis Arthritis - inflammatory Infection - unusual low-grade Amyloid Synovial sarcoma

P V N S: Treatment and Results

• • • •

Surgical resection/synovectomy Recurrence rate ➢ GCT - TS (10%–20%) ➢ Diffuse form (40%–50%) Radiation - internal synovectomy - yttrium 90 and dysprosium 165

Soft Tissue Ganglion: Clinical Features

• • •

PVNS knee (diffuse type) with large amount of low signal intensity intraarticular hemosiderin laden tissue (*)

Young adults (25–45 years old) Most common mass hand/wrist (60% of masses) Pain, tenderness or functional impairment (50%), rarely nerve palsy

Figure 4-9-12

Soft Tissue Ganglion: Pathology Etiology - unknown - neoplasm, inflammation, trauma Thick walled unilocular/multilocular cystic spaces Gelatinous - mucinous fluid rich in hyaluronic acid and mucopolysaccharides

Soft Tissue Ganglion: Radiology • •

[Figures 4-9-12 to 4-9-16]

• • • •

Soft tissue mass (1.5–2.5 cm) - dorsum hand/wrist Attached to tendon sheaths usually no communication with joint Rarely cause adjacent bone erosion; periosteal reaction, wall calcification CT/Sono/MRI - cystic mass May have higher attenuation on CT or signal T1W MR image – high protein mucin Wall/septae may show mild enhancement Large ganglion in the most frequent location dorsal to the proximal carpal row with low to intermediate signal intensity mass (*) on axial T1-weighted MR image and homogeneous high signal intensity on T2-weighting

Figure 4-9-13

Figure 4-9-14

Large ganglion in the most frequent location dorsal to the proximal carpal row with anechoic appearance on sonography (*) Ganglion in Guyon canal with intermediate signal intensity mass (*) on axial T1-weighted MR image [upper image] and marked high signal intensity on axial T2-weighted MR image (*) [lower image] causing ulnar nerve entrapment symptoms

Musculoskeletal Radiology

795

Juxtaarticular Masses

Myxoma: Clinical Features • • • • •

Figure 4-9-15

Figure 4-9-16

Location - heart, subcutaneous, intramuscular, juxtaarticular Adults 40–70 years of age Slightly more common in women Painless palpable mass

Myxoma: Pathology • • • • • • •

Ovoid/globular whitish appearance Intraarticular ganglion (*) in the knee on sagittal Contain gelatinous T2-weighted MR image with septations and marked high signal intensity (same patient as material Intraarticular ganglion in the knee on CT previous CT) with low attenuation and multiple Unusual to have cystic septations (*) spaces No fibrous capsule, but edema and muscle atrophy surround mass

Myxoma: Radiology [Figures 4-9-17 to 4-9-19]

• • • • •

Soft tissue mass - location - thigh, shoulder, buttock, upper arm Fluid characteristics CT/MRI Not simple fluid on sonography High protein material may increase CT attenuation or signal on T1W MR image Peripheral rim may enhance with contrast or mild diffuse (57%) pattern Septations (43%) thick and mildly nodular regions Small rim of fat-like tissue CT (25%), MR (71%) Edema surrounding mass MR (79%) Rare to recur after removal (partial or complete)

• • • •

Abscess Chronic hematoma Ganglion/synovial cyst/bursa Other myxomatous neoplasms MFH/liposarcoma/neural tumors

Figure 4-9-17

Myxoma: Differential Diagnosis

Figure 4-9-18

Intramuscular myxoma in paraspinal location on CT with low attenuation mass (*) simulating a cyst

Figure 4-9-19

Paraspinal intramuscular myxoma (same patient as previous CT) with low echogenicity mass but some internal echoes (*) are present suggesting that the lesion is not cystic Intramuscular myxoma in the forearm on sagittal MR imaging with low signal intensity on T1-weighting and high signal intensity on T2-weighting (*) simulating a cyst. However, the intramuscular location, subtle rim of fat (arrowheads on T1) and surrounding edema (arrows on T2) exclude a cyst as a reasonable diagnostic consideration Juxtaarticular Masses

796

Musculoskeletal Radiology

•

Synovial Cyst: Definition

• • •

Figure 4-9-20

A herniation or continuation of the synovial membrane through the joint capsule

Synovial Cyst: Location and Etiology

• • •

Most commonly recognized - popliteal Shoulder, elbow, hip, hand, foot and ankle Types ➢ Primary - unknown cause - children ➢ Secondary - any cause joint distention ➢ Adults - rheumatoid arthritis

Synovial Cyst: Pathology

• • • •

Fluid filled may be multilocular Dense fibrous wall Lined by synovium

Popliteal Cyst - Baker Cyst

• • • • • • •

Synovial Cyst: Radiology

•

• • •

[Figure 4-9-20]

Often asymptomatic or pain from other causes Uncommon to present as mass May dissect in calf simulate DVT Imaging shows infiltration of calf (long fusiform lesion) caused by extension of cyst with dissection and surrounding edema Results from communication between knee joint and gastrocnemius – semimembranosus bursa Increase incidence with age – 50% autopsy series Incidence varies – arthrography (7% – 42%), sonography (15%), MRI (5%) [Figures 4-9-21 to 4-9-25 continues overleaf]

Arthrogram showing the morphology of a ruptured popliteal cyst with long fusiform shape and irregular margins caused by infiltration into surrounding muscle

Fluid filled mass - Sono/CT/MRI May have septations Arthrography can show joint communication Can have solid components if complicated (rupture) with hemorrhage, dissection or superimposed infection Contrast enhancement of rim/septae ➢ Noncomplicated – thin walls Contrast enhancement more complex in complicated/ruptured popliteal cysts Complicated cysts difficult to exclude other causes of mass; must look at morphology Imaging shows infiltration of calf (long fusiform lesion) caused by extension of cyst with dissection and surrounding edema

Figure 4-9-21

Figure 4-9-22 Figure 4-9-23

Synovial cyst (popliteal) on CT with low attenuation (*) and single septation (arrowhead) in typical location with neck between the semimembranosus and gastrocnemius tendons

Synovial cyst (popliteal) on axial T1weighted MR image with low signal intensity mass (*) and typical location (neck between gastrocnemius and semimembranosis tendons-arrowheads) as described in previous CT Musculoskeletal Radiology

Synovial cyst (popliteal - same patient as previous MR) on axial T1-weighted postcontrast MR image with peripheral/septal but nonnodular enhancement (arrowheads)

797

Juxtaarticular Masses

Figure 4-9-24

Figure 4-9-25

Synovial cyst (popliteal - same patient as previous two MR’s) on axial T2-weighted post-contrast MR image with diffuse high signal intensity (*) and neck extending into joint (arrowhead)

• • • •

Ruptured popliteal cyst on sagittal T1 and T2-weighted MR image with evidence of hemorrhage (*) and extensive surrounding edema both superiorly and inferiorly (arrowheads)

Meniscal Cyst: Clinical Features

• • • • •

Adults (20–40 years); M>F ratio 2:1 Cystic masses related to meniscal tears (1%–2% incidence) Fluid accumulates from joint through tear Pain at night or after exercise

Figure 4-9-26

Meniscal Cyst: Radiology

• • •

Radiographs - soft tissue mass CT/SONO/MRI - fluid collection adjacent to meniscus Lateral > Medial 3–10:1 now more equal Medial - small cystic mass within or adjacent to meniscus Lateral - larger fluid collection filling potential space between meniscus and collateral ligament MRI - best to evaluate meniscal tear and extension into cyst Must repair tear and resect cyst

Synovial Lipoma

• • • •

Two types ➢ Localized form ➢ Diffuse form - lipoma arborescens

Lipoma arborescens with villonodular fronds of fatty tissue (arrows) extending into the knee joint on sagittal T1-weighted MR image

Synovial Lipoma: Localized

• • •

Figure 4-9-27

Rare - knee most frequent Solid fatty intraarticular mass Filling defect on arthrogram CT/MRI - lipomatous tissue

Lipoma Arborescens: Clinical Features Diffuse infiltration of synovium by fat Monoarticular - knee most common Often secondary (but can be primary) to chronic arthritis from trauma or inflammatory disease

Lipoma Arborescens: Radiology • • • •

[Figures 4-9-26 and 4-9-27]

Radiographs - soft tissue swelling Arthrography - multiple filling defects CT fatty infiltration MRI best to identify frond-like fatty projections

Juxtaarticular Masses

Lipoma arborescens (same patient as previous MR ) showing high signal intensity fluid surrounding fatty nodules (arrows) on sagittal T2-weighted MR image

798

Musculoskeletal Radiology

• • • • •

Synovial Chondromatosis: Clinical Features

• •

Figure 4-9-28

Formerly synovial osteochondromatosis Cartilage metaplasia in synovium Knee (50%), hip, elbow, any joint can be involved M > F 2:1; 3rd to 6th decade Joint pain, decrease range of motion

Synovial Chondromatosis: Pathology • •

Hyaline cartilage metaplasia in synovium Cartilage nodules (2–3 cm) can break away into joint, grow, reattach to synovium Hypercellularity and nuclear atypia simulate cartilage malignancy

Synovial Chondromatosis: Radiology [Figures 4-9-28 to 4-9-32] • • • • • •

Radiographs - calcified bodies (70%–75%), may ossify, extrinsic erosions, joint widened, OA changes Bone scan - mild increased activity Arthrography - filling defects CT thickening about joint, effusion often small if present, calcification/ossification MRI - variable depending on degree of mineralization, some hyperintensity T2W images Can also involve tendons and bursa Secondary chondromatosis - trauma, OA, RA, AVN, osteochondritis dissecans

Figure 4-9-29

Synovial chondromatosis wit multiple round filling defects on hip arthrography. No calcification was seen on pre-arthrography radiographs (not shown)

Figure 4-9-30

Synovial chondromatosis of right hip with subtle calcifications (arrowhead) difficult to detect on radiograph, although joint is widened (arrow)

Synovial chondromatosis of the shoulder with innumerable calcified intraarticular osteochondral bodies all similar in size and shape on radiograph

Figure 4-9-32

Figure 4-9-31

Synovial chondromatosis of right hip (same patient as previous radiograph) with multiple calcifications (arrowheads) about hip on CT

Musculoskeletal Radiology

799

Synovial chondromatosis of right hip (same patient as previous radiograph and CT) with extensive high signal intensity intraarticular tissue (*) but calcification is not apparent on T2-weighted MR image Juxtaarticular Masses

• • • • •

Surgical synovectomy Recurrence common External radiation therapy Internal RT - nuclear medicine synovectomy? Rare degeneration into chondrosarcoma

• • • •

Less common than synovial chondromatosis 3rd and 4th decades, M>F Slow growing masses, painless Fingers (80%), hands, toes, feet, trunk

Synovial Chondromatosis: Treatment and Prognosis

Figure 4-9-33

Soft Tissue Chondroma: Clinical Features

• • • •

Soft Tissue Chondroma: Pathology Usually < 3 cm, often attached to tendon Mature hyaline cartilage lobular pattern Can show ossification Fibrous capsule not tenosynovium unlike synovial chondromatosis

Soft tissue chondroma of the finger on radiographs with large calcified mass (arrowheads)

Soft Tissue Chondroma: Radiology • • • •

[Figures 4-9-33 and 4-9-34]

• • • •

Nonspecific soft tissue mass related to IP joint Also common in infrapatellar fat Chondroid matrix, can ossify Unusual to erode underlying bone

Heterotopic Bone Formation: Myositis Ossificans

• • • •

Young adults, M>F, usually trauma history No history trauma 25%–50%; also paraplegics Can involve muscles, fascia, tendons, subcutaneous fat Initially pain/tenderness and localized mass; pain decreases with time

Heterotopic Bone Formation: Location

Same patient: CT with large calcified mass (arrowheads). Noncalcified portion is low attenuation consistent with a chondroid lesion

Extremities - 80%, anterior compartments Lower extremity - quadriceps muscle Upper extremity - brachialis muscle Subcutaneous fat - 30% of cases

Figure 4-9-34

Same patient: MR imaging with high water content soft tissue mass (arrowheads) consistent with a chondroid lesion

Soft tissue chondroma in Hoffa fat pad on radiograph with large calcified mass Juxtaarticular Masses

800

Musculoskeletal Radiology

•

Heterotopic Bone Formation: Pathology •

Zonal pattern of maturation ➢ Central immature osteoid/fibroblastic tissue ➢ Periphery calcifying osteoid to mature lamellar bone Cortical bone with further maturation

Figure 4-9-35

Heterotopic Bone Formation: Radiology • •

[Figure 4-9-35]

• • • •

Early soft tissue mass and edema Calcification 2–4 weeks then matures (zonal phenomena) to central trabecular and peripheral cortical bone Usually separable from cortex but may be attached Bone scan marked increased activity Angiography - staining and neovascularity early CT – best to see early ossification pattern with peripheral rim – enhances with contrast

Heterotopic Bone Formation: MR Imaging •

[Figure 4-9-35]

• •

Early to intermediate ➢ Normal with displaced fascial planes (T1W) ➢ Increased intensity mass with prominent edema (T2W image) Late - heterogeneous well defined mass marrow fat on T1W/T2W MR images, no edema, low intensity rim Often misinterpreted as malignant tumor

Heterotopic bone formation (myositis ossificans) with peripheral rim of calcification on radiograph (arrow)

Heterotopic Bone Formation: Treatment and Prognosis • • • • • •

May resorb or be asymptomatic Resect after maturation (12–18 months) Premature resection - recurrence with vengeance Rare report malignant transformation Malignant myositis (mucinous carcinoma )

Nodular Fasciitis: Clinical Features • • •

Very common; most frequent tumor-like lesion fibrous tissue Rapidly growing mass 1–2 weeks duration Young adults (20–35 years), M
• • • •

Upper extremity (50%) – volar forearm Trunk - chest wall and back Head and neck in children Rare hand/feet/lower extremity

Same patient: peripheral rim of calcification (arrow) separated from femoral cortex on CT

Nodular Fasciitis: Location

• • • •

Nodular Fasciitis: Pathology Subcutaneous type (70%) - soft tissue nodule Intramuscular type (15%) - not circumscribed multinodular Immature fibroblasts in irregular fascicles Reticulin meshwork, collagen minimal, inflammatory and mucoid component

Musculoskeletal Radiology

801

Same patient: axial T2-weighted MR image with heterogeneous mass (arrow) suggesting a more aggressive neoplastic process as peripheral calcification is less apparent

Juxtaarticular Masses

Nodular Fasciitis: Radiology and Treatment • •

Figure 4-9-36

Figure 4-9-36]

• •

Nonspecific soft tissue mass; may show fascial extensions CT/MRI-mass with irregular margins and heterogeneous on MRI, surrounding edema Suggests malignancy imaging and pathology Surgical resection-recurrence rare (1%–2%) even if incomplete

• • • • •

Malignant mesenchymal tumor Young adults 15–40 years of age Fourth to fifth most common soft tissue sarcoma Painful deep soft tissue mass Often indolent slow growing mass (4 years to diagnosis)

• • • • • • •

MFH/Fibrosarcoma 20%–30% Liposarcoma 16%–19% Rhabdomyosarcoma 10%–19% Nonspecific spindle cell sarcoma 5%–15% Leiomyosarcoma 5%-10% Dermatofibrosarcoma protuberans (DFSP) 5%-10% Synovial sarcoma 5%–10%

• • • •

Extraarticular adjacent to tendons bursa, ligaments > 90% Intraarticular < 10% Lower extremity 60% - around knee Upper extremity 25% - around wrist

Synovial Sarcoma: Clinical Features

Musculoskeletal Soft Tissue Sarcoma: Incidence Nodular fasciitis of the forearm on coronal STIR MR image with a high signal intensity subcutaneous mass (*) with linear fascial extensions (fascial tail sign arrows) both superiorly and inferiorly

Figure 4-9-37

Synovial Sarcoma: Location

•

Synovial Sarcoma: Pathology • • • • • • •

Two cell lines ➢ Epithelial (keratin positive) ➢ Spindle cells Monophasic / biphasic Variable - calcification, hemorrhage

Synovial Sarcoma: Radiology [Figures 4-9-37 to 4-9-40]

• • • • • • •

Radiographs - normal (50%) or nonspecific soft tissue mass near joint Bone erosion or periosteal reaction (11%–20%), bone invasion (5%) Soft tissue calcification up to 30% - best by CT Bone scan - increased activity MRI ➢ T1W images - similar to muscle ➢ T2W images - usually high intensity ➢ Triple sign on T2W MR (35-57%) (nonspecific) ➢ Very heterogeneous (bowl of grapes) Not uncommonly well defined with pseudocapsule - simulates benign characteristics Fluid - fluid level 10%–25% (hemorrhage) worse prognosis in highly vascular lesions

Synovial sarcoma of the foot with indolent appearing extrinsic erosions on radiograph (arrow)

Figure 4-9-38

Synovial Sarcoma: Treatment and Prognosis Surgical resection/amputation Radiation therapy/chemotherapy Local recurrence 30% – 50% 5 year survival 36% – 76%; 10 year survival 20% – 63% Metastases (16%-25%) – lung (94%), lymph node (10%), marrow Synovial sarcoma about elbow with calcification (arrowhead) in mass near but not in the joint on radiograph Juxtaarticular Masses

802

Musculoskeletal Radiology

• • • • •

Malignant melanoma of soft parts Arise in tendons/aponeurosis Deep tissue without skin involvement Foot/ankle (43%), knee, thigh, hand Adults 20–40 years; F>M

• • • •

Cells with clear cytoplasm Framework of fibrocollagenous tissue Intracellular melanin 60%–75% Hemosiderin also present

Clear Cell Sarcoma: Clinical Features

Figure 4-9-39

Clear Cell Sarcoma: Pathology

• • • •

Clear Cell Sarcoma: Radiology [Figure 4-9-41]

• • • •

Soft tissue mass at/in tendon/aponeurosis Bone erosion/destruction CT/MRI - infiltrative soft tissue mass MRI ➢ T1W image - intermediate intensity ➢ T2W image - may be low intensity

Clear Cell Sarcoma: Treatment and Prognosis Surgical resection/radiation/chemotherapy Poor prognosis Local recurrence and metastases Mets - lungs, lymph nodes, bone

Synovial sarcoma about the ankle on coronal T1weighted MR image with heterogeneous hemorrhagic mass (*) invading bone (arrowhead)

Figure 4-9-40

Figure 4-9-41

Synovial sarcoma about the ankle (same patient as previous MR) on axial T2 weighted MR image with heterogeneous multicompartment mass

Clear cell sarcoma on MR imaging with origin in the quadriceps tendon as evidenced on the axial image with low signal intensity both anterior and posterior to the mass (arrowheads). Sagittal T2-weighted MR shows nonspecific intermediate signal intensity

Musculoskeletal Radiology

803

Juxtaarticular Masses

Noncalcified Juxta/Intraarticular Soft Tissue Masses Differential Diagnosis • • • • • •

Synovial/Meniscal cyst Ganglion/myxoma Gouty tophus Hemangioma/PVNS Lipoma Synovial sarcoma

Calcified juxta/Intraarticular Soft Tissue Mass Differential Diagnosis • • • • • • • •

Myositis ossificans Aneurysm Gouty tophus Hyperparathyroidism/hemangioma Osteochondromatosis (synovial) Synovial sarcoma Tumoral calcinosis Soft tissue sarcoma

References 1.

Al-Nakshabandi NA, Ryan AG, Choudur H, Torreggiani W, Nicoloau S, Munk PL, Al-Ismail K. Pigmented villonodular synovitis. Clin Radiol. 2004 May;59(5):414-20. Review. 2. Kransdorf MJ, Meis JM, Jelinek JS. Myositis ossificans: MR appearance with radiologic-pathologic correlation. AJR Am J Roentgenol. 1991 Dec;157(6):1243-8. 3. Martinez S, Vogler JB 3rd, Harrelson JM, Lyles KW. Imaging of tumoral calcinosis: new observations. Radiology. 1990 Jan;174(1):215-22. 4. Murphey MD, Choi JJ, Kransdorf MJ, Flemming DJ, Gannon FH. Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation. Radiographics. 2000 Sep-Oct;20(5):1407-34. Review. 5. Murphey MD, McRae GA, Fanburg-Smith JC, Temple HT, Levine AM, Aboulafia AJ. Imaging of Soft Tissue Myxoma with Emphasis on CT and MRI and Comparison of Radiologic and Pathologic. Radiology 2002; 225:215-224. 6. Ortega R, Fessell DP, Jacobson JA, Lin J, Van Holsbeeck MT, Hayes CW. Sonography of ankle Ganglia with pathologic correlation in 10 pediatric and adult patients. AJR Am J Roentgenol. 2002 Jun;178(6):1445-9. 7. Robinson P, White LM, Kandel R, Bell RS, Wunder JS. Primary synovial osteochondromatosis of the hip: extracapsular patterns of spread. Skeletal Radiol. 2004 Apr;33(4):210-5. Epub 2004 Feb 18. 8. Steinbach LS, Johnston JO, Tepper EF, Honda GD, Martel W. Tumoral calcinosis: radiologic-pathologic correlation. Skeletal Radiol. 1995 Nov;24(8):573-8. 9. Tschirch FT, Schmid MR, Pfirrmann CW, Romero J, Hodler J, Zanetti M. Prevalence and size of meniscal cysts, ganglionic cysts, synovial cysts of the popliteal space, fluid-filled bursae, and other fluid collections in asymptomatic knees on MR imaging. AJR Am J Roentgenol. 2003 May;180(5):1431-6. 10. Valenzuela RF, Kim EE, Seo JG, Patel S, Yasko AW. A revisit of MRI analysis for synovial sarcoma. Clin Imaging. 2000 Jul-Aug;24(4):231-5.

Juxtaarticular Masses

804

Musculoskeletal Radiology

Musculoskeletal Angiomatous Lesions Mark D. Murphey, MD • • • • • • •

Hemangioma Lymphangioma Glomus Tumor Angiomatosis and associated syndromes Hemangioendothelioma Hemangiopericytoma Angiosarcoma

• • • •

M > F (2:1); 4th – 5th decade Majority asymptomatic May have soft tissue components Common sites : vertebral body (11% of spines), calvarium

Angiomatous Lesions

Osseous Hemangioma: Clinical Characteristics

• • • •

Soft Tissue Hemangioma: Clinical Characteristics

• •

7% of all benign S.T. neoplasms 1.5% of the general population Most frequent S.T. neoplasm in children More common in young women – may increase in size with pregnancy Can be subcutaneous, intramuscular or synovial

Figure 4-10-1

Hemangioma: Pathology • • • • •

Subtype based on predominant vascular component – but usually mixed tumor Capillary – most common – first years of life – skin, subcutaneous, vertebrae (low flow) Cavernous – childhood – larger and deeper (low flow) Arteriovenous – deep or superficial – persistent fetal capillary bed (high flow) Venous – adults – deep involvement – retroperitoneum, mesentery or extremities (low flow) Epithelioid – dermis/subcutis

Vertebral hemangiomas with thickened vertical trabeculae (arrows-corduroy appearance) on radiograph and coronally sectioned gross specimen (different patients)

Osseous Hemangioma: Radiology •

Figure 4-10-2

[Figures 4-10-1 to 4-10-5]

• • • •

Vertebrae – focal or diffuse – vertical striations (corduroy or polka dot) posterior element involvement more likely symptomatic Calvarium/mandible radiating web-like trabecular pattern Long bone - multifocal lytic honeycomb pattern, cortical lesions/erosions in diaphysis Bone overgrowth Arthritis from intraarticular bleeding

Vertebral hemangioma (asymptomatic) of lumbar spine with "polka dot" appearance and fat between trabeculae on CT

Musculoskeletal Radiology

805

Musculoskeletal Angiomatous Lesions

Figure 4-10-3

Figure 4-10-4

Vertebral hemangioma (symptomatic patient) with "polka dot" appearance on CT (arrowheads) and soft tissue extension. Sagittal T1 and T2-weighted MR images show vertebral fracture and spinal canal compromise caused by anterior epidural soft tissue component (arrows) but diagnostic trabecular thickening is difficult to appreciate

S.T. Hemangioma Radiology: S.T. Changes [Figure 4-10-6] • • •

Calcification – curvilinear or amorphous, phlebolith (30%-50% of lesions) Angiography – irregular enlarged feeding arteries, contrast pooling, arteriovenous shunting Venous lesions seen only with venography

Calvarial hemangioma with spoke wheel pattern of trabecular thickening (arrows) on radiograph, CT and vascular channels/spaces on gross specimen (arrowheads)

Figure 4-10-5

S.T. Hemangioma: Imaging •

[Figures 4-10-7 to 4-7-12]

• • • • • •

T1W images – low to intermediate heterogeneous mass; look for fat overgrowth Very high intensity T2W images (low flow) Serpentine vessels / cavernous spaces may help distinguish types; high vs. low flow Enhance with contrast Phleboliths – CT > MRI Bone scan – often only limited activity Sonography – solid mass Doppler may show low resistance flow

Femoral hemangioma on coronal T1-weighted MR image before and after contrast shows multifocal round areas of marrow replacement (arrowheads) representing vascular channels with enhancement and serpentine feeding vessels

Figure 4-10-6

Figure 4-10-7

Soft tissue hemangioma of the hand with phleboliths on radiograph (arrows) and intraoperative photograph

Soft tissue hemangioma of axilla (intramuscular and cavernous) on CT showing enhancing vascular channels (large arrows), fat overgrowth (small arrows), and phlebolith (arrowhead) Musculoskeletal Angiomatous Lesions

806

Musculoskeletal Radiology

Figure 4-10-8

Figure 4-10-9

Soft tissue hemangioma of forearm (intramuscular and cavernous) on sagittal T1-weighted MR images before and after gadolinium showing intermediate signal intensity serpentine vascular channels and spaces (arrows) that enhance following contrast and fat overgrowth (arrowheads). Axial T2-weighted MR reveals multiple circular high signal areas corresponding to slow flow cavernous spaces (*)

Soft tissue hemangioma of the thigh on sagittal T1-weighted (right image) and axial STIR (left image) MR images with associated fat atrophy (arrowheads) and slow flow circular vascular spaces (arrow) corresponding to the gross specimen and histology [4-10-10]

Figure 4-10-11 Soft tissue hemangioma (high flow arteriovenous) about knee with low signal intensity serpentine vessels (arrows) on coronal T2weighted MR image

Figure 4-10-10

Figure 4-10-12

Soft tissue hemangioma (arrows) with associated fat atrophy (arrowheads) in surrounding thigh muscle on gross specimen (left image). Histology (right image) reveals phleboliths (*) with calcification peripherally (arrows) and fat atrophy of muscle (arrowheads)

Musculoskeletal Radiology

Capillary hemangioma on T2-weighted MR image (arrow) with nonspecific high signal intensity in the face with typical extensive strawberry nevus clinically. No characteristic features of fat overgrowth or serpentine vascular structures are seen to suggest hemangioma as the vessels in this type of lesion are to small (capillary) to discern on imaging as demonstrated on the histology

807

Musculoskeletal Angiomatous Lesions

• • • • •

Hemangioma: Treatment

Figure 4-10-13

Surgical resection / laser therapy Embolization Radiation in symptomatic unresectable lesions – spine Vertebroplasty Recurrence (15% – 30%) – large lesions

Lymphangioma: Clinical Characteristics • • • • • • • • •

Rare lesion in bone, usually S.T. Often present at birth (50% – 65%) 90% apparent by age 2 years Head, neck, axilla – 75% of cases Soft fluctuant mass Lymphangioma of the neck on CT with a homogeneously low attenuation mass (*)

Lymphangioma: Pathology

• •

Sequestrated noncommunicating lymphoid tissue Large multiloculated cystic spaces Lined by lymphatic endothelium Filled with proteinaceous material

Figure 4-10-14

Lymphangioma: Radiology [Figures 4-10-13 and 4-10-14]

•

Radiographs – soft tissue mass Imaging – large cystic spaces less common serpentine component may appear complex – solid components (high signal on T1 25%) Cystic hygroma – hydrops fetalis, Turner syndrome

• • • •

Patients 4th to 5th decade Tumor of neuromyoarterial glomus Almost all terminal phalanx soft tissue Bone erosion/invasion 15%–65%

Glomus Tumor [Figure 4-10-15]

•

Angiomatosis Multifocal or diffuse infiltration of bone by hemangiomatous or lymphangiomatous lesions with or without soft tissue involvement

• • • • •

Young patients – first 3 decades M > F (2:1) Osseous involvement only – benign course Visceral involvement – poor prognosis No malignant potential

• • •

Capillary or cavernous hemangioma’s Lymphangioma’s – lymphatic backflow Mixed vascular lesion difficult to distinguish

Angiomatosis: Clinical Characteristics

Lymphangioma of the neck in an infant on coronal T1-weighted MR image with heterogeneous mass (arrow) showing both high and low signal intensity areas extending along the chest wall (arrowhead)

Figure 4-10-15

Angiomatosis: Pathology

Glomus tumor with erosion of the terminal phalanx of the long finger on radiograph (arrows) and sagittal macrosection (*)

Musculoskeletal Angiomatous Lesions

808

Musculoskeletal Radiology

Angiomatosis: Radiology [Figure 4−10-16] • • • • • •

MR imaging/CT – some as solitary angiomatous lesions more extensive Imaging to evaluate visceral involvement/extent Lymphangioma – proven with lymphangiography and contrast in lesion Diffuse round/oval medullary lytic lesions May have sclerotic margins Location: femur, ribs, spine, pelvis, humerus, scapula, other long bones, clavicle

• • • • •

Maffucci syndrome Osler – Weber – Rendu Klippel – Trenaunay – Weber Massive osteolysis of Gorham Associated osteomalacia and thrombocytopenia

• • • •

Multiple enchondromata Cavernous soft tissue hemangiomata Often hands/feet, unilateral predominance Malignant potential both lesions and viscera

Figure 4-10-16

Angiomatous Syndromes

Maffucci Syndrome

•

Angiomatosis (lymphangiomatosis) with extensive infiltration of the entire lower extremity causing elephantiasis on coronal T1weighted MR image and clinical photograph

Osler-Weber-Rendu • • •

Figure 4-10-17

Hereditary hemorrhagic telangiectasia Dilated capillaries and veins Autosomal dominant GI, GU, lung, spinal; bone – rare

Klippel-Trenaunay-Weber • •

[Figure 4-10-17]

• •

Nonhereditary, lower extremity Unilateral cutaneous capillary hemangioma Varicose veins and local gigantism Can have arteriovenous component

Massive Osteolysis of Gorham: “Vanishing Bone Disease” • • • •

Klippel-Trenaunay-Weber syndrome on clinical photograph and coronal T1weighted and T2-weighted MR images showing classic triad of hemihypertrophy,varicose veins and extensive predominantly slow flow angiomatous lesion (arrowheads). Smaller high flow component is also seen on T2-weighted image (arrows)

[Figure 4-10-18]

• •

Patients < age 40 years History trauma 50% Upper extremity favored, may extend across joint Progressive bone resorption and fragmentation (simulate neuropathic) Pathology - proliferating vascular channels

Figure 4-10-18

Osteomalacia and Thrombocytopenia • •

Tumor induced osteomalacia: most frequent vascular lesions Hemangioma / hemangiopericytoma Kasabach - Merritt syndrome - hemangioma/ hemangiopericytoma associated with thrombocytopenia and purpura

Gorham vanishing bone disease involving the foot with radiograph and gross specimen showing extensive sharply defined bone resorption (arrowheads and arrow)

Musculoskeletal Radiology

809

Musculoskeletal Angiomatous Lesions

Intermediate to Malignant Musculoskeletal Angiomatous Lesions • • •

Hemangioendothelioma Hemangiopericytoma Angiosarcoma

• • • • •

Intermediate – benign or malignant Composed of vascular endothelial cells Often in young patients Bone or soft tissue Locally aggressive, unusual to metastasize

• • • • •

Intermediate – benign or malignant Tumor of cells around vessels – pericytes Tumor of middle-aged adults Sites – soft tissue of thigh, pelvis and retroperitoneum Rare in bone

Figure 4-10-19

Hemangioendothelioma (HE)

Hemangiopericytoma (HPC)

• •

Angiosarcoma (ASC) • • • •

Malignant; M > F (2:1) Composed of hemangiosarcoma or lymphangiosarcoma cellular elements Location: skin, muscular, viscera, bone Associated with lymphedema post-mastectomy (Stewart-Treve syndrome)

Osseous HE, HPC, ASC: Skeletal Location •

Hemangioendothelioma: skull, vertebrae, lower extremity Hemangiopericytoma (rare): pelvis, proximal long bones, vertebrae, mandible Angiosarcoma: long tubular bone lower extremity

Figure 4-10-20

Osseous HE, HPC, ASC : Radiographic Findings • •

T1

[Figure 4-10-19]

Multifocal lytic lesions – honeycomb appearance Aggressive bone destruction with expansion and soft tissue mass

T2

Radiology of HE, HPC, ASC : Advanced Imaging •

Malignant hemangioendothelioma of bone with multifocal lytic lesions (arrowheads) in the tibia and fibula on radiograph and osseous replacement by hemorrhagic tissue (*) on photograph of coronally sectioned gross specimen

T1 GD

[Figures 4-10-20 to 4-10-21]

• • • • •

Angiography – intensely vascular with peripheral vessels displaced by tumor early, dense blush late Sonography – hypo or hyperechoic mass Doppler – arteriovenous shunting MRI ➢ T1W – usually similar to muscle ➢ Can be high intensity hemorrhage Look for prominent serpentine vessels Fluid - fluid levels, contrast enhancement Dominant skin mass in chronic lymphedema (ASC) Angiosarcoma developing in a patient with chronic leg lymphedema. Axial T1-weighted, T1-weighted post contrast and T2-weighted MR images show the enlarged leg with subcutaneous edema (arrows) and dominant skin mass (arrowheads) representing the angiosarcoma. The superficial angiosarcoma is also seen on the clinical photograph (grey arrows)

Musculoskeletal Angiomatous Lesions

810

Musculoskeletal Radiology

Figure 4-10-21 T1

T2

Hemangiopericytoma in of the thigh showing high flow vessels (arrows) in the soft tissue mass (*) and feeding the lesion on both axial T1-weighted and coronal T2-weighted MR images. Photograph of the sectioned gross specimen also shows the soft tissue mass (*) and the high flow vessels (arrowheads)

Cannot distinguish HE, HPC or ASC from other soft tissue masses if prominent serpentine vessels are not recognized Hemangioendothelioma, Hemangiopericytoma and Angiosarcoma cannot be differentiated from each other radiologically • • •

Large masses Aggressive characteristics with infiltration No fat overgrowth

• • • •

Surgical resection Malignant lesions – radiation and chemotherapy Local recurrence common Metastases common to lung in ASC

Distinction of HE, HPC and ASC from Hemangioma

Treatment and Prognosis: HE, HPC and ASC

Summary: Musculoskeletal Angiomatous Lesions • • • • •

Osseous – Multifocal bone lysis – Honeycomb appearance Look for serpentine vascular pattern – MRI Overgrowth of fat – MRI Multiple associated syndromes and angiomatosis Higher grade lesions – HE, HPC, ASC ➢ Larger aggressive lesions ➢ Infiltrative characteristics

References 1. 2. 3. 4. 5. 6.

Baudrez V, Galant C, Vande Berg BC. Benign vertebral hemangioma: MR-histological correlation. Skeletal Radiol. 2001 Aug;30(8):442-6. Coldwell DM, Baron RL, Charnsangavej C. Angiosarcoma. Diagnosis and clinical course. Acta Radiol. 1989 NovDec;30(6):627-31. Fayad L, Hazirolan T, Bluemke D, Mitchell S. Vascular malformations in the extremities: emphasis on MR imaging features that guide treatment options. Skeletal Radiol 2006; 35:127-137. Laredo JD, Assouline E, Gelbert F, Wybier M, Merland JJ, Tubiana JM. Vertebral hemangiomas: fat content as a sign of aggressiveness. Radiology. 1990 Nov;177(2):467-72. Lorigan JG, David CL, Evans HL, Wallace S. The clinical and radiologic manifestations of hemangiopericytoma. AJR Am J Roentgenol. 1989 Aug;153(2):345-9. Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995 Jul;15(4):893-917.

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Musculoskeletal Angiomatous Lesions

Paget Disease Mark D. Murphey, MD • • •

Figure 4-11-1

Paget Disease: Clinical Characteristics

• • • • • • • • •

Described 1877 by Sir James Paget Osteitis deformans Common disease ➢ 3% over age 40 ➢ 10% over age 80 Slightly more common in men Common in Great Britain and descendents (USA, Australia) ➢ Continental Europe Uncommon in Asia Many patients asymptomatic (20%)

Paget Disease: Clinical Presentation

• • •

Pain Osseous bowing and enlargement Neurologic symptoms High output congestive failure Lab ➢ Serum alkaline phosphatase (blastic phase) ➢ Urinary and blood hydroxyproline (lytic phase)

Marrow replacement by fibrovascular tissue (*) in active Paget disease (left image) versus fat (F) in marrow with inactive disease (right image)

Figure 4-11-2

Paget Disease: Etiology

• • • • •

Unknown Possible etiologies Infection; viral; intranuclear inclusions; paramyxovirus (measles) Autoimmune Connective tissue disease Neoplastic

Paget Disease: Pathology [Figure 4-11-1] Initially osteoclastic resorption Subsequently osteoblastic response (active) ➢ Excessive and disorganized ➢ “Mosaic or jigsaw” pattern ➢ Marrow: fibrovascular reaction ➢ Marrow: fat (inactive)

• • • •

Calvarium, spine (lumbosacral) and pelvis: 25%-75% Proximal long bones: 25%–30% Humerus (31%), scapula (24%), clavicle (11%) Initially monostotic 10%–35%: most polyostotic

• • •

Radiographs – diagnosis Bone Scan – assess areas involved CT/MRI: to assess complications or unusual cases

Paget Disease: Skeletal Distribution [Figure 4-11-2]

Paget Disease: Radiologic Evaluation

Paget Disease

812

Musculoskeletal Radiology

•

Paget Disease: Radiologic Stages • • •

Active ➢ Lytic - osteoclastic activity ➢ Mixed - majority of cases Inactive ➢ Blastic - osteoblastic activity Usually progresses through these phases but not always ➢ Recrudescent lytic phase in patients at rest; simulates tumor

Paget Disease: Radiologic Lytic Phase [Figures 4-11-3 to 4-11-5 ] •

Skull: large well-defined areas; involve both inner and outer tables of frontal/occipital bones (osteoporosis circumscripta) Long Bones: subchondral location with advancing wedge/V shape “blade of grass/candle flame”

Figure 4-11-3

Figure 4-11-4 Figure 4-11-5

Lytic phase of Paget disease with sharp margins and subchondral extension

Osteoporosis circumscripta with sharply marginated large area of bone lysis Musculoskeletal Radiology

813

Paget Disease

• •

Paget Disease: Radiographs – Mixed/Blastic Disease

•

Figure 4-11-6

Sclerosis and lucency Trabecular and cortical thickening ➢ Along the lines of stress ➢ But some disorganization Bone enlargement

Paget Disease: Radiographs: Mixed/Blastic Disease •

[Figures 4-11-6 to 4-11-9]

•

Skull: “cotton wool” appearance obscures inner and outer tables, often spares facial bones Spine: vertebral body (picture frame); ivory vertebral body; posterior elements may be involved

Figure 4-11-7

“Cotton wool” appearance on radiograph with multifocal areas of sclerosis and thickening of the diploic space anteriorly

Figure 4-11-8

Picture frame appearance of Paget disease of the spine, multiple levels, on radiograph and coronal macrosection

Figure 4-11-9

Mixed lytic/blastic Paget disease in skull with diploic space expansion and hyperemic bone on CT, gross specimen and histology

Ivory Vertebral Body: Differential Diagnosis • • • •

Blastic metastasis: breast, prostate, adenocarcinoma GI tract, carcinoid, transitional cell carcinoma bladder Lymphoma Chronic infection Chordoma

Ivory vertebral body in Paget disease on radiograph and intense uptake on radionuclide bone scan

Paget Disease: Radiographs Mixed/Blastic Phase •

[Figures 4-11-10 and 4-11-11]

•

Pelvis: asymmetric involvement ➢ Thickened iliopubic and ischial lines ➢ Enlarged pubic rami and ischium Long Bones: epiphyseal involvement ➢ Rarely diaphyseal (tibia) ➢ Enlarged bone

Paget Disease

814

Musculoskeletal Radiology

Figure 4-11-10

Figure 4-11-11

Figure 4-11-12

Paget disease mixed lytic and blastic in left pubic rami

Paget disease (noncomplicated) in right iliac crest with trabecular and cortical thickening (arrowheads)

Paget disease with coarsened trabecular pattern and thickening of the iliopectineal line involving the entire hemipelvis

•

Paget Disease: Bone Scintigraphy • • •

Figure 4-11-13

Active disease – marked uptake ➢ Dynamic and static images Abnormal before radiographs Overview of disease – look at distribution Monitor disease and therapy

T1

Paget Disease: CT/MRI Noncomplicated Diseases • •

[Figure 4-11-12]

Not usually needed for diagnosis CT- thickened trabeculae ➢ Bone enlargement ➢ Lytic areas

Paget disease involving the calcaneus (noncomplicated), mixed lytic/blastic on radiograph, with maintained yellow marrow on T1- weighted MRI

Paget Disease: MRI - Noncomplicated Cases •

[Figures 4-11-13 to 4-11-16]

•

T1-weighted images ➢ Cortically trabecular thickening ➢ Enlarged bone ➢ Low signal (sclerosis) ➢ Yellow marrow/fat (inactive disease) ➢ Heterogeneous signal (active disease) ➢ Marrow replacement non-masslike T2-weighted images ➢ Low signal (sclerosis) ➢ Fat signal intensity (inactive disease) ➢ Heterogeneous intermediate/high signal (fibrovascular marrow active disease) ➢ No focal mass

Figure 4-11-14

Paget disease mixed lytic/blastic with cortical thickening (arrow) involving the tibia Musculoskeletal Radiology

815

Paget Disease

Figure 4-11-16

Figure 4-11-15 T1

T1 GD

Coronal T1-weighted (noncontrast) and axial T1-weighted (after contrast) MR images show speckled marrow pattern with enhancement in more active Paget disease (noncomplicated) (*) and more intense enhancement of the intracortical component that is most active

• • • • •

T2

Axial T2-weighted MR image shows speckled pattern of increased intensity (*) in more active Paget disease (noncomplicated-same patient as previous MRI and radiograph)

Paget Disease: Complications

Figure 4-11-17

Osseous deformity Fractures Neurologic symptoms Arthropathy Neoplasm

Paget Disease: Osseous Deformity • • • •

Effects of bone softening Bowing – common in long bones Acetabulae protrusio Basilar invagination – 30% of patients with skull involvement

• • • • • • • • • •

Partial or complete (insufficiency) True acute fractures Horizontal lucencies (“banana fracture”) Convex surface or bone Single or multiple Often symptomatic Sites: femur, humerus, pelvis Spine: central compressions May heal but high nonunion rate At risk for sarcoma (biopsy?)

[Figure 4-11-17]

Paget Disease: Fractures [Figure 4-11-18]

Acetabulae protrusio on radiograph and coxa varus deformity of the femur on the coronally sectioned macrosection in patients with Paget disease. Note the axial narrowing of the hip joints

Figure 4-11-18

Paget Disease: Neurologic Symptoms • • •

[Figure 4-11-19]

•

Symptoms – impingement of cranial and spinal nerves Caused by skull and spine involvement Bone enlargement, fractures, bone softening with basilar invagination and increased osseous vascularity with cord hypoxia CT/MRI for evaluation

Paget disease of femur with complete and incomplete fractures (arrowheads)

Paget Disease

816

Musculoskeletal Radiology

•

Paget Disease: Arthropathy • •

• •

Figure 4-11-19

Rheumatic diseases with poor association: rheumatoid arthritis, calcium pyrophosphate deposition (CPPD), ankylosing spondylitis Gout – higher incidence hyperuricemia (40%) from increased cell turnover Osteoarthritis ➢ Hip and knee most common ➢ Abnormal mechanics from deformity ➢ Bone weakening ➢ Hip narrowing can be axial Soft tissue calcification ➢ Tendinitis and with vitamin D treatment

Paget Disease: Neoplasm • • • • • • •

Sarcomatous transformation ➢ 1% patients with Paget disease ➢ 5% - 10% patients with extensive disease Patients 55 to 80 years old Common sites – femur, pelvis, humerus Osteosarcoma (50% - 60%) MFH/fibrosarcoma (20% - 25%) Chondrosarcoma (10%) GCT Metastasis, myeloma, lymphoma

Paget disease at C2 (*) with marked osseous enlargement

Figure 4-11-20

Paget Disease: Neoplasm Radiology • • • • • • • •

[Figures 4-11-20 to 4-11-22]

Bone destruction predominates Cortical involvement and soft tissue mass No periosteal reaction Persistent nonhealing fracture Compare with old radiographs Bone scan: photopenic area Gallium scan: increased uptake MRI ➢ T1W: masslike marrow replacement ➢ T2W: focal mass of high intensity ➢ Soft tissue mass

Figure 4-11-21

Pictoral representation of malignant transformation in Paget disease

Paget disease with malignant transformation to osteosarcoma in the proximal tibia where there is mass-like marrow replacement (*) and soft tissue extension (arrow) on radiograph and multiple sagittal T1-weighted MR images

Musculoskeletal Radiology

817

Paget Disease

•

Paget Disease: Neoplasm [Figure 4-11-23]

•

Figure 4-11-22

GCT – skull or facial bones ➢ More often benign ➢ Rarely multiple (familial) ➢ Lytic expansile lesion Metastasis – likely related to hyperemia

Paget Disease: Differential Diagnosis • • •

• •

Diffuse sclerosis – chronic renal failure (CRF), myelofibrosis, metastasis, lymphoma, sickle cell anemia Trabecular thickening – hemangioma, chronic infection, osteomalacia, fluorosis Polyostotic lesions – CRF (hyperparathyroidism), Langerhans cell histiocytosis, unusual infection, metastasis, fibrous dysplasia, lymphoma, Gaucher, mastocytosis

Paget disease with malignant transformation to osteosarcoma in the humeral midshaft where there is mass-like marrow replacement (*) and soft tissue extension (arrows) on radiograph and multiple sagittal T1-weighted MR images, CT and gross specimen

Figure 4-11-23

Paget Disease: Treatment • •

Calcitonin – inhibits resorption Biphoshonates – inhibits bone resorption and production Mithramycin – cytotoxic antibiotic Often relieve pain

Paget Disease: Radiology Post-Treatment • • • •

Often subtle or no change Occasionally improved radiographs Watch for fractures: may increase with diphosphonates Bone scans best treatment indicator

Hereditary Hyperphosphatasia: Juvenile Paget Disease • • • •

Paget disease with benign giant cell tumor of the clavicle associated with Described 1956: Bakwin/Elger pathologic fracture showing cortical thickening (arrows) and destructive lesion Autosomal recessive (*) on CT scans, gross specimen radiograph and gross specimen Disorder of infants/children Elevated alkaline and acid phosphatase, uric acid

Figure 4-11-24

Juvenile Paget Disease: Radiographic Findings • • • • •

[Figure 4-11-24]

Generalized cortical thickening All bones including skull involved Osteopenia and bowing Epiphyses may be spared Patients severely deformed

Juvenile Paget disease (hereditary hyperphosphatasia) on radiographs with osseous bowing and shortening as well as trabecular thickening Paget Disease

818

Musculoskeletal Radiology

• •

Paget Disease: Summary

• • • •

•

Common disease: 3% - 4% Diagnosis: Radiographs ➢ Lytic: well defined, subchondral (v/wedge shape – candle flame) ➢ Thickened trabeculae and cortex ➢ Osseous enlargement Bone scan – overview Complications – fractures, osseous deformity, neurologic symptoms, arthritis, neoplasm CT/MRI to evaluate complications CT/MRI: noncomplicated case ➢ Bone enlargement ➢ Trabecular thickening ➢ T1W: low signal (sclerosis), yellow marrow, heterogeneous non-masslike marrow replacement ➢ T2W: low signal (sclerosis), yellow marrow, heterogeneous high signal, no focal mass CT/MRI: complicated case ➢ CT: focal bone destruction / soft tissue mass ➢ T1W: masslike marrow replacement ➢ T2W: focal mass in marrow with high signal and soft tissue mass

References 1. 2. 3. 4. 5. 6.

Boutin RD, Spitz DJ, Newman JS, Lenchik L, Steinbach LS.. "Complications in Paget disease at MR imaging." Radiology. 1998 Dec;209(3):641-51. Cooper C, Dennison E, Schafheutle K, Kellingray S, Guyer P, Barker D.. "Epidemiology of Paget's disease of bone." Bone. 1999 May;24(5 Suppl):3S-5S. Moore TE, Kathol MH, el-Khoury GY, Walker CW, Gendall PW, Whitten CG.. "Unusual radiological features in Paget's disease of bone." Skeletal Radiol. 1994 May;23(4):257-60. Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnik CS, Gannon FH.. "From the archives of the AFIP. Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation." Radiographics. 2002 Sep-Oct;22(5):1191-216. Vande Berg BC, Malghem J, Lecouvet FE, Maldague B.. "Magnetic resonance appearance of uncomplicated Paget's disease of bone." Semin Musculoskelet Radiol. 2001;5(1):69-77. Vellenga CJ, Bijvoet OL, Pauwels EK.. "Bone scintigraphy and radiology in Paget's disease of bone: a review." Am J Physiol Imaging. 1988;3(3):154-68.

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Paget Disease

Musculoskeletal Infection: Part I Mark D. Murphey, MD • • •

Musculoskeletal Infection: Routes of Inoculation Hematogenous Contiguous spread Direct implantation/post-surgical

Hematogenous Vascular Supply Tubular Bones •

Figure 4-12-1

[Figure 4-12-1]

• • • •

Diaphyseal ➢ Enter through cortex ➢ Ascending/descending branches Metaphyseal ➢ Sharp turns beneath growth plate Epiphyseal ➢ Artery from epiphysis/metaphysis Periosteal ➢ From diaphyseal/muscle/soft tissue vessels Synovial ➢ From epiphysis/surrounding vessels

Hematogenous Vascular Supply Tubular Bones • • • •

[Figure 4-12-2]

Vascular supply to long bone and joint

Age dependent Infant – vessels penetrate growth plate Child – vessels don’t extend across plate Adult – vessels cross closed growth plate

Figure 4-12-2

CHILD

INFANT

ADULT

Vascular supply of tubular bone by patient age

• • • • • •

Pathophysiology of Acute Osteomyelitis [Figure 4-12-3, opposite] Inoculation/colonization/immunologic response Marrow and soft tissue edema Bone destruction – trabecular/cortical Subperiosteal/soft tissue/medullary abscess Vascular thrombosis – infarction (sequestrum) Periosteal new bone (involucrum)

Musculoskeletal Infection I

820

Musculoskeletal Radiology

Figure 4-12-3 a) Deposition

b) Extension

d) Superiosteal Lift

e) Stripping

c) Lateral Spread

Progression of osteomyelitis through the cortex into the subperiosteal space (*)

• • • • •

Radiographic Abnormality: Acute Osteomyelitis

•

Deep soft tissue swelling (within 3 days) Osteoporosis to focal bone lysis (7–14 days); magnification views Periosteal reaction (10–14 days) Increased blood flow: scintigraphy (early) Decreased blood flow: scintigraphy (later)

Other Radiologic Studies: MSK Infection • • • •

Nuclear Medicine – BS: 3 phase studies ➢ Gallium: with BS, active infection ➢ WBC: increased specificity Sonography – fluid collections/effusions CT – bone destruction/sequestra/abscess Sinography – sinus tract extent MRI: marrow involvement, abscess very sensitive (STIR, GADO)

• • • • • •

Staph aureus (80% - 90% of pyogenic cases) H. flu, strep (shoulder, trauma from delivery) Gram negatives, uncommon except enterics (25% of infections) Pseudomonas – penetrating trauma/IVDA Salmonella – sickle cell Blood cultures positive 50%

Osteomyelitis: Organisms

Musculoskeletal Radiology

821

Musculoskeletal Infection I

Hematogenous Osteomyelitis: Infant (Up to 1–2 Years) • • • • • • • •

Figure 4-12-4

[Figures 4-12-4 to 4-12-7]

May be clinically silent (NICU/catheters) Group B strep more common than other ages Metaphysis/epiphysis location Extend into joint Most prominent sequestrum/involucrum Common soft tissue/subperiosteal abscess Fracture/sinus tracts uncommon May lead to late sequelae

Figure 4-12-5 Pictorial representation of infection deposition sites in an infant long bone

Figure 4-12-6

Osteomyelitis in an infant with soft tissue swelling (circle and arrow) as the only initial finding. Compare to normal left arm (right image)

Figure 4-12-7

Subsequent radiographs 1 week (left image) and two weeks (right image) later reveal development of periosteal reaction initially (arrows) followed by extensive involucrum (arrowheads) and sequestrum (*) formation

Figure 4-12-8

Continued follow-up radiographs 1 month (left image), 3 months (middle image) and 1 year (right image) later show progressive resorption of sequestra representing the majority of the humerus (*-”bone within bone appearance”) and subsequent remodeling to nearly normal appearance

Hematogenous Osteomyelitis: Child (1–16 Years) • • • • • • •

[Figures 4-12-8 to 4-12-13]

Tubular bones 75% Metaphysis/lower extremity Can involve joint (hip /shoulder) Sequestrum/involucrum common Soft tissue/subperiosteal abscess common Fracture uncommon Sinus tracts can occur

Musculoskeletal Infection I

Pictorial representation of infection deposition sites in a child long bone

822

Musculoskeletal Radiology

Figure 4-12-9

Figure 4-12-10

Osteomyelitis in the distal femoral metaphysis of the femur with bone destruction (arrows).

Aggressive bone destruction (arrow) and periosteal reaction (arrowheads) in the distal ulnar metaphysis on radiograph and marked uptake of radionuclide on bone scan resulting from acute bacterial osteomyelitis in a child

Figure 4-12-12

Figure 4-12-11

There is marrow replacement on the T1-weighted MR image with focal rim enhancing subperiosteal abscess (arrowheads) posteriorly showing high signal on T2-weighting (*)

Figure 4-12-13

Subperiosteal Abscess •

[Figure 4-12-14]

• • • • •

Common in children/infants ➢ Loose periosteum Femur/tibia – long extension Adults – sinuses/orbit Often don’t recognize on Figure 4-12-14 radiographs Nuclear Medicine – rim with photopenia (BS, WBC) CT/MR/SONO – fluid collection bone surface

Pictorial presentation of subperiosteal abscess

Musculoskeletal Radiology

Civil war gross specimen and radiograph showing extensive involucrum (arrows) and sequestrum (*) resulting from war injury induced osteomyelitis 823

Musculoskeletal Infection I

Hematogenous Osteomyelitis: Adult (Over 16 Years) • • • • •

[Figures 4-12-15 to 4-12-17]

Tubular bones less common Spine/pelvis/small bones more common: subchondral focus Joint involvement/sinus tracts common Involucrum/sequestrum/abscess uncommon Fracture uncommon except neglected cases

Figure 4-12-15

Figure 4-12-17

Figure 4-12-16

Acute bacterial osteomyelitis in an adult. Initial radiograph reveals only soft tissue swelling posterior to olecranon (*). Subsequent radiographs two weeks (right images) later show motheaten to permeative bone destruction resulting from staph aureus osteomyelitis

Pictorial representation of infection deposition sites in an adult long bone

Subacute Osteomyelitis: Brodie Abscess • • • • • •

[Figures 4-12-18 and 4-12-19]

Described in 1832 – chronic/subacute Walled-off with central fluid, often sterile (staph) Children (M>F), metaphysis, tibia Intramedullary - channel-like lucencies May cross growth plate or be cortical Periosteal reaction/sequestra may be seen

Figure 4-12-18

Figure 4-12-19

Brodie abscess with sequestra (arrows) in the cortex of the proximal femur on radiograph, axial CT and coronal CT reconstruction. Debrided sequestered fragment is also demonstrated (*)

Musculoskeletal Infection I

824

Musculoskeletal Radiology

• • • •

Chronic Osteomyelitis [Figure 4-12-20]

•

Bone formation results in sclerosis Sclerosing osteomyelitis of Garre No pus, may have mixed radiolucency Active infection: new bone destruction, sequestra, aggressive periosteal reaction on radiographs MRI, scintigraphy (bone, gallium, WBC)

Figure 4-12-20

Osteomyelitis/Septic Arthritis Contiguous Spread: Source •

Soft tissue infection ➢ Trauma ➢ Human/animal bites ➢ Puncture wounds ➢ Ulcers ➢ Surgery

Osteomyelitis/Septic Arthritis Contiguous Spread: Radiographs • • • •

Soft tissue swelling/air Periosteal reaction Cortical destruction into marrow Joint space loss

Chronic osteomyelitis of the ulna with diffuse sclerosis on radiograph

Figure 4-12-21

Osteomyelitis/Septic Arthritis Contiguous Spread: Hand • • • •

Tendon sheaths, fascial planes, lymphatics Felon-volar tuft destruction Paronychia-dorsal tuft destruction Bites ➢ Human: MCP joint with fight (Staph/strep) ➢ Animal: dog (90%), 5% infected; cat (10%), 20%–50% infected (Pasteurella multocida)

Osteomyelitis/Septic Arthritis Contiguous Spread: Other Sites [Figure 4-12-21] •

Foot – pathways: medial, intermediate, lateral ➢ Puncture: pseudomonas ➢ Diabetes: multiple organisms ➢ Aerobic/anaerobic • Pelvis – decubitus ulcers (paraplegics) ➢ Ischial tuberosity chronic osteomyelitis • Post-operative: ring sequestra • • • •

Diabetic patient with ulcer lateral to fifth MTP joint and radiograph shows underlying destruction of distal fifth metatarsal head and proximal phalanx resulting from osteomyelitis and pyarthrosis. Amputation specimen shows similar findings

Osteomyelitis: Diabetes Mellitus Bone destruction/periosteal reaction Bone scan – normal – no osteomyelitis WBC scan – good predictive value MRI – marrow replacement geographic area T1 and increased signal STIR, post gado ➢ Normal on T1 or subtle/subcortical only; then probably marrow reaction not osteomyelitis (Collins et al AJR 185:2005) ➢ Focal fluid collections

Figure 4-12-22

Osteomyelitis vs Neuropathic Diabetes Mellitus • •

Factors favoring infection: Sinus tract (84%/0%); ST replacement (68%/32%); Fluid collection (95%/48%); Extensive marrow abnormality (41%/12%) Factors favoring neuropathic: Thin rim enhancement of effusion (68%/21%); Presence of subchondral cysts (76%/2%); Intraarticular bodies (53%/12%)

Chronic osteomyelitis with sinus tract and secondary epidermoid carcinoma. Aggressive bone destruction is seen distally and medially corresponding to malignancy (arrow and *) as sinus tract enters bone

Ahmadi et al, Radiology 238; 622-631, 2006 Musculoskeletal Radiology

825

Musculoskeletal Infection I

• • • • • • •

Complications of Osteomyelitis [Figure 4-12-22]

Figure 4-12-23

Sequestra discharged through sinus tracts Avascular necrosis Fracture/slipped epiphysis Growth plate disturbance Osteolysis Systemic amyloidosis (rare) Epidermoid carcinoma ➢ 0.5% long term draining sinus ➢ M>F, 20–40 year latent period ➢ Tibia/femur ➢ Arise deep in sinus tract

•

Septic Arthritis: Bacterial Cause ➢ Hematogenous, contiguous spread, direct implantation, postsurgical • Polyarticular – 20% • Organisms ➢ H. flu – leading cause up to age 2 years ➢ Staph, alpha/beta hemolytic strep. ➢ Pneumococcus, E.coli, Pseudomonas • • • • •

Figure 4-12-24

Septic Arthritis: Pathology [Figure 4-12-23]

• • • •

Pictorial representation of septic arthritis

Synovial inflammation /hyperemia/fluid production Fibrin deposits inhibit cartilage nutrition Attract WBC’s release enzymes (collagenase) Pannus formation Cartilage destruction/bone erosion

Septic Arthritis: Radiology [Figure 4-12-24] Soft tissue swelling / joint effusion (sonography) Periarticular osteopenia Increased vascularity – scintigraphy Pannus – arthrography – gadolinium – enhanced MRI

Septic arthritis and osteomyelitis of the fourth MTP joint with joint narrowing and bone destruction (arrowhead)

Figure 4-12-25

Septic Arthritis: Hip Infant/Childhood [Figure 4-12-25] • • •

Staphylococcus aureus Spread from adjacent osteomyelitis Radiologic signs ➢ Widened hip joint/effusion (sonography) ➢ Displaced pericapsular fat planes • Surgical emergency

Septic Arthritis: Complications/Sequelae [Figure 4-12-26] • • • • • • •

Avascular necrosis Slipped epiphysis Growth disturbance Osteomyelitis Secondary osteoarthritis Synovial cyst, tendon/capsule injury

Septic arthritis in the right hip of a young child with joint widening indicating a joint effusion on radiography

Figure 4-12-26

Soft Tissue Infection • • • •

Septic bursitis – usually injury ➢ Staph/prepatellar (child) Septic tenosynovitis Cellulitis Necrotizing fasciitis Soft tissue abscess – pyomyositis ➢ Staph (90%) Complication of septic arthritis with right hip osseous ankylosis on radiography

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•

Soft Tissue Infection: Radiology [Figure 4-12-27]

Figure 4-12-27

Radiographs ➢ Soft tissue swelling ➢ Air (rare): clostridia, E. Coli (coliform), bacteroides • CT/MRI/Sonography ➢ Inflammatory changes ➢ To detect focal abscess ➢ Contrast enhancement of rim • •

Cellulitis Acute inflammatory process of deep subcutaneous tissues Location ➢ Extremities, thorax, abdomen, neck • Organisms ➢ Streptococci, staphylococci, H. influenza • • • • •

Necrotizing Fasciitis: Clinical Infection and necrosis of fascia (Staph/Strep) Important to distinguish from cellulitis Systemic severe toxicity - IVDA Extremities, neck, face, perineum High mortality (>70% if not treated) ➢ Need prompt aggressive treatment • Treatment–Fasciotomy, debridement, antibiotics • • • • •

Necrotizing Fasciitis: Imaging Skin thickening Subcutaneous edema and air Involves deeper tissue unlike cellulitis Focal fluid collections (abscess) Gadolinium enhancement

Chronic Granulomatous Disease of Childhood • • • • • • •

X-linked recessive (males) WBC’s cannot kill organisms Skin lesions, lymphadenitis, Soft tissue abscess in the buttock on CT and MR showing focal fluid collection hepatosplenomegaly (*) with internal foreign material (arrows) after accident ➢ Pneumonias, chronic osteomyelitis (25%–35%) Fatal – 40% cases – low virulent organisms Symptoms of osteomyelitis limited Bone destruction/limited sclerosis Small bones hands/feet, chest wall, spine

Figure 4-12-28

Chronic Recurrent Multifocal Osteomyelitis (CRMO) [Figure 4-12-28] • • • • • • • • • • •

Chronic symmetric plasma cell osteomyelitis Indolent/recurrent bone infection Age 5–15 years; knee metaphyses, clavicle, face Mixed lysis/sclerosis May be sterile, plasma cells, lymphs and histiocytes SAPHO (Synovitis, Acne, Pustular rash palms/soles, Hyperostosis, Osteitis)

Musculoskeletal Infectionn Drug Abusers Altered WBC function and infected needles “S” joints – spine, sacroiliac, and sternal joints AC joint, symphysis, ischial tuberosity Pseudomonas, Klebsiella, Serratia Candida in heroin addicts

Musculoskeletal Radiology

CRMO with patchy areas of lucency and sclerosis in the distal tibial metaphysis on radiography. Contralateral ankle, ipsilateral knee and iliac bone showed similar findings (not shown)

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•

Spine Infections: Routes of Contamination Hematogenous ➢ Arterial ➢ Venous: Batson Plexus • Contiguous source • Direct implantation/post-operative ➢ Discography (-ectomy), biopsy, laminectomy • • • • • • •

Spondylodiskitis 2%–4% of all osteomyelitis cases M>F (1.5–3:1); age 40–60 years Lumbar > thoracic > cervical/sacrum Vertebral body History: recent primary infection Symptoms: back pain, fever, neurologic

• • • •

Staph aureus (55%–90%) Localizes to anterior subchondral bone Rapidly extends into disc (1–3 weeks) Can extend into paravertebral soft tissue

Spine Infections: Clinical Characteristics Figure 4-12-29

Spine Infections: Pathology

•

Spine Infections: Radiography • • • •

Initially radiographs normal or subtle subchondral destruction Usually seen radiologically after disc involved Rapid disc narrowing with irregular endplate destruction Later osteosclerosis (10–12 weeks) Treatment: diffuse sclerosis, DDD, disc ankylosis

Bacterial spondylodiskitis with rapid disk space loss and endplate destruction over two weeks (right image) due to staph aureus infection. Left image initial radiograph

• • •

Spine Infections: CT/MR Imaging [Figure 4-12-29] MR imaging optimal Bone destruction/marrow replacement (T1W) High signal T2W MR images ➢ Disc and adjacent vertebra • Post gadolinium images helpful ➢ Focal abscess detection • Paravertebral soft tissue masses (20% pyogenic cases) ➢ Anterior and lateral (psoas) ➢ Epidural •

Spine Infections: Differential Diagnosis Amyloid in CRF ➢ Low signal T2W MR images • Tumor-crossing disc ➢ Chordoma, lymphoma, myeloma, GCT • Other causes disc narrowing (well-defined bone margins) ➢ DDD, CPPD, neuropathic, RA, trauma, sarcoid

Invertebral Discitis: Clinical Characteristics •

Hematogenous – children (1–16 years) ➢ Disc vascularized • Symptoms – mild after primary infection • Cultures negative (50%–90%); staph aureus • Antibiotics given empirically • • • • •

Intervertebral Discitis: Radiology Scintigraphy positive early Late disc narrowing and erosion MRI findings similar to adults Paravertebral soft tissue changes unusual Disc often reconstitutes after therapy but may remain deformed with sequelae

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Musculoskeletal Infection Part II: Atypical Organisms Mark D. Murphey, MD • •

Unusual Bacterial Musculoskeletal Infection Brucella Mycobacteria (acid-fast bacilli) ➢ Tuberculosis ➢ Atypical ➢ Leprosy • Actinomycosis • • • • •

Brucellosis

• • • •

Malta fever – undulant fever WHO – 500,000 cases annually B. – abortis, melitensis, suis, canis Endemic Midwest USA, Saudi Arabia, South America, Spain, and Italy Ingested milk/meat – reticuloendothelial system (marrow)

Brucellosis [Figure 4-13-1] Musculoskeletal infections 30%–85% Septic arthritis – knee, sacroiliac joint, shoulder Prepatellar bursitis Spine – 53% – lumbar (lower L4) ➢ Focal: subchondral anterior superior endplate (parrot beak phyte) ➢ Diffuse: vertebral body maintained, less disc and paravertebral involvement, disc gas (25%–30%)

Al-Shahed, Radiographics 94:14:333-348

Figure 4-13-1

Brucellosis spondylodiscitis on radiograph, CT and sagittal T1- and T1- weighted postcontrast MR images shows multilevel involvement of the discs and vertebral bodies (arrows,open arrows,arrowheads and *) and paraspinal and anterior epidural inflammatory changes (curved arrows and arrows on MR). Note the lack of vertebral collapse and “parrot beak” (open arrows on radiographs) osteophytes

Musculoskeletal Tuberculosis: Clinical Characteristics • • • • • •

Increasing – immunocompromised patients 1%–3% of TB patients – MSK involvement Pain, swelling, stiffness – long delay to diagnosis Mortality still significant Negative skin test – MSK TB unlikely Negative CXR (child) – MSK TB unlikely

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• •

Musculoskeletal Tuberculosis: Pathology Hematogenous, pulmonary changes 50% Tubercles: central giant and epithelioid cells ➢ Central caseating necrosis (may calcify) ➢ Peripheral: lymphocyte mantle • Synovial tissue/fluid: 80%–90% positive culture • • • • • •

Musculoskeletal Tuberculosis: Sites of Involvement

• • • •

Spine: 25%–60% of MSK cases Other osseous sites unusual Dactylitis Arthritis – knee, hip Bursitis/tenosynovitis – hand/wrist BCG related

Tuberculous Spondylodiscitis May result in neurologic symptoms Hematogenous – venous (Batson) plexus L1 most common: decreases above/below More than one level frequent ➢ Usually contiguous ➢ Separate foci: 1%–4%

Tuberculous Spondylodiscitis: Radiology •

[Figures 4-13-2 to 4-13-7]

• • • • • •

• •

Subchondral vertebral body (2–5 months) ➢ Anterior 82%, posterior 18% Disc extension Less common involvement – posterior elements, isolated vertebral body (ivory) Subligamentous extension – “gouge” defects Kyphosis (gibbus) – thoracic Paraspinal extension – psoas (L1-L5) ➢ Calcification: amorphous/teardrop ➢ Abscess (5% psoas) MR imaging usually optimal ➢ Assess extent/relationship to canal ➢ Abscess shows rim enhancement

Tuberculous Spondylodiscitis: Differential Diagnosis Pyogenic

• • • • •

Can be difficult Findings favoring tuberculosis ➢ Multilevel involvement ➢ Slow vertebral/disc destruction ➢ Calcified paraspinal mass ➢ Lack of sclerosis

Figure 4-13-2

Tuberculous Osteomyelitis Not common, usually with arthritis Often epiphyseal, any bone, joint Children metaphyseal cross plate Cystic variety – multifocal defined lytic foci Dactylitis – < age 5 (0.5%–14% cases) ➢ Multifocal (25%–35%) ➢ Spina (spike-like) ventosa (puffed full of air)

Pictoral representation of tuberculous spondylodiscitis

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Figure 4-13-3

a

b Tuberculous spondylodiscitis: a) Radiographs show myelographic block (arrowhead) with endplate destruction, collapse, and disc involvement (arrow); b) CT reveals large paraspinal mass (*); c) Sagittal T1-weighted MR images show marrow replacement and disc involvement at two levels (arrowheads) as well as anterior paraspinal and posterior epidural masses (arrows) ; d) Sagittal and axial post-contrast T1-weighted MR images reveal rim enhancement about anterior paraspinal and posterior epidural abscesses (arrows); e) High signal intensity is seen on the axial and sagittal T2weighted MR images in the involved vertebrae, discs and paraspinal abscesses (*)

c

d

e

Figure 4-13-5

Figure 4-13-4

Anterior gouge defects on sagittal T2-weighted MR image and sagittal macrosection resulting from tuberculous spondylodiscitis extending under the anterior longitudinal ligament (arrows) and invading other vertebral segments (arrowheads)

Musculoskeletal Radiology

Sagittal dried bone specimen and gross specimen show effects of tuberculous spondylodiscitis with gibbus deformities (arrows) and anterior epidural inflammatory mass (*) displacing the spinal cord (c) posteriorly

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Figure 4-13-7

Figure 4-13-6

Teardrop paraspinal calcification (*) in a tuberculous paraspinal abscess related to spondylodiscitis

CT of the spine in a patient with tuberculous spondylodiscitis and calcified paraspinal abscesses (arrows)

Figure 4-13-8 • • • • •

Tuberculous Arthritis [Figure 4-13-8] Large joints (knee/hip); monoarticular Synovial thickening covered by fibrin Granulation tissue erodes cartilage bone Slow process/lack proteases Areas of cartilage contact spared

Tuberculous Arthritis: Radiology •

[Figures 4-13-9 and 4-13-10]

Phemister triad ➢ Juxtaarticular osteopenia ➢ Slow joint space loss ➢ Peripheral erosions • Joint effusion • MRI – nodular synovial thickening • Less reactive bone, periostitis, osseous ankylosis

Figure 4-13-9

Pictorial representation of tuberculous arthritis (right image, left image normal joint)

Figure 4-13-10 Pelvis radiographs over a two month interval show slow pancompartmental loss of the right hip joint resulting from tuberculous arthritis

Radiograph of tuberculous arthritis with diffuse joint space loss, marginal erosions (arrows) and osteopenia (Phemister triad) and sagittal macrosection showing fibrinous exudate (*) throughout the joint

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• • •

Atypical Mycobacterium [Figure 4-13-11]

•

Figure 4-13-11

Immunocompromised patients Early diagnosis/treatment important – skin/pulmonary Types – photochromogens (M. Kansasii), nonchromogens (M. avium); rapid growers Bone/joint – multiple lesions, less osteopenia, hand/wrist, metaphysis/diaphysis lysis/sclerosis, abscess/sinus tracts common

• • • • •

Africa, South America, Asia USA – Texas, Louisiana, Hawaii, Florida Long incubation 3–6 years Infection through skin/mucous membranes M>F; usually detected before age 20

• • • •

Lepromatous – many bacilli more severe/generalized Tuberculoid – more reaction/less bacilli/skin & nerve involvement Dimorphous – features of both Indeterminate

• • • • •

Related to bacilli presence Uncommon 1%–2% findings Small bones hands/feet (direct spread) Punched out or lacelike osseous destruction phalanges, nasal bone Periosteal reaction, fragmentation, arthritis

Leprosy: Mycobacterium Leprae

Leprosy: Pathology Types

Leprosy Direct: Specific Signs [Figures 4-13-12 and 4-13-13]

Figure 4-13-12

Sagittal T1-weighted and axial T2-weighted MR images reveal marked thickening about the second flexor tendon (arrowheads) related to atypical mycobacteria tenosynovitis. Chronic fibrosis causes the intermediate signal on T2-weighting (arrow)

Figure 4-13-13 Lattice-like lucencies in the phalanges representing a direct sign of leprosy infection on radiography

• • • • •

Actinomycosis: Clinical Characteristics Anaerobic higher bacteria; acid fast-like Actinomyces (Israelii), nocardia (asteroides) Normal flora in mouth Trauma often results in inoculation Immunocompromised patients

Xeroradiography shows medial distal arm calcification that is located in the ulnar nerve (*) on the resected gross specimen resulting from leprosy

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•

Actinomycosis: Radiology [Figure 4-13-14]

Figure 4-13-14

• • •

Sites mandible, axial skeleton (rib, spine, pelvis), large joints Lysis and sclerosis (bone proliferation) Spine (posterior elements) and rib Sinus tracts/abscesses (no calcification) common

• • • • •

Syphilis Yaws Lyme disease Tropical ulcer Bejel, rat bite, fever, leptospirosis

Spirochetes Actinomyces osteomyelitis in the mandible on radiograph showing patchy areas of destruction and sclerosis (arrowheads)

• •

Congenital Syphilis: Early Changes [Figure 4-13-15] Toxic effects degenerating spirochetes/infection Osteochondritis – metaphyseal lucent bands/irregularity ➢ Long bone (tibia: Wimberger sign), rib, spine, sternum ➢ Epiphyseal widening/separation ➢ Heal quickly with treatment • Osteomyelitis – diaphyseal lysis/sclerosis/periostitis • Diffuse periostitis • •

Figure 4-13-15

Congenital Syphilis: Late Changes • • • • • •

Congenital syphilis – 75% diagnosed after age 10 Hutchinson triad (Hutchinson teeth, interstitial keratitis, nerve deafness) May be similar to early changes Usually more like acquired findings Dactylitis – fingers more common Painless knee effusions (Clutton joints)

Congenital Syphilis: Early Changes

• • • •

Congenital syphilis of the lower extremity showing periosteal reaction (arrows) and osteochondritis (arrowheads) of the proximal tibiae (Wimberger sign)

Bone changes in up to 20% (early as 6 weeks) Proliferative periostitis ➢ Most common finding ➢ Tibia (saber shin), skull, ribs, sternum ➢ Solid/laminated (rarely perpendicular) Osteomyelitis/osteitis/septic arthritis Much less common Skull: outer table aggressive lysis Nasal bone collapse – saddle nose

• •

Congenital Syphilis: Late Changes Periostitis/osteomyelitis/osteitis Gumma – any bone caseous necrosis related to degenerating spirochete ➢ Bone resorption: Carries sicca ➢ Bone lysis/reactive sclerosis • Arthritis – uncommon – ankles, MTP, elbows, knees ➢ Swelling, effusion, narrowing, destruction ➢ Neuropathic (5%–10%) – knee, hip, ankle, spine • • • •

Treponema pertenue Africa, South America, South Pacific Very similar to syphilis – less nose changes, more phalanges (spares distally) Exostosis maxilla – goundou

• • • • •

Central/East Africa Lower leg ulcer destroys muscle/tendon To bone focal osseous production tibia/fibula Multiple organisms including spirochetes Epidermoid carcinoma 25% (>10 years latency)

Yaws

Tropical Ulcer

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• • • • • • •

Lyme Disease: Clinical Characteristics

Figure 4-13-16

Recognized 1975; 23,763 new cases (2002) Most common vector borne illness in USA USA (NE/MidAtlantic), Europe, Far East, Australia Tick bite - ixodes dammini (transmissiom) Spirochete - Borrelia burgdorferi (deer, mice) Skin lesions (erythemia chronicum migrans 20%) Joint symptoms (usually 2-6 mths; 2 weeks - 2 yrs) ➢ 80% cases reported May-August

•

Lyme Disease: Radiology [Figure 4-13-16] Knee (80%), shoulder, elbow, temporomandibular, ankle, wrist, hip, hand/foot • Monoarticular, oligo, or polyarticular • Soft tissue swelling/effusion (MRI synovitis) ➢ Adenopathy, myositis, lack subcutaneous edema • Chronic changes (10%) – osteopenia, joint loss (25%), erosions, secondary carcinoma • • • • • • • •

Musculoskeletal Infection: Fungal [Figures 4-13-17 to 4-13-20] Aspergillosis Blastomycosis Candidiasis Cryptococcosis Histoplasmosis Mucormycosis Sporotrichosis Madura Foot

Fungal Musculoskeletal Infection: Common Changes • • • • •

Frequently in immunocompromised Osteomyelitis large punched out lytic lesions May have surrounding sclerosis Often multifocal/may involve bone protuberances Joint involvement – slow progressive destruction ➢ MRI: nodular synovial thickening

• •

Aspergillosis

Lyme arthritis of the knee on lateral radiograph with effusion (* ) and MR revealing synovitis (arrows), myositis (M) and adenitis (A)

A. Fumigatus – normal URT organism Sites related to pulmonary changes (children) or ➢ Hematogenous (adult): rib, sternum, spine • Arthritis rare •

Figure 4-13-17

Blastomycosis • • • • •

B. Dermatiditis (N. American) ➢ Ohio, Miss. River Valleys, Mid-Atlantic B. Brasiliensis – Mexico, Central/South America Skin/pulmonary entry from soil Bone involved in up to 60% patients Ribs, spine, tibia, carpus, tarsus, skull Arthritis <10% patients

• • • • •

C. Albicans but many other species Patients on hyperal, antibiotics, intraarticular steroids Bone involvement rare (1%–2%) Monostotic/monoarticular – long bone, sternum, spine, knee Arthritis more often precedes osteomyelitis

• • • • •

C. Immitis – soil- inhalation SW USA, Mexico <1% disseminated; (10%–50% MSK changes) Metaphyseal, may be symmetric Joints – ankle, knee, also migratory arthritis

Candidiasis (Moniliasis)

Coccidioidomycosis

Musculoskeletal Radiology

Aspergillus infection with pulmonary and rib (arrowheads) involvement

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• • • •

Torulosis, C. Neoformans Soil – inhalation Disseminated disease, 5%–10% MSK changes Arthritis unusual

• • • • •

H. Capsulatum – USA (Miss. River Valley) H. Dubosii – Africa – MSK changes 80% cases Soil – inhalation; most common fungal in USA Pelvis, skull, ribs, small tubular bones Arthritis – knee, ankle, wrist, hand

• • • • •

Phymycetes – rhizopus, mucor, absidia Diabetes, uremia, burns Entry via sinuses Skull/face (maxillary/ethmoid sinuses) Bone destruction

• • • • • • •

Blastomycosis of the humerus with Sporothrix schenckii extensive involvement demonstrating Saprophyte on vegetation mixed lysis and sclerosis Inhalation/skin wound (rose thorn) Disseminated form 80% MSK changes Arthritis (monoarticular) common – knee (66%) hand wrist (52%) ankle, elbow Osteopenia often not prominent Osteomyelitis – due to arthritis

Cryptococcosis

Figure 4-13-18

Histoplasmosis

Mucormycosis

Sporotrichosis

Figure 4-13-19

Figure 4-13-20 Sporotrichosis of the knee with CT (post-arthrogram) and MR showing enhancing nonspecific nodular synovial thickening (arrowheads) after intravenous contrast Histoplasmosis with multifocal area of lysis, many of which involve tuberosities and trochanteric regions ("lumps and bumps" of bone)

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• • •

Madura Foot: Mycetoma [Figure 4-13-21]

• • • •

Figure 4-13-21

Chronic granulomatous infection Foot (65%–70%), hand, legs, back/head Many organisms can be cause – Eumycetes (Madurella), Actinomycetes, Monosporium Apiospermum (USA) Tropics – India, Africa, Arabia, Latin America Tarsals/metatarsals most involved

Viral/Protozoan Musculoskeletal Infection

• • •

Torch – metaphyseal lucent bands (celery stalking) Cat-scratch disease – viral like bacteria AFIPIA felis (R. Hensalae and Bartanella species) ➢ Look for adenitis (epitrochlear)

Aids: Musculoskeletal Changes [Figure 4-13-22]

• • •

Seronegative arthropathy Osteomyelitis, septic joint/bursitis any organism Pyomyositis (staph); lower extremity (95%), multiple (50%) Bacillary angiomatosis (rochalimaea hensalae, quintana) skin lesion/bone destruction (cortex prominent) Neoplasm – Kaposi sarcoma, lymphoma

Helminths/Worms • • • •

Musculoskeletal changes usually soft tissue calcification Loa loa - Africa – subcutaneous calcification (fine lace-like or thicker bead-like) Filariasis – lymphatic obstruction (elephantiasis) Guinea worm (dracunculosis) – long calcification female worm lower extremity hand – can cause arthritis Cysticercosis – linear/oval rice grain calcification along axis of muscle (up to 20–25 mm length)

Echinococcus: Musculoskeletal Changes • • • • •

[Figure 4-13-23]

Echinococcus (E. multilocularis/granulosis) Bone disease: 0.5%–4% (E. granulosis) Spine, long bone epiphysis,iIlium, skull, rib Multiloculated (bundle of grapes) lysis/expansion May be soft tissue loculated, cyst margins may calcify

Mycetoma (Madura foot) on radiographs, sagittal T1-and T2weighted MR images and gross specimen show extensive multifocal destruction with sclerosis/fibrosis representing chronicity

Figure 4-13-22

• •

Sarcoid: Musculoskeletal Usually have chest changes (80%–90%) Muscle – myositis (50%–80% patients) ➢ MRI low signal central scar • Subcutaneous soft tissue nodules (5%) • Arthritis (10%–35%) – acute/chronic ➢ Hand, wrist, ankle, knee, elbow

Sarcoid - Musculoskeletal: Osseous Changes [Figures 4-13-24 and 4-13-25] • • • • • • •

1%–13% of patients; may be asymptomatic Lattice like lysis (hands) Punched out lytic lesions May appear aggressive Acroosteolysis, acrosclerosis (30%–50%) Areas of sclerosis Marrow replacement creating mottled appearance (MRI)

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Coronal STIR MR shows a focal fluid collection (*) representing pyomyositis in an HIV patient

Musculoskeletal Infection II

Figure 4-13-23

Figure 4-13-24

Typical lattice like multifocal lucencies of several phalanges resulting from sarcoid

Paraspinal echinococus infection with multiloculated fluid filled cysts (*) on axial T1- and T2-weighted MR images and gross specimen

Figure 4-13-25

Sarcoid marrow involvement showing heterogeneous or mottled marrow signal intensity diffusely on sagittal T1-weighted cervical spine MR images

References (Musculoskeletal Infection Parts 1 and 2) 1. 2. 3. 4. 5. 6. 7.

Erdman WA, Tamburro F, Jayson HT, Weatherall PT, Ferry KB, Peshock RM. Osteomyelitis: characteristics and pitfalls of diagnosis with MR imaging. Radiology. 1991 Aug;180(2):533-9. Jung NY, Jee WH, Ha KY, Park CK, Byun JY. Discrimination of tuberculous spondylitis from pyogenic spondylitis on MRI. AJR Am J Roentgenol. 2004 Jun;182(6):1405-10. Lawson JP, Rahn DW. Lyme disease and radiologic findings in Lyme arthritis. AJR Am J Roentgenol. 1992 May;158(5):1065-9. Review. Palestro CJ, Kipper SL, Weiland FL, Love C, Tomas MB. Osteomyelitis: diagnosis with (99m)Tc-labeled antigranulocyte antibodies compared with diagnosis with (111)In-labeled leukocytes--initial experience. Radiology. 2002 Jun; 223(3):758-64. Sharif HS, Aideyan OA, Clark DC, Madkour MM, Aabed MY, Mattsson TA, al-Deeb SM, Moutaery KR. Brucellar and tuberculous spondylitis: comparative imaging features. Radiology. 1989 May;171(2):419-25. Sharma P. MR features of tuberculous osteomyelitis. Skeletal Radiol. 2003 May; 32(5):279-85. Epub 2003 Mar 25. Unger E, Moldofsky P, Gatenby R, Hartz W, Broder G. Diagnosis of osteomyelitis by MR imaging. AJR Am J Roentgenol. 1988 Mar;150(3):605-10.

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Imaging of Cervical Spine Trauma Mark D. Murphey, MD • • • •

Cervical Spine Trauma: Demographics Most common portion of spine injured Responsible for 65% of all spinal injuries Mechanism: MVA/Fall/Sports injury Spinal cord injury: 40% (10,000 annually)

•

Cervical Spine Trauma: Patterns Areas most commonly involved ➢ C1 – 2 (particularly in children) ➢ C5 – 7 • Other fractures 20% • Particular association of low cervical fracture with high thoracic and thoracolumbar injury • •

Cervical Spine Trauma: Radiographic Signs - Normal ABC’S - alignment, bone integrity, cartilage (joint/disc space), soft tissues Lateral view - anterior/posterior vertebral body arcs ➢ Spinolaminal arc (except childhood pseudosubluxation C2-3) • AP view-spinous and lateral mass arcs •

Cervical Spine: Normal Measurements • • • • • • • •

Lateral atlantoaxial offset ➢ (“open mouth” view) - 2 mm Predental space - 3 mm adult; 5 mm child Anterior vertebral height vs. posterior ➢ 2 mm (except C5) Pretracheal space at C6 - 22 mm adult, ➢ 14 mm child Facet width – 2 mm Listhesis with flexion/extension - 2 mm Retropharyngeal space at C2 - 7-8 mm ➢ Exceptions: ET/NG tubes; inflammatory process/crying child Interspinolaminar space - 2 mm ➢ Between 3 continuous levels

Cervical Spine Trauma: Radiographic Evaluation • • • • • • • •

Standard 3 view series ➢ AP, lateral, open mouth ➢ Kasabach view modified oblique open mouth Oblique views Trauma oblique views Swimmer (Twining) views Upright, lateral, flexion, extension

Trauma Oblique: Radiographs Developed by Gehweiler and Abel X-ray tube angled 30o - 40o from horizontal Add 15o cranial tube tilt Better than Swimmer view for cervico-thoracic junction

• • •

Flexion and Extension: Radiographs Use upright lateral first to evaluate cervical spine straightening/reversal To evaluate abnormal alignment/stability False negative from muscle spasm ➢ Repeat after delay • Performed under physician guidance ➢ Passive motion

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Imaging of Cervical Spine Trauma

•

Cervical Spine Trauma: CT Indispensable, widely available, rapid study ➢ 1-3 mm sections, coronal/sagittal reconstructions ➢ Spiral/Multichannel/Holography ➢ 3D helpful to depict spatial relationships

•

Cervical Spine Trauma: MR Imaging/Myelography MRI indications ➢ Post traumatic cervical myelopathy/radiculopathy ➢ Clinical symptoms unexplained by other radiologic studies ➢ Assess ligamentous injury • Myelography (CT) largely replaced by MRI ➢ CSF obstruction ➢ Nerve root avulsion, dural tear •

Figure 4-14-1

Cervical Spine Trauma: Stability • •

Mechanical – ability to not deform under physiologic stress Neurologic – potential to produce new or increase previous deficit Acute/Chronic

Cervical Spine Trauma: Radiographics Signs Instability • • • • • •

Widened interspinous spaces (>2 mm) Widened apophyseal joints (>2 mm) Anterior listhesis > 3.5 mm Narrowed/widened disc space Focal angulation of >11o Vertebral compression > 25%

Pictoral representation of flexion sprain cervical injury with distraction forces posteriorly causing interspinous widening or fanning (*)

Cervical Spine Trauma: Classification by Mechanism •

Hyperflexion ➢ Modified by rotation/lateral flexion • Hyperextension ➢ Modified by rotation • Axial loading – burst • Complex, poorly understood mechanism

Figure 4-14-2

Cervical Spine Trauma: Hyperflexion Injuries • • • • • • •

Account for 50% - 80% of injuries Flexion forces maximal at C4 – C7 anterior; distraction posterior Sprain; compression fracture Facet fracture/subluxation/dislocation Flexion teardrop fracture Clay (coal) shovelers fracture Lateral flexion fractures – unilateral occipital condyle/lateral mass C1 ➢ Uncinate or transverse process

Cervical Spine Trauma: Hyperflexion Sprain • • •

Lateral radiograph of flexion sprain cervical injury with distraction forces posteriorly causing interspinous widening or fanning at C6-7 (arrows)

[Figures 4-14-1 and 4-14-2]

Disrupted one-level posterior ligaments by distraction Acute focal pain/limited ROM Delayed instability (30% - 50%) - lack symptoms (delayed flexion/extension views) • Radiographic findings ➢ Focal kyphosis, mild anterolisthesis ➢ Widened facet, interspinous/interlaminar spaces ➢ Widened posterior, narrowed anterior disc ➢ Compression fracture often associated ➢ All findings accentuated with flexion; MRI to confirm ligament injury

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Musculoskeletal Radiology

Cervical Spine Trauma: Compression Fracture • • •

Figure 4-14-3

[Figure 4-14-3]

Associated hyperflexion sprain common Usually stable unless > 25% compression Radiographs ➢ Loss of height superior endplate ➢ Focal cortical angulation ➢ Band of increased density from impaction

Cervical Spine Trauma: Unilateral Facet Injury • • • • • •

Hyperflexion and rotation Common – 13% of cervical injuries Radicular symptoms common Most frequent C4 – C6 Often mechanically stable, PLL partially intact Unstable with prominent articular mass/laminar fractures

Unilateral Facet Injury: Radiologic Characteristics • • •

[Figures 4-14-4 and 4-14-5]

Anterolisthesis < 50% vertebral width Dislocated facet anterior (oblique view in foramen) Abnormal spinolaminar space/facet rotation ➢ “Bow-tie” sign • Spinous process rotation toward side of dislocation

Sagittal MR (proton density and T2) of flexion sprain cervical injury with superior endplate fracture (arrow) and distraction forces posteriorly causing interspinous widening or fanning at C6-7 (*)

Figure 4-14-4

Unilateral Facet Injury: Radiologic Characteristics [Figure 4-14-6] •

CT ➢ “Naked” facet (may be subtle and partial) ➢ Contralateral facet subluxation common ➢ Articular mass fracture (73%) isolating pillar (17%), posterior vertebral body fracture (25%) ➢ MRI/MRA – disc herniation and vertebral artery injury not uncommon

Figure 4-14-5

Pictoral representation of a unilateral facet injury (circle)

Figure 4-14-6

Radiographs (AP and lateral) of a unilateral facet injury (circle) with subluxation. Note that on the AP film the spinous processes above the level of injury are shifted to the left. Also on the lateral film the facets below the level of injury are projected as in a lateral projection whereas above the level of injury they are obliqued consistent with the rotational component of force

Musculoskeletal Radiology

Sagittal CT of a unilateral facet injury with locked facets (IF-inferior facet of the level above; SF-superior facet of the level below) 841

Imaging of Cervical Spine Trauma

Cervical Spine Trauma Facet Injury: Bilateral [Figures 4-14-7 and 4-14-8]

Figure 4-14-7

• • • • •

Hyperflexion may be some rotation At least as common as unilateral injury Disrupted PLL, disc, and often ALL Unstable injury High incidence of cord damage ➢ (72% quadriplegia) • Bilateral facet dislocation may be partial or complete

Bilateral Facet Dislocation: Radiologic Characteristics [Figures 4-14-9 and 4-14-10] • • • • • •

Anterolisthesis > 50% vertebral body diameter Dislocated inferior facets, anterior to superior facets Dislocated facets in foramen – oblique views Findings of hyperflexion – fanning, focal kyphosis, disc narrowing Spinous processes not rotated CT – “naked” facets, small fracture fragments often not seen on radiographs

Pictoral representation of a bilateral facet injury with perched facets

Figure 4-14-8

Figure 4-14-9

Pictoral representation of a bilateral facet injury with locked facets

Figure 4-14-10 Lateral radiograph of a bilateral facet dislocation with anterolisthesis (arrow) at C4-5. Also on the lateral film the inferior facets (IF) of the level above is anterior to the superior facets (SF) of the level below

Sagittal CT’s of bilateral facet dislocation in two different patients with the inferior facets (IF) of the level above anterior to the superior facets (SF) of the level below

Imaging of Cervical Spine Trauma

842

Musculoskeletal Radiology

Cervical Spine Trauma: Flexion Teardrop Fracture • • • •

Figure 4-14-11

[Figure 4-14-11]

Most severe devastating flexion injury Usually lower cervical spine C5-6 (70% of cases) Diving accident shallow pool common cause Immediate, complete and permanent quadriplegia (90% of cases) • Acute anterior cord syndrome – loss pain, temperature, and touch ➢ Retention position, motion, vibration (posterior column senses)

Flexion Teardrop Fracture: Radiologic Characteristics [Figure 4-14-12] • • • • •

Involved vertebrae and levels above in severe flexion Vertebral body fracture with triangular fragment from anteroinferior corner Central vertebral body not severely involved but posteriorly displaced Bilateral facet subluxation/dislocation MRI/MRA – disc herniation and vertebral artery injury not uncommon

Pictoral representation of a flexion teardrop injury

Figure 4-14-12

Cervical Spine Fracture: Clay Shoveler Fracture • • • • • •

Avulsion C7, C6, T1 spinous process Result of abrupt flexion against opposing interspinous ligament Stable injury Oblique fracture spinous process May see “double’ spinous process sign (AP radiograph) Spinous process fractures can also result from extension/direct trauma

Cervical Spine Trauma: Hyperextension Injuries • • • • • • •

[Figure 4-14-13]

Usually caused by force to face/forehead Compression posteriorly, distraction anterior Less common than hyperflexion injuries (19% - 38%) Atlas and laminar fractures Hyperextension teardrop fracture Hangman fracture Pillar fracture

Radiographs (AP and lateral) of a flexion teardrop injury with facet widening (circles and solid arrows), interspinous fanning (*) and vertebral fracture with teardrop fragment anteriorly (open arrow)

Figure 4-14-13

•

Atlas Fractures Avulsion of anterior arch C1 ➢ Rare stable injury ➢ Results from anterior atlantoaxial ligament ➢ Horizontal cleft in anterior arch (difficult on CT) • Posterior C1 arch fracture ➢ Bilateral posterior fractures (no anterior component) ➢ No anterior soft tissue swelling; stable ➢ Distinguish from normal congenital cleft • • • • • • •

Laminar Fractures Lamina crushed on extension from above/below Pictoral representation of an extension injury Often in older patients with spondylosis Usually C5 to C7 Difficult to detect on radiographs CT optimal Mechanically stable (Intact anterior column/facets) Neurologically unstable due to cord impingement by fragments

Musculoskeletal Radiology

843

Imaging of Cervical Spine Trauma

•

Hyperextension Dislocation • • • •

Figure 4-14-14

Common in older patients with spondylosis ➢ Also bone forming diatheses AS, DISH Rupture of ALL, disc and stripping of PLL (unstable) Patients usually severe neurologic symptoms ➢ Acute central cord syndrome Spinal cord impinged by subluxation and intact posterior elements Often recoils back to relatively normal position

Hyperextension Dislocation: Radiographic Characteristic [Figure 4-14-14] • •

Relatively normal cervical alignment in quadriplegic patient Soft tissue swelling (100%) ➢ Only finding 33% • Avulsed fragment anteroinferior vertebrae (65%) ➢ Longer horizontally (unlike extension teardrop fracture) ➢ In young patients ring apophysis, no neurologic deficit • Widened disc anteriorly and vacuum (15%)

Hyperextension Fracture/Dislocation: Pedicolaminar Fracture-Separation • • • •

Combined hyperextension, compression and rotation Fractures of pillar, lamina, pedicles and spinous process opposite side of translation Vertebral body often mildly (3 – 6 mm) anteriorly displaced Spinous process not rotated

Lateral radiograph of an extension fracture subluxation at C5-6 (arrow) in a patient with ankylosing spondylitis (syndesmophytesarrowhead)

Hyperextension Fracture/Dislocation: Pedicolaminar Fracture-Separation Radiologic Characteristics • • •

Disc narrowing and vertebral rotation above injury Opposite facet may be widened/dislocated Commonly involve foramen transversarium ➢ Vertebral artery (MRA) • Important to distinguish from flexion injury • • • •

Pillar Fracture Not common, 3% - 11% of cervical injuries (C6 – C7) Hyperextension and rotation Articular mass compressed on side of rotation Stable, radiculopathy common without cord damage

• • • •

Pillar Fracture: Radiologic Characteristics [Figure 4-14-15] Subtle on radiographs Disrupted lateral cortical margin (AP view) Visualize facets on AP radiographs Loss of posterior articular mass overlap ➢ Lateral radiograph (“double outline” sign) • CT optimal – degree of fragmentation and additional other fractures ➢ Pedicle, transverse process, lamina

Figure 4-14-15

Radiograph, AP tomogram and axial CT of an articular pillar fracture with offset at the facet (solid arrows) and fracture (open arrow) seperating the articular pillar from the remaninder of the vertebrae

Imaging of Cervical Spine Trauma

844

Musculoskeletal Radiology

Hyperextension: Teardrop Fracture

Figure 4-14-16

• • • •

[Figures 4-14-16 and 4-14-17]

Often occur in older osteoporotic patients Avulsion by ALL of triangular fragment Anterioinferior vertebral body (usually C2) Fragment vertical height same or larger than length ➢ Unlike avulsion with hyperextension dislocation • Soft tissue swelling more prominent in younger patients • Unstable in extension

Traumatic Spondylolisithes: “Hangman” Fracture (“Hangee” Fracture) • •

Common 5% of all cervical spine injuries Hyperextension is probably transient modified by flexion/compression/distraction • Unstable injury • Neurologic symptoms unusual unless distraction ➢ Large canal relative to cord at C2 ➢ “Autodecompression” from bilateral posterior fractures

Pictoral representation of an extension teardrop fracture

Figure 4-14-17

Traumatic Spondylosithes: Radiologic Characteristics •

[Figures 4-14-18 and 4-14-19]

Effendi classification ➢ I: Minimally displaced fracture ➢ II: More displacement, involves C2 3 disc (widening) ➢ III: Bilateral facet dislocation • Oblique C2 pedicle fracture – lateral view • Mild anterolisthesis, posteriorly displaced spinolaminar line • Associated injuries-anterior corner fractures C2/C3 ➢ C1/high thoracic fractures (10%) ➢ Vertebral artery injuries

Figure 4-14-18

Lateral radiograph and tomogram of an extension teardrop fracture with avulsed fragment (arrow) from the attached anterior longitudinal ligament (ALL)

Figure 4-14-19 Pictoral representation of the different types of traumatic spondylolistheses

Lateral radiograph and axial CT of a type 1 traumatic spondylolisthesis showing the linear nondisplaced fracture (arrows)

Musculoskeletal Radiology

845

Imaging of Cervical Spine Trauma

Axial Compression Injury: Burst Fracture • • • •

Figure 4-14-20

Not common, 4% of cervical injuries Only occurs where cervical spine in neutral position C1 – Jefferson fracture Lower cervical burst fracture C3 - 7

•

Jefferson Fracture [Figure 4-14-20] Axial compression drives occipital condyles toward atlas • Bilateral fractures anterior/posterior ➢ Lateral displacement • Unstable, neurologic symptoms unusual ➢ Large neural canal ➢ Outward displacement of fragments

Jefferson Fracture: Radiologic Characteristics •

[Figure 4-14-21]

• • • •

Pictoral representation of a Jefferson fracture

Open mouth view best ➢ Laterally displaced lateral masses Lateral radiograph may only show soft tissue swelling; look for posterior fractures CT optimal for bilateral fractures ➢ Jefferson variants Lateral mass displacement > 7 mm / predental space > 6 mm = ruptured transverse atlantal ligament Small nondisplaced fragment medial to articular mass – intact ligament

Figure 4-14-21

•

Cervical Burst Fracture Caused by vertical force driving nucleus pulposis through endplate with body exploding from within • Mechanically stable unless posterior ligament injury • Neurologically unstable – deficit may progress ➢ Fragments change position ➢ Symptoms transient paresthesias to quadraplegia Axial CT of a Jefferson fracture with four components (arrows)

Cervical Burst Fracture: Radiologic Characteristics [Figure 4-14-22] • • • • •

Soft tissue swelling with straightening (but no kyphosis) Retropulsed fragments disrupted posterior vertebral body line Degree of vertebral body comminution variable Vertical fracture – midline/eccentric Disrupted joints of Lushka (facets)

Figure 4-14-22

Lateral radiograph, axial CT and sagittal CT of burst type fractures in different patients showing the comminuted fracture (circle), retropulsed fragments (solid arrows) and fracture at the junction of the lamina and spinous process (open arrow)

Imaging of Cervical Spine Trauma

846

Musculoskeletal Radiology

• • • •

Indeterminate Mechanism: Cervical Injuries

• • • •

Figure 4-14-23

Odontoid fractures Occipitoatlantal dissociation Torticollis Rotary atlantoaxial subluxation/dislocation

Odontoid Fracture

•

Most common of C2 fractures (41%) 11% - 13% of all cervical spine injuries Mechanism – flexion and or extension Other fractures (13%) – face, mandible, posterior arch C1, extension teardrop, hangman, atlantoaxial dissociation Anderson/D’Alonzo classification

Odontoid Fracture: Radiologic Characteristics [Figures 4-14-23 to 4-14-26] •

Prevertebral soft tissue swelling(may be only finding) Pictoral representation of different types of odontoid fractures • Type I ➢ Rare (if occurs) avulsion at tip from alar Figure 4-14-24 ligaments • Type II ➢ At base (60%-70%) ➢ May miss on CT ➢ High nonunion rate (72%), higher if displacement > 5 mm ➢ Open mouth view (simulated by Mach effect); atlantoaxial instability ➢ Os odontodeum distinguished by sclerotic margins • Type III (30%-40%) ➢ C2 body ➢ Disruption of Harris ring ➢ “Fat” C2 sign, invariably heal

Figure 4-14-25 Lateral radiograph of a type 2 odontoid fracture (arrows)

Figure 4-14-26 Open mouth radiograph of a type 3 odontoid fracture (arrows)

Coronal and sagittal CT of a type 3 odontoid fracture (arrows) Musculoskeletal Radiology

847

Imaging of Cervical Spine Trauma

• •

Cervical Spine Trauma: Role of MR Imaging Thecal sac/spinal cord impingement Disc herniation/extrusion: 20% - 40% patients ➢ Highest (100%) in patients with anterior cord syndrome • Epidural hematoma (1% - 2%); spinal cord edema/hematoma • Ligamentous disruption; cervical spondylosis • Subsequent complications ➢ Syringomyelia, myelomalacia

Figure 4-14-27 T1

T2

•

MR Imaging: Spinal Cord Injury Intramedullary swelling ➢ T1W – increased cord caliber ➢ T2W – increased signal • Intramedullary edema ➢ T2W – increased signal • Intramedullary hemorrhage ➢ Variable MR appearance (often heterogeneous) ➢ Poor prognostic sign

Sagittal T1 and T2-weighted MR images show high signal intensity in the spinal cord (arrows) that subsequently reveals low signal intensity on T2-weighting on a follow-up MR all indicative of hemorrhage as seen on the gross specimen

T2

MR Imaging: Intramedullary Hemorrhage [Figure 4-14-27] •

Oxyhemoglobin ➢ Hyperacute (minutes – hours) ➢ Intermediate signal T1W ➢ High signal T2W • Deoxyhemoglobin ➢ Usually first 24 hours ➢ Can be up to 8 days with hypoxia ➢ Intermediate signal T1W ➢ Low signal T2W • Methemoglobin ➢ Usually after 24 hours ➢ High signal T1W (begins peripherally) ➢ Low signal T2W (early subacute intracellular) ➢ High signal T2W (late subacute extracellular)

References 1. 2. 3. 4.

Blackmore CC, Mann FA, Wilson AJ.. "Helical CT in the primary trauma evaluation of the cervical spine: an evidence-based approach." Skeletal Radiol. 2000 Nov;29(11):632-9. Review. Jarolimek AM, Coffey ECC, Sandler CM, West OC. "Imaging of uppercervical spine injuries -- Part III: C2 below the dens." Applied Eadiology. 2004 July; 9-21. Murphey MD, Batnitzky S, Bramble JM. "Diagnostic imaging of spinal trauma." Radiol Clin North Am. 1989 Sep;27(5):855-72. Stabler A, Eck J, Penning R, Milz SP, Bartl R, Resnick D, Reiser M.. "Cervical spine: postmortem assessment of accident injuries--comparison of radiographic, MR imaging, anatomic, and pathologic findings." Radiology. 2001 Nov;221(2):340-6.

Imaging of Cervical Spine Trauma

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Musculoskeletal Radiology

Radiographic Differential Diagnosis of the Jaws Christopher G. Fielding, COL, DC, USA • •

Generalities

•

Dentists like plain films Act as radiologist ➢ Oral & Maxillofacial Surgery ➢ Oral & Maxillofacial Radiology

Dental Anatomy Review

•

Primary dentition (deciduous) ➢ 20 teeth ➢ Eruption starts at 6 months ➢ Completed eruption sequence 3 years Permanent dentition ➢ 32 teeth ➢ Eruption starts at 6 years ➢ Completed eruption sequence 12 years ❖ Except 3rd molars (wisdom teeth)

Primary Dentition Eruption/Exfoliation Tooth Numbering Primary Tooth Numbering Primary tooth “numbering”

Tooth Numbering •

Imaging Techniques

•

Intraoral ➢ Bitewing ➢ Periapical ➢ Occlusal Extraoral ➢ Panoramic ➢ AP, PA, lateral, oblique, Water’s, Townes ➢ CT, MRI, technetium scan

Bitewing • •

Radiographic Description • • • • • •

Size : in centimeters Border: well circumscribed, poorly circumscribed, illdefined Shape: unilocular, multilocular, uniform, irregular Number: focal, multifocal “Color”: radiolucent, radiopaque, mixed; ➢ buzzwords: “ground glass”, “cotton wool” Location: exact location within the maxilla or mandible; location in relation to adjacent structures (periapical, interradicular, pericoronal, etc) Teeth ➢ Impaction, displacement, or resorption Periodontal supporting structures ➢ Periodontal ligament space enlargement or loss of the lamina dura

Musculoskeletal Radiology

849

Tooth numbering (adult)

Radiographic Differential Diagnosis of the Jaws

•

Features “Unique” to Dental Radiographs

•

Figure 4-15-1

Dental anatomy ➢ Crown of tooth ➢ Periodontal ligament ➢ Lamina dura ❖ Artifactual thin white line around roots of teeth ➢ Root canal ➢ Apex of root ➢ Inferior alveolar canal ❖ Above - think odontogenic ❖ Below - think fibro-osseous & developmental

Overview

•

•

Radiolucent lesions ➢ Periapical ➢ Pericoronal ➢ Multilocular Radiopaque/mixed density lesions ➢ Periapical ➢ Interradicular ➢ Multifocal confluent ➢ Target lesion Osteosarcoma of the gnathic skeleton Periapical cyst/periapical granuloma

• • • • •

Radiolucent Lesions: Periapical Periapical granuloma Periapical cyst Traumatic bone “cyst” Nasopalatine duct cyst Early focal cemento-osseous dysplasia

Figure 4-15-2

Periapical Granuloma and Periapical Cyst •

[Figure 4-15-1]

• • •

•

• •

Inflammatory lesion progressing from nonvital pulp as a sequelae of caries or trauma Pain with or without swelling and drainage Radiographic ➢ Circumscribed or diffuse radiolucency which destroys the periodontal ligament space and lamina dura ➢ Usually limited in size Differential diagnosis: ➢ Immature periapical cemental dysplasia (tooth is vital) ➢ Immature cementoblastoma (vital tooth, usually mandibular molars) ➢ Traumatic bone cyst (vital teeth, intact lamina dura) Treatment ➢ Endodontic (root canal therapy) ➢ Extraction

Nasopalatine Duct Cyst [Figure 4-15-2] • • • •

Aka incisive canal cyst Nasopalatine duct cyst (must exceed 6 mm in diameter, the upper limit for the normal incisive Most common non-odontogenic cyst in the oral cavity canal) (developmental cyst) Swelling of anterior palate, pain, drainage 4th – 6th decades Well-circumscribed ULRL midline of palate, apical to central incisors Treatment ➢ Surgical enucleation ➢ Recurrence is rare

Radiographic Differential Diagnosis of the Jaws

850

Musculoskeletal Radiology

•

Fibro-osseous lesions

• • •

Figure 4-15-3

Cemento-osseous dysplasia ➢ Periapical ➢ Focal ➢ Florid Ossifying fibroma Fibrous dysplasia

Cemento-osseous Dysplasia

•

•

Focal ➢ Single site ➢ 90% occur in F ➢ Whites > blacks ➢ Posterior mandible most common site ❖ Many occur in extraction sites ➢ Most lesions smaller than 1.5 cm in diameter ➢ Well defined RL ---> mixed density ---> RO ➢ Early lesions RL ➢ Calcification with maturation of lesion

Cemento-osseous Dysplasia [Figures 4-15-3 and 4-15-4]

•

•

Periapical ➢ Anterior mandible ➢ F predilection (10:1, 14:1) ➢ 70% occur in blacks ➢ Pulps are vital ❖ Teeth are usually unrestored ➢ Asymptomatic ➢ Incidental finding RL --->mixed density --->RO ➢ Early lesions RL ➢ Calcification with maturation of lesion

Periapical cemento-osseous dysplasia (early)

Figure 4-15-4

Cemento-osseous Dysplasia [Figures 4-15-5 and 4-15-6]

•

Florid ➢ Multifocal, not limited to anterior mandible ➢ Black F ❖ Usually middle-aged ➢ Pulps are vital ➢ Asymptomatic ❖ Dull pain ❖ Occasional expansion or sinus tract ➢ Incidental finding RL --->mixed density --->RO ➢ Early lesions RL ➢ Calcification with maturation of lesion

Periapical cemento-osseous dysplasia (late/mature)

Figure 4-15-5

Figure 4-15-6

Florid cemento-osseous dysplasia

Musculoskeletal Radiology

851

Radiographic Differential Diagnosis of the Jaws

Gardner Syndrome •

Figure 4-15-7

Cemento-osseous Dysplasia

• • • •

Treatment/prognosis ➢ No treatment required ➢ Clinical & radiographic diagnosis in most cases ➢ Surgery, extraction, biopsy of sclerotic lesions ❖ Chronic osteomyelitis

Radiolucent Lesions: Pericoronal

• • •

Dentigerous cyst Ameloblastoma Ameloblastic fibroma Odontogenic keratocyst Dentigerous cyst involving an impacted mandibular 3rd molar

Dentigerous Cyst [Figure 4-15-7]

•

The most common developmental odontogenic cyst Derived from reduced enamel epithelium of the dental follicle Radiographic ➢ Well demarcated radiolucency that may be extensive ➢ Encompasses the crown of an unerupted or impacted tooth ➢ Third molars and cuspids of young adults most commonly involved Rarely may give rise to: ➢ Ameloblastoma ➢ Mucoepidermoid carcinoma ➢ Squamous cell carcinoma Treated by enucleation

• • • • • • • • •

Odontogenic keratocyst Central giant cell granuloma Ameloblastoma Cherubism Odontogenic myxoma Hemangioma/AV malformation Odontogenic myxoma Botryoid odontogenic cyst Hyperparathyroidism

• • • • •

Multilocular Aneurysmal bone cyst Cherubism Hyperparathyroidism Odontogenic fibroma

•

Figure 4-15-8

Radiolucent Lesions: Multilocular (Macho-Macho)

Multilocular RL of the Mandible

• • • • •

• • • •

Can Cross Midline Myxoma (odontogenic) Ameloblastoma Central giant cell lesion Hemangioma/A-V malformation Odontogenic keratocyst

Odontogenic Keratocyst [Figure 4-15-8] The most common multilocular radiolucency Usually presents in adults Up to 3/4 in posterior mandible and ramus Radiographic ➢ Well-defined unilocular, or more commonly multilocular radiolucency with sclerotic borders ➢ Usually grows in a relatively linear direction

Radiographic Differential Diagnosis of the Jaws

852

Odontogenic keratocyst involving the posterior mandible and ramus

Musculoskeletal Radiology

•

• • • • •

❖ Obvious expansion may NOT be seen, even with large cysts ➢ Often associated with an impacted tooth May grow aggressively with: ➢ Cortical perforation ➢ Tooth displacement and/or resorption ➢ Soft tissue extension

Odontogenic Keratocyst Treated by thorough enucleation and curettage Occasionally, en bloc resection required Up to 1/3 will recur Long term radiographic follow-up requiredfollow-up quiredfo If multiple, evaluate for nevoid basal cell carcinoma syndrome (Gorlin syndrome)

Figure 4-15-9

Nevoid Basal Cell Carcinoma Syndrome (Basal Cell Nevus Bifid Rib Syndrome; Gorlin Syndrome) [Figure 4-15-9] • • •

Teenagers, both male and female Autosomal dominant disease Symptom complex characterized by: ➢ Numerous basal carcinomas of the skin ➢ One or more bifid ribs ➢ Multiple odontogenic keratocysts ❖ The keratocysts are identical to the solitary ones ❖ Recurrence of OKC’s is the rule in this syndrome

Multiple odontogenic keratocysts associated with nevoid basal cell carcinoma syndrome (Gorlin Syndrome)

Bifid Rib – Basal Cell Carcinoma Syndrome (Nevoid Basal Cell Carcinoma Syndrome; Gorlin’s Syndrome) •

• • • •

Other findings include: ➢ Palmer/planter pitting pitting ➢ Frontal bossing ➢ Calcified falx cerebri ➢ Tendency to develop medulloblastomas ➢ Many others… Long-term periodic radiographic follow-up is needed

Figure 4-15-10

Central Giant Cell Granuloma [Figure 4-15-10]

•

Aggressive reactive process more often in mandible Young adults with female predilection Radiographic ➢ Unilocular, or more commonly multilocular radiolucency with well demarcated margins ➢ The most common anteriorly located multilocular lucency ❖ May cross the midline ➢ May cause expansion of the involved bone with thinning of the cortex ➢ Root divergence and smooth, concave root resorption is common

Central giant cell granuloma

Central Giant Cell Granuloma

•

•

Histology ➢ Highly cellular fibrovascular stroma with… ❖ Dispersed multinucleated giant cells ❖ Osteoid or osseous trabeculae ❖ Extravasated blood and hemosiderin ➢ Brown tumor of hyperparathyroidism is histologically indistinguishable ❖ Serum calcium determination required Surgical enucleation with extraction(s) if necessary ➢ May also try hormonal therapy with calcitonin May recur if incompletely removed

Musculoskeletal Radiology

853

Radiographic Differential Diagnosis of the Jaws

• • • •

Ameloblastoma [Figure 4-15-11]

• •

•

Figure 4-15-11

Most common odontogenic neoplasm Average age is 34 with no sex predilection Presents as a painless expansile lesion Most common location is posterior mandible and ramus Maxillary lesions may extend to nasal cavity and base of skull Radiographic ➢ Classically a “soap-bubble” multilocular radiolucency with clearly demarcated borders ➢ Unilocular (“unicystic”) ameloblastomas possible ➢ Most are unassociated with impacted teeth ➢ May displace and/or resorb teeth ➢ Centrifugal growth ❖ May reach gigantic proportions

Ameloblastoma

•

Treatment: ➢ Curettage or en bloc resection for mandibular lesions ➢ Resection for maxillary lesions ➢ Recurrence rate is high ➢ Unilocular ameloblastomas have a better prognosis with rare recurrence and requiring only simple enucleation ➢ Rare malignant transformation has been reported

Cherubism [Figure 4-15-12] • • • •

• • • • • • •

Rare developmental jaw condition that is generally inherited as an AD trait Usually occurs between ages 2-5 YO Bilateral involvement of the posterior mandible developing characteristic “cherub-like facies” ➢ Maxillary involvement can occur Expansile multilocular radiolucency Ameloblastoma ➢ May cause tooth displacement, eruption failure, impair mastication, speech difficulties Treatment: ➢ Prognosis in any given case is unpredictable. Most cases demonstrate varying degrees of remission and involution after puberty. ➢ Radiotherapy contraindicated

Radiopaque and Mixed Lesions: Periapical Periapical cemento-osseous dysplasia Cementoblastoma Erupting teeth Endodontic procedures Hypercementosis Idiopatic osteosclerosis Focal scerosing osteomyelitis

Figure 4-15-12

Cherubism (bilateral multilocular radiolucencies)

Radiographic Differential Diagnosis of the Jaws

854

Musculoskeletal Radiology

•

Cementoblastoma [Figure 4-15-13]

• • • •

•

•

Figure 4-15-13

Benign cemento-osseous tumor, most commonly associated with a root of a mandibular molar ➢ Usually 1st molar ➢ Some authorities consider this a simple variant of osteoblastoma M=F; < 25y of age Slow growth with possible expansion Sometimes painful Radiographic ➢ Calcified, highly radiodense mass intimately associated with the root ➢ Root outlines obscured ➢ Usually surrounded by radiolucent rim Tx/Prognosis ➢ Excision or root amputation/endodontics ➢ May continue growing if incompletely excised, otherwise no recurrence

Cementoblastoma

Idiopathic Osteosclerosis [Figure 4-15-14] • •

• •

• • •

Focal area of increased radiodensity of unknown cause ➢ Vital pulp M=F; arise 1st – 2nd decade ➢ May remain static or slowly increase in size ➢ Once skeletal growth stops, the lesions become static 90% in mandible, usually 1st molar region ➢ Usually involving a root apex Multifocal in some cases XR ➢ Well-defined, rounded or elliptic radiodense mass ➢ No RL rim ➢ 3 mm – 2 cm

Figure 4-15-14

Idiopathic osteosclerosis

Focal Sclerosing Osteomyelitis [Figure 4-15-15]

•

•

Aka condensing osteitis Localized increased radiodensity Apex ➢ Widened PDL space or periapical RL Molar-premolar areas of mandible commonly

Figure 4-15-15

Condensing Osteitis

•

Tx/prognosis ➢ Endodontic/ext ➢ 85% regress or resolve ➢ Bone scar ❖ Residual lesion

Radiopaque and Mixed Lesions: Interradicular • • • •

Ossifying fibroma ➢ Active ossifying fibroma Focal cemento-osseous dysplasia Osteoblastoma Adenomatoid odontogenic tumor Odontoma

Musculoskeletal Radiology

855

Focal sclerosing osteomyelitis (condensing osteitis)

Radiographic Differential Diagnosis of the Jaws

•

Ossifying Fibroma [Figure 4-15-16] • • •

•

Figure 4-15-16

Only true neoplasm in the “benign fibro-osseous lesion” category Seen more often in teenagers and young adults No sex predilection Radiographic ➢ Well defined mixed lesion with sclerotic borders ➢ Density will vary with the maturity of the lesion ➢ Tooth bearing and non-tooth bearing areas, especially posterior mandible ➢ Growth is radial instead of linear (as in fibrous dysplasia) ❖ Expansion buccally and lingually will thin cortex ❖ Bowing of inferior border is characteristic ❖ May expand alveolar crest Treatment ➢ Thorough excision of mass ➢ Mass tends to “shell out” easily from the surrounding bone ➢ Up to 1/3 may recur

Radiopaque and Mixed Lesions: Multifocal Confluent • • • • •

Florid cemento-osseous dysplasia Paget’s disease of bone Chronic sclerosing osteomyelitis Gardner syndrome Multiple tori and exostoses

• • •

Fibrous dysplasia Hyperparathyroidism Osteopetrosis

Radiopaque and Mixed Lesions: Ground Glass

• •

Fibrous Dysplasia

• • • •

Occurs in children, teenagers and young adults of both sexes Four main forms: monostotic, polyostotic, McCune-Albrights, and craniofacial ➢ Craniofacial FD may involve multiple contiguous bones in the midface and cranium ➢ McCune-Albright’s disease includes polyostotic FD, focal skin hyperpigmentation (“café-au-lait spots”) and endocrine disturbances (usually precocious puberty and/or hyperthyroidism) Painless expansile process of osteoprogenitor tissue Slow growth with facial deformity More common in the maxilla

Ossifying fibroma

Figure 4-15-17

Fibrous dysplasia (“ground glass” appearance)

Fibrous Dysplasia [Figure 4-15-17]

• • • •

Radiographic ➢ Mature maxillary lesions are homogeneous, “ground glass” or “peau d'orange” ➢ No margination or borders and blends with adjacent trabecular bone ➢ May obliterate the maxillary sinus ➢ Mandibular lesions more likely mottled or multicystic ➢ Skeletal survey to rule out polyostotic disease ➢ Use plain films or CT as MRI does not demonstrate the traditional radiographic findings Defer surgical treatment (cosmetic recontouring) until skeletal maturity Growth may cease, continue, or resume after periods of quiescence Quarterly follow-up Small chance of sarcomatous transformation, usually osteosarcoma or fibrosarcoma ➢ Especially in patient’s with a history of radiotherapy

Radiographic Differential Diagnosis of the Jaws

856

Musculoskeletal Radiology

• • • • • •

Radiopaque and Mixed Lesions: Target Lesion, Dense

• • • • •

Figure 4-15-18

Odontoma Osteoma Focal cemento-osseous dysplasia Ameloblastic fibro-odontoma Ossifying fibroma Osteoblastoma

Odontoma [Figure 4-15-18]

• • • • •

Equal sex predilection More common in teenagers and young adults A mixed odontogenic tumor / hamartoma Asymptomatic but may prevent tooth eruption Types: ➢ Compound ❖ Target lesion with central tooth-like structures ❖ More common in anterior jaws between teeth ➢ Complex ❖ Well demarcated opacity with frayed margins ❖ May have a radiolucent rim ❖ More common in posterior jaws (pericoronal) Treated by enucleation Does not recur

Compound odontoma

Osteosarcoma [Figure 4-15-19]

• •

•

•

Malignancy of mesenchymal cells M>F Extragnathic ➢ Bimodal (2nd – 3rd decades, 6th decade) ❖ Distal femur/proximal tibia ❖ Axial skeleton/flat bones Gnathic ➢ 3rd – 4th decades ➢ Maxilla = mandible XR ➢ RL, mixed, RO ➢ Ill-defined periphery ➢ “Sunburst” or “sunray” ❖ 25% of gnathic lesions ➢ “Spiking” root resorption ➢ Widening of PDL space Treatment/prognosis ➢ Gnathic lesions low-grade?? ➢ Radical surgical extension ❖ Best hope for cure ➢ Preoperative chemotherapy ➢ Local uncontrolled dz ❖ Leading cause of death ❖ Usually within 2 years of initial tx Risk factors ➢ Paget’s disease ➢ H/O radiation

Musculoskeletal Radiology

Figure 4-15-19

Osteosarcoma (widening of the periodontal ligament space)

857

Radiographic Differential Diagnosis of the Jaws

MRI of the Knee: Part 1 Mark Anderson, MD •

Lecture Outline • •

• • • •

Part 1 ➢ Technique ➢ Menisci Articular cartilage Part 2 ➢ Bones ➢ Stabilizers ➢ Miscellaneous

Technique

•

Surface coil High resolution T1, T2, fat suppression Sagittal, coronal, axial planes

Technique: Pulse Sequences

• •

•

•

•

T1 ➢ Overall anatomy ➢ Menisci ➢ Bones (marrow) ➢ Fat/hemorrhage ➢ Muscle T2 ➢ Fluid / edema ➢ Tendons, ligaments ➢ Soft tissue injury Fast Spin Echo-T2 ➢ T2 contrast – faster acquisition ➢ Caution: ❖ Bright fat (marrow pathology) ❖ Blurring effect – proton density (meniscal tears) Gradient Echo (T2*) ➢ Menisci ➢ Articular cartilage (3D) ➢ Susceptibility effects ➢ Caution: Marrow Pathology STIR (Fat suppressed T2) ➢ Marrow pathology ➢ Soft tissue injury ➢ Articular cartilage

Figure 4-16-1

Cadaveric specimen of the menisci

Figure 4-16-2

Summary: Pulse Sequences • •

•

Menisci ➢ Short TE (T1, PD, GRE) - caution with FSE Bone Marrow ➢ Fat saturation (STIR, Fat Sat FSE T2) - not GRE ➢ T1W in one plane Other soft tissues (ligaments, tendons) ➢ T2W with fat saturation (STIR, Fat Sat FSE T2) Cartilage ➢ Contrast between fluid and cartilage Schematic diagram of a meniscus cut in cross-section (upper); normal sagittal image of posterior horn of the medial mensicus (lower); normal meniscus at arthroscopy (right)

MRI of the Knee: Part 1

858

Musculoskeletal Radiology

•

Technique: Imaging Planes

•

•

• • •

Figure 4-16-3

Sagittal ➢ Menisci ➢ Cruciate ligaments ➢ Extensor tendons ➢ Articular cartilage ➢ Bones Coronal ➢ Collateral ligaments ➢ Menisci ➢ Articular cartilage ➢ Bones Axial ➢ Patellofemoral joint ➢ Muscles / tendons ➢ Popliteal fossa

Sagittal gradient echo images (corresponding to lines on diagram) through the medial meniscus

Figure 4-16-4

Menisci [Figures 4-16-1 and 4-16-2] Fibrocartilage Medial/lateral Functions: ➢ Joint congruity ➢ Shock absorption ➢ Load transmission

• • • •

Larger “C” Posterior horn > anterior horn Attached more tightly to the capsule Covers 1/2 contact surface of tibial plateau

• • • • •

Tighter “C” Posterior horn = anterior horn Attached more loosely to the capsule Covers ¾ contact surface of tibial plateau Popliteus tendon (fascicles)

Sagittal gradient echo images (corresponding to lines on diagram) through the lateral meniscus

Medial Meniscus [Figure 4-16-3]

Lateral Meniscus

[Figure 4-16-4]

Figure 4-16-5

Menisci: Attachments • • •

[Figures 4-16-5 and 4-16-6]

Tibia Capsule Ligaments ➢ Transverse ➢ Meniscofemoral ❖ Humphrey ❖ Wrisberg ➢ Oblique meniscomeniscal

Figure 4-16-6 Normal transverse intermeniscal ligament

Diagram of the knee (posterior view) demonstrating the meniscofemoral ligament of Wrisberg (arrow). Musculoskeletal Radiology

859

MRI of the Knee: Part 1

Menisci: Variants •

Figure 4-16-7

[Figures 4-16-7]

•

•

•

Discoid ➢ Enlarged meniscus ➢ Embryologic, congenital? ➢ Lateral > medial ➢ Prone to tear ➢ Complete / incomplete ➢ Wrisberg variant Buckled ➢ “Lax”, “Flounce” ➢ Medial meniscus ➢ Ligament injury/laxity ➢ Positional Ossicle ➢ Vestigial, post-traumatic? ➢ May be symptomatic ➢ PHMM most common ➢ Variable MRI signal intensity

A. Diagram illustrating a discoid lateral meniscus (L) from above. B. Normal sized medial meniscus (thin arrow) and enlarged, discoid lateral meniscus (large arrow)

Figure 4-16-8

Meniscus

• •

Microstructure ➢ Collagen bundles ❖ Circumferential ❖ Transverse (“tie fibers”) ➢ Resist longitudinal loading ❖ “hoop stresses”

Menisci: Pathology •

•

Degeneration Tear ➢ traumatic vs. degenerative 20% asymptomatic pts > 50 y.o. show MR evidence of tear

Surgical Considerations

• •

Primary goal ➢ Preserve as much meniscal tissue as possible

A. Diagram of a meniscus cut in cross-section revealing an oblique undersurface tear. B. Similar tear in the posterior horn of the medial meniscus on sagittal image

Meniscal Tear: MRI

•

Abnormal Signal intensity -Morphology

Meniscal Tear: MRI [Figure 4-16-8]

•

Signal intensity (Grades) ➢ 1 – Globular ➢ 2 – Linear ➢ 3 – Contact with articular surface ==> tear

Meniscal Tear: “Close” • • •

Kaplan ➢ 13/20 (65%) no tear DeSmet ➢ 1 image only… 30%–55% ➢ > 1 image….... 90% Don’t overcall… be descriptive

Meniscal Tear: MRI Morphology ➢ Blunted, truncated ➢ Size

MRI of the Knee: Part 1

860

Musculoskeletal Radiology

•

Meniscal Tears [Figure 4-16-9]

•

•

Figure 4-16-9

Vertical ➢ Radial ➢ Longitudinal ➢ Traumatic ➢ Divides into ant/post or med / lat fragments Horizontal ➢ Degenerative ➢ Divides into sup / inf fragments

Meniscal Tears [Figures 4-16-10 and 4-16-11] Radial ➢ Perpendicular to axis ➢ Vertical ➢ Traumatic or degenerative ➢ Meniscal subluxation ➢ Irreparable?

Figure 4-16-10

Diagram of radial tear

Figure 4-16-11

Radial tear involving the body of the lateral meniscus (arrow)

A. Radial tear. B. Longitudinal tear. C. Horizontal tear.

Figure 4-16-13

Figure 4-16-12 •

Meniscal Tears [Figure 4-16-12] Longitudinal ➢ Vertical ➢ Along axis of meniscus ➢ Bucket handle ❖ displaced fragment ❖ medial meniscus ❖ locking ➢ Peripheral ❖ potentially reparable ❖ outer 1/3 (red zone) Longitudinal tear

• •

Peripheral Tears [Figure 4-16-13] •

Outer 1/3 of meniscus Vascular region ➢ (red/red zone) Tend to heal ➢ primary repair ➢ conservative therapy

Musculoskeletal Radiology

A. Diagram of a peripheral tear. B. Peripheral tear involving the posterior horn of the medial meniscus (arrow).

861

MRI of the Knee: Part 1

• • •

Meniscocapsular Separation

•

Figure 4-16-14

Figure 4-16-15

PHMM Fluid at meniscocaps interface Poor sensitivity/PPV

Meniscal Tears [Figure 4-16-14]

•

“Parrot Beak” Tear ➢ Combination ❖ Radial + longitudinal ❖ Oblique to long axis ➢ Meniscal Flap Parrott beak tearParrott beak tear

Meniscal Tears [Figure 4-16-15] •

Horizontal ➢ Often degenerative ➢ May be asymptomatic Meniscal cysts

• • •

Parameniscal/intrameniscal Lateral > medial Horizontal tear

• • • •

Bucket Handle (medial) Flipped (lateral) Gutter Extruded

Meniscal cyst [Figure 4-16-16] A. Diagram of a horizontal tear. B. Horizontal tear of the body of the meniscus (white arrow) and an associated parameniscal cyst (black arrow)

Displaced Meniscal Tears [Figures 4-16-17 to 4-16-21]

Figure 4-16-16

Figure 4-16-17

Bucket Handle Tear •

[Figures 4-16-17 to 4-16-19]

•

Coronal Sensitivity ➢ Displaced fragment ➢ Blunted body Sagittal ➢ Too few “bowties” ➢ Double PCL

(94%) (64%) (97%) (30%)

Helms AJR 1998

Diagram of a bucket handle tear of the meniscus

Figure 4-16-18

Figure 4-16-19

Sagittal image corresponding to the dashed line shows the large bucket handle fragment within the notch creating the “double PCL” sign (P = PCL)

Coronal image corresponding to the dashed line demonstrates the displaced meniscal fragment of this bucket handle tear (large arrow) and irregular, truncated body of the meniscus (thin arrow)

MRI of the Knee: Part 1

Horizontal tear with associated parameniscal cyst

862

Musculoskeletal Radiology

Figure 4-16-20

Figure 4-16-21

A. Diagram of a “flipped” (longitudinal) meniscal tear. B. Sagittal image at the level of the dashed line shows the flipped fragment (short arrow) adjacent to the anterior horn (long arrow) creating the “double anterior horn” sign. Note also the small residual posterior horn (circle).

• • •

Menisci: Post-surgical

•

Truncated, absent, “normal” Healed tear can look like new tear MR arthography ➢ 0.2 cc Gd + 20 cc saline ➢ T1W with fat-saturation ➢ Gd extending into tear

Menisci: Post-surgical

• •

White et al. Radiology, February 2002 ➢ 364 patients ➢ Conventional MR ➢ Indirect MR arthrography ➢ Direct MR arthrography Direct slightly more accurate, but no significant difference

A. Coronal image demonstrating a displaced meniscal fragment along the medial joint line (arrow). B. Diagram illustrating the horizontal tear and flipped fragment

Menisci: Post-surgical

•

Signs of new or recurrent tear? ➢ Fluid in tear ➢ Displaced fragment ➢ Tear in new area

Meniscal Tear: MRI

•

•

Accuracy > 90% ➢ 50% - sagittal only ➢ 3% - coronals only ➢ FSE ~ 80% (?) Problem areas: ➢ Free edge ➢ PHLM

Meniscal Tear: Pitfalls

•

Anatomy ➢ Transverse ligament ➢ Meniscofemoral ligaments ➢ Oblique meniscomeniscal lig ➢ Lateral inf geniculate artery ➢ Popliteus tendon ➢ Edge artifact

Meniscal Tear: Pitfalls Artifacts ➢ Patient motion ➢ Phase artifact (artery) ➢ Magic angle ➢ Gas/hemosiderin ➢ Chondrocalcinosis

Musculoskeletal Radiology

863

MRI of the Knee: Part 1

•

Take Home Points • • •

Meniscal tear? ➢ Signal intensity and morphology Small meniscus? ➢ Find the missing fragment Healed tear? ➢ Can look just like a new tear True pathology or pitfall?

To most easily identify pathology, know the normal anatomy

References 1.

Helms CA, Laorr A, Cannon WD, Jr. The absent bow tie sign in bucket-handle tears of the menisci in the knee. AJR Am J Roentgenol 1998; 170:57-61.

MRI of the Knee: Part 1

864

Musculoskeletal Radiology

MRI of the Knee: Part 2 Mark Anderson, MD •

Lecture Outline

•

• •

Figure 4-17-1

Part 1 ➢ Technique ➢ Menisci ➢ Articular Cartilage Part 2 ➢ Bones ➢ Stabilizers ➢ Miscellaneous

Bones

• • • •

Femur, tibia, patella, fibula Cortical ➢ compact ➢ subchondral plate Cancellous ➢ trabecular

Spectrum of acute osseous injuries

Figure 4-17-2

Trabecular Bone

•

10 x load-bearing capacity of cortical bone Dissipates forces Support for subchondral plate

Bones: Injuries • • •

Acute ➢ Impaction (contusion, occult fracture) ➢ Avulsion Chronic ➢ Fatigue, insufficiency fracture Spontaneous osteonecrosis Osteochondritis dissecans

Acute Impaction Injuries: Spectrum [Figure 4-17-1] • • • •

Focal contusion involving the posterolateral tibial plateau

Acute Trauma: Impaction

•

Contusion, bone bruise Edema, hemorrhage Trabecular fx Detection ➢ Explains symptoms ➢ Avoids unnecess arthroscopy ➢ Mechanism of injury ➢ May change Rx plan

Acute Trauma: Contusion [Figure 4-17-2] • •

MRI ➢ Reticular ➢ Ill-defined margins − T1, + STIR 100% heal; 2–4 months

Musculoskeletal Radiology

865

MRI of the Knee: Part 2

• •

Geographic Contusion [Figure 4-17-3]

• • •

Figure 4-17-3

Subchondral Cartilage damage ➢ softening, fissuring, ➢ chondral fx − proteoglycans* Long term sequelae? Protect during healing

*Johnson, AJSM, 1998

Focal subchondral contusion along the posterior weight-bearing portion of the lateral femoral condyle

• • •

Figure 4-17-4

Contusion Patterns: ACL Tear [Figure 4-17-4]

•

• •

Lat. femoral condyle (sulcus terminalis) Post-lateral tibial plateau Specific ➢ 95% (adults) ➢ 75% (children) “Contrecoup” ➢ Post-medial tibial plateau ➢ Peripheral injury

Patterns: Patellar Dislocation [Figure 4-17-5]

• • •

Lateral dislocation Contusions ➢ Lat. femoral condyle ❖ Anterior / non-wgt bearing ➢ Medial patella Medial retinacular injury Cartilage injury Avulsion fracture

“ACL” contusion pattern (lateral femoral condyle and posterolateral tibial plateau)

Figure 4-17-5 •

Acute Trauma: Fracture [Figure 4-17-6] Linear ➢ T1 ❖ – signal intensity ➢ STIR ❖ + or – signal intensity

Figure 4-17-6

Patellar dislocation contusion pattern (lateral margin of lateral femoral condyle and medial patella) Occult fracture of the lateral tibial plateau on sagittal T1weighted and coronal fat-saturated T2-weighted images

MRI of the Knee: Part 2

866

Musculoskeletal Radiology

• • •

Knee Stabilizers

•

Central: ACL, PCL Medial: MCL Lateral: ➢ Iliotibial band ➢ Fibular collateral lig. ➢ Biceps femoris tendon Anterior: Patellar Retinacula

• • • •

Intracapsular, extrasynovial Intercondylar notch Anterior (lateral) Posterior (medial)

Figure 4-17-7

Cruciate Ligaments [Figure 4-17-7]

• • •

ACL [Figure 4-17-8]

•

LFC --> anterior tibia Restricts ant displacement of tibia MRI ➢ low SI ➢ usually striated ➢ taut

Diagram of the knee (frontal view) demonstrating the anterior (A) and posterior (P) cruciate ligaments

Figure 4-17-8

ACL Tear •

• • •

75% of all ligament injuries ➢ Twisting + valgus force ➢ Hyperextension Associated injuries ➢ Meniscal tear (40%–70%) ❖ O’Donoghue’s Triad ❖ ACL, MCL, medial meniscus (?) (Lateral / Medial about equal) 70% mid substance; 20% proximal; 10% distal

ACL Tear: MRI [Figure 4-17-9]

•

MRI – 90%–95% accurate Primary Signs ➢ Edematous mass (48%) ➢ Non-visualization (“empty notch”) (18%) ➢ Disruption (11%) ➢ Irregular, wavy, horizontal contour ➢ Focal + SI

Normal anterior cruciate ligament

Figure 4-17-9

ACL Tear: MRI

• • • • •

Secondary Signs ➢ Bone contusions ➢ Deep notch LFC ➢ Segond fracture ❖ 10% ACL tears fx ❖ 75–100% fx - ACL tear ➢ “Anterior drawer” ❖ (uncovered PHLM)

Chronic ACL Tear: MRI Non-visualization Focal angulation Fragments “Normal” (scar) Without Edema

Musculoskeletal Radiology

Complete ACL rupture

867

MRI of the Knee: Part 2

•

Post-Op ACL [Figure 4-17-10]

• • •

Figure 4-17-10

Graft ➢ Integrity ➢ Signal Intensity ❖ Variable, especially early ➢ Roof Impingement ➢ “Cyclops” lesion ❖ Anterior arthrofibrosis

PCL [Figure 4-17-11]

•

MFC -> posterior tibia Restricts post tibial displacement MRI ➢ Low signal intensity ➢ Arched

Left: Normal ACL graft (dashed arrow). Right: Cyclops lesion (arthrofibrosis) along ventral margin of ACL graft (arrow).

Figure 4-17-11

PCL Tear [Figure 4-17-12] • • •

Force -> ant tibia – flexed knee ➢ “Dashboard” injury Complete Tear (45%) ➢ Midsubstance Partial Tear (47%) Avulsion (8%)

Medial Collateral Ligament • • •

[Figure 4-17-13]

Superficial component Bursa Deep component ➢ Meniscofemoral ➢ Meniscotibial Normal PCL

MCL Injuries: MRI [Figure 4-17-14] Grade Clinical 1 Sprain

2 3

MRI Thickened Irregular Adjacent edema Partial Tear Focal SI Complete Tear Discontinuity

Figure 4-17-12

Figure 4-17-13

Full thickness PCL tear (midsubstance)

Normal MCL

MRI of the Knee: Part 2

868

Musculoskeletal Radiology

• •

Lateral Stabilizers [Figure 4-17-15] •

• •

Figure 4-17-14

Biceps femoris tendon Fibular (lateral) ➢ collateral ligament Iliotibial tract

Posterolateral Corner Injuries

•

• • •

Hyperextension with varus force Isolated injuries rare ➢ often with cruciate injuries ➢ PCL most common Urgent exploration indicated ➢ within 3 days – 3 weeks ➢ reconstruct with cruciate(s)

ITB Friction Syndrome

•

Lateral pain Abnormal contact ITB/LFC Bursa develops ➢ Fluid collection/edema ➢ Lateral recess?

Tendons

• •

•

•

Medial ➢ Semimembranosus ➢ Sartorius ➢ Gracilis ➢ Semitendinosus Lateral ➢ Biceps femoris ➢ Iliotibial tract Posterior ➢ Gastrocnemius (med/lat) Anterior ➢ Quadriceps tendon ➢ Patellar tendon ➢ Patellar retinacula

Extensor Mechanism

• •

Partial tear of proximal MCL (arrow)

Figure 4-17-15

Diagram of lateral stabilizers (B = Biceps femoris tendon; F = Fibular collateral ligament; I = Iliotibial tract).

[Figure 4-17-16]

Quadriceps tendon ➢ Striated ❖ Vastus lateralis ❖ Vastus medialis ❖ Intermedius ❖ Rectus femoris Patellar tendon ➢ Magic angle

Figure 4-17-16

Extensor Tendons: Injuries Tears ➢ Trauma ➢ Degeneration ❖ Renal disease, steroids ❖ RA, SLE ➢ Partial vs. complete

Extensor mechanism (quadriceps and patellar tendons)

Musculoskeletal Radiology

869

MRI of the Knee: Part 2

• • • •

Patellar Tendinitis [Figure 4-17-17]

•

Figure 4-17-17

“Jumpers Knee” Enlarged (proximal) + intrasubstance SI Spectrum ➢ partial --> complete tears

Patellofemoral Joint Patellar subluxation ➢ Lateral ➢ Hypoplastic intercondylar notch

• • •

Anterior pain Patellar tilt / subluxation Impingement of infrapatellar fat

• • •

Recesses Bursae Ganglia

• • • • •

Suprapatellar “bursa” Infrapatellar cleft Popliteus hiatus Gastrocnemius/ Semimembranosus Posterior recesses

Patellofemoral Syndrome

“Cystic” Structures

Patellar tendinits with thickening and high grade partial tearing in its proximal fibers

Normal Recesses

• •

Bursae • • • •

• •

Figure 4-17-18

[Figure 4-17-18]

Prepatellar Infrapatellar ➢ superficial/deep Semimembranosus Pes anserine Tibial collateral LCL-Biceps Femoris

Other Cystic Masses [Figure 4-17-19]

•

Meniscal cysts Ganglia ➢ Intraarticular (cruciates) ➢ Extraarticular (infrapatellar fat) ➢ Intraosseous (cruciate insertions) Vascular masses

A. Prepatellar bursitis. B. Semimembranosus bursitis.

Figure 4-17-19

Popliteal artery aneurysm (A)

MRI of the Knee: Part 2

870

Musculoskeletal Radiology

• • • •

Cystic Adventitial Disease

• • • •

Figure 4-17-20

Cystic degeneration vessel wall Popliteal artery common Sudden onset claudication MRI findings ➢ intramural cysts ➢ along long axis of vessel ➢ extrinsic compression ➢ MR angiography

Synovial Plica Embryologic remnants Infrapatellar Suprapatellar Medial

Loose Bodies • • • •

Intercondylar notch Baker’s cyst Popliteus sheath GRE (T2*)

• • • • • •

Bone contusions? ACL torn? ACL graft? MCL? Lateral ligaments? “Cyst”?

[Figure 4-17-20] Loose body in posterior joint recess (arrow)

Take Home Points Look for pattern Taut...primary/secondary signs Taut...roof impingement...cyclops Deep and superficial fibers Biceps, LCL, Iliotibial Band Recess, bursa, ganglion, meniscal cyst

References 1.

Johnson DL, Urban WP, Jr., Caborn DN, Vanarthos WJ, Carlson CS. Articular cartilage changes seen with magnetic resonance imaging-detected bone bruises associated with acute anterior cruciate ligament rupture. Am J Sports Med 1998; 26:409-414.

Musculoskeletal Radiology

871

MRI of the Knee: Part 2

MRI of the Wrist Mark Anderson, MD • • •

Figure 4-18-1

Technique: Positioning Supine (arm at side) Prone (arm extended overhead) Surface Coil ➢ Thickness 1–3 mm ➢ Matrix 256–512 ➢ FOV 10 cm

• • • •

T1 T2* STIR Gd

• • •

Bones Intrinsic ligaments. TFCC

Technique: Pulse Sequences Anatomic overview Ligaments, tendons Marrow, fluid Cyst/solid, infxn Synovitis “screen”

Os styloideum

Anatomy: Coronal

• •

Anatomy: Axial

• •

Tendons Three levels ➢ Distal radioulnar joint ➢ Pisotriquetral joint ➢ Hamate Median nerve (Carpal tunnel) Ulnar nerve (Guyon’s canal)

• • •

Carpal alignment Pisotriquetral joint Triangular fibrocartilage

• • • • • •

Bones Intrinsic ligaments (SL, LTL) TFCC Tendons Nerves Masses

Anatomy: Sagittal

Anatomy / Pathology

• Signal intensity Alignment ➢ sagittal alignment ➢ ulnar variance

Bones: Normal

• • •

Os Styloideum [Figure 4-18-1] Normal variant Base of 2nd/3rd metacarpals +/- Pain ➢ Bursitis ➢ Ganglion ➢ Trauma

MRI of the Wrist

872

Musculoskeletal Radiology

•

Occult Osseous Injuries [Figure 4-18-2] •

•

Figure 4-18-2

Contusion ➢ Bone marrow edema Fracture ➢ Edema + fx line

Occult Fractures [Figure 4-18-3] • •

Trauma Screening Protocol

Scaphoid Fracture [Figure 4-18-4] 16% not detected initially Complications ➢ AVN ➢ Nonunion

• • • •

Normal T1 = Normal − T1 − T2 = Necrotic − T1 + T2 = Ischemia vs. traumatic edema Contrast enhancement?

• • • •

Kienbock’s Disease Repetitive trauma, fracture, ulna (-) variance End arteries Central position

Scaphoid AVN

Occult fracture of triquetrum

Figure 4-18-3

[Figure 4-18-5]

AVN Lunate

• •

Mid scaphoid fracture Left: Coronal T1 - Right: Coronal STIR

Figure 4-18-4

AVN Lunate: MRI [Figure 4-18-6]

• •

More than 50% of lunate Abnormal marrow signal ➢ − T1 − T2 = Diagnostic ➢ − T1 + T2 = Earlier stage

Ulnolunate Impaction [Figure 4-18-7 •

Ulna plus variance Degenerative changes ➢ especially lunate TFC tears

Scaphoid fracture

Figure 4-18-5

Figure 4-18-7

Scaphoid fracture with ischemic changes in proximal pole

Figure 4-18-6

Avascular necrosis of the lunate (Kienbock’s disease).

Ulnolunate impaction syndrome

Musculoskeletal Radiology

873

MRI of the Wrist

•

Intrinsic Ligaments [Figure 4-18-8]

• •

•

Figure 4-18-8

Scapholunate ➢ Volar Trapezoidal ➢ Middle Triangular ➢ Dorsal Band-like Lunotriquetral ➢ Smaller (2mm) Other ➢ Distal carpal row ➢ Incomplete

Intrinsic Ligaments Pitfalls ➢ Intermediate signal ➢ Attach to bone or articular cartilage

• • • • •

Absent Distorted / Elongated Widened joint Discontinuous Fluid signal on T2

•

Scaphoid <-> Lunate <-> Triquetrum

Ligament Pathology [Figure 4-18-9] Normal scapholunate ligament

Figure 4-18-9

Carpal Stability •

Scapholunate Dissociation: DISI [Figure 4-18-10]

• • • • • •

Tear or stretching of SLL ➢ dorsal fibers ➢ scaphoid palmar flexes ❖ “signet ring” deformity ➢ lunate dorsiflexes ❖ S-L angle > 600• Scaphoid fracture DISI deformity ➢ can result from scaphoid fx

Small scapholunate ligament perforation

Figure 4-18-10

Scapholunate Instability: SLAC Wrist [Figure 4-18-11] Scapho Lunate Advanced Collapse Trauma, RA, CPPD

Figure 4-18-11

DISI deformity SLAC wrist

MRI of the Wrist

874

Musculoskeletal Radiology

• • • •

LTL tear Associated with TFC tears VISI deformity Difficult diagnosis

• • • • •

Triangular fibrocartilage Radioulnar ligaments Meniscus homologue UCL and ulnocarpal ligaments ECU tendon sheath

Lunotriquetral Instability [Figure 4-18-12]

Figure 4-18-12

Triangular Fibrocartilage Complex

• • • • •

TFC: Normal Anatomy [Figure 4-18-13]

•

Fibrocartilage “Bow tie” Ulnar styloid → dist radius Attaches to radial cartilage Central portion / periphery ➢ Peripheral 20% vascularized

VISI deformity

Figure 4-18-13

TFC: Pathology [Figure 4-18-14]

• • • •

Tear / Perforation ➢ 95% accuracy ➢ Partial vs. full thickness ➢ Radial / ulnar ➢ Central / peripheral Associated injuries ➢ LTL, ECU sheath

Radioulnar Ligaments

[Figure 4-18-15]

Volar / Dorsal margins of TFC Flat margins - Attach directly to bone Injury → DRUJ instability

Figure 4-18-15

Normal triangular fibrocartilage (TFC)

Figure 4-18-14

Normal volar and dorsal radioulnar ligaments (arrows), and normal TFC (open arrow).

• •

Extensor Carpi Ulnaris Sheath [Figure 4-18-16] Ulnar-sided support Injury leads to ➢ subluxation, tenosynovitis, tears

Figure 4-18-16 Small perforation of the TFC

Subluxed extensor carpi ulnaris tendon (arrow) Musculoskeletal Radiology

875

MRI of the Wrist

• •

Tendons •

•

Figure 4-18-17

Axial plane Flexors ➢ Carpal tunnel Extensors ➢ dorsal compartments ➢ Extensor retinaculum

Extensor Compartments • • • • • • •

1st

Abd. pollicis longus Ext. pollicis brevis 2nd Ext. carpi radialis longus/brevis Lister’s Tubercle 3rd Ext. pollicis longus 4th Ext. digitorum Ext. indicis 5th Ext. digiti minimi 6th Ext. carpi ulnaris

A. Artifactual intermediate signal in the flexor pollicis longus tendon on gradient echo image. B. Tendon appears normal on fat-saturated T2-weighted image

Figure 4-18-18

Tendon Pathology

•

Tenosynovitis ➢ Surrounding fluid, +/- enlargement ➢ Stenosing (loculated, septations) Partial tear ➢ Enlarged / thinned / focal signal Complete tear

• • • •

Artifactual signal within tendon ~ 55º to main magnetic field Short TE images Disappears on long TE images

•

Magic Angle Phenomenon [Figure 4-18-17]

•

DeQuervain’s tenosynovitis

Tendon Pathology [Figures 4-18-18 and 4-18-19] •

•

• • •

Figure 4-18-19

Extensor Carpi Ulnaris ➢ 6th dorsal compartment DeQuervain’s Syndrome ➢ 1st extensor compartment ➢ Tenosynovitis ➢ DDX: ❖ Scaphoid fracture ❖ 1st CMC arthritis ❖ Flexor carpi radialis tenosynovitis Flexor tendons ➢ Tenosynovitis ➢ Carpal tunnel syndrome

Severe tenosynovitis of the flexor tendons

Carpal Tunnel [Figure 4-18-20]

Figure 4-18-20

Floor – carpal bones Roof – flexor retinaculum Contents ➢ Flexor tendons ➢ Median nerve

Normal carpal tunnel (long arrow = flexor retinaculum; short arrow = median nerve) MRI of the Wrist

876

Musculoskeletal Radiology

• • •

Median Nerve [Figure 4-18-21]

• •

Figure 4-18-21

Volar / radial position in carpal tunnel Stable to decreasing size May appear flattened at hamate

Carpal Tunnel Syndrome • •

Compressive neuropathy Pain, paresthesias ➢ Thumb, index, long, radial 1/2 ring Worse at night DX: clinical exam, nerve conduction

• • • •

Swelling (pisiform) Flattening / angulation (hamate) Increased signal intensity – T2 Bowing of flexor retinaculum

• • •

Volar displacement of tendons/nerve Free edges of retinaculum Retinaculum not seen

• • • • •

Incomplete retinacular release Proximal swelling of median nerve Scarring around nerve Mass lesion in carpal tunnel Median nerve neuroma

Normal median nerve within the carpal tunnel

Figure 4-18-22

Carpal Tunnel Syndrome: MRI

Carpal Tunnel: Post-op

CT: Post-op Complications

• • •

Normal ulnar tunnel (Guyon’s canal)

Figure 4-18-23

Guyon’s Canal [Figure 4-18-22]

• • •

“Ulnar Tunnel” Ulnar nerve, artery, vein Boundaries ➢ Floor – flexor retinaculum ➢ Roof – fascia ➢ Lat. to pisiform and hook of hamate

Ulnar Tunnel Syndrome [Figure 4-18-23]

•

Ganglion cyst or other mass Fracture (hook of hamate) Repetitive trauma

Masses: Anomalous Muscles [Figure 4-18-24] • •

Accessory palmaris longus ➢ volar ➢ superficial to flexor tendons Ext. digitorum manus brevis ➢ dorsal ➢ near extensor indicis tendon Isointense to muscle ➢ on all sequences

Lipoma compressing the structures within the ulnar tunnel

Figure 4-18-24

Accessory palmaris longus muscle (M)

Musculoskeletal Radiology

877

MRI of the Wrist

• • • • •

Take Home Points

•

High resolution imaging! SLL and LTL?...Coronal thin section images TFCC?...TFC, radioulnar ligaments, ECU tendon Tendons?...Flexor and extensor – axial images Masses?...Ganglia 70% dorsal @ SLL 20 % volar @ distal radius Nerves?...Median – carpal tunnel Ulnar – Guyon’s canal

MRI of the Wrist

878

Musculoskeletal Radiology

MRI of the Ankle and Foot Mark Anderson, MD • • • •

Technique

• • • •

Figure 4-19-1

Surface Coil One ankle/foot only T1, T2, Fat Sat Gd? ➢ Cyst vs. Solid ➢ Infection ➢ Synovitis “screening”

Ankle/Foot: Imaging Planes

• •

Ankle Axial Coronal Sagittal

Foot Long Axis Short Axis Sagittal

Bones

•

Fracture of the anterior process of the calcaneus

Marrow Edema Differential Diagnosis ➢ Activity related ➢ Contusion/occult fracture ➢ Osteonecrosis ➢ Osteomyelitis ➢ Tumor

Figure 4-19-2

Bones: Acute Trauma [Figure 4-19-1]

• •

Contusion, bone bruise ➢ Marrow edema ➢ Hemorrhage ➢ Trabecular fx Fracture

Osteochondral Lesion [Figure 4-19-2]

•

•

Terminology ➢ Osteochondral fracture ➢ Transchondral fracture ➢ Osteochondritis dissecans (OCD) Ankle ➢ Acute trauma ➢ Talar dome ❖ Mid 1/3 lateral (inversion, dorsiflexion, LCL) ❖ Posteromedial (inversion, plantarflexion)

Osteochondral lesion, medial talar dome

Figure 4-19-3

Osteochondral Lesion [Figure 4-19-3] •

Talar Dome ➢ Mid 1/3 lateral (inversion, dorsiflexion, LCL) ➢ Posteromedial (inversion, plantarflexion) Staging ➢ 0 Normal cartilage ➢ 1 Abnl SI but intact ➢ 2 Fissuring not to bone ➢ 3 Flap or exposed bone ➢ 4 Loose fragment ➢ 5 Displaced fragment Mintz DN, et al. Arthroscopy 2003;19:353-9

Musculoskeletal Radiology

Osteochondral lesion, medial talar dome with overlying cartilage loss 879

MRI of the Ankle and Foot

• •

Bones: Os Trigonum [Figure 4-19-4]

• • • •

Ununited tubercle Os Trigonum Syndrome ➢ Post. Pain ❖ Plantar flexion (ballet) ➢ MRI ❖ Marrow edema ❖ FHL tenosynovitis (stenosing)

Figure 4-19-4

Bones: Access. Navicular [Figure 4-19-5]

• • • •

Type I – distal PT tendon Type II – close proximity to bone Cornuate navicular Pain syndrome ➢ Type II and Cornuate ➢ MRI: marrow edema Os trigonum syndrome

Tarsal Coalition [Figure 4-19-6]

•

2nd – 3rd Decade Vague hindfoot pain Calcaneonavicular Talocalcaneal ➢ 2° signs: talar beak, “C” sign, etc. Cartilaginous, Fibrous, Osseous

• • • •

Flexor hallucis brevis tendons Stress reaction/fracture (medial) Osteonecrosis (lateral) DJD (subchondral changes)

• • • • • • •

Syndesmotic Lateral Medial Spring Lisfranc Sinus Tarsi Plantar Fascia

Figure 4-19-5

Bones: Hallux Sesamoids

Ligaments

Accessory navicular

Figure 4-19-6

Ligaments: Syndesmotic [Figure 4-19-7] • • • •

Interosseous ligament Anterior tibiofibular Posterior tibiofibular Talus = rectangular Calcaneonavicular coalition

Figure 4-19-7

Anterior and posterior tibiofibular ligaments MRI of the Ankle and Foot

880

Musculoskeletal Radiology

• • • • •

Ligaments: Lateral [Figure 4-19-8]

•

Figure 4-19-8

“Fibular collateral lig complex” Anterior talofibular Calcaneofibular Posterior talofibular Talus = elongated

Ligaments: Medial [Figure 4-19-9] • •

• • • • •

Deltoid ➢ “Tibial collateral lig complex” Deep (tibiotalar) Superficial ➢ Tibionavicular ➢ Tibiocalcaneal (strongest) ➢ Posterior tibiotalar

Ligaments: Injuries [Figure 4-19-10]

Normal anterior talofibular ligament

Interruption Laxity Thickening/irregularity Edema (acute) Non-visualization

Figure 4-19-9

Figure 4-19-10

Deep fibers of the deltoid ligament

Torn anterior tibiofibular ligament (syndesmotic injury)

•

Ligaments: Chronic Injury [Figure 4-19-11]

•

Figure 4-19-11

Anterolat Impingement Syndrome ➢ ATAF ligament injury ➢ Persistent pain ➢ Scar tissue in lateral gutter ➢ MRI ❖ Intermediate SI tissue ❖ T1 and T2WI

Ligaments: Spring / Lis Franc •

Spring ligament ➢ plantar calcaneonavicular ➢ medial and plantar bands Lisfranc ligament ➢ medial cuneiform ➢ base of 2nd metatarsal Scar tissue in anterolateral gutter (arrow) secondary to chronic anterior talofibular ligament injury

Musculoskeletal Radiology

881

MRI of the Ankle and Foot

• • •

Sinus Tarsi [Figure 4-19-12]

• • • • •

Figure 4-19-12

Cone-shaped space Wide lateral – tarsal canal medial Fat, nerves, vessels, ligaments ➢ Inferior extensor retinaculum ➢ Cervical ligament ➢ Talocalcaneal interosseous lig

Sinus Tarsi Syndrome

•

Lateral pain Sense of hindfoot instability 70% – Prior trauma 30% – Inflammatory arthritis PTT tear/dysfunction

Normal sinus tarsi

Figure 4-19-13

Sinus Tarsi Syndrome

• •

MRI Findings ➢ Replacement of normal fat ➢ - SI T1 ➢ + or - SI T2 Calcaneus → toes Two bands ➢ Medial ➢ Lateral

Plantar Fascia [Figure 4-19-13]

•

Plantar Fasciitis

•

•

Inflammation ➢ Mechanical (pes cavus, etc.) ➢ Degenerative (age related) ➢ Systemic disease (RA, seronegative) DDx: ➢ Calcaneal stress fx ➢ Tendinitis ➢ Heel pad trauma/inflammation

Normal plantar fascia

Figure 4-19-14

Plantar Fasciitis [Figure 4-19-14]

•

MRI ➢ Thickened fascia (> 4 mm) ➢ + SI ❖ Fascia and perifascial tissues ❖ Calcaneus

Plantar Fibromatosis [Figure 4-19-15] • •

• •

Fibrous proliferation ➢ Fibroblasts and collagen Solitary or multiple MRI ➢ T1 / - SI ➢ T2 / low to intermediate SI ➢ Variable enhancement

Severe plantar fasciitis with partial tearing at its origin (arrow).

Figure 4-19-15

Tendons •

Change orientation Pulleys ➢ Osseous or soft tissue Magic angle effect Small, enhancing plantar fibroma (arrow) on post-contrast, fat-saturated T1-weighted image

MRI of the Ankle and Foot

882

Musculoskeletal Radiology

Tendon Pathology [Figure 4-19-16] • • •

Figure 4-19-16

Achilles Tendon [Figure 4-19-17]

•

Gastrocnemius/Soleus No tendon sheath (paratenon) Bursae ➢ Retrocalcaneal ➢ Tendo Achilles (acquired) Flat/concave ventral margin

Achilles Tendon: Pathology •

[Figures 4-19-18 and 4-19-19]

•

• • •

Insertional Tendinitis ➢ Haglund’s Syndrome ➢ Bursitis ➢ Thickened tendon ➢ “Pump bump” Non-Insertional ➢ Overuse ❖ 30-50 y.o.-“weekend warrior” ➢ Systemic disease ❖ RA, SLE ➢ Local/systemic steroids Peritenonitis Chronic Tendinitis Partial / Complete Tear

MR images and schematic diagrams of the spectrum of tendon pathology

Figure 4-19-17

Figure 4-19-18

Normal Achilles tendon

Figure 4-19-19

Haglund’s syndrome (insertional Achilles tendinopathy and partial tearing; retrocalcaneal bursitis)

Chronic, non-insertional Achilles tendinopathy and partial tearing

Musculoskeletal Radiology

883

MRI of the Ankle and Foot

• •

Plantaris Tendon

•

• • • •

Figure 4-19-20

Origin near lateral Gastrocnemius Long tendon ➢ Between med head Gastroc/Soleus ➢ Medial margin of Achilles Pitfalls ➢ “Partial tear” of Achilles ➢ “Residual fibers” of Achilles

Medial Tendons [Figure 4-19-20]

• •

Post Tibial Flex Digitorum Artery, vein, nerve Flex Hallucis

“Tom” “Dick” “and” “Harry”

Posterior Tibial Tendon

• •

Oval – 2X size of FDL Insertion sites ➢ Medial navicular ➢ Cuneiforms ➢ Bases of Metatarsals 1–4

Normal medial flexor tendons (T = posterior tibial; D = flexor digitorum; H = flexor hallucis; A = neurovascular structures in tarsal tunnel).

Figure 4-19-21

PTT: Pathology

•

Tenosynovitis, Tears Factors ➢ Degenerative (middle aged women) ➢ RA ➢ Abnormal stresses Loss of arch

PTT Pathology: MRI • • •

B

A

C

[Figure 4-19-21]

•

Tenosynovitis...Fluid Partial Tear...Thick, thin, split Complete Tear...Disruption

Spectrum of posterior tibial tendon pathology (A = tenosynovitis; B = partial tear; C = complete tear).

PTT Pathology: MRI •

• •

Secondary Signs ➢ Pes planus ➢ Spur/edema post medial malleolus Also look for: ➢ Deltoid ligament ➢ Spring ligament ➢ Sinus Tarsi

Figure 4-19-22

Lateral Tendons [Figure 4-19-22] • •

Peroneus Longus and Brevis Posterior to lateral malleolus ➢ Retrofibular groove Peroneus Brevis ➢ Anterior or medial Common Sheath

Normal peroneus tendons (L = peroneus longus; B = peroneus brevis).

MRI of the Ankle and Foot

884

Musculoskeletal Radiology

• •

Peroneus Tendons [Figure 4-19-23]

• • • • •

Figure 4-19-23

Tenosynovitis Subluxation/Dislocation ➢ Lateral margin of fibula ➢ Retinacular injury or small avulsion fx ➢ Acute or chronic Entrapment (calcaneal fracture) Partial/Complete Tear

Peroneus Brevis Split Syndrome [Figure 4-19-24]

•

Longitudinal tear (lateral malleolus) May be asymptomatic MRI ➢ C-shaped ➢ Two tendons ➢ Adjacent fluid/edema

Tarsal Tunnel Syndrome [Figure 4-19-25] • •

Fibro-osseous tunnel ➢ PT, FDL, FHL tendons ➢ Tibial nerve, artery, vein Pain, paresthesias – sole of foot Etiologies: ➢ Tumor, ganglion cyst, dilated veins, post-traumatic fibrosis

Lateral dislocation of the peroneus tendons (arrow)

Figure 4-19-24

Figure 4-19-25

Split peroneus brevis tendon (arrow = intact peroneus longus tendon)

Ganglion cyst (G) displacing the neurovascular bundle (arrow) within the tarsal tunnel

• • • •

Morton’s Neuroma [Figure 4-19-26] Plantar digital nerve Perineural fibrosis 3rd (2nd) web space MRI ➢ - SI T1 - SI T2 ➢ Variable enhancement

Figure 4-19-26

Enhancing Morton’s neuroma (arrow)

Musculoskeletal Radiology

885

MRI of the Ankle and Foot

• •

Masses: Accessory Muscles [Figure 4-19-27] •

• •

Figure 4-19-27

Isointense to Muscle on MRI Accessory Soleus ➢ Ventral to Achilles tendon Peroneus Quartus ➢ Adjacent to Peroneus Brevis

Take Home Points

•

• • •

Bone Marrow Edema?... Ligaments?... elongated talus Tendons?... brevis split Sinus tarsi?... Tarsal tunnel... Morton’s neuroma, plantar fibroma?...

Differential Syndesmotic – rectangular talus Lateral / Medial Collateral – Magic Angle; PTT – 20 signs; P. Normal fat on T1W images Space-occupying mass Low SI on T2W images – Give Gadolinium!

Accessory soleus muscle

References 1.

Mintz DN, Tashjian GS, Connell DA, Deland JT, O'Malley M, Potter HG. Osteochondral lesions of the talus: a new magnetic resonance grading system with arthroscopic correlation. Arthroscopy 2003; 19:353-359.

MRI of the Ankle and Foot

886

Musculoskeletal Radiology

Osseous Lesions Unknown Histogenesis Mark J. Kransdorf, MD • •

Figure 4-20-1

Unknown Histogenesis

•

Ewing sarcoma Langerhans cell histiocytosis ➢ Eosinophicic granuloma ➢ Hand-Schüller-Christian disease ➢ Letterer-Siwe disease

Learning Objectives • • •

Recognize the spectrum of imaging appearances of these specific osseous lesions Identify differentiating features

Ewing Sarcoma • • • • • • •

Highly malignant primary bone sarcoma Ewing provided first comprehensive description in 1921, designating it “diffuse endothelioma” of bone Later (1924) termed “endothelial myeloma” of bone, and “Ewing Tumor” by Codman Origin controversial but likely derived from primitive mesenchyme

Sheets of monotonous malignant "round cells“ with indistinct cytoplasmic margins. Areas of necrosis and hemorrhage are frequent

Ewing Sarcoma: Incidence & Distribution

• • • • • • • • •

About 5% of all biopsied tumors Usually major long bones, femur most common (25%), then humerus (8%) Long bones involved most commonly In flat bones, most common pelvis (20%) followed by ribs (11%) Rare in hands, sternum, T-spine

Ewing Sarcoma: Clinical Presentation

• • • • • •

Seventy-five percent 10–25 years Peak incidence 10 to 15 years Range 5 months to 83 years Slight male predominence (1.5:1) Pain & swelling most common symptoms Constitutional signs to include local heat, fever, anemia, leukocytosis, etc. Chromosomal trans in 90%; t(11;22) most common, others t(21;22), t(7;22) Predilection for Caucasions (95%) Usually solitary and nonfamilial; 10% are reported to be multiple at presentation Exceedingly rare familial cases (siblings), case reports in patients with retinoblastoma

Ewing Sarcoma: Pathologic Features [Figure 4-20-1] Characterized by sheets of monotonous malignant “round cells” Indistinct cytoplasmic borders Frequent areas of necrosis and hemorrhage Virtually all PAS positive (glycogen) Ewing family includes Ewing sarcoma and primitive neuroectodemal tumor [PNET]

Musculoskeletal Radiology

887

Osseous Lesions: Unknown Histogenesis

Radiologic Features: Intergroup Ewing Sarcoma Study •

Figure 4-20-2

[Figures 4-20-2 to 4-20-10]

• • • • • • • •

Distribution: diaphysis 35%, metadiaphysis 59%, metaphysis 5%, epiphysis <1% Lesions medullary, symmetric or eccentric Soft tissue mass in about 90% Reactive bone 40%, but tumor produces no cartilage or bone Cortical thickening 20% Periosteal reaction due to irritation or edema or tumor permeation 85% “Onion skin” appearance due to cyclic pattern of periosteal irritation 55% Perpendicular striations due to rapid continuous lifting of periosteum 30% Pathologic fracture in 10%–15%; soft tissue calcification 10%

Figure 4-20-3

Figure 4-20-4

MR imaging. Typical features. MR shows large heterogeneous circumferential soft tissue mass. Soft tissue changes seen to better advantage on MR. Coronal T1 (Left) and T2 (Right).

Note diaphyseal location, complex periosteal reaction and cortical thickening

Figure 4-20-6

Figure 4-20-5

Ewing sarcoma with pathological fracture. Radiograph (left) and bone scan (right)

Hair-on-end” periosteal reaction

Osseous Lesions: Unknown Histogenesis

Ewings sarcoma. Radiograph. Note metadiaphyseal location and absence of identifiable matrix

888

Musculoskeletal Radiology

Figure 4-20-7

Figure 4-20-8

Ewing sarcoma. Flat bone. MR T2 (left) and T1 (right).

Ewing sarcoma. Flat bone. Radiograph

Figure 4-20-10

Figure 4-20-9

Ewing sarcoma. Flat bone (rib). Large soft tissue mass obscure osseous origin

• • • •

Ewing sarcoma. Note permeative osteolysis with evidence of associated mass

Treatment & Prognosis: Ewing Sarcoma [Figures 4-20-11 and 4-20-12] Ablative surgery, chemotherapy and radiation therapy About 30% present with metastases Mets typically to lungs (85%), bones (69%), pleura (46%), CNS (12%) The 5-year survival rate for patients w/o mets at presentation: 55–70%

Figure 4-20-11

Figure 4-20-12

Ewing sarcoma. Post treatment change

Musculoskeletal Radiology

Ewing sarcoma. Local recurrence.

889

Osseous Lesions: Unknown Histogenesis

• • •

Langerhans Cell Histiocytosis (LCH)

• • •

Eosinophilic granuloma Hand-Schuller-Christian disease Letterer-Siwe disease

LCH: History

• • • •

1940: Jaffe & Lichtenstein eosinophilic granuloma 1941: Farber, Green & Farber EG could be solitary or multiple 1953: Lichtenstein proposed the name histiocytosis X for the inflammatory histiocytoses

LCH: Phases

• •

Solitary or multiple lesions localized to bone: Eosinophilic granuloma (> 5 y) Chronic disseminated histiocytosis: Hand-Schuller-Christian disease (1–5 y) Acute or subacute disseminated histiocytosis: Letterer-Siwe disease (<1 y) Supports the concept that this is a disorder of immune regulation

LCH: Phases • • • •

LCH, localized to bone: limited to a single or a few bones LCH, chronic disseminated: multifocal bone lesions and es involvement of lymph nodes, skin and abdominal viscera LCH, acute or subacute disseminated: disseminated multisystem involvement

LCH: More Recently Classification challenged as vague with overlapping clinical syndromes Classification includes benign and malignant LCH More recently classified as localized or multifocal ➢ Single bone lesion or single organ system ➢ Multifocal

Figure 4-20-13

Eosinophilic Granuloma: Incidence & Distribution • • • • •

About 1% of all biopsied tumors Solitary EG is about twice as common as multifocal EG About 70% involve flat bones, most commonly skull (25%), pelvis (20%) In long bones, femur then humerus Hands and feet rare in solitary disease

Eosinophilic Granuloma: Clinical Presentation • • • • •

Histiocytosis. Note histiocytes with reniform shape and clefts. Scattered eosinophils are seen

About 90% are 5–15 years (average 10–12) Male:female about 2:1 More than 95% of patients are white Most patients present with pain/tenderness Fever may be present and presentation may suggest osteomyelitis

Figure 4-20-14

Eosinophilic Granuloma: Pathologic Features [Figures 4-20-13 and 4-20-14] • • • •

Characterized by a collection of histiocytes Histiocytes are either oval, lobulated or reniform, w/ clefts or indentations Eosinophils may be seen singly, in sheets, clusters or not at all Birbeck bodies on EM

Histiocytosis. EM. Note Birbeck bodies. Osseous Lesions: Unknown Histogenesis

890

Musculoskeletal Radiology

•

Eosinophilic Granuloma: Radiologic Features • • •

Figure 4-20-15

Usually permeative destruction in early phase with periosteal reaction More sharply defined with time, although lesion may still enlarge May have a rind of sclerosis There may be an associated soft tissue mass in 5%–10% of patients

Eosinophilic Granuloma: Radiologic Features • • •

[Figures 4-20-15 to 4-20-25]

• •

Skull: beveled edge, button sequestrum Flat bone: hole within a hole Long bone distribution: diaphysis (58%), metadiaphysis (18%), metaphysis (28%), epiphysis (2%) Spine: vertebra plana Mandible/maxilla: floating teeth

Skull. Beveled edge

Figure 4-20-16

Skull. Button sequestrum.

Figure 4-20-17 Figure 4-20-18

Figure 4-20-19

Flat bone. “Hole within a hole”

Figure 4-20-20

Indolent radiographic appearance. Note epiphyseal lesion of proximal femoral lesion

Figure 4-20-23

MRI. Spine. Single lesion. T1 and T2 Musculoskeletal Radiology

Flat bone (a) and long bone (b). “Hole within a hole”

Figure 4-20-21

Flat bone (rib). “Hole within a hole”

Figure 4-20-22

Spine. “Almost” vertebral plana Spine. Vertebral plana.

Figure 4-20-25

Figure 4-20-24

Floating teeth 891

Clavicular lesion. Radiograph (a) and macrosection (b) Osseous Lesions: Unknown Histogenesis

• • •

Eosinophilic Granuloma: Prognosis & Treatment

• • • • •

Benign course Simple curettage or intralesional prednisone Large lesions and vertebral lesions may be treated with low dose RTX (300–1000 rad) May regress spontaneously

Hand-Schuller-Christian: LCH Chronic Disseminated Initially described by Hand (1893), then by Schuller (1916); Christian (1920) Classic triad: destructive skeletal lesions, exophthalmos and diabetes insipidus Histologically identical to lesions of EG About 10% of patients with unifocal EG will develop multifocal and extraskeletal disease

Hand-Schuller-Christian Disease: LCH Chronic Disseminated • • • • • •

Patients are young, usually less than 5 years Classic triad in 10%–15%, <50% have DI, exophthalmos about 25% Any bone may be involved, 90% have cranial involvement, 7% hand or foot lesion(s) Hepatosplenomegaly and adenopathy Anemia, fever, neurologic complaints Fatal in about 15%, morbidity may be high

• • • • •

Initial reported by Letterer in 1924 (one case) and Siwe in 1933 (7 cases) Usually develops within the first year of life Disease disseminated and bone lesions small Symptoms may be severe Fatal in about 95% of those who develop disease before 1 year of age

Letterer-Siwe: LCH Acute Disseminated

•

Summary •

Review the imaging appearances of Ewing sarcoma and the family of lesions know as Langerhans cell histiocytosis Demonstrate how the radiologic images reflect the underlying pathophyiology and appropriate differentiating features

References 1. 2. 3. 4.

Davis et al. Radiographic features of eosinophilic granuloma of bone. AJR 1989;153:1021 Shapeero et al. Ewing sarcoma. Radiology 1994;191:825 Stull et al. Langerhans cell histiocytosis of bone. RadioGraphics 1992;12:801 Wilkins et al. Ewing's sarcoma of bone. Cancer 1986;58:2551

Osseous Lesions: Unknown Histogenesis

892

Musculoskeletal Radiology

Soft Tissue Lipomatous Tumors Mark J. Kransdorf, MD • • •

Recognize the spectrum of common lipomatous soft tissue masses Identify the radiologic appearance of the common fatty masses Identify imaging limitations and pitfalls

• • • • • • •

Fundamental definitions Incidence of soft tissue tumors Overview Common lipomatous tumors Liposarcoma Mimics Cases

Learning Objectives

Outline:

•

Definitions • • • • • • •

Soft tissue is the nonepithelial extraskeletal tissue, excluding the RES, glia and supporting tissue of parenchymal organs It is derived primarily from mesenchyme, and by convention is comprised of skeletal muscle, fat, fibrous tissue and the serving vessels and nerves.

Incidence: New Cancers by site 2000 Breast Lung Colon/rectum CNS Soft Tissue Bone

184,200 164,000 130,200 16,500 8,100 2,500

CA Cancer J Clin 2000;50:12 •

Incidence: Variations • • • •

It is estimated that the relative frequency of benign to malignant tumors is 100:1 US overall annual incidence: 1.4 per 100K Age specific incidence ≥ 80 years: 8.0 per 100k

Classification World Health Organization subdivides benign lipomatous tumors into 9 groups For imaging purposes, it is more useful to use the classification proposed by Weiss and Goldblum

Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors, 4th ed. St. Louis; 2001 •

Classification: Weiss and Goldblum

•

Lipoma ➢ Superficial ➢ Deep ➢ Multiple Variants of lipoma ➢ Lipoblastoma ➢ Spindle cell lipoma ➢ Pleomorphic lipoma ➢ Angiolipoma ➢ Chondroid lipoma

Musculoskeletal Radiology

893

Soft Tissue Lipomatous Tumors

•

• • • • • • •

Lipomatous tumors ➢ Intramuscular lipoma ➢ Intermuscular lipoma ➢ Lipomatosis nerve ➢ Lipoma tendon sheath ➢ Lipoma joint Infiltrating lipomas ➢ Lipomatosis ➢ Symmetric Lipomatosis ➢ Adiposis Dolorosa Hibernoma

Figure 4-21-1

Lipoma: Clinical

• • • • • • •

CT. Subcutaneous lipoma

Tumor of mature fat Incidence ? Presents 40–60 yrs, uncommon <20 M ~ F, recent reports male predominence Superficial and deep (deep: chest wall, retroperitoneum, deep tissues hands and feet) Deep seated tumors rare, < 1% lipomas Typically asymptomatic, local pain or tenderness unusual More common in obese, tumor fat not available for metabolism Usually small, 80% < 5cm; 1% > 10 cm Clinical diagnosis 85% accurate Usually solitary, 5%–15% multiple 50%-80% transloc chromosome 12 q13-15

Figure 4-21-2

MR. Subcutaneous lipoma

• • • • • • • • • • •

Lipoma: Radiology [Figures 4-21-1 to 4-21-8] Radiographs may demonstrate a fat density mass Fatty nature well demonstrated on CT/MR CT tissue attenuation -65 to -120HU Visual comparison more reliable than # Signal intensity equals SQ fat on MR No enhancement with contrast (CT/MR) May contain other mesenchymal elements The most commom is fibrous tissue Termed fibrolipoma when significant fibrous tissue present May be associated with cortical thickening Occasional chondroid and/or osseous metaplasia ; when long standing-termed “benign mesenchymoma”

Figure 4-21-3

CT. Subcutaneous lipoma right shoulder. Imaging contralateral side may be useful in identifying subcutaneous lesions

Figure 4-21-4

T1

T2 MR. Fibrolipoma

Soft Tissue Lipomatous Tumors

894

Musculoskeletal Radiology

Figure 4-21-5

Figure 4-21-6

Deep lipoma. Retroperitoneum

MR: Unencapsulated lipoma

Figure 4-21-7

Lipoma with metaplastic bone formation (benign mesenchymoma). Radiograph (left) and macrosection (right)

Figure 4-21-8

Lipoma with metaplastic bone formation (benign mesenchymoma). Radiograph (left), CT (middle) and MR (right)

• •

Intramuscular Lipoma •

Lipoma arising in skeletal muscle Most common member of subgroup of lipomatous tumors (fatty tumors arising in intimate association with non-adipose tissue) Other entities in this category include: intermuscular lipoma, lipoma of tendon sheath, and lipomatosis of nerve

Musculoskeletal Radiology

895

Soft Tissue Lipomatous Tumors

Figure 4-21-9

Figure 4-21-10

Intermuscular lipoma. Note infiltrating margin.

Intramuscular Lipoma: Clinical • • •

[Figures 4-21-9 to 4-21-11]

• •

Most common 4th – 7th decades Men more commonly affected Most frequent large muscles extremities (thigh, shoulder, upper arm) Typically asymptomatic Often incidental finding

Intramuscular lipoma

Figure 4-21-11

“In general, the concept that lipomas give rise to liposarcomas is not accepted...Based on consultation material reviewed at the AFIP over several decades, we never encountered a clear-cut example of malignant transformation of lipoma, although a few possible cases have been reported in the literature.” Weiss and Goldblum Enzinger and Weiss’s Soft Tissue Tumors, 4th ed. 2001 • • •

Lipoblastoma [Figure 4-21-12]

• • • • • •

Relatively rare cellular immature lipoma Originally described as embryonic lipoma Occurs almost exclusively in infants, usually presents by 3 yrs of age, occasionally at birth Usually in superficial soft tissues or subcutis of the extremities Males affected 2–3X more commonly Two-thirds to three-quarters are discrete When diffuse, termed lipoblastomatosis Radiologically may be indistinguishable from a liposarcoma Liposarcoma is exceedingly rare in children & most pediatric fatty masses are lipoblastoma

Intramuscular lipoma

Figure 4-21-12

Lipoblastoma in 18 month old. This appearance in an adult suggests liposarcoma Soft Tissue Lipomatous Tumors

896

Musculoskeletal Radiology

• • • • • •

Diffuse overgrowth mature adipose tissue Rare, but mild cases may go undiagnosed Usually present before age 2 Considered congenital Bone hypertrophy frequent association Nerve not affected, not confined to extremity

• • • • • • •

Malignant mesenchymal tumor Second most common soft tissue tumor after MFH Approximately 16%–18% all sarcomas Presents ages 40–60 years Exceedingly rare in children Usually extremities or retroperitoneum Extremity lesions present 5–10 yrs earlier

Lipomatosis [Figures 4-21-13 to 4-21-15]

Figure 4-21-13

Liposarcoma

Lipomatosis. Clinical photo. MR different patient

Classification: World Health Organisation (WHO) (From low to high) • • • •

Figure 4-21-14

Well differentiated Myxoid Pleomorphic Dedifferentiated

Classification: World Health Organisation (WHO) • • •

Well differentiated ---> Dedifferentiated Myxoid ---> Round cell * Pleomorphic * Round cell liposarcoma was previously a distinct subtype, now considered the hypervascular variant of myxoid liposarcoma

Liposarcoma: Well-Differentiated [Figure 4-21-16] • • • •

Lipomatosis trunk

Predominantly fatty mass, usually more than 75% fat Irregularly thickened or nodular septa Presence of nodular/globular areas A small number of lipomas will have a similar imaging appearance

Figure 4-21-15

Figure 4-21-16 A

B

Mild lipomatosis right lower extremity

C

D

Well-differentiated liposarcoma. Typical features. Radiograph (a), CT (b), MR T1 (c) and T2 (d).

Musculoskeletal Radiology

897

Soft Tissue Lipomatous Tumors

Liposarcoma: Atypical Lipoma •

Figure 4-21-17

[Figure 4-21-17]

•

Lesion histologically indistinguishable from welldifferentiated liposarcoma Used for lesions in which wide surgical margin is possible, such as those in subcutaneous tissue

Liposarcoma: Dedifferentiated •

[Figures 4-21-18]

• • • •

Bimorphic lesion with: ➢ WD liposarcoma ➢ Juxtaposed high grade sarcoma (MFH) Most common dedifferentiated sarcoma May be a time-related phenomenon Imaging typically shows a well differentiated fatty mass Fatty mass associated with a focal dominant nonadipose component

Well-differentiated liposarcoma. Typical features

Figure 4-21-18

Liposarcoma: Higher Grade Lesions •

[Figure 4-21-19]

• • •

Only 50%-80% of the myxoid or pleomorphic types show fat on imaging studies Fat usually minor component (<25%) Hypervascular myxoid (round cell) and pleomorphic types are typically more heterogeneous

Liposarcoma: Myxoid Lesions [Figure 4-21-20] •

Myxoid and round cell lesions are now accepted as ends of a common spectrum About 20% of myxoid lesions will have a “cyst-like” appearance

Figure 4-21-19

Dedifferentiated liposarcoma, well-differentiated component

Figure 4-21-20

Myxoid liposarcoma, typical imaging features. T1 (upper left), T2 (upper right), CT (lower left) and gross (lower right)

Myxoid liposarcoma, cyst-like appearance. Radiograph (upper left), MR T1 (upper right), T2 (lower left), bone scan (lower right) Soft Tissue Lipomatous Tumors

898

Musculoskeletal Radiology

• • • • •

Liposarcoma: Distribution

• • • •

Type Well-differentiated Myxoid Pleomorphic Dedifferentiated

% 54 28 7 10

Retro 54 10 5 32

Extrem 54 34 8 4

Liposarcoma: Mimics

•

Hemorrhage Malignancies engulfing portion fat Muscle atrophy with fat replacement Myxoid tumors: intramuscular myxoma, ES myxoid chondrosarcoma, myxoid MFH Neural tumors

• • •

Fatty tumors are common There is a wide spectrum of lipomatous tumors Imaging of fatty tumors is frequently characteristic

Summary

References 1. 2. 3. 4.

Christopher et al. WHO Classification of tumors. Lyon, France: IARC Press; 2002 Kransdorf et al. Fat-containing masses of the extremities. RadioGraphics 1991;11:81 Peterson et al. Malignant fatty tumors. Skeletal Radiol 2003;32:493 Weiss & Goldblum. Enzinger and Weiss's Soft Tissue Tumors, 4th ed. St. Louis: CV Mosby; 2001

Musculoskeletal Radiology

899

Soft Tissue Lipomatous Tumors

Metabolic Bone Disease Mark J. Kransdorf, MD • •

Figure 4-22-1

Part I

•

Rickets and osteomalacia Scurvy

Learning Objectives • •

Identify the pathophysiologic alterations that occur in rickets and osteomalacia and scurvy Recognize the spectrum of radiological features of these diseases

Metabolic Bone Disease Systemic diseases which effect the skeleton diffusely and are the result of a metabolic disorders Biochemistry of vitamin D

•

Rickets

•

Figure 4-22-2

Abnormal mineralization and development of the growth plate

Osteomalacia Inadequate or delayed mineralization of mature cortical or spongy bone

Pitt MJ. Rad Clin No Amer:1991;29:97 •

Insufficient quantity of normal bone

•

Increased radiolucency of bone

•

Paucity of bone

•

Malformed bone

• •

D2 – Synthetic D3 – Natural

Osteoporosis Osteopenia OsteoPorosis

Normal enchondral bone formation

Figure 4-22-3

OsteoMalacia Vitamin D: Prohormone

Normal bone formation (left); rachitic bone formation (right)

Vitamin D: Biochemistry [Figure 4-22-1]

Figure 4-22-4

Vitamin D: 1, 25 Dihydroxyvitamin D • • •

Most active form of vitamin D Calcium/phosphorus homeostasis Maintenance bone mineralization

• • •

Nonspecific features Growth plate abnormalities Skeletal deformities

[Figures 4-22-2 to 4-22-4]

Rickets: Radiographic Features

Osteoid seams

Metabolic Bone Disease

900

Musculoskeletal Radiology

• •

Rickets: Nonspecific Features

Figure 4-22-5

Osteopenia Growth retardation

Rickets: Growth Plate Abnormalities • • •

[Figures 4-22-5 to 4-22-9]

Axial widening Metaphyseal lucency Metaphyseal cupping

Figure 4-22-6

Radiographic changes of rickets

Figure 4-22-7

Healing rickets with metaphyseal lucent bands (a) and macrosection (b)

Dietary rickets with treatment; presentation (a), one month (b), two months (c), and four months (d)

Rickets: Skeletal Deformities • • • • • •

[Figures 4-22-8 to 4-22-11]

Craniotabes Rachitic rosary Bowing of long bones Scoliosis Basilar invagination Triradiate pelvis

Figure 4-22-9

Figure 4-22-8

Craniotabes Musculoskeletal Radiology

Overgrowth wrist cartilage (a) with corresponding clinical photo (b) 901

Metabolic Bone Disease

Figure 4-22-10

Figure 4-22-11

Skeletal deformities

Basilar invagination (a); triradiate pelvis (b)

Osteomalacia: Classic Radiographic Features • • • •

[Figures 4-22-12 to 4-22-14]

Figure 4-22-12

Osteopenia Coarse trabecular pattern with unclear margins Looser’s zones Features can be seen in rickets

Figure 4-22-13

Coarse trabecular pattern with pseudofracture; radiograph (a) and macrosection (b)

Figure 4-22-14 Looser zone with (a) and without (b) fracture

• • •

Vitamin D-deficient GI malabsorption Neonatal

• •

Liver disease Anticonvulsant therapy

Vitamin D [Figures 4-22-15 and 4-23 16]

25-OH Vitamin D

Looser zones

Metabolic Bone Disease

902

Musculoskeletal Radiology

Figure 4-22-15

Figure 4-22-16

Rachitic disease with fracture distal left femur

Rachitic disease from nec

Figure 4-22-17 Renal Related •

[Figures 4-22-17 to 4-22-19]

•

1, 25 Dihydroxyvit D ➢ Renal osteodystrophy ➢ Vitamin D dep rickets ➢ Tumor related Renal Tubular Disorders ➢ X-linked hypophosphatasia ➢ Familial vitamin D res rickets ➢ Fanconi syndromes ➢ Tumor related ➢ Ifosfamide

Renal osteodystrophy (a) and Fanconi syndrome (b)

Figure 4-22-18 Figure 4-22-19

Rickets due to ifosfamide therapy Oncogenic osteomalacia

Musculoskeletal Radiology

903

Metabolic Bone Disease

• • •

No Abnormality [Figure 4-22-20]

•

Figure 4-22-20

Axial osteomalacia Hypophospatasia Metaphyseal chondrodysplasia

Scurvy: The Stinking Disease [Figure 4-22-21] “…the whole army was infected by a shocking disorder…those affected had …sore complaint in the mouth that rotted the gums and caused a most stinking breath. Very few escaped death…“…the surest sign of its being fatal was bleeding at the nose…barbers were forced to cut away very large pieces of flesh from the gums, to enable their patients to eat…it was pitiful to hear the cries and groans on those on whom this operation was performed.” Metaphyseal chondrodysplasia

De Joinville , 7th Crusade (1249–1254) •

Scurvy

• • •

Figure 4-22-21

Sir James Lind, Ships Surgeon, conducted the first documented controlled study in 1747 and proved oranges and lemons were effective treatment for scurvy

Scurvy: Pathophysiology Deficiency of dietary vitamin C Decrease cellular activity Decreased collagen and osteoid production

Scurvy: Radiographic Features • • • • • •

[Figure 4-22-21 and 4-22-22]

Dense metaphyseal bands Ring epiphysis Lucent metaphyseal bands Metaphyseal beaks Periostitis Subpepiphyseal infractions

Henry VIII was thought to have scurvy due to his illtemperament and horrid breath

Figure 4-22-22

Figure 4-22-23

Frankels line and Trummerfeld’s zone

Scurvy with treatment; presentation (left), one month (middle), six months (right) Metabolic Bone Disease

904

Musculoskeletal Radiology

•

Summary •

The radiographic features of metabolic bone disease are frequently characteristic These changes accurately reflect the underlying pathophysiology

References 1. 2. 3. 4. 5.

Holick. Vitamin D deficiency: what a pain it is. Mayo Clin Proc 2003;78:1457 Leggett et al. Scurvy. NEJM 2001;345:1818 Narchi et al. Symptomatic rickets in adolescence. Arch Dis Chil 2001;84:501 Pitt. Rickets and osteomalacia. In: Resnick. Diagnosis of bone and joint disorders, 4th ed. Philadelphia, W.B. Saunders Company, 2002:1901 Sundaram et al. Oncogenic osteomalacia. Skeletal Radiol 2000; 29:117

Musculoskeletal Radiology

905

Metabolic Bone Disease

Osteonecrosis and Related Conditions Mark J. Kransdorf, MD • • •

Identify the spectrum of radiological features of osteonecrosis Recognize various associated conditions Identify differentiating features

• • • • • • •

Definitions Pathophysiology of osteonecrosis Infarct geometry Radiologic-pathologic correlation Associations Complications Related conditions

Learning Objectives

Outline

• • • •

Definitions

•

Osteonecrosis – ischemic death of cellular components of bone and marrow Aseptic necrosis – equivalent to ischemic necrosis and avascular necrosis Bone infarct – osteonecrosis involving the metaphysis or diaphysis Osteochondrosis – variety of conditions in which there is increased bone density

Figure 4-23-1

Pathophysiology: Osteonecrosis

•

• • • •

Cellular changes from ischemic injury ➢ Interruption of intracellular enzymes ➢ Cessation intracellular metabolic activity ➢ Cell death Cellular sensitivity to anoxia ➢ Hematopoietic elements (6 hrs – 12 hrs) ➢ Bone cells (12 hrs – 48 hrs) ➢ Marrow fat cells (48 hrs – 5 days)

Infarct Geometry: Zones [Figure 4-23-1]

• •

Central zone of cell death Ischemic injury Active hyperemia Normal tissue Infarct geometry

Location Osteonecrosis is most common in the epiphysis Ischemic necrosis or bone infarct occur almost exclusively in areas of predominantly fatty marrow

Figure 4-23-2

Osteonecrosis: Radiologic-Pathologic Correlation • • • • •

[Figures 4-23-2 and 4-23-3]

Phase I: Phase II: Phase III: Phase IV: Phase V:

Cellular death initial response Cell modulation Emergence reactive interface Remodeling reactive interface Crescent sign & collapse

Bilateral femoral head osteonecrosis Osteonecrosis and Related Conditions

906

Musculoskeletal Radiology

• • • • •

Associations

•

Trauma Hemoglobinopathy Steroids Alcoholism Collagen vascular disease

• • • • •

Dysbaric disorders Gaucher disease Pregnancy Irradiation Pancreatitis

Figure 4-23-3

Osteonecrosis: Causes • •

Thrombophilia (increased tendency to develop thrombosis) Hypofibrinolysis (reduced ability to lyse thrombi) Found in 76% of patients with osteonecrosis*

*Glueck et al. Osteonecrosis. AAOS 1997 •

Increased size fat cell → compresses sinusoid vascular bed → impedes blood flow

Osteonecrosis: Causes [Figure 4-23-4]

Radiograph and specimen radiograph showing osteonecrosis with collapse and crescent sign

Figure 4-23-4

Corresponding gross and macro section showing osteonecrosis with collapse and crescent sign

Figure 4-23-5

Radiograph showing typical serpentine margin of infarct Radiograph showing flattening with collapse and crescent sign

• • • •

Alcoholism • Sickle cell anemia • Exogenous sterosis • Pancreatitis

“Aseptic”

Trauma Idiopathic Caisson disease (dysbaric)

Figure 4-23-6

Imaging Features: Radiographs • • • • • •

[Figures 4-23-5 and 4-23-6]

Patchy lucent/sclerotic areas Serpentine sclerosis Arc-like subchondral lucencies Articular collapse Preservation of joint space Surrounding osteopenia Osteonecrosis of proximal pole of scaphoid with surrounding osteopenia

Musculoskeletal Radiology

907

Osteonecrosis and Related Conditions

Imaging Features: Scintigraphy • •

Figure 4-23-7

[Figures 4-23-7]

• • •

Decreased or absent uptake initially Increased uptake with repair & revascularisation

Imaging Features: CT [Figures 4-23-8 and 4-23-9] Variable findings with age of lesion Alterations in osseous architecture Useful to evaluate the integrity of the articular surface

Bilateral osteonecrosis with increased tracer accumulation on right, left is normal

Figure 4-23-8

Figure 4-23-9

Bilateral osteonecrosis with collapse on left

CT showing reactive interface bilaterally

Figure 4-23-10

Imaging Features: MRI • • • • • •

[Figures 4-23-10 and 4-23-11]

Ring or band pattern Homogeneous or heterogeneous ”Double line sign” Joint effusion Marrow edema Articular collapse

Osteonecrosis and infarcts with “double line “ sign

Figure 4-23-11

Osteonecrosis with edema pattern

Osteonecrosis and Related Conditions

908

Musculoskeletal Radiology

• • •

Transient Osteoporosis [Figures 4-23-12 to 4-23-14]

•

Figure 4-23-12

Described originally in 3rd trimester Typically young and middle-aged adults Progressive hip pain, symptoms regress in 2-6 months Edema pattern on MR, osteoporosis on radiographs

Figure 4-23-13

Transient osteoporosis with edema pattern and no osteonecrosis

Figure 4-23-14

Note regional osteoporosis of right hip

• • • • • • •

Radiographic Staging [Figure 4-23-15] Stage 0 1 2 3 4 5

Findings Clinically suspected, imaging normal Clinical findings, abnormal scintigram Osteopenia, cysts, bone sclerosis Crescent sign without collapse Flattening with normal joint space Joint narrowing with abnormal acetabulum

Note regional osteoporosis of left hip

Figure 4-23-15

Complications: Osteonecrosis • • • •

[Figures 4-23-16]

Cartilaginous abnormalities Intra-articular loose bodies Cyst formation Malignant transformation

Figure 4-23-16 Bilateral osteonecrosis

Note “screw treads” extending through infarct with associated high grade sarcoma

Infarct with malignant transformation

Musculoskeletal Radiology

909

Osteonecrosis and Related Conditions

Spontaneous Osteonecrosis • • • • •

Figure 4-23-17

[Figures 4-23-17 and 4-23-18]

Middle-aged to elderly Abrupt onset – pain, swelling, < rom Weight bearing surface Medial femoral condyle ? traumatic, ? vascular, ? meniscal tear

Osteochondritis Dissecans •

[Figures 4-23-19 to 4-23-21]

• • • • •

Fragmentation and possible separation of articular surface Typically childhood to adolescent Variable symptoms Non-weight bearing surface Classic: lateral medial femoral condyle Probably traumatic in origin

Spontaneous osteonecrosis medial femoral condyle. Presentation (a) and at 5 months (b)

Figure 4-23-18

Figure 4-23-19

Spontaneous osteonecrosis medial femoral condyle. T1 (left) and T2 (right)

Osteochondritis dissecans, classic location

Figure 4-23-20

Figure 4-23-21

MR-Osteochondritis dissecans, classic location

Osteonecrosis and Related Conditions

Osteochondritis dissecans, classic location, Radiograph (left) and MR (right) 910

Musculoskeletal Radiology

•

Osteochondroses

• •

Varied disorders characterized by: ➢ Predilection for children ➢ Involvement of epiphysis or apophysis ➢ Radiographs showing fragmentation, collapse, sclerosis and reossification Osteonecrosis is not a feature in many, and is secondary (to trauma) in others Some are normal variations

• • • • •

Lunate: Kienböck 2nd metatarsal: Frieburg Femoral head: Legg-Calvé-Perthes Tarsal navicular: Köehler Capitulum: Panner

Osteochondroses: Characterized by Osteonecrosis

• •

Figure 4-23-22

Kienböck’s Disease [Figures 4-23-22] • • • • • • •

Most common in young adults (20–40 yrs) Trauma hx +/-, pain, swelling, increased density, altered shape, collapse and fragmentation

Osteochondroses: Without Osteonecrosis Spine: Scheuermann Tibial tubercle: Osgood-Schlatter Tibial epiphysis: Blount Patella: Sinding-Larsen-Johansson

Kienbocks disease

Figure 4-23-23

Scheuermann Disease • • • • •

[Figures 4-23-23]

Described in adolescent farm workers Common, seen in about 4%–8% population Presents second decade, M=F Variable clinical presentation Radiographs show end plate irregularity, narrowed disc spaces, and Schmorl’s nodes involving three or more vertebrae

Osteochondroses: Variations in Normal Ossification • • •

Calcaneous: Sever Ischiopubic synchondrosis: Van Neck Scheurmann disease

Summary • •

Morphologic changes in osteonecrosis are relatively characteristic, although they will vary with location. There are a variety of predisposing conditions, as well as those patients in which no cause is found. The term osteochondrosis is used for a variety of conditions, many of which show no evidence of osteonecrosis

References 1. 2. 3. 4.

Sweet et al. Osteonecrosis: pathogenesis. In: Resnick D, ed. Diagnosis of bone and joint disorders, 4th ed. Philadelphia: WB Saunders, 2002 Iida et al. Correlation between bone marrow edema and collapse of the femoral head in steroid-induced osteonecrosis. AJR 2000;174:735 Vande Berg et al. MR imaging of avascular necrosis and transient marrow edema of the femoral head. RadioGraphics 1993;13:501 Glueck et al. Thromophilia, hypofibrinolysis, and osteonecrosis. Clin Orthop 1997;334:43

Musculoskeletal Radiology

911

Osteonecrosis and Related Conditions

Approach to the Inflammatory Arthropathies Donald J. Flemming, MD • •

Figure 4-24-1

Inflammatory Arthropathies

•

Rheumatoid Arthritis Spondyloarthropathies ➢ Ankylosing spondylitis ➢ Enteropathic arthritis ❖ Crohn, Ulcerative Colitis, Whipple ➢ Psoriatic arthritis ➢ Reiter Disease Juvenile Chronic Arthritis

• • • • • • • •

Soft Tissue Swelling Soft Tissue Calcification Mineralization Joint Space Change Erosion Bone Production Subluxation Distribution

• • •

Symmetrical around joint (fusiform) Diffuse (“Sausage digit”) Lumpy, bumpy

Radiographic Assessment

AP radiograph with non specific fusiform soft tissue swelling surrounding the proximal interphalangeal joint of the ring finger. Clinical photograph in a different patient with rheumatoid arthritis and synovitis and fusiform soft tissue swelling involving the proximal interphalangeal of the index and middle fingers

Soft Tissue Swelling [Figure 4-24-1]

Sausage Digit [Figures 4-24-2 • • •

Figure 4-24-2

and 4-24-3]

Mineralization Normal Juxta-articular Diffuse

AP radiograph of patient with psoriatic arthropathy and diffuse swelling of the second toe producing a sausage appearance of the digit. Clinical photograph in a different patient with psoriatic arthropathy and sausage enlargement of the third and fourth toes

Juxtaarticular Osteoporosis [Figure 4-24-4]

Figure 4-24-3

Figure 4-24-4

MR of sausage digit with intermediate signal of tenosynovitis surrounding the flexor tendon sheath of second digit. Tendon sheath of first digit is normal. MIP projections following intravenous contrast administration show diffuse enhancement of second, third and fourth toes in patient with psoriatic arthritis and sausage digits

27 year old man with reactive arthritis and erosive disease in the right first metatarsal phalangeal joint. Loss of subchondral bone is seen in the second, third, fourth and fifth metatarsal phalangeal joints indicating hyperemia associated with inflammation Approach to the Inflammatory Arthropathies

912

Musculoskeletal Radiology

• • • • •

Joint Space Change [Figures 4-24-5 and 4-24-6]

Figure 4-24-5

Widening Normal Uniform narrowing Asymmetrical narrowing Ankylosis

Figure 4-24-6

The interphalangeal joints should all be similar in dimension as should the metacarpal phalangeal joints when compared to the neighboring articulations. A “horizontal” scan pattern is useful to detect subtle joint space narrowing. Note the loss of joint space in the distal interphalangeal joint of the ring finger in this patient with posttraumatic osteoarthritis

Joint space narrowing is important for differential diagnosis. Both patients have erosions involving the wrist presenting as lucencies in the carpal bones. Patient A has rheumatoid arthritis with diffuse narrowing of all of the carpal articulations. Patient B has gout with maintenance of joint space despite extensive erosive disease

•

Figure 4-24-7

Erosions [Figures 4-24-7 and 4-24-8] • •

Aggressive ➢ Marginal ➢ Central Nonaggressive Early erosions ➢ Thin cartilage ➢ Absent cartilage

Figure 4-24-8

Photomicrograph of axially sectioned fifth metatarsal head showing destruction of subchondral bone that would present as an erosion on radiography

Typical diarthrodial joint anatomy. In early disease, the articular cartilage (light blue) prevents synovial inflammation (red) from damaging subchondral bone (white). Erosions are seen earliest where cartilage is thinnest or where cartilage is absent. Minimal or no cartilage is present at the margins of a typical synovial joint adjacent to the attachment of fibrous capsule

Musculoskeletal Radiology

913

Approach to the Inflammatory Arthropathies

•

Bone Production [Figure 4-24-9]

• • •

Reparative Response ➢ “Whiskering”/ “brush” stroke erosions ➢ Overhanging edge of cortex ➢ Subchondral bone ➢ Osteophytes Enthesopathy Periostitis Ankylosis

• • • • •

Most common in females – 2–4:1 Most common in the fourth and fifth decades Incidence – 0.2-0.4/1,000 in females Prevalence – 0.5% – 1.0% Probably heterogeneous disorder

Figure 4-24-9

Rheumatoid Arthritis

• • •

Patient A has psoriatic arthritis with erosions and bone production in the metatarsal phalangeal and interphalangeal joints. Bone formation is present amongst the erosive changes producing a “whiskering” type appearance and the digits are dense secondary to osteitis. Patient B has rheumatoid arthritis with erosive disease in the metatarsal phalangeal joints but no bone production

Rheumatoid Arthritis

• • • • •

Genetic influence – HLA-DR4 (DRB1 allele) Pregnancy – increased risk postpartum Infectious agents ? ➢ Chlamydia

RA – Presentations

• • • • •

Gradual onset, polyarthritis – typical Mono or pauciarticular – unusual Abrupt, acute polyarthritis – unusual Systemic disease Felty Syndrome ➢ RA ➢ Splenomegaly ➢ Leukopenia

Figure 4-24-10

RA – Diagnostic Criteria

• •

Morning stiffness -Three or more joints involved Arthritis of hand joints Symmetric arthritis Rheumatoid nodules Rheumatoid factor – 90% patients ➢ Positive CCP increases specificity Radiographic changes Four criteria to have diagnosis

Classic manifestations of rheumatoid arthritis. Erosions and joint space narrowing are present in the proximal interphalangeal, metacarpal phalangeal and wrist joints in a bilateral and symmetric distribution

Rheumatoid Arthritis: Radiographic Manifestations • • • • • • • • • •

Figure 4-24-11

Fusiform soft tissue swelling Diffuse or juxta-articular osteoporosis Uniform joint space narrowing Aggressive marginal erosions No bone production Synovial/subchondral cysts Bilateral symmetrical – distribution PA view (A) of the wrist is shows subtle joint space narrowing in the wrist in this patient with rheumatoid arthritis. The Norgaard view (B) reveals erosive disease in the pisotriquetral joint the is impossible to appreciate in the PA projection

RA – Hand and Wrist [Figures 4-24-10 and 4-24-11]

• • •

“100%” of patients MCP, PIP joint space loss/ erosions Pancarpal joint space loss/ erosions ➢ ulnar styloid/ pisotriquetral early Ulnar drift – carpus and digits Swan-Neck, Boutonniere deformities Ankylosis rare – limited to carpus

Approach to the Inflammatory Arthropathies

914

Musculoskeletal Radiology

Ball-catcher’s View (Norgaard View)

Figure 4-24-12

[Figure 4-24-11]

Rheumatoid Arthritis – MR Findings •

[Figure 4-24-12]

• • • • • •

Synovial hypertrophy ➢ fat saturated fast spin echo T2 weighted images ➢ gadolinium “Erosions” ➢ low signal T1W ➢ low to high signal on T2W ➢ surrounding edema Hyaline cartilage loss

RA- Feet [Figure 4-24-13 ]

•

•

Coronal T1 weighted MR of the wrist shows numerous erosions depicted by intermediate signal replacing fat in subchondral bone that are difficult to appreciate on the PA radiograph (B)

80%–90% of patients May precede hand dz – 10%–20% Forefoot – MTP disease predominates Midfoot – talocalcaneonavicular joint ➢ May see osseous ankylosis Hindfoot – retrocalcaneal bursa

Figure 4-24-13

RA – Knee and Hip [Figure 4-24-14] • • •

•

Knee – 80% ➢ Pancompartmental joint space loss ➢ Minimal erosions Hips – 50% Axial migration Acetabular protrusio ➢ Medial deviation beyond ilioischical line ➢ 3mm in males; 6mm in females

RA – Shoulder and Elbow

• • • •

Shoulder – 60% of patients ➢ Erosion in humeral head tend to be lateral ➢ Rotator cuff tear common ➢ Bilateral AC joint erosive disease Elbow – ~ 34% of patients

Classic manifestations of rheumatoid arthritis of the feet. Erosions and joint space narrowing are seen in the metatarsal phalangeal joints. The erosions are more readily seen on the medial aspect of the first through fifth metatarsal heads and the lateral aspect of the fifth metatarsal head

Figure 4-24-14

RA- Cervical Spine [Figures 4-24-15]

• • •

60% – 80 % of patients Atlantoaxial subluxation Odontoid process erosion ➢ MRI best defines extent of pannus Apophyseal joint dz Erosion of joints of Luschka Spinous process erosions

Figure 4-24-15

Rheumatoid arthritis involving the knee. Note tricompartmental loss of joint space without erosions or osteophyte formation

Rheumatoid arthritis of the cervical spine with instability at C1C2. Widening of the atlantoaxial joint is seen only in flexion in this patient

Musculoskeletal Radiology

915

Approach to the Inflammatory Arthropathies

• • • •

Spondyloarthropathies

•

Family of inflammatory arthritides of synovium and entheses Axial and asymmetric peripheral arthritis Genetic predisposition – HLA B27 Infectious etiology

Spondyloarthropathy – Criteria • • • • • • • • • •

Inflammatory spine pain or synovitis and one or more of following Positive family hx Psoriasis/ IBD Urethritis/cervicitis/diarrhea – within 1 month Buttock pain Enthesopathy Sacroiliitis

HLA B27

• • •

Normal population -USA ~0–8% Ankylosing spondylitis – >90% Reiter Disease – 63%–75% Psoriasis – not increased without arthritis ➢ with peripheral arthritis – 20% ➢ with axial arthritis – 50% IBD with axial arthritis – 50%

Figure 4-24-16

Psoriatic Arthritis

• • •

Peak ages – 20–40 years M:F – 1:1 ➢ Spine and DIP – M>F ➢ Symmetric polyarthritis – F>M Arthritis in 5%–8% of patients with psoriasis Skin dz before arthritis in 75% Arthritis before skin dz in 10%

Psoriatic Arthritis: Radiographic Manifestations • • • • •

•

Fusiform soft tissue swelling Maintenance of mineralization Dramatic joint space loss Bone proliferation Marginal erosions predominate ➢ “Pencil-in-cup” erosions Bilateral asymmetric dz

Psoriatic arthropathy of the hands involving the interphalangeal joints of both hands in a bilateral but asymmetric pattern

Figure 4-24-17

Psoriatic Arthritis: Radiographic Manifestations •

[Figures 4-24-16 and 4-24-17]

•

Hand/Feet ➢ Distribution ❖ IP joints – asymmetric ❖ Ray distribution ❖ RA distribution ➢ Acroosteolysis ➢ Ankylosis – ~ 15% ➢ Calcaneal erosion – plantar bone proliferation Wrist – pancarpal

Typical central erosion in patient with erosive osteoarthritis compared to marginal erosions seen in patient with psoriatic arthritis Approach to the Inflammatory Arthropathies

916

Musculoskeletal Radiology

• • • • • • •

Erosive Osteoarthritis

Figure 4-24-18

Asymmetrical soft tissues around joint Normal mineralization Nonuniform loss of joint space Central “sea gull” erosions Osteophytes Subchondral sclerosis Distribution – symmetrical

Psoriatic Arthritis: Radiographic Manifestations •

•

• • • •

SI Joints – 30%–50% ➢ Bilateral asymmetrical (symmetrical) ➢ Erosion (iliac > sacral) and repair Spine – 17% ➢ Large, bulky, lateral bone outgrowths ➢ Unilateral or bilateral, asymmetrical ➢ Infrequent apophyseal involvement in lumbar spine

Reactive Arthritis: (Reiter Disease) Young adults M:F – 50–1:1 Annual incidence – 30–40/100,000 Frequently associated with infection ➢ Urethritis/cervicitis ➢ Diarrhea – Shigella, Salmonella, Campylobacter

Typical fluffy inflammatory plantar calcaneal enthesophyte that parallels the undersurface of the calcaneus in patient with reactive arthritis. The bone is dense and an erosion is present in the posterior superior aspect of the calcaneus

Figure 4-24-19

Reiter Disease/Reactive Arthritis: Radiographic Manifestations • • • • • • • •

Diffuse soft tissue swelling Early – juxta-articular osteoporosis Late – normal mineralization Uniform joint space loss Aggressive marginal erosions Bone production Bilateral asymmetrical distribution Feet, ankles, knees and SI joints

25 year old man with reactive arthritis. Erosions and subluxation are seen in the metatarsal phalangeal joints in a bilateral but asymmetric pattern. Subtle bone formation is present along the medial aspect of the navicular and the medial cuneiform of the left foot as a manifestation of the asymmetric nature of this disease

Reiter Disease/Reactive Arthritis: Radiographic Manifestations •

[Figures 4-24-18 to 4-24-20]

• •

• •

Figure 4-24-20

Feet – 40%–55% ➢ IP’s and MTP’s ➢ Erosions with repair ➢ Periostitis along diaphyses Calcaneus – 25%–50% ➢ May be “sole” sight of disease ➢ Plantar and posterior erosions ➢ Enthesophytes Ankle – 30%–50% ➢ Joint space loss and periostitis Knee ➢ Effusion ➢ Joint space loss and periostitis SI joints ➢ Bilateral asymmetric ➢ Erosions and repair

Bone formation at the posteromedial aspect of the distal tibia in a patient with psoriatic arthritis

Musculoskeletal Radiology

917

Approach to the Inflammatory Arthropathies

• • • • •

Reiter vs. Psoriatic

• • • •

Figure 4-24-21

Juxtaarticular osteoporosis Periostitis without joint findings Less ankylosis of IP joints Tendency to involve MTP vs. IP joints Lower extremity involvement predominates

Ankylosing Spondylitis

•

Peak age of onset – 15–35 years M:F – 3–5:1 Incidence ~ 6.6/100,000 Strong association with HLA B27 Rare in blacks Predilection for axial involvement

AS-Radiographic Manifestations •

Typical presentation of ankylosing spondylitis with erosions and sclerosis involving the inferior aspect of the SI joints in a bilateral and symmetric pattern

[Figures 4-24-21 and 4-24-22]

•

• • • • • • •

Sacroiliac disease ➢ Bilateral symmetric – same as enteropathic ➢ Erosions predominate iliac vs. sacrum ➢ Sclerosis ➢ Ankylosis Other pelvic dz ➢ Pubic symphysis – 16%–23 % erosion and ankylosis ➢ Enthesitis – ilium and ischium

Figure 4-24-22

Sacroiliitis: Differential Diagnosis Ankylosing Spondylitis Enteropathic Arthropathy Psoriasis Reactive Arthritis Hyperparathyroidism Osteiitis Condensans Infection Ferguson view of the pelvis with bilateral symmetric sacroillitis in patient with ankylosing spondylitis

AS-Radiographic Manifestations •

[Figures 4-24-23 to 4-24-25]

Spine Disease – ascends from lumbar to cervical ➢ Discovertebral destruction ❖ Romanus and Andersson lesion ➢ Shiny corner sign ➢ Squaring of vertebral body ➢ Syndesmophyte ➢ Bamboo spine ➢ Trolley track and Dagger signs ➢ Atlantoaxial disease

Figure 4-24-23

Density confined to the anterior superior and inferior end plates of the lumbar spine resulting in the classic “shiny corner” presentation of ankylosing spondylitis. Note the lack of anterior concavity of the vertebral bodies that contributes to its “squared” appearance

Approach to the Inflammatory Arthropathies

918

Musculoskeletal Radiology

Figure 4-24-24

Figure 4-24-25

Lateral radiograph of the cervical spine in patient with ankylosing spondylitis shows thin posterior and anterior syndesmophytes and fusion of the facet joints. Lateral radiograph of the thoracic spine also shows thin posterior and anterior syndesmophytes

The normal lumbar vertebral body is concave anteriorally. The 41 year old patient with ankylosing spondylitis shows bone formation at the anterior aspect of the vertebral bodies resulting is a squared appearance

Figure 4-24-26

DISH: Diffuse Idiopathic Skeletal Hyperostosis [Figure 4-24-26] • •

• • •

Common disease – 12% of elderly population Flowing bulky paravertebral ossification ➢ Four contiguous vertebral bodies Thoracic>lumbar>cervical Enthesophytes – particularly pelvis Absence of erosions/ joint abnormality

• • • •

Uveitis Ascending aortitis/ aortic valve disease Cardiac conduction abnormalities Interstitial lung dz - upper lobes

AS-Extraskeletal Manifestations

•

Juvenile Chronic Arthritis • • • • •

JRA (seronegative) – 70% ➢ Still Disease, pauci/monarticular, polyarticular Juvenile-onset adult type RA – 10% Juvenile-onset ankylosing spondylitis Psoriatic arthritis Enteropathic arthritis Reactive/ Reiter arthritis

• • • • • • •

M:F = 1:1 Age usually less than 5 years Acute febrile illness Rash Generalized adenopathy/hepatosplenomegaly Pericarditis Mild joint findings – arthralgias/mild arthritis

Bulky paravertebral ossification in patients with DISH is usually easily distinguished from the thin anterior syndesmophytes of ankylosing spondylitis that typically have no horizontal component

Still Disease: Systemic Disease (Classic)

Musculoskeletal Radiology

919

Approach to the Inflammatory Arthropathies

• •

JRA-Still Disease: Pauci or Monoarticular

• • • • • • •

Figure 4-24-27

Females more commonly than males Large joint disease ➢ Knees, ankles, elbows, and wrists

JRA: Polyarticular M:F = 1:1 Symmetric arthritis Hands – MCP, PIP Wrists Knees/Ankles Feet – intertarsal, MTT, MTP, IP’s Cervical spine

Juvenile-onset adult type RA (Seropositive JRA) • • • • •

Classic manifestation of mono or pauciarticular JRA. The left knee is enlarged and shows advanced bone age in comparison to asymptomatic right knee. Note the lack of erosions and joint space narrowing

F>M >10 years of age at onset Polyarticular Subcutaneous nodules Vasculitis

Figure 4-24-28

JRA: Radiographic Manifestations • • •

[Figures 4-24-27 to 4-24-31]

• • • • • • • •

Fusiform soft tissue swelling Osteoporosis Joint space loss ➢ Not prominent in monoarticular ➢ May be rapid in sero(+) JRA ➢ Ankylosis – hands, wrists, cervical spine Bone erosion – not prominent finding Periostitis ➢ Phalanges, metacarpals, metatarsals Ballooned epiphyses Accelerated skeletal growth Premature fusion of physes

17 year old man with polyarticular JRA and left hip pain. The femoral heads are enlarged resulting in “ballooned” appearance of the epiphyses

Osteoarthritis

• • •

Most common arthropathy ~ 80% of patients over 75 years Second only to CHD as cause of work disability for men > 50 years of age Primary – no underlying abnormality Secondary -preexisting metabolic, anatomic, traumatic, or inflammatory condition

Figure 4-24-29

Osteoarthritis: Definition American College of Rheumatology “ a heterogeneous group of conditions that lead to joint symptoms and signs which are associated with defective integrity of articular cartilage, in addition to related changes in the underlying bone at the joint margins.”

Coronal SPGR image of the left hip in patient with JRA showing marked irregularity in the articular cartilage

Approach to the Inflammatory Arthropathies

920

Musculoskeletal Radiology

Figure 4-24-30

Figure 4-24-31

Polyarticular JRA of the hands. Generalized osteoporosis and joint space narrowing is present with striking lack of erosions

• • • •

Adult patient with JRA as a child. Note ballooned appearance of the metacarpal heads right greater than left. Wrist involvement was also aymmetric in this patient. Minimal erosions are seen

Osteoarthritis: Clinical

• •

Increasing prevalence with age over 40 Pain, stiffness, and loss of range of motion Symptoms may regress or be cyclic Risk Factors ➢ Heredity – AD trait with Heberden nodes ➢ Obesity – risk factor for knee and possibly hand ❖ Not risk factor for hip ➢ Hypermobility – increases risk ➢ Occupation – increased risk in heavy manual labor ❖ No increased risk from recreational sports ➢ Diabetes – increases risk ➢ OA in one joint increases risk for other joints ➢ Osteoporosis – protective effect in hip OA ➢ Cigarette smoking – protective effect

Osteoarthritis and Pain

• • •

• • • • • • •

Most common and important complaint Source ➢ raised intraosseous pressure ➢ synovitis/bursitis/tenosynovitis ➢ periosteal elevation ➢ muscular imbalance Less common in very old or young Psychosocial factors Radiographic predictors ➢ Osteophytes in knee good predictor ➢ Joint space narrowing in hip predictor ➢ Good in first CMC joint ➢ Poor in hand IP joints

Osteoarthritis: Radiographic Manifestations Normal mineralization Nonuniform joint space loss Absence of erosions Subchondral new bone formation Osteophyte formation Subchondral cysts Subluxations

Musculoskeletal Radiology

921

Approach to the Inflammatory Arthropathies

• •

Osteoarthritis: Subchondral “Cysts” [Figure 4-24-32]

• • •

Figure 4-24-32

Not true cysts Intrusion ➢ Defect in cartilage leads herniation of joint fluid into bone ➢ Cyst size based on joint pressure Contusion ➢ Repeated insult to subchondral bone leads to resorption

Osteoarthritis: Osteophytes [Figures 4-24-33 to 4-24-35] • •

Tend to occur at the margins of joints Produce “enlargement” of joint ➢ attempt to stabilize joint Can be central – “button osteophyte” May not be dramatic in osteoporotic women

Figure 4-24-33

Typical subchondral lucencies in specimen radiograph of osteoarthritic femoral head

Osteophytes of the interphalangeal joints of the hands are usually best appreciated on lateral radiographs

Figure 4-24-34

Figure 4-24-35

Osteoarthritis of the hip with superior lateral joint space narrowing accompanied by subchondral sclerosis, subchondral cyst formation and osteophyte production

Large osteophytes projecting from the articular surfaces of the medial and lateral femoral condyles

Approach to the Inflammatory Arthropathies

922

Musculoskeletal Radiology

• • •

Osteoarthritis: Subchondral Sclerosis

Figure 4-24-36

Also known as eburnation Stimulated by loss of hyaline cartilage Combination of new bone on existing trabeculae from microfracture and repair

Osteoarthritis: Radiographic Manifestations •

[Figures 4-24-36 and 4-24-37]

•

Hands ➢ DIP joints – Heberden nodes ➢ PIP joints – Bouchard nodes Wrist ➢ First metacarpal-carpal joint

Figure 4-24-37 Patient A shows Heberdon nodes from osteophytes and soft tissue swelling at the distal interphalangeal joints. Bouchard nodes are seen at the proximal interphalangeal joints of patient B

Figure 4-24-38

Hooked osteophytes are seen involving the second and third metacarpal heads. These may be seen in hemochromatosis, CPPD arthropathy or osteoarthritis

Osteoarthritis - Knee: Radiographic Manifestations • • • • •

[Figures 4-24-38 and 4-24-39]

May require weight-bearing views Medial compartment – 75% Patellofemoral joint – 48% Lateral compartment – 26% Pancompartmental ➢ Think deposition dz or prior inflammatory arthropathy

Non weight bearing AP of the knee (A) shows osteoarthritis in the medial compartment with subchondral sclerosis and osteophyte formation but the joint space appears maintained. AP weight bearing view of the knee (B) shows the expected loss of joint space in the medial aspect of the knee

Figure 4-24-39

Osteoarthritis - Hip: Radiographic Manifestations •

• •

Superolateral migration ➢ 60% ➢ M>F Medial migration ➢ 25% ➢ F>M Axial migration ➢ Think deposition dz or prior inflammatory dz

56 year old woman with acromegaly. Manifestations of osteoarthritis are seen but the joint spaces are widened rather than narrowed

Musculoskeletal Radiology

923

Approach to the Inflammatory Arthropathies

Hip Joint Space Narrowing

• •

Osteoarthritis - Foot: Radiographic Manifestations

• •

Occurs along lines of weight bearing First MTP joint ➢ Hallux rigidus ➢ Hallux valgus First MTT joint Talonavicular joint ➢ Dorsal talar beak (coalition vs. DJD)

References 1. 2.

Brower A: Arthritis in Black and White, 2nd ed. Philadelphia, Pa: WB Saunders; 1997: 252. Resnick D ed. Diagnosis of bone and joint disorders, 4th Ed. Philadelphia: W.B.Saunders, 2002:

Approach to the Inflammatory Arthropathies

924

Musculoskeletal Radiology

MRI of the Rotator Cuff Donald J. Flemming, MD MRI of Rotator Cuff Tears Utopia CSS FT ESS FT CCS PT ESS PT

Sensitivity 89%–100% 56% 78% 0% 71%

Specificity 88%–97% 73% 83% 68% 71%

CCS-Clinical Community; ESS-Experienced Specialist Arthroscopy 1997; 13:710–719 • • • • •

Review anatomy/positioning Review MR appearance of tears Discuss problem tears Discuss clinical mimics of rotator cuff tear Discuss the radiologic report

• • • • • • • • • • •

Rotator Cuff Disease Impingement Arthritis Adhesive Capsulitis Cervical Spine Referred Pain Instability Fracture Osteonecrosis Nerve Entrapment Syndromes Bursitis

• • • • •

Radiographs Arthrography CT Arthrography Ultrasound MRI

• •

Humeroacromial space <7mm Massive tear

MRI of the Rotator Cuff: Goals

Shoulder Pain

Figure 4-25-1

Shoulder Imaging

AP radiograph of the shoulder shows superior narrowing of the humeral acromial space indicative of a large rotator cuff tear. Coronal oblique T2 weighted in the same patient confirms a large tear in the supraspinatus tendon

Radiography [Figure 4-25-1]

CT and Ultrasound [Figure 4-25-2] • • • • • • • • • • •

Figure 4-25-2

MRI of the Shoulder Rotator cuff Glenoid labrum Capsule Biceps tendon Bone marrow Acromial shape AC joint Sub-deltoid bursa Supraspinatus notch Coracohumeral lig Humeral head shape

Musculoskeletal Radiology

Coronally reconstructed CT image following a direct arthrogram shows a small full thickness rotator cuff tear in the supraspinatus tendon. Coronally acquired ultrasound image in the same patient demonstrates the full thickness tear as a focal hypoechoic defect in the hyperechoic tendon

925

MRI of the Rotator Cuff

• • • • •

MRI of the Rotator Cuff

• • •

Figure 4-25-3

Coil-dedicated shoulder Slice thickness – 3–4 mm Matrix – 256x192 or 256x256 FOV – 16 cm Patient position ➢ External rotation vs. neutral ➢ ABER Contrast – Indirect or Direct

Pulse Sequences • • •

•

Spin-echo Fast spin-echo (fat-sat) ➢ Sensitive for cuff tear STIR Gradient echo 3D volume

Axial gradient echo image (A) with typical planscan for coronal oblique images drawn perpendicular to the glenoid. Axial gradient echo image through the supraspinatus at the superior aspect of the shoulder in internal rotation (B) shows the plane of acquisition will cross the tendon obliquely. Axial gradient echo image through the supraspinatus at the superior aspect of the shoulder in external rotation (C) shows the plane of acquisition will parallel the tendon

Imaging Planes • •

Axial ➢ Assess subscapularis, biceps tendon Coronal obliques ➢ Parallel to supraspinatus tendon ➢ Assess all tendons Sagittal oblique (FSE T2) ➢ 900 to coronals ➢ Assess all tendons

Figure 4-25-4

Plane of Scan [Figure 4-25-3] •

Rotator Cuff • •

Dynamic stabilizer ➢ Complex coordination Five layers histologically Components ➢ SITS muscles ➢ Rotator cuff interval ❖ Coracohumeral ligament ➢ Long head biceps tendon

Coronal oblique T1 weighted image through the infraspinatus with corresponding gross anatomy

Figure 4-25-5

Coronal Anatomy [Figures 4-25-4 to 4-25-6] Sagittal Anatomy [Figure 4-25-7 Axial Anatomy

Figure 4-25-6

[Figure 4-25-8]

Coronal oblique T1 weighted image through the supraspinatus with corresponding gross anatomy

Coronal oblique T1 weighted image through the subscapularis with corresponding gross anatomy MRI of the Rotator Cuff

926

Musculoskeletal Radiology

Figure 4-25-7

Sagittal oblique T2 weighted image at the level of the glenohumeral joint shows the normal rotator cuff muscle anatomy

•

http://rad.usuhs.mil/rad/anatomy/shoulder/intro.html

• • • • • •

Impingement Overuse Aging Chronic inflammatory disease Acute trauma Instability

Normal Anatomy Figure 4-25-8

Rotator Cuff Tear

•

Pathogenesis RCT: Neer • • •

Stage I (<25 y/o) ➢ Edema/ hemorrhage Stage II (25-40 y/o) ➢ Fibrosis/ thickening Stage III (>40 y/o) ➢ Partial/ Complete Tear

Axial gradient echo image shows the normal subscapularis tendon anteriorally. The long head biceps tendon is normally situated in the bicipital groove

Figure 4-25-9

Impingement Syndrome

•

Clinical - not radiologic diagnosis ➢ Pain with abduction and external rotation ➢ Pain with elevation and internal rotation (Neer impingement sign) Mechanical causes ➢ Acromial shape, position ➢ AC joint osteophyte ➢ Thick coracoacromial ligament ➢ Instability

Impingement [Figure 4-25-9] •

Acromial Variation

• • •

Shape ➢ Type I ➢ Type II ➢ Type III Lateral Downsloping Anterior Downsloping Os acromiale

• • • • •

Increase in number increases risk of tear Type I - flat Type II – curved Type III – hooked Assess on sagittal images

Acromial Variation

Musculoskeletal Radiology

With abduction, flexion and internal rotation, the rotator cuff may impinge on the coracoacromial arch

927

MRI of the Rotator Cuff

• • •

Os Acromiale [Figures 4-25-10]

•

Figure 4-25-10

Increased risk of Cuff Tear Best seen on axial images Injury to Syndesmosis

Rotator Cuff Tear Types •

• •

Full thickness ➢ Communication between joint space and SA bursa Partial thickness ➢ Partial undersurface ➢ Partial Bursal surface ➢ Intrasubstance ➢ “Rim rent” Myotendinous Coronal oblique T1 weighted image posterior to the acromioclavicular joint (A) shows the syndesmosis of the accessory ossicle. Coronal oblique T1 weighted image at the acromioclavicular joint (B) shows the AC joint and supraspinatus tendon. The os acromiale is best seen on the axial image (C ) at the level of the AC joint

Rotator Cuff Tear •

Primary ➢ Increased signal in tendon ➢ Interruption of tendon Secondary ➢ Retraction of musculotendinous junction ➢ Obliteration of subacromial bursal fat plane ➢ Fluid in subacromial bursa ➢ Atrophy of muscles ➢ Fluid in muscle belly

Figure 4-25-11

Increased Signal within Tendon: Short TE images • • • • • • •

Magic angle Connective tissue between fascicles Tendon overlap (internal rotation) Degeneration Tear Partial volume Injection

• • •

Mild degeneration – Low Magic angle Severe degeneration – Intermediate Partial Tear Tear – High

Signal within Tendon: Long TE images

Axial gradient echo image at the superior aspect of the humeral head (A) shows the anterior aspect of infraspinatus tendon overlapping (lateral) the posterior aspect of the supraspinatus tendon (medial). Coronal T1 weighted image at the level of infra and supraspinatus tendon overlap shows normal high signal within the junction of the two tendons

Tendon Overlap [Figure 4-25-11] • • •

Partial Tears

• •

Figure 4-25-12

Twice as common as full thickness Intrasubstance – most common Bursal Surface – least common ➢ Poor response to conservative Rx Increased detection ➢ Contrast ➢ ABER Significant if >50% of tendon thickness

Coronal T2 weighted image through the supraspinatus tendon (left) shows a deep partial undersurface tear. Coronal T2 weighted image through the supraspinatus tendon of a different patient (right) shows fluid signal interrupting the articular surface of the supraspinatus tendon but the bursal surface is intact indicating an undersurface tear. Both patients have SLAP tears of the superior labrum

Partial Undersurface [Figure 4-25-12]

MRI of the Rotator Cuff

928

Musculoskeletal Radiology

Intrasubstance vs Partial US: Value of ABER [Figure 4-25-13] • • •

Figure 4-25-13

Rim Rent Tear [Figure 4-25-14]

• •

Seen in young patients Usually anterior Intrasubstance vs. partial undersurface

Subscapularis Tears [Figure 4-25-15] • • • • •

T1 fat saturated image of the shoulder following indirect arthrography in the ABER position (left) shows the undersurface of the infraspinatus tendon is intact. The conventional coronal oblique T1 weighted image through infraspinatus tendon was suggested an undersurface tear

Abnormal lift-off test on PE Uncommon – 2% of all tears ➢ LHBT dislocation-49% Look for on axial and anterior coronals ➢ Sagittals provide more clues Devastating to surgeon if missed Easy to miss on arthroscopy

Figure 4-25-14

Long Head of Biceps Tendon

•

Abnormality frequently associated with RCT Medial Dislocation ➢ Abnormal bicipital groove ➢ Chronic impingement ➢ Usually extra-articular ➢ Intra-articular with/without subscapularis tendon injury ➢ Associated with degeneration of tendon

Long Head of Biceps Tendon •

Tendonitis ➢ Increased signal in tendon ➢ Thickening of tendon Rupture of tendon ➢ Intracapsular ➢ Extracapsular ➢ Ovoid/ heart shaped – partial tear

Coronal oblique fat sat T2 weighted image shows horizontally oriented increased signal in the insertional portion of the posterior supraspinatus tendon consistent with a tear but it is difficult to determine whether the tear is an undersurface or intrasubstance defect

Figure 4-25-15

Subscapularis Insertion [Figure 4-25-16]

Figure 4-25-16 Sagittal T2 weighted image at the level of the lesser tuberosity shows a focus of high signal in the subscapularis tendon representing a partial undersurface tear. The tear is also seen on the axial T1 weighted gradient echo image following indirect arthrography (right) but is easier to see on the sagittal image

Axial gradient echo image through the subscapularis tendon (A) that appears to only insert on the lesser tuberosity on this image. However, photo of a gross specimen and a photomicrograph through the insertion of the subscapularis show that the tendon inserts on both the lesser and greater tuberosity. The transverse ligament covering the bicipital groove is not actually a ligament but represents the portion of the subscapularis tendon that inserts on the greater tuberosity [Courtesy Tim Sanders, M.D.]

Musculoskeletal Radiology

929

MRI of the Rotator Cuff

• • •

Rotator Cuff Atrophy

•

Figure 4-25-17

Rotator cuff tear Acute brachial neuritis Nerve entrapment syndromes ➢ Suprascapular nerve entrapment ➢ Quadrilateral space syndrome

Suprascapular Nerve Entrapment

• •

Suprascapular Notch ➢ Supraspinatus/ Infraspinatus innervation Spinoglenoid Notch ➢ Infraspinatus innervation Atrophy of SSM and ISM Look for mass in region of suprascapular notch

•

Infraspinatus atrophy

• • • • •

Axillary N. Compression Fibrous band Pain, paresthesia Atrophy of deltoid and/or teres minor Weightlifters

•

Spinoglenoid Notch Entrapment [Figure 4-25-17] Quadrilateral Space Syndrome [Figure 4-25-18]

Figure 4-25-18

Coronal oblique fat saturated T2 weighted image shows a paralabral cyst extending from a posterior superior labral tear into the spinoglenoid notch. A clinical photo (B) in the same patient shows marked atrophy of the right infraspinatus muscle belly as indicated by the loss of soft tissue inferior to the scapular spine. Sagittal T2 image (C) medial to the spinoglenoid notch shows atrophy and denervation edema in the infraspinatus muscle belly The quadrilateral space is bounded by teres minor, teres major, long head triceps and the humerus

• • •

Clinical Mimics of Rotator Cuff Tear

• •

Calcific tendonitis Adhesive capsulitis Subacromial bursitis

Calcific Tendonitis • • •

Rotator cuff most common site Primary or secondary disorder? HADD in tendon Concretion – low T1 and T2 Variable surrounding edema May erode cortex/ invade marrow

MRI of the Rotator Cuff

930

Musculoskeletal Radiology

• • •

Adhesive Capsulitis [Figure 4-25-19]

• •

Figure 4-25-19

Clinical mimic of cuff tear Capsule thickened Abnormal enhancement — IV gad

Pectoralis Major Tear

• • •

Weight-lifters Sternal and clavicular heads ➢ Sternal head superior on humerus ➢ Clavicular head inferior Sternal head tear most common Use torso coil and coronal obliques Myotendinous vs. tendon

Myotendinous Tear of Pectoralis [Figure 4-25-20] Figure 4-25-20

Axial T1 weighted gradient echo image (A) shows marked enhancement in the anterior inferior capsule following IV contrast administration indicating adhesive capsulitis in the atraumatic setting

Coronal oblique T1 (left) and fat sat T2 (right) weighted images through the pectoralis major muscle show hemorrhage associated with a myotendinous injury. The distal tendon is intact

• • •

Radiologic Report

• •

Acromion-os acromiale Tendon – normal, tendinosis, tear Size and location of tear ➢ Massive>5cm Partial thickness tear ➢ > or < 50% thickness of tendon Retraction/Muscle atrophy

References 1. 2.

Zlatkin MB, Needell SD, Hoffman C. MRI of the Shoulder, 2nd Edition. Lippincott Williams & Wilkins, Philadelphia, PA. 2003. Steinbach LS, Peterfy CG, Tirman PFJ, Feller JF eds. Shoulder Magnetic Resonance Imaging. Lippincott Williams & Wilkins, Philadelphia, PA. September 1998

Musculoskeletal Radiology

931

MRI of the Rotator Cuff

MR Arthrography of Glenohumeral Instability Timothy G. Sanders, MD •

Glenohumeral Joint Intrinsically Unstable joint ➢ Shallow glenoid fossa ➢ Large articular surface of the humeral head • Static Stabilizers ➢ Joint capsule ➢ Glenohumeral Ligaments ➢ Glenoid labrum • Dynamic Stabilizers ➢ Rotator cuff ➢ Long Head of the Biceps Tendon

Figure 4-26-1

•

Classification TUBS ➢ Traumatic ➢ Unidirectional ➢ Bankart ➢ Surgery • AMBRI ➢ Atraumatic ➢ Multidirectional ➢ Bilateral ➢ Rehabilitation ➢ Inferior Capsular Shift

Superior glenohumeral ligament

Figure 4-26-2

• •

Multidirectional Instability AMBRI Patient Causes of Multidirectional Instability ➢ Hypermobility or Laxity ➢ Stretching or Overuse of Support Structures • MR Imaging not usually Required ➢ MR Findings Nonspecific ➢ MR Useful if Direction Unknown to Rule Out Conventional Causes • • • • •

Anterior Stabilizers

• •

Middle glenohumeral ligament

Labrum Glenohumeral Ligaments Capsule Subscapularis Muscle Most Important Anterior Stabilizer: Inferior Glenohumeral Labroligamentous complex ➢ Anteroinferior labrum ➢ Anterior Band of the Inferior Glenohumeral Ligament

Figure 4-26-3

Normal Labrum

• •

Anterior and Posterior Labrum best seen in the Axial Plane LABRUM: ➢ Dark on all Pulse Sequences ➢ May be triangular, rounded, or blunted

Normal Superior Labrum Seen Best in the Coronal Plane Superior Labrum: ➢ Dark on all pulse sequences ➢ Triangular ➢ Extends off of Superior Glenoid

MR Arthrography of Glenohumeral Instability

Inferior glenohumeral ligament

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• • •

Superior Glenohumeral Ligament [Figure 4-26-1]

•

Prevents inferior subluxation with arm in 0º abduction Courses from superior glenoid tubercle to lesser tuberosity Parallels Coracoid process

Figure 4-26-4

Middle Glenohumeral Ligament [Figure 4-26-2] • • • • • •

Prevents external rotation of humeral head when arm is between 45º and 60º of abduction Originates at superior glenoid tubercle Courses obliquely superficial to the anterior labrum Blends with the deep fibers of subscapularis Most variable of the glenohumeral ligaments

Inferior Glenohumeral Ligament [Figure 4-26-3] • • •

Most important GHL Prevents anterior subluxation with arm in full abduction and external rotation Extends from anterior inferior labrum to humeral neck Lax with arm in neutral position Redundant when the arm is in neutral position

• • •

Scan plane along the long axis of the humeral shaft Coronal scout with arm in ABER position Stretches anterior band of IGHL

Left: Scan plane for the ABER view Right: Normal ABER view

Figure 4-26-5

Scout Position and Scan Plane for ABER [Figure 4-26-4]

•

Normal Anatomic Variants 1. Cartilage Undermining ➢ Articular cartilage hyaline- intermediate signal intensity ➢ Labrum- fibrocartilage- low signal intensity ➢ Smooth, tapering ➢ Does not Extend Completely Beneath Labrum • 2. Sublabral Foramen (Hole) ➢ Occurs only in the anterosuperior quadrant ➢ Complete detachment of the labrum from the glenoid • 3. Sublabral Recess [Figure 4-26-5] ➢ Smooth, tapering ➢ Extends toward the glenoid ➢ No signal extends into the black triangle of the superior labrum ➢ Can mimic a SLAP tear • Buford complex [Figure 4-26-6] ➢ 1.5 % of patients ➢ Can mimic anterior labral tear ➢ Thick cord-like MGHL ➢ Absent or diminutive anterior-superior labrum • •

Sublabral recess

Figure 4-26-6

Anterior Instability 95% of all dislocations Mechanism ➢ Fall on outstretched arm ➢ Abduction and external rotation

Bufford complex Musculoskeletal Radiology

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MR Arthrography of Glenohumeral Instability

•

Bankart Lesions [Figure 4-26-7] • • • •

Figure 4-26-7

The most common injury following anterior dislocation First-time dislocators under 35 y.o. Anterior labro-ligamentous avulsion with disruption of the medial scapular periosteum

Osseous Bankart Lesion [Figure 4-26-8]

•

Fracture of inferior glenoid Disruption of the cortex of the anteroinferior glenoid

Hill-Sachs Lesion • • • • •

Bankart lesion

Results from impaction of humeral head against anterior-inferior glenoid Associated with Bankart lesion Normally: Top 3 images round Hill-Sachs: flattening or concavity Acute: + Edema

Figure 4-26-8

Double Axillary Pouch Sign [Figure 4-26-9]

• • • • •

Double axillary pouch: small collection of contrast in inferior labrum seen on coronal images

Perthes Lesion Bankart variation (non-displaced) Labro-ligamentous disruption Medial scapular periosteum intact May resynovialize in place Best detected on ABER view

Osseous Bankart

Figure 4-26-9

Nondisplaced Tear Anteroinferior Labrum Perthes Lesion [Figure 4-26-10] • • • •

ALPSA Lesion

•

Anterior labroligamentous periosteal sleeve avulsion Intact medial periosteal periosteum Medialized Bankart lesion Surgical repair technique differs from Bankart Double axillary pouch

Medialized Bankart Lesion [Figure 4-26-11]

• •

ALPSA- Anterior labroligamentous periosteal sleeve avulsion

Figure 4-26-10

Chronic Medialized Bankart Lesion •

Labrum scars down medially Scar tissue mounds up covering medialized labrum and resynovializes Treatment: complete Bankart and reconstruction

Perthes Lesion

Figure 4-26-11

ALPSA lesion MR Arthrography of Glenohumeral Instability

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•

Axillary Nerve Neuropraxy [Figure 4-26-12]

Figure 4-26-12

Axillary nerve can be stretched at time of anterior dislocation resulting in denervation atrophy: Deltoid and Infraspinatus muscles • Denervation atropy: ➢ Acute: edema (high signal on T2) ➢ Chronic: fatty (high signal on T1) • • • • •

Clinical presentation can be confusing Tear supraspinatus tendon Fracture greater tuberosity Avulse subscapularis and anterior capsule from the humerus MRI can play pivotal role in directing patient therapy

• • •

First time dislocation over age 35: Bankart lesion uncommon Rotator cuff tendon becomes the weak link

•

Avulsion fracture of the greater tuberosity

First Time Dislocation Over Age 35

Tear of the Supraspinatus Tendon Denervation atrophy of Deltoid muscle

First Time Dislocation Over Age 35 •

Figure 4-26-13

Greater Tuberosity Fracture • • • •

Avulsion of the greater tuberosity is often occult radiographically Can mimic RCT Treated conservatively MRI can accurately distinguish

Avulsion of Subscapularis • •

Subscapularis muscle can avulse off of lesser tuberosity Associated with dislocation of the biceps tendon Seen best in axial plane

• •

Disruption of subscapularis at musculotendinous junction Requires surgical repair

Humeral Avulsion of the Glenohumeral Ligament (HAGL) Lesion

Disruption of Subscapularis

• • • •

Figure 4-26-14

Hagl Lesion Humeral avulsion of the glenohumeral ligament Results from dislocation No age predilection MR findings: contrast extravasation from joint capsule/ avulsion of subscapularis

• • • • •

Inferior GHL can disrupt anywhere along course Humeral attachment/ mid substance Difficult to detect with scope Cause of failed repairs Can present on MRI as avulsion of subscapularis muscle

• • • • •

2% - 4% of all traumatic dislocations 20% - 25% of shoulder instability cases in active duty military population Adduction with internal rotation Seizure, electrocution, weight lifting, swimming, lineman blocking “Reverse” Hill Sachs, Bankart

• • •

Repetitive microtrauma: non-displaced posterior labral tear Reverse Bankart Reverse Hill Sachs

Hagl Lesion [Figure 4-26-13] Reverse Bankart and Hill Sachs Lesion

Posterior Instability

Posterior Instability [Figure 4-26-14]

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MR Arthrography of Glenohumeral Instability

• • • •

Glenolabral articular disruption Forced adduction injury (humeral head impacts the glenoid fossa) Clinically a stable lesion Partial tear anteroinferior labrum / articular cartilage injury

• • • •

Non displaced tear anteroinferior labrum Best seen on ABER Articular Cartilage Injury Best seen on axial or coronal

Glad Lesion [Figure 4-26-15]

Figure 4-26-15

Glad Lesion [Figure 4-26-15]

• •

Posterior Superior Glenoid Impingement • • •

Also known as “Internal Impingement” Undersurface tearing of posterior rotator cuff (posterior SST or IST) Impingement between posterior labrum and greater tuberosity Throwing athletes- posterior shoulder pain Associated with anterior instability

[Figure 4-26-16]

Posterior Superior Glenoid Impingement • • •

Undersurface of posterior rotator cuff impinged between the greater tuberosity and the posterosuperior labrum Seen best on ABER view Glenolabral Articular Disruption (GLAD) Lesion

Posterior Superior Glenoid Impingement MR Findings: ➢ Undersurface tear of posterior rotator cuff ➢ Degenerative changes of posterosuperior labrum ➢ Cystic change in greater tuberosity ➢ Internal impingement seen on ABER view

Figure 4-26-16

[Figure 4-26-17] • Scarring and thickening of the posterior capsule and has recently been described as a source of potential pain in throwing athletes • MR imaging demonstrates thickening of the posterior capsule

Glenohumeral Internal Rotation Deficit (GIRD)

•

“SLAP” Tears The superior labrum, anterior-to-posterior lesion, can include biceps tendon • Mechanism ➢ Fall on outstretched arm ➢ Repetitive overhead activity (throwing, swimming) • Symptoms: pain with overhead activity, catching, popping sensation • •

Important factors to observe

Posterior Superior Glenoid Impingement

Figure 4-26-17

Abnormal signal in superior labrum Extent of lesion ➢ Posterior labrum ➢ Anteroinferior quadrant • Biceps involvement • Type of SLAP tear

Glenohumeral Internal Rotation Deficit (GIRD) MR Arthrography of Glenohumeral Instability

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•

SLAP Lesion

Figure 4-26-18

Type I [Figure 4-26-18] ➢ Fraying and degeneration; but labrum firmly attached • Type II [Figure 4-26-19] ➢ Fraying of labrum and superior labrum avulsed from glenoid ➢ Signal extends into the triangle of the superior labrum • Type III [Figure 4-26-20] ➢ Bucket-handle tear of the superior labrum; biceps tendon remains intact ➢ On MR imaging: fragment seen displaced into superior joint space • Type 4 [Figure 4-26-21] ➢ Bucket-handle tear of superior labrum involves biceps anchor ➢ Biceps involvement best seen on axial or sagittal images

SLAP Type I: Fraying of the undersurface of labrum

Figure 4-26-19

Figure 4-26-20

SLAP Type II: Avulsion of labrum from glenoid

Figure 4-26-21

SLAP Type III: Bucket-handle tear of the superior labrum

•

Pitfalls: SLAP Tears 1. Sublabral recess ➢ Smooth, tapering ➢ No signal in superior labrum ➢ SLAP tear: any signal extending into black triangle • 2. Sublabral recess: axial images ➢ Smooth linear collection of contrast ➢ SLAP on axial images: irregular contrast collection ➢ Sublabral recess: coronal images ➢ No displacement of superior labrum ➢ Type 2 SLAP tear ➢ Labrum pulled away from glenoid •

SLAP Type IV: Bucket-handle tear with involvement of biceps anchor

Paralabral Cyst • • • • • • • • •

High association with labral tears and GH joint instability Superior labral cyst: SLAP tears Posterior labral cyst: posterior labral tears Labral tear may resynovialize Can result in shoulder pain and adjacent nerve entrapment DDX: Intramuscular cysts of rotator cuff associated with PT tear of the cuff tendon

Figure 4-26-22

Paralabral Cysts [Figure 4-26-22] SLAP tear with superior paralabral cyst Suprascapular notch Suprascapular nerve entrapment Denervation edema: supraspinatus and infraspinatus muscles

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Left: SLAP tear with paralabral cyst Right: Denervation edema within supraspinatus and infraspinatus muscles

MR Arthrography of Glenohumeral Instability

• • • • •

Paralabral Cysts

• • • • • •

Figure 4-26-23

Posterior paralabral cysts Extend into spinoglenoid notch Entrapment of suprascapular nerve Persistent shoulder pain for 3 years Small anterior labral tear with small adjacent paralabral cyst

Paralabral cysts

• •

Small inferior labral cyst Teres Minor normal 3 years later; persistent pain Paralabral cyst larger Axillary nerve entrapment Atrophy Teres Minor

Left: Direct arthroscopic repair of Bankart lesion. Suture anchors at the 3, 4, and 5 o’clock positions Right: Sagittal MR image shows location of suture anchors

Figure 4-26-24

Labral Repair: Surgical Approach Direct repair of labral and capsular lesions Indirect repairs ➢ Staple capsulorapphy (Du Toit & Roux) ➢ Subscapularis manipulation to tighten anterior capsule (Putti Platt/ Magnuson Stack) ➢ Movement of the coracoid process (Bristow procedure)

•

Direct Repairs Arthroscopic/ open (deltopectoral interval) ➢ Suture anchors 3-,4-,5-, o’clock position • Capsulorapphy (open/ arthroscopic) ➢ Staple redundant capsule ➢ Done in conjunction with direct repair ➢ High failure rate if done as isolated procedure • Osseous Bankart

Recurrent displace Bankart lesion

MR Findings of Bankart Repair •

Figure 4-26-25

[Figure 4-26-23]

Suture anchor artifact from repair may obscure visualization

MR Findings of Failed Bankart Repair • • •

[Figure 4-26-24]

Recurrent displaced anterior labrum

Failed Bankart Repair [Figure 4-26-25] Missed HAGL lesion In one series– up to 30% of failed repairs

Left: Missed HAGL lesion. IGHL avulsed from humeral neck Center: Missed HAGL lesion. IGHL avulsed from humeral neck Right: HAGL lesion

• •

Displaced fragment anterosuperior labrum Osteochondral defect anterosuperior glenoid

• •

Fraying and irregularity of superior labrum; no displaced fragment Partial thickness articular surface tear rotator cuff

Recurrent SLAP Following Repair [Figure 4-26-26]

Figure 4-26-26

5 Months Following SLAP Repair: Recurrent Pain

Recurrent SLAP tear with a displaced fragment

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• •

Multidirectional Instability [Figure 4-26-27]

•

Figure 4-26-27

Treated first with rehabilitation Surgery ➢ Inferior capsular shift/plication ➢ Decrease volume of GHJ anteriorly, inferiorly, posteriorly ➢ MR capsular thickening

Hardware Complication [Figure 4-26-28] Proud suture anchor

Synovitis - Prior SA Decompression and Rotator Cuff Debridement: Recurrent Pain • •

•

Synovitis: 4 mm adhesive capsulitis Normal postop capsule: ➢ 2-4 mm after surgical procedure ➢ Thickened and nodular capsule

Postop Infection [Figure 4-26-29] • •

Infectious versus reactive synovitis difficult to differentiate with imaging Thickened enhancing capsule; effusion/ joint destruction/ cartilage loss/ cysts, erosions

. Normal postoperative MR appearance following inferior capsular shift. Thickened capsule

Acute Chondrolysis of the Glenohumeral Joint [Figure 4-26-30] • • • •

Rapid onset chondrolysis refers to a condition in which widespread chondrocyte death occurs within a joint over a relatively short period of time Devastating complication reported following arthroscopy and reconstruction of the GHJ in young individuals Rapid onset pain Marked loss of motion Treatment supportive; eventually total joint arthroplasty

Figure 4-26-28

Chondrolysis Shoulder: Proposed Etiologies • • • • • • •

Damage from use of thermal probe for capsular shrinkage Marcaine pump Left: MR appearance of a proud suture anchor Unknown infectious agent Right: CT appearance of a proud suture anchor Bioabsorbable material Mechanical trauma at time of arthroscopy Chemical trauma to the chondrocytes Event during arthroscopy that triggers an immune response and subsequent Figure 4-26-29 migration of inflammatory cells into the GH joint

Figure 4-26-30

Postoperative infection Acute Chondrolysis of the Glenohumeral Joint following Shoulder Arthroscopy

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MR Arthrography of Glenohumeral Instability

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19.

Bankart ASB: Recurrent or habitual dislocation of the shoulder-joint. Br J Surg 26: 23-29, 1938 Beltran J, Rosenberg ZS, Chandnani VP, et al: Glenohumeral instability: Evaluation with MR arthrography. Radiographics 17: 657-673, 1997 Chandnani VP, Gagliardi JA, Murnane TG, et al: Glenohumeral ligaments and shoulder capsular mechanism: Evaluation with MR arthrography. Rad 196: 27-32, 1995 Cvitanic O, Tirman PFJ, Feller JF, et al: Using abduction and external rotation of the shoulder to increase the sensitivity of MR arthrography in revealing tears of the anterior glenoid labrum. AJR 169 837-844, 1997 Kaplan PA, Bryans KC, Davick JP, et al: MR imaging of the normal shoulder: Variants and pitfalls. Rad 184: 519524, 1992 Linker CS, Helms CA, Fritz RC: Quadrilateral space syndrome: Findings at MR imaging. Rad 188: 675-676, 1993 Neviaser RJ, Neviaser TJ, Neviaser JS: Concurrent rupture of the rotator cuff and anterior dislocation of the shoulder in the older patient. JBJS 70-A: 1308-1311, 1988 Neviaser TJ: The anterior labroligamentous periosteal sleeve avulsion lesion: A cause of anterior instability of the shoulder. Arthroscopy 9: 17-21, 1993 Neviaser TJ: The GLAD lesion: Another cause of anterior shoulder pain. Arthroscopy 9: 22-23, 1993 Palmer WE, Brown JH, Rosenthal DI: Labral-Ligamentous complex of the shoulder: Evaluation with MR arthrography. Rad 190: 645-651, 1994 Petersilge CA, Witte DH, Sewell BO, et al: Normal regional anatomy of the shoulder. MRI Clin North Am 5: 667681, 1997 Sanders TG, Tirman PFJ, Linares R: The Glenolabral articular disruption lesion: MR arthrography with arthroscopic correlation. AJR 172: 171-175, 1999 Schweitzer ME: MR arthrography of the labral-ligamentous complex of the shoulder. Rad 190: 641-643, 1994 Synder SJ, Karzel RP, Pizzo WD, et al: SLAP lesions of the shoulder. Arthroscopy 6: 274-279, 1990 Tirman PFJ, Bost FW, Garvin GJ, et al: Posterosuperior glenoid impingement of the shoulder: Findings at MR arthrography and MR arthrography with arthroscopic correlation. Rad 193: 431-436, 1994 Tirman PFJ, Feller JF, Jansen DL, et al: Association of glenoid labral cysts with labral tears and glenohumeral instability: Radiographic findings and clinical significance. Rad 190: 653-658, 1994 Tirman PFJ, Feller JF, Palmer WE, et al: The Buford complex—A variation of normal shoulder anatomy: MR arthrographic imaging features. AJR 166: 869-873, 1996 Tirman PFJ, Steinbach LS, Feller, FJ: Humeral avulsion of the anterior shoulder stabilizing structures after anterior shoulder dislocation: demonstration by MRI and MR arthrography. Skeletal Radiol 25: 743-748, 1996 Wolf EM, Cheng JC, Dickson K: Humeral avulsion of glenohumeral ligaments as a cause of anterior shoulder instability. Arthroscopy 11: 600-607, 1995

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Imaging of Upper Extremity Trauma Timothy G. Sanders, MD •

Imaging of Upper Extremity Trauma

Figure 4-27-1

Anatomic locations ➢ Shoulder girdle, humerus, elbow, forearm, wrist and hand • Structures involved ➢ Bones, joints, articular cartilage, tendons, ligaments • Mechanism of injury ➢ Acute trauma, sports related, repetitive stress injury •

Sterno-clavicular Joint Dislocation most common injury ➢ Anterior more common than posterior Grade II separation of AC Joint • Best evaluated with limited CT scan • Normal distal clavicle extends above manubrium- use symmetry as guide • Plain film- 40º cephalic angulation • • • •

Clavicle Fractures Mechanism: indirect trauma- fall on outer prominence of shoulder Most common site of injury is middle third Healing may result in deformity (extensive callous) Distal third fracture must evaluate coracoacromial ligament integrity

Figure 4-27-2

• • •

Acromio-clavicular Joint Injuries Mechanism: fall on outer prominence of shoulder AC joint: weak capsule and inherently unstable Grade I injury ➢ mild strain of AC joint ➢ Ligaments intact; point tenderness over AC joint ➢ X-rays normal ➢ Treatment conservative- recovery is spontaneous • Grade II injury ➢ moderate strain [Figure 4-27-1] ➢ Disrupted AC ligament ➢ CC ligaments intact ➢ X-ray- widening of AC joint; slight uplifting of distal clavicle ➢ Treatment conservative- recovery is spontaneous • Grade III injury ➢ severe [Figure 4-27-2] ➢ Ruptured AC and CC ligaments ➢ Complete AC separation with increased distance between coracoid and clavicle • •

Grade III separation of the left AC joint

Figure 4-27-3

Surgical Repair of AC Joint Separation Internal fixation: 8-10 weeks Until CC ligaments heal

•

Osteolysis of Distal Clavicle [Figure 4-27-3] Post-traumatic osteolysis ➢ Complication of trauma (occurs within 2 months of injury, self limiting) ➢ Repetitive stress (wt. lifters) ➢ X-ray: loss of normal cortical line- distal clavicle • DDX: rheumatoid arthritis, infection, hyperparathyroid

Post-traumatic osteolysis of distal clavicle

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• • • • • • •

Scapular Fractures Mechanism: direct trauma to scapula (MVA) Uncommon fractures Frequently missed on X-ray Intra-articular fracture important clinically Fracture of scapular body- source of considerable pain Fracture of the neck of the scapula Non-articular fractures: clinically insignificant; musculature holds fragments in place; conservative treatment

•

CT- imaging modality of choice for evaluating scapula

• • •

Acromion process fractures Direct trauma Restore active range of motion; if severely comminuted- excise fragments

•

Os Acromiale: unfused acromial ossification center

• •

Coracoid fracture: may occur in conjunction with type III AC separation Treatment conservative

•

Trap shooter’s shoulder

Scapular Fractures Scapular Fractures

Scapular Fractures: Acromion Scapular Fractures: Coracoid Figure 4-27-4

Stress Fracture of Coracoid Process • •

Imaging of Glenohumeral Joint • • • • • • •

• • • • •

Standard AP view is oblique to the GH joint Excellent osseous detail (homogeneous distribution of soft tissues) Glenohumeral “True” AP View (Grashey view) Beam tilted 45° laterally Evaluate GH joint, subluxation, loss of articular cartilage Less value for fractures of surrounding structures Axillary view ➢ Evaluate for subluxation/ dislocation; fractures of ant/ post glenoid West Point View: variant of the Axillary View ➢ Improves visualization of the anterior glenoid (Bankart lesion) Scapular “Y” View ➢ Image along plane of scapula; 60° relative to the AP view ➢ Easily acquired in setting of acute trauma ➢ Evaluate for ant/ post dislocation ➢ Poor evaluation of the osseous structures

Figure 4-27-5

Anterior Dislocation [Figure 4-27-4]

• •

AP view of the shoulder demonstrates anterior dislocation of the humeral head

Mechanism: fall on outstretched arm X-ray: humeral head displaced anterior and medial Associated lesions Under 35 y.o. Hill-Sachs defect; Bankart lesion or variant Over 35 y.o. 1. Avulsion fracture greater tuberosity 2. RCT 3. Subscapularis tear

Anterior Dislocation Axillary view Scapular “Y” view

Lesions Associated with Anterior Dislocation •

[Figure 4-27-5]

Occurs secondary to impaction of humeral head against inferior glenoid rim

Imaging of Upper Extremity Trauma

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Hill-Sachs deformity

Musculoskeletal Radiology

•

Lesions Associated with Anterior Dislocation • • • •

Mechanism: results from impaction of humeral head against inferior glenoid rim Osseous Bankart lesion Fibrous Bankart lesion best evaluated with MR

Figure 4-27-6

Trauma [Figure 4-27-6]

• •

24 y.o. female: persistent painful shoulder after skiing accident Radiographically occult humeral head fractures best evaluated with MR imaging

Posterior Instability • • •

2% - 4% of all traumatic dislocations 20% - 25% of shoulder instability cases in active duty military population Adduction with internal rotation Seizure, electrocution, weight lifting, swimming, lineman blocking “Reverse” Hill-Sachs, Bankart

• • • •

Dislocates straight posterior Sometimes difficult to detect on AP view: 50% missed Locked in internal rotation; most reliable sign Very obvious on axillary view

Posterior Dislocation [Figure 4-27-7]

• •

MR appearance of a radiographically occult fracture of the greater tuberosity

“Trough” Sign • • •

Vertical line of dense cortical bone paralleling the medial cortex of the humerus Results from impaction fracture of the posterior medial aspect of humeral head “Reverse” Hills Sachs Fracture

Figure 4-27-7

“Positive Rim” Sign • •

Widening of the joint is termed the “positive rim sign” Normally the space between the anterior glenoid rim and medial humeral head is <6mm If posterior dislocation suspected- get axillary view

Calcific Tendonitis • • •

HADD: periarticular calcifications: shoulder most common Results from chronic repetitive micro-trauma Easily detected on x-ray; can be subtle on MR imaging

Left: AP view of the shoulder demonstrating posterior dislocation of the humeral head Right: Axillary view demonstrating posterior dislocation of the humeral head

Humeral Head Fractures [Figure 4-27-8]

• • • •

Neer’s Four-segment Classification ➢ Humeral head ➢ Humeral shaft ➢ Greater tuberosity ➢ Lesser tuberosity Fragment: >1 cm displacement or >45° angulation; considered significantly displaced No fragment >1 cm or 45°- considered a non-displaced fracture 1 fragment displaced = 2 part fracture; 2 fragments displaced = 3 part fracture; etc

Figure 4-27-8

Humeral Shaft Fractures

• • •

Mechanism: ➢ Indirect twisting force: spiral fx ➢ Direct force: transverse fx ➢ Usually involve mid-shaft Shaft fractures heal easily Rarely require internal fixation Ball-and-socket joint tolerates some degree of angular and rotational malalignment Neer’s Four-segment Classification

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Imaging of Upper Extremity Trauma

• • •

Humeral Shaft Fractures [Figure 4-27-9]

•

Figure 4-27-9

Common location for pathologic fractures Minor trauma Children: ➢ Unicameral bone cyst (fallen fragment) ➢ Fibrous dysplasia Adults: ➢ Metastatic lesion

• • • •

Radial head: most common fracture of upper limb in young adults Mechanism: fall on outstretched arm Frequently occult; oblique views; joint effusion Treatment: non-displaced- conservative; comminuted- resection

• • • • • • • • •

Supracondylar fracture: Most common elbow fracture in childhood Risk: brachial nerve injury Mechanism: Fall on outstretched arm Posterior fat pad sign Anterior “sail” sign In setting of trauma indicates occult elbow fracture Pathologic fracture through a unicameral bone Anterior humeral line cyst Intersect the middle 1/3 of capitellum on lateral view Subtle supracondylar fracture results in posteriorly displaced capitellum

• • •

Mechanism: fall on outstretched arm- common in child and adult Posterior or posterolateral Fractures (minor): coronoid/ radial head: child- medial epicondyle

Elbow Fractures: Adult

Elbow Fractures: Child [Figure 4-27-10]

Figure 4-27-10

Elbow Dislocations

• •

Complications of Dislocation

• • •

Brachial artery or nerve damage Post-traumatic ossification: stiffness- intra and periarticular adhesions: forms in subperiosteal and capsular hematoma

Posterior Dislocations in Child

•

Associated with medial epicondyle avulsion in 50% of cases Up to 30% become entrapped in the joint following reduction Treatment: open reduction for >1 cm displacement of medial epicondyle Abnormal anterior humeral line

Radiocapitellar Line [Figure 4-27-11]

• •

Line drawn along long axis of radius should intersect capitellum in any projection

Night Stick Fracture •

Mechanism: direct force to forearm (usually middle 1/3) Isolated fracture of the mid-shaft of the ulna: Usually no displacement or angulation Must exclude associated dislocations

Figure 4-27-11

Normal radiocapitellar line Imaging of Upper Extremity Trauma

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• • •

Isolated Fractures Ulna: Monteggia [Figure 4-27-12]

• • •

Figure 4-27-12

Ulnar fracture (usually proximal 1/3); radial head dislocation Mechanism: fall on outstretched arm (forced pronation of forearm) Treatment: ➢ Open adult/closed child ➢ Restrict pronation/ supination

Isolated Fractures Radius: Galeazzi

• •

Radius fracture/ distal radioulnar joint dislocation High incidence non-union, delayed union with closed reduction Tx: ORIF- still a tendency to dislocate after ORIF

Monteggia fracture

Essex-Lopresti [Figure 4-27-13] Comminuted radial head fracture/ DRUJ dislocation or instability Interosseous ligament: ➢ Intact: radial head resection ➢ Disrupted: radial head prosthesis

• • •

Childhood: distal radial buckle fracture Young adults: scaphoid fracture Older adults (>40): Colles fracture

• • •

Most common fracture of distal radius in patient over 40 y.o. FOOSH injury with wrist in dorsiflexion Distal fragment displaced dorsally

• • • • • •

A- Extra-articular fx radius B- (A) + ulnar styloid fx C- Intra-articular fx distal radius D- (C) + ulnar styloid fx E- Comminuted fxs of radiocarpal and radioulnar joints F- (E) + ulnar styloid fx

• • •

Volar angulation of distal fragment Younger patient High energy trauma with wrist in volar flexion

• • • •

Longitudinal shear injury of the distal radius Barton: Dorsal rim - Reverse Barton: Volar rim Transverse load with shearing forces Often requires internal fixation: unstable fracture

•

Intra-articular fracture of the radial styloid

Fall on Outstretched Hand

Figure 4-27-13

Colles Fracture

Colles Fracture Classification

Smith Fracture (Reverse Colles)

Comminuted fracture of the radial head

Barton Fracture (Reverse)

Radial Styloid Hutchinson’s/ Chauffeur’s Fracture • • •

Distal Radial Fracture in Childhood Transverse metaphyseal fracture Most common forearm fracture ages 4-10 y.o. May be complete or incomplete ➢ Torus/ Buckle fracture

Evaluation of Suspected Scaphoid Fracture Snuff Box Tenderness • 1. Additional radiographic views ➢ Oblique view ➢ Scaphoid view • 2. Cross-sectional imaging ➢ MRI ➢ CT ➢ Bone scan

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Top: MR imaging demonstrating an intact interosseous membrane of the forearm Bottom: MR imaging demonstrating disruption of the interosseous membrane of the forearm

Imaging of Upper Extremity Trauma

Scaphoid fracture: Complication rate is high • • • • • •

[Figures 4-27-14 and 4-27-15]

Figure 4-27-14

AVN Delayed/ nonunion Osteoarthritis AVN complication of scaphoid fracture Recurrent blood supply Risk factors: delayed diagnosis, displacement, proximal fracture site

Figure 4-27-15 Left: Avascular necrosis of the proximal pole of the scaphoid Right: Nonunion of a scaphoid fracture with secondary osteoarthritis

Figure 4-27-16

Recurrent blood supply to the scaphoid bone

• •

Scapholunate Ligament Disruption [Figure 4-27-16]

•

Normal scapholunate distance = 2mm Gap accentuated with “clenched” fist view

Scapholunate Ligament

•

Wrist MR arthrography can increase detection rate of subtle interosseous ligament disruption

Increased scapholunate distance indicating disruption of the scapholunate ligament

Carpal Dislocations [Figure 4-27-17] Dislocations ➢ Lunate: lunate dislocates in volar direction ➢ Perilunate: capitate dislocated dorsally/ lunate remains normal

Figure 4-27-17

Left: AP view of wrist demonstrates lunate dislocation Center: Lateral view of wrist demonstrate lunate dislocation Right: Perilunate dislocation: posterior capitate dislocation, lunate remains in normal position relative to the radius

Imaging of Upper Extremity Trauma

946

Musculoskeletal Radiology

•

Triquetrum Fracture [Figure 4-27-18]

Figure 4-27-18

• • • •

Shearing forces, dorsal avulsion fracture seen only on the lateral view Point tender over dorsal aspect of wrist Mechanism: fall on out-stretched hand Immobilize in plaster for 3 weeks Associated with lunate, perilunate dislocation

• •

Point tenderness over hook of hamate Direct trauma to volar aspect of wrist; surgical resection of hook

• • • • •

Oblique intra-articular fracture/subluxation of base of 1st MC The large fragment subluxes; smaller fragment maintains position Closed treatment: persistent subluxation/ traumatic arthritis Usually treat with ORIF Bennett “Bad”

Hamate Fracture [Figure 4-27-19]

Fracture of First MC- Bennett Fracture [Figure 4-27-20]

• Deforming forces ➢ Abductor pollicis longus pulls the distal fragment in a proximal/dorsal direction ➢ Adductor pollicis muscle stabilizes the volar ulnar lip of the articular surface in its normal position ➢ Thus results in distraction of the 2 fracture fragments

Bennett Fracture

• • •

Triquetrum fracture

Figure 4-27-19

Fracture of First MC- Rolando Fracture

• •

Comminuted Bennett fracture Dorsal subluxation and a separate dorsal fragment Usually treated closed as pinning does not work well on comminuted fragments Worse prognosis than the Bennett fracture Rolando “Ruined”

• • •

Disrupt Ulnar Collateral Ligament May have small avulsion fracture Pin or ORIF

Skier’s Thumb Injury (Gamekeeper’s thumb)

• •

Hook of Hamate Fracture

Figure 4-27-20

Stress Views [Figure 4-27-21] •

Stener Lesion: MR imaging Interposition of adductor pollicis apponeurosis between torn UCL and base of proximal phalanx Surgical lesion

Figure 4-27-21

Bennett Fracture

Disrupted ulnar collateral ligament

Musculoskeletal Radiology

947

Imaging of Upper Extremity Trauma

• • • •

Sudden resisted flexion of DIP joint Finger jammed or distal tip hit with a ball Avulsion of extensor digitorum tendon ORIF and splint in full extension 6 weeks

• •

Hyperextension avulsion injury MCP or PIP joint

Mallet Finger (Baseball Finger) [Figure 4-27-22]

Figure 4-27-22

Volar Plate Avulsion

Avulsion of Flexor Digitorum: Jersey Finger • • •

[Figure 4-27-23]

Forced hyperextension Avulsion of flexor digitorum profundus Osseous fragment displaced proximally

Figure 4-27-23

Isolated Tendon and Pulley Injuries [Figure 4-27• • •

24]

Rupture of pulley system occurs with forced extension Bowstringing of flexor tendon Common injury in rock climbers Mallet finger: avulsion of extensor digitorum tendon

Phalangeal Dislocations: Coach’s Finger • • • • • •

[Figure 4-27-25]

Usually dorsal; often associated with volar plate injury Simple: closed reduction; Complex: ST entrapment: open reduction

Boxer’s Fracture

• • • •

4th or 5th MC Dorsal angulation Treatment open >35º angulation Jersey Finger: Avulsion

Distal Tuft Fracture

injury of the flexor digitorum Fracture of distal tuft of phalanx profundus Mechanism: crushing injury Ignore the fracture and treat soft tissue injury Watch for complication of osteomyelitis with open fracture/ nail bed injury

Figure 4-27-24

Figure 4-27-25

Coach’s Finger: Interphalangeal dislocation

References 1. 2.

Manaster, BJ. Handbook of Skeletal Radiology, second edition. St Louis, MO: Mosby, 1997: 171-225 Rogers, LF. Radiology of Skeletal Trauma, third edition. Philadelphia, PA, Churchill Livingstone, 2002, 593-929 Rock Climber’s Finger: Flexor pulley disruption

Imaging of Upper Extremity Trauma

948

Musculoskeletal Radiology

Crystal Deposition Diseases and Neuropathic Osteoarthropathy Charles S. Resnik, MD •

Crystal Deposition Diseases • • •

Gouty arthritis ➢ Monosodium urate CPPD crystal deposition disease ➢ Calcium pyrophosphate dihydrate HA crystal deposition disease ➢ Calcium hydroxyapatite

Related Disorders •

Hemochromatosis ➢ Iron deposition Alkaptonuria ➢ Homogentisic acid

• • • •

Monosodium urate crystals Intra-articular, periarticular Acute inflammatory response Chronic granulomatous reaction

• • •

Male: Female = 20 : 1 40–50 years of age (Familial history)

• • • •

Hereditary diseases Myeloproliferative diseases Endocrine disorders Drug therapy

• • • • •

Asymptomatic hyperuricemia Acute gouty arthritis Interval phase Recurrent arthritis Chronic tophaceous gout

• • • • • •

Polyarticular Asymmetric 1st MTP joint (90%) Tarsometatarsal Carpometacarpal Olecranon bursitis

Gout

Gout: Primary (Idiopathic)

Gout: Secondary

Gout: Clinical Stages

Gouty Arthritis

Musculoskeletal Radiology

949

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

• • • • • •

Gouty Arthritis [Figures 4-28-1 to 4-28-3]

Figure 4-28-1

Soft tissue swelling Tophi (calcification rare) Well-defined erosions Overhanging edge Preserved joint space Extra-articular erosions

Figure 4-28-2

Gouty arthritis. Radiograph of the distal interphalangeal joint shows characteristic welldefined erosion of bone with overhanging edge of new bone (arrow). Note also preservation of joint space

Gouty arthritis. A. Pathological specimen shows tophaceous material (star) and sharp interface with adjacent bone (arrow). B. Clinical radiograph shows large well-defined erosions of the first metatarsal head with overhanging edge of new bone (arrow). Note multiple other erosions including the base of the first metatarsal

• • •

Pseudogout Chondrocalcinosis Pyrophosphate arthropathy

• • • •

Usually idiopathic Occasionally hereditary Over 50 years of age Incidence 5% to 34%

Figure 4-28-3

CPPD Crystal Deposition Disease: Terminology

CPPD Crystal Deposition Disease

CPPD Crystal Deposition Disease: Related Disorders • •

Primary hyperparathyroidism Hemochromatosis Gouty arthritis. There are multiple well-defined erosions, particularly at the bases of the metacarpals at the common carpometacarpal joint

CPPD Crystal Deposition Disease: Sites of Calcification • • • • • • •

Fibrocartilage Hyaline cartilage Synovial membrane Joint capsule Ligaments Tendons Bursae

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

950

Musculoskeletal Radiology

• • •

Fibrocartilage Calcification

• • • •

Knee menisci Triangular fibrocartilage Symphysis pubis

Figure 4-28-4

Pyrophosphate Arthropathy Joint space narrowing Bone sclerosis Osteophyte formation Subchondral cysts

Differentiation of Pyrophosphate Arthropathy from Degenerative Joint Disease • • • • • •

Unusual articular distribution Unusual intra-articular distribution Variable osteophyte formation Prominent subchondral cysts Progressive destruction Calcification

CPPD crystal deposition disease. A. Frontal radiograph of the knee shows chondrocalcinosis including meniscal fibrocartilage calcification (white arrows) and hyaline cartilage calcification (gray arrow). B. Lateral radiograph shows complete loss of patellofemoral joint space

CPPD Crystal Deposition Disease: Knee • • • • •

[Figure 4-28-4]

Meniscal calcification Hyaline cartilage calcification Patellofemoral arthropathy Subchondral cysts Osseous bodies

CPPD Crystal Deposition Disease: Wrist and Hand [Figures 4-28-5 and 4-28-6] • • • • •

Figure 4-28-5

Triangular fibrocartilage calcification Radiocarpal arthropathy “Stepladder” configuration (SLAC) Metacarpophalangeal arthropathy Absence of erosions

Figure 4-28-6

CPPD crystal deposition disease. Frontal radiograph of the wrist shows joint space narrowing between the radius and scaphoid and between the lunate and capitate (scapholunate advanced collapse [SLAC]). There is extensive chondrocalcinosis including the triangular fibrocartilage (arrow) as well as a large subchondral cyst in the radius (star)

CPPD crystal deposition disease. Frontal radiograph shows narrowing of the second and third metacarpophalangeal joints with sclerosis, osteophytes, and subchondral cysts. Note hyaline cartilage calcification (green arrow) and probable synovial and capsular calcification (yellow arrows)

Musculoskeletal Radiology

951

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

•

Hemochromatosis •

• • • • • • •

Primary ➢ Increased iron absorption Secondary ➢ Increased iron intake ➢ Multiple blood transfusions

Hemochromatosis: Clinical Findings Onset between 40 and 60 years Men more often than women Bronze pigmentation Cirrhosis Diabetes mellitus Cardiac failure Arthropathy

Figure 4-28-7

Hemochromatosis: Pathologic/Radiologic Findings • • • • • • •

Iron in synovioblasts CPPD crystal deposition Osteoporosis Symphysis pubis calcification Hyaline cartilage calcification Uniform MCP joint involvement Hook-like osteophytes

• • • •

Primary Secondary Periarticular Intra-articular

[Figure 4-28-7]

Hemochromatosis. Frontal radiograph shows narrowing of the second through fifth metacarpophalangeal joints as well as multiple large hook-like osteophytes (arrows)

HA Crystal Deposition Disease

Primary Periarticular HA Crystal Deposition Disease: (Calcific Tendinitis) • • • • • • •

Middle-aged Monoarticular (Asymptomatic) Localized pain Tenderness to palpation Restriction of motion (Fever and malaise)

Primary Periarticular HA Crystal Deposition Disease: (Calcific Tendinitis) [Figure 4-28-8] • • • •

Figure 4-28-8

Soft tissue swelling Poorly defined calcification Sharply defined calcification Resorption of calcification

Intra-Articular Hydroxyapatite Crystal Deposition Disease: Milwaukee Shoulder • • • • • • •

Elderly women Shoulder pain Decreased mobility HA crystal shedding Activated collagenase Neutral protease Tissue destruction

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

Calcific tendinitis. A. Radiograph of the shoulder in external rotation shows poorly defined calcification corresponding to the supraspinatus tendon (arrow). B. Radiograph of the shoulder of a different patient in internal rotation shows sharply defined calcification corresponding to the infraspinatus or teres minor tendon (arrow)

952

Musculoskeletal Radiology

• • • • • •

Amorphous calcification Glenohumeral joint narrowing Subchondral sclerosis Bone destruction Rotator cuff disruption Acromiohumeral abutment

• • • • • • • •

Homogentisic acid oxidase deficiency Onset between 20 and 30 years Bluish-black pigmentation (ochronosis) Dark colored urine Cardiovascular Genitourinary Upper respiratory Articular

• • • • •

Connective tissue pigmentation Fibrocartilage and hyaline cartilage Fibrillation, fragmentation Granulation tissue Osseous proliferation

Milwaukee Shoulder: Radiologic Findings

Alkaptonuria: Clinical Findings

Alkaptonuria: Pathologic Findings

•

Alkaptonuria: Radiologic Findings • • •

Disc calcification ➢ Annulus fibrosus ➢ Diffuse Multilevel disc narrowing Vacuum phenomena Osseous sclerosis

• • • • •

Knees, hips, shoulders Symmetric cartilage loss Osseous sclerosis Collapse and fragmentation Intra-articular bodies

• • • • •

Charcot joint Tabetic arthropathy Neurotrophic joint Neuropathic arthropathy Neuroarthropathy

• • • • • • • •

Diabetes mellitus Alcoholism Syringomyelia Syphilis Leprosy Meningomyelocele Congenital insensitivity to pain Steroid administration (intra-articular)

Alkaptonuria: Radiologic Findings [Figure 4-28-9] Figure 4-28-9

Neuropathic Osteoarthropathy

Neuroarthropathy: Etiologies

Musculoskeletal Radiology

Alkaptonuria. A. Lateral radiograph of the lumbar spine shows uniform loss of disc height and associated bone sclerosis. There is disc calcification bridging all levels anteriorly except for L4-L5, which shows large osteophytes and a vacuum phenomenon. B. Frontal radiograph of the knee shows uniform joint space loss and bone sclerosis

953

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

• • • •

French theory German theory Neurotraumatic Neurovascular

• • • • •

Mitchell (1831) Charcot (1868) Damage to CNS trophic centers Altered bone and joint nutrition Osseous and articular atrophy

• • •

Volkmann, Virchow Insensitivity to pain Recurrent trauma

• • • • •

Eloesser (1917) Posterior sensory nerve section Continued weightbearing Joint destruction Chemical analysis: no atrophy

Neuroarthropathy: Pathogenesis

Figure 4-28-10

Neuroarthropathy: French Theory

Neuroarthropathy: German Theory

Neuroarthropathy: Neurotraumatic Theory

• • • •

Neurally initiated vascular reflex Increased bone blood flow Osteoclastic bone resorption Fracture and repair

• • • •

Swollen, deformed joint Usually painless Detritic synovitis Rapid progression

Neuroarthropathy: Neurovascular Theory

Neuropathic osteoarthropathy. Frontal radiograph of the shoulder shows almost complete resorption of the humeral head except for some osseous debris medially (arrow). Note the extremely sharp margin of the remaining portion of the humeral shaft

Figure 4-28-12

Neuroarthropathy: Clinical/Pathologic Findings

Neuroarthropathy: Radiologic Findings • • • • • • •

[Figures 4-28-10 to 4-28-12]

Destruction (atrophy) Dislocation Disorganization Debris Detritus Density (sclerosis) Distension (effusion)

Figure 4-28-11

Neuropathic osteoarthropathy. Frontal radiograph of the foot shows characteristic destruction, disorganization, and debris around the tarsometatarsal joints

Neuropathic osteoarthropathy. A. Lateral radiograph of the foot shows extensive destruction of the bones of the midfoot with dislocation and disorganization. Note extensive vascular calcification from diabetes. B. Followup lateral radiograph less than one month later shows further bone destruction with almost complete disappearance of the talus and the anterior portion of the calcaneus

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

954

Musculoskeletal Radiology

MRI of the Elbow Mark E. Schweitzer, MD; William B. Morrison, MD •

Figure 4-29-1

Anatomy • •

• • • •

Osseous-radius ulna humerus Ligamentous-medial collateral lateral collateral Musculotendinous ➢ Posterior: triceps ➢ Anterior: biceps, brachialis ➢ Medial: flexor-pronator ➢ Lateral: common extensor Neurovascular

Articular Anatomy

• • •

Capitellum-hemispherical-articulates with radius Trochlea-spool 300 degree arch-articulates with ulna Coronoid fossa-ant/sup to trochlea, small-articulates with coronoid Olecranon fossa 180 degrees-art. with semilunar notch Lesser sigmoid notch- radial side of proximal ulna/PRUJ

Biomechanics

• •

Three articulations: ➢ Ulnar-tochlear ➢ Radiocapitellar ➢ Proximal radioulnar ➢ 0-140 degrees flex/ext ➢ 75 pronation ➢ 80 supination

Effusion and synovitis. Note complex fluid in the joint distending the anterior and posterior fat pads (arrows). The fat pads are intracapsular but extrasynovial

Effusion [Figure 4-29-1]

• • •

Fat pads are intracapsular and extrasynovial DDx: ➢ Fracture ➢ Internal Derangement (e.g., ligament, cartilage) ➢ Arthritis (e.g., RA, OA, septic)

Figure 4-29-2

Cartilage Loss

•

•

Difficult to see directly (cartilage thin) Subchondral marrow edema best sign Phytes-posterior/medial/coronoid ➢ Confirm cart loss / cause impingement Associated with effusion and bodies

OCD [Figure 4-29-2] • • • •

Capitellum- 3rd most common site in body (after ankle, knee) Repetitive microtrauma during valgus (assoc with MCL) Symptoms = pain, locking Sequelae- bodies/OA Staging-fluid/cyst under fragment=loose (unstable)

Musculoskeletal Radiology

955

Unstable OCD of the capitellum. Note contrast extending under osteochondral fragment (arrow) on this MR arthrogram

MRI of the Elbow

• • •

Pitfalls: NL variation

•

Trochlear sulcus Posterior capitellar pseudodefect DDx: location; no underlying edema

Figure 4-29-3

Intraarticular Bodies [Figure 4-29-3] • •

• •

• •

Often adherent to synovium ➢ Intraarticular, not loose Usually begin as cartilage defects and grow ➢ Often from OCDs) Variable signal characteristics ➢ Use GRE (TE>7), tend to bloom In the recesses, usually olecranon / coronoid MR imaging: arthrography, or effusion

Synovial Folds

•

Embryologic remnant Several locations ➢ Posterior/lateral catches ➢ Medial (meniscoid) most common May mimic bodies clinically and on MR

•

Lateral plica syndrome: Posterolateral catching/locking

Synovial Folds • •

Intraarticular body (arrow) surrounded by contrast in the olecranon recess in a patient with posterior impingement

Posterior Impingement • • • •

Part of tennis elbow spectrum Osteophytes- often related to chronic MCL overload, hyperextension (e.g., pitchers) Bodies in olecranon fossa Non-union of old triceps avulsion

Nerve Impingement

•

• • • • • • •

[Figure 4-29-4]

Potential locations of nerve impingement Median/radial nerve ➢ Proximal arcade of Struthers (avian spur) ➢ Impinged by fascicle of lacertus fibrosus ➢ Median nerve branch through pronator teres muscle (pronator syndrome) ➢ Supinator syndrome, radial tunnel syndrome, interosseous syndrome ➢ Look for muscle edema distally Ulnar nerve [Figure 4-29-4] ➢ Cubital tunnel ➢ Focal edema/STS/sublux/mass ➢ Look above and below ➢ Associated with epicondylitis, MCL injury

Figure 4-29-4

Epicondylitis Medial: golfer’s elbow (common flexor origin) Lateral: tennis elbow (common extensor origin) Spectrum from degeneration to partial to completetear Increased T1 signal Increased STIR, T2 signal Linear vs. diffuse Histologically- angioblastic changes/ fibrillar collagen degeneration

MRI of the Elbow

956

Enlargement of the ulnar nerve (arrow) with surrounding soft tissue inflammation in a patient with cubital tunnel syndrome

Musculoskeletal Radiology

• • • • •

Repetitive overload of extensors “Tennis elbow” 35-70 years old Pain/tenderness focally, may radiate proximally Usually extensor carpi radialis

• • • • • • •

Long head: superior glenoid Short head: coracoid Two heads merge distal to the bicipital groove Insertion onto radial tuberosity at elbow Intimate with brachialis Proximal synovial sheath Distally paratenon, bicipitoradialis bursa and lacertus fibrosus

Lateral Epicondylitis [Figure 4-29-5]

Figure 4-29-5

Biceps: Anatomy

•

Biceps Pathophysiology • •

Degeneration ➢ Primary (overuse injury) ➢ Or direct frictional effect Mechanical-pronation leads to impingement between radius and ulna Hypovascular-critical zone distally like rotator cuff

• • •

Common, but rare to image Imaging/ clinically overlap with partial tear, bursitis Very distal at insertion

• • • • •

Attritional Pain No pop, usually no ecchymosis More marrow edema and bursitis Surgery not usually needed unless large

Lateral epicondylitis (tennis elbow) with focal fluid signal (arrow) at the common extensor tendon origin indicating a partial tear

Figure 4-29-6

Biceps Tendinosis

Biceps-Partial Tears

• • • • • •

Biceps Injury: Distal vs Proximal [Figure 4-29-6]

• • •

Both: muscle belly retraction “popeye arm” Fluid dissects around muscle belly Both: sudden snap, arm hematoma Distal young/ proximal older Distal sports/ proximal – chronic impingement, RCT Proximal: surgery uncommon (two heads), except for repair of cuff, resection of spur Distal: surgery common Associated marrow edema Associated bursitis

• • • • •

Distal biceps lacks a sheath Apparent fluid around is bursal Close to insertion Ddx; vessel Assoc with biceps tears (esp partial), RA, mechanical –maybe 1st

Complete distal biceps tendon tear. Note end of retracted tendon (arrow) with muscle bunched up proximally resulting in a “popeye” arm

Bicipital Radialis Bursitis

Musculoskeletal Radiology

957

MRI of the Elbow

• •

Triceps Injuries • • • • • • • • •

Fairly uncommon Spectrum tendonitis (posterior tennis elbow/ posterior impingement), snapping, to tear Risk factors: steroids, SLE, CRF, RA, gout Within 2–3 cm of insertion, usually at Associated olecranon bursitis Associated soft tissue edema Look for avulsion fx

Figure 4-29-7

Olecranon Bursitis [Figure 4-29-7]

• • •

Anatomic bursa Normally no fluid visible Bursitis: Fluid, loss of subQ fat adjacent to olecranon Causes ➢ Trauma ➢ RA ➢ Gout ➢ Infection

Muscle Disorders

• • • •

Tears DOMS Neuropathy-Parsonage Turner Syndrome “edema-like” muscle signal Infection Olecranon bursitis

Osseous Injury [Figure 4-29-8]

•

Effusion on Xray: presumed fx F/U X-ray vs MR Bone marrow edema after trauma: Bone bruise vs. fracture ➢ T1: focal low signal (linear) = fracture ➢ No line, ill-defined edema = bruise

Figure 4-29-8

Avulsion •

Chronic avulsive stress ➢ Tendinopathy ➢ Usu subtle edema at enthesis Avulsion fx ➢ Ligamentous or tendinous ➢ Thin, longitudinally oriented edema at cortical margin

• • •

Analogous bruises from ACL tears Transient disloc/sublux Often both sides of joint

• • • • • • • • •

OA Bodies Capsular fibrosis Non union / malunion Associated ligament injury/instability (e.g., Essex Lopresti) AVN Pain Limited ROM Instability

Osteochondral Impaction

Fracture Complications

MRI of the Elbow

958

Occult radial neck fracture

Musculoskeletal Radiology

• •

AVN

• •

Figure 4-29-9

Older: typical risk factors for AVN Younger = Panner’s disease ➢ Capitellum ➢ Boys; 4-10 years ➢ Decreased vasc to growing epiphysis ➢ Usu. spontaneously resolves ➢ If >10, higher risk of complications

Ligament Disorders

•

Medial Lateral

Medial Collateral Ligament

•

Three segments ➢ Anterior bundle ❖ Most important soft tissue static constraint to valgus stability ➢ Posterior bundle ➢ Transverse bundle

MCL • • • • •

[Figures 4-29-9 and 4-29-10]

Anatomy ➢ Anterior/posterior/transverse bands ➢ Strongest is anterior Pathophysiology ➢ Overhead throwing /valgus overuse, weakens/incompletely heals, reinjures Partial tears — “T” sign vs. complete tears Old tears show thickening +/- bowing Association with epicondylitis

Medial collateral ligament tear (arrow) and osteochondral impaction injury (arrowhead)

Figure 4-29-10

Lateral Ligaments

• •

Components ➢ Lateral collateral ligament proper (LCL) (Radial collateral ligament) ➢ Annular ligament ➢ Lateral ulnar collateral ligament (LUCL)

Annular Ligament • • • • •

Fibro-osseous ring that encircles and stabilizes the radial head Attaches on the anterior and posterior edges of the lesser sigmoid notch Anterior portion taught in supination and posterior portion taught in pronation

Medial collateral ligament tear (arrow) and lateral ulnar collateral ligament tear (arrowhead) in a patient with recent elbow dislocation

Radial Collateral Ligament

•

Extends from the lateral epicondyle and attaches to the annular ligament Immediately deep to the common extensors About half to a third size of MCL Maintains humeroradial apposition in the presence of varus stress

Lateral Ulnar Collateral Ligament • •

With annular ligament (PRUJ) and radial collateral ligament (radial head) makes up lateral lig complex Sweeps posteriorly past the radial neck and inserts on the ulna Stabilizer for rotational and varus stress

Musculoskeletal Radiology

959

MRI of the Elbow

• • • •

Posterolateral Rotatory Instability Instability of the elbow manifested by painful clicking of the elbow in extension Radial head moves posteriorly in relation to the capitellum Essential lesion - tear of the LUCL Lateral pivot shift test ➢ Supination with axial and valgus stress Lateral pivot shift test - supination with axial and valgus stress

• •

Caused by a fall on an outstretched hand Iatrogenic injury during release or repair of lateral epicondylitis

• • • • •

Mimics a mass SubQ Inflammatory-like signal Cat scratch fever Assoc fasciitis

• • • •

Bodies MCL injuries OCDs Subtle cartilage loss

LUCL INJURIES

Epitrochlear Lymph Node

MR Arthrography Indications

•

Elbow MRA • •

Ligament tear ➢ Extracapsular leakage of contrast ➢ Medial or lateral collateral ligament tear IA bodies ➢ Anterior, posterior recesses OCD ➢ Same dx as knee ➢ Esp capitellum

References 1. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist, and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-379. 2. Bordalo-Rodrigues M, Rosenberg ZS. MR imaging of entrapment neuropathies at the elbow. Magn Reson Imaging Clin N Am. 2004 May;12(2):247-263. 3. Potter HG, Ho ST, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskelet Radiol. 2004 Mar;8(1):5-16 4. Chung CB, Kim HJ. Sports injuries of the elbow. Magn Reson Imaging Clin N Am. 2003 May;11(2):239-53. 5. Steinbach LS, Palmer WE, Schweitzer ME. Special focus session. MR arthrography. Radiographics. 2002 SepOct;22(5):1223-1246. 6. Zou KH, Carrino JA. Comparison of accuracy and interreader agreement in side-by side versus independent evaluations of MR imaging of the medial collateral ligament of the elbow. Acad Radiol. 2002 May9(5):520-5. 7. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist, and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-79 8. Bordalo-Rodriguez M, Rosenberg ZS. MR Imaging of entrapment neuropathies at the elbow. Magn Reson Imaging Clin N. Am. 2004 May; 12(2):247-63. 9. Chung CB, Chew FS, Steinbach L. MR imaging of tendon abnormalities of the elbow. Magn Reson Imaging Clin N. Am. 2004 May;12(2):233-45. 10. Kaplan LJ, Potter HG. MR imaging of ligament injuries to the elbow. Magn Reson Imaging Clin N. Am. 2004 May;12(2):221-32, v-vi. 11. Fowler KA, Chung CB. Normal MR imaging anatomy of the elbow. Magn Reson Imaging Clin N. Am. 2004 May;12(2):191-206, v. 12. Potter HG, Ho St, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskeletal Radiol. 2004 Mar;8(1):5-16. 13. Savnik A, Jensen B, Norregaard J, Egund N, Danneskiold-Samsoe B, Bliddal H. Magnetic resonance imaging in the evaluation of treatment response of lateral epicondylitis of the elbow. Eur Radiol. 2004 June;14(6):964-9. Epub 2003 Dec 11. MRI of the Elbow

960

Musculoskeletal Radiology

Skeletal Metastases, Myeloma, Lymphoma Michael E. Mulligan, MD Figure 4-30-1 • • • • • • • • •

Incidence of Metastases [Figure 4-30-1] 30% of all patients with Cancer Skeleton – 3rd most common site More than 80% due to PTBLK #1-Breast, #2-Prostate, #3-Lung Spine lesion – Breast 75% Femur lesion – Breast 50% Skull lesion – MM, B, L Hands/Feet – Lung P=prostate T=thyroid B=breast L=lung K=kidney

Mechanism of Spread to Bones Hematogenous/Contiguous • • •

Marrow vessels unusual, rich sinusoidal system with large endothelial gaps Batson’s plexus has direct connection to IVC/SVC with no valves Arterial – mechanism for distal mets?

Batson OV. Ann Surg 1940;112:138

48 year old man with renal cell carcinoma metastases mimicking multiple myeloma

Figure 4-30-2 • • •

Bone Metastases Different T1, T2 signal Different Gadoliniumenhancement The “holy grail” – DWI

Radiology 1998; 207:305-7

Acute Vertebral Collapse - Osteoporosis or Malignancy •

• • • • • • • •

[Figure 4-30-2]

Symptoms and Signs ➢ Pain, most common symptom, but only in 2/3 patients ➢ Pathologic fracture ❖ Common sign, esp bad in spine, femur

Pathologic Fractures [Figure 4-30-3] 5–10% of all patients with mets 50% or more cortex gone - 2/3 will develop pathologic fracture 61 year old woman with breast Less than 50% cortex gone - 1/5 will develop pathologic fracture cancer, focal depression of L2 Any lesion in femoral neck superior endplate is indicative of Avulsion of lesser trochanter metastatic disease Any lesion prox femur >2.5cm Figure 4-30-3 Mirels score - site, size, l/b, pain 3.0 cm lesion with persistent pain after XRT

Mirels, H. Clin Ortho Rel Res 1989; 249: 256-64

55 year old man with lung cancer and cortical “cookie bite” metastasis

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• •

Bone Metastases: Systemic Features [Figure 4-30-4]

Figure 4-30-4

Hypercalcemia Hypertropic Osteoarthropathy, triad of ➢ Periosteal reaction ➢ Clubbing ➢ Pain

• • • •

Classic Triad Joint swelling, 30%–40% patients 5% patients with lung cancer Cause? – may be paraneoplastic, due to a growth hormone releasing factor

• • • •

Pure lysis Lysis with blowout (renal, thyroid) Mixed lytic/blastic (breast, lung, GI) Pure blastic (prostate, carcinoid, medulloblastoma)

• • • •

Stimulates clasts to synthesize collagenase Produced by normal activated leukocytes Dependent on prostaglandin E Prostaglandin inhibitors can reduce/obliterate osteolysis

Hypertrophic Osteoarthropathy

Bone Metastases: Radiologic Features

Osteoclast – Activating Factor (A Cytokine)

JBJS 68A:310, FEB 1986 • • • • • •

Bone Metastases: Radiologic Features Ivory vertebra Pathologic fx Periosteal rx – prostate, lung neuroblastoma, GI tumors Soft tissue mass (lung) Missing pedicle Intracortical – lung cancer

Ivory Vertebra(ae): Differential Diagnosis [Figure 4-30-5] • • • •

#1 Paget’s #2 Hodgkin’s #3 Metastasis

Figure 4-30-5

2 year old Irish setter with lung cancer. Foreleg bones show classic periosteal changes of H.O

Figure 4-30-6

50% 30% 20%

Breast Carcinoma • • •

The most common source of bone mets in women Spine #1 site 65% lytic, 25% mixed, 10% blastic

Prostate [Figure 4-30-6] • • •

The most common source of metastases in men More than 1/3 of patients 75% blastic, 15% mixed, 10% lytic Humoral factor stimulates blasts

59 year old woman with multiple ivory vertebrae secondary to breast cancer metastases Metastases, Myeloma, Lymphoma

962

76 year old man with florid periosteal reaction around distal fibula metastasis Musculoskeletal Radiology

• • •

15% of patients have mets to bone 80% lytic, 15% mixed, 5% blastic Small cell – 20% blastic

• •

25%–30% of patients have mets to bone 90% Lytic

Lung Cancer

Renal Cell Carcinoma [Figure 4-30-7]

Figure 4-30-7

71 year old man with blowout type metastasis from renal cell cancer. Note active hyperemia on angiogram

Figure 4-30-8

• •

8% of patients have metastases Lytic

• • • • •

1st choice any patient under 10 years old Can mimic primary malignancy Usually multiple, often symmetric Histology confused with Ewing’s Periosteal rx – aggressive

• • • •

1 – History / physical 2 – Lab studies Direct workup based on 1, 2 Primary not found in up to 60% patients

Thyroid Cancer

Neuroblastoma [Figure 4-30-8]

Workup of Patient with Metastases

3 year old boy with neuroblastoma. Metastasis in proximal radius shows permeative appearance similar to Ewing’s sarcoma Musculoskeletal Radiology

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Metastases, Myeloma, Lymphoma

• • • • •

Solitary Focus Bone Scan [Figure 4-30-9]

Figure 4-30-9

Seen in 2%–15% of cancer patients % due to metastatic disease varies by site of involvement 10% solitary rib lesions 50%–60% in other locations (spine) 10% malignant even if there is “DJD” in the area

Rib – J Nucl Med 1985;26:1140–1143 All sites – Radiology 1976;121:663–667 • • • • • • •

Malignant Round Cell Tumors of Bone

•

Myeloma Lymphoma Ewing’s sarcoma Neuroblastoma Rhabdomyosarcoma Small cell carcinoma PNET

Myeloma Types • • • • • • • •

MGUS – monoclonal gammopathy of undetermined significance (1% of all SPEPs) Asymptomatic myeloma (no bone lesions) Symptomatic multiple myeloma (classic, generalized, osteosclerotic [POEMS], multiple myeloma with osteosclerosis, leukemic) Non-Secretory myeloma – 3% of all cases Solitary plasmacytoma in bone – 3%-5% of cases Extramedullary plasmacytoma – 5%

Myeloma: Pathologic Features

• • •

Plasma cells proliferate in erythropoietic areas Grossly – dark red, tan; soft Histology – sheets of malignant plasma cells obliterate the marrow Special studies – markers for light chains Amyloid – 10% Osteoclast stimulating factor

• • • • •

A cytokine (lymphotoxin alpha) Similar to O-AF Produced by myeloma cells, T cells Interferon is a cytokine antagonist Bisphosphonates used to counter osteoclastic resorption

65 year old man with solitary rib abnormality on whole body bone scan, proven to be esophageal cancer metastasis

Osteoclast-Stimulating Factor

Cancer 1997; 80:1557-63 • •

Classic Multiple Myeloma: Clinical Features • •

Signs and Symptoms: pain, bleeding diathesis, infection, renal insufficiency Lab findings: monoclonal spikes (IgG, IgA), B-J proteinuria, anemia, hypercalcemia, elevated alkaline phosphatase Imaging W/U: X-rays, CT/MRI, Nucs, PET, PET/CT MM has highly malignant course

B-J=Bence-Jones MM=multiple myeloma

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Musculoskeletal Radiology

Durie/Salmon PLUS Staging* [Figure 4-30-10] Radiologist’s role is to help determine the true tumor burden throughout the skeleton • Stage IA: normal skeletal survey or single lesion • Stage IB: < 5 focal lesions or mild diffuse disease • Stage IIA/B: 5-20 focal lesions or moderately diffuse • Stage IIIA/B: >20 focal lesions or severe diffuse disease • Subclasses A&B (A = nl renal function, B = abnl) • Stage is generally predictive of survival ➢ IA median survival = 60 months ➢ IIIB median survival = 15 months

Figure 4-30-10

*Durie et al. Myeloma management guidelines: a consensus report. The Hematology Journal 2003; 4: 379-398 • •

Myeloma: Imaging Features [Figure 4-30-11] • • • •

80% of new MM patients – abnormal skeletal survey Multiple myeloma - punched out lesions ➢ endosteal scalloping Solitary plasmacytoma: bubbly, any margin +/- soft tissue mass Generalized form- just osteopenia Sclerosing - < 3%, assoc with POEMS syndrome Spine MR – 3 patterns; mild, moderate, severe

Sclerotic Myeloma: Multiple myeloma with sclerosis or POEMS syndrome • Polyneuropathy • Organomegaly • Endocrinopathy • Monoclonal gammopathy • Skin changes

From left to right: mild, moderate, and severe. The three types of myelomatous spinal involvement for the Durie/Salmon PLUS staging system (T1-weighted images) [Figure 4-30-12]

Figure 4-30-11

NEJM 1992;327:1919–1923

Figure 4-30-12

56 year old man with myeloma. Humeral radiograph shows typical endosteal scalloping and macrosection shows plasma cells filling the marrow space with osteoclasts along the endosteal surface

Blastic or sclerotic lesions that are usually painless are typical in the POEMS syndrome Musculoskeletal Radiology

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• •

Myeloma: Differential Diagnosis [Figure 4-30-13]

Figure 4-30-13

Figure 4-30-14

Metastatic disease B cell malignancy ➢ ALL, NHL, CLL, Waldenstrom’s

Plasmacytoma Differential Diagnosis [Figure 4-30-14] • •

Metastasis: thyroid, renal Primary: Fibrosarcoma, MFH

Primary Lymphocytic Lymphoma of Bone [Figure 4-30-15] Figure 4-30-15 56 year old man with myeloma,. skull radiograph shows typical “punched out” lytic lesions 71 year old woman with aggressive looking solitary plasmacytoma mimicking blowout type metastasis

70 year old man with PLB, skull radiograph shows multiple lytic lesions, some with central sequestra (arrow) • •

Primary Lymphoma: Path Features •

Gross – pinkish-grey, “fish-flesh” Histology – similar to nodal lymphoma round cells of various sizes (Ewing’s monotonous) Reticulin stain – meshwork of fibers around each cell

• • • • • •

Non-Hodgkin’s (94%), Hodgkin’s (6%) Rare, @ 3% malignant tumors Any age, but rare under 10 years Stage like soft tissue lymphoma Solitary and multifocal (skull, femur, tibia) Osteoclast-stimulating factor

• • • • •

151 M, 86 F, ratio 1.8:1 Average age 42 years (range: 2 to 88 years) Long bones n=162 (71%) Flat bones n=78 (22%) Other sites (including spine, small bones) n=17

Figure 4-30-16

Primary Lymphoma: Clinical Features

PLB – 237 AFIP CASES

Mulligan, et al. AJR 1999; 173: 1691-1697 • • • • •

Typical Features – PLB [Figure 4-30-16] Location: Metadiaphyseal n=120 (54%) Pattern: Permeative n=130 (55%) Cortical involvement: n=148 (62%) Periosteal reaction: interrupted or solid single layer n=57 (65.5%) Soft tissue mass: n=113 (48%)

Mulligan, et al. AJR 1999; 173: 1691-1697 Metastases, Myeloma, Lymphoma

966

57 year old woman with PLB, tibia lesion shows all of the most common radiographic features Musculoskeletal Radiology

•

Variations – PLB [Figure 4-30-17]

•

•

Figure 4-30-17

Locations ➢ Epiphysis n=11 (5%) ➢ Diaphysis n=45 (19%) ➢ Intracortical n=16 (7%) Patterns ➢ Normal x-ray n=12 (5%) ➢ Geographic n=26 (11%) ➢ “Blow Out” n=2 (< 1%) ➢ Blastic n=4 (2%)

Radionuclide, CT and MRI Findings • •

•

•

Radionuclides n=56, markedly increased uptake n=36 (64%) CT n=45, MRI n=20 “Cortical holes” by CT or MR ➢ Large n=20 (31%), small n=45 (69%) Soft tissue mass ➢ Seen by CT n=36 (80%) ➢ Seen by MR n=20 (100%)

Variations – PLB • • •

Markedly abnormal bone scan and MR exam in 57 year old man with thigh pain and normal xrays. This type of extensive marrow replacement pattern with normal xrays is highly suggestive of “round blue cell tumors” like PLB and Ewing’s sarcoma

[Figure 4-30-18]

Periosteal reaction ➢ Multiple layers n=26 (10.2%) ➢ Sunburst n=4 (1.6%) Pathologic fracture n=52 (22%) Sequestra n=37 (15.6%) Crossing joint n=12 (5%)

Figure 4-30-18

AJR 1999; 173: 1691-1697 • • • • • •

Primary Lymphoma: Differential Diagnosis

•

Metastatic lymphoma Ewing’s sarcoma Neuroblastoma / PNET Rhabdomyosarcoma Osteomyelitis Eosinophilic granuloma

Summary – PLB •

Usually has an aggressive appearance ➢ CT or MRI showing large soft tissue mass without large cortical holes is typical Wide range of appearances ➢ Normal x-rays ➢ Geographic lesions ➢ “Blow out” lesions ➢ Blastic lesions

Large lytic lesions with soft tissue mass and sequestra should put PLB high up in the differential diagnosis

References 1. 2. 3. 4. 5. 6. 7.

Durie et al. Myeloma management guidelines: a consensus report. The Hematology Journal 2003; 4: 379-398 Mirels H. Metastatic disease in long bones. Clin Orthop Relat Res 1989;249:256-264 Mulligan M et al. Skeletal Metastatic Disease. In: Pope et al, Imaging of the Musculoskeletal System. Philadelphia: Elsevier, 2006 Mulligan M. Imaging techniques used in the diagnosis, staging, and follow-up of patients with myeloma. Acta Radiologica 2005;46:716-724 Mulligan M, McRae G, Murphey M. Imaging features of primary lymphoma of bone. AJR 1999; 173: 1691-1697 Roodman GD. Mechanisms of bone metastasis. N Engl J Med 2004;350:1655-1664 Weber K et al. An approach to the management of the patient with metastatic bone disease. Instr Course Lect 2004;53:663-676

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Imaging of Hematologic Disease

Figure 4-31-1

Thomas Lee Pope, Jr, MD, FACR • •

Objectives • •

To identify some of the common hematologic disorders To describe the clinical and epidemiological aspects of these entities To demonstrate the most significant MSK imaging findings

Hematologic Disease

•

• • • • •

Hereditary anemias ➢ Sickle cell anemia ➢ Thalassemia ➢ Rare anemias: Fanconi’s, thrombocytopenia with absent radii syndrome (TAR) Coagulation disorders ➢ Hemophilia ➢ Myelofibrosis

Characteristics of the Hereditary Anemias

•

Aberrations and/or abnormalities of RBC shape Molecularly distinctive Autosomal dominant Electrophoresis The Five “In’s” ➢ In sufficient ossification ➢ In farction ➢ In fection ➢ In failure (anemia) ➢ In volution (spleen)

Hand Foot Syndrome: Notice the diffuse involvement with regions of osteolysis and periosteal reaction

Figure 4-31-2

Sickle Cell Disease • • •

• • • • •

One of the most common inherited blood disorders (> 100,000 born with the disease worldwide per year) One of the most prevalent genetic disorders in the US (> 80,000 African Americans) Hemoglobin S gene (carrier state) ➢ Autosomal dominant ➢ Carried by 8% of African Americans or ~ 2 million US Blacks Hemoglobin SS disease (Sickle cell anemia) ➢ Autosomal recessive ➢ 1 birth in 400 in African Americans ➢ Occurs in 0.3%-1.3% of NA Blacks or ~ 50, 000 in the US

Economic Impact of SC Disease 75,000 hospitalizations yearly Average of $6, 300 per hospitalization $475 million in health care costs alone Does not include lost wages, productivity, etc… Likely > $1 billion yearly cost

Acute (upper image) and chronic (lower image) Salmonella osteomyelitis

Imaging of Hematologic Disease

968

Musculoskeletal Radiology

•

Characteristics of sickled and normal RBC’s

Figure 4-31-3

NORMAL ➢ Disc-shaped ➢ Soft and compressible ➢ Easily flows through vessels ➢ Life span of > 120 days • SICKLE ➢ Sickle-shaped ➢ Hard (tough and not malleable) ➢ Sticks in blood vessels ➢ Life span of < 20 days

Major Pathology-Vascular occlusion

[Figure

•

4-31-1]

Hand-Foot syndrome ➢ Dactylitis • Infarction (any site) • Infection • Marrow hyperplasia • • •

Hand foot syndrome (Dactylitis)

• • • • •

Up to 50% of “sickle cell anemia” children 2 months to 6 years Pain, low grade fever, diffuse non-pitting edema of the extremities Vaso-occlusion with osteonecrosis Infection is major DDx Distinction: Clinically

Infection

MR features of acute Salmonella osteomyelitis Courtesy of Dr. Hilary Umans New York, NY

Figure 4-31-4

[Figure 4-31-2]

50X less common than infarction Salmonella much more frequent pathogen in SCA patients • Infecting organisms: ➢ Salmonella – 70% ➢ Staph aureus – 10% ➢ Shigella sonnei, E coli, Arizona hinshawii and Serratia • Proposed mechanisms: ➢ Vascular insufficiency ➢ Decreased phagocytosis-low O ➢ Decreased splenic function Multiple hospitalizations

Classic features of avascular necrosis with areas of osteolysis and osteosclerosis with preservation of articular space

Figure 4-31-5 Figure 4-31-6

Radiographic and anatomic gross specimen correlation of the “rim” sign MR imaging of intramedullary infarcts

Musculoskeletal Radiology

969

Imaging of Hematologic Disease

Differential Diagnosis: “Bone within Bone” Appearance • • • • • •

Figure 4-31-7

S – Sickle cell disease T – Thoratrast O – Osteopetrosis P – Paget disease Heavy metal Hypervitaminosis D

http://chorus.mcw.edu

• • •

“H-Shaped” Vertebral Bodies [Figure 4-31-8]

• • • •

Another manifestation of ischemia and infarction >10 years of age Incidence: ➢ 43% of SS ➢ 36% of Sickle/Thalassemia ➢ 25% of SC

Thalassemia

• •

“Bone within a bone” apprearance in sickle cell anemia

1925 - Cooley and Lee Synonyms: Cooley’s anemia, mediterranean anemia, leptocytosis Impaired alpha or beta chain Hgb production Homozygous beta thalassemia (800-1000 US persons - NE corridor between Boston and NY) Heterozygous trait (2.5% of Italian Americans, 7%-10% of Greek Americans)

Figure 4-31-8

Types of Thalassemia

•

Alpha ➢ Least severe: ❖ Silent carrier = loss of 1 alpha globulin gene - often incidental finding ❖ Most severe: ❖ Hydops fetalis = loss of 4 alpha globulin genes - die in utero Beta: ➢ Spectrum ❖ Minor = slight anemia ❖ Major = life-threatening anemia requiring transfusions ❖ Risk of Fe++ overload “H” shaped vertebral bodies of sickle cell disease

Figure 4-31-10 Figure 4-31-9

Classic “Hair on end” appearance of Thalassemia Imaging of Hematologic Disease

Marrow expansion in Thalassemia with widening of the medullary canal and thinning of the cortices 970

Musculoskeletal Radiology

Imaging Features of Thalassemia •

Figure 4-31-11

[Figures 4-31-9 to 4-31-12]

• • • • •

Diffuse marrow expansion ➢ Skull - (“hair on end”) appearance ➢ Face - (“rodent-like facies”) ➢ Long bones – “Erlenmeyer flask” deformity Extramedullary hematopoeisis Rare minor features: Growth disturbances, fractures, crystal deposition

Gaucher’s Disease

• • • • • •

Ashkenazic Jews of Eastern European descent Defect of beta glucosidase Accumulation of glycosyl ceramide in the RE cells of BM, spleen, and liver Hepatosplenomegaly, yellow skin, scleral pigmentation, acid and alkaline phosphatase elevation

Imaging features

• • • • • •

AVN of the hip and femoral head Osteoporosis Marrow expansion with cortical thinning Erlenmeyer flask deformity Lytic lesions and sometimes periostitis

Erlenmeyer flask deformity

Differential Diagnosis: “Erlenmeyer Flask” Deformity

Figure 4-31-12

Osteoporosis Chronic anemia (Sickle cell disease) Gaucher disease Niemann- Pick (enzyme deficiency) Fibrous dysplasia Metaphyseal dysplasia (Pyle’s disease)

Extramedullary Hematopoiesis •

[Figures 4-31-13 and 4-31-14]

•

Blood production in fetal regions ➢ Liver, spleen, adrenal, thymus, heart, lung, nodes, renal pelvis, GI tract, dura mater (almost anywhere !) Major causes: ➢ Hematologic disease (SS and thalassemia) ➢ Myelofibrosis ➢ Leukemia ➢ Hodgkin’s ➢ Hyperparathyroidism ➢ Rickets ➢ Carcinomatosis

Figure 4-31-14

“Erlenmyer flask” deformity in Gaucher’s disease. “Crumpled tissue paper” cytoplasm on histology

Figure 4-31-13

Extramedullary hematopoesis with hepatosplenomegaly and posterior mediastinal masses

Extramedullary hematopoesis with MR correlation Musculoskeletal Radiology

971

Imaging of Hematologic Disease

• •

Fanconi’s Anemia [Figure 4-31-15] • •

Figure 4-31-15

Onset after first decade Severe anemia, pancytopenia, brown pigmentation Death 2–3 years after appearance Anomalies: ➢ Short stature, microcephaly, delayed ossification ➢ Hip dysplasia, renal anomalies ➢ Radius absent (50%) ➢ Thumb always absent

Thrombocytopenia with Absent Radii (TAR) [Figure 4-31-16] • • •

• • • • • • • • • • •

Fanconi’s anemia:

Congenital hypomegakaryocytic thrombocytopenia Apparent at birth or shortly thereafter Anomalies: ➢ Bilateral radial aplasia always present ➢ Thumb is present (differentiation from Fanconi’s) If kids survive for the first two years, the anemia often spontaneously resolves

Figure 4-31-16

Hemophilia

•

Oldest known bleeding disorder First noted in offspring of Queen Victoria of England Mutation in Queen Elizabeth’s X chromosomes Group of X-linked recessive disorders Gene carried by women and expressed in men All races affected 20, 000 hemophiliacs in US 400 new cases/year Severity – related to lack of clotting factor 70% have < 1% of normal clotting factor

TAR with absent radii syndrome: Note that the thumb is present

Figure 4-31-17

Major Types of Hemophilia

•

•

Hemophilia A ➢ 85% of all cases ➢ Factor VIII (antihemophiliac factor-AHF) deficiency ➢ 70% have < 1% of normal amounts of AHF Hemophilia B (Christmas disease) ➢ 15% of all cases ➢ Factor IX (Plasma thromboplastin componentPTC) deficiency

Acute hemorrhagic effusions in two patients with hemophilia

Figure 4-31-18

Joint Disease •

• • • • •

Acute hemarthroses: tense, swollen, red and tender joints, pain, LOM, fever, increase in WBC Stages of joint disease: ➢ Stage I: STS ➢ Stage II: Osteoporosis ➢ Stage III: Osseous lesions ➢ Stage IV: Cartilage destruction ➢ Stage V: Joint disorganization

Knee [Figures 4-31-17 and 4-31-18]

Marked articular space narrowing and cartilage destruction with massive subchondral cyst formation

Dense effusions Juxtaarticular osteoporosis Subchondral irregularity Epiphyseal overgrowth Squaring of inferior pole of patella (20%-30%)

Imaging of Hematologic Disease

972

Musculoskeletal Radiology

Elbow [Figure 4-31-19]

Figure 4-31-19

Low SI synovial proliferation [Figure 4-31-20] •

Differential Diagnosis of Hemophilia

• •

Juvenile chronic arthritis ➢ Single joint tough to differentiate ➢ Distribution may be helpful: ❖ JCA – hands/feet/big joints ❖ Hemophilia - knee, ankle, elbow PVNS, infection, especially TB (monoarticular) NM diseases: CP, muscular dystrophy, Figure 4-31-20 polio Hemophiliac involvement of two elbows in two different patients

Figure 4-31-21

Low signal intensity of synovial proliferation in hemophilia.

Medial talar tilt quite characteristic of hemophilia

Kerr R: Imaging of MSK complications of hemophilia. Sem in MSK

Figure 4-31-22 •

Other Less Common Imaging Findings • • • • • • •

Ectopic ST ossification (periarticular – pelvis, thigh, paraspinal, knee) Hemophiliac pseudotumor Osteonecrosis (epiphyseal fragmentation) Fractures Chondrocalcinosis

Pseudotumor [Figures 4-31-22 to 4-31-26]

• •

2% of patients Femur, pelvis, tibia, hands and feet Locations: ➢ Soft tissue, intraosseous, and subperiosteal ST ➢ Hard palpable subcutaneous masses Intraosseous and subperiosteal ➢ Lytic, expansile, destructive, aggressive process

Soft tissue pseudotumor of hemophilia Park JS, Ryu KN: AJR 2004;

Figure 4-31-23

Subperiosteal pseudotumor of hemophilia Park JS, Ryu KN: AJR 2004; 183:55-61

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Imaging of Hematologic Disease

Figure 4-31-24

Figure 4-31-25

Intraosseous pseudotumors of hemophilia Park JS, Ryu KN: AJR 2004; 183:55-61

Differential Diagnosis for Hemophiliac Pseudotumor •

• • • • •

Malignancy ➢ Osteosarcoma ➢ Chondrosarcoma ➢ Ewing tumor Metastases Infection

Intraosseous pseudotumor of the ilium in a hemophiliac Kerr R: Imaging of MSK complications of hemophilia in MSK Radiology 7:2, 2003

Figure 4-31-26

Myelofibrosis

•

• • • •

Affects progenitor (stem) cells of the bone marrow Primary (idiopathic) and secondary forms Major manifestations ➢ Fibrotic or sclerotic bone marrow ➢ Extramedullary hematopoiesis Other designations: ➢ Idiopathic myelofibrosis ➢ Myeloid metaplasia ➢ Agnogenic myeloid metaplasia

Primary (Idiopathic) Myelofibrosis Bone marrow replaced by fibrosis Unknown cause > 50 year old, incidence - 2/100,000 Findings: ➢ BM fibrosis with hepatosplenomegaly ➢ Anemia ➢ Increased nucleated RBC’s ➢ Leukocytosis or leukopenia ➢ Abnormal WBC’s

Intraosseous “blowout” lesions of hemophilia

Figure 4-31-27

Primary (Idiopathic) Myelofibrosis • • • •

[Figure 4-31-27]

• • • •

Diagnosis of exclusion Unknown cause > 50 year old, incidence - 2/100,000 Findings: ➢ BM fibrosis with hepatosplenomegaly ➢ Anemia ➢ Increased nucleated RBC’s ➢ Leukocytosis or leukopenia ➢ Abnormal WBC’s Diffusely dense bones characteristic of Diagnosis - BM aspiration myelofibrosis Rx: Transfusions, chemo, Interferon, splenectomy, radiation 50%-80% of patients have elevated serum or urinary uric acid levels Secondary gout occurs in 5-20% of patients

Imaging of Hematologic Disease

974

Musculoskeletal Radiology

•

Secondary Myelofibrosis • • • • • • •

Malignant disease ➢ Leukemias, Polycythemia vera, MM, Hodgkin’s disease, NHL, cancer Chronic infection ➢ Tuberculosis, osteomyelitis Toxins ➢ X- or gamma radiation, benzene exposure

Imaging Findings [Figure 4-31-15]

•

Generalized osteosclerosis (most common) Cortical thickening Osteopenia Rarely periostitis Extramedullary hematopoiesis

Review

•

•

•

Sickle Cell ➢ Vaso-occlusion ❖ “Hand-Foot” syndrome ❖ AVN and medullary bone infarcts ❖ “H-shaped” (“Lincoln log”) vertebral bodies ❖ “Bone within a bone” appearance ❖ Salmonella infection Thalassemia ➢ “Hair on end” ➢ “Pseudohemangiomatous” appearance ➢ “Erlenmeyer flask” deformity (differential-Gaucher’s) ➢ “Rodent” facies Hemophilia ➢ Wide intercondylar notch ➢ Erosions ➢ Medial slope of distal tibia at ankle ➢ “Pseudotumor” Myelofibrosis ➢ Primary and secondary forms ➢ Diffusely dense bones ➢ Hepatosplenomegaly ➢ BM bx to make dx

References 1.

2.

3.

4. 5. 6. 7.

"What is Sickle Cell Disease". Sickle Cell Information Center. December 16, 2003. Copyright © 1997. The Georgia Comprehensive Sickle Cell Center at Grady Health System, The Sickle Cell Foundation of Georgia, Inc., Emory University School of Medicine, Department of Pediatrics, Morehouse School of Medicine. http://www.scinfo.org/sicklept.htm Funaki B. "Sickle cell anemia: Bone manifestations", "Bone within a bone". Chorus: Collaborative Hypertext of Radiology. (Kahn CE ed). July 2004. Medical College of Wisconsin. February 1995. http://chorus.rad.mcw.edu/doc/01060.html Kahn CE. " Erlenmeyer flask deformity". Chorus: Collaborative Hypertext of Radiology. (Kahn CE ed). May 2004. Medical College of Wisconsin. < http://chorus.rad.mcw.edu/doc/00648.html> Kerr R. Imaging of musculoskeletal complications of hemophilia. Semin Musculoskelet Radiol 2003; 7:127-136. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001; 21:971-994. Park JS, Ryu KN. Hemophilic pseudotumor involving the musculoskeletal system: spectrum of radiologic findings. AJR Am J Roentgenol 2004; 183:55-61. Wong AL, Sakamoto KM, Johnson EE. Differentiating osteomyelitis from bone infarction in sickle cell disease. Pediatr Emerg Care 2001; 17:60-63; quiz 64.

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Imaging of Hematologic Disease

Generalized Musculoskeletal Disorders Thomas Lee Pope, Jr, MD, FACR •

Figure 4-32-1

Learning Objectives • • • • • •

To describe a group of entities not well covered in the rest of the course To outline the imaging features of these diseases To introduce the listener to these entities so that he/she can study about them further

Outline of Diseases Osteoporosis (and its sequelae) Osteogenesis imperfecta Neurofibromatosis Collagen vascular-like diseases ➢ SLE ➢ Scleroderma ➢ Polymyositis/dermatomyositis Disuse osteoporosis in a 76 yo with left sided CVA • •

Terminology

•

Osteopenia – “paucity of bone” Osteoporosis ➢ Decreased bone mineral density ➢ Normal in quality ➢ Decreased in quantity 30%-50% of cancellous bone must be gone to recognize

• • •

Localized Regional or segmental Generalized or diffuse

Types of Osteopenia

• •

Localized Osteopenia/Osteoporosis Focal areas of bone loss Differential diagnosis: ➢ Infection ➢ Arthritides

Figure 4-32-2

Regional Osteopenia/Osteoporosis • •

Segmental area of decreased BMD Differential diagnosis: ➢ Disuse (immobilization) ➢ Chronic regional pain syndrome (CRPS) (RSD (Reflex sympathetic dystrophy) [Figure 4-32-2]

➢ Transient osteoporosis (bone marrow edema) ➢ Regional migratory osteoporosis

Generalized Musculoskeletal Disorders

Chronic regional pain syndrome (Reflex sympathetic dystrophy) Note the increased radionuclide accumulation on the early and late views of the bone scan

976

Musculoskeletal Radiology

•

Disuse/Immobilization Osteoporosis

• • •

Figure 4-32-3

[Figure 4-32-1]

Major cause ➢ Immobilization for traumatic injury ➢ Motor paralysis ➢ Inflammatory lesions of bones and joints Changes take 7-10 days (maximal at 23 months) Patterns: uniform, spotty, bands, cortical lamination or scalloping May appear very aggressive!!!

Chronic Regional Pain SyndromeReflex Sympathetic Dystrophy • • •

[Figure 4-32-1]

Elderly Trivial trauma Pain, swelling, temperature changes

Transient Regional Osteoporosis • •

[Figure 4-32-3]

•

• • •

Transient regional osteoporosis (bone marrow edema) of the hip: Note the osteopenia of the right hip and the proximal diffuse increased radionuclide accumulation on the bone scan Note the marrow replacement in the proximal right femur on T1 and the increased signal intensity (edema) on the T2 weighted images

General term Conditions sharing features of: ➢ Rapidly developing, self-limited, reversible osteoporosis ➢ Absence of clear cut inciting events Major types: ➢ Transient osteoporosis (bone marrow edema) of the hip ➢ Regional migratory osteoporosis

Figure 4-32-4

Regional migratory osteoporosis Rapidly developing, self-limiting and reversible Knee, ankle, foot and hip Joint nearest involved likely to be next involved

Transient Regional Osteoporosis (bone marrow edema) of the hip • • • • • • • •

1st -women in the third trimester of pregnancy Middle aged males LE > UE Osteoporosis and BM edema Differential diagnosis: AVN, infection Generally spontaneously resolves in 9-12 months

Pathology of osteoporosis (rib specimens)

Generalized Osteopenia/Osteoporosis

• • •

Diffuse decreased BMD Differential diagnosis: ➢ Senile osteoporosis ➢ Medications (Steroids, heparin) ➢ Systemic diseases (Deficiency states) ❖ Scurvy ❖ Malnutrition ❖ Calcium deficiency

Senile Osteoporosis [Figure 4-32-4]

• •

Pommer-1985 — “increased porosity” Most commonly encountered metabolic disease Reduction in bone “quantity,” normal in “quality” F>M, 4:1, (equal incidence) > 80 yo Most pain source: compression fx’s and kyphosis PE – kyphosis, shortened stature, and spinal rigidity

Musculoskeletal Radiology

977

Generalized Musculoskeletal Disorders

•

Epidemiological data

• • •

Figure 4-32-5

Surgeon General Report, October, 2004 ➢ Half population in US > 50 yo with low bone mass and risk for fracture ➢ 1.5 million/year osteoporosis-related fx ➢ 34 million with hip osteopenia Caucasian females > 50 yo – 40% chance of fx in lifetime (13% for males) Hip fracture: ➢ Risk of mortality within 3 months is 4X greater than normal ➢ 20% of fx victims die or wind up in nursing home within year after event Annual cost of treating osteoporosis: $18 BILLION

Pathology of osteoporosis [Figure 4-32-4] Osteoporosis “life cycle” - Fracture index [Figure 4-32-5] • • • • • •

Osteoporosis Measurement [Figure 4-32-6] Dual energy X-ray absorptiometry (DEXA) Conventional X-ray (radiogrammetry) Single photon absorptiometry (SPA) Dual photon absorptiometry (DPA) Neutron activation analysis Quantitative CT (QCT)

Figure 4-32-6

Lumbar

• • • • • •

Hip

Distal Radius

Dual Energy X-ray Absorptiometry (DEXA)

• • • •

Relative tissue attenuation from dual energy X-ray source Easy to perform Most reproducible technique with the least coefficient of variation (COV) Detects changes of 1–3% Expressed in gm/cm2 Primary indication: Estrogen deficiency to determine therapy

BMD Terms BMD measured in gm/cm2 T-score: Patient’s BMD compared to normative data (Normal = 25 yo women) Z-score: Patient’s BMD compared to her aged-matched controls World health organization uses T scores to classify a patient’s bone mineral status

Generalized Musculoskeletal Disorders

978

Musculoskeletal Radiology

• •

WHO Classification of BMD • • • •

STANDARD: Mean BMD of 25 yo women NORMAL: BMD from the mean to 1 standard deviation below the mean (mean to -1SD) OSTEOPENIA: T-score from 1 to 2.5 SD below mean (-1SD and -2.5 SD)) OSTEOPOROSIS: T-score below 2.5 SD below mean (> -2.5 SD) Osteoporosis also established by presence of a non-traumatic vertebral compression fracture

Figure 4-32-7

Important implications • • •

• • •

Fracture risk doubles with each drop of 0.1 below the mean of the T-score Risk of fracture also doubles for each decade the patient is > 50 yo Goal is to eventually be able to calculate an “absolute fracture risk”- more holistic measurement method Patients respond better to “you have a 70% of Photomicrograph of senile vertebral osteoporosis developing a fracture” than they do to “your T-score is whatever”

Figure 4-32-8

Senile Osteoporosis: Imaging Features

•

Increased radiolucency on X-ray (“osteopenia”) Cortical thinning Altered trabecular patterns

Senile Osteoporosis: Complications

• •

Acute fractures ➢ Spine (L>T>C) ➢ Distal radius (Colles) ➢ Proximal femur ➢ Humerus (neck) ➢ Ankle (malleoli) Insufficiency fractures ➢ Covered in “osseous stress injury” talk

Lateral radiographs of three patients with “codfish” vertebral bodies

Spinal effects of osteoporosis • •

Decreased bone density ➢ Accentuation of primary trabeculae Cortical thinning Changes in vertebral shape ➢ “Biconcave” – CODFISH (“fish”) shape ➢ Endplate deformities (Schmorl’s nodes, cortical irregularities) ➢ Wedged vertebrae ➢ Vertebrae plana (“pancake”/silver dollar)

Senile vertebral osteoporosis “Codfish” vertebral bodies

Figure 4-32-9

[Figure 4-32-7]

[Figure 4-32-8]

Femoral neck fracture locations

[Figure 4-32-9]

A = subcapital B = neck C = basicervical D = intertrochanteric E = subtrochanteric

Musculoskeletal Radiology

979

Generalized Musculoskeletal Disorders

• •

Garden classification [Figure 4-32-10] < II = Percutaneous pinning > II = THA (AVN risk)

Figure 4-32-10

Bohndorf, Imhoff, Pope: Musculoskeletal Imaging: A Multimodality Approach George Thieme Verlag, 2001

Figure 4-32-11

Garden Type I [Figure 4-32-11] • • • •

Intertrochanteric Fractures

• • • • • • • • •

Extracapsular Periosteum present Low incidence of nonunion or AVN (~1%) Distinction from basicervical often difficult (no clinical concern) Most comminuted, 15% severely GT/LT may be displaced by gluteus or iliopsoas May have other non-suspected injuries in pelvis

Osteogenesis Imperfecta (OI)

• •

[Figure 4-32-12]

Skeletal, skin, sclera and dentin abnormality 1/30K affected 1/50K severely 20-50K in US 85% AD Major types: ➢ Congenita ➢ Tarda

Garden I type of femoral neck fracture treated with Knowles’ pins

Figure 4-32-12

OI vs Child Abuse • •

Metaphyseal corner fx’s uncommon in OI Sternal, rib, scapular, skull and bucket handle fx’s common Fx’s continue to occur in protective custody Other non-MSK findings not present: ➢ Retinal hemorrhage ➢ Visceral intramural hematomas ➢ Intracranial bleeding ➢ Pancreatitis ➢ Splenic trauma

OI-congenital type OI-tarda

Congenital type of Osteogenesis Imperfecta

Figure 4-32-13

[Figure 4-32-12]

[Figures 4-32-13 and 4-32-14]

Tarda form of Osteogenesis Imperfecta: Note healing fractures with exuberrant callus formation

Generalized Musculoskeletal Disorders

980

Musculoskeletal Radiology

Figure 4-32-14

Figure 4-32-15

Note multiple fractures, intramedullary rods and dynamic hip screw and Harrington rods for scoliosis

Tarda form of Osteogenesis Imperfecta: Note exuberrant callus formation and intramedullary rod placement for fractures • •

Neurofibromatosis (NF) • • • • • • • • •

Described first by Tiresius (1773) and Smith (1849) Named for von Recklinghausen - noticed association of neural and cutaneous elements in 1882!! Defects of all three cell layers = phakomatosis 1/3000 births Estimated ~100,000 in US One of humanity’s most common genetic disorders Mutation rate is 1/10,000 gametes/generation Greater than that for ALL OTHER COMMON GENETIC DISORDERS AD with variable gene expression (FH in 60%) Equal incidence in male and female and Caucasian and non-Caucasian

Neurofibromatosis (NF)

• • •

Two distinct clinical forms: ➢ NF-1 (vonRecklinghausen’s) - Café-au-lait spots, neurofibromas, skeletal deformities ➢ NF-2 - Acoustic neuromas Can lead to disfigurement, blindness, deafness, dermal/brain/spinal tumors, loss of limbs, malignancies, learning disabilities WAS NOT “Elephant Man’s Disease”…John Merrick had Proteus Syndrome (cell growth disturbance with hemihypertrophy and macrodactyly)

Imaging features [Figures 4-32-16 to 4-32-20 overleaf]

•

•

Spinal changes: ➢ Dural ectasia ❖ Vertebral scalloping ❖ Foraminal enlargement ❖ Pedicle erosion ➢ Mesodermal dysplastic changes: ❖ Scoliosis - Typical - Dysplastic, sharply angulated, < 6 segments of lower T spine (pathognomonic of NF) ❖ Pencilling and spindling of the transverse processes Long bones (due to neurofibromas or mesodermal dysplastic changes) ➢ Pencilling ➢ Bone erosions ➢ Pseudarthrosis (characteristically of the tibia) ➢ Associated with nonossifying fibromas Ribs ➢ Scalloped and irregular (“twisted ribbons”) ➢ Erosions of inferior rib surfaces

Musculoskeletal Radiology

981

Generalized Musculoskeletal Disorders

Figure 4-32-16

Figure 4-32-17

Neurofibromatosis: Note accentuated scoliosis

Neurofibromatosis: Note kyphosis, vertebral anomalies and widened neural foramen

Figure 4-32-19 Figure 4-32-18

Neurofibromatosis: Note mesodermal dysplastic changes in the pelvis of two different individuals

Figure 4-32-20

Neurofibromatosis: Note posterior vertebral scalloping and dural ectasia

Neurofibromatosis with the characteristic “pseudoarthrosis” of the tibia Generalized Musculoskeletal Disorders

982

Musculoskeletal Radiology

• • •

Meningoceles [Figures 4-32-21 and 4-32-22]

• • •

Figure 4-32-21

About 2/3 of patients with NF 70%-80% of all meningoceles in NF patients Most common presentation: Asymptomatic post mediastinal mass Protrusion of dura and arachnoid through IV foramen and posterior rib cage into the extrapleural thoracic cavity - nonca++ Presence of ca++ excludes meningocele

Systemic Lupus Erythematosis (SLE) • • • • • • • •

“Lupus” -Latin for wolf – malar erythema looked like the bite of a wolf Generalized connective tissue disorder F>M, second to fourth decade, rare over the age of 45 Higher incidence in AA and Hispanics Fever, anorexia, weight loss, polyarthralgias, skin rash Chronic disease with acute episodes Variable prognosis

Intrathoracic meningocele: Note the posterior mediastinal mass on the right

Figure 4-32-22

Three Categories of SLE •

Discoid-skin rash only, 20% of patients with SLE Systemic-chronic, inflammatory, multisystem disorder of the immune system Drug-induced-Chlorpromazine, hydralazine, isoniazid, methyldopa, procainamide CT and MR imaging of intrathoracic meningocele

Musculoskeletal Imaging Findings • •

Figure 4-32-23

[Figure 4-32-23]

•

Major: deforming nonerosive arthropathy Minor: ➢ Osteonecrosis ➢ Insufficiency fractures ➢ ST calcification ➢ Acroosteolysis ➢ Tendon weakening and rupture ➢ Subchondral cysts ➢ Myositis ➢ Polyarthritis ➢ Osteomyelitis and septic arthritis

Symmetric Polyarthritis • • • • • •

Articular symptoms and signs common-75%-90% of patients Non-deforming nonerosive arthropathy of SLE Frequently bilateral and symmetric (differential diagnosis is post-Streptococcal 5%-40% with disease (Jacoud’s) arthropathy Hands > knees > wrists > shoulders ST swelling, periarticular osteopenia Reversible and little functional effect !!Cartilage and osseous destruction rare without underlying osteonecrosis!!

• • • • • • •

Unknown cause F:M (4:1) 3rd to 5th decade < 20 cases/million/year Variable prognosis Up to 65% MSK involvement at presentation Death: Lung, heart and renal involvement

Scleroderma

Musculoskeletal Radiology

983

Generalized Musculoskeletal Disorders

• •

CREST Syndrome • • • • • • • •

Figure 4-32-24

First described by Winterbauer in 1964 as CRST Velayos added esophageal involvement to make CREST in 1979 50,000 to 100,000 in US 7 times more common in females Calcinosis Raynaud’s phenomenon Esophageal abnormalities Sclerodactyly Telangiectasia

Calcinosis [Figure 4-32-24] • • • • •

Abnormal calcium deposition in ST without calcium metabolism abnormality Fingers, forearms, and extensor surfaces of elbows and knees

Calcinosis of Scleroderma

Figure 4-32-25

Raynaud’s phenomenon [Figure 4-32-24] Often the first symptom of scleroderma Ischemia of fingers, toes and ears Numbness, tingling and burning pain Attacks precipitated by cold, vibration and emotional stimuli

Acroosteolysis

[Figure 4-32-25]

Acroosteolysis of scleroderma

• •

Fibrosis and atrophy of the smooth muscle Hypermobility, dysphasia, reflux esophagitis and strictures

• • •

Replacement of the normal connective tissue with dense collagen bundles Skin = thin, appears smooth and is tightly bound Fingers narrow and taper distally

• •

Permanent dilatation of capillaries and venules Face, lips, tongue and fingers

Gastrointestinal (esophageal) involvement

Sclerodactyly

Telangiectasia

• • • •

Idiopathic inflammatory myopathies

•

•

Dermatomyositis and Polymyositis 2:1 female to male ratio 5 cases/million/year (incidence increasing) Dermatomyositis ➢ Complement-mediated (terminal attack complex) vascular inflammation Polymyositis ➢ Direct cytotoxic effect of CD8+ lymphocytes on muscle

Idiopathic inflammatory myopathies

•

Dermatomyositis ➢ Men > 40 yo ➢ Skin rash and muscle weakness ➢ Primary malignancies: ❖ Lung, prostate, female pelvic organs, breast or GI tract ➢ Precedes detection of tumor months to years Polymyositis: ➢ Primary malignancies: Lung, NHL

Generalized Musculoskeletal Disorders

984

Musculoskeletal Radiology

•

Imaging Findings [Figures 4-32-26 to 4-32-29] •

Figure 4-32-26

ST abnormalities ➢ ST thickening and edema ➢ Soft and periarticular calcification (IM > SQ) Articular abnormalities ➢ Radial subluxation or dislocation of IP of thumb (“floppy thumb”) = quite characteristic ➢ Erosions of multiple sites in hands ➢ Flexion deformities (MCP) ❖ “Swan neck” deformity

Polymyositis with soft tissue calcification

Figure 4-32-28

Figure 4-32-27

“Floppy thumb” of dermatomyositis/polymyositis Dermatomyositis with extensive soft tissue calcification • •

Figure 4-32-29

“Swan neck” deformity

•

MC flexion, PIP hyperextension and flexion at the DIP Most common inrheumatoid arthritis

Other imaging Findings •

•

RBS ➢ Increased accumulation at sites of calcification (Technetium and gallium) MR Imaging ➢ Muscle atrophy ➢ Fatty replacement ➢ Decreased SI correlating with activity of disease ➢ Increased SI on T2WI and STIR

Remember! PM, DM, Scleroderma, SLE, mixed CVD and overlap syndromes may all look alike ➢ ST calcification ➢ Articular and osseous abnormalities

Dermatomyositis with increased signal intensity in the right gluteal region Musculoskeletal Radiology

985

Generalized Musculoskeletal Disorders

•

Summary • •

• • •

Osteoporosis ➢ Most common metabolic disease ➢ Insufficiency fractures may mimic mets/myeloma Osteogenesis imperfecta ➢ Diagnosis of exclusion in young patient with osteopenia out of proportion to age ➢ Easily fractured and exuberant callous formation Neurofibromatosis ➢ Pencilling, “pseudarthrosis,”, posterior scalloping, thoracic meningocele Systemic lupus erythematosis ➢ Ulnar deviation without erosions (differential is Jacoud’s (poststreptococcal arthritis) Scleroderma ➢ CREST, acroosteolysis Inflammatory muscle disease ➢ Dermatomyositis and polymyositis ➢ Nonspecific findings (look like scleroderma) ➢ Must look for malignancy in these patients

References 1.

Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York, NY, Thieme Medical Publishers, 2001

Generalized Musculoskeletal Disorders

986

Musculoskeletal Radiology

Osseous Musculoskeletal Stress Injuries Thomas Lee Pope, Jr, MD, FACR • • • •

Outline Biomechanical considerations Historical perspective Epidemiology and clinical manifestations Anatomic approach with examples ➢ Stress injury “look alikes” • Avulsive and muscular “tug” injuries • Unusual activities causing stress injury • Recommended work-up •

“Bone Fatigue” 1922 - Muller ➢ Isolated a segment from the radius of a dog ➢ Created a fatigue fracture of the ulna • 1949/50 - Rutishauser/Majno ➢ First description of the histologic aspects of fatigue fractures Muller W. Bruns Beitr. klin. Chir. 127:251-290, 1922 R/M. Schweiz. med. Wschr. 79: 281-88, 1949. 6:333-42, 1950. •

Historical Perspective • • • • •

1855 - Breithaupt - Prussian military surgeon ➢ Clinical features of painful feet on long marches 1887 - Pauzet - army doc ➢ Exostoses on PE from bone proliferation soldiers’ feet 1897 - Stechow - Prussian guard ➢ First imaging in 36 cases of MT stress fractures 1905 – Blecher ➢ First femoral neck stress fracture 1921 – Deutschlander - German physician ➢ Comprehensive study of stress lesions in > 50 yo 1936 - Asal – German ➢ First large series of 590 stress fractures in German troops

•

Types of MSK stress injury Soft tissue injury ➢ Bruises ➢ Muscle tears ➢ Musculotendinous injury • Collagen injury ➢ Tendon and ligament strain/tears • Cartilage injury ➢ Chondral injury (hyaline and fibrocartilage) • Osseous abnormalities ➢ Stress reaction (sclerosis/lucency/periosteal reaction) ➢ Stress fracture • •

Stress injuries Most common in lower extremities Dissipation of ground reaction forces (GRF) (running, walking, marching, jumping) • Bone exposed to stress (ie load) and strain (ie deformation) with weightbearing • Factors influencing bone response: ➢ Bone geometry and bone density • Jumping and landing – GRF up to 12 X body weight

Musculoskeletal Radiology

987

Osseous Musculoskeletal Stress Injuries

•

How Do Stress Injuries Develop? • • • • •

Figure 4-33-1

“Wolff’s law” ➢ Bone remodels in DIRECT reponse to the forces applied to it Normally a happy “marriage/relationship” between osteoblasts and osteoclasts Increased stresses cause increased osteoclastic activity with transient weakening Transient weakening predisposes to “microdamage” Coalescence of “microdamage” – stress reaction or injury Cascade ➢ Fissures ➢ Microfractures ➢ Osteoblastic response (periosteal reaction or cancellous “clouding”) ➢ Coalescence ➢ Fatigue reaction/injury

• • • •

Epidemiology 20% of all injuries seen in sports medicine clinics Between 4.7% and 15.6% of injuries in runners 20-25% of stress injuries in tibia, fibula or MT Females: 49% with very irregular menstruation, 39% with irregular menstruation • Study of 320 athletes with stress injury: ➢ 69% runners ➢ 8% fitness class participants ➢ 5% racket sports ➢ 4% basketball players • Track athletes have highest incidence • • • •

Clinical Features of Stress Injury Pain associated with activity Pain resolves without activity Pain in characteristic location associated with activity PE: ST swelling, point tenderness

Grade 4 Stress injury of the tibia, pathological fracture of the fibula in patient with RA on steroids Condensation of cancellous bone Perpendicular to the long axis

•

Risk Factors for Stress Injury Intrinsic ➢ Low BMD ➢ Lower limb misalignment ➢ Muscle fatigue ➢ Weakness/strength imbalance ➢ Pathologic bone ➢ Menstrual/hormonal irregularities ➢ Genetic predisposition • Extrinsic ➢ Excess volume/intensity of training ➢ Change in training surface (density or topography) ➢ Worn out training shoes ➢ Inadequate nutrition ➢ Cigarette smoking

Osseous Musculoskeletal Stress Injuries

988

Musculoskeletal Radiology

•

Activities associated with Stress Injury Lower extremity ➢ Running ➢ Marching ➢ Soccer (pelvis) ➢ Basketball ➢ Skating (fibula) ➢ Jumping (pelvis) ➢ Swimming (tibia, MT) ➢ Ballet (pelvis, spine) • Upper extremity ➢ Baseball: ❖ Throwing-Humerus, scapula, olecranon, first rib ❖ Batting-Ribs ❖ Catching-Patella, tibia ➢ Javelin throwing-ulna ➢ Basketball ➢ Volleyball • • • •

Activities associated with Stress Injury Rowing, kayaking-Second through tenth ribs Running with hand held weights-Scapula Coughing-ribs Trapshooting-Coracoid process

•

Major categories of Stress Injury “Fatigue” fracture ➢ Abnormal muscular stress of torque ➢ Bone of normal elastic resistance • “Insufficiency” fracture ➢ Normal or physiologic activity ➢ Bone deficient in mineral or elastic resistance *****Imaging findings are similar • • • • • • •

Osteoporosis Metabolic disease Hyperparathyroidism Osteomalacia/Rickets Cushing’s disease Paget disease Diabetes mellitus

• • • • •

0 – Normal study 1 – Subtle periosteal edema (IR, FS T2-W images) 2 – Periosteal edema and increased marrow SI on FS T2-W images 3 – More extensive edema (T1-W and T2-W) 4 – Discrete fracture line visible on MR or on radiography

Risk factors - Insufficiency fractures

MR Grading System Stress injuries

[Figure 4-33-1]

Fredericson M, Bergman AG et al: Am J Sports Med 1995;23:472-481 • • • • • •

Anatomic approach Lower extremity: Foot (MT, navicular, calcaneus), tibia, femur Pelvis (Insufficiency and adolescent) Upper extremity Unusual causes of stress injury Mimics of stress injury Recommended workup

Musculoskeletal Radiology

989

Osseous Musculoskeletal Stress Injuries

•

Metatarsal stress fractures [Figures 4-33-2 to 4-33-5]

Figure 4-33-2

Second most common stress fracture site behind the tibia • First described in military recruits ➢ Flat flexible feet = MT stress fractures ➢ Cavus feet = tibial stress fractures • Distance runners and ballet dancers • In order: 2, 3, 1, 4, 5 • •

Navicular stress fracture [Figures 4-33-6 and 4-33-7] • • • • •

0.7%-2.4% of all stress fx’s Activities: Track and field (59%), Australian football (19%), Basketball (10%) First description: 1958 in greyhounds 1970 - humans Most common in runners Increasing dorsal midfoot pain radiating down medial arch Rx: Non-weight bearing cast (86% success rate)

Figure 4-33-3

Second MT fatigue fracture

Figure 4-33-4

Right 2nd MT fatigue fracture and 1 month f/u in 42 yo male

Figure 4-33-5

MR imaging of third MT fatigue fracture, soccer player

Sesamoid necrosis in ballet dancer Osseous Musculoskeletal Stress Injuries

990

Musculoskeletal Radiology

Figure 4-33-6 Figure 4-33-7

Classic navicular fatigue fracture [Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]

Figure 4-33-8

Classic fatigue fracture of calcaneus

Microangiopathic studies cadaveric feet…navicular supplied by both ant and post tibial arteries, enter at small “waist” of cortical bone and branch out to supply the medial and lateral 1/3… Central 1/3, under greatest stress with relative avascularity •

[Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]

Tibia stress fracture

Figure 4-33-9

Three major types ➢ Medial tibial plateau ➢ Tibial diaphysis ➢ Anterior mid tibia

• •

Medial tibial plateau [Figure 4-33-9] Less frequent than tibial diaphysis Often misdiagnosed as pes anserinus bursitis or tendoninitis • Less critical stress injury • Treatment ➢ Rest for 4-6 weeks ➢ Then return to full activity

Medial tibial fatigue fracture

Figure 4-33-10

Medial tibial stress syndrome “Shin splints”[Figure 4-33-10]

MR features of medial tibial stress syndrome (“shin splints”)

Musculoskeletal Radiology

991

Osseous Musculoskeletal Stress Injuries

Figure 4-33-11

Figure 4-33-12

“Aggressive” periosteal reaction in the tibia in fatigue fracture Diaphyseal fatigue fracture with histologic correlation from the AFIP fascicles • •

Figure 4-33-13

Tibial diaphyseal Fatigue Fracture[Figures 4-33-11 and 4-33-12] • •

Posteromedial border of tibia Tensile forces produced along anterior convex side, compressive forces along posterior concave margin 465 injuries causing exertional leg pain – 75% in posteromedial tibial diaphysis Difficult to tell stress injury from “shin splints” (medial tibial stress syndrome)

• • •

Anterior mid-tibia [Figure 4-33-13] Most common in jumping and leaping athletes Focal cortical thickening and sclerosis “Dreaded black line” ➢ Propensity to nonunion ➢ Risk of complete displaces fracture • Require more aggressive treatment

Diaphyseal fatigue fracture with the “dreaded black line”

Figure 4-33-14

Longitudinal Tibial Stress Fracture • • •

[Figure 4-33-14]

Devas 1960 Patients MAY not give h/o increased activity Saifuddin (Clin Rad 1994): ➢ Two cases ➢ Stress fx located superomedial to the nutrient foramen of the tibia ➢ Foramen weakens bone at this site? ➢ ? insufficiency fracture

Longitudinal stress fractures of the tibia

Longitudinal fatigue fracture

Craig et al, Henry Ford Hospital, Detroit, Skeletal Radiology, 2003 • Six cases • All female (age range - 15-69 yo) • Diagnosis made by finding cleft on multiple axial images • 5/6 patients had: ➢ Edema starting at level of the entrance of the nutritent vessel into the medullary cavity ➢ Vertical fx identified below this level on the anteromedial tibial cortex

Figure 4-33-15

Compression type of femoral neck fatigue fracture

Osseous Musculoskeletal Stress Injuries

992

Musculoskeletal Radiology

•

Femoral neck stress injury [Figures 4-33-15 and 4-33-16]

Figure 4-33-16

Any athlete (jogger/runner) with hip, thigh or groin pain • Two types ➢ Tension type: ❖ Superior cortex ❖ Older osteoporotic patients ➢ Compression type: ❖ Younger athletic patients • Treatment: 2-3 months non-weight bearing with gradual return •

Pelvic Stress Injury [Figures 4-33-17 to 4-33-21] Running increases risk of stress lesions in sacrum and ischial rami • Sacral fractures more common in young women (sx’s mimic sacroiilitis) ➢ Fatigue: ❖ Anteroinferior sacral wing unilaterally ➢ Insufficiency: ❖ Elderly women, irradiated women ❖ Often bilateral (“Honda” sign)

Fatigue fracture which progressed to complete femoral neck fracture

Figure 4-33-17

Figure 4-33-19

Sacral insufficiency fracture

Figure 4-33-18

Bilateral sacral insufficiency fractures (the “Honda” sign)

Figure 4-33-20 CT of previous figure on left showing the classic CT findings of right sacral insufficiency fracture

Figure 4-33-21

Symphysis pubis stress reaction in soccer player Bilateral sacral and symphyseal insufficiency fractures in elderly female S/P external beam radiation for cervical cancer. Note the bilateral nature of the sacral fractures Musculoskeletal Radiology

993

Osseous Musculoskeletal Stress Injuries

•

Adolescent Stress Lesions [Figure 4-33-22] • • • •

Figure 4-33-22

Muscular “tug” (avulsive) lesions (Classic lesion: Cortical desmoid) Avulsion injury and sequelae Tendons, ligaments stronger than bone in adolescents May mimic primary soft tissue neoplasm in acute setting May mimic primary bone neoplasm after healing has occurred

Recommended Workup - Suspected Stress Injury • •

Correlate clinical situation with imaging Think “stress injury” in the correct setting (you may be the only clinician who does so!!) • Initial study is the conventional radiograph • Young patient-CT or MR imaging • Elderly patient ➢ Typical site-CT or MR ➢ Unusual site-RBS as screen, then CT or MR

16 yo with bilateral healed avulsions whose diagnosis was made at age 42!

References 1. 2. 3. 4. 5.

Chamay A. Mechanical and morphological aspects of experimental overload and fatigue in bone. J Biomech 1970; 3:263-270. Craig JG, Widman D, van Holsbeeck M. Longitudinal stress fracture: patterns of edema and the importance of the nutrient foramen. Skeletal Radiol 2003; 32:22-27. Müller W. Bruns Beitr. klin. Chir. 127:251-290, 1922 Rutishauser E, Majno G. [Lesions of normal and pathological bones due to overstrain.]. Bull Schweiz Akad Med Wiss 1950; 6:333-342. Tschantz P, Rutishauser E. [The mechanical overloading of living bone: initial plastic deformations and adaptation hypertrophy]. Ann Anat Pathol (Paris) 1967; 12:223-248.

Osseous Musculoskeletal Stress Injuries

994

Musculoskeletal Radiology

Pelvis and Lower Extremity Trauma: An introduction Thomas Lee Pope, Jr, MD, FACR Figure 4-34-1

•

Outline Caveats ➢ Major highlights ➢ Not enough time to cover in depth ➢ You must read more on your own to supplement this lecture • Pelvic trauma • Acetabular trauma • Lower extremity trauma ➢ Femur ➢ Knee ➢ Ankle ➢ Foot ❖ Talus ❖ Calcaneus ❖ Classic fx’s

Radiographic anatomy [Figure 4-34-1] Major mechanisms of pelvic injury [Figure 4-34-2]

Pelvic Radiographic Anatomy, ip = iliopubic line, ii = ilioischial line, SIJ = sacroiliac joints

•

Most Popular Classifications Pelvic Ring Fractures

Figure 4-34-2

Tile ➢ Pelvic stability • Young-Burgess ➢ Degree of injury • Major divisions ➢ “Ring sparing” ➢ AP compression ➢ Lateral compression ➢ Vertical shear ➢ Complex

Major mechanisms of pelvic injury (Tony Wilson, Seattle Washington)

Young-Burgess vs Tile Classifications FRACTURE TYPE

YOUNG-BURGESS

TILE

Ring sparing

Not included

Type A

Anterior compression

AP compression Types I-III

B1 (B1, 1.1-1.3)

Lateral compression

Lateral compression Types I-III

B2 (B2, B2.1-2.2)

Vertical shear

Vertical shear

C (C1-3)

Musculoskeletal Radiology

995

Pelvis-Lower Extremities Trauma

•

Young-Burgess Classification Pelvic Ring Fractures

Figure 4-34-3

Lateral compression (Most common) ➢ Types I and II • Anteroposterior (AP) compression ➢ Types I, II and III • Vertical shear • • • •

Lateral compression Most common mechanism of pelvic injury Lateral blow to the side of the pelvis Three types depending on severity “KEYS TO THIS INJURY:” ➢ Horizontal fx’s of pubic ramus/rami ➢ Crush (buckling) fx of sacrum

Lateral compression Type I injury-STABLE (Note the disruption of the sacral foraminal (arcuate) lines-arrows)

Figure 4-34-4

Lateral compression - Young-Burgess classification [Figures 4-34-3 to 4-34-5] • •

I – Ipsilateral sacral compression fx (stable) II- I + associated iliac wing fx ➢ Rotationally unstable Vertically stable • III – I + II with contralateral “open book” appearance (“windswept pelvis”)

“Windswept pelvis” - Lateral compressionipsilateral - AP compressioncontralateral [Figure 4-34-6] • • •

Lateral compression Type I injury

Severe anterior force Internal rotation of ipsilateral hemipelvis with external rotation of contralateral hemipelvis “Rolled over” look

Figure 4-34-5

Figure 4-34-6

“Windswept” pelvis Lateral compression Type I injury • • • • • • •

AP Compression Blows to front of pelvis MVA Three types depending on which ligaments involved Increases volume of pelvis Major risk = hemorrhage Often brain/abdominal injuries KEYS TO THIS INJURY: ➢ Vertical fx inf pubic rami (one or both sides) ➢ > 50% post acetabular wall ➢ < 10% sacral fx

Pelvis-Lower Extremities Trauma

996

Musculoskeletal Radiology

AP Compression - Young-Burgess classification • •

[Figures 4-34-7 to 4-34-9]

Figure 4-34-7

Type I = SP disrupted (all ligs intact) Type II ➢ SP diastasis < 2.5 cm ➢ Torn ligaments: ❖ SP, SS, ST and ventral (anterior SI) • Type III ➢ SP diastasis > 2.5 cm ➢ Torn ligaments: ❖ SP, ST, SS and both ventral (anterior) and dorsal (posterior) SI AP compression Type II injury

Figure 4-34-8

Figure 4-34-9

AP Compression injury with vertical fracture of the sacrum

AP compression injury (Note marked widening of the symphysis pubis) •

Figure 4-34-10

AP Compression, Type II • • • • •

Disruption of sacrospinous, sacrotuberous and ventral (anterior) SIJ ligaments Diastasis of SP > 2.5 cm Diastasis of both SIJ anteriorly “Open book” appearance Rotationally unstable Vertically and posteriorly stable

• • • • •

Type I and II Disruption of all SIJ ligaments Complete separation of iliac wing from sacrum Complete pelvic instability Rotationally, vertically and posteriorly unstable

AP Compression, Type III

• • •

Vertical Shear Fall from height or head and back trauma Least common KEYS TO THIS INJURY: ➢ Disruption of SP or SIJ ➢ Cephalad or caudad displacement of pelvis (best seen on OUTLET film) • Rotationally, vertically and posteriorly UNSTABLE • •

Vertical Shear [Figures 4-34-10 and 4-34-11] Disruption of SP, ST, SS, and ant/post SIJ ligaments Characteristics ➢ Vertical pubic rami fractures ➢ SIJ disruption +/- adjacent fractures • Hemipelvis vertically (cranially) displaced

Musculoskeletal Radiology

997

Vertical shear injury – Note vertical migration of left hemipelvis

Pelvis-Lower Extremities Trauma

•

Clues

Figure 4-34-11

Horizontally oriented pubic fracture ➢ Think lateral compression, look closely at sacral arcuate lines • Vertically oriented pubic fracture ➢ With AP displacement, think AP compression ➢ With vertical displacement, think vertical shear • Posterior wall acetabular fx ➢ Think AP compression • Central acetabular fx’s ➢ Think lateral compression •

Complications Pelvic Ring Disruption [Figure 4-34-12] Mortality 5%-50% (reflect severity) ➢ AP compression – 26% ➢ Vertical shear – 25% ➢ Complex – 17% ➢ Lateral compression – 13% • Head injury and hemorrhage (internal iliac branches or superior gluteal artery near sciatic notch) • • •

Acetabular Injury Significant trauma (MVA, falls) Associated pelvic ring fractures Pattern of acetabular injury depends on: ➢ 1. Position of femoral head at time of traumatic event ❖ FH externally rotated = anterior column ❖ FH adducted = acetabular roof ❖ FH abducted = forces transmitted inferiorly ➢ 2. Direction of force ❖ Anterior force = posterior wall and column ❖ Lateral force = medial acetabular wall (transverse type) • Therapy depends on proper classification

•

Soft tissue findings of vertical shear injury

Acetabular Columns Letournel and Judet Anterior ➢ Iliac wing to anterior acetabulum ➢ Incorporates superior pubic ramus • Posterior ➢ Sciatic notch to posterior acetabulum to ischium

Figure 4-34-12

“Inverted Y” column principle Radiographic Evaluation Acetabulum • •

[Figure 4-34-13]

AP pelvis Judet views ➢ 45 degree oblique views ➢ Right ❖ RPO = Iliac oblique ❖ RAO = Obturator oblique ➢ Left ❖ LPO = Iliac oblique ❖ LAO = Obturator oblique

Soft tissue complications of pelvic ring fractures

Figure 4-34-13

• •

Acetabular fractures Letournel and Judet classification, 1993 Ten different patterns ➢ Five elementary (run in single plane) ➢ Five associated (combination of elementary) • Difficult to remember • Most common: ➢ Posterior wall ➢ Transverse with posterior wall ➢ Both column (most common type) • Add T-shaped and transverse = 90% Pelvis-Lower Extremities Trauma

Pelvic CT anatomy (see Harris et al: AJR 2004;182:1363-75) 998

Musculoskeletal Radiology

•

Letournel and Judet, 1993

Figure 4-34-14

Elementary (simple) fractures ➢ Posterior wall ➢ Posterior column ➢ Anterior wall ➢ Anterior column ➢ Transverse • Complex (associated) fractures ➢ T-shaped ➢ Posterior wall posterior column ➢ Transverse posterior wall ➢ Anterior with posterior hemitransverse ➢ Both columns

CT patterns of acetabular fractures Hunter, RCNA 1997 •

• • •

Axial CT image through roof of acetabulum ➢ Column Fracture ➢ Transverse Fracture ➢ Wall Fracture ➢ Normal

Wall Fracture [Figures 4-34-14 and 4-34-15]

• • • •

Weight-bearing columns of acetabulum not disrupted Posterior wall most common Major complications: ➢ Hip joint instability ➢ Osteonecrosis

Transverse Fractures [Figures 4-34-16 and 4-34-17] Medial and lateral components Fx line anterior to posterior Separates “walls” from “columns” CT = sagittal plane

Figure 4-34-15 Posterior wall acetabular fracture

Figure 4-34-16

CT of posterior wall fracture (Same patient )

T-type transverse fracture Musculoskeletal Radiology

999

Pelvis-Lower Extremities Trauma

• • • • •

Column Fracture

• •

Figure 4-34-17

Craniocaudad (coronal) direction Front and back halves Ant/post only or both Associated with other fx’s (post column, post wall) Conceptualize: Grasp ASIS could move acetabulum freely Obturator fx = column type or T-shaped fracture

Questions To Ask Yourself… • • • • • • •

Obturator ring fx? ➢ T-shaped or column fx Ilioischial line disrupted? ➢ Posterior column or transverse fx patterns Iliopectineal line disrupted? ➢ Anterior column or transverse-type fx’s Is iliac wing above acetabulum fractured? ➢ Fracture of anterior column Is the posterior wall fractured? ➢ Isolated or combo with post column or transverse fx’s Is “spur” sign present? ➢ Almost assuredly “both column” fracture

CT of T-type transverse fracture with Sagittal (lower left) and Coronal (lower right) reconstruction

Hip Trauma

• • • • •

Dislocations Hip fx’s ➢ Common injury in multi-trauma ➢ Common in the elderly ❖ Osteoporosis and cerebrovascular disease ❖ Prone to falls

Hip Dislocations 5% of all dislocations High energy trauma (MVA, MCA, etc.) ~90+% posterior Commonly associated with femoral shaft, patella and post acetabular fx’s Clinically ➢ Limb shortening, internally rotated and adducted ➢ 10-15% transient sciatic nerve palsy (direct impingement)

•

Hip Fractures Intracapsular ➢ Subcapital ➢ Mid cervical ➢ Basicervical • Extracapsular ➢ Intertrochanteric ➢ Subtrochanteric • Femoral neck fx’s 3-6X > women • Intertroch fx = frequency • • • •

Subtrochanteric Fractures Fracture line extends between LT and point 5 cm distally Direct trauma Older patient, less force required High incidence of malunion or nonunion ➢ ? secondary to greater proportion of cortical bone to trabecular bone in this region • Rx: Intramedullary rod

Pelvis-Lower Extremities Trauma

1000

Musculoskeletal Radiology

•

Knee Injury Soft tissue signs ➢ ST swelling, lipohemarthrosis • Fractures ➢ *Supracondylar ➢ *Condylar ➢ *Tibial plateau ➢ Impaction (lateral femoral condylar notch) ➢ Tibial avulsion fractures ➢ Segond fracture ➢ Patellar fractures and dislocation • • • • • •

Valgus stress, 85% involve lateral tibial plateau Fat-fluid level Schatzker classification (6 types) Depression = cartilage thickness (3mm) Meniscal injury ~ 50% Rx: Lateral buttress plate and screw

• • • • • •

I=split fx (younger) II=split + depression of LTP (older) III=depression - splitting IV=MTP +/- depression V=split fx through MTP and LTP VI=dissociation of TP from underlying diaphysis

Tibial Plateau Fractures

Schatzker Classification

• • • •

Tibial Plafond (pilon) Fracture High energy axial loading (talus on tibial plafond) Ankle and distal tibial metaphyseal fx, intraarticular 20%-25% open Associated injuries: ➢ Compartment syndrome, vertebral compression fractures • Often require ORIF • Post-traumatic arthritis common • • • • •

Maisonneuve fracture

• • •

External rotation of ankle Fibular fx Serious injury Requires ORIF with screws Removed 8-12 weeks after injury

Ankle Fractures

•

Common injuries Soft tissue changes (STS, effusion) Classification schemes ➢ Lauge-Hansen ❖ Difficult to remember ❖ Not very reproducible ➢ Danis-Weber (AO) ❖ Easy to remember ❖ Reproducible

Danis-Weber Classification • •

Type A: Horizontal avulsion fx below mortise, stable, Rx: Closed reduction and casting (without displaced MM fx) Type B: Spiral fibular fx – level of mortise, external rotation, stable or unstable, Rx: Closed reduction unless fragments displaced Type C: Above mortise, disruption of lig attachment of tibia/fibula distal to fx, unstable, Rx: ORIF

Musculoskeletal Radiology

1001

Pelvis-Lower Extremities Trauma

• • •

Ankle Fractures

• • •

Talar Neck Fractures [Figure • • •

Figure 4-34-18

Key is re-establishing tibiotalar joint congruence Mortise view important 1-2 mm displacement of talus in mortise dramatically changes contact area and pressure 40% decrease in contact area with 1mm lateral talar shift 4-34-18]

3%-5 % of foot fractures Dorsally directed force on braced foot (“aviator’s astragulus” – WWI pilots), now most commonly MVA Main blood supply of talar body enters neck through sinus tarsi and proceeds retrograde to supply body Neck fx’s compromise vascularity Hawkins sign (no AVN) Hawkins II talar neck fracture

Talar Neck Fractures - Hawkins classification [Figure 4-34-19] Figure 4-34-19

Figure 4-34-20

Risk of AVN: I = 10%; II = 40%; III = 90%; IV = 100% Hawkins classification of talar neck fractures (Bohndorf K, Imhoff H, Pope T: Synopsis of MSK Imaging: A Multimodality Approach, Thieme) • • • •

Snowboarder’s fx Eversion Lat process caught between LM and calcaneus May be caused by inversion and dorsiflexion

• • • • • • • • •

Most frequently fractured tarsal bone (60% of all fx’s) 2% of all fx’s in adults 5% - 9% bilateral 10% LS compression fx’s Peroneal tendon entrapment or compartment Intraarticular 70%, extra-articular 30% Most common EA = calcaneal body fx Anterior process = 15% Difficult to treat if displaced

Lateral Process of Talus fx [Figure

Calcaneal Fractures [Figure

4-34-20]

4-34-21]

Figure 4-34-21

Lateral process of talus fracture (Snowboarder’s fracture)

Coronal reconstruction of CT of calcaneal fracture

CT of calcaneal fracture

Pelvis-Lower Extremities Trauma

1002

Musculoskeletal Radiology

• •

Classification systems Calcaneal Fractures

Figure 4-34-22

Bohler (1931) Essex-Lopresti (1952) ➢ Intraarticular vs extra-articular ➢ Types: Tongue and Joint depression • Rowe (1963) • Saunders – CT classification (1992) • Others: Hanover, Rowe, Palmer, Souer and Remy •

Sanders Classification • • • • • • •

I = Non-displaced ➢ Non-operative II = 2 parts (split) ➢ ORIF III = 3 parts (split and depression ➢ ORIF IV = Comminuted ➢ Defies open reduction Measures height of PF A = most cephalic point of tuberosity to posterior border of subtalar joint B = posterior border of subtalar joint to anterior process Normal: 20 - 40º Anatomy of the tarsal joints (Bohndorf K, Imhoff H, Pope T: Synopsis of MSK Imaging: A Multimodality Approach, Thieme)

•

Foot Injuries 5th MT ➢ Avulsion (pseudo-Jones or tennis fracture) ➢ Jones ➢ Stress fx (fatigue or insufficiency) • LisFranc • • • •

Figure 4-34-23

Jones Fracture Transverse fx 2-3 cm distally Displaces on weight bearing 35%-50% persistent non-union

Google Search: Sponsored links (Jones Fracture lawsuits-Recover medical expenses-Find attorneys and help nationwide-personal-injury-lawyer.com)

• • • • •

Lisfranc [Figures

•

4-34-22 and 4-34-23]

Napoleonic surgeon Developed quicker technique of forefoot amputation for gangrene Faster wiithout having to cut bone Injury in foot never described by him Commonly misdiagnosed

Divergent (left) and Homolateral (right) types of LisFranc injury

Summary • • • • • •

Reviewed major pelvic, acetabular and lower extremity traumatic lesions Meant as an introduction Supplement with reading and study Xerox major classifications of fractures Have readily available in MSK reading area Consult classifications frequently Supplement clinical experience with personal reading EVERY DAY

References 1. 2. 3. 4.

Bohler L: Diagnosis, pathology, and treatment of fractures of the os calcis. J Bone Joint Surg 13:75-89, 1931. Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York, NY, Thieme Medical Publishers, 2001 Borrill J, Funk L, Deakin S. Orthoteers: The guiding light in orthopaedic education. 2006.British Orthopaedic Association.. eMedicine (James WD, Adler J, Lutsep HL, Lorenzo CT, Lin EC, Ho SSW, Roy H, Gellman H, Meyers AD eds)

Musculoskeletal Radiology

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5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16.

1996-2005 eMedicine.com, Inc . WedMD. Essex-Lopresti P. The mechanism, reduction technique, and results in fractures of the os calcis. Br J Surg 1952; 39:395-419. GE: Healthcare reimagined. Copyright General Electric Company 1997-2006. GE Medical Systems. 2006. Harris JH, Jr., Lee JS, Coupe KJ, Trotscher T. Acetabular fractures revisited: part 1, redefinition of the Letournel anterior column. AJR Am J Roentgenol 2004; 182:1363-1366. Hunter JC, Brandser EA, Tran KA. Pelvic and acetabular trauma. Radiol Clin North Am 1997; 35:559-590. Letournel E, Judet R. Fractures of the acetabulum, 2nd ed. Heidelberg, Germany: Springer-Verlag,1993 MacLeod M, Powell JN. Evaluation of pelvic fractures. Clinical and radiologic. Orthop Clin North Am 1997; 28:299-319. Palmer I. The mechanism and treatment of fractures of the calcaneus: open reduction with the use of cancellous grafts. J Bone Joint surg 1948;30-A(1):2-8 Perry DC, DeLong W. Acetabular fractures. Orthop Clin North Am 1997; 28:405-417. Rowe CR, Sakellarides HT, Freeman PA, et al. Fractures of the os calcis: long term follow-up study of 146 patients. JAMA 1963;184:920-923 Sanders, R., Hansen, S.T. & McReynolds, I.S.: Fractures of the calcaneus, in Jahss, M. (Ed.): Disorders of the foot and ankle, Philadelphia, W.B. Saunders, 1991. p. 2326-2354. Souer, R. & Remy, R.: Fractures of the calcaneus with displacement of the thalamic portion. J Bone Joint Surg [Br] 57: 413-421, 1975. Wheeless' Textbook of Orthopaedics. Copyright 1996-2005 Data Trace Publishing Company. Duke University Medical Center's Division of Orthopaedic Surgery. Data Trace Internet Publishing Company. 2006.

Pelvis-Lower Extremities Trauma

1004

Musculoskeletal Radiology

Musculoskeletal Seminar I Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY 15 year old male with longstanding hindfoot pain

UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS LESIONS WITH SEQUESTRA-LIKE APPEARANCE • • • • •

Osteomyelitis Metastasis Fibrosarcoma/ Malignant Fibrous Histiocytoma (MFH) Lymphoma Osteoblastoma

UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS CORTICAL LUCENCY/CENTRAL CALCIFICATION • • • • • • •

Langerhans cell histiocytosis Osteoid osteoma Brodie abscess

UNKNOWN CASE #1: FINDINGS

• • • • • • •

Diffuse sclerosis of calcaneus CT-solid periosteal reaction causing sclerosis on radiographs Subchondral low density lesion with central calcification Diffuse edema/focal lesion related to posterior subtalar joint with joint effusion

T1

UNKNOWN CASE #1: OSTEOID OSTEOMA 10–25 years, M>F (3:1) Night pain relieved by ASA Lytic nidus (<1.5-2.0 cm); central calcification Intracortical-extensive periosteal reaction Intramedullary-often subtle little sclerosis Lymphofollicular synovitis CT/MRI important for surgical guidance

T2

UNKNOWN CASE #1: OSTEOID OSTEOMA TREATMENT OPTIONS • • • •

Surgical excision Percutaneous removal Percutaneous ablation Medical

Musculoskeletal Radiology

GRE 1005

Musculoskeletal Seminar I

•

UNKNOWN CASE #2: HISTORY 27 year old female with 5 years chronic left hip pain

UNKNOWN CASE #2: OSSEOUS LESIONS BOTH SIDES OF JOINT • • • • •

Arthritis Infection PVNS Synovial chondromatosis Amyloidosis

• • • • • • • •

Young males-3rd to 4th decade Synovial proliferation with hemosiderin deposition Extrinsic erosions common in hip Joint space normal; limited osteopenia Can appear like OA in hip Joint fluid-nodular thickening at arthrography MRI often characteristic low intensity, fluid foci RX -synovectomy-adjuvant radionuclide therapy

•

5 year old male with mild midfoot pain

UNKNOWN CASE #2: PVNS

Proton Density

T2

UNKNOWN CASE #3: HISTORY

Symptomatic side • • • •

Fragmentation Sclerosis Collapse AVN, trauma, normal

• • • • • •

Described 1908 M>F; 4–6:1; 3–7 years old Often asymptomatic Unilateral 75–80% AVN ? Treatment-immobilization

UNKNOWN CASE #3: OSTEOCHONDROSES

UNKNOWN CASE #3: KOHLER DISEASE

Musculoskeletal Seminar I

Asymptomatic side

1006

Musculoskeletal Radiology

•

13 year old female 1 year of pain now severe and worsening

• • • • •

Subacute osteomyelitis Medullary or cortical lucency surrounding sclerosis Channel-like lesion may extend to or across growth plate Staph aureus MRI or CT to evaluate soft tissue extension

•

30 year old female with progressive clubbing of fingers

UNKNOWN CASE #4: HISTORY

UNKNOWN CASE #4: BRODIE ABSCESS

T2

UNKNOWN CASE #5: HISTORY

UNKNOWN CASE #5: ACROOSTEOLYSIS BANDLIKE: DIFFERENTIAL DIAGNOSIS • • • •

Hyperparathyroidism Polyvinyl chloride Hajdu-Cheney syndrome Post-traumatic

UNKNOWN CASE #5: WORMIAN BONES: DIFFERENTIAL DIAGNOSIS • • • • • •

Normal Cleidocranial dysplasia Cretinism Osteogenesis imperfecta Hypophosphatasia Pyknodysostosis

Musculoskeletal Radiology

1007

Musculoskeletal Seminar I

• • • • •

Autosomal dominant; described 1948 Bathrocephaly, wormian bones, open sutures Acroosteolysis Poor dentition Osteoporosis

•

4 year old male with left hip pain

UNKNOWN CASE #5: HAJDU-CHENEY SYNDROME

UNKNOWN CASE #6: HISTORY

UNKNOWN CASE #6: DIFFERENTIAL DIAGNOSIS EPIPHYSEAL LESIONS • • • • • • •

Chondroblastoma Giant cell tumor (GCT) Subchondral cyst/Intraosseous ganglion Infection Langerhans cell histiocytosis (LCH) Osteoid osteoma/osteoblastoma Clear cell chondrosarcoma

• • •

Lytic lesion epiphysis and metaphysis Small amount surrounding sclerosis MRI and CT – no joint fluid or calcification

UNKNOWN CASE #6: FINDINGS

UNKNOWN CASE #6: EOSINOPHILIC GRANULOMA (LCH) • • • • • •

5–15 years; M:F-2:1 95% of patients Caucasian Solitary 67% Flat bones involved-70% Lytic hole within hole appearance Diaphysis (58%), metadiaphysis (18%), metaphysis (28%), epiphysis (2%)

Musculoskeletal Seminar I

1008

Musculoskeletal Radiology

Musculoskeletal Seminar II Mark D. Murphey, MD •

62 year old male with polyarticular joint pain

• • •

Asymmetric erosive arthritis hands and feet: MCP, and IP joints New bone formation Limited osteopenia

UNKNOWN CASE #1: HISTORY

UNKNOWN CASE #1: FINDINGS

UNKNOWN CASE #1: SERONEGATIVE SPONDYLOARTHROPATHY CHARACTERISTICS • • • •

Asymmetry Bone Production Less juxtaarticular osteopenia Distribution

UNKNOWN CASE #1: PSORIATIC ARTHRITIS POSSIBLE PRESENTATIONS • • • • •

DIP and PIP joints Arthritis mutilans Oligoarthritis or ray distribution Rheumatoid like (rare) Sacroiliitis/spondylitis

Musculoskeletal Radiology

1009

Musculoskeletal Seminar II

•

42 year old female with right low back pain

• • •

Unilateral destruction on both sides right sacroiliac joint Thickening of iliacus muscle Focal sclerotic fragments in joint

• • •

Unilateral-Infection, RA, Gout, Psoriatic, Reiter Bilateral asymmetric-RA, Gout, Psoriatic, Reiter Bilateral symmetric-AS, Enteropathic, Psoriatic, Reiter, RA

•

1 year old female with hand and foot pain

UNKNOWN CASE #2: HISTORY

UNKNOWN CASE #2: FINDINGS

UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS SACROILIITIS

UNKNOWN CASE #3: HISTORY

• •

UNKNOWN CASE #3: FINDINGS Soft tissue swelling about several fingers Periosteal reaction along several rays: phalanx hand, metacarpal and metatarsal

UNKNOWN CASE #3: DIFFERENTIAL DIAGNOSIS – DACTYLITIS •

• • •

Infection ➢ Pyogenic ➢ Unusual organism-TB Sickle cell anemia Thermal injury

UNKNOWN CASE #3: SICKLE CELL ANEMIA Musculoskeletal changes • Osteomyelitis (salmonella) • Avascular necrosis (AVN) • H-type vertebrae • Osteopenia • Diffuse sclerosis

Musculoskeletal Seminar II

1010

Musculoskeletal Radiology

•

UNKNOWN CASE #4: HISTORY 11 year old male with ankle pain after previous fracture

5 months after initial fracture • • • •

25%–33% growth sequelae Only 10% important Follow for 2 years-X-rays Look for bowing/shortening

• • • •

Initial Salter-Harris IV fracture without good reduction Subsequent lateral bowing of fibula and tibia Epiphyseal plate irregular CT/conventional tomography-osseous bar bridging plate

•

38 year old male with calf pain and mass, no history of trauma

UNKNOWN CASE #4: SALTER-HARRIS FRACTURE

UNKNOWN CASE #4: FINDINGS

UNKNOWN CASE #5: HISTORY

T2

T1

Musculoskeletal Radiology

1011

Musculoskeletal Seminar II

•

UNKNOWN CASE #5: FINDINGS • • •

Nonspecific enhancing inflammation and edema in calf with more focal mass medially Biopsy-soft tissue osteosarcoma 3 weeks later early calcification on CT predominantly peripheral 4 weeks later thick rind of calcification peripherally

UNKNOWN CASE #5: MYOSITIS OSSIFICANS (HETEROTOPIC BONE FORMATION) • • • • •

No history of trauma 25% Soft tissue mass Subsequent calcification Zonal phenomenon X-ray and path Follow-up for maturation

•

14 year old female with enlarging right foot mass

UNKNOWN CASE #6: HISTORY

UNKNOWN CASE #6: DIFFERENTIAL DIAGNOSIS CALCIFIED SOFT TISSUE MASS • • • • • • • •

Myositis ossificans Gout, collagen vascular disease Hyperparathyroidism, tumoral calcinosis Hemangioma Soft tissue chondro/osteosarcoma Synovial sarcoma

UNKNOWN CASE #6: FINDINGS • • • • •

Soft tissue mass plantar aspect mid to forefoot Faint calcification-CT and mag views; smooth erosion of 2nd and 3rd metatarsals Large soft tissue mass on MRI mildly heterogeneous and hyperintense T2W

UNKNOWN CASE #6: SYNOVIAL SARCOMA

• •

20–40 years old 68% lower extremity – particularly knee Most begin periarticular (< 10% intraarticular) Biphasic – epithelioid and spindle cell element on histo (also monophasic ) Radiographs – soft tissue mass, joint effusion (10–20%), calcification (30%), erosion or destruction adjacent bone Metastases-lungs and lymph nodes

Musculoskeletal Seminar II

1012

Musculoskeletal Radiology

Musculoskeletal Seminar III Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY 63 year old woman with vague calvarial pain

UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS – LYTIC SKULL LESIONS • • • • • • • •

Langerhans cell histiocytosis Metastases Myeloma Paget disease Brown tumor

UNKNOWN CASE #1: FINDINGS Focal skull lytic lesions: frontal and parieto-occipital Bone scan – multifocal area increased activity Radiographs – multiple lesions trabecular thickening

• • • •

Common – 3% of people over 40 years Lytic, blastic or mixed phases Most frequent to involve: spine, skull, pelvis Trabecular thickening – bone enlargement

• • • • •

Osseous deformity Fractures Neurologic symptoms Arthropaty Neoplasm

PAGET DISEASE

PAGET DISEASE: COMPLICATIONS

Musculoskeletal Radiology

1013

Musculoskeletal Seminar III

•

UNKNOWN CASE #2: HISTORY 14 year old boy with thigh pain

T1

T2 • • • •

Cortical scalloping – femur Hair-on-end periosteal reaction Broad based soft tissue mass No medullary involvement

• • • •

Most chondroblastic 85% diaphysis femur/tibia Same age group as conventional osteosarcoma Better prognosis

•

55 year old man with hip pain

UNKNOWN CASE #2: FINDINGS

UNKNOWN CASE #2: PERIOSTEAL OSTEOSARCOMA

UNKNOWN CASE #3: HISTORY T1

UNKNOWN CASE #3: FINDINGS • • • • •

Osteopenia of left hip MRI – decreased intensity left proximal femur T1W, and diffuse increased signal T2W No focal defects on MRI; effusion Enhances with gado; hot on bone scan Returns to normal in several months

Musculoskeletal Seminar III

T2 1014

Musculoskeletal Radiology

UNKNOWN CASE #3: TRANSIENT OSTEOPOROSIS HIP / BONE MARROW EDEMA SYNDROME • • • • • •

Middle aged males Spontaneous pain; worsened by weight-bearing Symptoms regress 2–6 months Migratory form may recur at nearby joint Cause unknown-bone marrow edema Relationship to AVN ?

•

82 year old man with slowly enlarging mass in the thigh

UNKNOWN CASE #4: HISTORY

T2 T1

UNKNOWN CASE #4: DIFFERENTIAL DIAGNOSIS – CALCIFIED SOFT TISSUE MASS • • • • • • •

Myositis ossificans Aneurysm Lipoma/liposarcoma Soft tissue osteosarcoma/chondrosarcoma Synovial sarcoma

UNKNOWN CASE #4: FINDINGS

• • •

Large mass thigh with mineralization calcification/ossification MRI/CT: ➢ Fat component ➢ Hemorrhagic component ➢ Myxoid component

UNKNOWN CASE #4: MYXOID LIPOSARCOMA

•

Myxoid variety most common liposarcoma (40–50%) Intermediate grade See lipomatous components with CT/MRI (40–50%) (We believe 90%-95% by MR) Mineralization not rare in liposarcoma

Musculoskeletal Radiology

1015

T1 GD

Musculoskeletal Seminar III

•

40 year old female with arthralgias

• • •

Interphalangeal joint subluxations No erosions Osteopenia

UNKNOWN CASE #5: HISTORY

UNKNOWN CASE #5: FINDINGS

UNKNOWN CASE #5: DIFFERENTIAL DIAGNOSIS SUBLUXATIONS/NO EROSIONS • • • • • •

Systemic Lupus Erythematosis (SLE) Mixed Connective Tissue Disease (MCTD) Juvenile chronic arthritis Ehlers-Danlos Jaccoud arthropathy

UNKNOWN CASE #5: SYSTEMIC LUPUS ERYTHEMATOSIS Musculoskeletal changes ➢ Deforming nonerosive arthropathy ➢ Tendon rupture ➢ Avascular necrosis (AVN) ➢ Joint and bone infection ➢ Acrosclerosis

Musculoskeletal Seminar III

1016

Musculoskeletal Radiology

•

UNKNOWN CASE #6: HISTORY 37 year old female with progressive ankle deformity ➢ What is the underlying disease? ➢ What process involves the ankle subsequently? ➢ How can the processes be correlated?

• • • • • • •

Langerhans cell histiocytosis Enchondromatosis Fibrous dysplasia Hereditary multiple exostoses Paget disease Neurofibromatosis (Type 1) Angiomatous lesions

• • •

Cause – pain sensation vs. neurovascular Destruction, debris, density increase, disorganization Diabetes, syphilis, cord-damage-syrinx

DIFFERENTIAL DIAGNOSIS – BENIGN POLYOSTOTIC LESIONS

UNKNOWN CASE #6: NEUROPATHIC JOINT

• • •

UNKNOWN CASE #6: FINDINGS

•

Lytic expansile benign appearing polyostotic lesions Fibula, femur, metatarsal Subsequently ankle-fragmentation, debris, destruction, increased density

UNKNOWN CASE #6: FIBROUS DYSPLASIA Endocrine Abnormalities • Sexual precocity • Cushings • Acromegaly 5 years after initial images • Hyperthyroidism • Diabetes mellitus (hypothalamic dysfunction) • Diagnosis case #6-fibrous dysplasia (polyostotic) with neuropathic ankle due to diabetes mellitus

Musculoskeletal Radiology

1017

Musculoskeletal Seminar III

Musculoskeletal Seminar IV Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY Several patients with various wrist subluxation patterns: Match with pattern ➢ Lunate ➢ Perilunate ➢ Barton fracture/subluxation

Musculoskeletal Seminar IV

1018

Musculoskeletal Radiology

• •

Perilunate – 75%, usually with transscaphoid fracture rest of carpus-dorsal Lunate – 25% lunate rotated volar, capitate remains aligned to radius

• •

Barton – fracture of dorsal rim of radius with dislocation of carpus Reverse Barton – fracture of volar rim of radius – with dislocation of carpus

•

31 year old female with underlying systemic disorder

• • •

Dysplastic changes thoracolumbar junction Short segment scoliosis Posterior vertebral body scalloping

UNKNOWN CASE #1: WRIST SUBLUXATIONS

UNKNOWN CASE #1: WRIST SUBLUXATIONS

UNKNOWN CASE #2: HISTORY

UNKNOWN CASE #2: FINDINGS

Musculoskeletal Radiology

1019

Musculoskeletal Seminar IV

UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS POSTERIOR VERTEBRAL SCALLOPING • • • • • • •

Normal variant L4–5 Neurofibromatosis (Type I) Tumor/increased intraspinal pressure Achondroplasia Acromegaly Ehlers-Danlos, Marfan , Osteogenesis Imperfecta Mucopolysaccharidosis

UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS DYSPLASTIC THORACOLUMBAR JUNCTION • • • •

Neurofibromatosis (Type 1) Cretinism Idiopathic Achondroplasia

UNKNOWN CASE #2: NEUROFIBROMATOSIS I MUSCULOSKELETAL MANIFESTATIONS • • • • • • •

Cranium-enlarged empty orbit, left lambdoid suture defect Spine – scoliosis, posterior vertebral scalloping, lateral meningocoeles Pseudoarthrosis (tibia), bowing, fractures Ribbon ribs Neurofibroma – 5% malignant degeneration Localized gigantism Multiple nonossifying fibromas

•

13 year old boy with bilateral hip pain

UNKNOWN CASE #3: HISTORY

T1

UNKNOWN CASE #3: DIFFERENTIAL DIAGNOSIS IRREGULAR EPIPHYSES (MULTIPLE) • • • • • • •

Normal variant Avascular necrosis (AVN) Hypothyroidism Epiphyseal dysplasia Trevor disease Mucopolysaccharidosis

UNKNOWN CASE #3: FINDINGS • •

Multiple irregular epiphyses ➢ Bilateral femora ➢ Right humerus Delayed skeletal maturation Changes of slipped capital femoral epiphysis (SCFE)

Musculoskeletal Seminar IV

1020

T2 Musculoskeletal Radiology

• • • • •

UNKNOWN CASE #3: CAUSES OF SCFE Idiopathic Rickets-renal Trauma, obesity Hypothyroidism, hypoparathyroidism Radiation

UNKNOWN CASE #3: MUSCULOSKELETAL CHANGES IN HYPOTHYROIDISM • • • • • •

Delayed skeletal maturation, Wormian bones Epiphyseal dysgenesis with osteoarthritis Thoracolumbar junction gibbus SCFE; ligamentous laxity Osteoporosis; soft tissue calcification Soft tissue edema, carpal tunnel syndrome

• • • • •

Ossification from multiple sites Femoral, humeral centers and talus Not due to vascular insufficiency May disappear with treatment May lead to premature osteoarthritis (OA)

•

45 year old man with wrist pain

UNKNOWN CASE #3: EPIPHYSEAL DYSGENESIS

UNKNOWN CASE #4: HISTORY

UNKNOWN CASE #4: DIFFERENTIAL DIAGNOSIS CHONDROCALCINOSIS • • • •

CPPD deposition/arthropathy Hemochromatosis Hyperparathyroidism All others poor association

Musculoskeletal Radiology

1021

Musculoskeletal Seminar IV

•

UNKNOWN CASE #4: FINDINGS • •

Osteoarthritic changes – unusual locations radiocarpal and MCP joints (2nd through 5th) Chondrocalcinosis – TFFC, no scapholunate separation Hook-like osteophytes metacarpal heads

UNKNOWN CASE #4: HEMOCHROMATOSIS MUSCULOSKELETAL CHANGES • • • •

•

Osteoporosis Chondrocalcinosis (20–60%) Arthropathy (24–50%) looks like osteoarthritis Differences from CPPD arthropathy-involvement of 4th and 5th MCP joints; hook-like osteophytes metacarpal heads; less scapholunate separation; pericapitate narrowing

UNKNOWN CASE #5: HISTORY 39 year old woman with low back pain

2 weeks prior to previous radiographs

Musculoskeletal Seminar IV

1022

Musculoskeletal Radiology

UNKNOWN CASE #5: DIFFERENTIAL DIAGNOSIS NARROW DISK SPACE • • • • • •

Degenerative disk disease (DDD); herniated disk (trauma) Inflammatory arthritis Scheuermann disease Osteomyelitis Neoplasm (very rare) Amyloid– chronic renal failure (CRF)

T1

T2

• • • •

Rapid disk space narrowing L2–3 over two week interval Subtle endplate destruction L2–3 level T1W-MR: marrow replacement L2–3 with disk involvement T2W-MR: increased marrow intensity L2–3 with disk involvement

• • •

Usually starts in anterior subchondral bone then spreads rapidly to disk Bacterial vs. unusual cause (TB) Drug abusers predisposed

•

65 year old man with right pain

UNKNOWN CASE #5: FINDINGS

UNKNOWN CASE #5: INFECTIOUS SPONDYLODISCITIS

UNKNOWN CASE #6: HISTORY

Musculoskeletal Radiology

1023

Musculoskeletal Seminar IV

UNKNOWN CASE #6: DIFFERENTIAL DIAGNOSIS CHONDROID LESION •

•

Benign ➢ Enchondroma, bone infarct, chondroblastoma, chondromyxoid fibroma (CMF), osteoblastoma Malignant ➢ Chondrosarcoma - intramedullary, juxtacortical, clear cell, mesenchymal, myxoid, dedifferentiated, extraskeletal

T2

T1 • • • •

UNKNOWN CASE #6: FINDINGS Lytic lesion proximal femur some areas of surrounding sclerosis Cortical permeation inferomedial on conventional tomography Matrix on CT and tomography-chondroid Soft tissue mass best seen on MRI

RADIOLOGIC DIFFERENTIATION OF CHONDROSARCOMATOUS LESIONS •

• •

Aggressive chondroid lesion with soft tissue mass ➢ High grade conventional chondrosarcoma ➢ Dedifferentiated chondrosarcoma ➢ Mesenchymal chondrosarcoma Large fluid component bone or soft tissue ➢ Myxoid chondrosarcoma Change in appearance or foci of more aggressive nature ➢ Diagnosis: Dedifferentiated chondrosarcoma

Musculoskeletal Seminar IV

1024

Musculoskeletal Radiology

Musculoskeletal Seminar V Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY 60 year-old man with 6 months of knee pain ➢ Biopsied and diagnosed as myeloma. ➢ Is this a tenable diagnosis? ➢ What is the correct diagnosis and why was the initial pathology incorrect?

T1

T2 Fat Sat T1 GD

T2 FAT SAT •

UNKNOWN CASE # 1: FINDINGS •

•

Radiographs – Geographic 1A lesion with channel/tract like component inferiorly (subtle) MRI – Marrow replacement T1W ➢ Rim enhancement (fluid filled mass) ➢ Homogeneous very high signal T2W ➢ Surrounding edema ➢ Tract like component inferiorly Differential diagnosis – UBC, ABC (no expansion) intraosseous hematoma, ablated lesion, Brodie abscess

Musculoskeletal Radiology

1025

Musculoskeletal Seminar V

UNKNOWN CASE #1: SUBACUTE OSTEOMYELITIS BRODIE ABSCESS • • • • • • •

Described in 1832 - chronic/subacute Walled-off with central fluid, often sterile (staph- only cultured in 50% of cases) Children (M>F), metaphysis, tibia Intramedullary; channel-like lucencies May cross growth plate or be cortical Periosteal reaction/sequestra may be seen

UNKNOWN CASE #1: BRODIE ABSCESS •

•

Biopsied at margin in reactive tissue ➢ Led to erroneous diagnosis Myeloma (untreated) not a tenable diagnosis ➢ asthis is a solid lesion ➢ Importance of radiologic/pathologic correlation

UNKNOWN CASE #1: HISTORY 59 year old veteran involved in mild MVA (first film) with progressive pain (second set of films 2 weeks later and MRI)

First Radiograph

Second radiographs 3 weeks prior to first radiograph

T2

T1

Musculoskeletal Seminar V

1026

Musculoskeletal Radiology

• • • • •

UNKNOWN CASE # 2: FINDINGS Relatively rapid destruction of shoulder Fragments in joint Sharp “surgical” margin MRI – replacement of humeral head with high intensity on T2W History of drained syrinx 40 years ago and cervical spine MRI shows severe myelomalacia

• • •

Cause – pain sensation vs. neurovascular Radiologic- destruction, debris, density increase, disorganization Diabetes, syphilis, cord damage - syrinx

•

15 year-old boy with hip pain

UNKNOWN CASE # 2: NEUROPATHIC SHOULDER-SYRINX

UNKNOWN CASE # 3: HISTORY

T1

•

STIR

UNKNOWN CASE # 3: FINDINGS • • •

Marrow replacement right femoral neck T1W ➢ medial transcervical region High signal on STIR ➢ surrounding edema periosteum/ST ➢ horizontal low signal linear band medially Subsequent near total resolution

STRESS FRACTURE: FEMUR •

•

Medial femoral neck - fatigue type ➢ heal with symptomatic treatment (3 to 12 months) ➢ crescentic MR abnormality above lesser trochanter Lateral femoral neck - insufficiency type ➢ possible cause most subcapital fractures ➢ DO NOT HEAL: COMPLETE/DISPLACED ➢ Garden staging (< Grade 2 percutaneous pins) (> Grade 2 THA due to development of AVN) Usually horizontal/oblique rarely longitudinal ➢ thigh splints (stress reaction)

Musculoskeletal Radiology

1027

Musculoskeletal Seminar V

•

UNKNOWN CASE # 4: HISTORY 23 year-old man with knee pain, masses and lesions on radiographs ➢ Diagnosis of bone lesions ➢ Underlying condition

T1

T1

T2

T2 WITH FAT SAT

Musculoskeletal Seminar V

1028

Musculoskeletal Radiology

• • • • • • • • • •

NEUROFIBROMATOSIS 1: SKELETAL MANIFESTATIONS

•

•

Mesodermal dysplasia Kyphoscoliosis Facial, orbital, lambdoid suture (left) defects Multiple nonossifying fibromas Meningocele Posterior vertebral scalloping Rib deformity (ribbon ribs) Congenital pseudarthrosis (tibia) Focal hypertrophy (gigantism) Localized neurofibroma - most common ➢ least characteristic, often deep/multiple ➢ superficial lesions (fibroma molluscum) Plexiform neurofibroma - pathognomonic ➢ early childhood ➢ precedes cutaneous neurofibromas

UNKNOWN CASE # 5: HISTORY 53 year-old man with lateral knee mass and pain

T1

STIR

STIR

Musculoskeletal Radiology

1029

Musculoskeletal Seminar V

PROTON DENSITY

STIR • •

UNKNOWN CASE # 5: FINDINGS

•

Tibiofibular/lateral tibiofemoral joint osteoarthritis High fluid content multilocular mass laterally ➢ multilocular / surrounding edema ➢ appears to arise from tibiofibular joint ➢ components in bone (tibia and femur) and soft tissue D/DX -- Ganglion/Synovial cyst, myxoid tumor

Musculoskeletal Seminar V

1030

Musculoskeletal Radiology

• • • •

GANGLION/SYNOVIAL CYST

• • •

Etiology unknown- neoplasm, trauma, inflammatory Young adults-most common mass hand/wrist Pain-may affect adjacent nerves Location ➢ ST: Hand, foot, knee, hip, shoulder ➢ Intraosseous: medial malleolus, wrist, knee ➢ BOTH Thick walled unilocular/multilocular ➢ high protein content affects CT/T1W MR ➢ walls/septa may enhance CT/MRI/Sono - cystic mass ➢ may rupture cause surrounding edema

UNKNOWN CASE # 6: HISTORY 47 year-old woman with mid to low back pain ➢ Most likely diagnosis? ➢ Two other possible diagnoses?

T1

T2

T2

STIR

Musculoskeletal Radiology

1031

Musculoskeletal Seminar V

•

UNKNOWN CASE # 6: FINDINGS • • • •

Multifocal bone scan areas increased radionuclide activity ➢ spine, SC joints, SI joints CT-multifocal sclerosis ➢ anterior/posterior paralleling endplates ➢ erosions/bone production costovertebral joints ➢ cause of hot bone scan MRI - multifocal areas marrow abnormality ➢ low T1W, high T2W/STIR ➢ anterior/posterior paralleling endplates ➢ no soft tissue mass Radiographs- subtle sclerosis, sacroiliitis D/DX - Metastases, myeloma, lymphoma

Musculoskeletal Seminar V

1032

Musculoskeletal Radiology

• • • • • •

ANKYLOSING SPONDYLITIS: CLINICAL CHARACTERISTICS

• • •

Peak age of onset 15-35 years M:F 3-5:1 Incidence 6.6/100,000 HLA-B27 > 90% Rare in blacks Predilection axial involvement

ANKYLOSING SPONDYLITIS: DISTRIBUTION

• •

• • • • • • • •

Osseous ankylosis Ligament/Tendon ossification Spine/SI joints ➢ symmetric Pelvis - symphysis, ischium, iliac, hips Peripheral changes unusual early (10% - 50%) ➢ asymmetric

ANKYLOSING SPONDYLITIS: SPINE CHANGES Osteitis -”shining corners” Squared vertebral bodies Syndesmophytes Bamboo spine Calcified disc, fused facets/ligaments Pseudarthrosis/fractures Atlantoaxial disease/Cauda equina Other diagnoses- Reiter/Psoriatic, SAPHO

Musculoskeletal Radiology

1033

Musculoskeletal Seminar V

Radiologic Pathology 2006-2007 - Volume 2 - Index 25-OH Vitamin D 902 Abscess 796 Soft Tissue Infection 827 Subperiosteal 823 Access. Navicular 880 Accessory Muscles Masses (Ankle and Foot) 886 Accessory Soleus 886 Acetabulae protrusio 816 Acetabular Columns Letournel and Judet 998 Injury 998 Trauma 995 Acetabulum 998 Achilles Tendon 883 Achondroplasia 1020 ACL 867 Post-operative 868 Tear 866, 867 Acromegaly 773 Acromial Variation 927 Acromio-clavicular Joint Injuries 941 Acromion 942 Acroosteolysis 983, 1007, 1008 Actinomyces 833 Actinomycosis 829, 833 Active infection 825 Acute Osteomyelitis 821 Adamantinoma 721, 771, 776 Adductor pollicis 947 Adenomatoid odontogenic tumor 855 Adhesive Capsulitis 931 Adolescent Stress Lesions 994 Adult Palmar Fibromatosis 779 Adult Plantar Fibromatosis 780 Aggressive “Malignant” Osteoblastoma 750 Aggressive infantile Fibromatosis 778 Aggressive Osteoblastoma 750 Alcoholism 907, 953 Alkaline phosphatase 812 Alkaptonuria 949, 953 Allograft 707 ALPSA Lesion 934 Aluminum Toxicity 716 Ameloblastic fibroma 852 Ameloblastic fibro-odontoma 857 Ameloblastoma 852, 854 Amyloid 795 Amyloidosis 716, 1006 B2 – microglobulin 716 Carpal tunnel syndrome 716 Destructive spondyloarthropathy 716 Discovertebral erosions 717 Anatomy of the tarsal joints 1003 Aneurysm 804 Aneurysmal Bone Cyst 749, 784, 787 Angioblastoma 776 Angiomatoid 780 Angiomatosis 805, 808

Angiomatous lesions 1017 Angiosarcoma 721, 805, 810 Ankle 879, 995 Fractures 1001 Ankylosing Spondylitis 912, 916, 918, 1033 Ankylosis 914 Anterior Dislocation (Glenohumeral) 942 Anterior Drawer 867 Anterior Instability (Glenohumeral) 933 Anterior talofibular 881 Anterior tibiofibular ligament 880 Anterolisthesis 840 Anteroposterior (AP) compression (Pelvic Trauma) 996 Arthritis 795, 1006 Juvenile Chronic 919 Psoriatic 916 Septic 825 Tuberculous 832 Articular cartilage 859 Aseptic necrosis 906 Aspergillosis 835 Aspirin/nonsteroidals (Osteoma) 746 Atlantoaxial subluxation 915 Atlas Fractures 843 Atypical Mycobacterium 833 Autograft Healing 706 Avascular Necrosis 719, 959, 1010, 1020 Aviator’s astragulus 1002 Avulsion 865 Avulsive cortical injury 777 Axial Compression Injury 846 Axial Osteomalacia 904 Axillary Nerve Neuropraxy 935 Axillary View 942 Bacillary angiomatosis 837 Ballooned epiphyses 920 Bamboo spine 918 Banana fracture 816 Bankart Lesion 932 Bankart Repair 938 Barton Fracture (Reverse) 945 Baseball Finger 948 Basilar invagination 816, 901 Bathrocephaly 1008 Batson’s plexus 961 Benign Bone Tumors: Age Distribution by Decade 723 Benign Fibrosis Histiocytoma 773 Benign Polyostotic Lesions 1017 Bennett Fracture 947 Biceps Injury 957 Partial Tears 957 Tendinosis 957 Biceps Femoris 869 Biceps Tendon 929 Bicipital Radialis Bursitis 957 Bilateral Facet Dislocation 842 Birbeck bodies (Eosinophilic Granuloma) 890 Blade of grass 813 I1

Blastic Disease 814 Blastic Lesions 967 Blastic Phase 812 Blastomycosis 835 Blount 911 Blow out lesions 967 BMD 978 BMD (WHO Classification) 979 Bohler 1003 Bone Autograft 706, 707, 710 Bone bruise 865, 879, 958 Bone enlargement 814 Bone Graft Complications 708 Fracture 709 Joint Instability 709 Nonunion and Pseudarthrosis 708 Resorption 709 Bone Graft Substitutes 710 Bone Infarct 769, 906, 1024 Osteonecrosis 769 Bone Island 743 Bone Metastases 961 Bone Production 914 Bone scan 744 Bone Tumors 720 Cartilage 720 Histiocytic 721 Marrow 720 Notochord 721 Osteoid 720 Unknown Origin 721 Vascular 721 Bone Tumors (Incidence) 722 Important Factors in the Diagnosis of 723 Primary Benign 722 Primary Malignant 722 Bone within Bone 970 Botryoid odontogenic cyst 852 Bouchard nodes 923 Bowing of long bones 901 Boxer’s Fracture 948 Brachial artery 944 Brachial nerve injury 944 Breast Carcinoma 962 Bristow procedure 938 Brodie abscess 748, 824, 1007, 1026 Brown tumor 711, 853, 1013 Brucella 829 Brucellosis 829 Bucket handle 861, 862 Bumpy (Soft Tissue Swelling) 912 Bursae (Knee) 870 Bursitis (Bicipital Radialis) 957 Bursitis (septic) 826 Burst Fracture 846 Button osteophyte 922 Cafe-au-lait spots 773, 981 Calcaneal Fractures 1002 Calcaneofibular 881 Calcaneus 995

Calcific Myelitis 792 Tendinitis 952 Tendonitis 930 Tendonitis (Glenohumeral) 943 Calcified falx cerebri 853 Calcified Soft Tissue Mass 1012, 1015 Calcinosis 984 Calcitonin 818 Calcium deficiency 977 Calcium hydroxyapatite 718, 792, 949 Cancellous (Osteoid Osteoma) 746 Candidiasis 835 Candle flame 813 Cap thickness 758 Capillary hemangioma 809 Capitate (Dislocation) 946 Capitellum 955 Capsulorapphy 938 Carcinomatosis 971 Carpal Dislocations 946 Carpal Stability 874 Carpal Tunnel 872, 876 Syndrome 877 Cartilage metaplasia 799 Cartilage nodules 799 Cartilaginous Lesions 757 Chondroblastoma 757 Chondromyxoid fibroma 757 Chondrosarcoma 757 Enchondroma 757 Juxtacortical chondroma 757 Osteochondroma 757 Caseating necrosis 830 Cavernous hemangiomas 761 Cavernous spaces 806 Cellulitis 826 Cementoblastoma 854 Central giant cell granuloma 852 Cervical Burst Fracture 846 Cervical Spine Trauma 839 Cervical spondylosis 848 Charcot joint 953 Chondroblastoma 720, 1008, 1024 Codman Tumor 763 Chondroblasts 764 Chondrocalcinosis 950, 1021 Chondroid 738 Chondroid Lesion 1024 Chondroid Matrix (Enchondroma) 761 Chondroid Matrix (Intramedullary Chondrosarcoma) 765 Chondromyxoid Fibroma 720, 1024 Chondrosarcoma 762, 764, 1024 Dedifferentiated 769 Extraskeletal 768 Mesenchymal 768 Myxoid 768 Chondrosarcomatous Lesions 1024 Chordoma 721, 786 Christmas disease 972 Chronic ACL Tear 867 Chronic Granulomatous Disease of Childhood 827 I2

Chronic hematoma 796 Chronic infection 818 Chronic Osteomyelitis 825, 852 Chronic Recurrent Multifocal Osteomyelitis (CRMO) 827 Chronic regional pain syndrome 976 Chronic renal failure 793 Chronic sclerosing osteomyelitis 856 Chronic symmetric plasma cell osteomyelitis 827 Clavicle Clay Shoveler Fracture 843 Clear Cell Chondrocytes 767 Clear Cell Chondrosarcoma 763, 764, 767, 1008 Clear Cell Sarcoma 792, 803 Cleidocranial dysplasia 1007 Clutton joints 834 Coach’s Finger 948 Coccidioidomycosis 835 Codman Tumor 763 Collagen Vascular Diseases 793, 907, 1012 Collagen vascular-like diseases 976 Collateral ligament 869 Collateral ligaments 859 Colles fracture 945 Column Fracture (Pelvis) 1000 Complete pelvic instability 997 Compression Fracture 841 Congenital insensitivity to pain 953 Congenital Syphilis 834 Contiguous spread 820 Contusion 865 Cooley’s anemia 970 Coracoid 942 Coronoid fossa 955 Cortical Osteoid Osteoma 746 Desmoid 777 Fibrous Dysplasia 775 Involvement 738, 739 Lucency/Central Calcification 1005 Resorption 712 Thickening (Chondrosarcoma) 765 Cotton wool 814 CPPD 718, 949 Arthropathy 792 Deposition/arthropathy 1021 Cranial Sclerosis 744 Craniotabes 901 CREST Syndrome 984 Cretinism 1007, 1020 Crohn's disease 912 Cruciate ligaments 859 Cryptococcosis 836 Crystal deposition (Thalassemia) 971 Crystal Deposition Disease 718, 949 Cushing syndrome 773 Cyst (Paralabral) 938 Cystic hygroma 808 Cystic Masses (Knee) 870 Cysticercosis 837 Dactylitis 830, 834, 969, 1010 Dagger sign 918 Danis-Weber Classification 1001

Decubitus ulcers 825 Dedifferentiated Chondrosarcoma 764, 769 Deep Endosteal Scalloping 765 Deforming nonerosive arthropathy 983 Degenerative Joint Disease 951 Dental Anatomy 849 Dentigerous cyst 852 Dentition 849 Deoxyhemoglobin 848 DeQuervain’s Syndrome 876 Dermatofibrosarcoma Protuberans (DFSP) 771, 781, 782, 802 Dermatofibrosis lenticularis disseminata 744 Dermatomyositis 976 DeSmet 860 Desmoid 746 Extraabdominal 779 Desmoplastic fibroma 771, 777 DEXA 978 Diabetes 825 Insipidus 892 Mellitus 773, 825, 953 Diffuse sclerosis 1010 Diphoshonates 818 Direct implantation 820 Discitis 828 Discography 828 Discoid 860 DISI deformity 874 Distal radial buckle fracture 945 Distal radioulnar joint 872 Distal Tuft Fracture 948 Disuse/Immobilization Osteoporosis 977 Double Axillary Pouch Sign 934 Double line sign 908 Doughnut sign 785 Drug Abusers 827 Du Toit & Roux 938 Dual Energy X-ray Absorptiometry (DEXA) 978 Dupuytren Exostosis 759 Durie/Salmon PLUS Staging (Myeloma) 965 Dysbaric disorders 907 Dysplasia Epiphysealis Hemimelica: Trevor Disease 759 Dysplastic Thoracolumbar Junction 1020 Dysprosium 165 795 Early focal cemento-osseous dysplasia 850 Echinococcus 837 ECU tendon sheath 875 Ehlers-Danlos 1016 Elbow 955 Dislocations and Fractures 944 Embolization 748, 808 Enchondroma 720, 760, 769, 1024 Enchondroma vs. Chondrosarcoma 769 Enchondromatosis 1017 Endocrinopathies 773 Endodontic procedures 854 Endosteal scalloping 770 Enostosis 720, 743 Enteropathic arthritis 912 Enthesopathy 914 Enucleation 852

I3

Eosinophilic Granuloma 721, 887, 890, 967 LCH 1008 Epicondylitis (Elbow) 956 Epidermoid Carcinoma 826, 834 Epidermoid Inclusion Cyst 784, 790 Epidural hematoma 848 Epiphyseal Lesions 1008 Dysgenesis 1021 Dysplasia 1020 Epiphysis 767 Epithelial nests 776 Epitrochlear Lymph Node 960 Erlenmeyer flask deformity 971 Erosions 913 Erosive Osteoarthritis 917 Erupting teeth 854 Essex-Lopresti 945, 1003 Ewing Sarcoma 721, 887, 964, 967 Ewing Sarcoma (Intergroup Study) 888 Exophthalmos 892 Exostoses 856 Exostosis (Subungual - Dupuytren Exostosis) 759 Extensor Carpi Ulnaris Sheath 875 Extensor tendons 859 Extent of Musculoskeletal Neoplasm 738 Extra-abdominal Desmoid Fibromatosis 778 Extra-articular erosions (Gouty Arthritis) 950 Extramedullary Hematopoiesis 971 Extraskeletal Chondrosarcoma 764, 768 Facet 840 Fallen fragment sign 787 Familial vitamin D res rickets 903 Fanconi syndromes 903, 968 Fanconi’s Anemia 972 Felon 825 Felty Syndrome 914 Femur 995 Fibrocartilage Calcification 951 Fibroma molluscum 1029 Fibromas 746 Fibromatosis 771, 778 Fibromatosis: Types 778 Fibrosarcoma 771, 780, 781, 802 Fibrosarcoma/MFH 748 Fibrosis Histiocytoma 773 Fibrous cortical defect 771 Fibrous dysplasia 771, 773, 856, 944, 1017 Fibrous Histiocytoma (Malignant) 721 Fibrous medullary defect 771 Fibroxanthoma (Nonossifying fibroma) 771 Fibular (lateral) 869 Fibular collateral lig complex 881 Fibular collateral ligament 867 Filariasis 837 Finger (Trauma) 948 First MC (Fracture) 947 Flexion Teardrop Fracture 843 Flexor Digitorum (Avulsion) 948 Flipped (meniscal tear) 862 Florid cemento-osseous dysplasia 856 Fluid - fluid level 764, 785, 802 Fluorosis 818

Focal cemento-osseous dysplasia 855, 857 Focal scerosing osteomyelitis 854 Foot 879, 995 Injuries 1003 Fracture (First MC) 947 Fractures 816 Fractures (Pathologic) 961 Frieburg 911 Full thickness (Rotator Cuff Tear) 928 Galeazzi 945 Ganglia 870 Ganglia (Knee) 870 Ganglion 792 Ganglion/Synovial Cyst 1031 Ganglion/synovial cyst/bursa 796 Gardner Syndrome 852, 856 Gardner Syndrome (Osteoma) 746 Gastrocnemius 869 Gastrocnemius/ Semimembranosus 870 Gaucher disease 818, 907, 971 GCT 769 Geodes 790 Geographic Contusion (Knee) 866 Geographic Pattern (Bone Tumors) 725 Geographic 1A: Differential Diagnosis 725 Geographic IB: Differential Diagnosis 726 Geographic IC: Differential Diagnosis 726 Giant Bone Island 743 Giant Cell (Reparative) Granuloma 780 Giant Cell Tumor 721, 784, 1008 Giant Cell Tumor Tendon Sheath (GCT-TS) 794 Giant Cells 784 Gigantism 1029 Glad Lesion 936 Glenohumeral 932 Instability 932 Joint 932 Ligaments 932 Glenohumeral Internal Rotation Deficit (GIRD) 936 Glenohumeral Labroligamentous complex 932 Glenohumeral ligament (avulsion) 935 Glenoid labrum 932 Glomus 721 Glomus Tumor 805, 808 Gnathic Osteosarcoma 754 Golfer’s elbow 956 Goltz syndrome 744, 784 Gorham 809 Gorlin syndrome 853 Gout 718, 817, 949, 1010, 1012 Gouty arthritis 949, 950 Gouty tophus 804 Gracilis 869 Granulomatous Disease of Childhood 827 Grashey view 942 Greater Tuberosity Fracture 935 Ground glass 774 Group B strep 822 Guinea worm (dracunculosis) 837 Gumma 834 Guyon’s canal 872, 877 HA Crystal Deposition Disease 952 I4

Hagl Lesion 935 Hajdu-Cheney Syndrome 1008 Hallux 924 rigidus 924 valgus 924 Hamartoma 743 Hamate Fracture 947 Hand-Foot syndrome 969 Hand-Schüller-Christian disease 887, 892 Hangee Fracture 845 Hangman Fracture 845 Hanover 1003 Hawkins classification of talar neck fractures 1002 Hawkins sign 1002 Heberden nodes 923 Hemangioendothelioma (HE) 721, 805, 810 Hemangioma 721, 804, 805, 1012 Arteriovenous 805 AV malformation 852 Capillary 805, 809 Cavernous 805 Venous 805 Hemangiopericytoma (HPC) 721, 768, 805, 810 Hematogenous Osteomyelitis: Adult 824 Hematogenous Osteomyelitis: Child 822 Hematogenous Osteomyelitis: Infant 822 Hematogenous Vascular Supply 820 Hematologic Disease 968 Hemochromatosis 949, 950, 952, 1021 Hemodialysis elbow 718 Hemoglobinopathy 907 Hemophilia 968, 972 Hemophiliac pseudotumor 973 Hemorrhage 781, 790 Hereditary Hyperphosphatasia: Juvenile Paget Disease 818 Hereditary multiple exostoses (HME) 757, 760, 1017 Heterotopic Bone Formation: Myositis Ossificans 800 High - Grade Surface 754 High output congestive failure 812 Hill-Sachs Lesion 934, 943 Hip Fractures 1000 Joint instability 999 Trauma 1000 Histoplasmosis 836 HLA B27 916 Hodgkin 966, 971 Hook of hamate 877 Hoop stresses 860 H-Shaped Vertebral Bodies 970 H-type vertebrae 1010 Human/animal bites 825 Humeral Fractures 943 Humphrey 859 Hutchinson triad 834 Hyaline cartilage cap 757, 758 Hyaluronic acid 795 Hydrops Fetalis 808 Hydroxyapatite 715 Hydroxyapatite Crystal Deposition 952 Hydroxyproline 812 Hygroma 808

Hypercementosis 854 Hyperextension 840 Hyperextension Dislocation 844 Hyperextension Injuries 843 Hyperextension: Teardrop Fracture 845 Hyperflexion 840 Hyperflexion Injuries 840 Hyperflexion Sprain 840 Hyperparathyroidism 711, 773, 852, 856, 950, 971, 1007, 1012, 1021 Hyperthyroidism 773 Hypertrophic Osteoarthropathy 962 Hypophosphatasia 904, 1007 Hypothyroidism 1020, 1021 Idiopatic osteosclerosis 854 Ifosfamide 903 Iliotibial band 867 Iliotibial tract 869 Imaging for Staging Musculoskeletal Neoplasm 738 Immature cementoblastoma 850 Immature periapical cemental dysplasia 850 Impingement Syndrome 927 anterolateral 881 Infantile dermal/digital fibromatosis 778 Infantile myofibromatosis 778 Infarction 969 Infection 1006 Infectious Spondylodiscitis 1023 Inferior Glenohumeral Labroligamentous complex 932 Inferior Glenohumeral Ligament 933 Inflammatory 780 Infrapatellar 870 Infrapatellar cleft 870 Insufficiency fractures 979, 983 Interbody Fusion 710 Intermedius 869 Interosseous ligament 880 Interosseous syndrome (Elbow) 956 Interspinous widening 840 Intertrochanteric Fractures 980 Intraarticular Bodies (Elbow) 956 Intra-Articular Hydroxyapatite Crystal Deposition Disease 952 Intracortical fibrous dysplasia 775 Intramedullary (Chondrosarcoma) 764 Intramedullary Extent 738 Intramedullary Hemorrhage 848 Intranuclear inclusions 812 Intraosseous ganglion 784, 790, 1008 Intrasubstance Tear (Rotator Cuff) 929 Invisible Margin (Bone Tumors) 728 Involucrum 820 Irradiation 907 Irregular Epiphyses (multiple) 1020 Isolated Fractures Radius: Galeazzi 945 Isolated Fractures Ulna: Monteggia 945 Isolated Tendon Injuries 948 ITB Friction Syndrome 869 Ivory vertebra 814, 962 Differential Diagnosis 962 Jaccoud arthropathy 1016 Jaffe-Campanacci syndrome 773 I5

Jaws 849 Jefferson Fracture 846 Jersey Finger 948 Joint Arthroplasty (Complications of ) 699 Dislocation / Abnormal Alignment 702 Fractures and Nonunion 702 Heterotopic Bone Formation 699, 703 Loosening and/or infection 699 Radionuclide Evaluation 700 Small Particle Disease 699, 701, 702 Joint involvement 738, 739 Abscess 741 Bursitis 741 Diabetic muscle ischemia 741 Fibromatosis 741 Gadolinium 742 Hematoma 741 intraarticular resection 739 Lymphocele 741 Muscle flap 741 Myositis ossificans 741 Myxoid liposarcoma 741 Post-Operative Imaging (Bone Neoplasm) 741 Radiation necrosis 741 Reactive lymph node 741 Seroma 741 Soft Tissue Mass - Benign 740 Soft Tissue Mass - Malignant 740 Subtraction MRI 742 Synovial cell sarcoma 741 Joint Replacement 699 Jones Fracture 1003 JRA 919 Polyarticular 920 Still Disease: Pauci or Monoarticular 920 Jumpers Knee 870 Juvenile aponeurotic fibroma 778 Juvenile Chronic Arthritis 912, 919, 973, 1016 Juvenile Paget Disease 818 Juvenile-onset adult type RA 920 Juvenile-onset ankylosing spondylitis 919 Juxtaarticular Osteoporosis 912 Juxtacortical Chondroma 720, 762, 767 Juxtacortical Chondrosarcoma 767 Juxtacortical Osteosarcoma 752 Kaplan 860 Kaposi sarcoma 837 Kasabach - Merritt 809 Keloid formation 744 Keratocyst 852 Kienböck 911 Kienböck’s Disease 911 Klippel-Trenaunay-Weber 809 Knee 995 Knee Injury 1001 Knee Stabilizers 867 Köehler 911 Kohler Disease 1006 Kyphosis 830, 840 Labral Repair 938 Labrum 932 Lamina dura 850

Laminar Fractures 843 Langerhans Cell Histiocytosis (LCH) 748, 887, 890, 1013, 1008, 1017 Laser therapy 808 Lateral compression (Pelvis Trauma) 996 Lateral Epicondylitis (Elbow) 957 Lateral inferior geniculate artery 863 Lateral Meniscus 859 Lateral Process of Talus Fractures 1002 Lateral Stabilizers 869 Lateral Tendons (Ankle and Foot) 884 Lateral Ulnar Collateral Ligament 959 Lauge-Hansen 1001 LCL-Biceps Femoris 870 Ledderhose disease 780 Legg-Calvé-Perthes 911 Leiomyosarcoma 781, 802 Leprosy 829, 833, 953 Lesion matrix 738 Letournel and Judet classification 998 Letterer-Siwe disease 887, 892 Leukemia 971 Ligament and Tendon Involvement 739 ligament(ous) injury 840 Limb Salvage Procedures 737 Lipoblastoma 896 Lipoma 720, 804, 1015 Lipoma Arborescens 798 Lipoma Intramuscular 895 Lipomatosis 897 Liposarcoma 720, 781, 802, 893, 897, 1015 Atypical 898 Dedifferentiated 898 Higher Grade Lesions 898 Myxoid Lesions 898 Pleomorphic Liposarcoma 899 Well-Differentiated 897 Loa loa 837 Location in Bone: Axial (Bone Tumors) 724 Locked facets 842 Long Head of Biceps Tendon 929 Long Head of the Biceps Tendon 932 Longitudinal Tibial Stress Fracture 992 Loose Bodies 871 Looser zones (Osteomalacia) 715 Looser’s zones 902 Lower Extremity Trauma 995 Low-Grade Chondroid Lesion 769 Low-Grade chondrosarcoma 769 Lumpy 912 Lunate 946 Lung Cancer 963 Lunotriquetral Instability 875 Lyme disease 834, 835 Lymphangioma 721, 805 Lymphoma 720, 748, 961, 964 Lymphoma of Bone (Primary Lymphocytic) 966 Lytic Patterns (Bone Tumors) 728 lytic phase 812 Lytic Phase 813 Lytic Skull Lesions 1013 Macho-Macho 852 I6

Madura Foot 837 Maffucci syndrome 760, 761, 809 Magic Angle Phenomenon 876 Magnuson Stack 938 Malignant Bone Tumors: Age Distribution by Decade 723 Malignant Fibrous Histiocytoma (MFH) 721, 771, 780, 781 Malignant melanoma of soft parts 803 Malignant myositis 801 Malignant transformation (Multiple Enchondromatosis) 761 Mallet Finger 948 Malnutrition 977 Marrow Edema (Osteoid Osteoma) 747 Marrow hyperplasia 969 Massive Osteolysis of Gorham 809 Mastocytosis 818 Matrix Formation 723 Matrix Formation (Bone Tumors) 729 Mazabraud syndrome 773 McCune Albright syndrome 773 Medial Collateral Ligament (MCL) 867 Injuries 868 Medial Collateral Ligament (Elbow) 959 Medial Meniscus 859 Medial migration (Osteoarthritis: Hip) 923 Medial Tendons (Ankle and Foot) 884 Median nerve 872, 877 Impingement 956 Melorheostosis 744 Meningoceles 983 Meningomyelocele 953 Meniscal Cyst 798, 870 Meniscal Flap 862 Meniscal tears 798, 858, 861 Menisci (Calcification) 951 Menisci (Post-surgical) 863 Meniscofemoral ligaments 863 Meniscus homologue 875 Mesenchymal cells 768 Mesenchymal Chondrosarcoma 764, 768 Metabolic Bone Disease 900 Metachronous Osteosarcoma 750 Metaphyseal chondrodysplasia 904 Metastases 748, 768, 1013 Skeletal 961 Metastatic lymphoma 967 Methemoglobin 848 MFH (Malignant Fibrous Histiocytoma) 802 MFH/ fibrosarcoma 769 Middle Glenohumeral Ligament 933 Milk - Alkali syndrome 793 Milwaukee Shoulder 952 Mithramycin 818 Mixed Connective Tissue Disease (MCTD) 1016 Mixed/Blastic Disease 814 Monostotic 773, 812 Monteggia 945 Morton’s Neuroma 885 Motheaten (Bone Tumors) - Differential Diagnosis 727 Mucoepidermoid carcinoma 852 Mucopolysaccharidosis 1020 Mucormycosis 836 Multidirectional Instability (Glenohumeral) 939

Multiple enchondromatosis 760, 761 Multiple Myeloma 964 Multiple myeloma with sclerosis or POEMS syndrome 965 Multiple tori and exostoses 856 Musculoskeletal Infection 820 Musculoskeletal Neoplasm - Extent 738 Musculoskeletal Neoplasm - Staging - Surgical Implications 737 Musculoskeletal Tumors - Staging 734 Histologically Benign 735 Histologically Malignant 736 Mycetoma 837 Mycobacteria 829 Mycobacterium 833 Mycobacterium Leprae 833 Myelitis (Calcic) 792 Myelofibrosis 818, 968, 971, 974 Myelography 840 Myeloma 720, 961, 964, 1013 Myeloma/plasmacytoma 786 Myelomalacia 848 Myeloproliferative diseases 949 Myositis 837, 983 Myositis Ossificans 792, 800, 1012 Myotendinous (Rotator Cuff Tear) 928 Myotendinous Tear of Pectoralis 931 Myxoid 780 Myxoid Chondrosarcoma 764, 768 Myxoma 773, 792 Myxomatous neoplasms 796 Narrow Disk Space 1023 Nasopalatine duct cyst 850 Navicular 880 Neck of the scapula 942 Necrosis - Avascular 719 Neoplasm (Paget Disease) 817 Nerve Impingement (Elbow) 956 Nerve root avulsion 840 Neuroarthropathy 953 Neuroblastoma 963, 964, 967 Neurofibromatosis (NF) 976, 981 Neurofibromatosis (Type 1) 773, 1017, 1020, 1029 Neurogenic tumor 786 Neuropathic 834 Joint 1017 Osteoarthropathy 949, 953 Shoulder-Syrinx 1027 Neurovascular involvement 738, 739 Nevoid basal cell carcinoma syndrome 853 NF (Neurofibromatosis) 981 NF-1 (vonRecklinghausen’s) 981 NF-2 - Acoustic neuromas 981 Nidus (Osteoid Osteoma) 746 Night Stick Fracture 944 Nocardia 833 Nodular Fasciitis 792, 801 Non-Hodgkin 966 Non-Insertional Achilles Tendon Pathology 883 Nonossifying fibroma (NOF) 771, 981 Nonosteogenic fibroma 771 Nonspecific spindle cell sarcoma 781, 802 O’Donoghue’s Triad 867 I7

Oblique meniscomeniscal ligament 863 Occult fracture 865 OCD 955 Odontogenic cyst 852 Odontogenic keratocyst 852 Odontogenic myxoma 852 Odontoid Fracture 847 Odontoma 855, 857 OI (Osteogenesis Imperfecta) 980 Olecranon bursitis 949, 958 Olecranon fossa 955 Ollier disease 760 Ollier Syndrome 762 Oncogenic osteomalacia 773 Os Acromiale 928, 942 Os odontodeum 847 Os Trigonum 880 Osgood-Schlatter 911 Osler-Weber-Rendu 809 Osseous bowing 812 Osseous deformity 816 Osseous Lesions both Sides of Joint 1006 Osseous Neoplasm 733 Ossicle 860 Ossifying fibroma 775, 855, 857 Osteitis deformans 812 Osteoarthritis 920 Osteoarthritis (secondary) 826 Osteoarthropathy (Hypertrophic) 962 Osteoarthropathy (Neuropathic) 949, 953 Osteoblastic Metastasis 743 Osteoblastoma 720, 743, 748, 763, 855, 857, 1008, 1024 Osteochondral fracture 879 Osteochondral Lesion 879 Osteochondritis 834 Osteochondritis Dissecans 799, 865, 879, 910 Osteochondroma 720, 757 Osteochondroses 911, 1006 Osteochondrosis 906 Osteoclast 962 Osteofibrous dysplasia 771, 775, 776 Osteogenesis Imperfecta (OI) 976, 980, 1007 Osteoid 738 Osteoid Osteoma 720, 743, 746, 748, 763, 1005, 1008 Osteolysis (Post-Traumatic - Clavicle) 941 Osteolysis of Gorham 809 Osteoma 720, 743, 745, 857 Osteomalacia 711, 714, 809, 900 Osteomyelitis 718, 748, 821, 824, 825, 827, 830, 856, 967, 1026 Osteomyelitis (salmonella) 1010 Osteonecrosis 769, 906, 999 Osteonecrosis (Spontaneous) 910 Osteopathia striata 744 Osteopenia 714, 900, 979, 1010 Osteopetrosis 856 Osteophyte (button) 922 Osteophyte formation 921 Osteopoikilosis 744 Osteoporosis 711, 900, 976, 979, 1008 Osteoporosis (transient) 909 Osteoporosis circumscripta 813

Osteosarcoma 743, 750, 751, 769, 857, 1012 Intramedullary 751 Juxtacortical 752 Parosteal 753 Telangiectatic 752 Osteosarcoma (Sclerosing) 754 Osteosarcoma: Low Grade Intramedullary 754 Osteosarcoma: Soft Tissue 755 Extraskeletal 755 Osteosarcoma : Intracortical 756 Osteosarcomatosis 755 Osteosclerosis 711, 714 Overhanging edge 914 Overhanging edge (Gouty Arthritis) 950 Oxalosis 718 Oxyhemoglobin 848 P V N S 793 Paget Disease 812, 1013, 1017 Paget’s disease 856 Pain (Congenital Insensitivity) 953 Palmar Fibromatosis 779 Palmer 1003 Pancreatitis 907 Panne 911 Panner’s disease 959 Paralabral Cyst 937 paramyxovirus (measles) 812 Parathormone (PTH) 711 Paravertebral soft tissue 828 Paronychia 825 Parosteal Osteosarcoma 753 Parrot Beak 862 Parsonage Turner Syndrome 958 Partial thickness (Rotator Cuff Tear) 928 Patellar Dislocation 866 Patellar Retinacula 867, 869 Patellar Tendinitis 870 Patellar tendon 869 Patellofemoral Joint 870 Patellofemoral Syndrome 870 Pathologic Fractures 961 Pattern of Bone Destruction and Lesion Margin 725 Geographic 725 Motheaten 725 Permeative 725 Transition Zone 725 PCL 867, 868 PCL Tear 868 Pectoralis Major Tear 931 Pedicle erosion 981 Pedicle sclerosis 749 Pedicolaminar Fracture-Separation 844 Pelvic Ring Disruption 998 Pelvic Ring Fractures 995 Pelvic Stress Injury 993 Pelvis Trauma 995 Pencilling (Long Bones) 981 perched facets 842 Percutaneous ablation (radiofrequency) 748 Percutaneous removal (Osteoid Osteoma) 748 Embolization 748 Radiofrequency 748 I8

Periapical cemento-osseous dysplasia 854 Periapical cyst 850 Periapical granuloma 850 Periarticular Calcification 715 pericapsular fat planes 826 Periodontal ligament 850 Periosteal Osteosarcoma 753, 1014 Periosteal reaction 723 Periosteal Reaction (Intramedullary Chondrosarcoma) 765 Periosteal reaction (PTH) 711 Periosteal Reaction: Aggressive 731 Codman triangle 731 Hair-On-End 731 Laminated 731 Sunburst 731 Periosteal Reaction: Nonaggressive 730 Buttressing 730 Expansion 730 Septation 730 Solid 730 Periosteal/juxtacortical (Chondrosarcoma) 764 Periostitis 914 Permeative (Bone Tumors) - Differential Diagnosis 727 Peroneus Brevis 884 Peroneus Brevis Split Syndrome 885 Peroneus Longus and Brevis 884 Peroneus Quartus 886 Perthes Lesion 934 Pes anserine 870 Phemister triad 832 Phlebolith 806 Picture frame 814 Pigmented Villonodular Synovitis (PVNS) 793 Pillar Fracture 844 Pisotriquetral joint 872 Plantar Fascia 882 Plantar Fasciitis 882 Plantar Fibromatosis 780, 882 Plantaris Tendon 884 Plasmacytoma 720, 966 PNET 964, 967 POEMS syndrome 964, 965 Polyarthritis 983 Polymyositis 976 Polyostotic 773, 812 Polyostotic Lesions 732 Angiomatous lesions 732 Malignant 732 Neurofibromatosis (type 1) 732 Paget disease 732 Popliteus hiatus 870 Popliteus tendon 859 Positive Rim Sign 943 Posterior Dislocation (Glenohumeral) 943 Posterior Impingement (Elbow) 956 Posterior Instability (Glenohumeral) 935, 943 Posterior recesses 870 Posterior Superior Glenoid Impingement 936 Posterior talofibular 881 Posterior Tibial Tendon 884 Posterior tibiofibular ligament 880 Posterior Vertebral Scalloping 1020

Posterolateral Rotatory Instability (Elbow) 960 Post-traumatic cyst 784, 790 Pregnancy 907 Prepatellar 870 Primary Lymphocytic Lymphoma of Bone 966 Primary Lymphoma 966 Primary Periarticular HA Crystal Deposition Disease: (Calcific Tendinitis) 952 Prostaglandin (Osteoma) 746 Prostate Carcinoma 962 Pseudarthrosis (Tibia) 981 Pseudogout 950 Pseudomonas 821 Pseudotumor (Hemophiliac) 973 Pseudoxanthoma Elasticum 792 Psoriasis 916 Psoriatic arthritis 912, 916, 1009 Psoriatic Sacroiliitis 1010 Pubic fracture 998 Pulley Injuries 948 Puncture wounds 825 Putti Platt 938 PVNS 792, 973, 1006 Pyknodysostosis 1007 Pyomyositis 826 Pyrophosphate arthropathy 950, 951 Quadriceps tendon 869 Quadrilateral Space Syndrome 930 RA 869 Rachitic rosary 901 Radial Collateral Ligament 959 Radial Fracture 945 Radial head 944 Radial head dislocation 945 Radial nerve impingement 956 Radial Styloid Hutchinson’s/ Chauffer’s Fracture 945 Radial tunnel syndrome 956 Radiation 757 Radiation - internal synovectomy 795 Radiation Induced Chondrosarcoma 764 Radiocapitellar Line 944 Radiolucent Lesions Multilocular (Macho-Macho) 852 Periapical 850 Pericoronal 852 Radiopaque and Mixed Lesions Ground Glass 856 Interradicular 855 Multifocal Confluent 856 Periapical 854 Target Lesion, Dense 857 Radioulnar ligaments 875 Raynaud’s phenomenon 984 Reactive Arthritis 917 Rectus femoris 869 Recurrent Multifocal Osteomyelitis 827 Reflex sympathetic dystrophy 976 Reiter Disease 912, 916, 917, 1010 Renal Cell Carcinoma 963 Renal disease 869 Renal Insufficiency - Chronic (MSK Manifestations) 711 Renal Osteodystrophy 711, 903 I9

Renal Tubular Disorders 903 Rhabdomyosarcoma 769, 781, 802, 964, 967 Rheumatoid Arthritis 797, 817, 912, 914, 941 Rheumatoid arthritis, JRA 748 Ribbon ribs 1029 Rickets 714, 900, 971 Acetabuli Protrusio 714 Basilar invagination 714 Triradiate pelvis 714 Rim Rent Tear (Rotator Cuff) 929 Ring sequestra 825 Rolando Fracture 947 Romanus and Andersson lesion 918 Rotator cuff 932 Atrophy 930 Tears 915, 925 Types 928 Rowe 1003 Saber shin 834 Sacral Lesions: Differential Diagnosis 786 Sacroiliac disease 918 Sacroiliitis 1010 Sacrospinous ligaments 997 Sacrotuberous ligament 997 Saddle nose 834 Salmonella 821 Salter-Harris Fracture 1011 Sanders Classification 1003 SAPHO 827 Sarcoid 837 Sarcomatous transformation 817 Sartorius 869 Saucerization (Juxtacortical Chondroma) 762 Saunders 1003 Sausage digit 912 Scaphoid fracture 945 Scapholunate Ligament 946 Disruption 946 Scapular “Y” View 942 Scapular Fractures 942 Schatzker Classification (Tibial Plateau Fractures) 1001 Scheuermann Disease 911 Sclerodactyly 984 Scleroderma 793, 976, 985 Sclerosing osteomyelitis of Garre 825 Sclerosing Osteosarcoma 754 Scoliosis 901, 981 Scurvy 900, 904, 977 Secondary chondromatosis - trauma 799 Secondary Chondrosarcoma 764 Secondary osteoarthritis 826 Segond fracture 807, 1001 Semimembranosus 869, 870 Semitendinosus 869 Senile osteoporosis 977 Septic Arthritis 718, 748, 825 Septic bursitis 826 Septic tenosynovitis 826 sequestra 824, 967 Sequestra-Like Appearance 1005 Sequestrum 820 Seronegative Spondyloarthropathy 1009

Serpentine sclerosis 907 Serpentine vessels 806 Sever 911 Shepherd’s Crook 774 Shiny corner sign 918 Sickle cell anemia 818, 968, 1010 Simple Bone Cyst 786 Sinding-Larsen-Johansson 911 Sinus lesions 745 Sinus Tarsi Syndrome 882 Sinus tracts 822 Skeletal Metastases 961 Skull: beveled edge, button sequestrum 891 SLAC Wrist 874 SLAP Tears 936 SLE 869, 976 SLE (Systemic Lupus Erythematosis) 983 Slipped Epiphyses 715, 826 Small cell carcinoma 964 Smith Fracture (Reverse Colles) 945 Soft Tissue abscess 826 Soft Tissue Chondroma 800 Soft tissue chondrosarcoma 1012 Soft Tissue Ganglion 795 Soft Tissue Hemangioma 805 Soft Tissue infection 825 Soft Tissue Lipomatous Tumors 893 Soft Tissue Masses Differential Diagnosis 804 Soft Tissue Neoplasm 733 Angiomatous lesions 733 Elastofibroma and fibromatosis 733 Lipomatous lesions 733 Neurogenic tumors 733 PVNS and ganglion 733 Soft Tissue Sarcoma Incidence 781 Solitary Focus Bone Scan 964 Souer and Remy 1003 Sphenoid 745 Spina ventosa 830 Spinal cord edema/hematoma 848 Spine Infections 828 Spinoglenoid Notch Entrapment 930 Spirochetes 834 Spondylitis 918 Spondyloarthropathies 912, 916 Spondylodiscitis 828, 830, 1023 Spondylolisithes 845 Spontaneous healing (Osteoid Osteoma) 748 Spontaneous osteonecrosis 865, 910 Sporotrichosis 836 Squamous cell carcinoma 852 Staph aureus 821, 828 Stener Lesion 947 Sterno-clavicular Joint 941 Steroid administration 953 Steroids 869, 907 Steroids (Osteopenia/Osteoporosis) 977 Stewart-Treve syndrome 810 Still Disease 919 Storioform 780 Stress fracture 748 Femur 1027 I 10

Stress Injuries 987 Subacute osteomyelitis 748, 824, 1007, 1026 Subchondral cyst 784, 790, 921, 1008 Subchondral cyst/intraosseous ganglion 763 Subchondral Resorption 713 Subchondral Sclerosis 923 Sublabral Foramen 933 Subligamentous extension 830 Subligamentous/Subtendinous Resorption 713 Subperiosteal (Osteoid Osteoma) 746 Subperiosteal abscess 822, 823 Subperiosteal Resorption 712 Subscapularis (Avulsion) 935 Subscapularis (Disruption) 935 Subscapularis Muscle 932 Subscapularis Tears 929 Subtrochanteric Fractures 1000 Subungual Exostosis 759 Sunburst 967 Super bone scan 718 Superior Glenohumeral Ligament 933 Superolateral migration (Osteoarthritis - Hip) 923 Supinator syndrome (Elbow) 956 Supracondylar fracture 944 Suprapatellar “bursa” 870 Suprascapular Nerve Entrapment 930 Supraspinatus Tendon (Tear) 935 Swan-Neck, Boutonniere deformities 914 Symmetric Polyarthritis 983 Symphysis pubis (Calcification) 951 Synchronous Osteosarcoma 750 Syndesmophyte 918 Synovial Chondroma 792 Synovial Chondromatosis 792, 799, 1006 Synovial Cyst 790, 792 Folds (Elbow) 956 Lipoma 792, 798 Osteochondromatosis 799 Plica 871 Sarcoma 781, 792, 793, 795, 802, 1012 Synovitis (Postoperative - Glenohumeral) 939 Syphilis 834, 953 Syringomyelia 848, 953 Systemic Lupus Erythematosis (SLE) 983, 1016 Tabetic arthropathy 953 Talar Neck Fractures 1002 Talus 995 Tarsal Coalition 880 Tarsal joints 1003 Tarsal Tunnel Syndrome 885 Telangiectasia 984 Telangiectatic Osteosarcoma 751 Tendinitis (Calcific) 952 Tendon Sheath 794 Tennis elbow 956 Tenosynovitis 876 Tenosynovitis (septic) 826 Tetracycline 748 TFCC 872 Thalassemia 968, 970 Thickened trabeculae 815

Thrombocytopenia 809 Thrombocytopenia with Absent Radii (TAR) 968, 972 Thumb Injury 947 Thyroid Cancer 963 Tibial collateral 870 Tibial Plateau Fractures 1001 Tile 995 Tile Classification 995 Tophi 950 Tori 856 Torus/ Buckle fracture 945 Transchondral fracture 879 Transient Osteoporosis 909 Transient Osteoporosis Hip 1015 Transient Regional Osteoporosis 977 Transverse Fractures (Pelvis) 999 Transverse ligament 863 Trap shooter’s shoulder 942 Trauma 839 Trauma (Dysbaric Disorders) 907 Trauma (Pelvis and Lower Extremity) 995 Trauma (Upper Extremity) 941 Traumatic bone “cyst” 850 Traumatic Spondylolisithes 845 Trevor Disease 759, 1020 Triangular Fibrocartilage (Calcification) 951 Triangular Fibrocartilage Complex 875 Triceps Injuries 958 Triquetrum Fracture 947 Triradiate pelvis 901 Trochlea 955 Trochlear sulcus 956 Trolley track sign 918 Tropical ulcer 834 Trough Sign 943 T-score 978 Tuberculosis 829, 973 Tuberculous Arthritis 748, 832 Osteomyelitis 830 Spondylodiscitis 830 Tubulation (Osteochondroma) 758 Tumoral Calcinosis 792, 1012 Turner syndrome 808 UCL and ulnocarpal ligaments 875 Ulcer (tropical) 834 Ulcerative Colitis 912 Ulcers 825 Ulnar fracture 945 nerve 872 Tunnel Syndrome 877 Unicameral bone cyst (UBC) 784, 944 Unilateral Facet Injury 841 Unusual infection 818 Upper Extremity Trauma 941 Van Neck 911 Vanishing Bone Disease 809 Vastus lateralis 869 Vastus medialis 869 Ventral (anterior) SIJ ligament 997 Vertebra Plana 891 I 11

Vertebral scalloping 981 Vertebroplasty 808 Vertical shear (Pelvic Trauma) 996 VISI deformity 875 Vitamin D: Prohormone 900 Volar Plate Avulsion 948 Voorhoeve Disease (Osteopathia Striata) 744 Wall Fracture (Pelvis) 999 West Point View 942 Whipple 912 Whiskering 914 Widened hip joint 826 Wimberger sign 834 Wormian Bones 1007 Wrisberg 859 Wrist 872 Wrist Subluxations 1019 Xenograft 709 X-linked hypophosphatasia 903 Yaws 834 Young-Burgess 995 Young-Burgess Classification 996 Young-Burgess vs Tile Classifications 995 Yttrium 90 795 Zonal pattern 801 Zonal phenomena 801 Z-score 978

I 12

Radiologic Pathology Fifth Edition VOLUME 3 Neuroradiology and Pediatric Radiologic Pathology Correlation

2006 2007

Editors Angela D. Levy, COL, MC, USA Chairman and Registrar Chief, Gastrointestinal Radiology

Ellen M. Chung, LTC, MC, USA Chief, Pediatric Radiology

Jeffrey R. Galvin, MD Chief, Chest Radiology

Kelly K. Koeller, MD Chief, Neuroradiology

Mark D. Murphey, MD Six Week Course Director Chief, Musculoskeletal Radiology

Paula J. Woodward, MD Chief, Genitourinary Radiology

Associate Editor Jean-Claude Kurdziel, MD

Illustrators Aletta A. Frazier, MD Dianne D. Engelby, MAMS, RDMS Heike Blum, MFA

Department of Radiologic Pathology Armed Forces Institute of Pathology Washington DC, USA

American Registry of Pathology Armed Forces Institute of Pathology Washington, DC 20306-6000 _____________________________________ © Copyright 2006 by the American Registry of Pathology. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means: electronic, mechanical, photocopy, recording, or any other information storage and retrieval system without written permission of the publisher. Made in the United States of America _____________________________________ Great care has been taken to guarantee the accuracy of the information contained in this volume. However, neither the American Registry of Pathology, Armed Forces Institute of Pathology, nor the editors and contributors can be held responsible for errors or for any consequences arising from the use of the information contained herein. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as representing the views of the Departments of the Army, Air Force, Navy, or Defense. 987654321 Library of Congress Cataloging-in-publication Data [in process] ISBN 1-933477-00-8

Preface The Armed Forces Institute of Pathology’s Radiologic Pathologic Correlation course presented by the Department of Radiologic Pathology enters its 59th year of educating radiology residents worldwide. For the fifth year, our staff and visiting lecturers have contributed their lecture material and images to compile Radiologic Pathology 2006 – 2007, continuing the tradition of presenting richly illustrated material that teaches the pathologic basis of disease to improve our understanding of the imaging appearance of disease. We hope the efforts of our authors and editors have once again accomplished our goal of bringing the outstanding and unique Radiologic Pathologic Correlation course to your fingertips.

Acknowledgements The annual production of the Radiologic Pathologic Correlation course and syllabus is made possible through the tremendous support, dedication, and selfless service of countless individuals who work in the AFIP and the various institutions and organizations throughout the world that believe in the importance of teaching the principles of disease through radiologic pathologic correlation. The Department of Radiologic Pathology of the Armed Forces Institute of Pathology expresses our deepest appreciation and sincerest gratitude to: - All radiologists and radiology residents who have contributed case material to the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of Pathology, - All pathologists in the AFIP who have donated their time and expertise to radiologic pathologic correlation, - All of our outstanding authors, illustrators, and department staff members who make the course and the syllabus happen effortlessly year after year, - And, to the extraordinary efforts of our production team, headed by JeanClaude Kurdziel, MD, who have tirelessly dedicated the spring and summer of the last five years to the production of this syllabus.

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Faculty – VOLUME 3 Neuroradiology

Pediatric Radiology

Kelly K. Koeller, MD

Ellen M. Chung, LTC, MC, USA

Kelly K. Koeller, MD, FACR Chief, Neuroradiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Associate Professor of Radiology Mayo Clinic Rochester, MN

Chief, Pediatric Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Dorothy I. Bulas, MD Professor of Radiology and Pediatrics Children's National Medical Center The George Washington University School of Medicine and Health Sciences Washington, DC

Patricia A. Hudgins, MD Professor of Radiology Emory University Medical Center Atlanta, GA

Gael J. Lonergan, MD Chief of Radiology Children's Hospital of Austin Austin, TX

Mary E. (Lee) Jensen, MD Director of Interventional Neuroradiology Professor of Radiology and Neurosurgery University of Virginia Health System Charlottesville, VA

William E. Shiels II, D.O. Chairman, Department of Radiology Children 's Hospital Columbus, OH

Erin Simon Schwartz, MD Assistant Professor of Radiology University of Pennsylvania School of Medicine Pediatric Neuroradiologist The Children's Hospital of Philadelphia Philadelphia, PA

Marilyn J. Siegel, MD Professor of Radiology and Pediatrics Mallinckrodt Institute of Radiology Washington University Medical School St. Louis, MO and Former Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

James G. Smirniotopoulos, MD Professor of Radiology, Neurology, and Biomedical Informatics Chair, Radiology and Radiological Sciences Uniformed Services University of the Health Sciences Bethesda, MD Wendy R. K. Smoker, MS, MD, FACR Professor of Radiology University of Iowa Medical Center Iowa City, IA

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Table of Contents – VOLUME 3 Neuroradiology Kelly K. Koeller, MD, FACR Imaging of Demyelinating Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1037 Lymphoma and Uncommon Neuroepithelial Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1045 Cerebral Intraventricular Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1058 Imaging of the Temporal Bone: Anatomy and Congenital Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1068 Imaging of the Temporal Bone: Infectious and Neoplastic Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1076 Imaging of the Orbit: The Globe and Conal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1088 Imaging of the Orbit: Intraconal and Extraconal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1097

James G. Smirniotopoulos, MD Patterns of Location: Infratentorial and Supratentorial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1106 Patterns of Enhancement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1126 The WHO 2000 Brain Tumor Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1137 Non-Astrocytic Gliomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1149 Extraaxial Tumors: Other Non-Glial Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1158 Neoplasms of the Meninges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1164 Pinealomas and, other Pineal Region Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1175 The Phakomatoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1184

Mary E. Jensen, MD Subarachnoid Hemorrhage and Intracranial Aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1210 Intracranial Vascular Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1220

Patricia A. Hudgins, MD Intracranial Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1231 Paranasal Sinuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1240 Sella and Parasellar Region . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1250

Erin Simon Schwartz, MD Congenital Spinal Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1260

Wendy R. K. Smoker, MS, MD, FACR Imaging of the Suprahyoid Neck: Superficial, Parapharyngeal and Carotid Spaces . . . . . . . . . . . . . . . . .1266 Imaging of the Suprahyoid Neck: Masticator and Parotid Spaces . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1274 Imaging of the Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity . . . . . . . . . . . . . . . . . . . .1282 Spine: Degenerative Disease and Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1290 Spinal Tumors, Cysts, and Mimics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1298 Congenital Abnormalities of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1307

Kelly K. Koeller, MD, FACR Neuroradiology Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1318 Neuroradiology Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1322

Pediatric Radiology Ellen M. Chung, LTC, MC, USA Childhood Urinary Tract Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1329 Neonatal GI Tract Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1341 Acute GI Disorders of Infants and Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1353 Diseases Affecting The Pediatric Airway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1363 Vascular Rings and Slings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1374 Cystic Renal Disease of Childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1381

Marylin J. Siegel, MD Pediatric Renal Tumors: Infancy and Young Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1390 Pediatric Adrenal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1402 Pediatric Pelvic Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1414 Bone Marrow Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1425 Congenital Lung Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1435 Lung Diseases in Neonates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1444 Pediatric Cardiac Imaging Part I: Vascular Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1453 Pediatric Cardiac Imaging Part II: Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1463

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Gael J. Lonergan, MD Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1472 Forensic Radiology of Child Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1491

Dorothy I. Bulas, MD Neonatal Brain: Radiologic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1501

William E. Shiels II, DO Pediatric Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1509 Pediatric Hip Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1518

Ellen M. Chung, LTC, MC, USA Pediatric Radiology Seminar I: Pulmonary Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1521 Pediatric Radiology Seminar II: Skeletal Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1527 Pediatric Radiology Seminar III: Cystic Fibrosis & Pulmonary Infections of Immunocompromised Child .1535

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Neuroradiology

Imaging of Demyelinating Diseases Kelly K. Koeller, MD, FACR Imaging Hallmarks •

• • •

White matter location ➢ May involve basal ganglia Little or no mass effect Usually no calcification May or may not enhance

Figure 5-1-1

Normal Lesions [Figure 5-1-1] •

•

Virchow-Robin spaces ➢ Perivascular space of deep penetrating vessels ➢ Follows CSFsignal intensity Ependymitis granularis ➢ Frontal horn

Ependymitis granularis [Figure 5-1-2] • •

• •

Frontal horn “capping” “Swamp” of the brain Perivascular spaces follow CSF signal ➢ Axons with low myelin content on all MR sequences ➢ Interstitial CSF backed up ➢ Loss of ependyma Periventricular hyperintensity: increased in ischemic WM states No lymphatics in the brain

Demyelinating Disorders • • • • •

Multiple sclerosis Vascular Viral / post-viral demyelination Toxic / metabolic encephalopathies Iatrogenic white matter degeneration

Figure 5-1-2

Multiple Sclerosis •

• • •

Unknown etiology ➢ Viral: measles, Epstein-Barr virus (EBV) ➢ Genetic: chromosome 6, human lymphocyte antigens (HLA) loci ➢ Autoimmune: associations with Graves’, myasthenia gravis, ulcerative colitis, Crohn's, SLE Females (60%): especially with optic neuritis 95% cases: 18-50 years old Cooler climates: northern Europe, North America; southern South America

Multiple Sclerosis •

• •

Relapsing-remitting: 70% ➢ Numbness, dysesthesia, burning sensations ➢ 2 clinical attacks from 2 separate lesions ❖ At least 24 hours and at least 30 days apart Ependymitis granularis ➢ Partial or complete remission for months or years Progressive: 20% ➢ Primary progressive: slow onset without distinct attacks ➢ Secondary progressive: relapsing-remitting form with progressive disability “Monosymptomatic demyelinating”: 10%

McDonald et al, Ann Neurol 2001: 50-121-127

Neuroradiology

1037

Imaging of Demyelinating Disease

Multiple Sclerosis - Clinical • • •

Figure 5-1-3

Uthoff's phenomenon: symptoms worsened with exposure to heat Children: very rare especially before puberty Pregnancy ➢ Decreased risk during 2nd and third trimester ? ➢ ...but exacerbation rate of 75% in first 6 months post-partum

Multiple Sclerosis - Pathology • • •

Microglial infiltration Myelin disintegration and Perivascular cuffing of focal hypercellularity lymphocytes --> Predilection for periventricular zone

Active (enhancing) MS plaque compared to chronic (non-enhancing) MS plaque

Multiple Sclerosis - Imaging •

• • •

Periventricular distribution: classic ➢ 3 or more lesions during “event”: sensitive indicator for MS within 10 years Ovoid lesions often perpendicular to walls of ventricles (Dawson's fingers) Little mass effect for size of lesion Corpus callosum-septum pellucidum interface lesions: increase specificity and sensitivity of diagnosis

Figure 5-1-4

Radiology 1991;180:467-474; Radiology 1991; 180:215-221; Neurology 2003; 61:602-611

Multiple Sclerosis - Imaging [Figure 5-1-3] • •

Active plaques enhance (ring-like in some) Chronic lesions: do not enhance

Atrophy in chronic MS

McDonald Criteria - MR •

•

Dissemination in space (3 or more) ➢ 1 enhancing lesion or 9 T2 hyperintense lesions ➢ 1 or more infratentorial lesion ➢ 1 or more juxtacortical lesion ➢ 3 or more periventricular lesions Dissemination in time (at least 1) ➢ MRI more than 3 months after clinial event, enhancing lesion at different site ➢ No enhancing lesion: new T2 lesion or enhancing lesion on f/u study at least 3 months later ➢ MRI less than 3 months, new enhancing lesion on f/u study

Figure 5-1-5

Multiple Sclerosis – MR [Figure 5-1-4] • • •

Chronic cases: atrophy Fluid-attenuated inversion recovery (FLAIR): hyperintense MR Spectroscopy ➢ Decreased N-acetyl aspartate (NAA) in chronic plaques ➢ Increased choline, lipids and lactate

Imaging of Demyelinating Disease

1038

Tumefactive MS plaque. Lack of mass effect and “non-neoplastic’ (NAA > choline) MR spectrogram are important clues to correct diagnosis

Neuroradiology

“Tumefactive” Multiple Sclerosis [Figure 5-1-5] • •

Tumor-like but… Lack of mass effect: most important clue for demyelination

Figure 5-1-6

Multiple Sclerosis – Variant Types [Figure 5-1-6] •

• •

Concentric sclerosis (Balo's) ➢ Alternating bands of myelination and demyelination, often in concentric fashion Acute: (Marburg) ➢ Rapid course ➢ Death in months ➢ Severe axonal loss Neuromyelitis optica (Devic syndrome) ➢ Both visual and spinal cord signs

Multiple Sclerosis vs. Transverse Myelitis • •

Balo concentric sclerosis

[Figure 5-1-7]

•

Spinal cord MS plaques: 7% Multiple sclerosis: peripheral, usually less than two segments, limited to one side ➢ Clinical cord syndrome: 60% had brain lesions Transverse myelitis: usually holocord, commonly involves gray matter

Figure 5-1-7

Tartaglino et al, Radiology. 1995;195:725-32; Tartaglino et al, Radiology. 1996;201:661-9

Vascular White Matter Disease • • • • • • •

Microangiopathy Arteriosclerosis / venous collagenosis Hypoxic-ischemic encephalopathy Posterior Reversible Encephalopathy Syndrome (PRES) Amyloid angiopathy Vasculitis Migraine

Transverse myelitis

Edwards, ed, Neuroimaging clinics 1993

Senescent White Matter Changes [Figure 5-1-8] •

•

•

“Microangiopathy”, “deep white matter ischemia, leukoariosis, etc.” ➢ Demyelination, axonal loss, gliosis, ischemic changes 30%-80% "normal" elderly patients ➢ More lesions, more likely to have neuropsychologic and cognitive problems ➢ ? correlation with dementia ➢ “Binswanger's”: clinical diagnosis, reserved only for dementia cases Do not involve corpus callosum

Figure 5-1-8

Arteriosclerosis •

• •

Long penetrating end arteries ➢ Few or no collateral vessels Pons, thalami, basal ganglia, deep white matter Prevalence increased with age and history of stroke

Venous Collagenosis • • •

Noninflammatory stenosis Occlusion of subependymal veins Associated with periventricular white matter changes

Senescent white matter changes. Note subcortical location

Moody et al, Radiology 1995; 194:469-476 Neuroradiology

1039

Imaging of Demyelinating Disease

Posterior Reversible Encephalopathy Syndrome (PRES) •

Figure 5-1-9

[Figure 5-1-9]

• •

Hypertensive events: renal failure, pre/eclampsia, immunosuppressive drugs Loss of normal autoregulation: elevated hydrostatic pressure mediated by venous vasoconstriction? Posterior cerebral circulation: less sympathetic innervation, less ability to vasoconstrict ➢ Visual field deficits, headache, somnolence ➢ T2 hyperintensity ➢ Diffusion: usually increased (not restricted) ➢ Perfusion (CBV, CBF): decreased, normal, or increased

Casey et al, AJNR 2000; 21:1199-1206 Brubaker et al, AJNR 2005; 26:825-830; Schuuring et al AJNR 2003; 24:2085-2088

Senescent white matter changes. Note subcortical location

Viral and Postviral Demyelination • • • •

Encephalitis Acute disseminated encephalomyelitis Subacute sclerosing panencephalitis Human immunodeficiency virus infection and complications ➢ HIV encephalitis ➢ Progressive multifocal leukoencephalopathy

Acute Disseminated Encephalomyelitis (ADEM) • • • • • •

1-3 weeks post-infection or vaccination Monophasic: rubeola, vaccinia, varicella, mycoplasma, mumps, rubella ➢ No virus or bacteria isolated on autopsy Hemorrhagic type (Hurst variant): rapidly progressive onset Children > adults Good prognosis overall but 10%-20% significant neurological deficit or death Diagnosis of exclusion: long-term follow-up needed to rule out MS

Honkaniemi et al, AJRN 2001; 22:1117-1124; Rosman et al, J Child Neurol. 1997; 12:448-54

Figure 5-1-10

ADEM – Pathology and Imaging • • •

Autoimmune response —-> perivenous demyelination CT: normal or nonspecific hypoattenuation No mass effect

ADEM: MR Findings [Figure 5-1-10] •

• •

Asymmetric WM lesions ➢ Varying in size and number ➢ Little or no mass effect Variable enhancement Optic neuritis, myelitis

Kesselring et al, Brain 1990;113:291-302

Imaging of Demyelinating Disease

1040

Neuroradiology

Progressive Multifocal Leukoencephalopathy (PML) • •

• • •

Figure 5-1-11

Defective cell-mediated immunity Marked decrease in prevalence with highly active anti-retroviral therapy (HAART) ➢ Pre-HAART: 1-7% of AIDS patients; 55-85% cases related to AIDS 5–84 y/o; peak: 6th decade JC virus (papovavirus) reactivation ➢ Affects oligodendrocytes: demyelination Extremely poor prognosis (death in 6 months) if untreated

Baqi et al, AIDS 1997; 11:1526-7

PML – Path and Imaging [Figure 5-1-11] •

• • •

Predominantly parieto-occipital and frontal ➢ Posterior fossa: 1/3 cases ➢ Subcortical white matter Typically no mass effect or enhancement ➢ Enhancement indicative of long-term survival? Characteristic scalloped lateral margin at gray matter-white matter junction May show hemorrhage

Whiteman et al, Radiology 1993; 187:233-240; Thurnher et al AJNR 2001; 22:977-984 PML with relative lack of mass effect and sparing of cortical gray matter

HIV Encephalitis [Figure 5-1-12] • • • • •

Much less common with anti-retroviral therapy Deep white matter and gray matter Psychomotor slowing, mental status changes, memory problems, apathy Direct or indirect infection of oligodendrocytes Demyelination and vacuolation ➢ Axonal loss and microglial nodules

Figure 5-1-12

Thurnher et al AJNR 2001; 22:977-984

HIV Encephalitis • •

•

Imaging often normal early in course Diffuse mild cerebral atrophy ➢ Cortical first, then central Ill-defined patchy areas ➢ Central white matter, basal ganglia, thalamus ➢ Bilaterally symmetric ➢ Usually no necrosis or edema ➢ No enhancement

Thurnher et al AJNR 2001; 22:977-984; Olson et al, Radiology 1988; 169:445-448

HIV encephalitis with characteristic cortical atrophy

Toxic Demyelination • •

• • •

Alcohol Ion balance disorders ➢ Osmotic myelinolysis ➢ Extrapontine myelinolysis Organic toxins (lipophilic solvents) Carbon monoxide poisoning (“interval” form) Drug abuse (poisoned heroin)

Neuroradiology

1041

Imaging of Demyelinating Disease

Alcohol and the Brain [Figure 5-1-13 and 5-1-14] •

• •

Figure 5-1-13

Atrophy ➢ Cerebral hemisphere ➢ Superior vermis Marchiafava-Bignami disease ➢ Corpus callosum demyelination, necrosis ➢ Rare: cortical laminar necrosis Wernicke encephalopathy ➢ Thiamin deficiency ➢ Ophthalmoplegia, ataxia, confusion ➢ Medial thalamic nuclei ➢ Mamillary bodies: atrophy

Arbelaez et al, AJNR 2003; 24:1955-57; Johkura et al, AJNR 2005; 26:670-3; Donnal et al, AJNR 1990; 11:893-894 Marchiafava-Bignami disease with corpus callosal lesions. Note atrophy of cerebral hemisphere and superior cerebellar vermis

Osmotic Myelinolysis [Figure 5-1-15] • •

• • • •

“Central pontine myelinolysis” Variable clinical presentation ➢ Spastic quadraparesis, pseudobulbar palsy Incidence?: 0.16–3.7 % of autopsy cases Rapid osmotic change ➢ Vascular injury in gray matter - white matter apposition regions Demyelination: spares periphery of pons CT: Hypoattenuated

Figure 5-1-14

Ruzek et al, AJNR 2004; 25:210-213

Osmotic Myelinolysis [Figures 5-1-15 and 5-1-16] •

MR: ➢ T1WI: hypointense ➢ T2WI: hyperintense ➢ May return to normal in months to year ➢ Extrapontine (10%): basal ganglia, other sites

Iatrogenic Demyelinating Disorders: Chemotherapy •

Mineralizing microangiopathy ➢ Methotrexate ❖ Periventricular, centrum semiovale ❖ Patients < 5 y/o, meningeal leukemia, high-dose therapy: greatest risk

Wernicke encephalopathy with pathognomonic hyperintensity of both medial thalami

Figure 5-1-16

Davis et al, AJR 1986; 147:587-592; Cajade-Law et al in Neuroimag Clin, Edwards, ed. 1993;3:361-377

Figure 5-1-15

Osmotic (central pontine) myelinolysis on CT and T2 MR images Imaging of Demyelinating Disease

Extrapontine myelinolysis 1042

Neuroradiology

Radiation Injury [Figure 5-1-17] • •

•

Figure 5-1-17

Acute: no imaging findings Early delayed: >2 months after therapy ➢ White matter, basal ganglia, cerebral peduncles Late: ➢ Focal radiation necrosis: > 1 year ➢ Diffuse radiation injury: > 1 year ❖ Geographic pattern: conforms to radiation port ➢ Necrotizing leukoencephalopathy: as early as 3 months posttherapy

Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404

Radiation Necrosis [Figure 5-1-18] • •

• •

Tumor-like Metabolic imaging ➢ Increased activity: high-grade tumors ➢ Normal or decreased activity: radiation necrosis ➢ Less reliable for low-grade tumors MRS: increased lactate and choline in tumors vs. increased lactate in necrosis Diffusion-weighted imaging: ➢ Tumors: usually hypointense ➢ Necrosis: usually hyperintense

Radiation injury to white matter with typical "geographic" pattern of involvement

Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404

Demyelination Imaging Hallmarks •

• • •

Figure 5-1-18

White matter location ➢ May involve basal ganglia Little or no mass effect Usually no calcification May or may not enhance

Summary •

• •

• • •

Normal ➢ Virchow-Robin spaces: follow CSF ➢ Ependymitis granularis: frontal horn Multiple Sclerosis ➢ Periventricular ➢ Clinical diagnosis Vascular demyelination ➢ Senescent white matter changes ❖ Subcortical, do not involve corpus callosum ➢ Posterior Reversible Encephalopathy Syndrome (PRES) Viral / postviral demyelination ➢ ADEM ➢ PML ➢ HIV encephalitis Toxic / metabolic demyelination ➢ Alcohol ➢ Osmotic myelinolysis: centra pons Iatrogenic demyelination ➢ Chemotherapy and radiation injury

Radiation necrosis mimicking appearance of a glioblastoma multiforme

References 1. Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J Neuroradiol 2003; 24:1955-1957. 2. Baqi M, Kucharczyk W, Walmsley SL. Regression of progressive multifocal encephalopathy with highly active antiretroviral therapy. Aids 1997; 11:1526-1527. Neuroradiology

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3. Brubaker LM, Smith JK, Lee YZ, Lin W, Castillo M. Hemodynamic and permeability changes in posterior reversible encephalopathy syndrome measured by dynamic susceptibility perfusion-weighted MR imaging. AJNR Am J Neuroradiol 2005; 26:825-830. 4. Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluidattenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000; 21:1199-1206. 5. Davis PC, Hoffman JC, Jr., Pearl GS, Braun IF. CT evaluation of effects of cranial radiation therapy in children. AJR Am J Roentgenol 1986; 147:587-592. 6. Donnal JF, Heinz ER, Burger PC. MR of reversible thalamic lesions in Wernicke syndrome. AJNR Am J Neuroradiol 1990; 11:893-894; discussion 895-896. 7. Frohman EM, Goodin DS, Calabresi PA, et al. The utility of MRI in suspected MS: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2003; 61:602-611. 8. Gean-Marton AD, Vezina LG, Marton KI, et al. Abnormal corpus callosum: a sensitive and specific indicator of multiple sclerosis. Radiology 1991; 180:215-221. 9. Honkaniemi J, Dastidar P, Kahara V, Haapasalo H. Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol 2001; 22:1117-1124. 10. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol 2005; 26:670-673. 11. Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis. Brain 1990; 113 ( Pt 2):291-302. 12. McDonald WI, Compston A, Edan G, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001; 50:121-127. 13. Moody DM, Brown WR, Challa VR, Anderson RL. Periventricular venous collagenosis: association with leukoaraiosis. Radiology 1995; 194:469-476. 14. Nesbit GM, Forbes GS, Scheithauer BW, Okazaki H, Rodriguez M. Multiple sclerosis: histopathologic and MR and/or CT correlation in 37 cases at biopsy and three cases at autopsy. Radiology 1991; 180:467-474. 15. Olsen WL, Longo FM, Mills CM, Norman D. White matter disease in AIDS: findings at MR imaging. Radiology 1988; 169:445-448. 16. Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. J Child Neurol 1997; 12:448-454. 17. Rowley HA, Dillon WP: Iatrogenic white matter diseases. Neuroimaging Clin N Am 3:379–404, 1993 18. Ruzek KA, Campeau NG, Miller GM. Early diagnosis of central pontine myelinolysis with diffusion-weighted imaging. AJNR Am J Neuroradiol 2004; 25:210-213. 19. Schuuring J, Wesseling P, Verrips A. Severe tacrolimus leukoencephalopathy after liver transplantation. AJNR Am J Neuroradiol 2003; 24:2085-2088. 20. Tartaglino LM, Croul SE, Flanders AE, et al. Idiopathic acute transverse myelitis: MR imaging findings. Radiology 1996; 201:661-669. 21. Tartaglino LM, Friedman DP, Flanders AE, Lublin FD, Knobler RL, Liem M. Multiple sclerosis in the spinal cord: MR appearance and correlation with clinical parameters. Radiology 1995; 195:725-732. 22. Thurnher MM, Post MJ, Rieger A, Kleibl-Popov C, Loewe C, Schindler E. Initial and follow-up MR imaging findings in AIDS-related progressive multifocal leukoencephalopathy treated with highly active antiretroviral therapy. AJNR Am J Neuroradiol 2001; 22:977-984. 23. Whiteman ML, Post MJ, Berger JR, Tate LG, Bell MD, Limonte LP. Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology 1993; 187:233-240.

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Lymphoma and Uncommon Neuroepithelial Tumors Kelly K. Koeller, MD, FACR CNS Lymphoma •

• •

6.6%-15.4% of all primary brain tumors ➢ Only GBM, meningioma, and low-grade astrocytoma are more common Less than 1% of all body lymphomas Primary lymphoma much more common than secondary

Miller et al, Cancer 1994; 74:1383-1397; Henry et al, Cancer 1974; 34:1293-1302

Clinical •

•

Wide age range ➢ Peak: 4th to 5th decades ➢ Smaller peak: 1st decade (AIDS) Nonspecific clinical presentation ➢ Expanding mass lesion ➢ Encephalitis ➢ Stroke ➢ Cranial nerve palsies

Figure 5-2-1

Koeller et al, Radiographics 1997; 17:1497-1526

Immunocompromised Patients •

• • •

“Opportunistic neoplasm” ➢ Incidence much higher than in immunocompetent patients AIDS-defining diagnosis in HIV+ patients 2% of AIDS patients develop CNS lymphoma CNS mass lesion in AIDS patient ➢ Toxoplasmosis #1, lymphoma #2 ➢ Lymphoma: #1 in pediatric AIDS patient

Rosenblum et al, Ann Neurol 1988: 23:S13-S16; Koeller et al, Radiographics 1997; 17: 1497-1526 •

Two masses, both lymphoma, with one located around the ventricle while the other arises from the leptomeninges

Gross Pathology [Figure 5-2-1] • • •

Intra-axial nodule ➢ Grayish-pink, homogeneous, circumscribed ➢ Multifocal: 50% Leptomeningeal Uveal Intradural spinal

Figure 5-2-2

Koeller et al, Radiographics 1997; 17:1497-1526

Histopathology [Figure 5-2-2] • • •

“Small blue cell tumor” Almost always B-cell type Perivascular space

Koeller et al, Radiographics 1997; 17:1497-1526

Perivascular distribution of small blue lymphocytes (arrow, vessel) Neuroradiology

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Lymphoma & Uncommon Neuroepithelial Tumors

CT / MR Findings [Figures 5-2-3 to 5-2-5] •

• •

Figure 5-2-3

NCCT: Hyperattenuated mass ➢ Negative CT does not exclude diagnosis MR: ➢ T1WI: iso-to-hypointense to gray matter ➢ T2WI: hypointense ➢ Little mass effect for size of lesion Virtually all enhance ➢ Ring-like: necrosis; common in immunocompromised hosts

Lee et al, AJR 1986; 147:747-752; Schwaighofer et al, AJNR 1989; 10:725-729; Dina, Radiology 1991; 179:823-828

Figure 5-2-4 Lymphoma with characteristic CT hyperdensity and T2 hypointensity

Figure 5-2-5

CNS Lymphoma with true water restricted diffusion (DWI hyperintensity, left; ADC hypointensity, right)

General Neuroimaging Features [Figure 5-2-6] •

• •

Supratentorial location ➢ Deep gray matter: “classic”, 33% ➢ Cerebral white matter: 55% ➢ Cerebellar lesions: 10% Multiple lesions: 11%-47% ➢ More common in immunocompromised Recurrences: 50% at original site

Lymphoma in AIDS patient with ring-like enhancement secondary to necrosis

Koeller et al, Radiographics 1997; 17:1497-1526

Figure 5-2-6

General Neuroimaging Features • • •

•

“Hugs “ ependyma or leptomeninges “Butterfly” pattern: corpus callosum “Ghost” tumor: vanishes with steroid or radiation therapy ➢ Avoid prior to biopsy Rare: calcification, hemorrhage

Jiddane et al, J Neurosurg 1986; 65:592-599; Vaquero et al, J Neurosurg 1984; 60:174-176

Lymphoma of right basal ganglia

Lymphoma & Uncommon Neuroepithelial Tumors

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PET / SPECT-Thallium [Figure 5-2-7] • • • •

Figure 5-2-7

Hypermetabolic Non-neoplastic lesions: hypometabolic High specificity and sensitivity False positives: interpretation errors, occasional hypermetabolic abscess

Hoffman et al, J Nucl Med 1993; 34:567575; Villringer et al, J Comput Assist Tomogr 1995; 19:532-536

Neuroepithelial Tumors •

• • • • • • • •

•

Astrocytic ➢ Pleomorphic xanthoastrocytoma Toxoplasmosis or lymphoma ? Oligodendroglial PET image shows hypermetabolic activity consistent with Mixed glial lymphoma Ependymal Choroid plexus Pineal parenchymal Neuroblastic Glial of uncertain origin Neuronal and mixed neuronal-glial ➢ Ganglioglioma / Gangliocytoma ➢ Desmoplastic Infantile Ganglioglioma ➢ Dysplastic cerebellar gangliocytoma ➢ Dysembryoplastic neuroepithelial tumor ➢ Cerebellar Liponeurocytoma Embryonal ➢ Supratentorial PNET ➢ Atypical teratoid / rhabdoid tumor

Ganglioglioma / Gangliocytoma • •

• •

About 1% of all brain tumors Children and young adults ➢ 80% <30 years old; peak: 10-30 years of age ➢ Males slightly more common Most common tumor seen in chronic temporal lobe epilepsy ➢ 15%-25% of medically refractory seizures Cerebral hemisphere predilection ➢ Temporal lobe: most common (38%) ➢ Optic nerves, pituitary and pineal glands, spinal cord, ventricles, cranial nerve (1 report)

Johnson et al, Pediatr Neurosurg 1997; 27:203-207; Athale et al, Neuroradiology 1999; 16:790-792

Ganglioglioma / Gangliocytoma [Figure 5-2-8] • •

•

Good prognosis overall Ganglioglioma: WHO I or II ➢ Biphasic tumor: neoplastic ganglion cells and glial cells ➢ Malignant degeneration of glial cells (WHO III): 6% Gangliocytoma ➢ Only mature ganglion cells ➢ WHO grade I ➢ AFIP: really cortical dysplasia?

Figure 5-2-8

Silver et al, Surg Neurol 1991; 35:261-266; Kalyan-Raman and Olivero, Neurosurgery 1987; 20:428-433 Ganglioglioma with biphasic appearance on histology. Arrow points to a ganglion cell Neuroradiology

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Lymphoma & Uncommon Neuroepithelial Tumors

Ganglioglioma [Figure 5-2-9] •

• • • •

Figure 5-2-9

Typically peripheral mass ➢ Cystic / solid: 52% ➢ Solid: 43% ➢ Purely cystic: 5% Little mass effect or surrounding edema Calcification common Skull remodeling Enhancement variable

Zentner et al, AJNR 1994; 57:1497-1502; Dorne et al, AJNR 1986; 7:281-285; Castillo et al, AJNR 1990; 11:109-114

Ganglioglioma [Figures 5-2-10 to 5-2-12] •

•

Typical MR findings ➢ T1WI: iso-to-hypointense ➢ T2WI: hyperintense ➢ Occasional T1 hyperintensity ➢ May or may not enhance Leptomeningeal spread: rare

Castillo et al, AJNR 1990; 11:109-114; Tampieri et al, AJNR 1991; 12:749-755; Tien et al, AJR 1992; 159:391-393

Ganglioglioma with typical peripheral location and calcification

Ganglioglioma • •

Positron Emission Tomography (PET): heterogeneous metabolic activity MR Spectroscopy: increased choline-creatine ratio

Figure 5-2-10

Provenzale et al, AJR 1999; 172:1103-1107; Kumabe et al, Neurosurgery 1999; 45:183-187

Desmoplastic Infantile Ganglioglioma / Astrocytoma •

•

• •

First described in 1987 ➢ “Superficial cerebral astrocytoma” ➢ “Desmoplastic cerebral astrocytoma of infancy” Rare: 0.6% of brain tumors ➢ 16% of all infant brain tumors ➢ Vast majority: less than 1 year (range: up to 17 years) ➢ Males more common (2:1) Rapid onset: increasing head circumference Usually more than one lobe: typically frontal and parietal

Ganglioglioma in temporal lobe

VandenBerg et al, J Neurosurg 1987; 66:58-71; Taratuto et al, Cancer 1984; 54:2505-2512

Figure 5-2-12

Figure 5-2-11

Ganglioglioma in medial portion of right temporal lobe

Large ganglioglioma Lymphoma & Uncommon Neuroepithelial Tumors

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Desmoplastic Infantile Ganglioglioma / Astrocytoma [Figure 5-2-13] • •

•

Figure 5-2-13

WHO grade I Desmoplastic stroma ➢ Neuronal component: ganglioglioma ➢ Neoplastic astrocytes: astrocytoma Meningocerebral: involves cortex & leptomeninges

Duffner et al, Neurosurgery 1994; 34:583-589

Desmoplastic Infantile Ganglioglioma / Astrocytoma •

[Figure 5-2-14]

Large heterogeneous mass ➢ Cyst-like areas ➢ Superficial soft tissue area ❖ Slightly hyperattenuated ❖ Frequently attached to dura ❖ Intense enhancement ➢ No calcification ➢ Occasional vasogenic edema

Desmoplastic infantile ganglioglioma

Figure 5-2-14

Duffner et al, Neurosurgery 1994; 34:583-589; Martin et al, AJNR 1991; 12:1195-1197; Tenreiro-Picon et al, Pediatr Radiol 1995; 25:540-543

Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) •

• •

Original case, 1920 (Lhermitte and Duclos) ➢ 36-year-old male with progressive neurologic deficits ➢ Cerebellar mass: “diffuse ganglioneuroma” Dysplastic cerebellar gangliocytoma ➢ Numerous other names proposed Histogenesis?: hamartoma vs. neoplasm ➢ Hamartoma favored

Lhermitte and Duclos, Bull Assoc Fr Etude Cancer, 1920;9:99-107; Wiestler et al, WHO classification, 2000; 235-237

Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) •

50%: Cowden disease ➢ Autosomal dominant: susceptibility gene 10q23 ➢ Colonic polyps, cutaneous tumors, thyroid DIG with intensely enhancing soft tissue component tumors, breast tumors, meningioma, glioma along meningocerebral interface ➢ MR screening of family members recommended ➢ Diagnosis established when Lhermitte-Duclos combined with either: ❖ Thyroid cancer ❖ Breast cancer ❖ Macrocephaly

Robinson and Cohen, Neurosurgery 2000; 46:371-383; Kulkantrakorn et al, Neurology, 1997; 48:725-731; Nelen et al, Nat Genet 1996; 13:114-116

Neuroradiology

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Lymphoma & Uncommon Neuroepithelial Tumors

Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) •

Figure 5-2-15

CT findings ➢ Usually hypoattenuated ➢ Isoattenuated: “normal” ➢ Calcification uncommon

Meltzer et al, Radiology 1995; 194:699-703

Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) [Figure 5-2-15] • •

•

MR findings “Classic” appearance ➢ Cerebellar mass ➢ “Striated” ➢ No edema ➢ No enhancement Non-classic cases: non-specific appearance

Classic “striated cerebellum” appearance in Lhermitte-Duclos disease

Meltzer et al, Radiology 1995; 194:699-703

Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) •

T1 hypointensity / T2 hyperintensity: ➢ Inner molecular layer, granular cell layer, and loss of central white matter within folia

Figure 5-2-16

Kulkantrakorn et al, Neurology 1997; 48:725-731

Cerebellar Liponeurocytoma •

• •

First described in 1978 ➢ “Lipomatous medulloblastoma” ➢ 15 cases reported by year 2000 Similar to central neurocytoma except: ➢ Cerebellar location ➢ Older age group Peak age: 5th-6th decades (mean: 51 years old)

Kleihues et al, WHO classification 2000, 110-111

Cerebellar Liponeurocytoma • • •

•

No gender predilection Cerebellum or cerebellopontine angle WHO grade I or II ➢ Well-differentiated neuronal cells ➢ Focal lipomatous differentiation ➢ Low mitotic activity Good overall prognosis but few cases ➢ No role for radiation therapy or chemotherapy?

Kleihues et al, WHO classification 2000, 110-111

Cerebellar Liponeurocytoma [Figure 5-2-16] • • • •

Cerebellar mass CT: hypoattenuated with focal areas of fat attenuation MR: hypointense with scattered focal T1 hyperintensity Moderate enhancement

Kleihues et al, WHO classification 2000, 110-111; Cacciola et al, Acta Neurochir 2002; 144:829-833; Alkadhi et al 2001; J Neurosurg 95:324-331 Inherent T1 hyperintensity and mild enhancement in cerebellar liponeurocytoma Lymphoma & Uncommon Neuroepithelial Tumors

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Dysembryoplastic Neuroepithelial Tumor • • •

• •

Figure 5-2-17

First described in 1988 (Daumas-Duport et al) Benign tumor of cortex or deep gray matter Children and young adults with partial seizures ➢ Peak age: 10-30 years ➢ Males more common Neurologic deficits not common Excellent prognosis even with partial resection ➢ Very rare malignant transformation

Daumas-Duport et al, Neurosurgery 1988;23:545-556; Daumas-Duport et al, WHO classification, 2000; Hammond et al, J Neurosurg 2000;92:722-725

Dysembryoplastic Neuroepithelial Tumor • • • • •

[Figure 5-2-17]

•

WHO Grade I Simple form vs. complex form: controversial Multinodular: complex form Cortical dysplasia: focal “Specific glioneuronal element”: columnar pattern ➢ “Freely floating neurons” Temporal lobe: 62% ➢ Frontal lobe: 31% ➢ Other sites: caudate nucleus, cerebellum, pons

DNT with “floating neuron”

Figure 5-2-18

Daumas-Duport et al, Neurosurgery 1988;23:545-556; Daumas-Duport et al, WHO classification, 2000

Dysembryoplastic Neuroepithelial Tumor •

[Figure 5-2-18]

•

CT findings ➢ Hypoattenuated ➢ Calcification: uncommon (~ 5%) ➢ May produce remodel skull ➢ No surrounding edema ➢ May or may not enhance MR findings ➢ T1WI: hypointense ➢ T2WI: hyperintense ➢ May or may not enhance ➢ Occasional “soap-bubble” appearance ➢ More multinodular than gangliogliomas?

Koeller and Dillon, AJNR 1992;13:1319-1325; Kuroiwa et al, JCAT, 1994;18:352-356; Ostertun et al, AJNR 1996;17:419430

Pleomorphic Xanthoastrocytoma •

• • •

Originally described in 1979 (Kepes et al) ➢ 12 cases of supratentorial tumors involving the leptomeninges Believed to arise from subpial astrocytes of the cortex Less than 1% of all brain neoplasms Importance: characteristic imaging appearance, highly amenable to surgical resection

Kepes et al, Cancer 1979:44:1839-1852

DNT in classic cortical location and showing exophytic extension with pressure erosion of inner table of skull Neuroradiology

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Pleomorphic Xanthoastrocytoma •

• • •

Usually adolescents or young adults (average age: 26 years) ➢ Wide age range: 5-82 years No gender predilection Long history of seizures Supratentorial: 98% ➢ Temporal lobe: 47% ➢ Multi-lobe: 10% ➢ Rare: thalamus, cerebellum, spinal cord, orbital globe

Giannini et al, Cancer 1999; 85:2033-2045; Pahapill et al, Neurosurgery 1996; 38:822-829

Figure 5-2-19

Pleomorphic Xanthoastrocytoma [Figure 5-2-19] •

WHO Grade II: ➢ Pleomorphism ➢ “Lipidized” neoplastic glial cells: xanthoma-like ➢ Overall good prognosis ❖ 81% 5-year survival ❖ 70% 10-year survival ➢ Higher recurrence rate ➢ Malignant transformation: 20%

Kepes et al, WHO classification 2000, 52-54; Giannini et al, Cancer 1999; 85:2033-2045

Pleomorphic Xanthoastrocytoma • • • •

“Classic”: large cystic lesion with an enhancing mural nodule (<50%) Meningocerebral location ➢ Meningeal infiltration rare ➢ Cortical involvement common ➢ Perivascular space extension Calcification rare Usually no surrounding edema

PXA with vacuoles secondary to “lipidized” neoplastic glial cells

Figure 5-2-20

Tien et al, AJR 1992:159:1397-1404; Lipper et al, AJNR 1993; 14:1397-1404; Pahapill et al, Neurosurgery 1996; 38:822-829 •

Pleomorphic Xanthoastrocytoma [Figure 5-2-20] MR findings ➢ Heterogeneous mass ➢ T1WI: hypo-to-isointense ➢ T2WI: hyper-to-isointense ➢ Soft tissue component usually enhances intensely ➢ Leptomeningeal enhancement: characteristic

PXA as large heterogeneous mass and enhancing soft tissue component

Lipper et al, AJNR 1993; 14:1397-1404

Supratentorial Primitive Neuroectodermal Tumor • •

•

“Cerebral medulloblastoma” 1% of all pediatric CNS neuroepithelial tumors ➢ 6% of all pediatric PNETs Age range: 4 weeks to 10 years (mean age: 5.5 years) ➢ Males more common (2:1)

Rorke et al, WHO classification, 2000, 141-144

Lymphoma & Uncommon Neuroepithelial Tumors

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Supratentorial Primitive Neuroectodermal Tumor •

•

Nonspecific clinical features related to site ➢ Cerebrum: seizures, increased intracranial pressure, motor deficits ➢ Suprasellar region: visual or endocrine problems ➢ Not pineal: pineoblastoma Poor prognosis, especially in children < 2 years old ➢ 34% 5-year survival rate (up to 85% for cerebellar medulloblastoma)

Rorke et al, WHO classification, 2000, 141-144

Supratentorial Primitive Neuroectodermal Tumor • • • •

WHO grade IV Virtually identical to medulloblastoma Undifferentiated or poorly differentiated neuroepithelial cells Dense cellularity: “small blue cell tumor” ➢ Pleomorphism ➢ Rosette formation

Rorke et al, WHO classification, 2000, 141-144

Supratentorial Primitive Neuroectodermal Tumor •

Figure 5-2-21

[Figure 5-2-21]

Large heterogeneous mass ➢ CT: iso-to-hyperattenuated ➢ Cysts or necrosis common ➢ Calcification: 50%-70% ➢ Vasogenic edema present but not usually extensive ➢ T1WI: hypointense to gray matter ➢ T2WI: hypointense predominantly

Rorke et al, WHO classification, 2000, 141-144

Atypical Teratoid / Rhabdoid Tumor • •

•

First reported in 1978 Various names ➢ “Rhabdomyosarcomatoid variant of Wilms tumor” ➢ “Embryonal small cell tumor” ➢ “Rhabdoid tumor” Biologic behavior and some histologic features similar to malignant rhabdoid tumor of the kidney

Beckwith and Palmer, Cancer 1978;41:1937-1948; Rorke and Biegel, WHO classification 2000, 145-148; Rorke et al, J Neurosurg 1996; 85:56-65; Hanna et al, AJNR 1993; 14:107-115

Atypical Teratoid / Rhabdoid Tumor • •

• • •

2.1% of all primary CNS tumors in children 94%: < 5 years old ➢ Rarely presents in adults (4 cases) ➢ Males more common (1.4:1) Non-specific symptoms (lethargy, failure to thrive) Head tilt Cranial nerve palsy: usually VI or VII

Rorke and Biegel, WHO classification 2000, 145-148; Burger et al, Am J Surg Pathol 1998; 22:1083-1092

Supratentorial PNET with CSF dissemination Neuroradiology

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Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-22] • •

• •

Figure 5-2-22

WHO grade IV Soft lobulated mass ➢ Necrosis and hemorrhage common Rhabdoid cells ➢ Mixed with primitive neuroepithelial, epithelial, and mesenchymal elements ➢ Not a germ-cell tumor Poor prognosis: most die within one year

Rorke and Biegel, WHO classification, 2000, 145-148

Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-23] •

• • • •

Posterior fossa: most common location (52%) ➢ Especially cerebellopontine angle Supratentorial: 39% ➢ Including intraventricular Pineal: 5% Spinal: 2% Multifocal: 2%

90% have monosomy or deletion of chromosome 22

Figure 5-2-23

Rorke and Biegel, WHO classification, 2000, 145-148

Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-23] • •

Imaging: often mimics medulloblastoma CT: hyperattenuated ➢ Cysts and hemorrhage common ➢ Vasogenic edema common ➢ Variable enhancement

Hanna et al, AJNR 1993:14:109-114; Caldemyer et al, Pediatr Neurosurg 1994; 21:232-236; Rorke and Biegel, WHO classification, 2000, 145-148

Atypical Teratoid / Rhabdoid Tumor [Figure 5-2-24] • •

Large heterogeneous mass MR findings ➢ T1WI: hypo-to-isointense ➢ T2WI: variable ❖ Soft tissue: iso-to-hypointense ❖ Cyst-like: hyperintense ➢ Heterogeneous enhancement ➢ Subarachnoid seeding: 33% at presentation

Posterior fossa ATRT

Hanna et al, AJNR 1993;14:109-115; Rorke and Biegel, WHO classification, 2000, 145-148

Figure 5-2-24

Posterior fossa ATRT. Hyperattenuation on CT is similar to medulloblastoma but shows foraminal extension on MR images Lymphoma & Uncommon Neuroepithelial Tumors

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• • • • • •

Summary - Lymphoma Rapid increase in prevalence Fourth most common primary cerebral neoplasm Second most common intracranial mass in immunocompromised patient Periventricular CT: hyperattenuation T2W: hypointense

Peripheral Mass •

•

Ganglioglioma ➢ Variable size with common calcification ➢ Most common cause of chronic temporal lobe epilepsy ➢ Gangliocytoma: lacks glial component Dysembryoplastic neuroepithelial tumor (DNT) ➢ Almost always involves cortical margin ➢ Uncommon calcification ➢ Temporal lobe: 62% ➢ “Soap bubble” appearance

Cerebellar Mass •

•

“Striated” cerebellar mass ➢ Dysembryoplastic cerebellar gangliocytoma (Lhermitte-Duclos) With fat content ➢ Cerebellar liponeurocytoma

Large heterogeneous mass •

Meningocerebral interface ➢ Desmoplastic infantile ganglioglioma ❖ Large heterogeneous cerebral hemisphere mass ❖ No calcification ➢ Pleomorphic xanthoastrocytoma ❖ “Cyst-like mass with enhancing mural nodule” ❖ Calcification rare ❖ Older patients: adolescents and young adults

Large heterogeneous mass •

Young child ➢ Supratentorial PNET ❖ Large heterogeneous cerebral hemisphere mass ❖ “Cerebral medulloblastoma” ➢ Atypical teratoid / rhabdoid tumor ❖ Predilection for posterior fossa ❖ Mimics medulloblastoma ❖ Subarachnoid seeding common

References 1. 2. 3. 4. 5.

6. 7.

Akhaddar A, Zrara I, Gazzaz M, El Moustarchid B, Benomar S, Boucetta M. Cerebellar liponeurocytoma (lipomatous medulloblastoma). J Neuroradiol 2003; 30:121-126. Alkadhi H, Keller M, Brandner S, Yonekawa Y, Kollias SS. Neuroimaging of cerebellar liponeurocytoma. Case report. J Neurosurg 2001; 95:324-331. Athale S, Hallet KK, Jinkins JR. Ganglioglioma of the trigeminal nerve: MRI. Neuroradiology 1999; 41:576-578. Beckwith JB, Palmer NF. Histopathology and prognosis of Wilms Tumour: Results from the first National Wilms Tumor Study. Cancer 1978; 41:1937-1948. Burger PC, Yu IT, Tihan T, et al. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 1998; 22:1083-1092. Cacciola F, Conti R, Taddei GL, Buccoliero AM, Di Lorenzo N (2002) Cerebellar liponeurocytoma. Case report with considerations on prognosis and management. Acta Neurochir (Wien) 144: 829-833 Caldemeyer KS, Smith RR, Azzarelli B, Boaz JC. Primary central nervous system malignant rhabdoid tumor: CT and MR appearance simulates a primitive neuroectodermal tumor. Pediatr Neurosurg 1994; 21:232-236.

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8. 9.

10.

11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23.

24. 25. 26. 27.

28. 29. 30. 31. 32. 33. 34.

35. 36.

Castillo M, Davis PC, Takei Y, Hoffman JC, Jr. Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients. AJNR Am J Neuroradiol 1990; 11:109-114. Daumas-Duport C, Pietsch T, Lantos PL. Dysembryoplastic neuroepithelial tumour. In: Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and genetics of tumours of the nervous system. Lyon, France: IARC, 2000; 103-106. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER, Jr., Vedrenne C. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery 1988; 23:545-556. Dina TS. Primary central nervous system lymphoma versus toxoplasmosis in AIDS. Radiology 1991; 179:823-828. Dorne HL, O'Gorman AM, Melanson D. Computed tomography of intracranial gangliogliomas. AJNR Am J Neuroradiol 1986; 7:281-285. Duffner PK, Burger PC, Cohen ME, et al. Desmoplastic infantile gangliogliomas: an approach to therapy. Neurosurgery 1994; 34:583-589; discussion 589. Giannini C, Scheithauer BW, Burger PC, et al. Pleomorphic xanthoastrocytoma: what do we really know about it? Cancer 1999; 85:2033-2045. Hammond RR, Duggal N, Woulfe JM, Girvin JP. Malignant transformation of a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 2000; 92:722-725. Hanna SL, Langston JW, Parham DM, Douglass EC. Primary malignant rhabdoid tumor of the brain: clinical, imaging, and pathologic findings. AJNR Am J Neuroradiol 1993; 14:107-115. Henry JM, Heffner RR, Jr., Dillard SH, Earle KM, Davis RL. Primary malignant lymphomas of the central nervous system. Cancer 1974; 34:1293-1302. Hoffman JM, Waskin HA, Schifter T, et al. FDG-PET in differentiating lymphoma from nonmalignant central nervous system lesions in patients with AIDS. J Nucl Med 1993; 34:567-575. Jiddane M, Nicoli F, Diaz P, et al. Intracranial malignant lymphoma. Report of 30 cases and review of the literature. J Neurosurg 1986; 65:592-599. Johnson JH, Jr., Hariharan S, Berman J, et al. Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg 1997; 27:203-207. Kalyan-Raman UP, Olivero WC. Ganglioglioma: a correlative clinicopathological and radiological study of ten surgically treated cases with follow-up. Neurosurgery 1987; 20:428-433. Kepes JJ, Rubinstein LJ, Eng LF. Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases. Cancer 1979; 44:1839-1852. Kleihues P, Chimelli L, Giangaspero F. Cerebellar liponeurocytoma. In: Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and genetics of tumours of the nervous system. Lyon, France: IARC, 2000; 110-111. Koeller KK, Dillon WP. Dysembryoplastic neuroepithelial tumors: MR appearance. AJNR Am J Neuroradiol 1992; 13:1319-1325. Koeller KK, Smirniotopoulos JG, Jones RV. Primary central nervous system lymphoma: radiologic-pathologic correlation. Radiographics 1997; 17:1497-1526. Kulkantrakorn K, Awwad EE, Levy B, et al. MRI in Lhermitte-Duclos disease. Neurology 1997; 48:725-731. Kumabe T, Shimizu H, Sonoda Y, Shirane R. Thallium-201 single-photon emission computed tomographic and proton magnetic resonance spectroscopic characteristics of intracranial ganglioglioma: three technical case reports. Neurosurgery 1999; 45:183-187; discussion 187. Kuroiwa T, Kishikawa T, Kato A, Ueno M, Kudo S, Tabuchi K. Dysembryoplastic neuroepithelial tumors: MR findings. J Comput Assist Tomogr 1994; 18:352-356. Lee YY, Bruner JM, Van Tassel P, Libshitz HI. Primary central nervous system lymphoma: CT and pathologic correlation. AJR Am J Roentgenol 1986; 147:747-752. Lhermitte J,Duclos P. Sur un ganglioneurome diffus du cortex du cervelet. Bull Assoc Fr Etude Cancer 1920;9:99107. Lipper MH, Eberhard DA, Phillips CD, Vezina LG, Cail WS. Pleomorphic xanthoastrocytoma, a distinctive astroglial tumor: neuroradiologic and pathologic features. AJNR Am J Neuroradiol 1993; 14:1397-1404. Martin DS, Levy B, Awwad EE, Pittman T. Desmoplastic infantile ganglioglioma: CT and MR features. AJNR Am J Neuroradiol 1991; 12:1195-1197. Meltzer CC, Smirniotopoulos JG, Jones RV. The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). Radiology 1995; 194:699-703. Miller DC, Hochberg FH, Harris NL, Gruber ML, Louis DN, Cohen H. Pathology with clinical correlations of primary central nervous system non-Hodgkin's lymphoma. The Massachusetts General Hospital experience 19581989. Cancer 1994; 74:1383-1397. Montagna N, Moreira D, Vaz LC, Reis M. Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity. Arq Neuropsiquiatr. 2002 Sep; 60(3-B):725-9. Nelen MR, Padberg GW, Peeters EA, et al. Localization of the gene for Cowden disease to chromosome 10q22-23. Nat Genet 1996;13:114-116

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37. Ostertun B, Wolf HK, Campos MG, et al. Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR Am J Neuroradiol 1996; 17:419-430. 38. Pahapill PA, Ramsay DA, Del Maestro RF. Pleomorphic xanthoastrocytoma: case report and analysis of the literature concerning the efficacy of resection and the significance of necrosis. Neurosurgery 1996; 38:822-828; discussion 828-829. 39. Provenzale JM, Arata MA, Turkington TG, McLendon RE, Coleman RE. Gangliogliomas: characterization by registered positron emission tomography-MR images. AJR Am J Roentgenol 1999; 172:1103-1107. 40. Robinson S, Cohen AR. Cowden disease and Lhermitte-Duclos disease: characterization of a new phakomatosis. Neurosurgery 2000; 46:371-383. 41. Rorke LB, Biegel JA. Atypical teratoid/ rhabdoid tumor. In Kleihues P and Cavenee WK, In: Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and Genetics, Tumours of the Nervous System. IARC Press Lyon, 2000, page 145-148. 42. Rorke LB, Hart MN, McLendon RE: Supratentorial primitive neuroectodermal tumour (PNET). In: Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of the Nervous System. Edited by: Kleihues P, Cavenee WK. Lyon: IARC Press; 2000:141-144. 43. Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 1996; 85:56-65. 44. Rosenblum ML, Levy RM, Bredesen DE, So YT, Wara W, Ziegler JL. Primary central nervous system lymphomas in patients with AIDS. Ann Neurol 1988; 23 Suppl:S13-16. 45. Schwaighofer BW, Hesselink JR, Press GA, Wolf RL, Healy ME, Berthoty DP. Primary intracranial CNS lymphoma: MR manifestations. AJNR Am J Neuroradiol 1989; 10:725-729. 46. Silver JM, Rawlings CE, 3rd, Rossitch E, Jr., Zeidman SM, Friedman AH. Ganglioglioma: a clinical study with long-term follow-up. Surg Neurol 1991; 35:261-266. 47. Tampieri D, Moumdjian R, Melanson D, Ethier R. Intracerebral gangliogliomas in patients with partial complex seizures: CT and MR imaging findings. AJNR Am J Neuroradiol 1991; 12:749-755. 48. Taratuto AL, Monges J, Lylyk P, Leiguarda R. Superficial cerebral astrocytoma attached to dura. Report of six cases in infants. Cancer 1984; 54:2505-2512. 49. Tenreiro-Picon OR, Kamath SV, Knorr JR, Ragland RL, Smith TW, Lau KY. Desmoplastic infantile ganglioglioma: CT and MRI features. Pediatr Radiol 1995; 25:540-543. 50. Tien RD, Cardenas CA, Rajagopalan S.Pleomorphic xanthoastrocytoma of the brain: MR findings in six patients. AJR Am J Roentgenol. 1992 Dec;159(6):1287-90. 51. Tien RD, Tuori SL, Pulkingham N, Burger PC.Ganglioglioma with leptomeningeal and subarachnoid spread: results of CT, MR, and PET imaging. AJR Am J Roentgenol. 1992 Aug;159(2):391-3. 52. VandenBerg SR, May EE, Rubinstein LJ, et al. Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential ("desmoplastic infantile gangliogliomas"). Report on 11 cases of a distinctive embryonal tumor with favorable prognosis. J Neurosurg 1987; 66:58-71. 53. Vaquero J, Martinez R, Rossi E, Lopez R. Primary cerebral lymphoma: the "ghost tumor". Case report. J Neurosurg 1984; 60:174-176. 54. Villringer K, Jager H, Dichgans M, et al. Differential diagnosis of CNS lesions in AIDS patients by FDG-PET. J Comput Assist Tomogr 1995; 19:532-536. 55. Wiestler OD, Padberg GW, Steck PA. Cowden disease and dysplastic gangliocytoma of the cerebellum/LhermitteDuclos disease. In: Kleihues P, Cavenee WK, eds. World Health Organization Classification of Tumours: Pathology and genetics of tumours of the nervous system. Lyon, France: IARC, 2000; 235-237. 56. Zentner J, Wolf HK, Ostertun B, et al. Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry. 1994;57:1497-1502.

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Cerebral Intraventricular Neoplasms Kelly K. Koeller, MD, FACR Ten Most Common Intraventricular Tumors* • • • •

• •

Ependymoma (18%) Subependymoma (11%) Central Neurocytoma (10%) Subependymal Giant Cell Astrocytoma (6%) Other Astrocytomas (9%) Colloid Cyst (4%)

• • • •

Choroid Plexus Papilloma (24%) Choroid Plexus Carcinoma (2%) Meningioma (6%) Metastasis (2%)

*Based on 397 cases in AFIP Archives

Ependymoma •

• •

Arise from ependymal cells of ventricular wall ➢ Also central canal of spinal cord 3%-9% of all neuroepithelial tumors ➢ 6%-12% of pediatric brain tumors ➢ 30% of all brain tumors in children < 3 years of age No gender predilection

3rd WHO classification, 2000

Ependymoma [Figure 5-3-1] •

•

Broad age range ➢ Posterior fossa: 6 years old (mean) ➢ Supratentorial: 18-24 years old (mean) Fourth ventricle: 58% ➢ Lateral & third ventricle: 42% ➢ Supratentorial: more commonly extraventricular ➢ Rare: ovaries, soft tissues, mediastinum, sacrococcygeal region

JCAT 1995; 19:518-526; Childs Nerv Syst 1991; 7:177-182; J Neurosurg 1979; 51:383-391

Figure 5-3-1

Ependymoma • • •

• •

Increased intracranial pressure and hydrocephalus Adults: 5-year survival rate = 57%, 10-year = 45% Less favorable prognosis ➢ Children: especially those under 2 years of age ➢ Fourth ventricle location Recurrence common Gross total resection “curative” ➢ Radiation therapy in partial resection cases

3rd WHO classification, 2000; Childs Nerv Syst 1990; 6:375-378; Neurosurgery 1995; 37:655-667; Neurosurgery 1993; 32:169-175

Ependymoma • • •

Well-circumscribed mass May extend into brain Fourth ventricle: foraminal extension common

3rd WHO classification, 2000

Ependymoma extending into cerebellopontine angle from fourth ventricle Cerebral Intraventricular Neoplasms

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Neuroradiology

Ependymoma •

•

Figure 5-3-2

WHO grade II ➢ Perivascular pseudorosettes ➢ Rare mitotic figures Variants ➢ Cellular ➢ Papillary ➢ Clear cell ➢ Tanycytic ➢ Anaplastic

3rd WHO classification, 2000

Ependymoma •

•

[Figures 5-3-2 and 5-3-3]

CT: iso- to hyperattenuated ➢ Calcification: 40%-80% ➢ Enhancing soft tissue component ➢ Non-enhancing cyst-like portion MR: heterogeneous ➢ Isointense to gray matter on T1WI ➢ Hyperintense to gray matter on T2WI ➢ Post-op residual: decreased survival

Ependymoma with calcification on non-contrast CT

Radiology 1982; 143:97-101; Br J Radiol 1994; 67:223-243; JCAT 1995; 19:518-526; Neurosurgery 1991; 28:666-672

Figure 5-3-3

Subependymoma • • • •

•

First described in 1945 Arise from subependymal glial layer Incidence: 0.4 (asymptomatic) - 0.7% (symptomatic) Most smaller than 2 cm diameter ➢ Symptomatic: 3-5 cm Hydrocephalus (80%), focal neurologic deficit (27%), seizures (9%), subarachnoid hemorrhage (4.5%)

J Neurosurg 1945; 2:232-240; Acta Neurochir 1989; 96:15-25; AJR 1995; 165:1245-1250; Neurosurgery 1986; 19:594-598

Ependymoma with foraminal extension on MR

Subependymoma • • •

•

Males more common Older than 15 years of age: 82% Fourth ventricle: > 50% ➢ Lateral ventricle: ~ 45% Well-circumscribed avascular mass ➢ Pedicular attachment to ventricular wall

Neurosurgery 1986; 19:594-598; J Neurosurg 1978: 49:689-696

Subependymoma • •

• •

Expansive but not infiltrative WHO grade I ➢ Dense fibrillary matrix ➢ Cysts and nests ➢ Low mitotic activity Low recurrence rate 10% mixed with ependymoma or other tumor

Neurosurgery 1986; 19:594-598; 3rd WHO classification, 2000 Neuroradiology

1059

Cerebral Intraventricular Neoplasms

Subependymoma •

•

Figure 5-3-4

[Figures 5-3-4 and 5-3-5]

CT: lobulated mass ➢ Iso-to-slight hypoattenuated ➢ Calcification: 32% ➢ Cystic degeneration: 18% ➢ At least some enhancement: 84% MR: hypointense compared to white matter on T1WI ➢ Hyperintense on T2WI ➢ Variable enhancement

Neurosurgery 1986; 19:594-598; AJR 1995; 165:1245-1250; AJNR 1995; 16:2121-2129; AJNR 1990; 11:83-91; Surg Neurol 1990; 33:329-335

Ependymoma

vs.

Subependymoma

Iso- to hyperattenuated

Iso-to-hypoattenuated

Calcification, cysts more common

Calcification, cysts less common

ntense enhancement more common

More variable enhancement

Extraventricular extension

Rarely extends beyond ventricular margin

Subependymoma with calcification in right frontal horn on CT

Figure 5-3-5

Neurosurgery 1986; 19:594-598

Central Neurocytoma •

• • •

First described in 1982 ➢ Confusion with intraventricular oligodendroglioma “Central”: lateral and third ventricles ➢ “Extraventricular central neurocytoma” for those located elsewhere (brain, cerebellum, spinal cord) 0.25%-0.5% of all intracranial tumors No gender predilection

Acta Neuropathol 1982;56:151-156; 3rd WHO classification, 2000; Brain Pathol 1993; 3:297-306

Central Neurocytoma •

•

Broad age range: 8 days to 67 years ➢ Mean age: 29 years ➢ 50%: 20-30 years of age ➢ 75%: 20-40 years of age Short clinical course (mean: 3 months) ➢ Increased intracranial pressure, mental status changes, visual deficits

3rd WHO classification, 2000; Brain Pathol 1993; 3:297-306

Central Neurocytoma • •

Arise from septum pellucidum or ventricular wall Lateral ventricle near foramen of Monro: 50% ➢ Lateral and third ventricles: 15% ➢ Bilateral: 13% ➢ Third ventricle alone: 3%

Surg Neurol 1998: 49:197-204; 3rd WHO classification, 2000

Cerebral Intraventricular Neoplasms

1060

Subependymoma of right lateral ventricle on axial T1-weighted pre-contrast and post-contrast images Neuroradiology

Central Neurocytoma [Figure 5-3-6] • •

•

Figure 5-3-6

WHO grade II Striking resemblance to oligodendroglioma ➢ “Fried egg appearance” ➢ Pineocytomatous rosettes ➢ Calcification: 50% Neuronal differentiation ➢ ? Glial differentiation

3rd WHO classification, 2000

Central Neurocytoma [Figure 5-3-7] • • •

• •

Well-circumscribed lobulated mass Broad attachment to septum pellucidum or ventricular wall CT: hyperattenuated compared to brain ➢ Cyst-like areas: 66% ➢ Calcification: 50% MR: hyperintense compared to white matter on T1WI and T2WI Hemorrhage rare

Central neurocytoma with characteristic "fried egg" histologic appearance

Neuroradiology 1991; 33:143-148; JCAT 1989: 13:495-497

Figure 5-3-7

Central neurocytoma with bilateral involvement on CT and MR in a 24-year-old woman with headaches and no neurological deficit

Subependymal Giant Cell Astrocytoma •

• • •

Most common brain neoplasm in tuberous sclerosis (6%-16%) ➢ Neurocutaneous phakomatosis ➢ Autosomal dominant in 20%-50% of cases ➢ Tubers and subependymal nodules: 90%-100% Can it occur without TS? - controversial 1.4% of all pediatric brain tumors Most: first or second decades (mean: 11 years)

Figure 5-3-8

Neurosurgery 1991;28:864-868; 3rd WHO classification; Pediatr Radiol 1992;22:485-489; Pediatr Neurosurg 1994: 20:233-239

Subependymal Giant Cell Astrocytoma [Figure 5-3-8] • • • • •

Virtually always located near the foramen of Monro Hydrocephalus, seizures Higher incidence of cardiac rhabdomyomas Surgical resection for symptomatic SEGA or with documented growth Non-responsive to radiation therapy

3rd WHO classification, 2000; Pediatr Radiol 1992; 22:485-489 Subependymal giant cell astrocytoma with characteristic location near foramen of Monro Neuroradiology

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Cerebral Intraventricular Neoplasms

Subependymal Giant Cell Astrocytoma •

• •

WHO grade I ➢ Slow growth with benign biologic behavior and low recurrence rate ➢ Earlier diagnosis associated with longer survival Probably arise from subependymal nodules Mixed glioneuronal pattern

Figure 5-3-9

Pediatr Neurosurg 1994: 20:233-239

Subependymal Giant Cell Astrocytoma [Figure 5-3-9] •

CT: iso- to slightly hypoattenuated ➢ Calcification common

Subependymal Giant Cell Astrocytoma •

• •

MR: hypointense compared to white matter on T1WI, heterogeneously hyperintense on T2WI ➢ Neonates: hyperintense on T1WI, hypointense on T2WI Intense enhancement on CT and MR ➢ Distinguishes SEGA from most subependymal nodules Annual surveillance screening post-op and in first-degree relatives

Pediatr Neurosurg 1994; 20:233-239; AJNR 1999: 20: 907-916; Pediatr Radiol 1991; 21:432

Subependymal Giant Cell Astrocytoma •

Other TS manifestations ➢ Cortical tubers ➢ Adenoma sebaceum ➢ Shagreen patch ➢ Retinal hamartoma ➢ Renal angiomyolipoma ➢ Cardiac rhabdomyoma ➢ Rectal polyps

Subependymal giant cell astrocytoma with calcification on CT

Figure 5-3-10

Colloid Cyst • • • • •

Most common neuroepithelial cyst: probably arises from endoderm Young to middle-aged adults Positional headache: acute CSF obstruction Antero-superior third ventricle Variable composition: mucoid material with old blood, cholesterol crystals, serous fluid, paramagnetic ions

Lach et al, J Neurosurg 1993; 78:101-111; Shaungshotti et al, Arch Pathol Lab Med 1965; 80:214224

Colloid Cyst [Figure 5-3-10] •

• •

Variable appearance ➢ Well-circumscribed mass ➢ Hyperattenuated on CT ➢ Hypo-to-hyperintense on T1WI ➢ Hypo-to-hyperintense on T2WI May ring enhance Solid enhancement: not colloid cyst

Waggenspack and Guiunto, AJNR 1989;10:105-110; Lach et al, J Neurosurg 1993; 78:101-111

Cerebral Intraventricular Neoplasms

1062

Colloid cyst on non-contrast axial CT, axial T2weighted, sagittal T1-weighted, contrast-enhanced coronal T1-weighted images

Neuroradiology

Choroid Plexus •

Neuroepithelial tissue ➢ Cerebrospinal fluid (CSF) production: 450 ml/day (avg.) ➢ Atrium of the lateral ventricle ➢ Foramen of Monro and third ventricle ➢ Fourth ventricle and foramen of Luschka ➢ Absent in cerebral aqueduct

3rd WHO classification, 2000

Choroid Plexus Tumors •

• • • •

Lateral ventricle (50%), fourth ventricle (40%), third ventricle (5%) ➢ Multiple: 5% Lateral ventricle: no gender predilection Fourth ventricle: males more common 0.4%-0.6% of all intracranial tumors ➢ 2%-4% of pediatric brain tumors ➢ 10%-20% of brain tumors in less than 1 year of age ➢ 50% manifest in first decade Incidence: 0.3 per million

Figure 5-3-11

J Neurosurg 1988: 69: 843-849

Choroid Plexus Tumors •

•

Hydrocephalus ➢ Increased CSF production ➢ CSF flow obstruction ➢ Hemorrhage: CSF absorption Association with Li-Fraumeni and Aicardi syndromes

Neurosurgery 1989; 25:327-335; J Neurosurg 1952; 9:59-67; J Neurosurg 1998; 88:521-528; Radiology 1989; 173:81-88

Choroid Plexus Tumors •

•

Choroid plexus papilloma with Pedicular attachment common histology similar to that of normal ➢ Lateral ventricle: trigone choroid plexus tissue ➢ Third ventricle: roof ➢ Fourth ventricle: posterior medullary velum ➢ “Bobble-head doll” syndrome: intermittent ventricular obstruction CSF Seeding: occurs in both papillomas and carcinomas

3rd WHO classification, 2000

Papilloma •

vs.

Choroid plexus papilloma • ➢ 80% ➢ WHO grade I ➢ 100% 5-year survival ➢ Children and adults ➢ Lateral ventricle: 1st decade ➢ Fourth ventricle: first 5 decades

Carcinoma Choroid plexus carcinoma ➢ 20% ➢ WHO grade III ➢ 26-50% 5-year survival ➢ Much more common in children ➢ Post-op residual disease: very poor prognostic factor ➢ Necrosis, parenchymal invasion

3rd WHO classification, 2000

Choroid Plexus Papilloma [Figure 5-3-11] •

•

Histology resembles normal choroid plexus ➢ Prominent fronds of fibrovascular connective tissue WHO grade I

Neuroradiology

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Cerebral Intraventricular Neoplasms

Choroid Plexus Carcinoma [Figure 5-3-12] • • • • • •

Figure 5-3-12

Hypercellularity Nuclear pleomorphism High nucleus-cytoplasm ratio Mitotic activity Brain parenchyma invasion WHO grade III

3rd WHO classification, 2000

Choroid Plexus Papilloma •

•

Well-circumscribed cauliflower-like mass ➢ Prominent lobulated margin CT: iso-to-hyperattenuated ➢ Calcification: 24% ➢ Lateral ventricle: more common on left side? - Probably not ➢ Foraminal extension characteristic

Choroid plexus carcinoma with markedly heterogeneous histologic appearance

3rd WHO classification, 2000; J Neurosurg 1998; 88:521-528; Radiology 1989; 173:81-88

Choroid Plexus Papilloma [Figure 5-3-13] • •

•

MR: iso-to-hypointense compared to normal brain parenchyma on T1WI Flow voids common ➢ Enlarged choroidal artery ➢ Amenable to pre-operative embolization US: lobulated echogenic mass ➢ Bi-directional flow throughout diastole

3rd WHO classification, 2000; Radiology 1989: 173:81-88

Figure 5-3-13 Choroid Plexus Carcinoma [Figure 5-3-14] • • • •

More heterogeneous Extraventricular extension Vasogenic edema Slightly less hydrocephalus?

3rd WHO classification, 2000; J Neurosurg 1998; 88:521-528; Radiology 1989; 173:81-88

Choroid plexus papilloma of left lateral ventricle atrium on MR

Figure 5-3-14

Choroid plexus carcinoma with extension into adjacent brain parenchyma and spread within ventricles Cerebral Intraventricular Neoplasms

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Neuroradiology

Intraventricular Meningioma •

• • • •

Most common atrial mass in adults ➢ Usually older than 30 years of age ➢ Mean: 42 years Lateral ventricle >> third or fourth ventricle Arise from arachnoidal cap cells within choroid plexus, tela choroidea, or velum interpositum 0.7% of all meningiomas Almost all are benign ➢ Predilection for sarcomatous change in children

Figure 5-3-15

Neurosurgery 1987; 20:465-468; AJNR 1995; 16:1378-1381; Radiology 1984; 153:435-442

Intraventricular Meningioma •

CT: well-defined globular mass ➢ Hyperattenuated compared to brain ➢ Calcification: 50%

Neurosurgery 1987: 20:465-468; AJNR 1995; 16:1378-1381; Surg Neurol 1994: 42:41-45

Intraventricular Meningioma [Figure 5-3-15] •

• •

MR: iso-to-hypointense compared to gray matter on T1WI ➢ Iso-to-hyperintense on T2WI Heterogeneous enhancement MRS: decreased NAA, creatine ➢ Increased choline

Intraventricular meningioma on contrast-enhanced axial T1weighted MR image

AJNR 1999; 20:882-885; AJNR 1994; 15:435-444

Choroid Plexus Metastasis • •

• •

Rare: 0.9-4.6% of all cerebral metastasis Renal cell carcinoma and lung carcinoma: most common in adults ➢ Children: neuroblastoma, Wilms’ tumor, retinoblastoma ➢ Others: melanoma, gastric carcinoma, colon carcinoma, lymphoma Lateral ventricle: most common Renal cell carcinoma metastasis may mimic meningioma

Figure 5-3-16

South Med J 1998; 91:1159-1162; Neurosurgery 1983; 13:430-434

Choroid Plexus Metastasis • • •

[Figure 5-3-16]

CT: iso- or hyperattenuated MR: hypointense on T1WI, hyperintense on T2WI Intense enhancement usually

Br J Radiol 1994; 67:223-243

Summary •

• •

Fourth Ventricle ➢ Ependymoma ➢ Subependymoma ➢ Choroid Plexus Papilloma Third ventricle ➢ Colloid Cyst ➢ All the others: less common Lateral Ventricle (anterior half) ➢ Subependymoma ➢ Central Neurocytoma ➢ Subependymal Giant Cell Astrocytoma ➢ Ependymoma ➢ Astrocytoma

Neuroradiology

Choroid plexus metastasis from renal cell carcinoma

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•

Lateral Ventricle (posterior half) ➢ Choroid Plexus Papilloma / Carcinoma ➢ Meningioma ➢ Metastasis

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20.

21.

22. 23. 24. 25. 26.

Baron Y, Barkovich AJ. MR imaging of tuberous sclerosis in neonates and young infants. AJNR Am J Neuroradiol 1999; 20:907-916. Bolen JW, Jr., Lipper MH, Caccamo D. Intraventricular central neurocytoma: CT and MR findings. J Comput Assist Tomogr 1989; 13:495-497. Chiechi MV, Smirniotopoulos JG, Jones RV. Intracranial subependymomas: CT and MR imaging features in 24 cases. AJR Am J Roentgenol 1995; 165:1245-1250. Coates TL, Hinshaw DB, Jr., Peckman N, et al. Pediatric choroid plexus neoplasms: MR, CT, and pathologic correlation. Radiology 1989; 173:81-88. Darling CF, Byrd SE, Reyes-Mugica M, et al. MR of pediatric intracranial meningiomas. AJNR Am J Neuroradiol 1994; 15:435-444. Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery 1989; 25:327335. Furie DM, Provenzale JM. Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr 1995; 19:518-526. Hassoun J, Gambarelli D, Grisoli F, et al. Central neurocytoma. An electron-microscopic study of two cases. Acta Neuropathol (Berl) 1982; 56:151-156. Hassoun J, Soylemezoglu F, Gambarelli D, Figarella-Branger D, von Ammon K, Kleihues P. Central neurocytoma: a synopsis of clinical and histological features. Brain Pathol 1993; 3:297-306. Healey EA, Barnes PD, Kupsky WJ, et al. The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 1991; 28:666-671; discussion 671-672. Hoeffel C, Boukobza M, Polivka M, et al. MR manifestations of subependymomas. AJNR Am J Neuroradiol 1995; 16:2121-2129. Kahn EA, Luros JT. Hydrocephalus from overproduction of cerebrospinal fluid, and experiences with other parillomas of the choroid plexus. J Neurosurg 1952; 9:59-67. Killebrew K, Krigman M, Mahaley MS, Jr., Scatliff JH. Metastatic renal cell carcinoma mimicking a meningioma. Neurosurgery 1983; 13:430-434. Kleihues P, Cavenee WK eds. World Health organization Classification of Tumours, Pathology & Genetics: Tumours of the Nervous System. IARC, Lyon, France, 2000. Kudo H, Oi S, Tamaki N, Nishida Y, Matsumoto S. Ependymoma diagnosed in the first year of life in Japan in collaboration with the International Society for Pediatric Neurosurgery. Childs Nerv Syst 1990; 6:375-378. Lach B, Scheithauer BW, Gregor A, Wick MR. Colloid cyst of the third ventricle. A comparative immunohistochemical study of neuraxis cysts and choroid plexus epithelium. J Neurosurg 1993; 78:101-111. Lang I, Jackson A, Strang FA. Intraventricular hemorrhage caused by intraventricular meningioma: CT appearance. AJNR Am J Neuroradiol 1995; 16:1378-1381. Lobato RD, Sarabia M, Castro S, et al. Symptomatic subependymoma: report of four new cases studied with computed tomography and review of the literature. Neurosurgery 1986; 19:594-598. Majos C, Cucurella G, Aguilera C, Coll S, Pons LC. Intraventricular meningiomas: MR imaging and MR spectroscopic findings in two cases. AJNR Am J Neuroradiol 1999; 20:882-885. Matsumura A, Ahyai A, Hori A, Schaake T. Intracerebral subependymomas: clinical and neuropathological analyses with special reference to the possible existence of a less benign variant. Acta Neurochir Wien 1989; 96:15–25. McConachie NS, Worthington BS, Cornford EJ, Balsitis M, Kerslake RW, Jaspan T. Review article: computed tomography and magnetic resonance in the diagnosis of intraventricular cerebral masses. Br J Radiol 1994; 67:223-243. McGirr SJ, Ebersold MJ, Scheithauer BW, Quast LM, Shaw EG. Choroid plexus papillomas: long-term follow-up results in a surgically treated series. J Neurosurg 1988; 69:843-849. Menor F, Marti-Bonmati L, Mulas F, Poyatos C, Cortina H. Neuroimaging in tuberous sclerosis: a clinicoradiological evaluation in pediatric patients. Pediatr Radiol 1992; 22:485-489. Morantz RA, Kepes JJ, Batnitzky S, Masterson BJ. Extraspinal ependymomas. Report of three cases. J Neurosurg 1979; 51:383-391. Morrison G, Sobel DF, Kelley WM, Norman D. Intraventricular mass lesions. Radiology 1984; 153:435-442. Palma L, Celli P, Cantore G. Supratentorial ependymomas of the first two decades of life. Long-term follow-up of 20 cases (including two subependymomas). Neurosurgery 1993; 32:169-175.

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27. Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, et al. Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 1998; 88:521-528. 28. Pollack IF, Gerszten PC, Martinez AJ, et al. Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 1995; 37:655-666; discussion 666-657. 29. Raila FA, Bottoms WT, Jr., Fratkin JD. Solitary choroid plexus metastasis from a renal cell carcinoma. South Med J 1998; 91:1159-1162. 30. Rieger E, Binder B, Starz I, Oberbauer R, Ebner F, Urban C. Tuberous sclerosis complex: oligosymptomatic variant associated with subependymal giant-cell astrocytoma. Pediatr Radiol 1991; 21:432. 31. Scheinker IM: Subependymoma: A newly recognized tumor of subependymal derivation. J Neurosurg 1945, 2: 232-240 32. Scheithauer BW. Symptomatic subependymoma. Report of 21 cases with review of the literature. J Neurosurg 1978; 49:689-696. 33. Schiffer D, Chio A, Giordana MT, et al. Histologic prognostic factors in ependymoma. Childs Nerv Syst 1991; 7:177-182. 34. Sgouros S, Carey M, Aluwihare N, Barber P, Jackowski A. Central neurocytoma: a correlative clinicopathologic and radiologic analysis. Surg Neurol 1998; 49:197-204. 35. Sgouros S, Walsh AR, Barber P. Intraventricular malignant meningioma in a 6-year-old child. Surg Neurol 1994; 42:41-45. 36. Shepherd CW, Scheithauer BW, Gomez MR, Altermatt HJ, Katzmann JA. Subependymal giant cell astrocytoma: a clinical, pathological, and flow cytometric study. Neurosurgery 1991; 28:864-868. 37. Shuangshoti S, Roberts MP, Netsky MG. Neuroepithelial (colloid) cyst: pathogenesis and relation to choroid plexus and ependyma. Arch Pathol Lab Med 1965; 80:214-224, 38. Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Schut L. Subependymal giant cell astrocytomas in children. Pediatr Neurosurg 1994; 20:233-239. 39. Spoto GP, Press GA, Hesselink JR, Solomon M. Intracranial ependymoma and subependymoma: MR manifestations. AJNR Am J Neuroradiol 1990; 11:83-91. 40. Strenger SW, Huang YP, Sachdev VP. Malignant meningioma within the third ventricle: a case report. Neurosurgery 1987; 20:465-468. 41. Swartz JD, Zimmerman RA, Bilaniuk LT. Computed tomography of intracranial ependymomas. Radiology 1982; 143:97-101. 42. Waggenspack GA,Guinto FC Jr. MR and CT of masses of the anterosuperior third ventricle. AJNR, 1989; 10:105110 43. Wichmann W, Schubiger O, von Deimling A, Schenker C, Valavanis A. Neuroradiology of central neurocytoma. Neuroradiology 1991; 33:143-148. 44. Yamasaki T, Kikuchi H, Higashi T, Yamabe H, Moritake K. Two surgically cured cases of subependymoma with emphasis on magnetic resonance imaging. Surg Neurol 1990; 33:329-335.

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Imaging of the Temporal Bone: Anatomy and Congenital Lesions Kelly K. Koeller, MD, FACR Middle Ear •

•

Epitympanum ➢ Malleus head ➢ Short process of incus Mesotympanum ➢ Muscles: tensor tympani (V3), stapedius (VII) ➢ Ossicles: rest of malleus and incus, stapes ➢ Ligaments ➢ Nerves: chorda tympani (VII), Jacobson’s nerve (IX)

Inner Ear: Cochlea • • • • • •

Anterior to vestibule Promontory: bony ridge Modiolus: central axis, cochlear nerve Apex (cupola) Basal turn Cochlear aqueduct: perilymphatic duct

Inner Ear: Vestibule • • • •

Posterior to cochlea Oval window niche: partition from middle ear Lamina cribrosa: partition from internal auditory canal (IAC) Vestibular aqueduct: endolymphatic duct

Inner Ear: Semicircular Canals (SCC) [Figures 5-4-1 to 5-4-15] • • • • •

Lateral (horizontal) Superior: arcuate eminence Posterior Oriented ≈ 90° to each other Rotational acceleration

Figure 5-4-1

Axial CT at superior portion of temporal bone shows lumen of superior semicircular canal and upper portion of mastoid antrum and epitympanum

Figure 5-4-2

Subarcuate artery canal traversing through “hoop” of superior semicircular canal

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Figure 5-4-3 Superior portion of vestibule with malleus (anterior) and incus (posterior) within the epitympanum

Figure 5-4-4 Superior portion of internal auditory canal with labyrinthine canal containing the facial nerve on its way to the geniculate ganglion and tympanic facial segment along medial wall of middle ear

Figure 5-4-5 Superior portion of cochlea and internal auditory canal. Note vestibular aqueduct arising from posterior margin of temporal bone

Mid-portion of internal auditory canal and cochlea. Sinus tympani is located just lateral to vestibule. Small bony peak lateral to vestibule is pyramidal eminence. Facial nerve canal is located posterolateral to pyramidal eminence. Middle ear shows “parallel lines sign”: tensor tympani tendon anteriorly and incudostapedial junction with stapedial struts posteriorly. Struts mark the site of the oval window

Figure 5-4-6

Figure 5-4-7 Inferior portion of cochlea. Bony plate separating it from the middle ear is the cochlear promontory. Note jugular bulb in posterior temporal bone

Figure 5-4-8

Neuroradiology

Basilar turn of cochlea. Internal carotid artery is seen anterior to the cochlea and is delimited from the middle ear by a bony plate. Eustachian tube arises just lateral to the artery and heads along an anteromedial pathway towards the nasopharynx

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Coronal view shows internal carotid artery inferiorly separated from middle ear by bony plate. Cochlea is located immediately superior. Geniculate ganglion is located just superolateral to cochlea. Middle ear contains ossicles (malleus anterior to incus) within epitympanum. Tegmen tympani is bony plate separating middle ear from brain

Figure 5-4-9

Figure 5-4-10 Moving posteriorly, facial nerve segments are seen above and lateral to cochlea

Figure 5-4-11 Cochlea is separated by cochlear promontory from middle ear. Anterior portion of internal auditory canal is just coming into view. Scutum is well seen along superior margin of medial external auditory canal

Figure 5-4-12

Mid-portion of internal auditory canal with crista falciformis along its lateral margin. Vestibule now appears with lateral and superior semicircular canals. Facial nerve is located immediately inferior to lateral semicircular canal and above oval window

Figure 5-4-13 Posterior margin of vestibule with facial nerve as small soft tissue just prior to reaching posterior genu

Figure 5-4-14

Facial nerve at posterior genu

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Figure 5-4-15 Mastoid segment of facial nerve extending inferiorly to the stylomastoid foramen

Congenital Anomalies • • • •

• •

Outer ear Middle ear Inner ear Vascular ➢ Internal carotid artery ➢ Jugular vein Cholesteatoma Encephalocele

Congenital Malformations • • •

External and middle ear (1st and 2nd branchial arch) develop independent of inner ear (ectodermal) IAC development separate from inner ear development Anomalies of all 3 parts are rare ➢ Dysplasias and trisomies 13, 18, 21

Fisher and Curtin, Otolaryngol Clin North Am 1994; 27:511-531

Outer Ear Anomalies • • • •

Congenital aural dysplasia Bilateral: 33% Genetic disorder association External auditory canal (EAC) atresia: failure of recanalization (26th gestational week) ➢ Fibrous vs. bony plate ➢ CT: middle ear dysplasia or cholesteatoma

Figure 5-4-16

Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al, AJNR 1997; 18:53-65

Middle Ear Anomalies • • •

Temporomandibular joint (TMJ) anomalies TMJ higher and more posterior than normal Facial nerve displaced ➢ Vertical portion more anterior than normal ➢ Very important pre-operative finding

Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al, AJNR 1997; 18:53-65

Inner Ear Anomalies [Figure 5-4-16] •

Lateral semicircular canal anomaly ➢ Last semicircular canal to form ➢ Usually short and wide, less commonly narrow

Anomalous shortening and widening of lateral semicircular canal

Jackler and Luxford, Laryngoscope 1987; 97:2-14

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Complete Labrynthine Aplasia • • • •

Figure 5-4-17

Michel’s deformity 3rd gestational week Inner ear absent Small cystic cavity: single or multiple

Jackler and Luxford, Laryngoscope 1987; 97:2-14

Incomplete Partition / Dilatational Defects [Figure 5-4-17] • • • • • •

Mondini’s dysplasia (1791): cochlea with 1 and 1/2 turns Second most common form of congenital deafness (Schiebe’s deafness #1) 7th gestational week Small cochlea with incomplete or absent intrascalar septum Basilar turn present Common cavity in place of middle and apical turns

Paparella, Ann Otol Rhinol Laryngol Suppl 1980; 89(2 Pt 3):1-10

Other Cochlear Anomalies [Figure 5-4-18] •

• •

Common cavity ➢ 4th gestational week

Mondini dysplasia

➢ Cochlea and vestibule fused ➢ 25% of all cochlear anomalies Cochlear aplasia ➢ 5th gestational week ➢ Rest of inner ear normal or malformed Cochlear Hypoplasia ➢ 6th gestational week

Figure 5-4-18

➢ Small cochlear bud Jackler and Luxford, Laryngoscope 1987; 97:2-14

Large Endolymphatic Duct and Sac (LEDS) • •

Large vestibular aqueduct syndrome Dilated vestibular aqueduct ➢ Most common radiologic finding in early-onset SNHL ❖ > 1.5 mm diameter (lateral SCC) ❖ MR: look at T2W images ➢ Often associated with incomplete partition cochlear anomalies ➢ Progressive sensorineural hearing loss (SNHL) ➢ Etiology: hyperosmolar protein transmission?

Valvassori and Clemis, Laryngoscope 1978; 88:723-728; Mafee, AJNR 1992;13:805-819; Jackler and De la Cruz, Laryngoscope 1989; 99:1238-1243; Dahlen et al, AJNR 1997; 18:67-75; Davidson et al, AJNR 1999; 20:1435-1441

Inner Ear Anomalies: Associations •

• •

Otocraniofacial ➢ Crouzon’s, Apert’s, etc. Otocervical ➢ Klippel-Feil, Goldenhar’s, etc. Otoskeletal ➢ Osteogenesis imperfecta, osteopetrosis, etc.

Enlarged vestibular aqueduct. Note size in comparison to lateral semicircular canal

Romo, Casselman, and Robson in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

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Aberrant Internal Carotid Artery [Figure 5-4-19] • • • •

Figure 5-4-19

90% females More common on right side Pulsatile tinnitus, conductive hearing loss (HL), otalgia Enhancing mass in hypotympanum (inferior tympanic canaliculus)

Sinnreich et al, Otolaryngol Head Neck Surg 1984; 92:194-206; Thiers et al, AJNR 2000; 21:1551-1554

Absent Internal Carotid Artery • • •

May be incidental discovery High association with intracranial aneurysms Nearly 30% present with subarachnoid hemorrhage

Aberrant internal carotid artery with soft tissue attenuation within middle ear and absent bony margin (Case courtesy of Wendy Smoker, MD, FACR)

Keen, Clin Proc 1946; 4:588-594; Martinez-Granero et al, Rev Neurol 1997; 25:1207-1209

Persistent Stapedial Artery • • • • • •

Figure 5-4-20

Rare: most seen at surgery Precursor for middle meningeal artery Small canal from carotid canal Crosses cochlear promontory Widened facial canal Absence of foramen spinosum

Thiers et al, AJNR 2000; 21:1551-1554

High Jugular Bulb (“Megabulb”) [Figure 5-4-20] • •

• • • •

Various definitions described Most common vascular anomaly of petrous temporal bone ➢ 3%–7% incidence More common on right side ➢ 75% jugular vein larger on right Usually poorly pneumatized mastoids No bony dehiscence Importance: surgical impact

Overton and Ritter, Laryngoscope 1973; 83: 1986-1991; Caldemyer et al, RadioGraphics 1997; 17:1123-1139 High jugular bulb with dehiscence along internal auditory canal

Other Jugular Vein Anomalies •

•

Dehiscent jugular bulb ➢ Direct communication with middle ear ➢ Lateral: pulsatile tinnitus, conductive hearing loss ➢ Medial: Meniere’s disease Jugular diverticulum ➢ Above, medial, posterior to petrous pyramid ➢ More common on left-side and in females

Figure 5-4-21

Couloigner et al, Eur Arch Otorhinolaryngol 1999; 256:224-229; Pappas et al, Otolaryngol Head Neck Surg, 1993; 109:847-852

Pial siderosis with thin bands of T2 hypointensity secondary to subarachnoid hemorrhage Neuroradiology

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Pulsatile Tinnitus Lesions •

Figure 5-4-22

[Figures 5-4-21 and 5-4-22]

• •

Congenital ➢ Aberrant internal carotid artery ➢ Dehiscent jugular bulb Tumor ➢ Paraganglioma ➢ Hemangioma Vascular ➢ Arteriovenous malformation / fistula ➢ Aneurysm ➢ Pial siderosis: VIII n. especially prone

Congenital Cholesteatoma (Epidermoid) • • •

Abnormal flow voids secondary to arteriovenous fistula

[Figures 5-4-23 and 5-4-24]

• • •

Child with conductive HL Aberrant epithelial rests Epitympanum, incudostapedial joint > petrous apex Globular mass +/– bone destruction Follows fluid signal intensity May have peripheral enhancement

Figure 5-4-23

Figure 5-4-24

Peron and Schuknecht, Arch Otolaryngol 1975: 101:498-505; Gao et al, AJNR 1992; 13:863-872

Congenital Dehiscence of Tegmen Tympani [Figure 5-4-25] •

Fusion of petrosquamosal suture by 1 year of age • Tiny openings: up to 34% of population ➢ Encephalocele, fistula: rare (requires dural weakening) Ossicular epidermoid with • Coronal plane best bone erosion Gavilan et al, Arch Otolaryngol 1984; 110-206-207; Gottlieb et al, Arch Otolaryngol 1998; 124:1274-1277

Contrast-enhanced coronal T1-weighted image shows rim enhancement of epidermoid involving right temporal bone

Figure 5-4-25

Axial T2-weighted image shows focal hyperintensity in region of epitympanum. Coronal CT images show soft-tissue density in epitympanum. Surgical exploration confirmed encephalocele Temporal Bone Anatomy and Congenital Lesions

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References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23.

Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: a review of anatomy, masses, and imaging characteristics. Radiographics 1997; 17:1123-1139. Couloigner V, Grayeli AB, Bouccara D, Julien N, Sterkers O. Surgical treatment of the high jugular bulb in patients with Meniere's disease and pulsatile tinnitus. Eur Arch Otorhinolaryngol 1999; 256:224-229. Dahlen RT, Harnsberger HR, Gray SD, et al. Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18:67-75. Davidson HC, Harnsberger HR, Lemmerling MM, et al. MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol 1999; 20:1435-1441. Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation. Epidermoid tumor of the cerebellopontine angle. AJNR Am J Neuroradiol 1992; 13:863-872. Gavilan J, Trujillo M, Gavilan C. Spontaneous encephalocele of the middle ear. Arch Otolaryngol 1984; 110:206207. Gottlieb MB, Blaugrund JE, Niparko JK. Imaging quiz case 1. Tegmental encephalocele. Arch Otolaryngol Head Neck Surg. 1998 Nov;124(11):1274, 1276-7. Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99:1238-1242; discussion 1242-1233. Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987; 97:2-14. Keen JA. Absence of both internal carotid arteries. Clin Proc 1945-1946;4:588 Mafee MF, Charletta D, Kumar A, Belmont H. Large vestibular aqueduct and congenital sensorineural hearing loss. AJNR Am J Neuroradiol 1992; 13:805-819. Martinez-Granero MA, Martinez-Bermejo A, Arcas J, et al. [Unilateral agenesis of the internal carotid artery in childhood: description of a case]. Rev Neurol 1997; 25:1207-1209. Mayer TE, Brueckmann H, Siegert R, Witt A, Weerda H. High-resolution CT of the temporal bone in dysplasia of the auricle and external auditory canal. AJNR Am J Neuroradiol 1997; 18:53-65. Overton SB, Ritter FN. A high placed jugular bulb in the middle ear: a clinical and temporal bone study. Laryngoscope 1973; 83:1986-1991. Paparella MM. Mondini's deafness. A review of histopathology. Ann Otol Rhinol Laryngol Suppl 1980; 89:1-10. Pappas DG, Jr., Hoffman RA, Cohen NL, Holliday RA, Pappas DG, Sr. Petrous jugular malposition (diverticulum). Otolaryngol Head Neck Surg 1993; 109:847-852. Peron DL, Schuknecht HF. Congenital cholesteatomata with other anomalies. Arch Otolaryngol 1975; 101:498505. Robson CD, Robertson RL, Barnes PD. Imaging of pediatric temporal bone abnormalities. Neuroimaging Clin N Am 1999; 9:133-155. Romo LV, Casselman JW, Robson CD. Temporal Bone: Congenital Anomalies. In: Som P.M., Curtin H.D. (eds) Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1275-1360. Sinnreich AI, Parisier SC, Cohen NL, Berreby M. Arterial malformations of the middle ear. Otolaryngol Head Neck Surg 1984; 92:194-206. Smith ME, Fisher C, Weiss SW. Pleomorphic hyalinizing angiectatic tumor of soft parts. A low-grade neoplasm resembling neurilemoma. Am J Surg Pathol 1996; 20:21-29. Thiers FA, Sakai O, Poe DS, Curtin HD. Persistent stapedial artery: CT findings. AJNR Am J Neuroradiol 2000; 21:1551-1554. Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope 1978; 88:723-728.

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Imaging of the Temporal Bone: Infectious and Neoplastic Lesions Kelly K. Koeller, MD, FACR Figure 5-5-1

Conductive Hearing Loss • •

Ossicular motion impeded Differential Diagnosis ➢ Cholesteatoma ➢ Hemangioma ➢ Glomus tympanicum ➢ Trauma: disruption ➢ Congenital ➢ Otosclerosis

Inflammatory Disease •

•

Mechanism ➢ Eustachian tube dysfunction ➢ Decreased intratympanic pressure ➢ Children: otitis media ➢ Adults: nasopharyngeal carcinoma Thin-section CT: soft tissue and fluid look alike ➢ Hounsfield units not helpful

Nemzek and Schwartz in Som and Curtin, Head and Neck Imaging, 4th ed, 2003, Mosby

Acquired Cholesteatoma • •

• •

[Figures 5-5-1 to 5-5-5]

Exfoliated keratin within sac of stratified squamous epithelium 98% of middle ear cholesteatomas ➢ Probably from retraction pocket in pars flaccida Prussak’s space: ossicles displaced medially Bone destruction ➢ Scutum and ossicles:coronal plane best ➢ Pars tensa: lateral semicircular canal, axial plane best

Artist rendition of retraction pocket at superior tympanic annulus caused by negative intratympanic pressure

Figure 5-5-2

Buckingham and Valvassori, Otolaryngol Clin North Am 1973; 6:363

Figure 5-5-3

Retraction pocket fills with epithelial debris from external auditory canal, creating a cholesteatoma Gross photograph of an acquired cholesteatoma, a sac of keratin lined with squamous epithelium

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Acquired Cholesteatoma • • • •

Figure 5-5-4

May not be able to distinguish from simple debris early in course MR: T1 & T2 prolongation Does not enhance (granulation tissue does) Treatment: excision or exteriorization ➢ Open cavity (canal wall down) mastoidectomy ❖ Radical: stapes left ❖ Modified radical (Bondy): all ossicles left

Phelps and Lloyd, Radiology 1986; 37:359-364; O’Donoghue et al, Clin Otolaryngol 1987; 12:89; Ishii et al, JCAT 1991; 15:934-937; Nemzek and Schwartz in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003 Coronal CT image of acquired cholesteatoma with erosion of the scutum and ossicles

Acquired Cholesteatoma: Complications •

[Figure 5-5-6]

• •

Labyrinthine fistula: lateral SCC most common ➢ Labyrinthitis Facial nerve canal ∗Tegmen tympani: intracranial

Figure 5-5-5

• ∗Sigmoid sinus erosion / thrombosis • “Automastoidectomy”: into EAC ∗MR recommended

Silver et al, Radiology 1987; 164:47; Schwartz, Radiology 1984; 153:443-447; Nemzek and Schwartz in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003

Middle ear “soft tissue” without bone erosion (not cholesteatoma!). Left: granulation tissue. Right: middle ear fluid

Figure 5-5-6

Cholesteatoma with erosion of mastoid bone adjacent to sigmoid dural sinus

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Mastoiditis •

• •

Figure 5-5-7

[Figures 5-5-7 and 5-5-8]

Osteomyelitis: patchy opacification ➢ Loss of mastoid septations ➢ Demineralization Coalescent: single cystic cavity Complications ➢ Bezold abscess: zygomatic root, EAC ➢ Gradenigo syndrome: petrous apicitis ❖ VI palsy, V neuralgia, chronic otitis ➢ Sigmoid sinus thrombosis ➢ Meningitis, epidural abscess

Castillo et al, AJR 1998; 17:1491-1495; Mafee et al, Radiology 1985; 155:391

Necrotizing External Otitis

• • • • • •

Osteomyelitis: bone destruction Diabetics: Pseudomonas AIDS: Aspergillus Cartilage portion: fissures of Santorini ➢ Spreads rapidly into adjacent spaces ➢ Parotid, facial nerve, intracranial Goal: determine extent of disease by CT and MR In-111 WBC study: post-therapy

Coalescent mastoiditis. Only a single cavity remains within the mastoid bone as a result of osteomyelitis

Slattery and Brackmann, Otolaryngol Clin North Am 1996; 29:795-806; Ress et al, Laryngoscope 1997; 107:456-460; Grandis et al, Radiology 1995: 196:499-504

External Ear Masses • • • •

Figure 5-5-8

[Figure 5-5-9]

Exostosis: chronic cold water exposure Usually broad-based and bilateral Bony portion of EAC Not an osteoma ➢ Usually unilateral, pedunculated, and lateral to EAC

DiBartolomeo, Ann Otolaryngol 1979; 88(suppl 61):2-20; Turetsky et al, AJNR 1990; 11:1217-1218

External Ear Masses •

•

Keratosis obturans ➢ < 40 years old ➢ Sinusitis, bronchiectasis ➢ Hearing loss ➢ Smooth external auditory canal (EAC) widening ➢ Entire EAC often filled EAC cholesteatoma: 0.5% of all cholesteatomas ➢ Otorrhea ➢ Focal erosions

Mastoiditis with posterior fossa epidural abscess (Case courtesy of Vanessa Albernaz, MD)

Figure 5-5-9

Piepergerdes et al, Laryngoscope 1980; 90:383-391

Bilateral exostoses Temporal Bone Infectious and Neoplastic Lesions

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External Ear Neoplasms [Figure 5-5-10] •

• • •

Figure 5-5-10

Skin cancers ➢ Squamous cell carcinoma ❖ Most common malignant ear tumor ➢ Basal cell carcinoma ➢ Melanoma “Ceruminoma” Parotid tumors Metastasis

Schuknecht, Pathology of the Ear, Harvard, 1974; Maya et al in Som and Curtin, Head Neck Imaging, 4th ed, Mosby, 2003

Cerebellopontine Angle Masses: The “AMEN” Differential Diagnosis • • • • •

Acoustic schwannoma (60–91%) Meningioma (3%-7%) Epidermoid (2%-6%) Nonacoustic schwannoma (1%-5%): V, VII Others ➢ Ependymoma, medulloblastoma, pilocytic astrocytoma ➢ Paraganglioma ➢ Arachnoid cyst ➢ Lipoma, dermoid, teratoma

External auditory canal erosion secondary to neoplasm

Brackmann and Bartels, Otolaryngol Head Neck Surg 1980; 88:555-559; Valavanis et al, Clinical Imaging of the Cerebellopontine Angle, Springer-Verlag, 1980; Gonzalez-Revilla, Johns Hopkins Hosp Bull 1948(83):187-189

Figure 5-5-11

Acoustic Schwannoma [Figure 5-5-11] • • • •

8%-10% of intracranial tumors 60%-90% of CPA tumors Most: 30-70 years old Neurofibromatosis type 2 (NF2): children, bilateral in 96% ➢ Schwann cell tumors, multiple meningiomas, gliomas ➢ First-degree relative counseling +/- imaging screening

Kasantikul et al, J Neurosurg 1980; 52:28-35; Martuza and Eldridge, N Engl J Med 1988; 318:684-688; Kishore and O’Reilly, Clin Otolaryngol 2000; 25:561-565

Canalicular vestibular schwannoma with smooth remodeling of the canal wall and loss of crista falciformis

Acoustic (Vestibular) Schwannoma [Figure 5-5-12] •

• • •

Sensorineural hearing loss, vertigo, tinnitus ➢ Speech discrimination impaired: telephone use Arise from vestibular division CN VIII usually ➢ Direct pressure on cochlear division Benign neoplasm ➢ Slow growth (0.2 cm per year) ➢ Well circumscribed globular mass Histology: Antoni A and B fibers [Figure 5-12-8]

Figure 5-5-12

Komatsuzaki and Tsunoda, J Laryngol Otol 2001; 115:376-379; NIH Consensus Development Conference, Arch Neurol 1994; 51:201-207 ; Lanser et al, Otolaryngol Clin North Am 1992; 25:499-520

Antoni A (left) and Antoni B (right) cell populations of a schwannoma Neuroradiology

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Vestibular Schwannoma: Imaging •

Figure 5-5-13

[Figures 5-5-13 to 5-5-16]

• • • •

IAC widening with “mushroom expansion ➢ “Giant”: usually no IAC involvement CT: usually isodense to cerebellum ➢ Calcification and hemorrhage rare T1WI: iso- to hypointense T2WI: hyperintense Intense enhancement: into porus acousticus and no dural tail

Maya et al in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003; Moller et al, Neuroradiology 1978; 17:25-30; Tali et al, AJNR 1993; 14:1241-1247; Schmalbrock et al, AJNR 1999; 20:1207-1213 Vestibular schwannoma with classic mushroom morphology on pre-contrast and post-contrast axial T1-weighted images

Figure 5-5-14

Figure 5-5-15

Coronal T2-weighted FSE image of right-sided vestibular schwannoma

Figure 5-5-16 Focal enhancement of deep portion of internal auditory canal secondary to arteriovenous malformation (not vestibular schwannoma)

Cystic degeneration of large vestibular schwannoma

Temporal Bone Infectious and Neoplastic Lesions

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Neuroradiology

Vestibular Schwannoma: Therapy •

• •

Figure 5-5-17

Surgical resection ➢ Larger masses ❖ Translabyrinthine: protect facial nerve ➢ Smaller masses ❖ Retrosigmoid: suboccipital approach ❖ Middle cranial fossa Stereotactic radiosurgery (gamma knife) up to 4 cm Poor surgical risk patients: serial MR

Jackler and Pitts, Otolaryngol Clin North Am 1992; 25:361-387; House and Shelton, Otolaryngol Clin North Am 1992; 25:347-359; Fucci et al, Am J Otol 1999; 20:497-508; Nakamura et al, AJNR 2000; 21:1540-1546

Meningioma •

• • • •

[Figures 5-5-17 and 5-5-18]

Usually eccentric to porus acousticus ➢ IAC involvement uncommon (16%) ➢ Frequently “trans-spatial” Broad dural base: hemispherical ➢ Obtuse bone-tumor angle ➢ Dural tail: 52%-72% ➢ Hyperostosis: highly characteristic NCCT:usually hyperdense (calcification: 25%) T1WI: isointense to gray matter T2WI: variable

Tentorial meningioma with extension into cerebellopontine angle

Figure 5-5-18

House and O’Conner, Handbook of Neurotological Diagnosis, Marcel-Dekker, 1987; Valavanis et al, Neuroradiology 1981; 22:111-121; Moller et al; Neuroradiology 1978; 17:25-30

Epidermoid • • • • • •

[Figure 5-5-19]

Soft, “pearly tumor” Irregular margins: “cauliflower” Follows CSF density and signal Usually no enhancement Diffusion-weighted imaging: hyperintense to CSF Differential Diagnosis: arachnoid cyst, cysticercosis, atypical dermoid, lipoma Cerebellopontine angle meningioma with numerous flow voids and fluid-fluid level. Note extension through foramina

Berger and Wilson. J Neurosurg 1985; 62:214-219; Gao et al, AJNR 1992; 13:863-872; Tampieri et al, AJNR 1989; 10:351-356; Tsuruda et al, AJR 1990; 155:1059-1065

Facial Nerve Palsy • •

•

Figure 5-5-19

MR: imaging study of choice Bell’s palsy: > 50%, nerve not enlarged ➢ Idiopathic, by definition (probably HSV) ➢ Imaging usually not performed Tumors: 6%, enlarged nerve ➢ Geniculate ganglion ❖ Schwannoma ❖ Hemangioma ➢ Epidermoid ➢ Parotid tumor spread

Tien et al, AJNR 1990; 11:735-741; Daniels et al, Radiology 1989; 17:807-809 Epidermoid of middle cranial fossa with extension into posterior fossa Neuroradiology

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Temporal Bone Paragangliomas •

• • •

Figure 5-5-20

Glomus jugulare: jugular foramen ➢ Jacobson’s (IX) and Arnold’s (X) nerve Glomus tympanicum: cochlear promontory ➢ Most common middle ear tumor ➢ Most common etiology of retrotympanic vascular mass Vagal paraganglioma: jugular ganglion Females 5:1; peak age: 40-60 years old

Rao et al, RadioGraphics 1999; 19:1605-1632

Jugulotympanic Paraganglioma [Figure 5-5-20] •

• •

• •

Neuroendocrine tumor ➢ Paraganglia: chemoreceptor function ➢ Functioning: 1%-3%, catecholamine secretion Early symptoms ➢ Conductive HL, pulsatile tinnitus Slow growth but locally invasive ➢ Mortality rate: 15% Metastasis very rare Path: chief cells (“Zellballen”), sustentacular cells “Zellballen” histologic appearance of paraganglioma

Rao et al, RadioGraphics 1999; 19:1605-1632

Figure 5-5-21

Jugulotympanic Paraganglioma: Imaging • • •

[Figures 5-5-21 to 5-5-23]

• • •

CT: irregular margins, “moth-eaten” erosion Glomus jugulare: may extend down carotid sheath MR: “Salt and pepper” appearance ➢ “Salt”: hyperintense foci (slow flow, hemorrhage) ➢ “Pepper”: serpentine flow voids Intense enhancement Angiography: ascending pharyngeal artery Radiologist’s goal: define extent

Rao et al, RadioGraphics 1999; 19:1605-1632

Figure 5-5-22

Glomus tympanicum (paraganglioma) (Case courtesy of William Kelly, MD)

Glomus jugulotympanicum (paraganglioma) Temporal Bone Infectious and Neoplastic Lesions

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Figure 5-5-23

“Salt-and-pepper” appearance of glomus jugulare on MR images

Jugular Foramen Masses • • • •

Paraganglioma: 90% Schwannoma: 9% Meningioma: <1% Malignant neoplasms: <1% ➢ Carcinoma ➢ Sarcoma ➢ Mets

Figure 5-5-24

Papillary Endolymphatic Sac Tumor [Figure 5-5-24] • • • • • •

Papillary endolymphatic sac tumor in different patients

Ipsilateral hearing loss, facial nerve palsy, vestibular dysfunction Females more common von Hippel-Lindau association Adenomatous tumor Bone destruction, intratumoral bone spicules T1WI: hyperintense

Heffner, Cancer 1989; 64:2292-2302; Lo, AJNR 1993;14:13221323; Palmer et al, Otolaryngol Head Neck Surg 1989; 100:6468; Mukherji et al, Radiology 1997; 202:801-808

Differential Diagnosis Petrous Apex • •

• • • •

Cholesterol granuloma (cyst): most common Epidermoid ➢ Follows cerebrospinal fluid (CSF) signal ➢ Solid mass: resection Chondrosarcoma Mucocele Carotid artery aneurysm Meningocele

Curtin and Som, Otolaryngol Clin North Am 1995; 28:473-496

Cholesterol Granuloma (Cyst) • • • •

Retention cyst: obstruction in petrous apex ➢ “Chocolate cyst”, “Blue-domed cyst”: within mastoidectomy cavity Young, middle-aged adults Hearing Loss, tinnitus, cranial nerve palsies Hemorrhage and foreign-body reaction: cholesterol crystals and blood (brownish fluid)

Lo et al, Radiology 1984;153:705-711; Graham et al, Laryngoscope 1985; 95:1401-1406; Latack et al, AJNR 1985; 6:409-413; Griffin et al, AJNR 1988; 8:825-829; Greenberg et al, AJNR 1988; 9:1205-1214

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Cholesterol Granuloma (Cyst) •

•

Figure 5-5-25

[Figure 5-5-25]

CT ➢ Isodense to brain ➢ Expansile, especially posterior ➢ Sharp smooth margins MR: hemorrhage ➢ Hyperintense on T1WI ➢ Hyperintense on T2WI

Lo et al, Radiology 1984;153:705-711; Latack et al, AJNR 1985; 6:409-413; Chang et al, Laryngoscope 1998; 108:599-604; Muckle et al, Am J Otol 1998: 19:219-225; Palacios and Valvassori, Ear Nose Throat J 1999; 78:234 Cholesterol granuloma

Chondrosarcoma • • • • • •

[Figure 5-5-26]

Most common primary neoplasm of petrous apex Off the midline: sutures ➢ Petrosphenoidal ➢ Petro-occipital Locally invasive Bone destruction: no sclerosis T1 and T2 prolongation Intense but heterogeneous enhancement

Grossman and Davis, Radiology 1981; 141:403-408; Meyers et al, Radiology 1992; 184:103-108; Bourgouin et al, JCAT 1992; 16:268-273

Temporal Bone Fracture Frequency Axis Blow Middle ear injury Inner ear injury Facial paralysis

Tegmen tympani disruption

Longitudinal 80% Long Temporoparietal Likely Rare 10–20%, usually incomplete & delayed Common

Transverse 20% Short Occipital Rare Common 40–50%, usually acute & complete Less common

Schwartz and Curtin in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

Figure 5-5-26

Chondrosarcoma Temporal Bone Infectious and Neoplastic Lesions

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Ossicular Derangement • •

•

[Figure 5-5-27]

Need 1 mm CT images or thinner Incus: most vulnerable ➢ Subluxation from malleus ➢ Dislocation Incudostapedial disruption ➢ Most common cause of post-traumatic conductive HL ➢ Normal: <1 mm between lenticular process of incus & stapes head

Figure 5-5-27

Lourenco et al, Am J Otol 1995; 16:387-392; Swartz et al; Radiology 1989; 171:309-317

Otosclerosis • • •

• •

Primary endochondral bone within otic capsule replaced by spongy vascular bone (“otospongiosis”) Slowly progressive Presents 10–30 years old with tinnitus ➢ Hearing loss later Females more common (70%) Bilateral 80% (usually asynchronous)

Valvassori, Otolaryngol Clin North Am, 1973; 6:379-389; Reudi, Arch Otolaryngol 1963; 78:469-477

Otosclerosis •

• • • •

Fenestral type (80%): CHL ➢ Begins at anterior oval window Cochlear type (20%): SNHL ➢ Almost always with fenestral type ➢ “Double Ring” sign Demineralized areas: active disease Chronic disease: may appear normal MR: punctate enhancement

Ossicular derangement

Mafee et al, Radiology 1985; 156:703-708; Swartz et al, Radiology 1985; 155:147150; Sakai et al, Am J Otolaryngol 2000; 21:116-118

Summary • • • • • •

• • • •

Challenging complex anatomy Facial nerve course: critical for pre-operative evaluation Bony plate between hypotympanum and ICA canal Tegmen tympani Cholesteatoma: bone erosion Cerebellopontine Angle: “AMEN” ➢ Vestibular schwannoma: most common ➢ Meningioma ➢ Epidermoid ➢ Non-acoustic schwannoma Jugular foramen ➢ Paraganglioma ➢ Schwannoma Petrous apex ➢ Cholesterol granuloma vs. epidermoid ➢ Chondrosarcoma, Chordoma, Metastasis Otosclerosis Radiologist’s Goals ➢ Define extent of lesion

References 1.

Berger MS, Wilson CB. Epidermoid cysts of the posterior fossa. J Neurosurg 1985; 62:214-219.

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2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32.

Bourgouin PM, Tampieri D, Robitaille Y, et al. Low-grade myxoid chondrosarcoma of the base of the skull: CT, MR, and histopathology. J Comput Assist Tomogr 1992; 16:268-273. Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg 1980; 88:555559. Buckingham RA, Valvassori GE. Tomographic evaluation of cholesteatomas of the middle ear and mastoid. Otolaryngol Clin North Am 1973; 6:363-378. Griffin C, De La Paz R, Enzmann D MR And CT correlation of cholesterol cysts of petrous bone. AJNR, 1987, 8:825-829. Castillo M, Albernaz VS, Mukherji SK, Smith MM, Weissman JL. Imaging of Bezold's abscess. AJR Am J Roentgenol 1998; 171:1491-1495. Chang P, Fagan PA, Atlas MD, Roche J. Imaging destructive lesions of the petrous apex. Laryngoscope 1998; 108:599-604. Curtin HD, Som PM. The petrous apex. Otolaryngol Clin North Am 1995; 28:473-496. Daniels DL, Czervionke LF, Millen SJ, et al. MR imaging of facial nerve enhancement in Bell palsy or after temporal bone surgery. Radiology 1989; 171:807-809. DiBartolomeo JR. Exostoses of the external auditory canal.Ann Otol Rhinol Laryngol Suppl. 1979 Nov-Dec;88(6 Pt 2 Suppl 61):2-20. Fucci MJ, Buchman CA, Brackmann DE, Berliner KI. Acoustic tumor growth: implications for treatment choices. Am J Otol 1999; 20:495-499. Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation. Epidermoid tumor of the cerebellopontine angle. AJNR Am J Neuroradiol 1992; 13:863-872. Gonzales-Revilla A. Differential diagnosis of tumors at the cerebellopontile recess. Bulletin of the Johns Hopkins Hospital1948, 83: 187 Graham MD, Kemink JL, Latack JT, Kartush JM. The giant cholesterol cyst of the petrous apex: a distinct clinical entity. Laryngoscope 1985; 95:1401-1406. Grandis JR, Curtin HD, Yu VL. Necrotizing (malignant) external otitis: prospective comparison of CT and MR imaging in diagnosis and follow-up. Radiology 1995; 196:499-504. Greenberg JJ, Oot RF, Wismer GL, et al. Cholesterol granuloma of the petrous apex: MR and CT evaluation. AJNR Am J Neuroradiol 1988; 9:1205-1214. Grossman RI, Davis KR. Cranial computed tomographic appearance of chondrosarcoma of the base of the skull. Radiology 1981; 141:403-408. Heffner DK. Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer 1989; 64:2292-2302. House JW; O'Connor AF, Marcel Dekker, ed. Handbook of Neurotological Diagnosis, Inc., Science and Practice of Surgery, Marcel-Dekker,1987. House WF, Shelton C. Middle fossa approach for acoustic tumor removal. Otolaryngol Clin North Am 1992; 25:347-359. Ishii K, Takahashi S, Kobayashi T, Matsumoto K, Ishibashi T. MR imaging of middle ear cholesteatomas. J Comput Assist Tomogr 1991; 15:934-937. Jackler RK, Pitts LH. Selection of surgical approach to acoustic neuroma. Otolaryngol Clin North Am 1992; 25:361-387. Kasantikul V, Netsky MG, Glasscock ME, 3rd, Hays JW. Acoustic neurilemmoma. Clinicoanatomical study of 103 patients. J Neurosurg 1980; 52:28-35. Kishore A, O'Reilly BF. A clinical study of vestibular schwannomas in type 2 neurofibromatosis. Clin Otolaryngol Allied Sci 2000; 25:561-565. Komatsuzaki A, Tsunoda A. Nerve origin of the acoustic neuroma. J Laryngol Otol 2001; 115:376-379. Lanser MJ, Sussman SA, Frazer K. Epidemiology, pathogenesis, and genetics of acoustic tumors. Otolaryngol Clin North Am 1992; 25:499-520. Latack JT, Graham MD, Kemink JL, Knake JE. Giant cholesterol cysts of the petrous apex: radiologic features. AJNR Am J Neuroradiol 1985; 6:409-413. Lo WW, Solti-Bohman LG, Brackmann DE, Gruskin P. Cholesterol granuloma of the petrous apex: CT diagnosis. Radiology 1984; 153:705-711. Lourenco MT, Yeakley JW, Ghorayeb BY. The "Y" sign of lateral dislocation of the incus. Am J Otol 1995; 16:387-392. Mafee MF, Singleton EL, Valvassori GE, Espinosa GA, Kumar A, Aimi K. Acute otomastoiditis and its complications: role of CT. Radiology 1985; 155:391-397. Mafee MF, Valvassori GE, Deitch RL, et al. Use of CT in the evaluation of cochlear otosclerosis. Radiology 1985; 156:703-708. Martuza RL, Eldridge R. Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 1988; 318:684688.

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33. Maya M.M., Lo W.W.M., Kouvanlikaya I. Tumors and cerebellopontine angle lesions. In: Som P.M., Curtin H.D. (eds) Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1275-1360. 34. Meyer JR, Gebarski SS, Blaivas M. Cerebellopontine angle invasive papillary cystadenoma of endolymphatic sac origin with temporal bone involvement. AJNR Am J Neuroradiol 1993; 14:1319-1321; Discussion Lo, 1322-1313. 35. Meyers SP, Hirsch WL, Jr., Curtin HD, Barnes L, Sekhar LN, Sen C. Chondrosarcomas of the skull base: MR imaging features. Radiology 1992; 184:103-108. 36. Moller A, Hatam A, Olivecrona H. Diagnosis of acoustic neuroma with computed tomography. Neuroradiology 1978; 17:25-30. 37. Muckle RP, De la Cruz A, Lo WM. Petrous apex lesions. Am J Otol 1998; 19:219-225. 38. Mukherji SK, Albernaz VS, Lo WW, et al. Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology 1997; 202:801-808. 39. Nakamura H, Jokura H, Takahashi K, Boku N, Akabane A, Yoshimoto T. Serial follow-up MR imaging after gamma knife radiosurgery for vestibular schwannoma. AJNR Am J Neuroradiol 2000; 21:1540-1546. 40. National Institutes of Health Consensus Development Conference Statement on Acoustic Neuroma, December 1113, 1991. The Consensus Development Panel. Arch Neurol 1994; 51:201-207. 41. Nemzek WR, Swartz JD. Temporal Bone: Inflamatory Disease. In: Som P.M., Curtin H.D. (eds) Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1173. 42. O'Donoghue GM, Bates GJ, Anslow P, Rothera MP. The predictive value of high resolution computerized tomography in chronic suppurative ear disease. Clin Otolaryngol Allied Sci 1987; 12:89-96. 43. Palacios E, Valvassori G. Petrous apex lesions: cholesterol granuloma. Ear Nose Throat J 1999; 78:234. 44. Palmer JM, Coker NJ, Harper RL. Papillary adenoma of the temporal bone in von Hippel-Lindau disease. Otolaryngol Head Neck Surg 1989; 100:64-68. 45. Phelps PD, Lloyd GA. Vascular masses in the middle ear. Clin Radiol. 1986 Jul;37(4):359–364. 46. Piepergerdes MC, Kramer BM, Behnke EE. Keratosis obturans and external auditory canal cholesteatoma. Laryngoscope 1980; 90:383-391. 47. Rao AB, Koeller KK, Adair CF. From the archives of the AFIP. Paragangliomas of the head and neck: radiologicpathologic correlation. Armed Forces Institute of Pathology. Radiographics 1999; 19:1605-1632. 48. Ress BD, Luntz M, Telischi FF, Balkany TJ, Whiteman ML. Necrotizing external otitis in patients with AIDS. Laryngoscope 1997; 107:456-460. 49. Ruedi L. Pathogenesis of Otosclerosis. Arch Otolaryngol 1963; 78:469-477. 50. Sakai O, Curtin HD, Fujita A, Kakoi H, Kitamura K. Otosclerosis: computed tomography and magnetic resonance findings. Am J Otolaryngol 2000; 21:116-118. 51. Schmalbrock P, Chakeres DW, Monroe JW, Saraswat A, Miles BA, Welling DB. Assessment of internal auditory canal tumors: a comparison of contrast-enhanced T1-weighted and steady-state T2-weighted gradient-echo MR imaging. AJNR Am J Neuroradiol 1999; 20:1207-1213. 52. Schuknecht, H. F. Pathology of the ear. Cambridge: Harvard University Press, 1974. 53. Silver AJ, Janecka I, Wazen J, Hilal SK, Rutledge JN. Complicated cholesteatomas: CT findings in inner ear complications of middle ear cholesteatomas. Radiology 1987; 164:47-51. 54. Slattery WH, 3rd, Brackmann DE. Skull base osteomyelitis. Malignant external otitis. Otolaryngol Clin North Am 1996; 29:795-806. 55. Swartz JD, Curtin HD. Temporal Bone: Trauma. In: Som P.M., Curtin H.D. (eds) Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1173. 56. Swartz JD, Mandell DW, Berman SE, Wolfson RJ, Marlowe FI, Popky GL. Cochlear otosclerosis (otospongiosis): CT analysis with audiometric correlation. Radiology 1985; 155:147-150. 57. Swartz JD. Current imaging approach to the temporal bone. Radiology 1989; 171:309-317. 58. Swartz JD. The facial nerve canal: CT analysis of the protruding tympanic segment. Radiology 1984; 153:443-447. 59. Tali ET, Yuh WT, Nguyen HD, et al. Cystic acoustic schwannomas: MR characteristics. AJNR Am J Neuroradiol 1993; 14:1241-1247. 60. Tampieri D, Melanson D, Ethier R. MR imaging of epidermoid cysts. AJNR Am J Neuroradiol 1989; 10:351-356. 61. Tien R, Dillon WP, Jackler RK. Contrast-enhanced MR imaging of the facial nerve in 11 patients with Bell's palsy. AJNR Am J Neuroradiol 1990; 11:735-741. 62. Tsuruda JS, Chew WM, Moseley ME, Norman D. Diffusion-weighted MR imaging of the brain: value of differentiating between extraaxial cysts and epidermoid tumors. AJR Am J Roentgenol 1990; 155:1059-1065; discussion 1066-1058. 63. Turetsky DB, Vines FS, Clayman DA. Surfer's ear: exostoses of the external auditory canal. AJNR Am J Neuroradiol 1990; 11:1217-1218. 64. Valavanis A, Schubiger O, Hayek J, Pouliadis G. CT of meningiomas on the Posterior surface of the petrous bone. Neuroradiology 1981; 22:111-121. 65. Valavanis A, Schubiger O, Naidich TP: Clinical Imaging of the Cerebello-Pontine Angle. Berlin: Springer-Verlag, 1987. 66. Valvassori GE. Otosclerosis. Otolaryngol Clin North Am 1973; 6:379-389.

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Imaging of the Orbit: The Globe and Conal Lesions Kelly K. Koeller, MD, FACR The Bony Orbit •

Figure 5-6-1 [Figure 5-6-1]

Bones ➢ Frontal ➢ Maxilla ➢ Sphenoid ➢ Zygoma ➢ Ethmoid ➢ Lacrimal ➢ Palatine

The Bony Orbit •

•

Superior orbital fissure ➢ Middle cranial fossa ➢ Cranial nerves III, IV, VI, V1 ➢ Superior and inferior ophthalmic veins Inferior orbital fissure ➢ Pterygopalatine (V2) & infratemporal fossae

Anterior Orbit •

• •

Figure 5-6-2

[Figures 5-6-2 and 5-6-3]

Orbital septal system ➢ Anterior: well-developped (“preseptal space”) ❖ Arises from periosteum of anterior bony orbit ❖ Attaches to tarsal plates of eyelids ➢ Posterior: incomplete Lacrimal gland Lacrimal sac and nasolacrimal duct

Globe • • • • •

The bony orbit

[Figure 5-6-4]

Anterior chamber: aqueous humor Iris and ciliary body Posterior chamber: aqueous humor Lens apparatus Vitreous body: gel-like (collagen fibrils) ➢ Most: free water

Lacrimal glands in superolateral portion of the bony orbit

Figure 5-6-3

Figure 5-6-4

Nasolacrimal ducts in inferomedial portions of bony orbit

Normal globe with anterior and posterior chambers located anterior to the lens and the vitreous body constituting most of the globe Imaging of the Orbit: The Globe and Conal Lesions

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Globe •

• • • •

Figure 5-6-5

[Figure 5-6-5]

Retina ➢ Neural sensory inner layer (photoreceptors) ➢ Retinal pigmented epithelium (RPE) outer layer ➢ Ora serrata Uvea: choroid (vascular); Bruch’s membrane ➢ Iris ➢ Ciliary body Sclera: fibrous layer; cornea anteriorly Tenon’s capsule (bulbar fascia) Normal: only one “layer” seen

Retrobulbar (“Postseptal”) Space • •

[Figures 5-6-6 to 5-6-8]

• •

Fat with fibrous septa Extraocular muscles (EOM) (“The Cone”) ➢ Rectus: medial, lateral, superior, inferior ❖ Annulus of Zinn: optic canal ❖ Intermuscular septa (incomplete posteriorly) ➢ Oblique: superior (trochlear), inferior ➢ Levator palpebrae superioris Optic nerve: glial-lined Vessels

Figure 5-6-6

Close-up view of the posterior globe layers. Retina is innermost, followed by choroid and sclera. The macula is located lateral to the optic disk

Figure 5-6-7

Extraocular muscles. The superior oblique muscle travels through the trochlea, a bony strut near the superomedial margin of the bony orbit

Coronal view of the six extraocular muscles and levator palpebrae superioris

Figure 5-6-8

Papilledema with increased fluid surrounding optic nerves bilaterally caused by a supratentorial oligodendroglioma Neuroradiology

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Cranial Nerves III, IV, VI • •

•

Figure 5-6-9

Motor control of EOMs Cranial Nerve III: all EOMs except ➢ Lateral rectus: Cranial Nerve VI ➢ Superior oblique: Cranial Nerve IV ➢ “LR6SO4” Sensory control: V1 primarily, V2 (infraorbital region)

Senile Macular Degeneration • •

• • •

Most common cause of legal blindness in the elderly Hyalinization of macula, thickening of Bruch’s membrane ➢ Pigment epithelium detachment ➢ Serous subretinal space fluid Hemorrhage: fibrous scar, macular loss Computed tomography (CT): mimics uveal melanoma Magnetic resonance (MR): variable

Mafee, in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

Posterior Hyaloid Detachment • • • •

Separation of the hyaloid base (posterior hyaloid membrane) from retinal sensory epithelium Usually adults with myopia ➢ Liquefaction of vitreous ➢ Children: persistent hyperplastic primary vitreous Association: macular degeneration Intravitreal and curvilinear layer ➢ Not connected to optic disk

Retinal detachment, with characteristic V-shape created by anchor points at optic disk and ora serrata

Mafee in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

Retinal Detachment (RD) • • • •

•

[Figure 5-6-9]

Sensory retina separates from RPE Rhegmatogenous RD: tear in retina RPE: can heal (laser therapy) Ultrasonography (US) superior to MR or CT for detection ➢ Causes: mass, fibroproliferative disease, toxocara, choroidal lesions V-shape: apex at optic disk

Figure 5-6-10

Mafee and Peyman, Radiol Clin North Am 1987;25:487-507

Choroidal Detachment • • •

•

[Figure 5-6-10]

Hemorrhagic: contusion Serous: ocular hypotony (choroidal inflammation, trauma, glaucoma therapy) U-shaped ➢ Anchor points: short posterior ciliary artery, vortex veins No connection with optic disk

Mafee and Peyman, Radiol Clin North Am 1987;25:487-507

Leukocoria • • • • • • •

Retinoblastoma Persistent hyperplastic primary vitreous (PHPV) Retinopathy of prematurity (ROP) Congenital cataract Coats’ disease Toxocariasis Total retinal detachment

Choroidal detachment, with typical Ushape created by anchor points at ciliary body and vessels away from the optic disk

Mafee, in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

Imaging of the Orbit: The Globe and Conal Lesions

1088 1090

Neuroradiology

Retinoblastoma •

• • • •

Figure 5-6-11

Most common intraocular tumor of childhood ➢ Incidence 1:15,000 ➢ Virtually all patients < 6 years-old ➢ 80% 3 years old or younger ➢ 13 months: average age at presentation No gender or racial predilection “Retinoblastoma gene”: chromosome 13q14 ➢ “Germinal” (inherited) 85% bilateral ➢ “Somatic” (not inherited) unilateral Unilateral: 60%-70% Association: osteosarcoma, other sarcomas

Abramson et al, Ophthalmology 1984; 91:1351-1355; Pendergrass and Davis, Arch Ophthalmol 1980; 98:1204-1210; Ellsworth, Trans Am Ophthalmol Soc 1969; 67:462-534; Kaufman et al, Radiol Clin North Am 1998; 36:1101-1117

Retinoblastoma •

• • • •

Gross photograph of retinoblastoma

[Figure 5-6-11]

Figure 5-6-12

Ophthalmoscopic diagnosis primarly ➢ Small gray-white intraretinal lesions, calcification, seeding ➢ Ultrasonography: 80% accurate Stage 1: confined to the globe Stage 2: extraocular extension to orbit or optic nerve Stage 3: extra-orbital extension 92% 5-year survival for intra-ocular lesions but near 100% mortality when extends beyond eye

Kodilyne, Am J Ophthalmol 1967; 63:467-481; Abramson et al, Arch Ophthalmol 1981; 99:1761-1762 • Neuroectodermal origin: primitive embryonal retinal cells (retinoblasts) • Rosettes: Flexner-Wintersteiner or Homer-Wright type • Highly malignant: necrosis, mitotic figures • Calcification

Retinoblastoma – Pathology

Kyritsis et al, Nature 1984; 307:471-473

Retinoblastoma - Imaging •

• •

[Figure 5-6-12]

CT: imaging study of choice Retinoblastoma with characteristic ➢ Calcification: >90% of cases calcification on CT ❖ Child < 3 y/o: highly suggestive for diagnosis Tri- / tetralateral retinoblastoma with pineal and/or suprasellar masses MR: not as specific as CT ➢ Hyperintense on T1WI and PD ➢ Hypointense on T2WI ➢ May miss lesions as large as 4mm ➢ Better for intracranial extension, extraocular spread

Char, Ophthalmology 1984; 91:1347-1350; Mafee, Radiol Clin North Am 1987; 25:667-681; Mafee et al, Ophthalmology 1989; 96:965-976

Persistent Hyperplastic Primary Vitreous (PHPV) [Figure 5-6-13] • •

• •

Failure of embryonic hyaloid system (primary vitreous) to regress normally and form the secondary vitreous by 5th-6th gestational month Isolated or part of more complex abnormality ➢ Bilateral: Norrie’s, Warburg’s Usually unilateral leukokoria and microphthalmos ➢ Lens opacity, RD, vitreous hemorrhage Persistent hyaloid (Cloquet’s) canal ➢ No calcification

Mafee and Goldberg, Radiol Clin North Am 1987; 25:683-692 Neuroradiology

1089 1091

Imaging of the Orbit: The Globe and Conal Lesions

Retinopathy of prematurity

Figure 5-6-13

Coats Disease [Figure 5-6-14] • • •

•

Juvenile males most common Usually unilateral (85%-90%) Peripheral telangiectasias ➢ Leak lipoproteinaceous exudate ➢ Retinal detachment May mimic retinoblastoma clinically

Coats, R Lond Ophthalmol Hosp Rep 1908; 17:440525; Reese, Am J Ophthalmol 1956; 42:1-8; Edward et al, Radiol Clin North Am 1988; 36: 1119-1131

Toxocariasis • • •

•

Chorioretinitis: Toxocara canis (nematode) Granuloma: eosinophilic abscess Persistent hyperplastic primary vitreous (PHPV) CT: homogeneous intravitreal density with hyaloid canal ➢ Retinal detachment, organized vitreous, inflammatory exudate ➢ Irregular thickening of uveoscleral coat MR: subretinal exudate, variably hyperintense on all sequences

Margo et al, Pediatr Ophthalmol Strabismus 1983; 20: 180-184; Wilder, Trans Am Acad Ophthalmol Otolaryngol 1950; 55:99-104

Figure 5-6-14

Uveal Melanoma •

• • •

Uvea ➢ Choroid, ciliary body, iris ➢ Derived from mesoderm & neuroectoderm ➢ Most highly vascular part of eyeball Melanoma: most common neoplasm of choroid Whites (15:1) ➢ Incidence increases with age Arises from choroid, elevates and may rupture Bruch’s membrane (mushroom shape)

Yanoff and Fine, Ocular Pathology, Harper and Row, 1975; Mafee, Radiol Clin North Am 1998; 36:1083-1099

Uveal Melanoma • • • •

•

Gross photograph of Coats Disease

[Figure 5-6-15]

Metastasis: liver > lung > bone > kidney > brain Diagnosis usually made by ophthalmoscopy or US CT: elevated, hyperdense, sharply marginated (usually) solid mass MR ➢ Hyperintense on T1WI and PDW ➢ Hypointense on T2WI Moderate enhancement

Duffin et al, Arch Ophthalmol 1981; 99:1827-1830; Enochs et al, Radiology 1997; 204:417-423; Mafee in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

Figure 5-6-15

Uveal melanoma Imaging of the Orbit: The Globe and Conal Lesions

1090 1092

Neuroradiology

Uveal Metastasis • • • • • •

Figure 5-6-16

[Figure 5-6-16]

Usually in the plane of the choroid with little increased thickness Mottled appearance, diffuse outline Breast and lung cancer most common Retina or choroid Bilateral 1/3 (melanoma rarely bilateral) Mucinous adenocarcinoma: mimic melanoma

Mafee, Radiol Clin North Am 1998; 36:1083-1099

Orbital Trauma • •

•

• • •

[Figures 5-6-17 and 5-6-18]

CT: imaging modality of choice Fractures ➢ Isolated: orbital rim (Waters view) ➢ Blowout: inferior wall; medial wall: 1/2 ➢ Nasoethmoidal (NOE) complex ❖ canthal injury common ➢ Zygomatic complex (ZC) ➢ Lefort types: I, II, III ➢ Orbital apex-optic canal Hemorrhage ➢ Retrobulbar: most common ➢ Extraconal ➢ Subperiosteal ➢ Sub-Tenon capsule ➢ Subdural (optic nerve sheath) Optic nerve injury Eyeball injury: phthisis bulbi Foreign Body

Bilateral uveal metastases

Figure 5-6-17

Figure 5-6-18

Blow-out fracture

Orbital fracture extending towards optic canal Neuroradiology

1091 1093

Imaging of the Orbit: The Globe and Conal Lesions

Conal Lesions • • •

Graves Pseudotumor Others ➢ Lymphoproliferative disease ➢ Metastasis: 7%, breast carcinoma, nodular ➢ Arteriovenous fistula or vascular congestion ➢ Acromegaly ➢ Amyloid ➢ Cysticercosis / Trichinosis

Thyroid Orbitopathy (Graves Disease) •

• • •

Autoimmune disorder: orbital soft tissues, thyroid, extremities ➢ Superior cervical lymph channel: drains both thyroid and orbit Incidence: 0.5% (U. S.) Most common orbital disorder Most common cause of exophthalmos in adults ➢ 15%–28% of unilateral exophthalmos ➢ 80% of bilateral exophthalmos

Figure 5-6-19

Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999; Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003

Graves Disease •

• • • •

Most (up to 80%) patients are or will be hyperthyroid ➢ Euthyroid (10%) Family history: 30% Range: 15–86 years old (peak: 30–50) Females much more common (4:1) Males, patients > 50y/o: more severe disease

Kendler et al, Arch Ophthalmol 1993; 111:197-201

Graves Disease •

•

Acute phase ➢ Inflammatory reaction: congestion, hypertrophy, fibrosis of orbital fat / muscles ➢ Mucopolysaccharides accumulate in EOMs Chronic phase: exophthalmos (34%–93%) ➢ Fibrosis ➢ Restrictive myopathy ➢ Diplopia

Graves Disease

Figure 5-6-20

Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999

Graves Disease: Imaging •

• • •

[Figures 5-6-19 and 5-6-20]

Muscle Enlargement ➢ Inferior rectus ➢ Medial rectus ➢ Superior muscle complex ➢ Lateral rectus ➢ ? Related to innervation and fiber size Tendon spared “Dirty” retrobulbar fat: inflammation Apex: optic nerve compression

Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003

Graves Disease with sparing of the tendinous insertions Imaging of the Orbit: The Globe and Conal Lesions

1092 1094

Neuroradiology

Pseudotumor • • • • •

Nongranulomatous inflammation No known cause Diagnosis of exclusion Second most common (~5%) orbital disease (after Graves disease) Children: 6%–16% of all cases, more frequently bilateral

Blodi and Gass, Br J Ophthalmol 1968; 52:79-93; Flanders et al, J Comput Assist Tomogr 1989; 13:40-47; Weber et al, Radiol Clin North Am 1999; 37:151-168

Figure 5-6-21

Pseudotumor •

• •

Acute form ➢ Abrupt onset of pain usually ➢ Lid swelling, redness, ptosis, proptosis Chronic form ➢ Fixation signs: diplopia, proptosis Sites ➢ Lacrimal gland (lacrimal adenitis) ➢ Extraocular muscles (myositic form) ➢ Cavernous sinus (Tolosa-Hunt)

Tolosa, J Neurol Neurosurg Psychiatry 1954; 17:300-302; Hunt, Neurology 1961; 11:56-62

Pseudotumor: EOM • • • • • •

Tendons involved (unlike Graves disease) Ragged “fluffy” muscle border Inward bowing of muscle contour at globe insertion “Dirty” retrobulbar fat May extend intracranially (apical orbital inflammation) or onto optic nerve (ON) sheath (perineuritis) Bone destruction rare

Trokel and Hilal, Am J Ophthalmol 1979; 87:503-512; Flanders et al, J Comput Assist Tomogr 1989; 13:40-47

Pseudotumor: Imaging •

•

[Figures 5-6-21 and 5-6-22]

CT ➢ Nonspecific ➢ Moderate enhancement MR ➢ Hypointense on T1WI and T2WI

Pseudotumor with tendinous involvement and rapid response to steroid therapy on follow-up

Figure 5-6-22

Pseudotumor with characteristic T1 and T2 hypointensity Neuroradiology

1093 1095

Imaging of the Orbit: The Globe and Conal Lesions

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18.

19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32.

Abramson DH, Ellsworth RM, Kitchin FD, Tung G. Second nonocular tumors in retinoblastoma survivors. Are they radiation-induced? Ophthalmology 1984; 91:1351-1355. Abramson DH, Ellsworth RM, Tretter P, Javitt J, Kitchin FD. Treatment of bilateral groups I through III retinoblastoma with bilateral radiation. Arch Ophthalmol 1981; 99:1761-1762. Blodi FC, Gas JD. Inflammatory pseudotumour of the orbit. Br J Ophthalmol 1968; 52:79-93. Char DH, Hedges TR, 3rd, Norman D. Retinoblastoma. CT diagnosis. Ophthalmology 1984; 91:1347-1350. Coats G, Lond R. Forms of retinal diseases with massive exudation. Ophthalmol Hosp Rep 1908; 17:440-525. Duffin RM, Straatsma BR, Foos RY, Kerman BM. Small malignant melanoma of the choroid with extraocular extension. Arch Ophthalmol 1981; 99:1827-1830. Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary vitreous. Role of MR imaging and CT. Radiol Clin North Am 1998; 36:1119-1131, x. Eller AW, Jabbour NM, Hirose T, Schepens CL. Retinopathy of prematurity. The association of a persistent hyaloid artery. Ophthalmology 1987; 94:444-448. Ellsworth RM. The practical management of retinoblastoma. Trans Am Ophthalmol Soc 1969; 67:462-534. Enochs WS, Petherick P, Bogdanova A, Mohr U, Weissleder R. Paramagnetic metal scavenging by melanin: MR imaging. Radiology 1997; 204:417-423. Flanders AE, Mafee MF, Rao VM, Choi KH. CT characteristics of orbital pseudotumors and other orbital inflammatory processes. J Comput Assist Tomogr 1989; 13:40-47. Hunt WE, Meagher JN, Lefever HE, Zeman W. Painful opthalmoplegia. Its relation to indolent inflammation of the carvernous sinus. Neurology 1961; 11:56-62. Jakobiec FA, Tso MO, Zimmerman LE, Danis P. Retinoblastoma and intracranial malignancy. Cancer 1977; 39:2048-2058. Kaufman LM, Mafee MF, Song CD. Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of Gd-DTPA contrast enhancement. Radiol Clin North Am 1998; 36:1101-1117. Kendler DL, Lippa J, Rootman J. The initial clinical characteristics of Graves' orbitopathy vary with age and sex. Arch Ophthalmol 1993; 111:197-201. Kodilinye HC. Retinoblastoma in Nigeria: problems of treatment. Am J Ophthalmol 1967; 63:469-481. Kyritsis AP, Tsokos M, Triche TJ, Chader GJ. Retinoblastoma--origin from a primitive neuroectodermal cell? Nature 1984; 307:471-473. Mafee MF, Goldberg MF, Cohen SB, et al. Magnetic resonance imaging versus computed tomography of leukocoric eyes and use of in vitro proton magnetic resonance spectroscopy of retinoblastoma. Ophthalmology 1989; 96:965-975; discussion 975-966. Mafee MF, Goldberg MF, Greenwald MJ, Schulman J, Malmed A, Flanders AE. Retinoblastoma and simulating lesions: role of CT and MR imaging. Radiol Clin North Am 1987; 25:667-682. Mafee MF, Goldberg MF. Persistent hyperplastic primary vitreous (PHPV): role of computed tomography and magnetic resonance. Radiol Clin North Am 1987; 25:683-692. Mafee MF, Peyman GA. Retinal and choroidal detachments: role of magnetic resonance imaging and computed tomography. Radiol Clin North Am 1987; 25:487-507. Mafee MF. The eye. In: Som PM, Curtin HD, eds. Head and neck imaging. 4th ed. St. Louis: Mosby–Elsevier Science; 2003 Mafee MF. Uveal melanoma, choroidal hemangioma, and simulating lesions. Role of MR imaging. Radiol Clin North Am 1998; 36:1083-1099 Margo CE, Katz NN, Wertz FD, Dorwart RH. Sclerosing endophthalmitis in children: computed tomography with histopathologic correlation. J Pediatr Ophthalmol Strabismus 1983;20:180-184 Pendergrass TW, Davis S. Incidence of retinoblastoma in the United States. Arch Ophthalmol 1980; 98:1204-1210. Reese AB. Telangiectasis of the retina and Coats' disease. Am J Ophthalmol 1956; 42:1-8. Rubin RM, Sadun AA. Ocular myopathies. In: Yanoff M, Duker JS, eds. Ophthalmology. St. Louis: Mosby; 1999. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954; 17:300-302. Trokel SL, Hilal SK. Recognition and differential diagnosis of enlarged extraocular muscles in computed tomography. Am J Ophthalmol 1979; 87:503-512. Weber AL, Romo LV, Sabates NR. Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin North Am 1999; 37:151-168, xi. Wilder HC. Nematode endophthalmitis. Trans Am Acad Ophthalmol Otolaryngol 1950:99-109. Yanoff K, Fine BS, Ocular Pathology . Hagerstown: Harper & Row, 1975.

Imaging of the Orbit: The Globe and Conal Lesions

1094 1096

Neuroradiology

Imaging of the Orbit: Intraconal and Extraconal Lesions Kelly K. Koeller, MD, FACR Figure 5-7-1

Intraconal Lesions • • • • • • •

Optic nerve glioma Optic nerve sheath meningioma Cavernous hemangioma Schwannoma Lymphoma Fibrous histiocytoma Varix, arteriovenous malformation

Optic Nerve Glioma • • •

• • •

3% of all orbital tumors; 4% of gliomas Peak age: 2–8 years (range: birth to 60 years) 50% associated with neurofibromatosis type 1 (NF-1) and frequently bilateral ➢ 10%–15% of all NF-1 cases Optic atrophy on ophthalmoscopy Arise from glial cells of optic nerve Slow growth usually; may grow in spurts

Azar-Kia et al, Radiol Clini North Am 1987; 25:561-581

Optic Nerve Glioma • • • •

• •

Optic nerve glioma with characteristic "kinking" of the nerve

[Figure 5-7-1]

Juvenile: pilocytic astrocytoma Adult: glioblastoma multiforme (non-NF-1 cases) Fusiform enlargement: kinking, buckling CT: iso- to hypodense ➢ Calcification rare T1WI: hypointense, T2WI: hyperintense Heterogeneous enhancement

Azar-Kia et al, Radiol Clin North Am 1987; 25:561-581; Haik et al, Ophthalmology 1987; 94:709-717

Figure 5-7-2

Optic Nerve Sheath Meningioma • • • •

5% of all orbital tumors Less than 1% of all meningiomas “Extradural” meningiomas: associated with “blistering” of adjacent bone Slowly progressive loss of vision, proptosis ➢ Optociliary venous shunts, disk pallor, visual loss: highly suggestive

Sibony et al, Ophthalmology 1984; 11:1313-1326

Optic Nerve Sheath Meningioma • • • • •

•

[Figure 5-7-2]

Well-defined tubular thickening of ON CT: Calcification common T1WI: Isointense to ON T2WI: Iso- to hypoattenuated “Tram-track” enhancement ➢ Fat suppression essential May be eccentric, extend intracranially

Optic nerve sheath meningioma with tram-track enhancement (upper Daniels et al, AJNR 1982: 3:181-183; Azar-Kia et al, Radiol Clin North image) and calcification (lower image) Am 1987; 25:561-581 in 2 different patients Neuroradiology

1097

Imaging of the Orbit: Intraconal and Extraconal Lesions

Cavernous Hemangioma • • •

•

•

• •

[Figure 5-7-3]

Most common vascular orbital tumor in adults Peak age: 25-40 y/o (range 25-70) Well-circumscribed mass (pseudocapsule) ➢ Intraconal (83%) Benign non-infiltrative hamartoma ➢ Large dilated sinusoid-like spaces ➢ Slowly progressive enlargement ➢ Prominent arterial supply usually absent CT ➢ Hyperattenuated ➢ Phleboliths ➢ Bone remodeling ➢ Variable enhancement MR ➢ T1WI: mixed ➢ T2WI: iso-to-hyperintense Hemorrhage occasionally

Bilaniuk, Radiol Clin North Am 1999; 37:169-183; Mafee et al, Radiol Clin North Am 1987; 25:529-559

Figure 5-7-3

Cavernous hemangioma with pressure erosion of orbital roof

Schwannoma •

• • •

1% of all orbital tumors: usually intraconal ➢ Arise from cranial nerves, not optic nerve ➢ Isolated or neurofibromatosis association Benign with slow growth ➢ Well-encapsulated Painless proptosis Compresses or engulfs optic nerve

Carroll et al, Radiol Clin North Am 1999; 37:195-202

Schwannoma • •

• • •

[Figure 5-7-4]

Fusiform to oval-shaped mass CT: Isoattenuated to extraocular muscles ➢ More hyperdense areas = Antoni A cells T1WI: Iso- to hypodense T2WI: Hyperintense Marked enhancement

Figure 5-7-4

Orbital Lymphoma •

• • • •

Lymphoid tumors: 10%-15% of orbital masses ➢ Lymphoma, pseudolymphoma, lymphoid hyperplasia 10% of all lymphomas as primary site ➢ 75% have or will have systemic lymphoma ➢ Lacrimal gland: most common site ➢ EOMs rarely involved Non-Hodgkin’s (B-cell): majority Proptosis, ptosis, diplopia Rubbery firm masses

Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et al, Radiol Clin North Am 1997; 25:601-612;

Schwannoma Imaging of the Orbit: Intraconal and Extraconal Lesions

1096 1098

Neuroradiology

Orbital Lymphoma •

• • • • •

Figure 5-7-5

[Figure 5-7-5]

Molds itself along margins of normal structures ➢ Bone erosion: late finding Usually well-defined, round to oval CT: homogeneous, mildly hyperattenuated T1WI: hypointense T2WI: iso-to-hypointense Mild to marked enhancement

Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et al, Radiol Clin North Am 1997; 25:601-612;

Fibrous Histiocytoma • • • •

• •

[Figure 5-7-6]

Most common primary orbital mesenchymal tumor in adults 1% of all primary orbital tumors Mean age: 42 y/o Usually benign (66%) ➢ Malignant: bone erosion, hemorrhage, post-radiation therapy for retinoblastoma Well-defined intra-or extraconal mass Moderate to marked enhancement

Font and Hidayat, Hum Pathol 1982; 13:199; Mafee in Valvassori, Mafee, and Carter, Imaging of the Head and Neck, Thieme, 1995

Orbital Varix • • •

• •

Orbital lymphoma with characteristic molding of the tumor against the orbital globe

[Figure 5-7-7]

Most common cause of spontaneous orbital hemorrhage Focal venous dilatation Valsalva: stress proptosis ➢ Lobulated mass Phleboliths Spontaneous thrombosis common

Figure 5-7-6

Bilaniuk, Radiol Clin North Am 1999; 37:169-183

Figure 5-7-7 Malignant fibrous histiocytoma

Orbital varix with enlargement upon Valsalva maneuver on post-contrast image (bottom) Neuroradiology

1097 1099

Imaging of the Orbit: Intraconal and Extraconal Lesions

Arteriovenous Fistula •

• • • •

Figure 5-7-8

[Figure 5-7-8]

Usually post-trauma ➢ Spontaneous less common (Ehlers-Danlos, osteogenesis imperfecta, pseudoxanthoma elasticum) Orbital bruit, proptosis, chemosis CT/MR: dilated superior ophthalmic vein Angiography diagnostic Endovascular occlusion: treatment of choice

Tan et al, Radiol Clin North Am 1987; 25:849-861

Extraconal Lesions • • • • •

Lymphangioma* Metastasis* Rhabdomyosarcoma* Dermoid/epidermoid Paranasal sinus disease ➢ Infection ➢ Neoplasm

*commonly “intercompartmental”

Lymphangioma • • •

• • •

Carotid-cavernous fistula with enlarged superior ophthalmic vein. Lateral view from cerebral angiogram shows abnormal flow through cavernous sinus, petrosal sinuses, and superior ophthalmic vein

[Figure 5-7-9]

Children and young adults Exophthalmos with viral infection Lymphoid follicles, dilated spaces ➢ Infiltrative; do not respect fascial planes ➢ Hemorrhage common Extraconal space primarily CT/MR: Heterogeneous appearance ➢ Hemorrhage or cystic fluid Variable enhancement (venous channels)

Bilaniuk, Radiol Clin North Am 1999; 37:169-183; Mafee et al, Radiol Clin North Am 1987; 25:529-559

Figure 5-7-9 Figure 5-7-10

Lymphangioma with hemorrhage in 2 different patients

Metastatic Lesions •

• •

[Figure 5-7-10]

10% of orbital tumors ➢ 1/3- bony orbit, 1/3- globe, 1/3- scattered Increasing incidence (longer survival) Primary site ➢ Breast: 42% ➢ Lung: 11% ➢ Unknown primary: 11% ➢ Prostate: 8% ➢ Melanoma: 5% ➢ Children: neuroblastoma, leukemia, Ewing’s

Imaging of the Orbit: Intraconal and Extraconal Lesions

Orbital metastasis from unknown primary neoplasm 1098 1100

Neuroradiology

Metastatic Lesions • • • • •

Figure 5-7-11

9 months average survival (lung carcinoma, melanoma worst) Hematogenous spread Diplopia, proptosis, pain, vision loss Enophthalmos: breast carcinoma Isolated lateral rectus enlargement metastasis or pseudotumor

Rhabdomyosarcoma [Figure 5-7-11] •

• •

Most common primary orbital malignancy tumor in children ➢ Most: 2-5y/o; 90% younger than 16 y/o ➢ Rapidly progressive but usually painless Arise from undifferentiated mesenchyme in orbital fat (not from extraocular muscles) ➢ Children: embryonal (70%) and alveolar types ➢ Adults: pleomorphic type 90% 5-year survival with complete resection ➢ 35% if significant residual disease

Rhabdomyosarcoma with characteristic bone destruction

Mafee et al, Radiol Clin North Am 1998; 36:1215-1227;

Rhabdomyosarcoma • • •

• • •

Figure 5-7-12

Superior orbit predilection Homogeneous mass CT: isoattenuated to muscle ➢ Bone destruction common in larger lesions ➢ Necrosis, calcification, hemorrhage uncommon T1WI: hypointense T2WI: hyperintense Moderate to marked enhancement

Mafee et al, Radiol Clin North Am 1998; 36:1215-1227

Dermoid / Epidermoid [Figure 5-7-12] •

• • • •

Most common congenital orbital lesion ➢ Many manifest in 2nd-3rd decades Superolateral: most comon location Arise at sutures or diplöe Well-defined mass with fat or fluid signal in upper corners of orbit Remodel bone without destruction

Kaufman et al, Radiol Clin North Am 1998; 36:1149-1163

Orbital Cellulitis [Figures 5-7-13 and 5-7-14] •

• •

Dermoid

Classification ➢ Pre-septal cellulitis: eyelid ➢ Post-septal cellulitis ➢ Subperiosteal phlegmon and abscess ➢ Cavernous sinus thrombosis Usually paranasal (ethmoid) sinusitis Usually does not extend into intraconal space

Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am 1998; 36:1165-1183

Neuroradiology

1099 1101

Imaging of the Orbit: Intraconal and Extraconal Lesions

Figure 5-7-14

Figure 5-7-13

Orbital cellulitis. Note involvement along lamina papyracea

Peri-orbital cellulitis

Fungal sinusitis [Figure 5-7-15] •

•

Immunocompromised patients ➢ Rhino-orbital mucormycosis ➢ Aspergillosis Reversal of typical findings in sinus disease ➢ Increasing protein, decreasing water content ➢ CT: hyperattenuated ➢ T1WI: hyperintense ➢ T2WI: hypointense (can mimic air)

Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am 1998; 36:1165-1183

Lacrimal Gland Lesions •

• •

50% inflammatory/lymphoproliferative ➢ Sarcoid ➢ Sjogren’s ➢ Lymphoma: frequent anterior/posterior extension ➢ Pseudotumor: 15% of all orbital pseudotumor 50%: epithelial tumors ➢ 50%: benign (pleomorphic adenoma, benign mixed cell tumor) ➢ 50%: malignant (adenoid cystic, malignant mixed, mucoepidermoid, adeno, squamous cell, anaplastic) Imaging: pre-op planning

Figure 5-7-15

Zimmerman et al, Int Ophthalmol Clin 1962; 2:337-367; Mafee et al, Radiol Clin North Am 1987; 25:767-779

Fungal sinusitis

Imaging of the Orbit: Intraconal and Extraconal Lesions

1100 1102

Neuroradiology

Lacrimal Gland Lesions [Figure 5-7-16 and 5-7-17] •

•

Figure 5-7-16

Inflammatory lesions ➢ Oblong mass ➢ Molded enlargment of lacrimal gland Pleomorphic adenoma ➢ Long duration ➢ Rounded mass ➢ Bone remodeling ➢ Bone destruction: malignant epithelial tumors

Jakobiec et al, Am J Ophthalmol Clin 1962; 2:337-367

Lacrimal Sac Lesions •

•

[Figure 5-7-18]

Malignant: 57% ➢ Epithelial: 75% ❖ Squamous cell ❖ Transitional cell ❖ Mucoepidermoid ➢ Mesenchymal: fibrous histiocytoma ➢ Lymphoid: lymphoma ➢ Neural ➢ Metastasis Benign: 43% ➢ Diverticulum ➢ Pneumatocele ➢ Mucocele ➢ Papilloma ➢ Polyp ➢ Fibroma ➢ Dermoid

Lacrimal lymphoma

Figure 5-7-17

Stefanyszyn et al, Ophthal Plast Reconstr Surg 1994; 10:169-184; Pe’er et al, Ophthalmology 1996; 103:1601-1605

Figure 5-7-18 Lacrimal pleomorphic adenoma

Lacrimal sac sarcoma with bone destruction

Neuroradiology

1101 1103

Imaging of the Orbit: Intraconal and Extraconal Lesions

Summary • • • •

•

•

•

•

Retinoblastoma: most common intraocular malignancy of childhood Uveal melanoma: most common malignancy of the globe in adults Uveal metastasis: frequently bilateral Most common diseases of the orbit ➢ 1. Graves: no tendon involvement ➢ 2. Lymphoma ➢ 3. Pseudotumor: involves tendon Intraconal lesions ➢ Optic nerve tumors ❖ Glioma ❖ Nerve sheath meningioma ➢ Cavernous hemangioma ➢ Nerve sheath tumors ➢ Lymphoma ➢ Fibrous histiocytoma ➢ Varix ➢ Carotid-cavernous fistula Extraconal lesions ➢ Lymphangioma ➢ Metastases: 10% of orbit masses ➢ Rhabdomyosarcoma ➢ Dermoid ➢ Sinus disease Lacrimal gland lesions ➢ “The 50% gland” ➢ 50% inflammatory/lymphoproliferative ➢ 50% neoplasms ❖ 50% benign, 50% malignant Lacrimal sac lesions: most are malignant

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

Azar-Kia B, Naheedy MH, Elias DA, Mafee MF, Fine M. Optic nerve tumors: role of magnetic resonance imaging and computed tomography. Radiol Clin North Am 1987; 25:561-581. Bilaniuk LT. Orbital vascular lesions. Role of imaging. Radiol Clin North Am 1999; 37:169-183, xi. Carroll GS, Haik BG, Fleming JC, Weiss RA, Mafee MF. Peripheral nerve tumors of the orbit. Radiol Clin North Am 1999; 37:195-202, xi-xii. Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis of orbital complications in acute sinusitis. Laryngoscope 1970; 80:1414-1428. Daniels DL, Williams AL, Syvertsen A, Gager WE, Harris GJ. CT recognition of optic nerve sheath meningioma: abnormal sheath visualization. AJNR Am J Neuroradiol 1982; 3:181-183. Eustis HS, Mafee MF, Walton C, Mondonca J. MR imaging and CT of orbital infections and complications in acute rhinosinusitis. Radiol Clin North Am 1998; 36:1165-1183, xi. Flanders AE, Espinosa GA, Markiewicz DA, Howell DD. Orbital lymphoma. Role of CT and MRI. Radiol Clin North Am 1987; 25:601-613. Font RL, Hidayat AA. Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol 1982; 13:199-209. Haik BG, Saint Louis L, Bierly J, et al. Magnetic resonance imaging in the evaluation of optic nerve gliomas. Ophthalmology 1987; 94:709-717. Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal fossa lesions. Am J Ophthalmol 1982; 94:785-807. Kaufman LM, Villablanca JP, Mafee MF. Diagnostic imaging of cystic lesions in the child's orbit. Radiol Clin North Am 1998; 36:1149-1163, xi. Mafee MF, Haik BG. Lacrimal gland and fossa lesions: role of computed tomography. Radiol Clin North Am 1987; 25:767-779. Mafee MF, Pai E, Philip B. Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT. Radiol Clin North Am 1998; 36:1215-1227, xii. Mafee MF, Putterman A, Valvassori GE, Campos M, Capek V. Orbital space-occupying lesions: role of computed tomography and magnetic resonance imaging. An analysis of 145 cases. Radiol Clin North Am 1987; 25:529-559.

Imaging of the Orbit: Intraconal and Extraconal Lesions

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15. Mafee MF. Imaging of the orbit. In: Valvassori GE, Mafee MF, Carter BL, eds. Imaging of the head and neck. New York: Thieme, 1995; 302-328 16. Pe'er J, Hidayat AA, Ilsar M, Landau L, Stefanyszyn MA. Glandular tumors of the lacrimal sac. Their histopathologic patterns and possible origins. Ophthalmology 1996; 103:1601-1605. 17. Sibony PA, et al: Optic Nerve Sheath Meningiomas. Ophthalmology 1984, 91(11): 1313-1326. 18. Stefanyszyn MA, Hidayat AA, Pe'er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994; 10:169-184. 19. Tan WS, Wilbur AC, Mafee MF. The role of the neuroradiologist in vascular disorders involving the orbit. Radiol Clin North Am 1987; 25:849-861. 20. Valvassori GE, Sabnis SS, Mafee RF, Brown MS, Putterman A. Imaging of orbital lymphoproliferative disorders. Radiol Clin North Am 1999; 37:135-150, x-xi. 21. Zimmerman LE, Sanders TE, Ackerman LV. Epithelial tumors of the lacrimal gland: prognostic and therapeutic significance of histologic types. Int Ophthalmol Clin 1962; 2:337-367.

Neuroradiology

1103 1105

Imaging of the Orbit: Intraconal and Extraconal Lesions

Patterns of Location: Infratentorial and Supratentorial James G. Smirniotopoulos, MD PATTERN ANALYSIS •

Basic Approach ➢ Where is the lesion ? ❖ Intraaxial ❖ Extraaxial ❖ Intraventricular ➢ Where is the lesion ? ❖ Supratentorial ❖ Infratentorial ➢ How old is the patient ? ❖ Child ❖ Adult ➢ What about Sex ?

INTRA-AXIAL • • • • • • • • • • •

Cortex Gray-white Junction Deep White Matter Deep Gray Matter Glioma Medulloblastoma Hemangioblastoma Metastases Infarct/hematoma AVM/congenital Abscess/inflammation

EXTRA-AXIAL LESIONS • • • • • • • • • • • • •

Subarachnoid Subdural Epidural Calvarium (Skull Base) Subgaleal Scalp (Soft-tissues) Meningioma Pituitary adenoma Craniopharyngioma Schwannoma Chordoma Dermoid/epidermoid, cyst, lipoma Hematoma, metastasis, infection

BASIC APPROACH CLASSIC LOCATIONS • • • • • • • • •

Foramen magnum Cerebellopontine angle (CPA) Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Cranial Nerves • • • • • • • •

Figure 5-8-1

Olfactory (I) Optic (II) Oculomotor (III) Trochlear (IV) Trigeminal (V) Abducens (V) Facial (VII) Vestibulocochlear (VIII)

When looking into the IAC (internal auditory canal) notice that 7 is UP and Coke (cochlear) is DOWN

Internal Auditory Canal • • • •

S – Schwannoma (8th >> > 5th) A – aneurysm, arachnoid cyst M – meningioma, mets E – epidermoid, ependymoma, CPP

• • • •

S – Schwannoma (8th >> > 5th) A – aneurysm, arachnoid cyst M – meningioma, mets E – epidermoid, ependymoma, CPP

[Figures 5-8-1 and 5-8-2]

CPA MASSES Differential Figure 5-8-2

CPA MASSES Demographics • • •

7/9 (Schwannoma, 8th > > 5th) 1/9 Meningioma (tentorial/petrous) 1/9 “Other”: ➢ Epidermoid Cyst (1/18) ➢ Mets, aneurysm, etc. ➢ Glioma (ependymoma, CPP) ➢ Arachnoid cyst ➢ Cystadenoma of endolymph ➢ Glomus tumor Vestibular Schwannoma begins as an intracanalicular mass; then it grows out of the canal into the cerebellopontine angle cistern

Intracanalicular Schwannoma [Figure 5-8-3]

Vestibular Schwannoma Figure 5-8-3

[left] T2W image shows CSF, normal nerve, and round mass. [right] T1WGd image shows enhancement of mass. The normal 7th and 8th nerves do NOT enhance in this location

Neuroradiology

1105 1107

Patterns of Location: Infratentorial and Supratentorial

Young Schwannoma – Old Schwannoma [Figure 5-8-4] •

Figure 5-8-4

Benign Cystic Degeneration

Vestibular Schwannoma •

• •

•

IAC origin ➢ IAC involved ➢ IAC Enlarged (70%) Spherical Mass ➢ encapsulated Heterogeneous if large ➢ > 20 mm Enhance “always”

Trigeminal Schwannoma [Figure 5-8-5] Figure 5-8-5

The larger – and older – Schwannoma is heterogeneous due to benign cystic degeneration

Figure 5-8-6

Trigeminal Schwannoma may present as a “dumbbell” mass, bilobed, with one lobe in the cavernous sinus and one in the poster fossa – lateral pontine cistern

Bilateral Vestibular Schwannoma

This mass is hemispheric – but does not extend into the canal. [Courtesy of Bob Peyster, MD]

Meningioma Hyperostosis Meningioma

Figure 5-8-7 Tentorial Meningioma Meningioma [Figures 5-8-6 and 5-8-7] • • • • •

•

Tentorium or Dura IAC Normal Hemispherical Enhance Homogeneous Hyperostosis ➢ 15%-40% Dural Tail ➢ 70%-90%

[left] T1W image – no enhancement, undulating (wavy) margin. Note the wispy internal structure. [right] T2W image – isointense to CSF Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Epidermoid vs. Arachnoid Cyst •

•

CLASSIC LOCATIONS • • • • • • • • •

Figure 5-8-8

Epidermoid Inclusion Cyst [Figure 5-8-8] ➢ CPA most common ➢ Extraaxial CPA Lesion ➢ IAC Normal ➢ Undulating Margin ➢ CSF - ‘like’ ❖ Not identical ➢ NO Enhancement ➢ Wispy internal structures Arachnoid Cyst ➢ Middle fossa common ➢ Extraaxial CPA Lesion ➢ IAC Normal ➢ Rounded Mass ➢ Identical to H2O on CT and all MR sequences ❖ T1, PD, T2, FLAIR, DWI, ADC ➢ NO Enhancement ➢ NO ‘structure’

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

This epidermoid inclusion cyst is only isointense to CSF on the T2W image

Figure 5-8-9

Central Posterior Fossa Lesion • • • •

Could be Intraaxial Could be Intraventricular Could be extending from vermis into ventricle Could be extending from ventricle into vermis

Fourth Ventricle - Schematic [Figure 5-8-9] Central Masses [Figure 5-8-10]

Schematic of central posterior fossa mass: Did it begin in the 4th ventricle, in the medullary velum, or in the cerebellum?

CHILD - CEREBELLAR/IVth • • • •

Medulloblastoma (PNET) Astrocytoma (usu. Pilocytic) Ependymoma Post fossa cysts

Figure 5-8-10

ADULT - CEREBELLAR/IVth • • • •

•

Metastasis Hemangioblastoma Hemorrhage, infarct Glioma ➢ Ependymoma ➢ Astrocytoma Abscess

Schematic diagram of medulloblastoma and ependymoma. Copyright 2005 Neuroradiology

1107 1109

Patterns of Location: Infratentorial and Supratentorial

Medulloblastoma [Figures 5-8-11 and 5-8-12] •

• • • •

Figure 5-8-11

‘Homogeneous’ ➢ finely irregular Cyst and Hemorrhage are uncommon <10% Hyperdense on NCT ➢ up to 75% ➢ densely cellular ➢ sm. Round blue-cells Center is behind 4th vent Rounder not angular

Pilocytic Astrocytoma •

[Figures 5-8-13 to 5-8-16]

• • • • • •

Cyst and Mural Nodule ➢ balanced morphology Wall may not enhance Cyst fluid with protein Nodule low density on CT ➢ may calcify up to 25% No increase in vascularity WHO Grade 1 Peak at ~10 yrs

Medulloblastoma, hyperdense on plain CT

Figure 5-8-12

Figure 5-8-13

Pilocytic astrocytoma: Classic cyst-with-nodule morphology Medulloblastoma

Figure 5-8-14 Figure 5-8-15

Pilocytic astrocytoma: Classic cyst-with-nodule morphology

Pilocytic astrocytoma: Classic cyst-with-nodule morphology

Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Good Rules for Practice [Figure 5-8-17] • • • • • • •

Figure 5-8-16

Cerebellum GBM is uncommon Pediatric GBM is uncommon If I am wrong … What’s the worst that it could be? What’s the best that it could be? The Probability of tandem events occurring is the product of multiplying the chance of the individual events. E.G. 1/1000 x 1/1000 = 1/million Uncommon X Uncommon = RARE

Hemangioblastoma [Figures 5-8-18 and 5-8-19] •

• • • • • • • •

Cyst and Mural Nodule ➢ some solid ➢ some nearly pure cyst Wall may not enhance Cyst fluid has protein Nodule high density No Ca++ Increased vascularity ➢ Flow void ➢ Blood products WHO Grade 1 Peak at ~ 35 yrs Multiple in VHL

Pilocytic astrocytoma: Classic cystwith-nodule morphology. Copyright 2005

Figure 5-8-17

Figure 5-8-18

What is this? – Come to the lecture and find out!

Figure 5-8-19 Hemangioblastoma may be a cyst and nodule in about 1/3 of cases. Solid forms, and almost completely “cystic” types occur

Hemangioblastoma may be a cyst and nodule in about 1/3 of cases. Solid forms, and almost completely “cystic” types occur. Copyright 2005 Neuroradiology

1109 1111

Patterns of Location: Infratentorial and Supratentorial

Expansile Mass in Brainstem

Figure 5-8-20

[Figure 5-8-20]

Intraluminal Mass of IVth [Figure 5-8-21]

Figure 5-8-21

Expansile lesion of the pons and medulla, encroaching on the 4th ventricle, does not enhance

Figure 5-8-22

Ependymoma, arising from the 4th ventricle floor, and remains within the lumen of the ventricle

Ependymoma - Schematic [Figure 5-8-22] Ependymoma [Figures • • •

• •

5-8-23 and 5-8-24]

Intraventricular “Soft” plastic lesion Angular extensions ➢ Luschka ➢ Magendie Heterogeneous ➢ Cystic areas ➢ Chunks of Ca++ Arise from floor of 4th

Schematic of ependymoma Copyright 2005

Figure 5-8-24

Figure 5-8-23

Ependymoma. Copyright 2005

Ependymoma Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Posterior Fossa Masses [Figure 5-8-25]

Figure 5-8-25

Lhermitte-Duclos [Figure 5-8-26] CLASSIC LOCATIONS • • • • • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial Schematic Localization of posterior fossa masses. Copyright 2005

SELLA/PARASELLAR REGION •

Differential: ➢ Pituitary adenoma ➢ Craniopharyngioma ➢ Aneurysm (ICA , etc.) ➢ Meningioma ➢ Optic/hypothalamic glioma ➢ Chordoma ➢ Granuloma, e.g., hamartoma, cyst(arachnoid, dermoid/epi) ➢ Germ Cell (Germinoma)

Figure 5-8-26

Pituitary Adenoma [Figure 5-8-27] • •

•

Adult Patient Microadenoma ➢ < 10 mm ➢ Entirely within gland ➢ Endocrine Sx ❖ Prolactinoma ❖ Acromegaly ❖ Gigantism ❖ Cushing Disease Macroadenoma ➢ > 10 mm ➢ balloon sella ➢ Visual Sx ❖ if >6 mm above sella ❖ bitemporal hemianopsia

Lhermitte-Duclos. T1 and T2 weighted images show a striated or “courduroy” appearance, classic for dysplastic gangliocyoma

Figure 5-8-27

Elevated Prolactin •

• • •

• •

Microadenoma ➢ < 10mm diameter ➢ Entirely intrasellar Macroadenoma ➢ > 10 mm Stalk Effect ➢ Blocks Prolactin Inhibitory Factor ➢ 40-150 ng PRL vs. 28 for nl. Hypothyroidism ➢ “Cross Reaction” from TSH Exogenous Pharmacologic Pituitary macroadenoma with hyperintensity from old hemorrhage

Neuroradiology

1111 1113

Patterns of Location: Infratentorial and Supratentorial

Macroadenoma [Figure 5-8-28]

Figure 5-8-28

Sella and Suprasellar [Figure 5-8-29] Craniopharyngioma – 2 Types •

•

Child ➢ Adam Ant – inomatous ❖ enamel organ of tooth ➢ Commonly Cystic ➢ ‘Machine Oil’ ➢ Commonly Calcified ➢ Adherent to brain ❖ pilocytic astrogliosis Adult ➢ Squamous and Papillary ➢ Commonly Solid ➢ Calcification less common ➢ Easier to resect

Pituitary macroadenoma with hyperintensity from old hemorrhage

Craniopharyngioma [Figure 5-8-30] Figure 5-8-29 Figure 5-8-30

Craniopharyngioma, you barely see how the hypothalamus is draped over the top of the mass

Craniopharyngioma, expansile remodeling of sella turcica

Craniopharyngioma “Machine Oil”

Figure 5-8-31

Craniopharyngioma [Figure 5-8-31]

Craniopharyngioma

Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Where is the Hypothalamus ? •

Figure 5-8-32

[Figure 5-8-32]

Hypothalamic Glioma – Pilocytic astrocytoma

Where is the Clivus? • • • •

[Figure 5-8-33]

Chordoma: Notochord rests Midline Bone destruction

Figure 5-8-33

Hypothalamic Glioma – Pilocytic astrocytoma

Figure 5-8-34

Chordoma of the clivus Schematic of location of remnant notochord tissue. Copyright 2003

Notochord: Chordoma & Thornwaldt [Figure 5-8-34]

SELLA/PARASELLAR •

Figure 5-8-35

Differential Features: ➢ ADULT – Pituitary adenoma ➢ CHILD – Craniopharyngioma or Glioma (hypothalamus or optic ) > EG, etc ➢ SELLA NORMAL – NOT pituitary ➢ Ca++ – Craniopharyngioma, but... ➢ HYPEROSTOSIS – Meningioma ( exp. “blistering” ) ➢ CLIVUS – Chordoma, mets, NP Ca ➢ Remember – rule out vascular lesions (aneurysms)

Pulsation Artifact: Phaseencoding direction [Figure 5-8-35]

Giant (> 2.5 cm) cerebral aneurysm

CLASSIC LOCATIONS • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle

Neuroradiology

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Patterns of Location: Infratentorial and Supratentorial

• • • •

Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Figure 5-8-36

Colloid Cyst [Figure 5-8-36] •

Hydrocephalus: Vents > Sulci

THIRD VENTRICLE [Figure 5-8-37] •

Differential: ➢ Colloid cyst ➢ Cysticercosis ➢ Craniopharyngioma ➢ Hypothalamic and thalamic glioma ➢ CPP, ependymoma ➢ Neurocytoma ➢ Basilar tip aneurysm

Colloid cyst

Figure 5-8-37

Colloid Cyst [Figure 5-8-38 to 5-8-40] Figure 5-8-38

Colloid cyst

Differential Diagnosis – 3rd ventricle. Copyright 2005

Figure 5-8-39 Figure 5-8-40

Colloid cyst. T1-weighted image shows slight hyperintensity before gadolinium and no definite enhancement

Patterns of Location: Infratentorial and Supratentorial

Colloid Cyst. Marked hypointensity on T2W image

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Neuroradiology

HYDROCEPHALUS •

• • •

Figure 5-22-41

Differential Diagnosis: ➢ Over production of CSF (CPP) ➢ Obstruction of CSF flow: ❖ Obstructive/internal hydrocephalus ❖ Communicating/external hydrocephalus Under reabsorption of CSF: SAH Compensatory: Ex vacuo/enlargement

CSF Homeostasis [Figure 5-8-41] Normal Ventricular System [Figure 5-8-42] Schematic of CSF Homeostasis Copyright 2004

Figure 5-22-42

Schematic of Normal Ventricular System Copyright 2004

Foramen of Monro Obstruction Aqueduct Obstruction Non-traumatic hemorrhage [Figures 5-8-43 and 5-8-44]

Figure 5-22-44

Figure 5-22-43

Non-traumatic hemorrhage in the right thalamus. Copyright 2006

Non-traumatic hemorrhage in the right thalamus

Neuroradiology

1115 1117

Patterns of Location: Infratentorial and Supratentorial

Hypertensive Hemorrhage

Figure 5-8-45

Hemorrhage into a mass •

NOTE: Vasogenic Edema

ARTERIOLOSCLEROSIS

What do they have in Common? [Figure 5-8-45] • • • • • •

Multiple Bilateral Symmetric Anatomic Basal ganglia Toxic and/or Metabolic: ➢ Acquired ➢ Congenital

CT – medial lenticular lesion – Globus Pallidus. MR – lateral lenticular lesion – Putamen

CO Poisoning [Figure 5-8-46]

Figure 5-8-46

MetOH Intoxication Tx for MetOH - Fomepazole • • •

Fomepazole (Antizole, 4-methylperazole) is a synthetic alcohol dehydrogenase inhibitor for IV administration Clear yellow liquid, mw 82.1, mp 25º C (77º F) INDICATIONS: Antidote for ethylene glycol, or methanol poisoning of suspected EG ingestion ➢ PRECAUTIONS: Dilute in > 100 mL NS, follow hepatic enzymes & WBC (eos) during Rx, interaction with ethanol (compete for ADH) ➢ DOSE: 15 mg/kg load, 10 mg/kg Q 12 h x 4 doses, then 15 mg/kg Q 12 h till EG < 20 mg/dL

Deep Lesions •

•

White Matter: ➢ Leukoencephalopathy ➢ “Bad White Matter Disease” ➢ Small vessel disease ➢ Hypertension ➢ Glial Neoplasm ➢ Astrocytoma (incl. GBM) ➢ Oligodendroglioma Deep White and Gray Matter ➢ Lymphoma ➢ Toxoplasmosis Both occur in HIV/AIDS, multiple lesions

Carbon monoxide toxicity. Notice there are bilateral medial lenticular (globus pallidus) signal abnormalities – hypointense on T1 and hyperintense on T2

Figure 5-8-47

Solitary Deep Lesion - Thalamus [Figure 5-8-47] Glioblastoma – WHO Grade 4 •

A solitary, deep, irregular, heterogeneous, ring-enhancing mass with vasogenic edema

Glioblastoma multiforme of the thalamus and temporal lobe Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Glioblastoma Multiforme [Figure 5-8-48]

Figure 5-8-48

Toxoplasmosis [Figure 5-8-49] •

2 patients with typical lesions

BASAL GANGLIA THALAMUS: •

• •

BILATERAL SYMMETRIC (toxic/metabolic): ➢ PUTAMEN – Methanol ➢ GLOBUS PALLIDUS – CO Poisoning BILATERAL ASYMMETRIC (hematogenous): ➢ INFECTION (TOXO, etc.) UNILATERAL (acquired/neoplastic): ➢ THALAMIC GLIOMA (astrocytoma) ➢ HYPERTENSIVE HEMATOMA (exclusion)

T2 - T1gad Glioblastoma multiforme with extensive vasogenic edema

CLASSIC LOCATIONS • • • • • • • • • •

Figure 5-8-49

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle Pineal Region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Patterns in Neuroradiology • • • • • • • • •

Cerebello-Pontine Angle Fourth Ventricle/Cerebellum Sella/Parasellar Basal Ganglia/Third Ventricle Lateral Ventricle Pineal Region Deep Hemispheric/Periventricular Cortical/Subcortical Convexity Extraaxial

Intraventricular Neoplasms • • • • • • • • •

Ependymoma (and subependymoma) Choroid plexus papilloma Subependymal giant cell astro. Meningioma Colloid cyst (3rd) Medulloblastoma (4th) Dermoid/epidermoid Central neurocytoma Mets, lymphoma, Germ Cell

Toxoplasmosis

Subependymal Giant Cell Astro

Neuroradiology

(From Vince Mathews, M.D. IU)

1117 1119

Patterns of Location: Infratentorial and Supratentorial

Lateral Ventricle @ f. Monro •

Figure 5-8-50

Lateral Ventricle/caudate ➢ Subependymal Giant Cell Astro. ❖ TUBEROUS SCLEROSIS, Enhances & Ca++ ➢ Subependymoma ❖ Variant of Ependymoma ❖ No Ca++, no enhancement ➢ Central Neurocytoma ❖ Septum pellucidum ➢ Cyst/Cavum septum pellucidum ➢ Huntington Chorea ❖ Atrophy

Lateral ventricular masses [Figure 5-8-50] Lateral Ventricle - Trigone • • • • • •

Schematic of lateral ventricular masses. Copyright 2005

Meningioma Choroid Plexus Papilloma Xanthogranuloma Metastasis Lipoma Choroid Cyst

Trigone or Atrium Trapped Temporal Horn Choroid Plexus Papilloma • •

• •

• • •

Attached to normal Choroid Plexus Lobulated ➢ Fronds ➢ Papillae Trigone of lateral vent ➢ Children Fourth ventricle ➢ Adults Third ventricle CPA cistern Hydrocephalus ➢ Obstruction ➢ Production =/= Resorption

CSF Overproduction? Central Neurocytoma •

Central ➢ Often centered on septum pellucidum ➢ Extension into both lateral ventricles ➢ Hyperdense on CT ➢ Gray matter on MR ➢ Spontaneous Bleed ➢ Calcifications

CLASSIC LOCATIONS • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle

Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

• • • •

Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Figure 5-8-51

Pineal/Quadrigeminal Cistern Region •

• •

•

“Pinealomas” ➢ Germ cell tumors ❖ Seminoma ❖ Teratoma Pineal cell tumors ➢ Pineoblastoma ➢ Pineocytoma Gliomas (regional) ➢ Brainstem, callosum, thalamus Other ➢ Dermoid, lipoma, arachnoid cyst ➢ Meningioma ➢ Vein of Galen malformations

Pineal region mass. Germinoma. Copyright 2005

Figure 5-8-52

Pineal Region Mass [Figure 5-8-51] Germinoma [Figure 5-8-52] •

• • • • • •

Central ➢ Pineal Region ➢ Suprasellar Cistern Homogeneous Hyperdense to GM Isointense to GM Uniform Enhancement CSF Seeding ? May ENGULF Pineal Ca++ Pineal region Germinoma

Pineal Cyst-Asymptomatic • • • • •

[Figure 5-8-53]

Figure 5-8-53

T1W sagittal and T1W-Gd axial Cyst is ovoid and hypointense Enlargement of pineal Cyst wall enhances minimally Quadrigeminal plate not compressed

Pineal Cyst CLASSIC LOCATIONS • • • • • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Pineal cyst

DEEP AND PERIVENTRICULAR • • • • • • •

Glioma (astrocytoma, oligodendro.) Lymphoma (usually primary in CNS) Toxoplasmosis, CMV (ependymitis) Leukoencephalopathy (WM) Arteriolar sclerosis (HT) Infarcts (lacunar, tri-watershed) Hemorrhage

Neuroradiology

1119 1121

Patterns of Location: Infratentorial and Supratentorial

Glioblastoma Multiforme [Figures 5-8-54 and 5-8-55]

Figure 5-8-54 Figure 5-8-55

Expansile lesion of the corpus callosum

Lymphoma PCNSL [Figure 5-8-56]

Two “butterfly” lesions. One with peripheral dense enhancement is a GBM; and, the other with softer more uniform enhancement is primary CNS lymphoma

Figure 5-8-56

Figure 5-8-57

FLAIR T2W T1W Gd+ Primary CNS Lymphoma – an expansile enhancing lesion of the corpus callosum

PCNSL: Immunocompetent Cytomegalovirus – note the thin rim of abnormal enhancement cause by ependymitis (Courtesy Vince Mathews, M.D.)

RIM PHOMA CMV [Figure 5-8-57] Multiple Sclerosis [Figure 5-8-58] CLASSIC LOCATIONS

Figure 5-8-58

Cortical and subcortical • • • • • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial Multiple Sclerosis. Classic Dawson fingers – ovoid lesions perpendicular to the ventricle from perivenous inflammation

Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

CONVEXITY INTRAAXIAL •

• •

Figure 5-8-59

Gray-white junction ➢ Hematogenous neoplasm ➢ Hematogenous infection ➢ Hematogenous thrombi (multiple infarcts) Infarction/ischemia Vasculitis (infectious,autoimmune)

Hematogenous dissemination [Figure 5-8-59] • • •

Multiple Cortical/subcortical Ring Lesions ➢ smooth ➢ round ➢ uniform thickness

CEREBRAL INFARCTION [Figure 5-8-60] • • • •

Abrupt Onset Gray Matter Involved Little Mass Effect Vascular Territory & Wedge Shape

Hematogenous dissemination. Multiple cortical ring-enhancing lesions – necrotic metastases from breast carcinoma on chemotherapy

Figure 5-8-60

Cerebral infarction. MCA territory, with matching lesions on DWI and ADC map

Figure 5-8-61

Time is Brain ! • • • •

Therapeutic Windows: 3 hours for IV tPA 6 hours for IA thrombolysis 9 hours for IV ‘Bat Spit’? ➢ an enzyme known as desmoteplase or DSPA ➢ isolated from the saliva of Desmodus rotundus ➢ vampire bat, Central and South America, 1oz NOTE: Clock starts with last time patient was observed ‘normal’. If you wake with a stroke, that might be bedtime … unless you get up at night

PCA Infarct

Lights up like a lightbulb on MRI DWI

PCA Infarct [Figure 5-8-61] Neuroradiology

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Patterns of Location: Infratentorial and Supratentorial

CVA : Progression of CT findings

Figure 5-8-62

Old Infarct •

Wallerian Degeneration

Dysembryoplastic Neuroepithelial Tumor (DNET) [Figures 5-8-62 and 5-8-63] •

Imaging Features ➢ Cortical, most in temporal lobe ➢ Well-circumscribed mass ➢ Hypointense T1W, Hyperintense T2W ➢ Nodular Cortical Mass ❖ Multicystic ❖ Megagyric - Assoc. calvarial erosion ➢ No Edema ➢ No or Minimal Mass Effect ➢ +/- Calvarial Erosion ➢ Occasional Enhancement

DNET – Usually a cortical lesion, often wedgeshaped

Figure 5-8-63

DDX: TUBEROUS SCLEROSIS •

• • •

Cortical Hamartomas (“Tubers): ➢ Appear to Spare Superficial Cortex ➢ Tend to be Multiple Accompanying Subependymal Nodules + Family History Generalized Seizures

CLASSIC LOCATIONS • • • • • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

DNET – Usually a cortical lesion, often wedgeshaped

CONVEXITY EXTRAAXIAL Differential [Figure 5-8-64] •

• •

EPIDURAL (sub-periosteal) ➢ (Hematoma, empyema, mets) ➢ (biconvex, acute, limited by sutures) SUBDURAL (epi-arachnoid) ➢ (Hematoma, empyema, mets) ➢ (Crescentic, subacute, crosses sutures) MENINGIOMA ➢ (hyperdense, hemispheric,hyperostosis, homogeneous enhancement)

Figure 5-8-64

Epidural = Subperiosteal [Figure 5-8-65] Epidural Metastasis

Schematic of epidural (left) and subdural (right) localization. Copyright 2006

Subdural = Epi-arachnoid [Figure 5-8-66] Subdural Metastasis Meningioma – Pre and Post Gd

Patterns of Location: Infratentorial and Supratentorial

1122 1124

Neuroradiology

Meningioma - Dural Tail

Figure 5-8-65

MENINGIOMA [Figure 5-8-67] •

The 4H+ Tumor ➢ homogeneous ➢ hyperdense ➢ homogeneous enhancement ➢ hemispheric ➢ hyperostosis ➢ hormonally modulated

Figure 5-8-66

Epidural hematoma

Bilateral chronic subdural hematomas

Figure 5-8-67

Meningioma. Hyperdense on plain CT, hemispheric, homogeneous enhancement, hyperostosis – the 4H+ tumor

Neuroradiology

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Patterns of Location: Infratentorial and Supratentorial

Patterns of Enhancement James G. Smirniotopoulos, MD Figure 5-9-1

Why Give Contrast? Contrast Enhancement •

Vascularity ➢ Blood Volume (rCBV) Perfusion MTT

•

➢ Blood Flow (rCBF) ➢ Arteries & veins > capillary Permeability ➢ Capillary (leakage)

Blood Brain Barrier Breakdown

Mechanisms of Enhancement [Figure 5-9-1] • •

Vascularity Permeability

Contrast Enhancement •

•

VASCULAR (intravascular) PHASE ➢ Inc. Blood Flow/Hypervascular ➢ AVM, Meningioma, GBM ➢ TRUE "Luxury" Perfusion ➢ Hyperemic Swelling (“malignant brain edema”) INTERSTITIAL (extravascular) PHASE ➢ Blood-brain-barrier breakdown) ➢ Acute inflammation (MS) ➢ Neoplasm, Abscess, “granulation” tissue ➢ Ischemia, “luxury” perfusion, contusion

Two mechanisms of Contrast Enhancement: Increased vascularity (rCBV and rCBF); and, increased permeability from breakdown of the blood-brain-barrier (BBBB)

Figure 5-9-2

Types of Radiology Contrast •

• •

Barium (BaSO4) for ingestion and enema ➢ Insoluble suspension Iodine for ingestion and enema ➢ Gastrograffin I+ and Gd+ Intravascular Contrast Agents ➢ Ionic Contrast ❖ High Osmolarity - Magnevist® – a chelate of Gd - di-N-methylglucamine salt of gadopentetate (Gd-DTPA) ❖ Iso and Low Osmolar - Iodixanol ➢ Non-Ionic Contrast ❖ ProHance® – Gadoteridol ❖ Omniscan® - Gadoimide

Time Density Curves [Figure 5-9-2] Variability in BBB permeability and perfusion [Figure 5-9-3]

Patterns of Enhancement

1124 1126

The bolus creates a high intravascular concentration gradient that pushes contrast across a permeable membrane into the tissue interstitial space

Figure 5-9-3

Variable degrees of permeability alteration may create variable time-density curves for interstitial (extravascular) enhancement

Neuroradiology

Double Dose of Contrast

Figure 5-9-4

Steroid Suppression of Enhancement [Figure 5-9-4]

MR vs. CT [Figures 5-9-5 and 5-9-6] Figure 5-9-5

Steroids may reduce or completely suppress visible enhancement

Enhancement on CT and MR are similar – except for intraarterial and pachymeningeal (dural) enhancement

What makes the BBB?

Figure 5-9-6

Semi-permeable Capillaries •

• •

Brain ➢ Blood-brain-barrier Testicle ➢ Blood-Testicle barrier Ovary ➢ Blood-Ovary barrier

Ultrastructure of BBB [Figure 5-9-7] •

•

Neural capillary ➢ astrocytic feet ➢ continuous BM ➢ tight junctions ➢ no pinocytosis Non-neural or ABBB ➢ no astrocytic feet ➢ fenestrated BM ➢ intercellular gaps ➢ pinocytosis

Figure 5-9-7 Enhancement on CT and MR are similar – except for intraarterial and dural enhancement

Schematic of Ultrastructure of blood-brain-barrier

Neuroradiology

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Patterns of Enhancement

The Berlin Wall and the BBB [Figure 5-9-8] •

• • •

•

Figure 5-9-8

Who built the Berlin Wall? ➢ The East Germans Why? ➢ To keep out the West Germans But … the barrier works in BOTH directions Some things are kept out ➢ Drugs, Contrast material Some things are kept in: ➢ Hemosiderin ➢ Vasogenic Edema

CNS: Normal Tissues w/o BBB • • • • • •

DURA (falx and tentorium) ARACHNOID ? (it’s avascular) CHOROID PLEXUS PINEAL GLAND (epiphysis) PITUITARY GLAND (hypophysis) CTZ (area postrema of medulla oblongata) ➢ one of the "Circumventricular Organs"

Hemosiderin cannot be cleared from the brain and spinal cord because of the blood-brain-barrier

Physiologic Why? •

•

Why do we have a BBB? ➢ To protect the brain ➢ To create the ionic environment for nerve conduction Why do we have tastebuds? ➢ So that we eat things good for us ❖ Salt ❖ Sweet ❖ Sour ❖ Umami (MSG) ❖ Bitter

Normal Enhancement • • • •

• • • •

Choroid Plexus Pineal Pituitary Stalk Pituitary Gland (anterior and posterior) ➢ Hypophyseal Portal System Cavernous sinus and dural reflections Nasal turbinates Sinonasal mucosa Extracranial muscles and mucosa

Plain vs. Enhanced Fat -Suppressed T1W - Gd Nasal Cycle • • • • • • • •

Vasocongestion ~/~ vasoconstriction 6 – 8 hour cycle alternation Humidify and warm the air Secrete mucus (1 – 2 liters/day) Chronic vasocongestion would cause submucosal edema Breathe mostly through the vasoconstricted side (~ 75%-85%) Yogi’s can control which nostril So can Tom Cruise (Minority Report)

Patterns of Enhancement

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Neuroradiology

Fat -Suppressed T1W – Gd

Figure 5-9-9

Musk Ox – Nasal Turbinates Fat -Suppressed T1W - Gd [Figure 5-9-9] Cranial Nerve Enhancement • •

•

Optic Nerve – Never normal * Seventh Nerve: ➢ Inside facial canal – Yes, asymmetric ~70% ➢ Geniculate ganglion – 98% ➢ Tympanic > labyrinthine > mastoid ➢ May represent perineural vessels Eighth Nerve – Never normal

Abnormal optic nerve enhancement – optic neuritis

* Neuroradiology 1997 Mar;39(3):207-12.

Figure 5-9-10

Plain vs. Enhanced [Figure 5-9-10] Contrast Enhancement – Phases ANGIO(I–) R-N (Tc+) C.T. (I–) MRI (Gd+)

VASCULAR ++++ + (flow) + +/–

BBB – + (static) +++ +++

Enhancement vs. Edema

Normal pineal enhancement. Visualization depends on the timing of injection and the molecular weight of the contrast

Contrast Enhancement •

Morphologic Patterns ➢ Homogeneous (solid) ➢ Heterogeneous (non-uniform) ➢ Ring (unilocular/multilocular) ➢ Serpentine ("Gyriform“) ❖ Serpiginous?

Serpiginous •

“A creeping skin eruption.”

Contrast Enhancement •

Location ➢ SUPERFICIAL (CORTICAL/GYRAL) ➢ GREY-WHITE JUNCTION ➢ DEEP WHITE MATTER ➢ PERIVENTRICULAR, EPENDYMAL

Neuroradiology

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Patterns of Enhancement

Contrast Enhancement •

Figure 5-9-11

Cortical/Gyriform ➢ Cerebral Ischemia / Infarction ➢ CSF or sub-pial spread ➢ Meningo-encephalitis ➢ S.A.H. ➢ Leptomeningeal Malformation (SW) ➢ Meningioangiomatosis (NF2)

Listeria Monocytogenes [Figure 5-9-11] CNS Bacterial Infections •

•

•

Meningitis - Listeria Monocytogenes

Birth to Four Weeks ➢ 2-10 cases / 10k births ➢ Group B streptococcus ➢ E. coli ➢ Listeria monocytogenes 3 mo. to 3 yrs ➢ Haemophilus influenzae (Type B) ➢ Strep pneumoniae ➢ Meningococcus (Neisseria meningitidis) Over 3 yrs to Adult ➢ Strep pneumoniae ➢ N. meningitidis

CSF Signal ? [Figure 5-9-12]

Figure 5-9-12

Flair shows “dirty CSF” from protein and pus in the SAS (Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)

Figure 5-9-13

Enhancement? [Figure 5-9-13] •

Leptomeningeal Enhancement Pneumococcal Meningitis

Pachymeningeal Enhancement •

[Figure 5-9-14]

Intracranial Hypotension

Figure 5-9-14 Multiple symmetric areas of abnormal leptomeningeal enhancement from meningitis Notice the abnormal ehancement of the entire suprasellar cistern (Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)

Pachymeningeal enhancement (Courtesy Laszlo Mechtler, DNI)

Patterns of Enhancement

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Neuroradiology

Hemorrhagic Infarction [Figure 5-9-15]

Figure 5-9-15

Ischemic Enhancement •

•

•

Acute and/or Reperfusion enhancement ➢ True “luxury perfusion” 2° to acidosis ➢ BBBB after 4-6 hours of ischemia Subacute to Chronic enhancement ➢ Ingrowth of capillaries from surface ➢ Primarily in GM (cortex and deep) ➢ Peak intensity at 2-3 weeks ➢ Fades away over weeks to months Atrophy replaces Enhancement

Blood Brain Barrier [Figure 5-9-16] • • • • • •

Hemorrhagic infarction shows early and dense enhancement due to reperfusion

Cirrhosis Hyperbilirubinemia Bilirubin bound to Albumin Albumin can’t cross the Blood-brain-barrier BBB is abnormal in infarct Mostly gray-matter

Figure 5-9-16

H&P •

Pt is a 25 yo woman, PMHx of BCP, presenting w/ acute mental status changes, afebrile Actually… • Pt is a 34 yo marine stationed at Guantanamo Bay Cuba, presenting w/ acute mental status changes, febrile

HSV Encephalitis [Figure 5-9-17] Figure 5-9-17

Gyral enhancement in Sturge-Weber disease

Figure 5-9-18

Herpes encephalitis

Meningioma – Dural Tail [Figure 5-9-18] Dural Tail • • • • •

Meningioma - Dural Tail

Curvilinear enhancement AKA “dural flair” First reported w/meningioma First reported to be neoplastic invasion What is it REALLY? ➢ Thickening of the dura ➢ Vasocongestion of the dura ➢ Edema of the dura

Neuroradiology

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Patterns of Enhancement

Contrast Enhancement [Figure 5-9-19] •

Figure 5-9-19

Ring Lesion ➢ Circumferential or peripheral/marginal enhancement, surrounding a central nonenhancing region. ➢ In turn, this is often surrounded by a large area of “edema”. ➢ May be unilocular or multilocular.

Rules for Ring Enhancing Masses [Figure 5-9-20]

Abscess

Figure 5-9-20

Differential appearance of ring enhancing lesions

Contrast Enhancement • • • •

Contrast leaks into interstitium from vessels without BBB Remains localized within millimeters of where it leaks out Not “simple diffusion” but rather “BULK FLOW” at a very slow rate (Glacier Not River)

Contrast Enhancement •

Ring Lesions Differential ➢ M – Metastasis, MS ➢ A – Abscess (Also Cerebritis) ➢ G – Glioblastoma, Granuloma ➢ I – Infarct (Esp. Basal Ganglia) ➢ C – Contusion (Rare) ➢ A – AIDS (Toxo, Etc.) ➢ L – Lymphoma (in Aids) ➢ D – Demyelination (Active) ➢ R – Resolving Hematoma Radiation Change (Necrosis)

Contrast Enhancement •

Ring Lesion Features For Infection ➢ ORGANIZED ABSCESS ➢ thin and uniform wall (3-7mm.) ➢ smooth inner margin does not “fill in” on CT, MR, even after time delay imaging ➢ CEREBRITIS (infection w/o organization): ➢ variable wall (may be smooth) smooth/variable inner margin ➢ often has “fill-in” on DDD ➢ (w/o fluid level)

Patterns of Enhancement

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Neuroradiology

Contrast Enhancement- Abscess • •

• • •

Figure 5-9-21

2 – 4 wks. for ORGANIZED WALL 2 LAYERS ➢ inner MESENCHYMAL (capillaries,fibroblasts, collagen) ➢ outer ASTROGLIAL (reactive astrocytes) WALL facing GM is well formed 3-5 mm WALL FACING WM IS THINNER/WEAKER (Daughter Abscess) Ventricular Spill (“pyocephalus”)

Abscess [Figures 5-9-21 to 5-9-23] • • • • • • •

Round Smooth Regular Convex all around Rim of Edema Restricted Diffusion MRS shows ➢ AA peaks ➢ Acetate ➢ Succinate

Figure 5-9-22

Cerebral abscess in thalamus

Figure 5-9-23

Abscess. Viscous Pus and Coagulation Necrosis cause restricted diffusion

Figure 5-9-24 Abscess

Contrast Enhancement [Figure 5-9-24] • •

•

Ring Lesion Features For Neoplasm NECROTIC NEOPLASM: ➢ thick and irregular wall ➢ shaggy inner margin (usually) ➢ may “fill in” heterogeneously on DDD CYSTIC NEOPLASM: ➢ thin wall +/– MURAL NODULE ➢ PART OF WALL MAY NOT ENHANCE ❖ smooth inner margin ❖ uniform fluid enhancement ❖ or FLUID LEVEL

Neuroradiology

Ring enhancing lesion: Glioblastoma

1131 1133

Patterns of Enhancement

Glioblastoma Multiforme

Figure 5-9-25

Pilocytic Astrocytoma [Figure 5-9-25] •

Cyst with mural nodule?

Tumefactive Demyelination [Figure 5-9-26]

Open (Incomplete) Ring Sign • •

Demyelinating Disease Fluid-secreting (“Cystic”) Neoplasms

Pilocytic Astrocytoma

Masdeau JC, Moreira J, Trasi S, Visintainer P, Cavaliere R, Grundman M: The open ring. A new imaging sign in demyelinating disease. J Neuroimaging 1996; 6(2):104-107.

Figure 5-9-26

Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P: Open-ring imaging sign: highly specific for atypical brain demyelination. Neurology 2000; 54(7):1427-1433

Contrast Enhancement: Hematoma • •

EARLY: Hyperdense, round/oval homogeneous mass of RBC’s with proportional mass effect for volume Edema “Halo”, not spreading LATER: Iso-/Hypodense, smaller Reactive capillaries form outside Uniform rim of enhancement May see “vasogenic” edema spreading

Hematoma – Halo of serum [Figure 5-9-27]

Tumefactive Demyelination

Figure 5-9-28

Figure 5-9-27

Acute Hematoma - halo of edema Subacute to chronic may have vasogenic edema

Acute Hematoma - halo of edema Subacute to chronic may have vasogenic edema

Figure 5-9-29

Reactive Ring Enhancement [Figure 5-9-28] MCA infarct [Figure 5-9-29] Ring Enhancing Mass •

•

Benign ➢ Round ➢ Smooth ➢ Thin wall Malignant ➢ Undulating ➢ Irregular ➢ Thick wall

Patterns of Enhancement

MCA infarct involving cortex and basal ganglia

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Neuroradiology

Post-Operative Enhancement •

• • •

•

Figure 5-9-30

RESIDUAL TUMOR ➢ Left behind RECURRENT TUMOR ➢ It grew back Infection Normal Postoperative Change ➢ surgical “trauma”, healing, gliosis Radiation Necrosis

Contrast Enhancement – Surgical Change and/or Residual Neoplasm? [Figure 5-9-30] •

• •

•

Surgical Enhancement typically after 24-48 hrs ➢ Scan early (24 hours) or scan late (4-6 wks) ➢ May fade after a few weeks but may last for months ➢ Gd+ enhancement may begin in 4-6 hours In the Operative Bed ➢ Mixed w/ residual tumor? ➢ Along the Margins of Resection ➢ Thin and uniform in brain (CT/MR) LINEAR meningeal/dural enhancement on MR ➢ Not lumpy-bumpy Small amts of air, blood are normal ➢ No instruments or sponges, etc.!!

Normal enhancement after ventricular shunt catheter insertion

References 1. 2. 3. 4. 5.

6. 7. 8. 9.

10. 11.

12. 13. 14.

Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura. Neurosurgery. 1994 Sep;35(3):370-7;discussion 377. Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8. Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5. Ekinci G, Akpinar IN, Baltacioglu F, et al. Early-postoperative magnetic resonance imaging in glial tumors: prediction of tumor regrowth and recurrence. Eur J Radiol 2003; 45:99-107. Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPAenhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment. Radiology. 1990 Aug;176(2):447-50. . Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French. Henegar MM, Moran CJ, Silbergeld DL. Early postoperative magnetic resonance imaging following nonneoplastic cortical resection. J Neurosurg 1996; 84:174-179. Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8. Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural spaceoccupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German. PMID: 9467021 Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo. 1996 Apr;164(4):314-7. German. Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90. Kaufman BA, Moran CJ, Park TS. Computer tomographic scanning within 24 hours of craniotomy for a tumor in children. Pediatr Neurosurg 1995; 22:74-80. Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels: the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5. Lai PH, Ho JT, Chen WL, et al. Brain abscess and necrotic brain tumor: discrimination with proton MR spectroscopy and diffusion-weighted imaging. AJNR Am J Neuroradiol 2002; 23:1369-1377.

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15. Martin-Duverneuil N, Sola-Martinez MT, Miaux Y, et al. Contrast enhancement of the facial nerve on MRI: normal or pathological? Neuroradiology 1997; 39:207-212. 16. Masdeau JC, Moreira J, Trasi S, Visintainer P, Cavaliere R, Grundman M: The open ring. A new imaging sign in demyelinating disease. J.Neuroimaging 1996; 6(2):104-107. 17. Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P: Open-ring imaging sign: highly specific for atypical brain demyelination. Neurology 2000; 54(7):1427-1433. 18. Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7. 19. Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16. 20. Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion 590-1. 21. Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995 Nov-Dec;19(6):890-2. 22. Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):42933. 23. Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI. Fukushima J Med Sci. 1998 Jun;44(1):1-11. 24. Sato N, Bronen RA, Sze G, et al. Postoperative changes in the brain: MR imaging findings in patients without neoplasms. Radiology 1997; 204:839-846. 25. Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese. 26. Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial meningiomas on fluid-attenuated inversion-recovery images. Neuroradiology. 2004 Feb;46(2):130-5. Epub 2004 Jan 28. 27. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):763-8. 28. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga H, Toya S. Prediction of consistency of meningiomas with preoperative magnetic resonance imaging. Surg Neurol. 1997 Dec;48(6):579-83.

Patterns of Enhancement

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Radiologic Grading of Astrocytoma and The WHO 2000 Brain Tumor Classification James G. Smirniotopoulos, MD Brain Neoplasia: Frequency [Figure 5-10-1]

Figure 5-10-1

Childhood CNS Tumor Demographics •

367 Syrian children, collected from 1993-2002 ➢ Supratentorial - 47% ➢ Infratentorial - 53% ➢ Male 52%:48% Female ➢ Overall Incidence: ❖ Medulloblastoma 27% ❖ Astrocytoma 26% ❖ Craniopharyngioma 14% ➢ Posterior Fossa Only: ❖ Medullo (PNET) 54% ❖ Astrocytoma 23% ❖ Ependymoma 17%

The frequency of the various primary central Kadri H,Mawla AA, Murad L: Incidence of childhood brain nervous system tumors ranges from 2% for tumors in Syria (1993-2002) Pediatric Neurosurgery meningioma and mixed oligoastrocytoma to 40% 2005; 41:173-177 for glioblastoma multiforme (GBM) and 42% for infiltrative astrocytoma Pediatric Posterior Fossa • • •

454 posterior fossa patients All under the age of 18 402 tumors: ➢ 37.1% Cerebellar astrocytoma (149) ➢ 34.6% Medulloblastoma (PNET) (139) ➢ 11.4% Brain stem astrocytoma (46) ➢ 7% Ependymoma (28) ➢ 9.9% "other" (40)

Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439.

Traditional Tumor Grading •

• •

PATHOLOGIST ➢ LOW GRADE ➢ HIGH GRADE RADIOLOGIST ➢ NON-ENHANCING ➢ ENHANCING NEUROSURGEON ➢ “SUCKABLE” ➢ “NON-SUCKABLE”

Kernohan-Sayre (AFIP) Grading System: • • •

• •

GRADE I – “BENIGN” or “Low-Grade” GRADE II – “BENIGN” or “Low-Grade” GRADE III – ANAPLASTIC ➢ atypia, pleomorphism, mitoses, etc. GRADE IV- MALIGNANT ➢ Mitoses, Vascularity, Endothelial changes ➢ Necrosis ➢ Glioblastoma Multiforme NOTE: Numerous modifications exist, most into three grades, e.g..: Low Grade (Benign), Anaplastic, and GBM (w/ NECROSIS).

Neuroradiology

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The WHO 2000 Brain Tumor Classification

Pathologic – Radiologic Correlation Pathology

Radiology

Cellularity

T2 SI, DWI & ADC

Endothelial proliferation and Vascularity Necrosis

Enhancement, PWI, and Permeability Imaging Ring Lesion, MRS, DWI & ADC

Hemorrhage

T1 and T2 SI

Labeling Indices

MRS, Th 201 and FDG

Infiltration

T1 and T2 SI, DTI

ASTROCYTOMA: Five Year Survival Grading Systems: Sem Rad Onc (1991); 1: 2-9 Kernohan

Berger

Benign (1)

Astrocytoma

WHO 2000 1 Pilocytic,SEGA 2 Astrocytoma

Benign (2) Anaplastic

3 Anaplastic

Glioblastoma

4 Glioblastoma

Anaplastic (3) Glioblastoma (4)

Define the Problem • • • •

Some Low Grade Enhance Some Low Grade Do Not Some Low Grade => GBM Some Low Grade Do Not

WHO Classification • • • •

Defines Histologic Subtypes Grades Biologic Potential Allows International Cooperation Ascending scale of Aggression from 1-4

WHO Correlation •

Low Grade ➢ Long-Term Survival: Possible Cure ➢ Stable History (No Progression): Possible Cure

WHO Grading CNS Tumors GRADE 1 GRADE 2 GRADE 3 GRADE 4

JPA PXA PXA GBM

SGCA

GANG

MENING HPC HPC

ANAPLASTIC

CNS NEOPLASM-GLIAL: Prognostic Factors • • •

Location Age Histology

The WHO 2000 Brain Tumor Classification

1136 1138

Neuroradiology

“BENIGN” ASTROCYTOMA •

Figure 5-10-2

Two types: ➢ Low grade (“benign”) ❖ Diffuse (Adults) ➢ Low grade “special” ❖ Circumscribed (Children)

Normal Diffuse Astrocytoma [Figure 5-10-2] WHO Gr1 - Pilocytic Astrocytoma [Figure 5-10-3] Circumscribed vs Diffuse Circumscribed Astrocytoma

Diffuse astrocytoma: Individual neoplastic cells spread out through the white matter. In WHO Gr 2, this is only noted as “increased cellularity”

Astrocytoma: Circumscribed •

• • •

“Special” astrocytomas ➢ Astrocytoma of young ➢ Various locations ➢ Well circumscribed (yet, no capsule) Do NOT spread along WM Do NOT change grade (except PXA) Constellation of findings correlates w/ Histology

Figure 5-10-3

Pilocytic Astrocytoma •

Cystic Cerebellar Astrocytoma Juvenile Pilocytic Astrocytoma (“PA” or “JPA”)

Pilocytic Astrocytoma • • • • • • • •

Synonyms: Polar Spongioblastoma, Cystic Cerebellar Astrocytoma Cell of Origin: Astrocyte (bi-polar, hairlike) Associations: in ON w/ NF-1 Incidence: 3%–6% of ALL Cranial, 32% of Child Age: 5–15 (Zulch 3–7) Sex: Slight F (11/9) Location: Cerebellum, Chiasm/Hypothal, Optic Treatment: Surgery, patience Prognosis: 77% at 5 yrs, 75% at 10 yrs, 75% at 15 yrs

Circumscribed astrocytoma, like pilocytic astrocytoma, have “pushing margins” and are often fluid-secreting

Pilocytic Astrocytoma: Radiology •

[Figures 5-10-4 to 5-10-6]

•

• •

Cerebellum, Diencephalon ➢ rare in BS or Cerebrum Majority have significant “cyst” ➢ “Cyst and Mural Nodule” ❖ part of lining does NOT enhance ➢ Nodule may be heterogeneous ➢ Exceptional purely solid Nodule NOT hyperdense Calcification in 5%–25%

Figure 5-10-4

Pilocytic astrocytoma with classic “cyst and nodule” morphology Neuroradiology

1137 1139

The WHO 2000 Brain Tumor Classification

Figure 5-10-5

Figure 5-10-6

Gross picture of pilocytic astrocytoma Pilocytic astrocytoma with classic “cyst and nodule” morphology

Figure 5-10-7

WHO Gr1 - Pilocytic Astrocytoma Pathology •

• • •

Biphasic pattern ➢ dense pilocytic glia ➢ Rosenthal fibers ➢ loose microcystic areas No necrosis Low grade Abnormal capillaries ➢ allow enhancement ➢ fluid production

Pilocytic Astrocytoma

Figure 5-10-8

Grading Problems in Gliomas 51 Pilocytic (WHO Gr. 1) KERNOHAN MAYO-ST.ANNE 1 26% 1 2% 2 69% 2 55% 3 6% 3 35% 4 0% 4 8% • By conventional “feature counting” most pilocytic astrocytomas were overgraded.

Pilocytic Astrocytoma • •

Pilocytic Astrocytoma (Courtesy of Paul Sherman)

Variant Appearance Variant Location

Pilocytic Astrocytoma [Figure 5-10-7] • •

A Cyst with mural nodule? Not Always !!!

Figure 5-10-9

Pilocytic Astrocytoma [Figure 5-10-8] Pilocytic Astrocytoma: Locations [Figure 5-10-9] • • • • •

CEREBELLUM Chiasm And Optic Nerve Hypothalamus/thalamus Cerebral Hemisphere Spinal Cord (Intramedullary)

Pilocytic astrocytoma of hypothalamus The WHO 2000 Brain Tumor Classification

1138 1140

Neuroradiology

•

Pilocytic Astrocytoma ➢ Enhance ➢ Cyst w/ Nodule Cystic ➢ Hypodense nodule ➢ Calcification ➢ NOT vascular ➢ Nodule location varies

Hemangioblastoma Enhance Solid <- cyst w/nodule -> Hyperdense nodule Never Ca++ Hypervascular, Flow Voids Nodule is “Subpial”

Figure 5-10-10

Pilocytic Astrocytoma (Juvenile Pilocytic) • • • • •

Childhood, Young Adults Benign, no mitosis/necrosis Circumscribed – Enhancing Cyst Formation, Mural Nodule Cerebellum and Diencephalon ➢ (Optic tracts, Hypothalamus)

WHO Grade I •

• •

Circumscribed Astrocytoma ➢ JPA (Pilocytic) ➢ SGCA (Subependymal Giant Cell) Ganglioglioma Meningioma

Subependymal Giant Cell Astro [Figure 5-10-10]

Subependymal Giant Cell Astrocytoma

Astrocytomas •

“SPECIAL” ASTROCYTOMAS ➢ Circumscribed Growth: ❖ Pilocytic ❖ Subependymal Giant Cell ❖ Pleomorphic Xantho-Astrocytoma

Circumscribed Astrocytoma Pleomorphic Xanthoastrocytoma • • • • • • •

Rare Variant of Astrocytoma Arises from Subpial Astrocytes Affects Superficial Cerebral Cortex and Meninges Skull erosion (scalloped excavation) Temporal > Frontal > Parietal WHO Grade 2,3 50% progress over time

Pleomorphic Xanthoastrocytoma •

IMAGING: ➢ CT APPEARANCE: ❖ Well-Circumscribed Hypodense or Cystic Mass ❖ Often Isodense Solid Nodule that Intensely Enhances ❖ May Mimic Juvenile Pilocytic Astrocytoma ❖ Calcifications Rare

Pleomorphic Xanthoastrocytoma •

MR APPEARANCE: ➢ Well-Circumscribed Mass of Variable Size ➢ Superficial Cortical Location ➢ T1: Low/Mixed Signal, ➢ T2: High/Mixed Signal ➢ Often with Cystic Component ➢ Solid Portion Intensely Enhances ➢ Adjacent Meninges May Enhance (Tail) ➢ Little or No Mass Effect

Neuroradiology

1139 1141

The WHO 2000 Brain Tumor Classification

Astrocytomas •

“Ordinary” Astrocytoma ➢ Diffuse Infiltration of WM by: ❖ Fibrillary Astrocytes ❖ Protoplasmic Astrocytes ❖ Gemistocytic Astrocytes ❖ WHO 2,3,4 (NOT 1) ❖ KS & Mayo Grades 1–4

Where do Glioblastomas come from?

• Progressive Transformation from lower grade diffuse astrocytoma - OR • Arise de novo

Diffuse Astrocytoma … too many cells ! •

Mild cellular atypia

KERNOHAN (KS) ANAPLASIA

1

2

3

4

0

Min

>1/2

Marked

CELLULARITY

Mild

Mild

Inc

Marked

MITOSIS

0

0

Plus

Marked

Min

Min

Marked

ENDOTHELIAL 0 Proliferation NECROSIS TRANSITION ZONE

Marked

<== Broad

Sharp ==>

Figure 5-10-11

Astrocytoma: Diffuse (Fibrillary, protoplasmic, etc.) • • • • • •

“Adult type” or “Hemispheric” Astrocytoma Diffusely infiltrate brain, along WM tracts Continuum, from low-grade to high-grade Genetic Alterations 17 => 9 => 10 Many Progress in Histology over time, changing from WHO Gr. 2 => Gr. 3 => Gr. 4 (GBM) Imaging tends to correlate with histology, especially at the ends of spectrum

Astrocyte Mutation [Figure 5-10-11] Successive mutations in Astrocytoma

Diffuse Astrocytoma [Figures 5-10-12 and 5-10-13] Figure 5-10-12

All three grades of astrocytoma in one patient The WHO 2000 Brain Tumor Classification

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Neuroradiology

Diffuse Astrocytoma • • • •

Figure 5-10-13

‘Astrocytoma’ Anaplastic Astrocytoma Glioblastoma Multiforme The Eastwood Method: ➢ The Good ➢ The Bad ➢ The Ugly

Astrocytoma: Radiologic Grading •

• •

TYPE 1 – WHO 2, KS Grade 1–2, “Benign” ➢ Homogeneous ➢ No Enhancement, No Vasogenic Edema TYPE 2 – WHO Grade 3, Anaplastic ➢ Variable Enhancement, Edema ➢ 50% enhance – 50% don’t TYPE 3 – WHO Grade 4 Glioblastoma ➢ Heterogeneous (Necrosis, Blood) ➢ Ring Enhancement, Edema

Diffuse astrocytoma is a spectrum of tumors, pathologically and by imaging. The classification into three grades (WHO 2,3, or 4) may be an artificial segmentation along a continuum

“Benign” Astrocytoma: WHO 2, KS 1–2, Mayo 1 •

•

YOUNGER PATIENT ➢ CHILDHOOD ➢ Young Adults (20’s – 40’s) NL VESSELS (NO NEOVASCULARITY) ➢ BBB INTACT ➢ NO EDEMA ➢ NO ENHANCEMENT ➢ NO TUMOR VESSELS

Figure 5-10-14

Benign – Diffuse •

HOMOGENEOUS ➢ NO NECROSIS ➢ NO HEMORRHAGE ➢ INCREASED WATER ❖ DARK and Poorly Demarcated on CT ❖ Dark and Sharp on T1W ❖ BRIGHT and Sharp on T2W ➢ MICROCYST >>> MACROCYST (macrocysts occur in JPA, etc.)

PD

T2 Gr 2 Fibrillary Astrocytoma

Gr 2 Fibrillary Astrocytoma [Figures 5-10-14 and 5-10-15]

Figure 5-10-15

T1T1gad non Gr 2 Fibrillary Astrocytoma – no enhancement after gadolinium Neuroradiology

1141 1143

The WHO 2000 Brain Tumor Classification

Gr 2 Astrocytoma: PWI [Figure 5-10-16] •

Figure 5-10-16

Reduced perfusion

Gliomatosis Cerebri [Figure 5-10-17] Figure 5-10-17 T1-gad

T2

Female, acute stroke (3 days), MCA occlusion

Figure 5-10-18 Gliomatosis Cerebri – a diffuse astrocytoma infiltrating two or more lobes of the brain

Gliomatosis Cerebri: Diffuse Astrocytoma – 2 lobes [Figure 5-10-18]

Spread along White Matter Tracts [Figure 5-10-19]

Figure 5-10-19 Gliomatosis Cerebri – a diffuse astrocytoma infiltrating two or more lobes of the brain

Figure 5-10-20

Diffuse Grade 2 astrocytoma spreading through white matter, including corpus callosum

Astrocytoma: Microcystic change

Gliomatosis Cerebri Astrocytoma: Microcystic Change [Figure 5-10-20]

Figure 5-10-21

Astrocytoma [Figure 5-10-21]

Pontine astrocytoma – WHO Gr 2 The WHO 2000 Brain Tumor Classification

1142 1144

Neuroradiology

Modes of Spread • • • •

1. 2. 3. 4.

Figure 5-10-22

Natural passages Along surfaces Along tracts Across the meninges

Spread Along Tracts: • • • • •

Corona Radiata Peduncles Corpus Callosum Anterior Commisure Arcuate Fibres

Astrocytes Track Along WM

Progressive transformation of WHO Grade 2 astrocytoma to Glioblastoma multiforme

Pontine Astrocytoma Figure 5-10-23

Pontine Astrocytoma: WHO 2 WHO 2 … GBM [Figure 5-10-22] Expanded Brain Anaplastic Astrocytoma: Overall Characteristics • • • • • •

Grade III malignant glioma Anaplastic astrocytoma – no enhancement in this example – Less aggressive than GBM, malignant about 50% will enhance with somewhat better prognosis Frequency: highest in young adults (30 – 40 years) Recurrence: often as a higher-grade glioma Challenge: difficult to remove completely with surgery Median survival: 3 – 4 years

Anaplastic Astrocytoma [Figure 5-10-23] Anaplastic Astrocytoma ( WHO 3 ) •

Increased Cellularity, +/- minimal vascular changes, no necrosis , no hemorrhage

GBM - Glioblastoma “Malignant” Astrocytoma: •

•

•

Older patient ➢ 40’s and up ➢ exceptions (PNET) ➢ ~ 1/2 arise from previous low grade (2–3) Abnormal Vessels (neovascularity) ➢ BBB abnormality ➢ vasogenic edema ➢ contrast enhancement ➢ irregular vessels, shunting, etc. HETEROGENEOUS ➢ hemorrhage (old/new) ➢ tumor necrosis ➢ tumor itself

Neuroradiology

1143 1145

The WHO 2000 Brain Tumor Classification

Astrocytoma Gr4: Angiogenesis

Figure 5-10-24

Glioblastoma Multiforme [Figures 5-10-24 and 5-10-25] Figure 5-10-25 T2

T1-gad

Glioblastoma Multiforme. Photomicrograph at high power shows both angiogenesis and pseuopalisading necrosis

Glioblastoma Multiforme

Figure 5-10-26 (Gr 4) Glioblastoma: PWI-CBV [Figure 5-10-26] Glioblastoma – WHO Grade 4 Mechanisms of Enhancement Ultrastructure of BBB •

Neural capillary ➢ astrocytic feet ➢ continuous BM ➢ tight junctions ➢ no pinocytosis

ABBB or Non-neural no astrocytic feet fenestrated BM intercellular gaps pinocytosis Increased perfusion due to angiogenesis in a glioblastoma multiforme

GBM •

• •

Center of Abnl Density/Intensity ➢ variegated necrosis ENHANCING RIM ➢ hypercellular, fleshy neoplasm ➢ greatest neovascularity Corona of Abnl Density/Intensity ➢ “edematous” white matter ➢ areas of microscopic neoplastic infiltration

Figure 5-10-27

GBM - Glioblastoma Glioblastoma multiforme with pseudopalisading necrosis

Pseudopalisading Necrosis [Figure 5-10-27] Ring Lesion and Infiltration [Figure 5-10-28]

Figure 5-10-28

Schematic of Glioblastoma multiforme – there are neoplastic cells infiltrating into the surrounding white matter The WHO 2000 Brain Tumor Classification

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Neuroradiology

Glioblastoma Multiforme (Surrounding Zone of Infiltration) •

•

Figure 5-10-29

GBM arose from a preexisting low grade ➢ surrounding lower grade neoplasm ➢ may also transform over time GBM arose de novo ➢ sends cells to invade the brain

Terrorist Cells Infiltrate Brain Glioblastoma Multiforme GBM - Multifocal [Figure 5-10-29] DWI of Glioblastoma (Gr 4) Multifocal glioblastoma multiforme. Microscopic infiltration does not always produce macrosopic changes that are visible on CT, MR, or even at gross pathology

Ring Enhancing Mass Ring Lesion Differences [Figure 5-10-30]

Figure 5-10-31

Figure 5-10-30

Glioblastoma Multiforme vs. Abscess (toxoplasmosis). The abscess rim is thinner, without a shaggy inner margin

Glioblastoma Multiforme vs. Abscess (toxoplasmosis). The viscous pus and white cell infiltrate in the abscess causes restricted diffusion – bright on DWI

DWI: Necrosis vs. PUS [Figure 5-10-31] Glioblastomas: Growth/Spread

Figure 5-10-32 Glioblastoma: Ependymal spread GBM [Figure 5-10-32] GBM – Thicker on Surface

Two different patients with GBM – the tumor flourishes when it reaches the rich vascularity of the cerebral cortex

Neuroradiology

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The WHO 2000 Brain Tumor Classification

X-Ray Perfusion Imaging [Figure 5-10-33]

Figure 5-10-33

MR Perfusion Imaging [Figure 5-10-34] GBM with increased rCBV New Tools for Grading and Staging •

•

Radiology ➢ Perfusion Imaging rCBV and rCBF ➢ Diffusion Imaging, ADC and DTI ➢ Spectroscopy ➢ PET/SPECT ➢ Monoclonal Ab. Pathology ➢ Labeling Index ➢ Chromosome Analysis ➢ Histochemical ➢ Electron Microscopy

Glioblastoma multiforme. The early draining veins reflect increased perfusion and a shortened mean transit time (MTT)

Figure 5-10-34

DTI and Tumor Imaging Astrocytes Track Along WM Glioblastoma Multiforme Two Port Radiotherapy •

•

Bad News ➢ Can’t define full extent of tumor by any current test Good News ➢ 90% of tumor recurrence within 2cm of enhancing rim

Glioblastoma multiforme

Figure 5-10-35

Glioblastoma Multiforme [Figure 5-10-35] Define the Problem • • • • • •

Some Low Grade Enhance* Some Low Grade Do Not Some Low Grade => GBM Some Low Grade Do Not* *These are the Circumscribed Astrocytomas The others are the Diffuse Astrocytomas

WHO Astrocytoma Summary Butterfly glioma – Glioblastoma multiforme References 1. 2.

3. 4.

Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (1993-2002) Pediatric Neurosurgery 2005; 41:173-177 Levin VA, Leibel SA, Gutin PH. Neoplasms of the central nervous system. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology. Vol 2. 5th ed. Philadelphia, Pa: Lippincott-Raven Publishers; 1997:2022-2082. Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439. Pobereskin LH, Chadduck JB: Incidence of brain tumours in two English counties: a population based study. J Neurol Neurosurg Psychiatry 2000; 69: 464-471.

The WHO 2000 Brain Tumor Classification

1146 1148

Neuroradiology

Non-Astrocytic Gliomas James G. Smirniotopoulos, MD PRIMARY NEOPLASMS – Neuroectodermal •

•

Figure 5-11-1

Neuroectoderm – ➢ Embryologic Neural Tube ➢ “Neuroepithelial” Broad Categories ➢ Glial Tumors (GLIOMAS) ➢ Embryonal/Immature (P.N.E.T.’s) ➢ Neuronal (Neurocytoma) ➢ Mixed (Ganglioglioma)

Neuroectodermal Tumors • • • • • • • • • • • •

Astrocytoma Circumscribed Diffuse Ependymoma Choroid Plexus Tumors Oligodendroglioma Medulloblastoma (PNET) Atypical Rhabdoid Tumor Ganglioglioma Central Neurocytoma Lhermitte-Duclos Dysembryoplastic Neuroepithelial Tumor

Ependymoma – Gross Axial section

Figure 5-11-2

EPENDYMOMAS – Brain and Cord •

•

Cell of Origin: Ependyma ➢ Lining of ventricles ➢ Central canal/filum terminale ➢ “rests” in parenchyma Subtypes: ➢ Anaplastic/Malignant ➢ Immature (Ependymoblastoma) ➢ Myxopapillary (cauda equina)

EPENDYMOMA •

WHO Classification ➢ Ependymoma (WHO grade II) ❖ Variants: cellular, papillary, clear cell, tanycytic, mixed ➢ Anaplastic ependymoma (WHO gr III) ➢ Myxopapillary ependymoma ➢ Subependymoma

Ependymoma – heterogeneous central mass

Figure 5-11-3

EPENDYMOMAS – Demographics • • • • • •

5%–6% of All Intracranial 70% occur in Males 70% arise in the Fourth Ventricle 70% present in Childhood 70% of All Intramedullary 5 year survival – 50%

EPENDYMOMAS – Gross Pathology •

[Figures 5-11-1 to 5-11-5]

Soft Intracavitary Mass ➢ “cast of ventricle” ➢ extrude out foramina ➢ invade floor of 4th (pons/medulla)

Neuroradiology

Ependymoma – Enhancing and heterogeneous, small “cysts” and calcified chunks 1147 1149

Non-Astrocytic Gliomas

• •

Heterogeneous ➢ small/large cystic areas ➢ calcification (often chunks) Spinal Cord – sharply circumscribed

Figure 5-11-4

Figure 5-11-5

Ependymoma – Tumor extends into lateral recess of 4th ventricle

Figure 5-11-6

Ependymoma – Tumor extends into vallecula of cisterna magna

Schematic Ependymoma [Figure 5-11-6] Ependymoma [Figure 5-11-7] Ependymoma CPA Cistern [Figure 5-11-8] Figure 5-11-7

Schematic of Ependymoma filling lumen of fourth ventricle. Copyright 2004

Figure 5-11-8

Ependymoma in the cerebellopontine angle cistern Ependymoma filling lumen of fourth ventricle Non-Astrocytic Gliomas

1148 1150

Neuroradiology

Extra-ventricular Ependymoma • • • •

Figure 5-11-9

[Figures 5-11-9 and 5-11-10]

•

More common in children More common in cerebral hemisphere Arise from *rests* of Ependymal Cells Better prognosis ➢ Not infiltrating ➢ Not communicating with CSF Often partially cystic

CHOROID PLEXUS NEOPLASMS – Introduction •

•

Normal Choroid Plexus (CP) ➢ Makes majority of CSF ➢ Forms papillary fronds: ❖ Vascular core ❖ Ependyma is modified into CHOROID EPITHELIUM Neoplasms: ➢ CP Papilloma (benign) WHO Grade 1 ➢ CP Carcinoma (malignant) WHO Grade 3

Extra-ventricular Ependymoma – Note fluid-fluid level formed by contrast layering within the cyst

Figure 5-11-10

CHOROID PLEXUS TUMORS • •

Choroid plexus papilloma (WHO grade I) Choroid plexus carcinoma (WHO grade III)

CHOROID PLEXUS NEOPLASMS – Demographics •

•

Tumor of Childhood: ➢ Uncommon (<1% of CNS) ➢ Intrauterine/congenital ➢ 40% < 1 year old ➢ 86% < 5 years Location: ➢ TRIGONE in children ➢ 4th vent. In adults ➢ Less often 3rd and CPA

CHOROID PLEXUS NEOPLASMS Gross Pathology • •

Extra-ventricular Ependymoma

[Figures 5-11-11 to 5-11-13]

Lobulated intraventricular mass with papillary fronds Secondary Effects: ➢ Ventricular and SAS Enlargement ➢ Spontaneous Hemorrhage ➢ CSF Seeding ➢ Parenchymal Invasion

Figure 5-11-11

Figure 5-11-12

Choroid plexus papilloma showing innumerable papillae Neuroradiology

Photomicrograph of CPP, showing choroid epithelium and vascular core 1149 1151

Non-Astrocytic Gliomas

Figure 5-11-13

Figure 5-11-14

Choroid plexus papilloma showing innumerable papillae Choroid plexus papilloma – Note pattern of bilateral hydrocephalus

Choroid Plexus Papilloma CSF Homeostasis [Figure 5-11-15] Figure 5-11-15

CSF Homeostasis

Normal Ventricular System: Lateral Ventricles All Ventricles Enlarged? •

Over Production vs. Under Resorption

Figure 5-11-16

Congenital CPP Choroid plexus papilloma [Figure 5-11-16] OLIGODENDROGLIOMA •

• • • • •

Cell of origin ➢ Oligodendrocyte ➢ Makes central myelin ➢ 51% - 90% oligos, remainder astrocytes 1%–8% of ALL CNS primary Adults > Children (8:1) Age peak 35 – 45 yrs) Supratentorial – 85% Slow growth, Long Hx (10 years) ➢ Prognosis better with 1p and 19q mutations

Choroid plexus papilloma – Note pattern of bilateral hydrocephalus Non-Astrocytic Gliomas

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Neuroradiology

OLIGODENDROGLIOMA • • •

•

Figure 5-11-17

Gr 1 - Rare Gr 2 - Conventional oligodendroglioma Gr 3 - Anaplastic oligo: ➢ Hypercellularity, atypia, mitoses, endothelial proliferation, necrosis Gr 4 - GBM-like (rare) ➢ Not biologically equivalent to Gr 4 fibrillary astrocytoma

OLIGODENDROGLIOMA – Gross Pathology • • • • •

•

Arises in White Matter Grow toward cortex! Unencapsulated Not as infiltrating as astro. Heterogeneous ➢ myxoid areas (“cystic”) ➢ hemorrhage C A L C I F I C A T I O. N !

OLIGODENDROGLIOMA – Radiology •

• •

• •

Heterogeneous Hemispheric Mass ➢ Ca++, Cysts – “myxoid change”, Blood products Oligodendroglioma. CT shows very dense Extend to Cortex and infiltrate GM calcifications, highly suggestive and characteristic ➢ Gyriform or dot-dash Ca++ of oligodendroglioma ➢ Scalloped erosion inner table Figure 5-11-18 2/3 will enhance ➢ +/– anaplasia MR +/- special pulse sequences for Ca++ detection MR Spectroscopy? ➢ Potential for tumor grade, but not subtype or genetics

MR - What is it? Oligodendroglioma [Figure 5-11-17] •

CT Shows DENSE Ca++

Oligo-astrocytoma •

Nothing Specific … Looks like Diffuse Astrocytoma Oligodendroglioma – Heterogeneous peripheral mass that involves the cortex with thick curvilinear calcifications

Oligodendroglioma [Figure 5-11-18] •

CT Shows DENSE Ca++

Chickenwire Vascularity [Figure 5-11-19]

Figure 5-11-19

Fried Egg: round dark nucleus surrounded by a clear halo – this is an artifact of fixation Chicken-wire: The capillary vessels form a "chicken wire“ pattern around nests of cells Neuroradiology

1151 1153

Non-Astrocytic Gliomas

Oligodendroglioma •

Figure 5-11-20

Combined 1p/19q loss ➢ Associated with prolonged survival ➢ Response to PVC (procarbazine, vincristine CCNU® [Lomustine]) chemotherapy ➢ 50% volume decrease in 100% ➢ Median survival 10 yrs vs 2 yrs ❖ 95% 5 yr survival ➢ Most powerful predictor on multivariate analysis

Cairncross et al. J NCI 1998;90:1473 External granular cell layer – normal in fetus and infants (up to 12 months old)

Old Elephants Age Gracefully Oligo Epend Astro GBM •

90% 50% 25% 15% (Incidence of Ca++)

Oligodendroglioma MEDULLOBLASTOMA [Figure 5-11-20] •

•

•

Cell of origin: ➢ “medulloblast” – NOT! Bi-potential embryologic cells: ➢ Migrate from 4th to form CRBLL ➢ Glial and neuronal differentiation ➢ External Granular Cells (fetus) ➢ Internal Granular Layer (mature) Primitive Neuroectodermal Tumor - PNET

MEDULLOBLASTOMA – Demographics •

• • •

1st or 2nd most common cerebellar neoplasm in children ➢ 1/5 – 1/3 of ALL pediatric CNS ➢ M:F 1.1 – 2:1 May be congenital (present at birth up to 60 days) ➢ most (1/2) < 15 yrs. ➢ however, 1/3 present from 15–35 yrs. 5 year survival >> 50% => approached 75%-85% Primitive Neuroectodermal Tumor

Pediatric Posterior Fossa • • •

454 posterior fossa patients All under the age of 18 402 tumors: ➢ 37.1% Cerebellar astrocytoma (149) ➢ 34.6% Medulloblastoma (PNET) (139) ➢ 11.4% Brain stem astrocytoma (46) ➢ 7% Ependymoma (28) ➢ 9.9% "other" (40)

Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439.

Childhood CNS Tumor Demographics •

367 Syrian children, collected from 1993-2002 ➢ Supratentorial - 47% ➢ Infratentorial - 53% ➢ Male 52%:48% Female ➢ Overall Incidence: ❖ Medulloblastoma 27% ❖ Astrocytoma 26% ❖ Craniopharyngioma 14% ➢ Posterior Fossa Only ❖ Medullo (PNET) 54%

Non-Astrocytic Gliomas

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Neuroradiology

❖ ❖

Astrocytoma 23% Ependymoma 17%

Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (19932002) Pediatric Neurosurgery 2005; 41:173-177

Figure 5-11-21

MEDULLOBLASTOMA – Gross Pathology •

[Figures 5-11-21 and 5-11-22]

•

Arise from: ➢ post. (inf.) Medullary Vellum ➢ Vermis (midline cerebellum) Morphology: ➢ expansile, spherical, Unencapsulated ➢ post. 4th ventricle ➢ residual ANT. Crescent of CSF ➢ “HOMOGENEOUS” ❖ (Ca++. Cyst. Heme are UN-common) Medulloblastoma (PNET) – Rounded mass in the central posterior fossa

Figure 5-11-22

Figure 5-11-23

Medulloblastoma – a small, round, blue-cell tumor

RADIATION CHEMOTHERAPY • • •

Dividing Cells Neovascularity Pharmaceuticals ➢ Tested against murine leukemia ➢ Small round blue cell tumor ➢ Cis-platinum ❖ Dividing cells ❖ Electrical field ❖ Platinum electrodes

MEDULLOBLASTOMA – Micro Pathology •

Small Round “BLUE CELL” Tumor ➢ Immature, high Nuclear:Cytoplasm ➢ Both Neuronal and Glial features (occasional astrocytic differentiation) ➢ Form “Rosettes” (Homer-Wright) cells arranged in a circle surround core with linear fibrils ➢ Densely cellular ➢ Necrosis/Hemorrhage are not rare

Medulloblastoma (PNET) – Rounded mass in the central posterior fossa

MEDULLOBLASTOMA – Radiology [Figure 5-11-23] • •

•

Post. Fossa, Behind/in 4th vent. “HOMOGENEOUS” (he’s lying!) ➢ grossly uniform, ❖ but, finely heterogeneous ➢ hyperdense on CT (w/o Ca++) ➢ hypo-/isointense to GM on MR (cellularity and high N:C ratio) ➢ (Ca++ and “cysts” < 15%) Enhance with contrast

Neuroradiology

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Non-Astrocytic Gliomas

Fourth Ventricle - Schematic [Figure 5-11-24]

Figure 5-11-24

Medulloblastoma (PNET) [Figures 5-11-25 and 5-11-26] Figure 5-11-25

Schematic of posterior fossa masses. Can you really determine where the mass originated when it is more than three centimeters in diameter? Copyright 2004

Medulloblastoma (PNET) – Rounded mass arising in the cerebellum – not from the 4th ventricle roof

Figure 5-11-26

Medulloblastoma •

[Figure 5-11-27]

• • • •

‘Homogeneous’ ➢ finely irregular Cyst and Hemorrhage are uncommon <10% Hyperdense on NCT ➢ up to 75% ➢ densely cellular ➢ sm. Round blue-cells Center is behind 4th vent Rounder not angular

Medulloblastoma (PNET) – Rounded mass in the central posterior fossa

Figure 5-11-27

Ependymoma - Schematic POSTERIOR FOSSA [Figure 5-11-28] •

INCIDENCE/LOCATION: ➢ Medulloblastoma (PNET) (1/4 - 1/3) ❖ Post. To IVth ➢ Brainstem glioma (1/6) ❖ Ant. To IVth ➢ Ependymoma (1/6) ❖ Inside IVth ➢ Pilocytic Astrocytoma (1/4 - 1/3) ❖ Lat. And/or post. IVth ❖ these are often a cyst w / nodule Medulloblastoma

Medulloblastoma (PNET) [Figure 5-11-29]

Figure 5-11-28

“Zuckerguss” or Sugar Icing (CSF dissemination) CSF DISSEMINATION •

Neuroectodermal: ➢ PNET (medulloblastoma) ➢ GBM (reaches ventricle or pia) ➢ Ependymoma ➢ Oligodendroglioma (micro curiosity - no Sx) ➢ CPP and CPC Medulloblastoma (PNET) – Rounded mass in the central posterior fossa

Non-Astrocytic Gliomas

1154 1156

Neuroradiology

•

Non-glial: ➢ Germinoma ➢ Lymphoma (usually secondary) ➢ Leukemia ➢ Carcinomatous Meningitis

Figure 5-11-29

CSF Spread - Zuckerguss [Figure 5-11-30] Lateral Medulloblastoma [Figure 5-11-31] Medulloblastoma - Desmoplastic • •

CSF spread of Medulloblastoma (PNET)

Lateral Hemispheric Location Older Patients More peripheral

Figure 5-11-30

Cerebral Neuroblastoma [Figure 5-11-32] Summary •

• • •

Ependymoma ➢ Intraventricular, soft, heterogeneous Choroid Plexus Papilloma ➢ Very young, Hydrocephalus ➢ Very Small Papillae and Lobulations Oligodendroglioma ➢ Superficial, skull remodeling ➢ Dense Calcification: dot-dash and linear Medulloblastoma (PNET) ➢ Hyperdense on CT ➢ Central posterior fossa

References 1.

2. 3.

4.

CSF spread of Medulloblastoma (PNET) Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (1993-2002. Pediatric Neurosurgery Figure 5-11-31 2005; 41:173-177 Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439. Jenkinson MD, Smith TS, Joyce K, Fildes D, du Plessis DG, Warnke PC, Walker C MRS of oligodendroglial tumors: correlation with histopathology and genetic subtypes. Neurology. 2005 Jun 28;64(12):2085-9. Cairncross JG, Ueki K, Zlatescu MC, et al. Specific genetic predictors of chemotherapeutic response and survival in patients with anaplastic oligodendrogliomas. J Natl Cancer Inst 1998; 90:1473-1479. Lateral Medulloblastoma – Desmoplastic variant

Figure 5-11-32

Cerebral Neuroblastoma – PNET

Neuroradiology

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Non-Astrocytic Gliomas

Extraaxial Tumors: Other Non-Glial Lesions James G. Smirniotopoulos, MD CHORDOMA [Figure 5-12-1] •

Rich Corinthian Leather vs. Physaliphorous Cells “Bubble” or Vacuolated

Figure 5-12-1

CHORDOMA: Normal Notochord • •

Nucleus Pulposis of Intervertebral Disk Ecchordosis of Clivus ➢ Size/Shape like a grain of rice ➢ Dorsal to clivus

Notochord Embryology Sclerotomes & Notochord [Figure 5-12-2] Figure 5-12-2

Chordoma - Physaliphorous Cells (Courtesy of Joe Parisi, M.D.)

Schematic of sclerotomes surrounding the notochord. Two adjacent sclerotomes fuse into one vertebral body. The notochord tissue is extruded into the intervertebral disc, forming the nucleus pulposis. Copyright 2003

Chordoma Notochord: Chordoma & Thornwaldt [Figure 5-12-3] CHORDOMA • ORIGIN: Notochordal Rests • AGE: (30–60)

•

Figure 5-12-3

LOCATION: ➢ Clivus 35% ➢ Spine 15% (esp. Cx) ➢ Sacrum 50%

CHORDOMA: Imaging •

• •

Location: midline clivus ➢ Extend lateral, dorsal , ventral NCT: Bone destruction ➢ Heterogeneous ➢ “Cysts”, Ca++ ECT: Heterogeneous

Extraaxial Tumors: Other Non-Glial Lesions

Chordoma (left) and Thornwaldt cyst (right). The orange line is the embryologic location of the notochord, the green triangle is the clivus

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Neuroradiology

Chordoma - Imaging •

• • • • • •

Figure 5-12-4

Location ➢ Midline Clivus ❖ May extend lateral, dorsal , ventral ➢ Midline Sacrum Lobulated Heterogeneous Bulky Mass Bone destruction NO remodeling Variable Density/Signal Bone sequestra Dystrophic calcifications

Clivus is Missing [Figure 5-12-4] Chordoma Chordoma – Destruction of the clivus, the basilar and vertebral arteries are stretched over a large bulky, hypovascular mass. Air and contrast remain from an earlier myelogram

Eccentric Growth [Figure 5-12-5] Figure 5-12-5

Figure 5-12-6

Chordoma with eccentric growth, there is a sharp margin between the tumor and brain, with a thin hypointense line – the dura

Chordoma [Figures 5-12-6 to 5-12-8] Chordoma – destruction of the sacrum and bulky soft-tissue mass. This patient presented with symptoms of constipation and rectal fullness

Figure 5-12-7

Figure 5-12-8

Chordoma – destruction of the sacrum and bulky soft-tissue mass. There are devitalized fragments of residual bone – sequestra – from the sacrum Chordoma – destruction of the sacrum and bulky soft-tissue mass

Neuroradiology

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Extraaxial Tumors: Other Non-Glial Lesions

T1 pre and post, T2 [Figure 5-12-9]

Figure 5-12-9

Chordoma Eccentric Mass [Figure 5-12-10] Chondrosarcoma [Figure 5-12-11] Figure 5-12-10 Sacrococcygeal chordoma

Figure 5-12-11

Chondrosarcoma – low grade. Notice the dense mineralization and the eccentric location of the mass

DERMOID/EPIDERMOID • • •

True Cysts Inclusion Cysts Lined by an Epithelium Chondrosarcoma

TRUE CYSTS OF THE CNS • • • • • • •

Epidermoid Dermoid Colloid Craniopharyngioma Rathke Cleft Ependymal Endodermal

TRUE CYSTS • •

A fluid filled space, lined by an epithelium. Classified by the type of epithelial lining: ➢ Epidermoid ❖ squamous epithelium - ectoderm ➢ Dermoid ❖ squamous and dermal adnexa - ectoderm ➢ Colloid cyst ❖ ciliated cuboidal/columnar epithelium, mucus secreting cells - similar to endoderm ? ❖ Craniopharyngioma (two types) ❖ Adamantinomatous - children, cystic, calcified ❖ Squamous and papillary - adult, solid ➢ Rathke Cleft cyst ❖ ciliated cuboidal/columnar epithelium - possibly endoderm ? ➢ Teratoma - a neoplasm of multipotential germ cells

NON-GLIAL MASSES •

Dermoid and Epidermoid ➢ MYTH OF THE MESODERM

Extraaxial Tumors: Other Non-Glial Lesions

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MYTH OF THE MESODERM •

• •

Figure 5-12-12

One germ cell layer = epidermoid ➢ Ectoderm Two germ cell layers = dermoid ➢ Ectoderm and Mesoderm Three germ cell layers = teratoma ➢ Ectoderm, Mesoderm, Endoderm

DERMOID/EPIDERMOID: Histology • • •

1. Epidermoid – Squamous Epithelium - ONLY 2. Dermoid – Sq. Epi PLUS Dermal Appendages (hair, sebaceous, sweat glands, etc.) 3. Teratoma – Complex tissues, 2 or more germ layers (often mainly ectoderm, “benign cystic”)

EPIDERMOID [Figure 5-12-12] • • • • •

•

AGE: 4 – 6TH Decade Location: Midline or lateral (CPA) Composition: Sq. epithelium, keratin Thin wall, no Ca++ or Vascularity NCT: Lipid to Brain ➢ Ca++/enhance. rare MRI: Hetero., CSF to Brain ➢ NOT bright on T1W ➢ **Fluid/Fluid Level RARE

Epidermoid Inclusion Cyst. Note: the lesion is nearly, but not exactly, like CSF in signal. The sagittal image demonstrates internal layers of keratin

Figure 5-12-13

Pearly Tumor Epidermoid - Dry Keratin [Figures 5-12-13 and 5-12-14] Epidermoid [Figures 5-12-15 and 5-12-16]

Epidermoid Inclusion Cyst - Dry Waxy Keratin

Figure 5-12-14 Figure 5-12-15

Squamous epithelium and flaky or dry keratin Epidermoid Inclusion Cyst. On T2W images the lesion is hyperintense – like CSF. However, the axial image on the right shows some internal structure

Figure 5-12-16

Epidermoid – Gd+ T1W

Midline posterior fossa/fourth ventricle

Neuroradiology

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Extraaxial Tumors: Other Non-Glial Lesions

Epidermoid [Figures 5-12-17 and 5-12-18]

Figure 5-12-18

Figure 5-12-17 T1W Gd

FLAIR

Epidermoid Inclusion Cyst

Figure 5-12-19

Epidermoid Inclusion Cyst. Faint peripheral enhancement from gliosis. Internal wisps from layers of keratin, and marked hyperintensity on the FLAIR image

DERMOID • • • • •

•

AGE: 3rd Decade Location: Midline Composition: Sq. epi. & appendages Thick wall, Ca++ & Vascularity NCT: Lipid to Brain, Fluid/Fluid ➢ Ca++/enhance. often MRI: Hetero., Lipid to Brain ➢ Bright on T1W ➢ **Dysraphism, Sinus tract

Dermoid with hair and sebaceous material

Figure 5-12-20 Dermoid Inclusion Cyst Dermoid [Figures 5-12-19 and 5-12-20] Ruptured Dermoid Dermoid - rupture [Figure 5-12-21] Figure 5-12-21 Epidermal surface, but with sebaceous glands, hair shafts and follicles – making this a dermoid inclusion cyst

Ruptured Dermoid Inclusion Cyst. Notice the “hairball” at the lipid-CSF interface

Extraaxial Tumors: Other Non-Glial Lesions

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Neuroradiology

Body Soil “As gross as it is, the average person excretes up to 50 grams of body soil per day! This is because on a normal day we each use 1 liter of sweat, eliminate 1 billion dead skin cells, and run off 10 grams of sebum, otherwise known as body oil.”

Figure 5-12-22

Clorox Commercial, May 2005 (http://www.clorox.com/health_body_soil.html)

Intradiploic Epidermoid [Figure 5-12-22] COLLOID CYST •

A benign mass, in a Malignant Location

COLLOID CYST [Figure 5-12-23] • • • • •

“Paraphyseal” cyst ependymal cyst, choroid cyst Congenital lesion Cuboidal, low columnar epithelium Scant connective tissue Foramen of Monro

Intradiploic Epidermoid Inclusion Cyst

Figure 5-12-23

COLLOID CYST [Figures 5-12-24 to 5-12-26] • •

•

Location: Foramen of Monro CT: sharply demarcated ➢ hyperdense to hypodense ➢ < half enhance MR: sharply demarcated ➢ T1W – iso. to bright ➢ T2W – bright to dark ➢ NOTE: Dark Cysts are too thick for Stereotactic Aspiration

Colloid cyst

Figure 5-12-25

Figure 5-12-24

Colloid Cyst – in the characteristic anterior 3rd ventricle location, and causing obstructive hydrocephalus

Colloid cyst

Figure 5-12-26

Aqueous Protein Solution Colloid Cyst – Black Hole

Colloid Cyst: two different patients – one cyst is markedly hyperdense, the other is hypodense. Why? Variable protein and viscosity

Neuroradiology

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Extraaxial Tumors: Other Non-Glial Lesions

Neoplasms of the Meninges James G. Smirniotopoulos, MD Educational Objectives • • •

• •

Meningioma is the most common non-glial primary tumor Meningioma is the most common extraaxial neoplasm Most meningiomas have “typical imaging” ➢ Hemispheric, homogeneous, broad based on the dura, hyperostosis, hormonally sensitive Some meningiomas have “atypical imaging” Hemangiopericytoma is NOT a meningioma

Meningioma • • •

• •

75% are histologically typical 75% are radiologically typical Not the same 75% ➢ CT ➢ MR ➢ Angiography Atypical Imaging =/= Atypical Histology “The atypical appearance of a common lesion may be seen more often than the classic appearance of an uncommon lesion.”

Primary Meningeal Neoplasms •

• • •

MENINGIOMA: ➢ Meningioma (typical and metaplastic ➢ Atypical Meningioma ➢ Anaplastic (Malignant) Meningioma ❖ Papillary Meningioma MESENCHYMAL (non-meningothelial) Primary MELANOCYTIC Lesions UNCERTAIN Origin ➢ Hemangiopericytoma (pericyte) ➢ Hemangioblastoma (mesenchyme)

Meningeal Tumors: WHO Grades •

• • • •

91% of Meningioma - Grade 1 ➢ Includes most subtypes / metaplastic changes ➢ Transitional, fibroblastic, meningothelial 8.3% are ATYPICAL Meningioma - Grade 2 HEMANGIOPERICYTOMA – Grade 2/3 PAPILLARY Meningioma - Grade 3 <1% are ANAPLASTIC Meningioma - Grade 3

Sandhyamani, Rao, Nair, Radhakrishan: Atypical Meningioma: A Clinicopathological Analysis.Neurology India 2000; 48: 338-342

Meningioma – Benign Subtypes – WHO I • • • • • •

SYNCYTIAL (Meningothelial) FIBROBLASTIC (Fibrous) TRANSITIONAL (Features of Both) PSAMMOMATOUS Microcystic (Humid), Secretory METAPLASTIC FEATURES ➢ Lipoblastic, Osteoblastic, Chondroblastic ➢ Myxoid, Xanthomatous, Melanotic

Neoplasms of the Meninges

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Neuroradiology

Meningiomas – Cell of Origin • •

Dural Fibroblast ? – No Arachnoid Cap Cell ➢ “Meningothelial cell” ➢ Arachnoid granulations ➢ Dural sinuses ❖ Sup. Sag. ❖ Sphenoparietal

Meningioma – Etiologic Factors • • • • •

Trauma Radiation Viruses Familial (Non-NF2) Meningioma Neurofibromatosis – Type - 2 ➢ MISME Syndrome

Meningioma – Radiation •

•

Low Dose (<800 cGray) ➢ Immigrants to Israel (1940’s) ➢ Tinea Capitis (ringworm) ➢ Superficial radiation ➢ High Incidence of Meningioma High Dose (>2000 cGray = 2000 RADS) ➢ Used for Skull Base Tumors ➢ Pituitary Adenoma

Meningioma – Molecular Biology • •

• • •

Postulate Tumor suppressor Gene Chromosome 22 – deletion in tumor cells ➢ both sporadic and inherited ➢ w/ or w/o NF-2 Homozygous for TWO wild-type copies is normal Heterozygous for 22 develops neoplasm ➢ Because there is a subsequent loss of the OTHER wild-type gene Inherited (germline) deletion of 22 ➢ W/Schwannoma = NF2

Meningiomas •

• • •

1/7 to 1/4 of all Intracranial Primary ➢ ~ 6/ 100k / year ➢ small ones in ~ 1.4% of autopsies 1/4 – 1/3 of all Intraspinal Tumors Middle age (40–60) Your current Age + Ten Years Female > Male ➢ Cranial 2–4:1 ➢ Spinal 4–8:1 ➢ Progesterone receptors in 2/3 ➢ Estrogen receptors less common

Figure 5-13-1

Morphology [Figure 5-13-1] • •

Globose (spherical, hemispherical) En plaque (like a flat bread) ➢ Pancake ➢ Crepe ➢ Wonton wrapper ➢ Tortilla ➢ Pita (Greece and Middle East) ➢ Naan (India) ➢ Injera (Ethiopia) ➢ Bolo de milho (Brazil)

Neuroradiology

Meningioma - parasagittal

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Neoplasms of the Meninges

Incidental Meningioma [Figure 5-13-2]

Figure 5-13-2

Not Incidental En Plaque Meningioma [Figure 5-13-3] Meningioma – Location • • •

• •

Parasagittal Convexity Ant. Basal ➢ Sphenoid ➢ Olfactory ➢ Suprasellar Tentorial Ventricular

25% 20% 40% 20% 10% 10%

Small incidental meningioma of the tentorium cerebelli

10% 5%

Figure 5-13-3

Meningioma – CT Imaging [Figure 5-13-4] •

•

Non-Contrast ➢ Sharply Circumscribed ➢ Homogeneous ➢ Hyperdense (+/– Ca++) ➢ NOT from psammoma bodies ! ➢ Broad Dural Surface ➢ Bone Changes (Hyperostosis) Enhanced CT ➢ Homogeneous Enhancement

Figure 5-13-4

En plaque meningioma, on the CT this blends into the bone

Figure 5-13-5 Meningioma – with classic features of hyperdensity and hyperostosis

Meningioma – CT Findings (193 pts) •

BENIGN Meningiomas: ➢ Homogeneous Enhancement ➢ Heterogeneous Enhancement ➢ Calcification ➢ Hyperostosis ➢ Midline Shift (large) ➢ “Mushrooming”

72% 23% 27% 18% 72% 0%

J. Neurosurg 71:665–672,1989

Psammomatous Meningioma

Psammomatous Meningioma [Figure 5-13-5]

Neoplasms of the Meninges

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Neuroradiology

Spreading Vasogenic Edema

Figure 5-13-6

Meningioma – Vasogenic Edema •

• • •

VASCULAR ➢ Parasitization of MCA, etc. ➢ Compression of cortical aa./vv. COMPRESSIVE TRAUMA SECRETORY EFFECT “Evil Humors” “TRANSCORTICAL FLOW” ➢ Close apposition of tumor to brain ➢ Thinned cortex ➢ +/- infiltration of brain ➢ Fluid gradient from meningioma into brain

Meningioma. WHO Grade 1 – benign, yet with extensive vasogenic edema

Meningioma and Edema [Figures 5-13-6 and 5-13-7]

Figure 5-13-7

Edema and Prognosis • • • •

• •

Edema =/= Histology Edema =/= Size Edema =/= Vascularity Edema IS Related to Resectability ➢ Smaller “pseudocapsule” ➢ Surgical “cleavage plane” ➢ Tumor “sticks” to underlying brain Resectability IS Related to Prognosis Edema IS INDIRECTLY Related to Prognosis

Meningiomas – MR Imaging •

• •

“Meningiomas are ISO-intense.” ➢ Usually on T1W ➢ Vary pulse sequence to see EXTRA-axial Features ➢ Gray-matter buckling ➢ Pseudo-capsule of vessels ➢ Meningeal/dural “TAIL” GADOLINIUM ENHANCEMENT

Bar graphs showing high frequency of vasogenic edema with meningioma

Meningioma – Isointense to GM [Figure 5-13-8]

Figure 5-13-9

Figure 5-13-8

Small peripheral round lesions, nearly isointense to gray-matter on T1W MR

Meningioma - CT Meningioma w/Gd+ [Figure 5-13-9]

Neuroradiology

Meningioma - Small peripheral round lesions, nearly isointense to gray-matter on T1W MR

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Neoplasms of the Meninges

Meningioma [Figure 5-13-10] • • • • • •

Figure 5-13-10

Meningioma Tentorium or Dura IAC Normal Hemispherical Homogeneous Hyperostosis

Tentorial Meningioma [Figure 5-13-11] Meningioma - Pseudocapsule Meningioma – Pre and Post Gd

Meningioma. Cerebellopontine angle cistern. This mass is hemispheric, and the enhancement does not involve the IAC (internal auditory canal) (Courtesy of Bob Peyster, MD)

MR Signal and Meningioma Types •

• • •

Most are Isointense to GM ➢ On Both T1W & T2W Hyperintense to GM on T1W ➢ “Lipoblastic” (Fatty) Meningioma ➢ Hemorrhage into Meningioma Hypointense on T2W ➢ Fibroblastic ➢ Transitional Hyperintense on T2W ➢ Meningothelial ➢ “Angioblastic” ➢ Microcystic (“Humid”) ❖ Some Good, Some “bad”

Figure 5-13-11

Meningioma - Pseudocapsule [Figure 5-13-12] Meningioma – Dural Tail [Figure 5-13-13]

Tentorial Meningioma

Figure 5-13-13

Figure 5-13-12

Meningioma – Pseudocapsule of CSF and vessels; and internal serpentine hypointensities

TMeningioma – Pseudocapsule of CSF and vessels; and internal serpentine hypointensities dural tail

Neoplasms of the Meninges

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Neuroradiology

DURAL TAIL • • • • •

Curvilinear enhancement “dural flair” First reported w/meningioma First reported to be neoplastic invasion What is it REALLY? ➢ Thickening of the dura ➢ Vasocongestion of the dura ➢ Edema of the dura

Dural tail: Histology Dural Tail: Differential Dx •

•

Figure 5-13-14

Extraaxial Lesions ➢ Meningioma ❖ Most common lesion w/dural tail ❖ Most Common Lesions Overall ➢ Schwannoma ➢ Hemangiopericytoma ➢ Sarcoidosis ➢ Gumma (syphilis) Intraaxial Lesions ➢ Pleomorphic Xanthoastrocytoma ➢ Superficial cerebral astrocytoma ➢ GBM (rare)

Cavernous Sinus Meningioma with extensive dural enhancement along the tentorium

Whorls of Spindle Cells Cavernous Sinus Meningioma [Figure 5-13-14] Meningioma: Named by region • •

Tentorial / Pineal Clival

Dural Tail - Schwannoma MENINGIOMA – *Imaging Features: CT vs. MR • • • • • • • • •

Mass effect Extraaxial Location Broad Dural Attach. Typical. Dense./Intensity Hyperostosis Homogeneous Enhancement (Homogeneous) Meningeal. Enhance (tail) “Capsule”

CT 88% 42% 74% 92% 10% 76%

MR 90% 70% 98% 74% 14% 76%

96%(78%) 2% 14%

96%(80%) 50% 68%

*Neuroradiology 1990;32:467–473

Meningioma - Transdural Other Locations for Meningioma • •

•

Intraventricular Orbit ➢ Intraconal ➢ Periorbital Nasal Cavity

Neuroradiology

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Neoplasms of the Meninges

Intraventricular: ~ 5% [Figures 5-13-15 and 5-13-16] • • • • • •

Figure 5-13-15

Usually Adult F>M Usually Lateral Ventricle Usually Trigone/Atrium ALWAYS attached to Choroid Plexus Vascular pedicle from choroid

Optic Nerve Meningioma Meningioma – Angiography - Supply •

• •

External Carotid 85% ➢ Some have dual supply Internal Carotid 63% Tumor Blush 95%

Intraventricular Meningioma

Figure 5-13-16

Meningioma – Angiography Transit Time [Figure 5-13-17] •

•

Blush or Stain ➢ early arterial ➢ prominent in VENOUS phase ➢ capillaries/sm. arterioles ➢ (too small to see individually) Venous Filling ➢ characteristic if delayed ➢ may fill with/ before NI. veins Normal Choroid Plexus: Nests of arachnoid cap cells

Spoke Wheel Vessels Meningioma – Angiography – Transit Time •

[Figures 5-13-18 and 5-13-19]

•

Figure 5-13-17

Venous Filling (Stattin, 1996) ➢ 170 Meningiomas ➢ delayed in 136 (80%) ➢ with Nl. vv. In 10 (6%) ➢ earlier in 24 (14%) ➢ (8 in early arterial phase) Leeds & Taveras (1969) ➢ EDV in 6/36 (16%)

Figure 5-13-18

Meningioma – Supplied by branches of the ECA, showing classic spoke-wheel vascularity

Meningioma – Venous phase, showing persistent and dense tumor blush

Neoplasms of the Meninges

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Neuroradiology

Meningioma – Pre-Op Embolization • •

Figure 5-13-19

Gd Pre Embo Gd Post Embo

AJNR Editorial - September 2003; 24: 1499 - 1500

Meningioma – Angiography – Tumor Blush • • • • •

May come early Usually very DENSE Stays way too long (Persistent!) Derek Harwood-Nash:The “In-Law Effect”

Meningioma – Effect on Skull •

• •

Hyperostosis (15%-25%) ➢ w or w/o micro invasion Pressure Erosion ➢ Periosteal remodeling Bone Destruction ➢ microscopic invasion

HYPEROSTOSIS IN MENINGIOMAS

MR Perfusion study, showing delayed washout from meningioma. (Courtesy of Dra. Perla Salgado, Mexico City, Mexico)

[Figures 5-13-20 to 5-13-22]

Figure 5-13-20

Figure 5-13-21

Variable patterns of hyperostosis from meningioma

Meningioma Hyperostosis • •

•

Variable patterns of hyperostosis from meningioma

Does NOT mean invasion of bone Implies CHRONICITY ➢ and benign behavior Skull Base ➢ Invasion via HAVERSIAN CANALS

Figure 5-13-22

Hyperostosis from meningioma Neuroradiology

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Neoplasms of the Meninges

Meningioma – Atypical Imaging (Yet, typical Histology) [Figure 5-13-23] • • • • • • •

Figure 5-13-23

Focal Lucency Outside (arachnoid cyst) Focal Lucency Inside (necrosis, cyst) Hypodensity (“humid”, lipoblastic) Focal Hyperdensity (metaplasia, hemorrhage) Heterogeneous Hyperintensity on T1W Hyperintensity on T2W

Fatty Metaplasia Fatty (Lipoblastic) Meningioma Fatty Metaplasia [Figure 5-13-24]

Meningioma with typical histology – yet aggressive radiologic appearance

Meningioma – Cysts •

• •

Figure 5-13-24

Inside of neoplasm ➢ (rim enhancement) Between tumor and brain ➢ (”arachnoid cysts”) Inside Brain ➢ PIA separates tumor from brain ➢ ?? Results of chronic Edema ➢ Vacuolization of White Matter

Cyst and Mural Nodule Meningioma [Figure 5-13-25] Meningioma – Atypical Histology • •

• • •

WHO Grade 2 Meningioma with Fatty Metaplasia

Atypical Meningioma ~ 5%–7% Anaplastic Meningioma ~ 1%–3% ➢ ~ 0.2% / 100k per year Higher incidence of Recurrence Shorter time to Recurrence “Atypical Histology” ➢ necrosis ➢ excessive mitoses ➢ invasion into brain

Figure 5-13-25

“Malignant Meningioma” • • • • • •

< 3% of all Meningioma Anaplastic (Malignant) Meningioma Papillary Meningioma “Benign” Metastasizing Meningioma Hemangio-Peri-Cytoma (HPC) Malignant Fibrous Histiocytoma (MFH)

Neoplasms of the Meninges

Meningioma – One level shows typical hemispheric shape, the next shows a peritumoral “cyst”

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Neuroradiology

Hemangiopericytoma [Figure 5-13-26] • • • • • •

Figure 5-13-26

Syn: “angioblastic meningioma” Cell of Origin – perivascular pericyte of Zahn and/or Zimmerman < 1% of primary CNS M 1.4:1 F Age – 40’s Dural based, bone destruction, lobulated

Hemangiopericytoma – (HPC) • • • • •

•

Narrow dural base (“Mushrooming”) No Hyperostosis No Calcification in tumor Lobulated (not hemispheric) Internal Signal Voids (on MRI) ➢ irregular and multiple Hypervascular on Angio ➢ irregular patterns

Meningioma – Radiologic Features – CT • • • • •

Feature Benign / ”Malignant” Homogeneous Enhancement 72% / 36% Heterogeneous Enhancement 23% / 64% Hyperostosis 18% / 7% Calcification 27% / 0% “Mushrooming” 0% / 57% ➢ Narrow attachment and larger “cap” ➢ invaginating into brain Hemangiopericytoma

Hemangiopericytoma vs. Meningioma Hemangiopericytoma Narrow Base Lobulated Heterogeneous Bone Destruction No Ca++ Irregular Vessels

Meningioma Broad Base Hemispheric Homogeneous Hyperostosis Psammomatous Ca++ Spoke Wheel Vessels

Meningioma •

The 4H+ Tumor ➢ homogeneous ➢ hyperdense ➢ homogeneous enhancement ➢ hemispheric shape ➢ hyperostosis ➢ hormonally modulated

Meningioma • • •

•

75% are histologically typical 75% are radiologically typical Not the same 75% ➢ CT ➢ MR ➢ Angiography Atypical Imaging =/= Atypical Histology

Neuroradiology

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Neoplasms of the Meninges

References 1. 2. 3. 4. 5.

6. 7. 8. 9. 10.

11. 12. 13. 14. 15.

16. 17.

18. 19. 20. 21.

22. 23.

Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura. Neurosurgery. 1994 Sep;35(3):370-7;discussion 377. Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8. Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5. Berger MS. Perfusion MR and the evaluation of meningiomas: is it important surgically? AJNR Am J Neuroradiol 2003; 24:1499-1500. (1) Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPAenhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment. Radiology. 1990 Aug;176(2):447-50. Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French. Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8. Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural spaceoccupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German. Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo. 1996 Apr;164(4):314-7. German. Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90. Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels: the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5. Maiuri F et al: Intracranial meningiomas: correlations between MR imaging and histology. Eur J Radiol. 1999; 31: 69-75 Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7. Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16. Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion 590-1. Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995 Nov-Dec;19(6):890-2. Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):42933. Sandhyamani, Rao, Nair, Radhakrishan: Atypical Meningioma: A Clinicopathological Analysis.Neurology India 2000; 48: 338-342 Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI. Fukushima J Med Sci. 1998 Jun;44(1):1-11. Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese. Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial meningiomas on fluid-attenuated inversion-recovery images. Neuroradiology. 2004 Feb;46(2):130-5. Epub 2004 Jan 28. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):763-8. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga H, Toya S. Prediction of consistency of meningiomas with preoperative magnetic resonance imaging. Surg Neurol. 1997 Dec;48(6):579-83.

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“PINEALOMAS” and other Pineal Region Masses James G. Smirniotopoulos, MD Pineal Gland Introduction • • • •

•

“Seat of the Soul” Daily (Diurnal) Biorhythms Life-Cycles (Puberty, Migration) Responds to Light/Dark ➢ Melatonin levels ➢ Accessory Optic Pathway ❖ Retinohypothalamic tract, RAS, Sympathetics Third Eye ➢ Phylogenetically ➢ Developmentally ➢ Embryologically

Figure 5-14-1

The normal pineal gland is ~10-14 mm in maximum sagittal diameter

Pineal – Third Eye •

•

Iguana ➢ Third Eye ➢ Photoreceptor ❖ Transparent scale ❖ Hole in skull ➢ Radiometer for sunlight In lower vertebrates it may have a lens and a retina

Figure 5-14-2

Biological Clock • •

Day-Night Diurnal Rhythms Pineal Melatonin Suppresses GnRH ➢ Longer daylight decreases melatonin and leads to increased Gonadotropin Releasing Hormone ➢ GnRH => LH and FSH ➢ Increased sexual drive and activity in the Spring … and I thought it was the Pollen …

Normal Pineal [Figures 5-14-1 and 5-14-2] Normal Pineal Calcification • • • • •

Pineal gland and surrounding region: Third ventricle, quadrigeminal plate and cistern, bilateral thalami

Figure 5-14-3

725 Normal Patients “Youngest was 6,5” 8%–11% from 8 to 14 yrs. 30% for 15 y.o. 39%–40% from 17 to 29 yrs.

Radiology 142:659–662, 1982

Pineal Region – Normal Anatomy [Figure 5-14-3] •

Pineal Region Mass: Symptoms and Signs • •

Parinaud Syndrome ➢ NOT Paranoid Precocious Puberty Headache, Nausea, Vomiting ➢ Non-specific mass effect ➢ +ICP

Neuroradiology

Pineal Region – Normal Anatomy: The internal cerebral veins are important landmarks

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Parinaud Syndrome: Aqueduct/Tectal Syndrome •

• • •

• • • •

Failure of conjugate vertical eye movement ➢ Upward >> downward Mydriasis, fixed pupils Failed ocular convergence ➢ Lateral midbrain tegmentum Blepharospasm ➢ Eyelid spasm

Pineal Region: Differential Germ Cell Neoplasms Pineal Cell Neoplasms Gliomas Non-neuroglial Masses

Germ Cell Tumors: WHO Classification

• • • • • •

Germinoma Embryonal Carcinoma Yolk Sac ➢ Endodermal sinus Choriocarcinoma Teratoma ➢ Immature, Mature, Malignant Mixed Germ Cell

Intracranial Germ Cell Tumors •

•

•

Usually primary in the CNS ➢ Arise from “Germ Cell Rests” ➢ Pineal/Quadrigeminal and suprasellar cistern Exceptional caese metastatic to CNS ➢ Usual testicular drainage to renal hilus ➢ Para-artic nodes ➢ Rare cases of testicular seminoma CNS mets ➢ Lance Armstrong had mixed Chorio/embryonal

Pineal Region Mass 369 pts. Hoffman series

Tumor

%

GERM CELL

59

Germinoma

59

Teratoma – Malignant

11

Teratoma – Benign

2

Yolk Sac

2

Choriocarcinoma

2

Embryonal CA

1

Mixed Germ Cell

1

PINEAL REGION MASS •

369 pts. Hoffman ➢ EPIDERMOID ➢ TRUE PINEAL ❖ Pineoblastoma ❖ Pineocytoma ➢ OTHER NEOPLASMS ❖ Gliomas ❖ Non-glial (meningioma, etc)

“PINEALOMAS” and, other Pineal Region Masses

1+ 14 12 2 27 26

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Dr. Hoffman’s Series • • • •

Figure 5-14-4

Dr Harold J Hoffman (1932 – 2004) Arguably the most famous pediatric neurosurgeon in the world. Harold joined the Neurosurgical Staff at the Hospital for Sick Children in 1964 1998, the Harold J Hoffman/Shoppers Drug Mart Chair in Pediatric Neurosurgery was established at the Hospital for Sick Children

Pineal Neoplasms: Demographics Germ Cell Tumors-AFIP Series [Figure 5-14-4] Pineal Region Germ Cell Tumors - AFIP Series

Basic Approach to Pineal Region

Figure 5-14-5 Intracranial Germinoma • • • • • • • •

Synonyms: Pinealoma, Seminoma, Dysgerminoma, Atypical Teratoma Cell of Origin: Germ Cell Rests, 2-cells pattern Incidence: 1%–2% of ALL Cranial neoplasms ➢ 2%–4% of Child. ➢ 9%–15% in Japan Age: 5–35 (remember precocious puberty) Sex: 2–7M/F Location: 60–80% Pineal, 22% Suprasellar Treatment: Bx, Radiation, ChemoTx Prognosis: >50% at 5yrs ➢ Radiosensitive and chemosensitive tumor ➢ Median survival ~19 yrs

Pineal Region Germinoma – Two cell pattern, one cell resembles a lymphocyte

Figure 5-14-6

Intracranial Germinoma •

• • • •

Central: ➢ pineal (para-pineal) ➢ Suprasellar cistern Homogeneous Solid Hyperdense on NCT Isointense on T1W “Hormonally silent” ➢ no AFP/HCG but PLAP+

Germinoma Imaging [Figures 5-14-5 to 5-14-8] • • •

• • • •

Pineal region seminomas are usually hyperdense on plain CT

Sharply circumscribed, midline mass Surrounds/Engulfs Pineal Ca++ Alternate locations ➢ thalamus, 3rd vent., suprasellar cistern NCT – Homogeneous Hyperdense ECT – Homogeneous Enhancement MR – Isointense to gray matter +/– CSF Spread, tumor Ca++

Figure 5-14-7

Surgical Planning • •

•

Find the Internal Cerebral Veins and the VOG If Tumor is BELOW these veins ➢ Suboccipital Infratentorial Approach If Tumor is ABOVE these veins ➢ Interhemispheric Approach ➢ Sub Temporal Approach ➢ Various other techniques

Neuroradiology

Pineal region germinoma

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Pineal Region Neoplasms •

• •

Figure 5-14-8

Germinoma: ➢ Iso on T1W ➢ slightly Hyper on T2W Choriocarcinoma: ➢ Hyper on T1W (blood) Dermoid, Teratoma: ➢ Hyper on T1W (lipid)

MR AJNR 11:557–565,1990

Basic Approach to Pineal Region Pineal Region [Figures 5-14-9 and 5-14-10] •

Teratoma ➢ Sharply circumscribed ➢ Lobulated and Loculated ➢ HETEROGENEOUS (mixture of lipid, soft-tissue, Ca++) ➢ Enhancement of solid areas

Pineal region germinoma, extending below the tentorium

Figure 5-14-10

Figure 5-14-9

Ruptured Pineal region teratoma. Note the lipid/fluid levels in the frontal regions of both lateral ventricles. The primary tumor is seen in the midline pineal region

Pineal region teratoma. Note the peripheral rim of T1-shortening from lipid

Teratoma vs. Dermoid •

•

Teratoma is a Neoplasm ➢ From Multipotential Cells/Tissues ➢ “Included Twin” from embryo/fetus ➢ Ectoderm (Skin, Occ. Brain) Common ➢ Lipid (Mesodermal FAT or Sebaceous) ➢ Multiloculated, Lobulated Dermoid is an Inclusion Cyst ➢ Only Skin (Ectoderm) ➢ Water and/or Sebaceous Lipid ➢ Unilocular

Famous Quote Aunt Voula: “You family now, so I tell you a story. All my life ... I have this lump on the back of my neck. When I reach the menopause, the lump get bigger. I go to the doctor, and he performs a...bo-bobopsy. And inside the lump, he finds teeth, and a spinal column.” “PINEALOMAS” and, other Pineal Region Masses

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Two Theories for Teratoma • •

Figure 5-14-11

Progressive differentiation from multi-potential (“omnipotential”) Germ Cells – “primordial germ cells” Inclusion of a twin during early gestation - embryogenesis

Dermoid Inclusion Cyst [Figure 5-14-11] Pineal Region Mass Endodermal Sinus Tumor [Figure 5-14-12] Figure 5-14-12

Dermoid Inclusion Cyst in the pineal region. Histology only revealed ectodermal elements Non-seminomatous germ cell tumor – NOT hyperdense on plain CT, but does engulf a central calcification

Figure 5-14-13

Basic Approach to Pineal Region Germinoma Germinoma - seeding [Figure 5-14-13] CSF Dissemination Pineal Neoplasms Laboratory Tests: Serum and CSF Neoplasm Germinoma Yolk-sac Chorio Ca. Embryonal

BHCG --inc. inc.

AFP -inc. -inc.

PLAP inc. ----

Germinoma – CSF seeding along the edge of the tentorium (arrows)

BHCG = Beta HCG AFP = Alpha Feto Protein PLAP = Placental Alkaline Phosphatase

Basic Approach to Pineal Region

Neuroradiology

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“PINEALOMAS” and, other Pineal Region Masses

Pineal Parenchyma • •

•

Figure 5-14-14

Pineoblastoma (PNET) ➢ Young Patients (1st two decades) ➢ Tumor ITSELF Calcifies ➢ ”Exploded” Pineal Ca++ Pineocytoma (Mature pineal cells) ➢ Young or Old Trilateral Retinoblastoma ➢ Inherited Rb (chromosome 13) ➢ 1/3 inherited but 2/3 heritable ➢ Look at ORBITS for signs of Tx

Pineal Parenchyma Mass [Figure 5-14-14] • •

•

Exploded Pineal Calcifications Pineoblastoma ➢ Some hyperdense on CT Pineocytoma ➢ Isodense on CT

Pineal Parenchyma Mass Schematic: “exploded calcifications” from a mass arising inside the pineal gland. This pattern is seen in both pineal cysts and neoplasms

Figure 5-14-15

Figure 5-14-16

Pineoblastoma – “exploded calcifications” around the outside rim of the tumor

Pineoblastoma

Figure 5-14-17

Figure 5-14-18

Pineoblastoma with seeding along the edge of the tentorium. Using this T1W MR alone, this mass is indistinguishable from a pineal region germinoma

“PINEALOMAS” and, other Pineal Region Masses

Pineoblastoma. Note how the lesion extends below the tentorial hiatus into the posterior fossa

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Pineoblastoma [Figures 5-14-15 to 5-14-18]

Figure 5-14-19

Pineal Cysts •

•

•

Autopsy ➢ ~ 5% of Adults ➢ < 2 mm in a Normal Size Gland MR Visible ➢ 2%-8% of Adults ➢ 2-7 mm common ➢ 14-25 mm cysts are not rare ➢ May expand the gland ➢ Ring enhancement should be smooth and thin 1-2 mm Why do they grow? – Unknown

Pineal Cyst •

•

Schematic for Vein of Galen malformations: Straight sinus obstruction, sinus hypoplasia, AVM or dural fistula draining into the VOG

Typical cysts: ➢ Round or Oval ➢ T1 ~ WM ➢ T2 ~ CSF ➢ T2 Homogeneous ➢ Rim Enhancement< 2mm ➢ No nodularity “These findings suggest that typical pineal cysts may be followed up on a clinical basis alone rather than on imaging.”

Figure 5-14-20

Pineal Region Mass: Other Lesions •

• •

Glial - Astrocytoma ➢ Splenium Of Corpus Callosum ➢ Tectum Of Midbrain ➢ Thalamus Congenital ➢ Lipoma ➢ Inclusion Cyst (Epidermoid/Dermoid) ➢ Vein of Galen Malformation Non-Glial - Meningioma

Vein of Galen Malformation

Vein Of Galen Malformation: Symptoms, Signs •

•

Figure 5-14-21

Childhood – Large shunt ➢ High Output Failure ➢ Persistent Ductus ➢ Hydrocephalus ➢ Cranial Bruit/thrill Adult – Small shunt ➢ Asymptomatic ➢ Pineal Symptoms

Vein Of Galen Malformation: Types and Causes • • • • •

[Figures 5-14-19 and 5-14-21]

Vein of Galen Malformation. Persistence of the falcine vein

Parenchymal Avm (Shunt) Direct Fistulae To Vein Dural Fistula (Drains To Vein) Sinus Thrombosis (Fetal) Hypoplastic Straight Sinus

Hydrocephalus •

•

Mechanical ➢ Aqueductal Obstruction Impaired CSF Resorption ➢ Venous Hypertension

Neuroradiology

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Intracranial Lipoma [Figure 5-14-22] • • • • •

Figure 5-14-22

Congenital, NOT a true neoplasm MIDLINE (usually) Usually around Corpus Callosum Occasional Tectal, Hypothalamic, CPA Abnormal Differentiation ➢ Meninx Primativa Into Fat

Meningioma [Figures 5-14-23 and 5-14-24] Figure 5-14-23

Intracranial Lipoma of the quadrigeminal plate. Notice how the mass presents below the tentorium because of the herniation caused by obstructive hydrocephalus

Figure 5-14-24

Meningioma of the quadrigeminal plate

Glioblastoma multiforme [Figure 5-14-25] Figure 5-14-25

Meningioma of the quadrigeminal plate – see the dural tail

Figure 5-14-26 Mass in the splenium of the corpus callosum – Glioblastoma multiforme

Astrocytoma - Splenium [Figure 5-14-25] Headaches and Parinaud Syndrome [Figure 5-14-26] Astrocytoma of Tectum

Astrocytoma of the quadrigeminal plate “PINEALOMAS” and, other Pineal Region Masses

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Pineal Region •

• •

60% Germ Cell Neoplasm ➢ Seminoma (2/3) ➢ Teratoma ➢ Other – “non-germinoma GCT” 15% Pineal Parenchymal ➢ Pineocytoma ➢ Pineoblastoma (PNET) OTHER Lesions ➢ Astrocytoma ❖ Splenium, Tectum, Thalamus ➢ Meningioma, Lipoma ➢ VOG Malformations

References 1. 2. 3. 4. 5. 6.

7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17.

Barboriak DP, Lee L, Provenzale JM. Serial MR imaging of pineal cysts: implications for natural history and follow-up. AJR Am J Roentgenol. 2001 Mar;176(3):737-43. Barbouriak DP, Lee L, Provenzale JM: Serial MR Imaging of Pineal Cysts: Implications for Natural History and Follow-Up.AJR 2001; 1737-743. Fain JS, Tomlinson FH, Scheithauer BW, Parisi JE, Fletcher GP, Kelly PJ, Miller GM. Symptomatic glial cysts of the pineal gland. J Neurosurg. 1994 Mar;80(3):454-60. Fleege MA, Miller GM, Fletcher GP, Fain JS, Scheithauer BW. Benign glial cysts of the pineal gland: unusual imaging characteristics with histologic correlation. AJNR Am J Neuroradiol. 1994 Jan;15(1):161-6. Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O, Tamura A, Sano K.J Neurooncol. CT and MRI features of intracranial germ cell tumors. 1994;19(3):217-26. Hayashida Y, Hirai T, Korogi Y, Kochi M, Maruyama N, Yamura M, Yamashita Y. Pineal cystic germinoma with syncytiotrophoblastic giant cells mimicking MR imaging findings of a pineal cyst. AJNR Am J Neuroradiol. 2004 Oct;25(9):1538-40. Jinkins JR, Xiong L, Reiter RJ. The midline pineal "eye": MR and CT characteristics of the pineal gland with and without benign cyst formation. J Pineal Res. 1995 Sep;19(2):64-71. Koenigsberg RA, Faro S, Marino R, Turz A, Goldman W. Imaging of pineal apoplexy. Clin Imaging. 1996 AprJun;20(2):91-4. Korogi Y, Takahashi M, Ushio Y. MRI of pineal region tumors. J Neurooncol. 2001 Sep;54(3):251-61. Lee DH, Norman D, Newton TH. MR imaging of pineal cysts. J Comput Assist Tomogr. 1987 Jul-Aug;11(4):58690. Mamourian A, Towfighi J. MR of pineal cysts. AJNR Am J Neuroradiol. 1994 Oct;15(9):1796-7. Mamourian AC, Towfighi J. Pineal cysts: MR imaging. AJNR Am J Neuroradiol. 1986 Nov-Dec;7(6):1081-6. Mamourian AC, Yarnell T. Enhancement of pineal cysts on MR images. AJNR Am J Neuroradiol. 1991 JulAug;12(4):773-4. No abstract available. Osborn RE, Deen HG, Kerber CW, Glass RF. A case of hemorrhagic pineal cyst: MR/CT correlation. Neuroradiology. 1989;31(2):187-9. Reis F, Faria AV, Zanardi VA, Menezes JR, Cendes F, Queiroz LS. Neuroimaging in pineal tumors. J Neuroimaging. 2006 Jan;16(1):52-8. Sener RN. The pineal gland: a comparative MR imaging study in children and adults with respect to normal anatomical variations and pineal cysts. Pediatr Radiol. 1995;25(4):245-8. Welton PL, Reicher MA, Kellerhouse LE, Ott KH. MR of benign pineal cyst. AJNR Am J Neuroradiol. 1988 MayJun;9(3):612. No abstract available.

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The Phakomatoses James G. Smirniotopoulos, MD Phakomatoses or Neurocutaneous Syndromes •

An Introduction

The Phakomatoses • •

Neuro-ectodermal or Nerves and Skin

Phakomatoses: Why Study Them? • • • • •

They are COMMON diseases DIAGNOSED by Imaging GENETIC Implications SCREEN Relatives SURVEILLANCE of Affected

Phakomatoses Mnemonic Tool •

• • •

•

NF-1 (von Reck’s) ➢ TRUE Neurofibromatosis #17 NF-2 (Bil. VS Syndrome) ➢ M.I.S.M.E. #22 STURGE-WEBER (Dimitri) Syndrome ➢ Congenital Vascular Lesion ➢ Perhaps NOT inherited Tuberous Sclerosis ➢ Pringle’s “HAMARTOMA” Disease Von HIPPEL-LINDAU Syndrome ➢ NO cutaneous lesions ➢ Hemangioblastomas and Visceral Lesions

Phakomatoses – Plan • • • • • • •

Demographics Diagnostic Criteria Ocular/Orbit Lesions Skin Brain Visceral Manifestations Complications of Disease

CNS Neoplasms: Chromosomes •

•

Loss of Heterozygosity (LOH) ➢ Schwannoma - 22q ➢ Meningioma - 22q (Long Arm) ➢ Ependymoma - 22 ➢ Medulloblastoma - 17p (Short Arm) ➢ Neurofibrosarcoma - 17p ➢ Retinoblastoma - 13q ➢ Pilocytic Astrocytoma - None ! TUMOR SUPPRESSOR GENES

Molecular Biology [Figures 5-15-1] •

Genetic “Two Hit Theory”

The Phakomatoses

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Neuroradiology

Phakomatoses •

• • •

NEUROFIBROMATOSES ➢ Type 1, Chromosome 17q11 ➢ Type 2, Chromosome 22q12 Tuberous Sclerosis ➢ Chromosome 9q, 16p STURGE-WEBER (? not inherited) von Hippel-Lindau ➢ Chromosome 3p25

Figure 5-15-1

Neurofibromatosis • • • • • • •

NF-1, von Recklinghausen (“peripheral” – bad term) NF-2, Bilateral Acoustic (“central” – bad term) NF-3, Overlap of 1 and 2 NF-5, Segmental (e.g. a quadrant) NF-6, Cafe-au-lait, w/o CNS/PNS NF-7, Late Onset NF-8, Other

Neurofibromatosis – Types •

•

Neurofibromatosis Type 1 (NF-1) ➢ von Recklinghausen Disease ➢ “True” Neurofibromatosis ➢ Prominent Cutaneous Signs ➢ Chromosome 17q Neurofibromatosis Type 2 (NF -2) ➢ Bilateral Acoustic Schwannoma ➢ “Central Neurofibromatosis” ➢ Minimal Skin Manifestations ➢ Chromosome 22q

Neurofibromatosis Type 1 or von Recklinghausen Disease •

Tumor Suppressor Gene: “Two Hit” Hypothesis

Chromosome 17

Neurofibromatosis • • • • •

1768 MARK AKENSIDE (New York) 1793 TILESIUS (Leipzig) 1849 R.W. SMITH (England) 1822 WISHART (Edinburg) NF-2 1882 von RECKLINGHAUSEN (Germany)

Neurofibromatosis - 1 •

Clinical ➢ Incidence: 1/2,500 births ➢ Inheritance: Autosomal Dominant ➢ Age at Presentation: Birth to Death ➢ Sx at Presentation: Spots, NFB ➢ Diagnostic Criteria: Cutaneous, PNS ➢ Chromosome Abnl.: 17 ➢ Ocular Findings: Myelinated retina ➢ Cutaneous Findings: cafe-au-lait, neurofibroma ➢ CNS Findings: Optic N. Glioma, Hamartoma, Heterotopia, macrocephaly, mentation problems

NIH Diagnostic Criteria: 2 from list • • •

Cafe-Au-Lait spots ➢ 6 or more ➢ 5 mm child, 15 mm adult Neurofibromas – 2 or more Plexiform Neurofibroma – 1

Neuroradiology

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The Phakomatoses

• • • • •

Axillary (Intertriginous) Freckling-1 Optic Glioma Lisch Nodules (Iris) – 2 or more “Distinctive Bone Lesions” 1st degree relative with NF-1

Figure 5-15-2

Neurofibromatosis – 1 • • • • •

Clinical Chromosome Abnl.: 17 Ocular: Myelinated retina Cutaneous: cafe-au-lait, neurofibroma CNS: Optic N. Glioma, Hamartoma, Heterotopia, Macrocephaly, Mentation

NF-1: Eye Manifestations •

Lisch Nodules – Named for the 19th century German physician who first described them, Dr. Augustus Nodule

LISCH Nodules (Iris Hamartomas) [Figure 5-15-2]

•

➢ Penetrance > 90% ➢ Specificity > 90% ➢ Translucent/pigmented ➢ Small ( < 3mm.), Slit-Lamp Exam OPTIC GLIOMA [Figures 5-15-3] ➢ Up to 15% of patients ➢ Pilocytic Astrocytomas ➢ Benign (“Hamartoma -like”), Tx? ➢ True Neoplasms, spread along SAS ➢ up to 1/2 of Childhood ONG w/NF -1

Figure 5-15-3

Neurofibromatosis – 1 •

Cutaneous Manifestations ➢ Cafe-au-Lait spots [Figure 5-15-4] ➢ Intertriginous Freckling ➢ Neurofibromas (Skin and SubQ) ➢ Fibroma Molluscum (TNTC NFB) ➢ Elephantiasis Neuromatosa ❖ diffuse skin thickening/plexiform NFB – ❖ or focal gigantism

Neurofibromatosis – 1 •

Bone Dysplasia and Remodeling ➢ Macrocephaly ➢ Craniofacial dysplasia ❖ especially sphenoid ➢ Vertebrae (scalloping, scoliosis) ➢ Pseudoarthrosis ❖ especially congenital ➢ Genu Valgum/Varum ➢ Twisted “Ribbon Ribs”

Optic Nerve Glioma

Figure 5-15-4

Neurofibromatosis – 1 [Figures 5-15-5 to 5-15-8] •

• • •

Skull and Spine Dysplasia ➢ Sphenoid Bone (“absent orbit”) ➢ Lambdoid Suture at Temporal Bone ➢ Optic and Auditory Canals (enlarged) Scoliosis ➢ Simple or Acute Cx Kyphosis Vertebral Scalloping (usu. Lumbar) Enlarged Spine Neural Foramina Café-au-lait spot – a macular (flat) area of hyperpigmentation

The Phakomatoses

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Embryo – Differential Development •

•

Figure 5-15-5

Molecular biology ➢ Chemical gradients ➢ Medial <> Lateral ➢ Proximal <> Distal ➢ Anterior <> Posterior ➢ Superior <> Inferior Sonic Hedgehog Gene (shh) ➢ Drosophila embryo spiked like a hedgehog ➢ Desert hedgehog (dhh), Indian hedgehog (ihh) ➢ Needed a new name – scientists like videogames … so

Nerve Sheath Tumors •

• •

•

Schwannoma (Sporadic >> NF-2 > NF-1) ➢ focal mass ➢ usually sensory root, cranial and spinal nerves Neurofibroma ➢ usually NF-1, esp. if spinal or paraspinal ➢ spindle or dumb -bell lesion Plexiform Neurofibroma (usually NF-1) ➢ diffuse or fusiform enlargement Malignant Peripheral Nerve Sheath Tumor ➢ NF-1 or Sporadic

“Empty Orbit” from sphenoid dysplasia. The left orbit shows the outline of normal sphenoid – lesser (superior) and greater (inferior) densities

Figure 5-15-6

Figure 5-15-7 Sphenoid Dysplasia

Figure 5-15-8

Progressive Pseudoarthrosis

Focal Gigantism – the overgrowth may affect all elements, bone, muscle, fat, vessels, etc. Neuroradiology

1185 1187

The Phakomatoses

Figure 5-15-9

Figure 5-15-10

Neurofibroma – a diffuse lesion, even at the microscopic level

Schwannoma – a focal mass, even at the microscopic level

Neurofibroma vs. Schwannoma •

•

Neurofibroma [Figure 5-15-10] ➢ Schwann cells ➢ Fibroblasts ➢ Acellular material ➢ Infiltrating ➢ Resect Parent Nerve Schwannoma [Figure 5-15-9] ➢ Schwann Cell Neoplasm ➢ Secondary vascular changes ➢ Mostly cellular ➢ Encapsulated ➢ Nerve Sparing Surgery

Distribution of Nerve Sheath Tumors •

• • • •

Intra-Cranial – Schwannoma ➢ Sporadic >> NF-2 Spinal – Both Types (S >> N) Dumbbell – Both (N >> S) PNS – Both Cutaneous – Neurofibroma ➢ Usually N in NF-1)

Figure 5-15-11

Intraspinal Neoplasms • • •

• •

•

68 Pts. 86 Spinal Nerve Sheath neoplasms Sporadic: 42 pts. (65%) ➢ 40 Schwannoma and 2 neurofibroma NF-1: 12 Pts. (18%) ➢ All were Neurofibroma NF-2: 7 Pts (11%) ➢ 6 Schwannoma/1 “mixed” tumor Unknown - 5 Pts.

Acute Cervical Kyphoscoliosis – one of the characteristic lesions of NF-1

Neurofibromatosis : Spine [Figure 5-15-11] •

•

Scoliosis (NF-1, only?) ➢ Simple ("idiopathic") ➢ Acute Cervical Kyphosis Dural Ectasia (NF-1, only?) ➢ Vertebral Scalloping ➢ Arachnoid "cysts" ➢ Lateral Thoracic meningocele

The Phakomatoses

1186 1188

Neuroradiology

Neurofibromatosis : Spine • •

• • •

Figure 5-15-12

Neurofibroma (NF-1) Osteoporosis (NF-1, only?) ➢ Idiopathic ➢ Parathyroid Adenoma Schwannoma (NF-2) Meningioma (NF-2 Ependymoma (NF-2)

Enlarged Neural Foramen •

DDx: ➢ Nerve Sheath Tumor ❖ Neurofibroma ❖ Schwannoma ➢ Arachnoid Cyst ➢ Bone Dysplasia

Enlarged neural foramina from multiple plexiform neurofibromata

Neurofibromatosis: Enlarged Neural Foramen [Figures 5-15-12 to 5-15-14] •

•

Nerve Sheath Tumor ➢ Neurofibroma ❖ NF-1 >> sporadic ❖ "dumbbell“ shape ➢ Schwannoma ❖ sporadic >> NF-2 Mesodermal Defect ➢ NF-1 only? ➢ Dural weakness ➢ Bone weakness

Figure 5-15-13

Rib Notching [Figure 5-15-15 ] •

• •

Aortic Coarctation ➢ Older than 5-6 years ➢ 3-9 possible ➢ Ribs 5-8 most often ➢ 1-2 arise from subclavian artery ➢ Usually Bilateral ❖ Unilateral on the Right ❖ if Coarctation involves Left Subclavian origin A-V Fistulae Nerve Sheath tumors

Multiple Dumbbell Lesions - neurofibromas

Figure 5-29-14

Figure 5-15-15

Neurofibroma vs. Schwannoma

Rib Notching from extensive plexiform neurofibromas involving all of the intercostal nerves

Neuroradiology

1187 1189

The Phakomatoses

Plexiform NF [Figure 5-15-16 ]

Figure 5-15-16

Plexiform neurofibromas

Neurofibromatosis - 1: Spine • • • •

Figure 5-15-17

[Figures 5-15-17 and 5-15-18]

Scoliosis (Acute Cx Kyphoscoliosis) Vertebral Scalloping Enlarged Neural Foramina Lateral Thoracic Meningocele

Neurofibromatosis - 1 [Figure 5-15-19] •

• •

Posterior Meningocele (sporadic) ➢ dorsal dysraphism, closure of tube Anterior Meningocele (sporadic) ➢ neurenteric canal/cyst ➢ anterior vertebral cleft Lateral Thoracic Meningocele (NF-1) ➢ “pulsion diverticulum” of SAS ➢ negative intrathoracic pressure ➢ no overlying paravertebral MM.

Vertebral Body Scalloping and one neurofibroma. Both lesions may also cause enlargement/erosion of the neural foramna

Figure 5-15-18

Figure 5-15-19

Arachnoid Cyst & Neurofibroma

Lateral Thoracic Meningocele The Phakomatoses

1188 1190

Neuroradiology

Neurofibromatosis – 1: DBO’s MR Signal Abnormalities •

Figure 5-15-20

[Figure 5-15-20]

• •

T1W Bright Foci ➢ globus pallidus T2W Bright Foci ➢ w/o mass, don’t enhance ➢ Cerebellar peduncles, Pons, midbrain ➢ globus pallidus, thalamus, optic radiations What in the heck are they?? ➢ Ectopic Schwann cells, Melanocytes?? ➢ Dysmyelination?? ➢ Intracellular proteinaceous fluid?

DBO’s and NF-1 • • •

Incidence: A considerable body of knowledge suggests that these Unidentified Deep Bright Objects or DBO’s are very common in children with NF-1. (>90% in some series) Age: They are most frequent from 4 –12 years of age. They are uncommon under the age of 4, and begin to fade away over the age of 16. Location: ➢ Globus Pallidus – 30% ➢ Cerebellum – 23% Deep Bright Objects ➢ Midbrain – 16%

DBO’s of NF-1: Globus pallidus • • • •

Neurofibromatosis Objects of Uncertain Significance ➢ “NOUS”

Neurofibromatosis •

• • • •

Malignant Nerve Sheath ➢ Tumor (malignant PNST, neurofibrosarcoma,...) Embryonal Malignancies:Wilms, Rhabdomyosarcoma Leukemia (CML) Melanoma, Medullary Thyroid Ca. Low Incidence of Lung Cancer”

Neurofibromatosis Deep Bright Objects – DBO’s

Malignant Peripheral Nerve Sheath Tumor Neurofibromatosis Type 2 or Wishart Disease • • • •

Chromosome 22 Bilateral Acoustic Schwannoma "Central Neurofibromatosis" Minimal Skin Manifestations

Neurofibromatosis – Type 2 • • • • • • • •

Incidence: 1/50,000 Inheritance: Autosomal Dominant Age at Presentation: Birth to 40’s (peak in 20’s) Sx at Presentation: Hearing loss from VS Diagnostic Criteria: VIII masses Chromosome Abnl.: 22 Cutaneous Findings: minimal (skin tags) CNS Findings: Schwannoma, Meningioma, Ependymoma (intramedullary spinal cord)

Neuroradiology

1189 1191

The Phakomatoses

CNS Neoplasms – Chromosome Loss of Heterozygosity •

• • •

Figure 5-15-21

NF-2 ➢ SCHWANNOMA – 22q ➢ MENINGIOMA – 22q (long arm) ➢ EPENDYMOMA – 22 NOT Neurofibroma NOT Astrocytoma NOT Optic Glioma

NF-2 (“Central”), 1 or More • •

Intracanalicular Schwannoma – they all begin Bilateral VIIIth Masses inside the IAC – because that is where the Relative with NF-2 and either: Schwann cells are. The cisternal segment of the ➢ Unilateral VIIIth Mass nerve has oligodendrocytes ➢ Any Two: ❖ “Neurofibroma”, Meningioma, Glioma, Schwannoma, (Congenital) Lens Opacity

Figure 5-15-22 Neurofibromatosis – Type 2 • • • • • • •

NEJM 319:278-83, 1988 (Gulf of Mexico) 23 Pts. (15M/8F), Kindred of 137 0.95 Penetrance 18 Acoustic Schwannoma (17 bil.) 8 Meningioma (3 mult.) 4 Ependymoma 2 Spinal “Neurofibroma”

Schwannoma [Figures 5-15-21 and 5-15-22] • • • • •

• •

5%-10% of All CNS Tumors Benign, Slowly growing F > M (Intracranial), M > F (Spinal) 30’s – 60’s, w/NF-2 10’s – 30’s Sensory Nerves (usually): ➢ CNN VIII (Sup.Vestibular), V, X ➢ Spine: Dorsal Roots Majority (>90%) are Sporadic Multiple in NF-2, Bilat.VIII Pathognomonic

Neurofibromatosis – 2 [Figure 5-15-23] •

•

Bilateral Vestibular Schwannoma

Meningiomas: ➢ multiple transitional type meningioma ➢ NOT meningothelial Meningioangiomatosis: ➢ cortical (intracortical) vascular tissue ➢ resembles a vascular malformation ➢ meningothelial and fibroblast -like cells

Figure 5-15-23

Multiple Schwannomas and Meningiomas and Ependymomas [Figure 5-15-24] Neurofibromatosis – 2 [Figure 5-15-24]

• • • •

Multiple Meningiomas (up to 45%) ➢ Intraventricular Meningiomas ➢ Childhood Meningiomas Multiple Meningiomas ➢ 1%-10% of all patients with meningioma ➢ SPORADIC in 80%-90% Intraventricular Meningiomas ➢ SPORADIC in 90% Childhood Meningiomas ➢ SPORADIC vs. Inherited (NF-2 or Not)

The Phakomatoses

Multiple Schwannomas and Meningiomas 1192

Neuroradiology

Neurofibromatosis Type - 2=> MISME • • • • •

Figure 5-15-24

M ultiple I nherited S chwannomas M eningiomas E pendymomas

Sturge-Weber Disease or EncephaloTrigeminal Angiomatosis • • • •

Inheritence ?? Autosomal Dominant ? Autosomal Recessive ? Sex-Linked ? Multiple Schwannomas, Meningiomas, and Ependymomas – the MISME syndrome

STURGE-WEBER: Definition •

A telangiectatic venous angioma of the leptomeninges, face, and choroid of the eye.

Figure 5-15-25

STURGE-WEBER SYNDROME: History • • • • • •

1879 STURGE, Clinical description 1897 Kalischer, Vascular nature 1922 Weber, published radiography 1923 Dimitri, “tram-track” Ca++ 1934 Krabbe, Ca++ in cortex 1937 van der Hoeve, Phakomatosis

STURGE-WEBER SYNDROME: Classic Triad •

• •

Facial Neveus Flammeus ➢ Port-Wine Stain Seizures Mental Deficiency

STURGE-WEBER: Manifestations •

• • • •

Seizures, Mental Decline ➢ Usually begins in first 24 months Facial Angioma ➢ At birth Angiomatous Overgrowth of soft-tissue and bone Leptomeningeal Angioma Cortical Atrophy w/Ca++

Trigeminal Angiomatosis in SWS

Figure 5-15-26

STURGE-WEBER: Variants • • •

•

Facial and Intracranial w/o Eye Intracranial and Eye w/o Face Intracranial Alone ➢ (Cerebral and Leptomeningeal) Klippel-Trenaunay (KT Weber) ➢ Extracranial soft-tissue angiomas)

STURGE-WEBER SYNDROME: Port Wine Stain (PWS) [Figures 5-15-25 and 5-15-26] • • •

Facial Neveus Flammeus Blanches w/ pressure Trigeminal Dermatome ➢ V1 – Ophthalmic ➢ V2 – Maxillary ➢ V3 – Mandibular Sturge-Weber: Facial overgrowth and extensive, bilateral, “port-wine stain”.

Neuroradiology

1191 1193

The Phakomatoses

Association of PWS with SWS • •

Figure 5-15-27

All 3 >> 1+2 >> 1 or 2 alone >> other medial aspect of eyelid (V1 or V2)

Medullary Veins [Figures 5-15-27 and 5-15-28] STURGE-WEBER: Vascular • • • • •

Absence of cortical veins Poor filling of sagittal sinus Persistent Primitive Plexus (SAS) Recruitment of Medullary Veins Prominent Choroid Plexus

Persistence of Primitive Plexus • • •

Medullary Veins are prominent, bridging veins are absent over the occipital and posterior parietal lobes

Persistence of primitive vascular plexus Absence of cortical veins Deoxygenated blood

Figure 5-15-28

Cranial Vascular Development • •

•

Begins with primitive plexus Progressive Differentiation ➢ Arteries ➢ Capillaries ➢ Veins Progressive Lamination ➢ Cerebral (brain) circulation ➢ Dura and Bone circulation ➢ Scalp Circulation

}

Connect via bridging veins

Sequential Induction: Eye Development • •

Optic nerve induces lens development Lens induces cornea development from surface ectoderm and adjacent mesenchyme

STURGE-WEBER: Etiology •

Abnormal Development of Capillaries ➢ Poor cortical venous drainage ➢ Absent cortical veins ➢ Prominent veins in SAS ➢ Prominent deep (medullary) veins ➢ Enlarged choroidal vessels ➢ Persistence of Primitive Plexus

T1W MR with contrast. Prominent medullary veins in SWS. Also note the widened diploic space of the frontal bone and gyriform surface enhancement posteriorly

Figure 5-15-29

STURGE-WEBER: Orbit/Eye [Figure 5-15-29] •

• • •

Buphthalmos (‘Ox Eye’) ➢ congenital glaucoma ➢ enlarged globe Choroidal Angioma Episcleral Telangiectasia Angiomatous Overgrowth of EOM’s

STURGE-WEBER: Manifestations • • • • •

Seizures, Mental Decline Facial Angioma Angiomatous Overgrowth Leptomeningeal Angioma Cortical Atrophy w/Ca++

The Phakomatoses

Buphthalmos or “Ox eye”. Congenital glaucoma, caused by a choroidal angioma, has led to enlargement of the ocular globe 1192 1194

Neuroradiology

Vessels in SAS [Figure 5-15-30]

Figure 5-15-30

Normal vs. Venous Outflow Obst. [Figure 5-15-31] Figure 5-15-31

Multiple small vessels in SAS in SWS

Figure 5-15-32

Impaired venous drainage leads to chronic cerebral ischemia

STURGE-WEBER: Calcification [Figure 5-15-32] • • • •

Abnormal (sluggish) circulation Chronic Cerebral Ischemia Progressive Cell Loss (Atrophy) Progressive Cerebral calcification ➢ early – subcortical WM (?) ➢ Later – middle layers of cortex

DYKE, DAVIDOFF, MASSON • •

“Cerebral Hemiatrophy with Homolateral Hypertrophy of the Skull and Sinuses” Surgery, Gynecology, & Obstetrics 1933 pp. 589-600 Sturge-Weber Disease. Cerebral hemiatrophy and calcification

Sturge-Weber- Hemiatrophy [Figure 5-15-33] Figure 5-15-33

Noncontrast and postcontrast axial CT (Images 1, 2, 3) sections show prominent subarachnoid spaces overlying atrophic left frontal lobe. Cortical calcification and hypodense white matter of the ipsilateral forceps minor are well shown also

Neuroradiology

1193 1195

The Phakomatoses

Progression in SWS [Figure 5-15-34]

Figure 5-15-34

Dyke, Davidoff, Masson [Figure 5-15-35] Figure 5-15-35

Dyke, Davidoff, Masson changes from cerebral hemiatrophy in SWS

STURGE-WEBER [Figures 5-15-36 • •

•

to 5-15-38]

Progression in SWS same patient two years apart

Gyral Gadolinium Enhancement Abnormal BBB in Cortex ➢ (Chronic ischemia) “Epi-Cortical” enhancement ➢ (slow flow in superficial veins)

Figure 5-15-36

STURGE-WEBER: Treatment • • • • •

Symptomatic (anticonvulsants) Cosmetic Tattooing Laser Treatment of Skin Lesions Hemispherectomy Aspirin (mild antiplatelet)?

Figure 5-15-37

Sturge-Weber Disease: Gyral Enhancement and Choroid Plexus enlargement

Figure 5-15-38

Sturge-Weber Disease. Gyral hypointensity from dense calcifications. Angiomatous overgrowth of the temporalis muscle (arrow) Sturge-Weber Disease. T2W MR and CT in the same patient show changes from gyral calcification The Phakomatoses

1194 1196

Neuroradiology

Half a Brain • • • • •

Uncontrolled Sz Under age 2 Up to age 5-7 Plasticity Uncrossed tracts ➢ 5%–15%

Tuberous Sclerosis or Bourneville Disease •

Chromosomes 9 and 16

Tuberous Sclerosis •

Original “VOGT TRIAD” ➢ FACIAL NEVUS (ADENOMA SEBACEUM) ➢ SEIZURES ➢ MENTAL DEFICIENCY

Tuberous Sclerosis • • • •

•

AUTOSOMAL DOMINANT No Racial/Sexual High Spontaneous Mutation High Penetrance ➢ “SPORADIC” over-reported Multiple Genes ➢ TSC1 – 9q ➢ TSC2 – 16p

Tuberous Sclerosis, NIH Consensus Conference •

Major Features: • ➢ Facial angiofibroma or forehead plaque ➢ Ungual or Periungual fibroma ➢ >3 Hypomelanotic macules ➢ Shagreen patch ➢ Multiple retinal nodular hamartomas ➢ Cortical Tuber ➢ Subependymal Nodule ➢ Subependymal Giant Cell Astrocytoma ➢ Cardiac rhabdomyoma ➢ Lymphangiomyomatosis ➢ Renal angiomyolipoma

Minor Features: ➢ Multiple dental enamal pits ➢ Hamartomatous rectal polyps ➢ Bone cysts ➢ White matter migration lines ➢ Gingival fibromas ➢ Non-renal hamartoma ➢ Retinal achromic patch ➢ "Confetti" skin lesions ➢ Multiple renal cysts

Definite TS - (2 Major) or (1 Major + 2 Minor) Probable TS - 1 Major + 1 Minor Possible TS - (1 Major) or (2 Minor) Hyman MH, Whittemore VH:"National Institutes of Health Consensus Conference:tuberous sclerosis Complex" Arch Neurol 2000; 57: 662-665

Tuberous Sclerosis •

Definitive (need 1) ➢ (1) facial angiofibroma ➢ (2) ungual fibroma ➢ (3) retinal hamartoma ➢ (4) cortical tubers ➢ (5) subependymal nodules ➢ (6) multiple renal AML

Neuroradiology

1195 1197

The Phakomatoses

•

Presumptive (need 2) ➢ (1) hypomelanotic nodules ➢ (2) shagreen patch ➢ (3) single renal AML ➢ (4) multicystic kidney ➢ (5) cardiac rhabdomyoma ➢ (6) pulmonary lymphangiomyomatosis ➢ (7) radiographic “honeycomb” lung ➢ (8) first degree relative with TS

Tuberous Sclerosis: • • • • • • •

Seizures 90% Adenoma Sebaceum 60%-90% Retardation 40%-60% Retinal Phakoma 50% Xr: Intracranial Ca++ 50% Ungual Fibromata 17% Giant Cell Astrocytoma 15%

INCIDENCE Of Tuberous Sclerosis: •

• •

CLASSIC TRIAD ➢ VARIABLE Incidence ➢ 1 In 10K- 500K ➢ 1 In 150K In HONG KONG MAYO Clinic Criteria ➢ 1 IN 10,000 AT MAYO CLINIC ➢ Local Population Olmsted Cty FORME FRUSTE ➢ Five Times More Common Than Classic

Tuberous Sclerosis •

•

“Hamartomas” ➢ CNS (Cortical Ventricular) ➢ Retina (Phakoma) ➢ Kidney (Angio Myo Lipoma – AML) Angiofibromas ➢ Face (“Adenoma Sebaceum”) ➢ Nail Bed (“Fibromas”)

Tuberous Sclerosis: •

•

Rhabdomyomas – Heart ➢ “Hamartomas” Angiomyomatosis – Lung ➢ smooth muscle proliferation

Tuberous Sclerosis: Cutaneous • • • •

“Adenoma Sebaceum” Peau D’orange Ash-Leaf Macule Ungual Angiofibromas

Adenoma Sebaceum • • • • •

AKA PRINGLE’S DISEASE NOT present at birth develop before puberty nasolabial fold ->bi-malar papules of angiofibroma

The Phakomatoses

1196 1198

Neuroradiology

Pringle’s Disease [Figure 5-15-39] • • • • •

Figure 5-15-39

Pringle’s Name ➢ Entire Disease ➢ Facial lesion only Mild Mental Retardation Seizures Hard Potatoes Tubular Can – ‘Tuberous’

Subungual/Periungual Fibroma [Figure 5-15-40] Figure 5-15-40

Pringle Disease: A papular (raised) reddish lesion, often in a bimalar pattern, caused by angiofibromata of the skin in Tuberous Sclerosis

Subungual (right) and Periungual (left) Fibroma in Tuberous Sclerosis. These are angiofibromata, similar to the Pringle facial lesion

Depigmentation: •

•

Ash-Leaf Spots ➢ (Lance- Ovate Shape) Confetti – Like Hypopigmentation ➢ (Inverse Freckle)

Figure 5-15-41

Other Cutaneous Manifestations •

Subepidermal Fibrosis: ➢ Dorsal Surfaces ➢ “Shagreen Patch” ➢ “Peau D’orange” ➢ “Pigskin” ➢ “Elephant Hide”

Tuberous Sclerosis: Ocular •

[Figure 5-15-41]

PHAKOMA ➢ benign astrocytic hamartoma ➢ LEUKOKORIA ➢ White light reflex ➢ Calcification Common ➢ Especially over Optic Nerve

Tuberous Sclerosis. Astrocytic hamartoma of the retina – the original “phakoma” of van der Hoeve

Tuberous Sclerosis – BRAIN: •

•

HETEROTOPIAS AND HAMARTOMAS ➢ in white and gray matter CORTICAL TUBERS ➢ “HAMARTOMAS” ➢ but with abnormal “N” cells ➢ neither Astrocyte nor Neuron ➢ Decreased Myelination ➢ No laminar architecture

Neuroradiology

1197 1199

The Phakomatoses

• • • •

SUBEPENDYMAL NODULES (almost 100%) ➢ “hamartomas” vs. neoplasia ➢ Caudothalamic groove ➢ Polypoid “Candle Gutterings” DILATED VENTRICLES ➢ variable ➢ obstructive, atrophic vs. “idiopathic” TUMORS 15% Sub-ependymal Giant Cell Astrocytoma ➢ True neoplasm, Benign WHO Grade I

Figure 5-15-42

Cortical Tubers [Figure 5-15-42]

Subependymal Nodules [Figures 5-15-43 and 5-15-44]

Figure 5-15-43

Cortical Tubers in Tuberous Sclerosis

Figure 5-15-44

Neonatal sonogram. Subependymal Nodules in Tuberous Sclerosis

Tuberous Sclerosis [Figures 5-15-45 to 5-15-47] •

Renal ➢ Angiomyolipoma ➢ Multiple Simple Cysts ➢ Another cause of APCKD ➢ RCC Reported

Figure 5-15-45

Subependymal nodules may enhance without neoplastic transformation

Tuberous Sclerosis is a disorder of neuronal migration and maturation

The Phakomatoses

1198 1200

Neuroradiology

Figure 5-15-46

Figure 5-15-47

Tuberous Sclerosis. Cortical tubers and white matter hyperintensities from the abnormal migration and maturation of the brain

Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis. This is a low grade – WHO 2 – neoplasm

Figure 5-15-48 Angiomyolipoma • • • • • • •

[Figure 5-15-48]

10% w/enough FAT for plain film 1/6 OF Solitary AML Pts. Have TS 1/3-1/2 of solitary AML Pts. Have other stigmata of TS 50-80% of Pts. W/TS will have AML 3/4 MULTIPLE 1/3 – 1/2 BILATERAL (probably more) variable amts. of FAT, Smooth mm., and vessels

Renal Cysts

[Figure 5-15-49]

Figure 5-15-49 Multiple renal angiomyolipomas in Tuberous Sclerosis

Tuberous Sclerosis. In addition to angiomyolipoma, the patients may also develop multiple and bilateral renal cysts [different patients] Neuroradiology

1199 1201

The Phakomatoses

Angiomyomatosis vs. Lymphangiomyomatosis •

Figure 5-15-50

[Figure 5-15-50]

•

“sporadic” cases, all are female ➢ 50% chylothorax ➢ Perilymphatic smooth mm. ➢ May have abdominal LN involvement In TS, males can be affected ➢ chylothorax is rare ➢ Periarterial smooth mm around pulmonary aa

Bone Islands [Figure 5-15-51] Hemangioblastomatosis or Von Hippel-Lindau Disease •

Chromosome 3

Pulmonary Lymphangioleiomyomatosis in Tuberous Sclerosis

von Hippel-Lindau • • • • • •

Incidence of 1/35K – 40K 6-7K pts in USA AUTOSOMAL DOMINANT NO RACIAL/SEXUAL PREDILECTION VARIABLE PENETRANCE / EXPRESSIVITY Chromosome 3p25-26

von Hippel-Lindau Syndrome: History • •

• •

•

1864 scattered reports of angiomatous lesions of both retina and cerebellum 1894 Collins (England) ➢ two sibs with retinal angioma 1904 von Hippel (Germany) ➢ familial retinal hemangioblastoma 1926 Lindau (Sweden) ➢ familial retinal and cerebellar hemangioblastomas 1964 Melmon and Rosen

von Hippel-Lindau • •

•

1. 2. ➢ ➢ ➢ 3. ➢ ➢ ➢ ➢

CNS and Retinal hemangioblastoma Hemangioblastoma and one: a. renal, pancreatic, hepatic, epididymal cyst b. pheochromocytoma c. renal cancer Family history and one: a. hemangioblastoma b. viscera c. pheochromocytoma d. renal cancer

Figure 5-15-51

von Hippel-Lindau SYNDROME: NIH Classification •

•

Type I – VHL w/o Pheo ➢ Renal/Pancreatic cysts, RCC ➢ most common type Type II – VHL with Pheo ➢ IIA Islet cell tumors (no cysts) ➢ IIB Renal/Pancreatic Disease ❖ least common type

Tuberous Sclerosis – Multiple bone islands. These could also be considered as “hamartomas”. The Phakomatoses

1200 1202

Neuroradiology

von Hippel-Lindau •

• • •

Figure 5-15-52

Hemangioblastoma ➢ Cerebellum ➢ Retina ➢ Medulla, Cord Cysts/Tumor ➢ Kidney ➢ Liver ➢ Pancreas Epididymis and Endolymphatic Cystadenoma Pheochromocytoma -Adrenal (Certain Families -Type II)

von Hippel-Lindau: Six Classic Lesions [Figure 5-15-52] • • • • • • •

Hemangioblastoma Retinal Angioma (Hemangioblastoma) Pancreatic Cyst Renal Cysts and Ca Pheochromocytoma Epididymal Cystadenoma Endolymphatic sac tumor

von Hippel-Lindau Syndrome. Retinal angioma demonstrated on flourescein angiogram

Figure 5-15-53

Hemangioblastoma: • •

•

True Neoplasm Endothelial Origin Hypervascular ➢ capillary to sinusoidal ➢ dilated feeding artery ➢ dilated draining vein ➢ slow flow Stromal Cells ➢ foamy, lipid -laden Hemangioblastoma – the classic “cyst with nodule” morphology

von Hippel-Lindau: Hemangioblastoma • • • •

Cerebellum 66% Retina (“angiomas”) 58% Spinal Cord / Roots 28% Medulla 14%

Figure 5-15-54

Hemangioblastoma [Figures 5-15-53 to 5-15-60]

Figure 5-15-55

Hemangioblastoma with capillaries and stromal cells

Hemangioblastoma – Sporadic on left, VHL on right (multiple lesions).

Neuroradiology

1203

The Phakomatoses

Figure 5-15-56

Figure 5-15-57

Hemangioblastomas span a spectrum from largely cystic to mostly solid

Hemangioblastoma – densely enhancing nodule persists into venous phase

Figure 5-15-58

Figure 5-15-59

Hemangioblastoma in the medulla oblongata

Hemangioblastoma – densely enhancing nodule persists into venous phase

Figure 5-15-60

Hemangioblastoma AND VHL • • • •

1/6-1/5 of solitary cerebellar hemangioblastomas are associated w/ VHL up to 1/2 of medullary HBL occur in VHL “ALL” Multiple HBL are VHL there was one family w/o VHL

Erythropoietin • • •

in cyst fluid Elevated ESR Elevated Hct

von Hippel-Lindau. Syringohydromyelia with multiple enhancing nodules of hemangioblastoma

The Phakomatoses

1202 1204

Neuroradiology

von Hippel-Lindau: Renal Manifestations CYSTS ANGIOMAS ADENOMAS CLEAR CELL CA

Figure 5-15-61

25%–63% 7% 14% 15%–50%

von Hippel-Lindau: Kidney •

[Figure 5-15-61]

Renal Cell Carcinoma ➢ Multiple ➢ Bilateral ➢ Conservative Surgery

von Hippel-Lindau. Multicystic renal cell carcinoma

von Hippel-Lindau: Pancreas [Figure 5-15-62] • •

• •

Pancreatic cysts 18%-72% Pancreatic adenoma 7% ➢ microcystic (“glycogen rich”) Pancreatic Ca ➢ reported in single family Islet Cell Tumors

Figure 5-15-62

Pancreatic Adenoma In VHL • • • • •

Microcystic (Not Macrocystic) Serous (Not Mucin Producing) Not Pre-Malignant Glycogen Rich Stellate Scar ➢ which may be visible, have Ca++

VHL – Visceral Manifestations

von Hippel-Lindau. Pancreatic Microcystic Adenoma – also called glycogen-rich adenoma - Note central stellate scar on gross image.

Pheochromocytoma and VHL • • • • • •

20% of ALL Pheochromocytoma are VHL Typically in Adrenal Present YOUNGER w/VHL Multiple with VHL Mortality (5% of VHL DIE from catecholamines) Workup: MR and MIBG (95% sensitive) ➢ 24hr NOREPINEPHRINE ➢ VMA (53% sensitive) ➢ US (40% sensitive)

Figure 5-15-63

Endolymphatic Sac Tumor [Figure 5-15-63] •

Petrous Apex Mass ➢ Cholesterol granuloma ➢ Glomus tumor ➢ Vascular variant ➢ Cystadenoma (endolymphatic sac tumor)

Von Hippel-Lindau •

•

•

Hemangioblastoma ➢ Cerebellum ➢ Retina ➢ Medulla, Cord Cysts/Neoplasms ➢ Kidney ➢ Liver ➢ Pancreas ➢ Epididymis ➢ Endolymphatic sac Pheochromocytoma -Adrenal

Neuroradiology

Endolymphatic Sac Tumor 1203 1205

The Phakomatoses

VHL - Multiple hemangioblastomas [Figure 5-15-64]

Figure 5-15-64

VHL - Multiple hemangioblastomas

Educational Objectives • • • • •

Describe why NF-1 is truly “Neurofibromatosis” Describe three neoplasms caused by the chromosome 22 mutation in NF-2 Describe the vascular abnormalities of Sturge Weber Syndrome Explain why Tuberous Sclerosis is a disorder of neuronal migration Distinguish von Hippel-Lindau from the ‘neurocutaneous’ phakomatoses

Summary - Phakomatoses Mnemonic Tool •

• • •

•

NF-1 (von Reck’s) ➢ TRUE Neurofibromatosis #17 NF-2 (Bil. VIII Syndrome) ➢ M.I.S.M.E. #22 STURGE-WEBER (Dimitri) Syndrome ➢ Congenital Vascular Lesion ➢ perhaps NOT inherited Tuberous Sclerosis ➢ Pringle’s “HAMARTOMA” Disease von Hippel-Lindau Syndrome ➢ NO cutaneous lesions ➢ Hemangioblastomas and Visceral Lesions

References Part 1 1. 2. 3. 4. 5. 6. 7. 8. 9.

Aizpuru RN, Quencer RM, Norenberg M, Altman N, Smirniotopoulos JG. Meningioangiomatosis: clinical, radiologic, and histopathologic correlation. Radiology 1991; 179:819-821. American Journal of Neuroradiology 8[6], 1031-1036. 1987 Aoki S, Barkovic AJ, Nishimura K, Kjos B, Brown EW, Riccardi VM et al. Neurofibromatosis Types-1 and Type2: Cranial MR Findings. Radiology 1989; 172(2):527-534. Baldwin D, King TT, Chevretton E, Morrison AW. Bilateral cerebellopontine angle tumors in neurofibromatosis type 2. J Neurosurg 1991; 74:910-915. Barker D, Wright E, Nguyen K, Cannon P. Gene for von Recklinghausen Neurofibromatosis is in the Pericentromeric Region of Chromosome 17. Science 1987; 236:1100-1102. Brown EW, Riccardi VM, Mawad M, Handel S, Goldman A, Bryan RN. MR Imaging of Optic Pathways in Patients with Neurofibromatosis. Brzowski AE, Bazan III C, Mumma JV, Ryan SG. "Spontaneous regression of optic glioma in a patient with neurofibromatosis.". Neurology 1992; 42(3):679-681. Chui MC, Bird BL, Rogers J. Extracranial and Extraspinal Nerve Sheath Tumors: Computed Tomographic Evaluation. Neuroradiology 1988; 30:47-53. Cohen MM. Invited Historical Comment: Further Diagnostic Thoughts About the Elephant Man. Am J Med Genetics 1988;777-782.

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10. DiMario FJ, Ramsby G, Greenstein R, Langshur S, Dunham B. "Neurofibromatosis Type 1: Magnetic Resonance Imaging Findings.". Journal of Child Neurology 1993; 8:32-39. 11. DiPaolo DP, Zimmerman RA, Rorke LB, Zacki EH, Bilaniuk LT, Yachnis AT. Neurofibromatosis Type 1: Pathologic Substrate of High-Signal-Intensity Foci in the Brain. 12. Domenicucci M, Santoro A, D'Osvaldo DH, Delfini R, Cantore GP, Guidetti B. Multiple Intracranial Meningiomas. J Neurosurg 1989; 70:41-44. 1989. 13. Eljamel MSM, Foy PM. Multiple Meningiomas and Their Relation to Neurofibromatosis. Review of the Literature and Report of Seven Cases. Surg Neurol 1989; 32:131-136. 14. Evans DG, Mason S, Huson SM, Ponder M, Harding AE, Strachan T. Spinal and cutaneous schwannomatosis is a variant form of type 2 neurofibromatosis: a clinical and molecular study. J Neurol Neurosurg Psychiatry 1997; 62(4):361-366. 15. Evans DGR, Huson SM, Donnai D, Neary W, Blair V, Newton V et al. A Clinical Study of Type 2 Neurofibromatosis. Quarterly Journal of Medicine 1992; 84:603-618. 16. Ferner RE, Chaudhuri R, Bingham J, Cox T, Hughes RAC. "MRI in neurofibromatosis 1. The nature and evolution of increased intensity T2 weighted lesions and their relationship to intellectual impairment.". J Neurol Neurosurg Psychiatry 1993; 56:492-495. 17. Halliday AL, Sobel RA, Martuza RL. Benign Spinal Nerve Sheath Tumors: Their Occurrence Sporadically and in Neurofibromatosis Types 1 and 2. J Neurosurgery 74:248-253, 1991. 18. Harkens K, Dolan KD. "Correlative Imaging of Sphenoid Dysplasia Accompanying Neurofibromatosis.". Ann Otol Rhinol Laryngol 1990; 99:137-141. 19. Harkin JC, Reed RJ. Tumors of the Peripheral Nervous System. Fascicle 3, Second Series, Atlas of Tumor Pathology. AFIP Washington D C 1969;-97. 20. Hurst RW, Newman SA, Cail WS. Multifocal intracranial MR abnormalities in neurofibromatosis. AJNR Am J Neuroradiol 1988; 9(2):293-296. 21. J Pediatr 125, 63-66. 1994 22. Kendall B, Symon L. Investigation of patients representing with cerebellopontine angle syndromes. 23. Listernick R, Charrow J, Greenwald M, Mets M. Natural History of Optic Pathway Tumors in Children with Neurofibromatosis Type 1: A Longitudinal Study. 24. Martuza RL, Eldridge R, Wertelecki W, Rouleau GA, Superneau DW, Forehand LW. Neurofibromatosis 2 (Bilateral Acoustic Neurofibromatosis) Neurofibromatosis 2: Clinical and DNA Linkage Studies of a Large Kindred. NEJM 1988; 319:278-283. 25. Mautner VF, Tatagiba M, Lindenau M, Funsterer C, Pulst SM, Baser ME et al. Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety [published erratum appears in AJR Am J Roentgenol 1996 May;166(5):1231]. AJR Am J Roentgenol 1995; 165(4):951-955. 26. Merten D, Gooding C, Newton T, Malamud N. Meningiomas of childhood and adolescence. J Peds 1974; 84:696700. 27. Mirowitz SA, Sartor K, Gado M. High-intensity basal ganglia lesions on T1-weighted MR images in neurofibromatosis. AJNR Am J Neuroradiol 1989; 10(6):1159-1163. 28. Moore BD, Slopis JM, Schomer D, Jackson EF, Levy BM. Neuropsychological significance of areas of high signal intensity on brain MRIs of children with neurofibromatosis. Neurology 1996; 46(6):1660-1668. 29. Mulvihill JJ, moderator. Neurofibromatosis 1 (Recklinghausen Disease) and Neurofibromatosis 2 (Bilateral Acoustic Neurofibromatosis): an update. Ann Intern Med 1990; 113:39-52. 30. National Institutes of Health Consensus Development Conference Statement on Acoustic Neuroma, December 1113, 1991. The Consensus Development Panel. Arch Neurol 1994; 51(2):201-207. 31. Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988; 45(5):575-578. 32. Neuroradiology 13, 65-84. 1977. 33. Pomeranz SJ, Shelton JJ, Tobias J, Soila K, Altman D, Viamonte M. MR of Visual Pathways in Patients with Neurofibromatosis. AJNR 1994; 8:831-836. 34. Radiology 195, 721-724. 1995 35. Riccardi V. Neurofibromatosis. Phenotype, Natural History, and Pathogenesis. 2 ed. Baltimore: The Johns Hopkins University Press, 1992. 36. Russell DS, Rubinstein LJ. Dysgenetic Syndromes (Phacomatoses) Associated with Tumors and Hamartomas of the Nervous System. Chap 11 (pgs 766-784) in Pathology of Tumors of the Nervous System Williams and Wilkins, Baltimore, 1989. 37. Seizinger BR, Martuza RL, Gusella JF. Loss of Genes on Chromosome 22 in Tumorigenesis of Human Acoustic Neuroma. Nature 322:644-647, 1986. 38. Smirniotopoulos JG, Murphy FM. The Phakomatoses. AJNR Am J Neuroradiol 1992; 13:725-746. 39. Stull MA, Moser RP, Kransdorf MJ, Bogumill GP, Nelson MC. Magnetic Resonance Appearance of Peripheral Nerve Sheath Tumors. Skeletal Radiology 20:9-14, 1991.

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References Part 2 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

15. 16. 17. 18. 19. 20. 21. 22.

23. 24. 25. 26. 27. 28. 29.

Afra D, Muller W, Slowik F, Firsching R. Supratentorial lobar pilocytic astrocytomas: report of 45 operated cases, including 9 recurrences. Acta Neurochir (Wien ) 1986; 81(3-4):90-93. Alexander GL. Sturge-Weber Syndrome. Chap 7 in The Phakomatoses Vinken PJ, Bruyn GW Eds., Vol. 14 of Handbook of Clinical Neurology. Elsevier Publishing Co , New York, 1972. Bachmann K, Markwalder R, Seiler RW. Supratentorial hemangioblastoma: Case Report. Acta Neurochirurgica 1978; 44:173-177. Barath B, Voros E, Bak Z, Bodosi M. Cerebral venous drainage via the ophthalmic veins in the Sturge-Weber syndrome. Neuroradiology 1994; 36(4):318-320. Bender BL, Yunis EJ. The Pathology of Tuberous Sclerosis. Path Annu 1982; 17:339-382. Choyke PL, Glenn GM, McClellan WM, Patronas NJ, Linehan WM, Zbar B. von Hippel-Lindau Disease: Genetic, Clinical, and Imaging Features. Radiology 1995; 194:629-642. Choyke PL, Glenn GM, Walther MM, Zbar B, Weiss GH, Alexander RB et al. "The Natural History of Renal Lesions in von Hippel-Lindau Disease: A Serial CT Study in 28 Patients.". AJR 1992; 159:1229-1234. Dahlen RT, Harnsberger HR, Gray SD, Shelton C, Allen R, Parkin JL et al. Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18:67-75. Dyke DG, Davidoff LM, Masson CB. Cerebral Hemiatrophy with Homolateral Hypertrophy of the Skull and Sinuses. Surgery, Gynecology and Obstetrics 1933; 57:588-600. Feghali JG, Levin RJ, Llena J, Bradley MK, Kantrowitz AB. Aggressive Papillary Tumors of the Endolymphatic Sac: Clinical and Tissue Culture Characteristics. Am J Otology 16[6], 778-782. 1995. Fetner CD, Barilla DE, Scott T, et al. Bilateral renal cell carcinoma in von Hippel-Lindau syndrome: Treatment with staged bilateral nephrectomy and hemodialysis. J Urol 17, 534-536. 1977. Filing-Katz MR, Choyke PL, Oldfield E, Charnas L, Patronas NJ, Glenn GM et al. Central nervous system involvement in Von Hippel-Lindau disease. Neurology 1991; 41:41-46. Filling-Katz MR, Choyke PL, Patronas NJ, et al. Radiologic scrreening for von Hippel-Lindau disease: The role of Gd-DTPA-enhanced MR imaging of the CNS. Journal of Computer Assisted Tomography 13, 745-755. 1989. Hirose T, Scheithauer BW, Lopes MB, Gerber HA, Altermatt HJ, Hukee MJ et al. Tuber and subependymal giant cell astrocytoma associated with tuberous sclerosis: an immunohistochemical, ultrastructural, and immunoelectron and microscopic study. Acta Neuropathol (Berl) 1995; 90:387-399. Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ. Radiologic-pathologic correlation: hemangioblastoma. AJNR 1992; 13:1343-1352. Hoang MP, Amirkhan RH. Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol 2003; 27(8):1152-1156. Huson SM, Harper PS, Hourihan MD, Cole G, Weeks RD, Compston DAS. Cerebellar Haemangioblastoma and von Hippel-Lindau Disease. Brain 1986; 109:1297-1310. Jelinek J, Smirniotopoulos JG, Parisi JE, Kanzer M. Lateral ventricular neoplasms of the brain: differential diagnosis based on clinical, CT, and MR findings. AJR 1990; 155:365-372. Koeller KK, Sandberg GD. From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologicpathologic correlation. Radiographics 2002; 22(6):1473-1505. Levine E, Weigel JW, Collins DL. Diagnosis and management of asympptomatic renal cell carcinomas in von Hippel-Lindau syndrome. Urology 21, 146-150. 1983. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM et al. von Hippel-Lindau disease. Lancet 2003; 361(9374):2059-2067. Megerian CA, McKenna MJ, Nuss RC, Maniglia AJ, Ojemann RG, Pilch BZ et al. Endolymphatic Sac Tumors: Histopathologic Confirmation, Clinical Characterization, and Implication in von Hippel-Lindau Disease. Laryngoscope 105, 801-808. 1995. Mukherji SK, Albernaz VS, Lo WW, Gaffey MJ, Mergerian CA, Feghali JG et al. Papillary endolymphatic sac tumours: CT, MR imaging, and angiographic findings in 20 patients. Radiology 202[3], 801-808. 1997. Murphy FM, Smirniotopoulos JG. Hemangioblastoma: Radiologic-Pathologic Correlation. Paper Presented at the 87th Annual Meeting of the Roentgen Ray Society . 1987. Neumann H. Basic criteria for clinical diagnosis and genetic couselling in von Hippel-Lindau Syndrome. VASA 1987; 16:220-226. Nixon JR, Houser OW, Gomez MR, Okazaki H. Cerebral Tuberous Sclerosis: MR Imaging. Radiology 170:869873, 1989. Nixon JR, Houser OW, Gomez MR, Okazaki H. Cerebral Tuberous Sclerosis: MR Imaging. Radiology 1994; 170:869-873. Ouallet JC, Marsot-Dupuch K, Van Effenterre R, Kujas M, Tubiana JM. Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel-Lindau disease. Case report. J Neurosurg 1997; 87:445-449. Ozek MM, Ozek E, Pamir MN, Ozer AF, Erzen C. Subependymal giant cell astrocytomas in tuberous sclerosis. Turk J Pediatr 1993; 35:145-150.

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30. Shepherd CW, Scheithauer BW, Gomez MR, Altermatt HJ, Katzmann JA. Subependymal giant cell astrocytoma. Neurosurg 1991; 28(6):864-868. 31. Sperner J, Schmauser I, Bittner R, Henkes H, Bassir C, Sprung C et al. MR-Imaging Findings in Children with Sturge-Weber Syndrome. Neuropediatrics 1990; 21:146-152. 32. Stimac GK, Solomon MA, Newton TH. CT and MR of Angiomatous Malformations of the Choroid Plexus in Patients with Sturge-Weber Disease. AJNR 1986; 7:623-627. 33. Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus 1992; 29(6):349-356. 34. Tibbs Jr. RE, Bowles Jr. AP, Raila FA, Fratkin JD, Hutchins JB. Should Endolymphatic Sac Tumors Be Considered Part of the Von Hippel-Lindau Complex? Pathology Case Report. Neurosurgery 40[4], 848-855. 1997. 35. Tishler PV. A Family with Coexistent von Recklinghausen's Neurofibromatosis and von Hippel-Lindau's Disease. Neurology 25:840-844, 1975. 36. van der Hoeve T. Eye Diseases in Tuberose Sclerosis of the Brain and in Recklinghausen's Disease. Transactions of the Ophthalmological Society of the United Kingdom 43; 1923;534-541. 37. Wippold FJ, Baber WW, Gado M, Tobben PJ, Bartnicke BJ. Pre- and Postcontrast MR Studies in Tuberous Sclerosis. J Comp Assist Tomogr 1992; 16:69-72. 38. Wippold II FJ, Baber WW, Gado M, Tobben PJ, Bartnicke BJ. Pre- and Postcontrast MR Studies in Tuberous Sclerosis. J Comp Assist Tomogr 1992; 16:69-72. 39. Wylie IG, Jeffreys R, MaClaine GN. Cerebral hemangioblastoma. Br J Radiol 1973; 46:472-476.

Neuroradiology

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Subarachnoid Hemorrhage and Intracranial Aneurysms Mary E. Jensen, MD Epidemiology • •

•

Most common cause of non-traumatic SAH is aneurysmal rupture Annual incidence of aneurysmal SAH: 1:10,000 ➢ Higher than primary brain tumors, MS ➢ Remained stable over last 30 yrs ➢ Females 1.6 x over males ➢ Blacks 2.1 x over whites ➢ Higher in Japan and Finland Accounts for 2-5% of all new strokes each yr ➢ 21,000 – 33,000 Americans ➢ Average age much lower than other strokes ❖ Peaks in 6th decade

Epidemiology - Prevalence of Unruptured Aneurysms •

•

Common incidental finding ➢ 3.6% on prospective autopsy series ➢ 6% on prospective angiography series ➢ Highest in patients with AD polycystic kidney disease, familial predisposition, atherosclerosis Multiplicity ➢ 20%-30% of patients have multiple aneurysms ❖ Usually 2 or 3

Figure 5-16-1

Types of Aneurysms •

[Figures 5-16-1 and 5-16-2]

•

• •

Saccular (“berry”) ➢ 90% of all aneurysms Fusiform ➢ Serpentine: Partially thrombosed aneurysm containing tortuous vascular channels ➢ Cirsoid: Dilated, elongated and tortuous Dissecting Blister/bleb

Saccular, paraophthalmic (left) Fusiform, vertebral (center) Serpentine, MCA (right)

Figure 5-16-2 Non-Modifiable Risk Factors •

•

•

Personal history of SAH ➢ Risk of developing a new aneurysm: 2% ❖ Annual incidence of SAH: 6:10,000 Family history of SAH ➢ 5%-20% of patients with SAH have a positive family history ❖ First-degree relatives of pts with SAH have 37X increased risk ➢ Second-degree relatives have same risk as general population ➢ Probably an autosomal transmission that does not follow a specific mendelian model Female gender Dissecting, PICA (left) Blister-bleb, AComA (right) ➢ Before the 5th decade, the risk is higher in men ➢ Risk is greater in post-menopausal women than men ❖ Supplemental low-estrogen hormones may impart some protection

Subarachnoid Hemorrhage-Intracranial Aneurysms

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Neuroradiology

Non-Modifiable Risk Factors •

•

•

Figure 5-16-3

Age ➢ Very rare in children Heritable disorders of connective tissue ➢ AD polycystic kidney disease ➢ Ehlers-Danlos disease IV ➢ Neurofibromatosis type 1 ➢ ? Fibromuscular dysplasia, Marfan’s syndrome Associated with anatomic variants ➢ Persistent trigeminal artery ➢ Fenestrations ➢ Azygous ACA Mid-basilar aneurysm with severe distal stricture (arrows)

Aneurysms in Children • • •

Figure 5-16-4 Incidental aneurysms rarely found at autopsy Etiology: trauma, infection, congenital Location, size, age and presentation are different from adults • Age of presentation bimodal ➢ 0-6 yrs, then 8-adolescence • Posterior circulation aneurysms 3x more prevalent • Large/giant aneurysms more common ➢ greater proportion of giant aneurysms, pts < 2 yrs 47 y.o. female with Marfan’s and rapidly growing mid-basilar Fusiform Basilar Aneurysm in a aneurysm Child [Figure 5-16-3] Aneurysm Associated with Connective Tissue Disorder [Figure 5-16-4]

Aneurysm Associated with Anatomical Variants

[Figure 5-16-5]

Modifiable Risk Factors •

• •

• •

Smoking ➢ Only risk factor that has been consistently identified in all populations studied ➢ Risk is 3-10X higher than non-smokers ➢ Risk is proportional to number of cigs ➢ Increased risk of new aneurysm formation in pts with SAH who continue to smoke Hypertension ➢ Probably a risk factor for both SAH and aneurysm formation Alcohol consumption ➢ Heavy, binge drinking Cocaine use ? Hypercholesterolemia, DM, obesity

Figure 5-16-5

Bilobed vertebrobasilar junction aneurysm associated with fenestration (left). Large aneurysm associated with an azygous ACA (right)

Neuroradiology

1209 1211

Subarachnoid Hemorrhage-Intracranial Aneurysms

Pathogenesis: Saccular Aneurysms •

•

Congenital defect in tunica media no longer considered the cause of aneurysm formation ➢ Gaps in muscles layers are also seen in unaffected individuals ➢ In aneurysms, the gap is not at the neck but in the sac Acquired changes due to environmental factors (HTN, smoking, EtOH abuse) are more likely ➢ Formation of intimal thickening proximal and distal to branch points ❖ May cause increased strain in the more elastic portion of the wall ➢ Structural abnormalities in structure proteins of the extracellular matrix

Figure 5-16-6 Pathogenesis: Saccular Aneurysms Hemodynamic factors • Wall shear stress ➢ Frictional force of viscous blood ❖ High WSS fragments the internal elastic lamina - Initiation of aneurysm formation ❖ Low WSS degenerates endothelial cells via apoptosis - Responsible for aneurysm growth/rupture • Increased flow ➢ 10%-20% of patients with brain AVMs have aneurysms

Pathogenesis: Fusiform Aneurysms •

Fusiform ➢ Four major histological findings ❖ Fragmentation of internal elastic lamina Double channel appearance of ❖ Neoangiogenesis within the thickened intima dissecting aneurysm aneurysm ❖ Intramural hemorrhage and thrombus formation ❖ Repetitive intramural hemorrhage from neovascularity within the Figure 5-16-7 thrombus ➢ Similar histologic features seen with ASVD ASVD starts with lipid deposition, not fragmentation of the IEL

Pathogenesis: Dissecting Aneurysms •

Cystic medial necrosis ➢ Widespread disruption of the arterial wall ❖ Medial disruption with subadventitial dissecting hemorrhage causing true lumen stenosis ❖ Formation of a dilated pseudoaneurysm covered only by thin adventitia

AP and lateral views show “pearl and string” appearance of dissecting aneurysm

CTA and MRA of Dissecting Vertebral Aneurysm [Figure 5-16-6] DSA of Dissecting Vertebral Artery Aneurysm [Figure 5-16-7] Clinical Presentation •

Subarachnoid hemorrhage ➢ Unique headache ➢ Nausea/vomiting ➢ Meningeal irritation ➢ Photophobia ➢ Focal or global neurological deficits ➢ Subhyaloid hemorrhages

Subarachnoid Hemorrhage-Intracranial Aneurysms

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Neuroradiology

Subarachnoid Hemorrhage Patterns [Figure 5-16-8]

Figure 5-16-8

Clinical Presentation •

•

Mass effect ➢ Headache ➢ Cranial nerve palsies ➢ Focal neurological findings ➢ Seizures ➢ Risk of rupture of unruptured aneurysm causing mass effect is 6%/yr Cerebral ischemia ➢ Distal embolization from intra-aneurysmal thrombus

Giant ICA Terminus Aneurysm [Figure 5-16-9] Outcomes of SAH • • •

•

12% die before reaching hospital 40% of hospitalized patients die within first month >1/3 of survivors have major neurological deficits ➢ Unable to live independently Many “good outcomes” have persistent cognitive deficits

AComA pattern (left images) - Basilar tip pattern (right images)

Outcomes of SAH - Major factors associated with poor outcome •

Patient’s level of consciousness on admission ➢ Based on the sum score of the Glasgow Coma Scale ❖ Eye opening (4 pts), best motor response (6 pts), best verbal response (5 pts) ➢ Age ➢ Amount of blood on CT ➢ Predicts risk of delayed cerebral ischemia using two parameters ❖ Amount of subarachnoid clot ❖ Ventricular hemorrhage ➢ Risk is additive

Figure 5-16-9

59 y.o. female evaluated for headache and cognitive dysfunction

Clinical Grading Scale [Figure 5-16-10] Radiologic Grading Scale [Figure 5-16-11]

Figure 5-16-10

Neuroradiology

Figure 5-16-11

1211 1213

Subarachnoid Hemorrhage-Intracranial Aneurysms

SAH-induced Vasospasm • • • • • •

Figure 5-16-12

Occurs angiographically in 30%-70% of patients Clinical symptoms seen in 20%-45% of patients Adds 10%-20% significant morbidity/mortality Smooth muscle constriction and vessel wall edema, infiltration and fibrosis leads to luminal narrowing and decreased compliance Time course ➢ Range: 4-14 days ➢ Peak: 7-10 days Treatment ➢ Fluid status, blood pressure, calcium channel Severe vasospasm of the supraclinoid ICA and M1 blockers segment leading to poor perfusion of the right MCA ➢ Induced hypertension territory ➢ Angioplasty, intra-arterial infusion of vasodilators

Figure 5-16-13

SAH-induced Vasospasm [Figure 5-16-12] Risk of Unruptured Aneurysm [Figure 5-16-13] Diagnostic Testing: Non-contrast Head CT •

•

First imaging study ➢ Detect 98%-100% of cases in first 12 hours ➢ Sensitivity goes down to 30% at 2 wks Reportable findings ➢ Presence, amount, location of blood ❖ Increased density in subarachnoid space ❖ Predominately in basilar cisterns ➢ Intraparenchymal hematoma ➢ Hydrocephalus ➢ Cerebral edema, herniation ➢ Negative image of aneurysm in SAH

Figure 5-16-14

CT of Subarachnoid Hemorrhage [Figure 5-16-14]

CT Findings of Ruptured Aneurysm [Figure 5-16-15] Diagnostic Testing: Lumbar puncture Usually done when CT is negative or equivocal • Should be done 6-12 hours after event ➢ Time it takes for xanthochromia to occur ➢ Xanthochromia is diagnostic ❖ Detectable in all patients between 12 hrs and 2 wks • Findings ➢ Elevated opening pressure ➢ Elevated RBCs that do not clear ❖ Unreliable way to r/o traumatic tap ➢ Xanthochromia

Acute SAH (left image)-SAH 5 days post bleed with CTA showing PComA aneurysm (right two images)

Figure 5-16-15

NCCT shows extensive SAH, intraparenchymal hematoma with fluid-fluid level, uncal and subfalcine herniation, small amount of intraventricular blood, and an MCA aneurysm (arrow) Subarachnoid Hemorrhage-Intracranial Aneurysms

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Neuroradiology

Diagnostic Testing: MRI •

Figure 5-16-16

FLAIR detects early SAH as well as CT ➢ Impractical ❖ Equipment availability ❖ Patient motion ➢ Better than CT for detecting older SAH ❖ Great for patients with negative CT, positive LP who are not referred immediately

SAH on CT and MRI FLAIR [Figure 5-16-16] Diagnostic Testing: CTA •

•

Advantages ➢ High sensitivity (97%-100%) ➢ Equal to IA DSA ➢ Good even if SAH present ➢ Source images, reconstructions Disadvantages ➢ Sensitivity varies with size ➢ Use of iodinated contrast and radiation ➢ Negative study requires IA DSA ➢ Technical issues ❖ Post-processing time is substantial ❖ Venous contamination with poor cardiac output

CTA with Active Extravasation [Figure 5-16-17]

CT three days and MR four days post-SAH (arrows) with interval development of intraventricular hemorrhage seen on MR

Figure 5-16-17

CTA shows the location, size and relationship of the aneurysm to the surrounding branches, and active extravasation into the hematoma

CTA of Small Aneurysms Figure 5-16-18

[Figure 5-16-18]

Diagnostic Testing: MRA •

•

Advantages ➢ Non-invasive ➢ High sensitivity ❖ 86%-100% in aneurysms 3-5 mm ❖ 81%-100% in cases of SAH ➢ Multiplanar viewing Disadvantages ➢ Complex/disturbed flow degrades image ➢ Availability of equipment ➢ Patient movement ❖ Anesthesia needed for uncooperative patients

MRA of Multiple Aneurysms [Figure 5-16-19]

CTA of Small Aneurysms

Figure 5-16-19

Diagnostic Testing: DSA • •

• •

Gold standard Carries risk of complication ➢ 1.8% transient or permanent ➢ SAH patients hypercoagulable Detects smallest aneurysms Gives important information for treatment decisions Following coiling of the three aneurysms larger than 2 mm, the MRA shows no change in the two untreated aneurysms

Neuroradiology

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Subarachnoid Hemorrhage-Intracranial Aneurysms

DSA: Evaluation of Aneurysm • • •

• • • • •

Figure 5-16-20

Location and number Size and configuration Characteristics of aneurysm neck ➢ neck to dome ratio; absolute size of neck ➢ slit, oval, round, wide ➢ relationship to adjacent vessels Suspected rupture site (“tits”) Collateral circulation distal to aneurysm Hemodynamics Calcification Intraluminal thrombus

Location •

•

Anterior circulation (80%-85%) ➢ ICA/PComA junction ➢ AComA complex ➢ MCA trifurcation Posterior circulation (15%-20%) ➢ Basilar terminus (5%) ➢ PICA ➢ SCA

Aneurysm Locations: Anterior Circulation

Common locations for aneurysms on the anterior circulation

[Figure 5-16-20]

Aneurysm Locations: Posterior Circulation

Figure 5-16-21

[Figure 5-16-21]

Aneurysm Measurements •

•

Aneurysm ➢ Small: less than 10 mm ➢ Large: 10-25 mm ➢ Giant: > 25 mm Aneurysm neck ➢ Absolute size ❖ Small: 4mm or less ➢ Dome to neck ratio ❖ Small: 2:1 or greater

Common locations for aneurysms on the posterior circulation

Aneurysm Sizes [Figure 5-16-22] Figure 5-16-22

Small superior hypophyseal (left) Large paraophthalmic (center) Giant ICA terminus (right)

Subarachnoid Hemorrhage-Intracranial Aneurysms

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Neuroradiology

3D Angiography [Figure 5-16-23]

Figure 5-16-23

Rupture Sites [Figure 5-16-24] Imaging Algorithm for Suspected SAH Non-contrast head CT • SAH ➢ CTA or DSA If negative, repeat CTA in 1-3 weeks If negative, image the brain and spinal cord • No SAH ➢ LP Abnormal or equivocal, go to SAH algorithm

Imaging Algorithm for Screening •

3D angiography better defines the aneurysm neck than biplane DSA

Figure 5-16-24

For high risk patients ➢ Family history of intracranial aneurysms ❖ Asymptomatic individuals with two or more affected members ❖ Non-invasive imaging every >1 year, < 5 years ➢ Asymptomatic adults with autosomal dominant polycystic kidney disease ❖ 5%-10% asymptomatic patients have aneurysms ❖ Clustering in some families with 20%-25% with aneurysms

Paraophthalmic (left) AComA (center) Basilar tip (right)

Other Causes of SAH • • • • • •

Non-aneurysmal perimesencephalic SAH Infectious intracranial aneurysms Brain AVMs ➢ Flow related aneurysms ➢ Intra/perinidal aneurysms Dural AVMs Intracranial dissections ➢ Spontaneous ➢ Iatrogenic Spinal vascular malformations

Non-aneurysmal Perimesencephalic Hemorrhage • •

•

•

Responsible for 10% of non-traumatic SAH Defined only by the characteristic location of blood and lack of aneurysm ➢ Blood confined to perimesencephalic cistern ➢ Centered anteriorly ➢ May have small amount of sedimentation in posterior horns ➢ 2.5%-10% of posterior fossa aneurysm hemorrhages mimic this pattern Clinical findings ➢ Gradual headache ➢ Focal findings, LOC uncommon and transient ➢ Seizure at presentation essentially excludes the diagnosis ➢ 1/3 with transient amnesia Outcomes ➢ Short convalescence ➢ No rebleeding ➢ No symptomatic vasospasm

Neuroradiology

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Subarachnoid Hemorrhage-Intracranial Aneurysms

Non-aneurysmal Perimesencephalic Hemorrhage [Figure 5-16-25]

Figure 5-16-25

Infectious Intracranial Aneurysms • • •

•

•

Rare- 2%-6% of all brain aneurysms Presentation: stroke,seizure, SAH Pathology ➢ Septic embolization of lumen or vasovasorum ❖ Focal arteritis, necrosis, and aneurysm formation Location ➢ Distal MCA>PCA and ACA Treatment ➢ Resection, parent artery occlusion ➢ Antiobiotic therapy

Infectious Intracranial Aneurysms [Figure 5-16-26]

Non-aneurysmal perimesencephalic hemorrhage

Figure 5-16-26

Other Causes of SAH • • • • •

Saccular aneurysm of spinal artery Atrial myxoma Pituitary apoplexy Coagulation disorders Superficial siderosis of the CNS

Aneurysms: Treatment Options • •

• •

Nothing Follow ➢ Very small unruptured aneurysms ➢ Unruptured aneurysms in cavernous carotid/carotid cave Surgical clipping Endovascular treatment ➢ Reconstructive: coiling +/- stent placement/balloon remodeling ❖ Saccular aneurysms ➢ Deconstructive: parent artery occlusion ❖ Giant, dissecting, septic

Separate patients with septic aneurysms (Courtesy A. Brooks, M.D.)

Figure 5-16-27

Treatment Outcomes •

•

ISAT trial Permanent occlusion of the left ICA with detachable balloons ➢ Comparative study of coiling vs. results in flow perpendicular to the neck of the aneurysm and clipping clotting of the dome ❖ 23.9% relative/7.4% absolute risk reduction for coiling vs. clipping Figure 5-16-28 ➢ Controversial study Should patients be offered the option of coiling vs. clipping in the acute setting?

Deconstructive Therapy [Figures 5-16-27 and 5-16-28]

A paraophthalmic aneurysm treated by endosaccular occlusion with coils

Subarachnoid Hemorrhage-Intracranial Aneurysms

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Neuroradiology

References 1. 2. 3. 4.

5.

6. 7. 8. 9. 10. 11. 12. 13.

Aryan H, Giannotta SL, Fukushima T, et al. Aneurysms in children: review of 15 years experience. J Clin Neurosci 2006;13(2):188-92. Claassen J, Bernardini GL, Kreiter K, et al. Effect of cisternal and ventricular blood on risk of delayed cerebral ischemia after subarachnoid hemorrhage: the Fischer scale revisited. Stroke 2001; 32:2012-20. Hamada Y, Mannoji H, Kaneko Y. A ruptured dissecting aneurysm of the vertebral artery: comparison of the angiographic and histological findings. Neuroradiology 2001; 375-8. Hoh BL, Cheung AC, Rabinov JD, et al. Results of a prospective protocol of computed tomographic angiography in place of catheter angiography as the only diagnostic and pretreatment planning study for cerebral aneurysms by a combined neurovascular team. Neurosurgery 2004; 54(6):1329-40. Molyneux A, Kerr RS, Yu LM, et al. International subarachnoid aneurysm trial (ISAT) of neurosurgical clipping versus endovascular coiling in 2143 patients with ruptured intracranial aneurysms: a randomised comparison of effects on survival, dependency, seizures, rebleeding, subgroups, and aneurysm occlusion. Lancet 2005; 366(9488):809-17. Nakatomi H, Segawa H, Kurata A, et al. Clinicopathological study of intracranial fusiform and dolichoectatic aneurysms: insight on the mechanism of growth. Stroke 200; 31:896-900. Schievink W. Intracranial aneurysms. [Review] NEJM 1997; 336(1):28-40. Shojima M, Oshima M, Takagi K, et al. Magnitude and role of wall shear stress on cerebral aneurysm: computation fluid dynamic study of 20 middle cerebral artery aneurysms. Stroke 2004; 35:2500-05. Suarez JI, Tarr RW, Selman WI. Aneurysmal subarachnoid hemorrhage. Review NEJM 2006; 354:387-96. Van Gijn J, Rinkel GJ. Subarachnoid hemorrhage: diagnosis, cause and management. [Review Article] Brain 2001; 124:249-78. Velthuis BK, Rinkel GJ, Ramos LM, et al. Subarachnoid hemorrhage: aneurysm detection and preoperative evaluation with CT angiography. Radiology 1998; 208: 423-430. Wani AA, Behari S, Sahu RN, et al. Paediatric intracranial aneurysms. J Pediatr Neurosci 2006; 1:11-15. Wiebers DO, Whisnant JP, Huston J 3d, et al. Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet 2003; 362(9378):103-110.

Neuroradiology

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Subarachnoid Hemorrhage-Intracranial Aneurysms

Intracranial Vascular Malformations Mary E. Jensen, MD Classification of IVMs •

•

Traditional (path-based) ➢ Arteriovenous malformations ❖ Pial ❖ Dural ➢ Venous vascular malformations ❖ Venous angioma ➢ Capillary telangectasias ➢ Cavernous angiomas Functional (flow-based) ➢ AV Shunting ❖ Pial AVM ❖ Cerebral AVF ❖ Dural AVF ➢ Non-shunting ❖ Capillary malformations ❖ Venous malformations ❖ Cavernous malformations

Epidemiology •

• •

Detection rate of AVM ➢ 1.1:100,000 (excluding autopsy cases) ➢ 2.1:100,000 (including autopsy cases) ➢ 1.2:100,000 person-years (symptomatic) Prevalence ➢ 0.1% of US population (300,000 people) ➢ Point prevalence 18:100,000 adults Account for ➢ 1%-2% of all strokes ➢ 3% of strokes in young adults ➢ 9% of subarachnoid hemorrhage ➢ 4% of all intraparenchymal hemorrhages ❖ 1/3 of young adult hemorrhages

Etiology •

•

Congenital ➢ Sporadic Genetic ➢ Hereditary hemorrhagic telangectasia ❖ 4%-13% with cerebral AVMs ❖ ?ENG, ACVR1 gene mutations ❖ Encode proteins in TGFB1 receptor complexes ➢ Neurocutaneous disorders

Pathogenesis •

•

Wedge-shaped abnormal tangle of arteries and veins ➢ Arteries and veins linked by fistulae ➢ No normal capillary bed ➢ Deficient muscularis in small arteries Hemodynamic effects ➢ Fistulous effects ❖ High flow, rapid shunting ❖ Induced hypotension in feeders and adjacent areas (“arterial steal”) ➢ Opening of collateral pathways, “angiomatous change” ➢ Dysplastic appearance of feeding pedicles, “flow-induced angiopathy” ❖ Luminal dilatation or stenosis ❖ Aneurysms

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Neuroradiology

Clinical Presentation •

•

Usually present before 40 years of age ➢ Men and women equally affected Hemorrhage is #1 complication (>50%) ➢ 2%-4% yearly rupture rate ❖ 6%-18% annual rate after first bleed - Increased rate for the first year reported but not consistently noted ➢ 10%-18% mortality from hemorrhage ❖ 1.0%-1.5% annual rate

Clinical Presentation •

• • • •

Seizures: 20%-25% ➢ Generalized more common than focal Headache: 15% Focal neurologic deficit: <5% ➢ Steal phenomenon is uncommon Pulsatile tinnitus Children (< 2 yr) ➢ Congestive heart failure ➢ Large head from hydrocephalus ➢ seizures

Location •

Supratentorial (85%), infratentorial (15%) ➢ Cortical AVMs (72%) ❖ Gyral, sulcal, mixed ➢ Subcortical AVMs (1%) ➢ Deep AVMs (27%) ❖ Subarachnoid, parenchymal, plexal, mixed

Figure 5-17-1

Hemorrhage Predictors •

Many are not consistent ➢ Radiologic findings ➢ Prior hemorrhage ➢ Small AVM in diameter or volume ➢ Increased feeding artery pressures ➢ Periventricular/intraventricular location

Radiologic Predictors •

An enlarged lenticulostriate running through the ventricle is the presumed source of hemorrhage

Features that may result in a higher risk of bleeding ➢ Arterial aneurysms (10%) ➢ Intranidal aneurysms (20%-58%) ➢ Arterial supply from perforators ➢ Location ❖ Intraventricular/periventricular ❖ Basal ganglia, thalamus ➢ Deep venous drainage ➢ Single venous drainage outlet ➢ Venous stenosis

Figure 5-17-2

Intraventricular Hemorrhage [Figure 5-17-1]

CTA, MRA and DSA all show intranidal aneurysm within a small AVM

Intraparenchymal Hemorrhage [Figure 5-17-2]

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Intracranial Vascular Malformations

Hemorrhage Risk: Summary •

•

Lowest-risk group (1%) ➢ No history of prior hemorrhage ➢ >1 draining vein Highest-risk group (8.9%) ➢ History of prior hemorrhage ➢ Single draining vein ➢ Diffuse nidus

AVM Grading • •

There are many grading systems The most commonly used is the Spetzler-Martin system ➢ Size (<3 cm = 1, 3-6 cm = 2, >6 cm = 3) ➢ Eloquence of surrounding brain (non-eloquent = 0, eloquent = 1) ➢ Pattern of venous drainage (superficial = 0, deep = 1)

Spetzler-Martin Grading System • •

•

This system is used to stratify surgical outcome Grades I through II ➢ Extremely low rates of surgically related morbidity and mortality Grade IV and V ➢ High risk

Spetzler-Martin Grading Scale •

Grade III ➢ Heterogeneous group ❖ S1V1E1 same risk as I/ II (III-) - Microsurgery ❖ S2V0E1 same risk as IV/V (III+) - Manage conservatively ❖ S2V1E0 - Intermediate risk - Judicious selection for surgery

Figure 5-17-3

Radiographic Evaluation •

•

CT ➢ Usual first study ➢ Hemorrhage, calcifications, parenchymal changes, iso- or hyperdense serpentine structures ➢ Contrast study outlines boundaries CTA ➢ Vascular elements ❖ Location of feeding arteries/draining veins ❖ Associated aneurysms ➢ Volumetric determination

NCCT shows enlarged MCA trunk and sylvian arteries, intraventricular hemorrhage, focal atrophy and hyperdense vessels

Figure 5-17-4

CT of Brain AVM [Figure 5-17-3] CTA of Brain AVM [Figure 5-17-4]

CTA shows AVM components and their relationship to the parenchymal hemorrhage Intracranial Vascular Malformations

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Neuroradiology

Radiographic Evaluation •

•

Figure 5-17-5

MR ➢ Location and topography ➢ Presence or absence of acute, subacute or chronic hemorrhage ➢ Associated parenchymal changes such as edema, ischemia, gliosis, atrophy, mass effect, radiation effects MRA ➢ Same as CTA ➢ Useful in stereotactic radiosurgery planning

MRI of Brain AVM [Figure 5-17-5] MRA of Brain AVMs [Figure 5-17-6] Radiographic Evaluation •

Angiography ➢ Arterial supply, regional and individual ➢ High flow arteriopathy ❖ stenosis ❖ dolichoectasia ❖ flow related aneurysms (10%) ➢ Arterial supply to the brain ❖ Pial ❖ Dural ➢ Assessment of nidus ❖ plexiform vs. fistulous ❖ intra-nidal aneurysms and ectasias ❖ true nidus vs. angiomatous change

T1 images without and with Gd (left images) T2 and FLAIR images (right images)

Figure 5-17-6

DSA of Cerebral AVMs [Figure 5-17-7] Nidal Components [Figure 5-17-8] En Passage Vascular Supply [Figure 5-17-9] AVM Associated Aneurysms [Figure 5-17-10] MRA of shows extent of parietal AVM, and the enlarged feeding arteries and draining veins

Angiomatous Change [Figure 5-17-11]

Figure 5-17-8 Figure 5-17-7 Superselective injections of a temporal lobe AVM (A) show different nidal components including a macrofistula (B) and a racemose nidus (C)

Typical appearance of gyral (left) and sulcal (right) AVM

Neuroradiology

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Intracranial Vascular Malformations

Radiographic Evaluation •

Figure 5-17-9

Angiography ➢ Venous drainage, regional and individual ➢ High-flow venopathy ❖ dural sinus high-flow ❖ venous thrombosis ❖ venous enlargement, stenoses, varix ➢ Normal drainage of the brain

Venous Abnormalities [Figure 5-17-12] Treatment •

Controversial ➢ Observation ➢ Microsurgery ➢ Embolization ➢ Stereotactic radiosurgery ➢ Combination therapy

Superselective study (right) of temporal AVM shows multiple “en passage” feeders with distal arterial supply to normal brain

Figure 5-17-10

Arterial feeder (left)

Perinidal (center)

Intranidal (right)

Figure 5-17-11

Figure 5-17-12

Difference between angiomatous change (circle) and nidus (box)

Intracranial Vascular Malformations

Two patients with high-flow venopathy—a venous aneurysm in a deep AVM (left) and a venous varix with a stricture (right, arrow)

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Neuroradiology

Pre-Radiosurgery Embolization [Figure 5-17-13]

Figure 5-17-13

Intracranial AVFs •

•

Dural ➢ Most common type of cerebral AV fistula ➢ Shunt occurs primarily from dural arteries to dural sinuses or cortical veins Cerebral ➢ Rare ➢ Pediatric population ❖ Vein of Galen malformation ❖ Pial

Pre- and post-embolization of distal pericallosal artery with coils and NBCA

Etiology/Pathogenesis •

•

Adults (Dural AVF) ➢ Acquired lesions ❖ Present in later life (40s-60s) ❖ Shunting within dural/venous wall ❖ Sinus thrombosis - Environment conducive to the development of DAVF - Triggered by factors which stimulate angiogenesis Children (Pial AVF) ➢ Congenital lesions ❖ ?vascular remodeling of the venous endothelial cells in the capillary bed caused by a trigger such as hypoxia

Figure 5-17-14

Newborn with congestive heart failure and cranial bruit

Pial A-V Fistula [Figure 5-17-14] Location of DAVFs • • • • • • •

Transverse - sigmoid sinus: Cavernous sinus: Tentorial - incisural: Convexity - sagittal sinus: Orbital - anterior falx area: Sylvian - middle fossa area: Others: marginal sinus, etc

62.6 % 11.9 % 8.4 % 7.4 % 5.8 % 3.7 %

Classification of DAVFs •

Borden, Djindjian and Merland, Cognard ➢ Venous drainage ❖ Sinus vs. cortical veins ➢ Direction of flow ❖ Antegrade, retrograde, both ➢ Involvement of cortical venous drainage ❖ Benign vs. aggressive ❖ Determines risk

Borden Classification

Neuroradiology

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Intracranial Vascular Malformations

Benign: All Classifications [Figure 5-17-15]

Figure 5-17-15

Aggressive: All Classifications [Figure 5-17-16] Clinical Presentation • •

Symptoms and signs vary with location Tinnitus, bruit ➢ Cranial nerve palsies ➢ Orbital congestion ➢ Focal neurological deficits ➢ Global neurological deficits ➢ Hemorrhage ❖ ICH: 35%-42% overall; 75%-95% in tentorial, ACF

Benign transverse sinus DAVF with fistulous flow only to sinus

Figure 5-17-16

Hemorrhage Associated with DAVF [Figure 5-17-17]

Clinical Outcomes •

• •

Without cortical venous involvement ➢ Symptom improvement or resolution ❖ No treatment: 81% ❖ Treatment: 86% With cortical venous involvement ➢ non-hemorrhagic neurological deficit: 6.9%/yr ➢ Hemorrhage: 8.1%/yr ➢ Mortality: 10.4%/yr Malignant transformation of benign lesion: <1%

Aggressive transverse sinus DAVF with all fistulous flow into the cortical veins

Figure 5-17-17

Radiographic Evaluation •

•

Acute symptoms ➢ CT to rule out hemorrhage ➢ MRI/A, CTA to show prominent vessels ❖ Not adequate for demonstration of shunt ➢ DSA ❖ Confirm diagnosis ❖ Identify angioarchitecture ❖ Evaluate hemodynamics Chronic symptoms ➢ Contrast CT/MR ➢ DSA Tentorial DAVF with SAH (left) and transverse sinus DAVF with ICH (right)

Aggressive DAVF [Figure 5-17-18]

Figure 5-17-18

Treatment •

•

Benign ➢ Observation Aggressive ➢ Endovascular therapy ❖ Transvenous, transarterial, combined ➢ Surgery ❖ Failed endovascular therapy ❖ Isolated sinus that required direct puncture/exposure

Developmental Venous Anomaly •

Most common type of vascular malformation

Intracranial Vascular Malformations

Diffusion study shows temporal lobe edema and old hemorrhage; Gd MRI shows marked venous congestion and a multi-channel left transverse sinus; DSA confirmed development of a DAVF

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Neuroradiology

➢ Accounts for 60% of CVM ➢ 2.5%-9% prevalence on enhanced MR • Congenital ➢ Develop during 30th to 45th day as embryo ➢ Focal underdevelopment of superficial or deep “adult” veins ➢ Anomalous medullary veins converge into a centrally located collector vein Caput medusa

Pathology •

• •

Veins are closely aggregated and dilated ➢ lack muscular and elastic fibers ➢ abnormally hyalinized Normal adjacent arteries Adjacent parenchyma ➢ Normal ➢ Gliosis, neuronal degeneration, demyelination ➢ May be associated with focal neuronal migrational abnormalities

Clinical Presentation •

•

•

•

Incidental finding in most cases ➢ Associated with H/N vascular malformations Rarely cause neurologic symptoms ➢ Posterior fossa ❖ Dizziness, ataxia, diplopia ➢ Cerebrum ❖ Seizures, headaches, focal deficits Hemorrhage ➢ Not the DVA that hemorrhages, but the associated cavernoma Treatment ➢ Surgery may be required to remove hemorrhage/cavernoma ➢ Leave DVA

Imaging Findings •

•

•

•

NCCT ➢ Normal ➢ Ill-defined hyperdense area without edema or mass effect CECT ➢ Diffuse enhancement of linear vessels adjacent to the ventricle ❖ Stellate pattern ❖ Converge on collector vein ❖ Well-visualized on CTA MR ➢ Variable degrees of T2 and T1 prolongation in the adjacent parenchyma ❖ Due to increased blood pool ➢ Signal intensity void in draining vein on T2 ➢ Occasionally gliosis ➢ Associated with cavernomas in 8%-33% of DVAs ➢ CE MR ❖ Dilated deep veins converge on a collector vein ❖ Follows a transhemispheric course to a normal vein Angiography ➢ Pathognomonic ➢ Normal arterial phase ➢ Radially oriented dilated medullary veins converge on an enlarged transcortical draining vein ❖ Caput medusa opacifies at the same time as normal veins ❖ Collector vein seen on late venous phase ➢ Location ❖ In the deep white matter near the margin of an adjacent ventricle ❖ Frontal>parietal>brachium pontis/dentate ❖ Frontal lobes may opacify earlier and show a capillary blush

Neuroradiology

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Intracranial Vascular Malformations

Developmental Venous Anomaly [Figure 5-17-19] DSA of Developmental Venous Anomaly [Figure 5-17-20] Cavernous Angioma • • • •

Prevalence: 0.5%-0.7% on MR/autopsy Account for 5%-13% of cerebral VMs Sporadic Familial form ➢ Autosomal dominant, Hispanic ➢ Multiple lesions ➢ Increased number of lesions with aging ➢ Correlation of symptoms with the presence of high signal intensity lesions on MR

Figure 5-17-19

Pathology •

Gross findings ➢ Small, reddish-purple (“mulberry”) lesions ➢ Few mms to several cms ➢ Multiple or single ➢ Often encapsulated and multilobar ➢ Occasionally calcified ➢ Often associated with DVA ➢ Found throughout the CNS

Pathology •

Histologic findings ➢ Thin-walled vascular sinusoids ➢ Endothelium lacks smooth muscle, elastin, and intervening parenchyma ➢ Lacks blood brain barrier ➢ Surrounded by hemosiderin deposits and gliosis ➢ May or may not be thrombosed

Clinical Presentation • •

NCCT and T1 and T2 weighted MRI shows dystrophic microcalcifications and/or hemosiderin staining in the right basal gangliar region

Usually present between age 30-50 years Symptoms ➢ Seizures ❖ Lesions usually in frontal or temporal lobe ➢ Focal neurologic deficits ➢ Acute hemorrhage ❖ Risk is 0.25%-6% per year ❖ Increased risk with previous hemorrhage, pregnancy ➢ Headache

Figure 5-17-20

Imaging Findings •

•

CT ➢ Variable density ❖ Hemorrhage ❖ Calcification - Rim, coarse, stippled, granular DSA ➢ Cavernous angioma not visualized ➢ Associated DVA

Intracranial Vascular Malformations

DSA shows “caput medusa” in early venous phase followed by filling of the collector vein

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Neuroradiology

CT of Cavernous Angioma [Figure 5-17-21]

Figure 5-17-21

Imaging Findings - MRI •

•

• •

Type 1 ➢ Hyperintense core on T1 ➢ Hyper or hypointense core on T2 ➢ Corresponds to subacute hemorrhage Type 2 “popcorn” ➢ Reticulated mixed signal on T1 ➢ Reticulated mixed signal on T2 with hypointense rim CT findings include a hypodense center with rim calcification ➢ Corresponds to lesions with multiple (left) and fine granular calcifications throughout the lesion hemorrhages of various age (right) Type 3 ➢ Iso or hypointense on T1 ➢ Hypointense lesion with hypointense rim on T2 ➢ Corresponds to chronic hemorrhage with hemosiderin staining Type 4 ➢ Not visible on T1 or T2 ➢ Punctate hypointense lesion on GRE ➢ Corresponds to tiny lesion or telangiectasia

Figure 5-17-22

MRI of Cavernous Malformations [Figure 5-17-22]

Cavernous Malformation with DVA [Figure 5-17-23]

Treatment •

• •

Stereotactic microsurgery ➢ Reasons for surgery ❖ Repeat hemorrhage ❖ Mass effect from enlarging lesion ❖ Seizure focus Radiosurgery ➢ Controversial ➢ Associated with hemorrhage and radiation-induced mass effect Follow ➢ Deep lesions ➢ Familial lesions

T1, T2 and GRE images show a Type 2 lesion in the right frontal lobe and a Type 4 lesion in the left occipital lobe

Figure 5-17-23 Capillary Telangiectasia • •

• •

Represents 16%-20% of all CVMs Incidental finding ➢ Nearly always asymptomatic ➢ Sx: headache, vertigo, ataxia, hearing loss Congenital vs. acquired lesions ➢ Obstructed venous drainage; radiation May be associated with VA, CM, AVM ➢ “Transitional malformations”

Gd MRA shows DVA associated with a cavernous malformation

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Intracranial Vascular Malformations

Pathology •

• •

•

Location ➢ Pons, cerebral/cerebellar hemispheres, spinal cord Histology ➢ Thin-walled, “capillary-type,” ectatic blood vessels ➢ Interspersed with normal brain Size ➢ Few millimeters to 2 cm Hemorrhage ➢ Rare ➢ Usually from an associated vascular malformation

Figure 5-17-24 Imaging [Figure 5-17-23] •

•

CT ➢ Usually normal MR ➢ T1: hypo- or isointense ➢ T2: iso- or slightly hyperintense ➢ GRE: hypointense ❖ Most consistent finding ❖ Due to intravascular deoxyhemoglobin ➢ CE-MR ❖ Faintly enhance in a “stippled” or “brushlike” pattern ❖ 2/3d with enlarged vessel Typical MR findings in a capillary telangiectasia

References 1. 2. 3. 4. 5. 6. 7. 8.

9. 10. 11. 12. 13.

Al-Shani R, Warlow C. A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain 2001; 124:1900-26. Augustyn GT, Scott JA, Olson E, et al. Cerebral venous angiomas: MR imaging. Radiology 1985; 156:391–395 Camacho DL, Smith JK, Grimme JD, et al. Atypical MR imaging perfusion in developmental venous anomalies. AJNR Am J Neuroradiol 2004; 25(9):549-52. Gault J, Sarin H, Awadallah N, et al. Pathobiology of human cerebrovascular malformations: basic mechanisms and clinical relevance. Neurosurgery 2004; 55(1):1-17. Lai CW, Agid R, van den Berg R, ter Brugge K. Cerebral arteriovenous fistulas induced by dural arteriovenous shunts. AJNR J Neuroradiol 2005; 26:1259-62. Lawton MT, UCSF Brain Arteriovenous Malformation Study Project. Spetzler-Martin Grade II arteriovenous malformations: surgical results and a modification of the grading scale. Neurosurgery 2003; 52(4):740-8. Mast H, Young WL, Koennecke HC, et al. Risk of spontaneous haemorrhage after diagnosis of cerebral arteriovenous malformation. Lancet 1997; 350:1065-8. Ogilvy C, Steig P, Awad I, et al. Recommendations for the management of intracranial arteriovenous malformations. A statement for health care professionals from a special writing group of the Stroke Council, American Stroke Association. Circulation 2001; 103:2644-57. Peebles TR, Vieco PT. Intracranial developmental venous anomalies: diagnosis using CT angiography. J Comput Assist Tomogr 1997; 21(4): 582-6. Sakata N, Takebayashi S, Kojima M, et al. Different roles of arteriosclerosis in the rupture of intracranial dissecting aneurysms. Histopathology 2001; 38(4):325-37. Stapf C, Mohn JP, Choi JH et al. Invasive treatment of unruptured brain arteriovenous malformations is experimental therapy. Current Opinion in Neurology 2006; 19(1):63-8. The Arteriovenous Malformation Study Group. Arteriovenous malformations of the brain in adults. NEJM 1999; 340(23):1812-18. The Scottish Intracranial Vascular Malformation Group. Prospective, population-based detection of intracranial vascular malformations in adults. Stroke 2003; 34:1163-69.

Intracranial Vascular Malformations

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Neuroradiology

Imaging of Intracranial Infections Patricia A. Hudgins, MD Summary •

•

Meningeal Disease ➢ Epidural abscess ➢ Subdural empyema ➢ Meningitis Parenchymal Infection ➢ Cerebritis to abscess ➢ Encephalitis – HSV, CJD, ADEM ➢ Parasitic – Cysticercosis, Lyme disease ➢ Tuberculosis ➢ Mycotic ➢ AIDS related infections

Intracranial Infection •

•

Location ➢ Intra-axial – Parenchymal ➢ Extra-axial – Epidural, subdural, leptomeningeal Response to infection ➢ Edema and swelling ➢ Mass and mass effect ➢ Abnormal enhancement ➢ Chronic – Atrophy

Figure 5-18-1

Blood-brain barrier • • • •

Tight junctions (no gaps) exist between normal endothelial cells Little communication between capillary & extracellular space or neurons Infection results in loss of tight junctions, with increased permeability of endothelial membranes = LOSS OF BBB Loss of BBB + increased local blood volume = ABNORMAL ENHANCEMENT

Intracranial infection – Location Meninges •

•

Dura mater – ➢ 2 layers ➢ Outer layer is calvarial periosteum ➢ Inner layer is separation between dura mater and arachnoid Collection between inner table of skull & dura is EPIDURAL

Subdural empyema (courtesy of Amirsys, Inc.)

Figure 5-18-2

Intracranial infection – Location Meninges [Figure 5-18-1] •

• •

Arachnoid Mater ➢ Thin connective tissue ➢ Parallels dura mater Collection between dura & arachnoid is SUBDURAL Subdural space is potential space w/ bridging veins

Intracranial infection – Location Meninges[Figure 5-18-2] •

• •

Pia mater – ➢ Blood vessels ➢ Covers surface of brain Space between arachnoid & pia is SUBARACHNOID space LEPTOMENINGES Arachnoid + Pia Leptomeningeal pattern of infection (courtesy of Amirsys, Inc)

Neuroradiology

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Imaging of Intracranial Infections

Extra-axial Infection Epidural [Figures 5-18-3 and 5-18-4] • • • • • • •

Figure 5-18-3

Usually starts in paranasal sinuses or mastoids Low density on CT Enhancing periphery ? SI on T1 ? SI on T2, FLAIR Enhancing dura Check for osteomyelitis of calvarium

Acquired CNS infections • • • • • •

Meningitis and complications Cerebritis and abscess Encephalitis Parasitic, rickettsial, spirochetal infections TB, other granulomatous infections Immunocompromised host, especially AIDS

Epidural abscess with typical enhancing dural surface

Acute Bacterial Meningitis •

• •

Organisms ➢ Neonates: group B streptococcus ➢ Children: H. influenza ➢ Adults: Streptococcus pneumoniae Pathogenesis ➢ Hematogenous seeding, choroid, leptomeninges ➢ Contiguous spread from sinusitis, mastoiditis ➢ Neonatal meningitis - maternal GU infxn, PROM Clinical - H/A, neck stiffness, photophobia, cranial nerve dysfunction, lethargy

Meningitis – role of imaging

Figure 5-18-4

Epidural collection crosses midline. Subdural abscess does not cross midline, and can extend along interhemispheric fissure

Complications • Hydrocephalus (especially communicating) • Subdural effusions, empyema • Venous sinus thrombosis/infarction • Arterial infarction • Vasculitis • Cerebritis or abscess • Ventriculitis/ependymitis

Figure 5-18-5

ACUTE MENINGITIS: Patterns [Figure 5-18-5] •

•

Dura –Arachnoid ➢ Pachymeningitis Pia-Subarachnoid Space ➢ Leptomeningitis

Two patterns of meningitis (courtesy of Amirsys, Inc).

Meningitis: CT Findings • • • • •

Figure 5-18-6

Early on, CT usually normal Sulcal or cisternal effacement Pia/subarachnoid enhancement Hydrocephalus ? Source – Paranasal sinus, mastoid

Acute Meningitis: MR Findings [Figure 5-18-6] • • • • •

T1 and T2 images may be normal FLAIR – ? SI in SAS Gd - Leptomeningeal enhancement ? Hydrocephalus ? Infection source Meningeal infection best detected on FLAIR image, with increased SI in subarachnoid

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Acute Meningitis: MR Findings [Figure 5-18-7] • • • • •

Figure 5-18-7

T1 and T2 images may be normal FLAIR – ? SI in SAS Gd - Leptomeningeal enhancement ? Hydrocephalus ? Infection source

Meningitis, complications • •

Hydrocephalus Ependymitis ➢ Subependymal enhancement ➢ In HIV disease, diff dx is lymphoma

Meningitis, complications [Figure 5-18-8] • •

• • • • •

Subdural effusions Often sterile ➢ If infected, restricted diffusion on DWI H. flu More common in children Bilateral, resolve spontaneously CT/MR similar to CSF Membranes may enhance

Meningitis with subarachnoid pattern of enhancement on T1 gad. Image

Figure 5-18-8

Meningitis, complications • • • •

Infarction May be arterial or venous Well-demarcated – best way to differentiate from cerebritis Typical CT/MR features with restricted diffusion on DWI

Diffusion weighted imaging (DWI) • • •

Powerful tool, physiologic information Differentiates unrestricted from restricted free water diffusion “Restricted” diffusion ➢ Acute and subacute infarction ➢ Intracranial abscess ➢ Rare MS plaque ➢ Epidermoid (vs. arachnoid cyst)

Meningitis, complications [Figure 5-18-9]

Figure 5-18-9

Summary •

•

Meningeal Disease ➢ Epidural empyema ➢ Subdural empyema ➢ Meningitis Parenchymal Infection ➢ Cerebritis to abscess ➢ Encephalitis – HSV, CJD, ADEM ➢ Parasitic – Cysticercosis, Lyme disease ➢ Tuberculosis ➢ Mycotic ➢ AIDS related infections

Thalamic and basal ganglia infarctions due to acute meningitis

Cerebritis to brain abscess • • •

Bilateral sterile effusions after H. flu meningitis

Early and late cerebritis Early and late capsule/abscess Etiology ➢ Direct spread (sinus, mastoid, odontogenic) ➢ Hematogenous ➢ Surgery or trauma ➢ 25% - no source found

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Imaging of Intracranial Infections

Cerebritis to brain abscess •

•

Figure 5-18-10

Early cerebritis ➢ 3-5 days after infection ➢ Unencapsulated – white cells, edema, necrosis, petechial hemorrhage Late cerebritis ➢ 4-14 days after infection ➢ Poorly delineated rim, necrotic core, white cells/inflammatory cells, granulation tissue

Cerebritis to brain abscess •

•

Early capsule ➢ 2-4 weeks ➢ Collagenous capsule +/- daughter abscesses ➢ Necrotic core ➢ Mild mass effect Late capsule ➢ Weeks/months ➢ Thick capsule ➢ Edema, mass effect resolve

Abscess is typically associated with vasogenic edema and rim enhancement

Figure 5-18-11

Abscess - pyogenic [Figure 5-18-10] • • • •

Supratentorial is most common Usually solitary (may have small surrounding cysts) Streptococcus, Staphylococcus Infants – Citrobacter, Proteus, Pseudomonas, Serratia

Abscess [Figures 5-18-11 and 5-18-12] • • •

• •

Mass effect, edema, enhancement T2 WI – ? SI rim DWI/ADC ➢ Restricted diffusion MR spectroscopy ➢ Lactate (1.3 ppm) Complications ➢ Herniation ➢ Intraventricular rupture ➢ Choroid plexitis ➢ Leptomeningitis

Abscess with restricted diffusion, confirmed on ADC map

Figure 5-18-12

Encephalitis “Inflammation of the brain” • •

•

Diffuse infection Post-infectious/immunization ➢ Acute disseminated encephalomyelitis Viral ➢ Herpes simplex virus ➢ Others: measles, mumps, etc.

Herpes encephalitis • • • • • •

Adults - HSV-1 (oral) Frontal sinusitis resulting in frontal abscess, with Neonates – HSV-2 (genital) low signal rim on T2, and enhancement Confusion, progressing to coma 50%-70% mortality, especially when delay to diagnosis (most common cause of fatal encephalitis) Necrotizing hemorrhagic encephalitis, often resulting in diffuse atrophy Axonal spread from reactivation in trigeminal ganglion

Imaging of Intracranial Infections

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Herpes encephalitis [Figure 5-18-13] •

Figure 5-18-13

Predilection for limbic system ➢ Temporal lobes ➢ Insula ➢ Cingulate gyrus ➢ Subfrontal region

Herpes encephalitis • • •

Imaging may be normal early in disease course MR > CT, especially early MR ➢ Inc. SI on T2 & FLAIR ➢ Mass effect ➢ +/- enhancement ➢ 60% bilateral ➢ May have small punctate hemorrhage

Herpes encephalitis of left temporal lobe

Figure 5-18-14

Herpes encephalitis [Figure 5-18-14] • • •

Subtle, gyral enhancement Unilateral early, then bilateral Temporal lobes, insular cortex, inferior frontal lobes, cingulate gyri

Creutzfeldt-Jakob Disease • • • • •

Transmissible spongiform encephalopathy (TSE) Prion: proteinaceous infectious particle Sporadic – most common (85%-90%) Genetic (10%-15%) Infectious (rare)

Creutzfeldt-Jakob Disease CLASSIC CJD • Older (mean 68 yrs) • Sporadic • Shorter duration • Dementia • Rare “pulvinar sign” •

VARIANT CJD Younger (mean 28 yrs) BSE contaminated food Longer duration Behavior changes “Pulvinar sign” on MR > 75% Variable amounts of PrPres Lots of PrPres

Herpes encephalitis, with high SI in temporal lobes and patchy enhancement

Creutzfeldt-Jakob Disease: Imaging • • • • • •

Symmetric high signal on T2 & FLAIR Caudate head, basal ganglia, thalamus (“pulvinar sign”) Diff. restriction > 2 weeks (c/w infarct) Cortical, limbic system involvement 1/3 Occipital lesions: 20% Rapidly progressive atrophy

CNS Tuberculosis • • • • • •

Increasing incidence – homeless, prisoners (2%-5% of pts. w/ TB) AIDS population Hematogenous dissemination from lungs Most have pulmonary TB Drug resistance increasing & ominous Either meningitic or tuberculoma, rarely both forms found together

CNS Tuberculosis Leptomeningeal • • • •

Cisterns fill with gelatinous exudate Hydrocephalus, infarctions, CN palsies Leptomeningeal enhancement, hydrocephalus Disease at COW – arteritis & infarctions, esp. in children

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Imaging of Intracranial Infections

CNS Tuberculosis Cerebritis [Figure 5-18-15]

Figure 5-18-15

CNS TuberculosisTuberculoma • • •

Most common parenchymal form(may be extra-axial) Supratentorial > infra Dense fibrous capsule with caseous necrotic core

CNS Tuberculosis Tuberculoma • • • •

[Figure 5-18-16]

TB cerebritis with typical pattern of right temporal edema and enhancement

Small or coalesced larger nodules Edema, mass effect Wall may have increased SI on T1, decreased SI on T2 Old/treated lesions may Ca+

Figure 5-18-16

Neurocysticercosis • • • • • • •

Taenia solium (Pork tapeworm) Worldwide, most common CNS infection Worldwide, most common cause of epilepsy Central/South America; East/SE Asia; India; Africa CNS disease: 60%-90% Seizures, intracranial hypertension, focal deficit Four variants ➢ 1. Parenchymal ➢ 2. Intraventricular ➢ 3. Cisternal ➢ 4. Spinal

Multiple tuberculomas, with low signal intensity rim on T2 and enhancement

Parenchymal Cysticercosis •

• •

•

Vesicular stage ➢ Eccentric nodule (scolex), no edema or enhancement Colloidal stage ➢ Dying scolex, capsule thickens, extensive edema & enhancement Granular nodular stage ➢ Cyst ? in size, small enhancing nodules, no edema Nodular calcified stage ➢ Cyst involutes, calcifies, no edema or enhancement

Figure 5-18-17

Parenchymal Cysticercosis Vesicular stage • • • •

Larva is fully grown,with thin intact capsule surrounding distended bladder Fluid in bladder is clear, no surrounding inflammatory reaction. Well-defined cyst, scolex enhances (mural nodule) Wall does not enhance, no edema

Parenchymal Cysticercosis Colloidal stage [Figure 5-18-17] • • • • • •

Colloidal stage of CNS cysticercosis, with thick capsule, edema, and mass effect

Larva degenerates, cyst fluid turbid Cyst wall thickens Vasogenic edema Cyst fluid ? SI on T1WI Cyst wall ? SI on T2WI Vasogenic edema & enhancement

Imaging of Intracranial Infections

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Neuroradiology

Parenchymal Cysticercosis Granular Nodule Stage • • • •

Figure 5-18-18

Cyst retracts, capsule thickens, and scolex calcifies +/- perifocal edema CT: Isodense cyst, calcified scolex MR: T1WI – isointense to brain T2WI – hypointense to brain

Intraventricular Cysticercosis [Figure 5-18-18] • • • • •

10-20% of neurocysticercosis Fourth ventricle - most common site +/- hydrocephalus Cyst can parallel CSF density and SI Does not calcify

Cisternal Cysticercosis • • • •

Basilar cisterns or sylvian fissures MRI more sensitive than CT Can incite leptomeningeal inflammatory response Hydrocephalus, infarctions

Intraventricular and subarachnoid cysticercosis, with hydrocephalus

CNS Lyme Disease • • • •

Borrelia burgdorferi Common tick borne disease in NE US Presents as meningitis, neuritis (incl. CN), vasculitis Multifocal wm lesions, +/- enh

Figure 5-18-19

Fungal Disease in CNS • • • • •

Cryptococcosis Coccidioidomycosis Mucormycosis Aspergillosis Others

AIDS Related Conditions • • • •

HIV Encephalitis Toxoplasmosis (vs lymphoma) Cryptococcal meningitis Progressive multifocal leukoencephalopathy (PML)

Human Retroviruses • • • • •

HIV (HIV-1 and HIV-2) and HTLV-1 Patchy symmetric abnormal signal HIV-1 found in CNS in AIDS (neurotrophic) intensity in patient with HIV Encephalopathy, myelopathy, peripheral neuropathy, myopathy encephalitis HIV replicates in multinucleated giant cells & macrophages in CNS Oligodendrocytes, astrocytes, neurons less frequently directly infected

Subacute HIV encephalitis AIDS Dementia Complex (ADC) • • • • •

[Figure 5-18-19]

Most common CNS complication (30%) Dementia, behavior changes, headache Virus in MNGC’s CT – normal MR – atrophy ➢ PVWM lesions ➢ Grey matter (late)

Neuroradiology

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Imaging of Intracranial Infections

HIV Leukoencephalopathy • •

Figure 5-18-20

Disease on MR parallels clinical progression NAA/Cho & NAA/Cr ratios reduced due to neuronal loss

PML [Figure 5-18-20] • • • • • •

Opportunistic infection Papovavirus (JC virus) Patchy non-enhancing white matter lesions No mass effect Hypointense on T1 Asymmetric

PML has low SI on T1 weighted images, with no enhancement

HIV Encephalitis vs PML HIV Diffuse Central T1 images nl

PML Asymmetric Peripheral Hypointense T1

Figure 5-18-21

Toxoplasmosis [Figure 5-18-21] • • • • • • • •

Protozoan Toxoplasma gondii Soil organism Endemic in US Reactivates in CNS in immunocompromised Most common opportunistic infection CD4 < 100 cell/mm3 Fever, HA, neurologic deficits, seizures Basal ganglia, cerebral hemispheres

Single large toxoplasmosis abscess

Toxoplasmosis • • • • • •

Figure 5-18-22

Necrotic debris, inflammatory cells, organisms Vasogenic edema Robust enhancement 15% are solitary Pyrimethamine + sulfa or clindamycin Life-long maintenance abs

Toxoplasmosis [Figure 5-18-22] • • •

With therapy, lesions may calcify Resolution of vasogenic edema New lesions or new edema develop if medication is stopped

Old, treated toxoplasmosis abscesses may calcify

Cryptococcus neoformans • • • • • •

Ubiquitous fungi within contaminated soil Inhaled, then hematogenous spread immuno- compromised hosts Most common fungus in AIDS (6%-7%) Subacute meningitis, HA, AMS, fever DX – India ink preparation, antigen in CSF, fungal culture of CSF Perivascular spaces distended with mucoid material and fungus

Figure 5-18-23

Cryptococcus meningitis • • •

Basal ganglia,often bilateral CT may be normal Non-enhancing low density lesions in Virchow-Robin spaces “gelatinous pseudocysts”

Cryptococcus meningitis [Figure 5-18-23] • •

On MR, pseudocysts are decreased SI on T1,increased SI on T2 No significant enhancement

Imaging of Intracranial Infections

Dilated peri-vascular spaces in cryptococcosis 1236 1238

Neuroradiology

Highly Active Antiretroviral TX (HAART) Immune reconstitution syndrome • • • • •

AIDS treatment regimen that includes protease inhibitor & reverse transcriptase inhibitor Suppresses viral replication Increase in CD4 counts, decrease in viral load Increased survival May result in “unexpected” imaging findings

HAART • •

Ability to mount immune response may change imaging findings, especially enhancement patterns Enhancement in crypto. meningitis

Summary •

•

Meningeal Disease ➢ Epidural empyema ➢ Subdural empyema ➢ Meningitis Parenchymal Infection ➢ Cerebritis to abscess ➢ Encephalitis – HSV, CJD, ADEM ➢ Parasitic – Cysticercosis, Lyme disease ➢ Tuberculosis ➢ Mycotic ➢ AIDS related infections

References 1. 2. 3. 4. 5.

6.

7. 8. 9. 10. 11. 12.

13. 14. 15. 16. 17.

Chang L, Ernst T. MR spectroscopy and diffusion-weighted MR imaging in focal brain lesions in AIDS. Neuroimaging Clin N Am. 1997 Aug;7:409-26. Collie DA, Summers DM, Sellar RJ, et al. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. AJNR Am J Neuroradiol. 2003l 24:1560-9. Enzman DR, Britt RH, Obana WG, et al. Experimental staphylococcus aureus brain abscess. AJNR 7:395-402, 1986 Fernandez RE, Rothbert M, Ferencz G, et al. Lyme disease of the CNS: MR imaging findings in 14 cases. AJNR 1990;11:479-481 Filippi CG, Sze G, Farber SJ, et al. Regression of HIV encephalopathy and basal ganglia signal intensity abnormality at MR imaging in patients with AIDS after the initiation of protease inhibitor therapy. Radiology 1998;206:491-498 Galassi W, Phuttharak W, Hesselink JR, et al. Intracranial meningeal disease: comparison of contrast-enhanced MR imaging with fluid-attenuated inversion recovery and fat-suppressed T1 weighted sequences. AJNR 2005; 26: 553-9 Gaviani P, Schwartz RB, Hedley-Whyte ET, et al. Diffusion-weighted imaging of fungal cerebral infection. AJNR Am J Neuroradiol. 2005 May;26(5):1115-21. Han XY, Weinberg JS, Prabhu SS, et al. Fusobacterial brain abscess: a review of five cases and an analysis of possible pathogenesis. J Neurosurg 2003; 99: 693-700 Kramer LD, Locke GE, Byrd SE, et al. Cerebral cysticercosis: documentation of natural history with CT. Radiology 1989;171:459-462 Lai PH, Li KT, Hsu SS, et al. Pyogenic brain abscess: findings from in vivo 1.5 T and 11.7 T in vitro proton MR spectroscopy. AJNR 2005; 26:279-88 Murata T, Shiga Y, Higano S, et al. Conspicuity and evolution of lesions in Creutzfeldt-Jakob disease at diffusionweighted imaging. AJNR Am J Neuroradiol. 2002; 23:1164-72 Nadal Desbarats L, Herlidou S, de Marco G, et al. Differential MRI diagnosis between brain abscess and necrotic or cystic brain tumors using the apparent diffusion coefficient and normalized diffusion-weighted images. Magn Reson Imaging 2003; 21: 645-650 Post MJD, Tate LG, Quencer RM, et al. CT, MR, and pathology in HIV encephalitis and meningitis. AJNR 1988; 9:469-476 Prusiner SB. Prions and neurodegenerative diseases. N Engl J Med. 1987; 317:1571-81. Sze G, Zimmerman RD. The magnetic resonance imaging of infections and inflammatory diseases. Radiol Clin North Am. 1988: 26:839-59. Tien RD, Felsberg GJ, Osumi AK. Herpesvirus infections of the CNS: MR findings. AJR Am J Roentgenol. 1993 Jul;161:167-76. Wehm SM, Heinz ER, Burger PC, et al. Dilated Virchow-Robin spaces in cryptococcal meningitis associated with AIDS: CT and MR findings. J Comput Assist Tomogr 1989;13:756-762

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The Paranasal Sinuses Patricia A. Hudgins, MD Summary • • •

•

Figure 5-19-1

Development Anatomy, especially for endoscopic sinus surgery (ESS) Infection ➢ Acute ➢ Chronic ➢ Complications Neoplasms ➢ Benign, including tumor-like lesions ➢ Malignant

Sinus – Anatomy & Development •

• • •

Nose and nasal cavities - Functions ➢ Respiration - humidifies and warms inspired air ➢ Defense – mucous blanket ➢ Olfaction – fibers pass through cribriform plate to CN I Nose – external nose, pyriform aperture Nasal septum – cartilage, ethmoid bone, septum Nasal cavities – internal nasal airways ➢ Inferior turbinate – nasolacrimal duct ➢ Medial turb. – max, frontal, ant ethmoid sinus ➢ Superior turb – post. ethmoid, sphenoid sinus

Normal ethmoid anatomy in coronal plane

Figure 5-19-2

Sinus – Anatomy & Development • • •

Lined by mucosa w/ serous and mucinous glands “Mucoperiosteum” – mucosa attached to bone Sinus functions – protect CNS (collapsible) is only definite function

Ethmoid sinus • • •

• •

Bilateral Groups of cells formed by septa and lamella Anterior ethmoids ➢ Multiple small cells ➢ Middle turbinate Posterior ethmoids ➢ Fewer but larger cells Basal lamella ➢ Lateral insertion of middle turbinate ➢ Separates ant from post ethmoids

Pneumatized agger nasi cell on left on coronal CT

Figure 5-19-3

Ethmoid sinus - anatomy [Figure 5-19-1] • • •

•

Lamina papyracea Roof of the ethmoids Drainage ➢ Infundibulum, lateral to uncinate process Ethmoid bulla

Ethmoid sinus - variants [Figures 5-19-2 and 5-19-3] •

• •

Agger nasi cell ➢ Most ant. ethmoid Supraorbital air cell ➢ Needs to be differentiated from frontal sinus Haller cell ➢ May obstruct max. sinus outflow

Paranasal Sinuses

Bilateral Haller cells, inferomedial ethmoid cells, between uncinate process and orbital wall 1238 1240

Neuroradiology

•

Onodi cell ➢ Close to optic nerve

Figure 5-19-4

Maxillary sinus [Figure 5-19-4] • • •

First to form in utero Rudimentary at birth, final form during 2nd decade Recesses ➢ Zygomatic ➢ Palatine ➢ Alveolar

Maxillary sinus •

•

Drainage ➢ Ostium is at superomedial portion ➢ Drains into infundibulum Uncinate process ➢ Medial wall of ostium ➢ Located at insertion of inferior turb on lateral nasal wall ➢ Contiguous with lacrimal bone anteriorly

Maxillary sinus cilia beat secretions up and medial, drain via maxillary sinus ostium into infundibulum

Figure 5-19-5

Maxillary sinus - variants [Figure 5-19-5] • • • •

Hypoplasia Sclerotic walls – chronic inflammation Atelectatic Lateralized medial wall

Maxillary sinus - variants [Figure 5-19-6] • • • • •

Hypoplasia Sclerotic walls – chronic inflammation Atelectatic “Silent sinus syndrome” Lateralized medial wall

CT finding of small sinus with thick sclerotic walls found in chronic sinus inflammation on right

Frontal sinus • • •

•

Absent at birth, finish pneumatizing in 2nd decade Essentially anterior ethmoid cell Drainage is variable ➢ Ethmoid infundibulum ➢ Frontal recess Usually asymmetric, may be septated

Figure 5-19-6

Ostiomeatal complex [Figure 5-19-7] • • •

Lateral nasal wall 3 projections – turbinates Divide nasal cavity into 3 separate passages - meatus

Figure 5-19-7

Right maxillary sinus is severely atelectatic, with depressed walls consistent with silent sinus syndrome

Ostiomeatal complex (courtesy of Amirsys, Inc.)

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Paranasal Sinuses

Normal OMU [Figure 5-19-8] • • •

Figure 5-19-8

3 turbinates/meati Frontal recess Infundibulum and middle meatus

Normal OMU – Frontal Recess • • •

Ant. coronal images Drains frontal sinus to ant. middle meatus Contents ➢ Frontal sinus ➢ Agger nasi cell ➢ Frontal cells ➢ Frontal recess ➢ Middle meatus

Normal OMU – Infundibulum [Figure 5-19-9] • • • •

Uncinate process Infundibulum – connects max sinus ostium to middle meatus Ethmoid bulla – largest ethmoid cell Hiatus semilunaris – space between uncinate process and ethmoid bulla

Normal OMU on coronal CT shows maxillary sinus, ostium, ethmoid infundibulum, uncinate process, middle turbinate and middle meatus.

Figure 5-19-9

Normal OMU - Checklist •

•

Anterior ➢ Frontal sinus, recess ➢ Agger nasi cell, frontal cells Posterior ➢ Uncinate process ➢ Maxillary sinus, ostium, infundibulum ➢ Ethmoid bulla (retrobullar recess) ➢ Middle turbinate/meatus ➢ Hiatus semilunaris

Uncinate process marks lateral aspect of infundibulum

Sphenoid sinus • • • • • •

Extremely variable pneumatization Dev. complete by 2nd decade Planum sphenoidale, posterior wall, anterior wall, inferolateral & pterygoid recesses Ant. wall is roof of nasopharynx Rarely non-pneumatized, consider anemia or ciliary dysmotility syndrome Sphenoethmoidal recess

Endoscopic sinus surgery • • •

Theory: mucosa is secondarily involved by inflammatory disease, usually due to sinus ostial obstruction Surgically relieve obstructing lesion (polyp, anatomic variant, etc) will allow sinus to drain normally and mucosal edema & inflammation will improve Recurrent or chronic sinusitis should improve

Figure 5-19-10

Ostiomeatal Complex Pattern [Figure 5-19-10] • • • • •

Middle meatus Maxillary sinus ostia Ethmoid infundibulum Anterior ethmoid cells Hiatus semilunaris

Ostiomeatal complex (Courtsey of Amirsys, Inc) Paranasal Sinuses

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Infection – Acute Sinusitis • •

• •

Figure 5-19-11

No indications for imaging common cold Secondary bacterial sinusitis ➢ Strep. pneum, H. flu, beta-hemolytic strep Facial pain, fever, discharge Clinical diagnosis

Acute Sinusitis – CT/MR • • •

•

Ethmoids often primary source Asymmetric Mucosal thickening ➢ Moderate or severe ➢ Non-specific ➢ Thickened enhancing mucosa with submucosal edema Air/fluid levels ➢ Frontal = sinusitis ➢ Maxillary = sinusitis in correct setting ➢ Ethmoid – rare ➢ Sphenoid - nonspecific Orbital sub-periosteal infection. Graphic and axial CECT show ethmoid opacification, and peripherally enhancing mass in medial extra-conal location. (courtesy of Amirsys, Inc.)

Acute sinusitis - complications •

• •

Local extension ➢ Orbital – sub-periosteal abscess ➢ Intra-cranial – epidural empyema Venous occlusion – cavernous sinus, transverse sinus (mastoid), superior sagittal sinus Chronic sinusitis ➢ Recurrent acute ➢ Chronic – patient has no periods without disease

Figure 5-19-12

Sinusitis – subperiosteal infection • • • •

[Figure 5-19-11]

Most common local complication Pre-septal swelling clinical dx Post-septal infection best detected with imaging CECT is test of choice, to exclude post-septal, subperiosteal abscess

Sinusitis – Complications - Local extension • •

Axial CECT shows small interhemispheric subdural abscess. Note posterior frontal wall is intact

[Figure 5-19-12]

•

Noncontrast sinus CT not enough Enhanced CT and MR are indicated for complicated sinusitis Intracranial infection can occur without bone defect

Figure 5-19-13

Frontal Sinusitis – Intracranial abscess [Figure 5-19-13] Sinusitis Mimics “Incidental” lesions found on screening sinus T2, sagittal and axial post-Gd T1 MR images show right frontal abscess, with surrounding edema. Note thin dural CT • • • • • •

Subarachnoid hem. GBM Colloid cyst Hydrocephalus Subdural hematoma Esthesioneuroblastoma

Neuroradiology

enhancement, opacified frontal sinuses and soft tissue inflammation

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Paranasal Sinuses

• • •

Lymphoma of maxillary antrum SCCa maxillary sinus Adenoid cystic ca

Figure 5-19-14

Sinusitis - Fungal • • • •

Two distinct forms Acute, fulminant invasive fungal sinusitis Allergic fungal sinusitis with sino-nasal polyps Imaging appearance variable for both

Allergic fungal sinusitis with polyps (AFS-SNP) • • •

[Figure 5-19-14]

•

Immune-competent Chronic nasal obstruction, “recurrent sinusitis” Cycle of sinusitis, mucosal edema, polyp formation, ostial stenosis, sinusitis… Polyps

Allergic fungal sinusitis with polyps (AFS-SNP) •

Coronal non-contrast CT shows massive expansion of ethmoid and frontal sinuses, with contents both low and high density

[Figure 5-19-15]

CT ➢ ➢ ➢ ➢ ➢

Pansinus/nasal cavity opacification Expanded airless sinuses Thin deossified sinus walls Sinus contents variable in density Mixed low and high density on noncontrast CT

Figure 5-19-15

Allergic fungal sinusitis with polyps (AFS-SNP) •

[Figure 5-19-16]

MRI ➢ Extremely complex SI ➢ May be increased or decreased on T1 ➢ Variable on T2, regions of frank signal void ➢ Marked expansion may encroach on surrounding structures, including orbit & skull base

Fungal sinusitis - invasive [Figure 5-19-17] • • •

Immune deficient Early – non-spec. presentation CT – early ➢ Mucosal disease ➢ Nasal cavity soft tissue due to mucosal or turbinate necrosis

Axial non-contrast CT shows marked thinning of posterior frontal sinus walls, without destruction

Figure 5-19-16

Figure 5-19-17

Complex, mixed signal intensity within expanded airless sinuses on T2 WI’s. (courtesy of Amirsys, Inc.)

Early invasive fungal sinusitis may have only a benign appearing nasal cavity mass Paranasal Sinuses

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Fungal sinusitis - invasive [Figure 5-19-18] •

Figure 5-19-18

CT/MR late ➢ Local invasion ➢ Dirty retro-antral space ➢ Intracranial/orbitalspread ➢ Bone destruction

Fungal sinusitis - invasive [Figure 5-19-19] • • •

Variable SI May have dramatic decrease of SI on T1 & T2 Heterogeneous enhancement pattern

Figure 5-19-19 CECT shows complete left nasal cavity obstruction, moderate left maxillary sinus mucosal thickening, and severe facial swelling

Complex sinus contents on T2 WI’s in invasive fungal sinusitis

Fungal sinusitis •

•

Figure 5-19-20

Acute invasive ➢ Immunesuppressed ➢ Acute ➢ Pain, fever, local invasion ➢ May be fulminant, rapidly progress ➢ Often treated surgically local resection, orbital exenteration ➢ High mortality Allergic fungal ➢ Clinically well ➢ Aspirin intolerance ➢ Chronic ➢ Presents with nasal obstruction ➢ Txed with endoscopic polyp resection ➢ High rate of recurrence

Benign Sinus Lesions • • • • • •

Antro-choanal polyp Mucocele Fibrous dysplasia Osteoma Juvenile nasopharyngeal angiofibroma (JNA) Inverted papilloma

Coronal graphic of left antrochoanal polyp. (courtesy of Amirsys, Inc.)

Antrochoanal polyp [Figure 5-19-20] • • • •

Benign maxillary polyp Extends from maxillary antrum through ostium into nasal cavity When large may extend to nasopharynx Entire lesion must be removed to avoid recurrence

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Paranasal Sinuses

Mucocele [Figure 5-19-21] • • • • •

Figure 5-19-21

Airless, expanded sinus Sinus walls thinned, may appear dehiscent Frontal, ethmoid, maxillary, sphenoid in order of frequency CT/MR signal intensity variable, depending on age of secretions Check sinus ostium for obstructing lesion

Fibrous Dysplasia [Figure 5-19-22] • • • • •

Medullary bone replaced by fibroosseous tissue T1 sagittal (top) and T2 axial (bottom) images Presents < 30 yrs show expanded, airless right frontal sinus Facial asymmetry, esp. cheek mucocele Most common – maxilla & mandible Obstruction of sinus ostium results in mucocele, especially Figure 5-19-22 ethmoid

Fibroosseous Lesions - Fibrous Dysplasia • • • •

Fibroosseous Therefore, often heterogeneous CT – ground glass MR – complex appearance ➢ Mixed increased & decreased SI ➢ Enhances robustly – often leads to misinterpretation as tumor

Benign Sinus Tumors - Fibroosseous - Osteoma • • • • • •

[Figure 5-19-23]

Benign proliferation of mature bone Frontal & ethmoid sinus – most common location Usually small & incidental May obstruct sinus ostium with sinusitis or mucocele formation CT – may be very dense, or more fibrous MR – decreased SI if primarily osseous

Figure 5-19-23

T1 sagittal (top) and T2 axial (bottom) images show expanded, airless right frontal sinus mucocele

Left ethmoid infundibulum osteoma

Juvenile Nasopharyngeal Angiofibroma (JNA) • • •

Benign but aggressive vascular mass Exclusively in males, adolescents Presenting sxs depend on location ➢ Nasal obstruction ➢ Minor or major epistaxis ➢ Facial asymmetry/deformity ➢ Proptosis ➢ Serous otitis media ➢ Headache

Paranasal Sinuses

1244 1246

Neuroradiology

JNA • • •

Figure 5-19-24

Arises in posterior nasal cavity (choana) Involves nasal cavity, nasopharynx, masticator space, sphenoid sinus, cavernous sinus Blood supply ➢ ECA - int. max & asc. pharyngeal arteries ➢ ICA supply usually implies intracranial extension ➢ Both ipsi and contralateral ECA/ICA supply

JNA - Imaging [Figure 5-19-24] • • • •

Nasal cavity & nasopharyngeal mass Expansion of pterygopalatine fossa Anterior bowing of posterior max. sinus wall CECT & MR with gado – robust enhancement Left JNA expands PPF, displaces posterior maxillary sinus wall

JNA - Imaging [Figure 5-19-25] • • • •

Heterogeneous on T1 & T2 sequences with flow voids Axial & cor best Check for sphenoid sinus, cavernous sinus invasion T2 images best map entire lesion

Benign sinus lesions - Inverted Papilloma • • • • • •

Epithelial tumor of mucosa Endophytic growth pattern Benign appearing mass in nasal cavity/middle meatus Associated with SCCa 10%-20% of time Bone remodeling without destruction On MR ➢ Enhancement ➢ Convoluted cerebriform pattern

Figure 5-19-25

Malignant Sinus Lesions • • •

• •

“Sinusitis” mimic Adults, M > F Usually advanced when detected ➢ Early small lesions clinically attributed to inflammatory sinus disease Maxillary, ethmoid most common SCCa – 80%-90% JNA shows robust enhancement and intracranial extension

Malignant Sinus Lesions • • • • • •

Squamous cell ca (most common) Glandular origin Olfactory neuroblastoma Sinonasal undifferentiated ca Melanoma Lymphoma

Figure 5-19-26 COMMON IMAGING FINDINGS • • •

Bone destruction Local extension/invasion Intracranial extension

Most important imaging goal extension of disease [Figure 5-19-26]

Graphics show patterns of spread of sinus malignancy Neuroradiology

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Paranasal Sinuses

SCCa Staging – critical findings •

Figure 5-19-27

Primary site ➢ Size ➢ Bone - maxillary or orbit walls, skull base ➢ Local - cheek, nasal cavity, nasopharynx, orbit

SCCa Staging (maxillary) • • • •

T1 – Antral mucosa w/o bone involvement or erosion T2 - Hard palate or sinus walls T3 – Cheek, orbital walls, pterygoid plates, ethmoids T4 - Skull base (cribriform plateor sphenoid sinus), frontal sinus, nasopharynx, orbital apex

Squamous cell carcinoma [Figure 5-19-27] • • • •

Low SI left nasal cavity SCCa, with obstructed secretions

Most arise in max sinus or nasal cavity M > F, adults > 50 Usually advanced at detection Obstructed sinus secretions may make imaging appearance complex

Figure 5-19-28

Malignant sinus - SCCa [Figure 5-19-28] • •

CT – bone thinning or destruction MR – T2 best differentiates tumor from benign secretions ➢ Tumor - decreased SI ➢ Secretions - increased SI

Malignancies of Glandular Origin • • •

10% of all sinus malignancies Typical local extension & bony erosion May be higher SI on T2 ➢ Adenocarcinoma ➢ Adenoid cystic ca ❖ Propensity for perineural spread ➢ Mucoepidermoid ca

Subtle erosion of planum sphenoidale (top) from nasal cavity & sphenoid sinus SCCA (bottom)

Figure 5-19-29

Adenoid cystic ca [Figure 5-19-29] • •

Sinus mass with osseous erosion Perineural spread ➢ Widened foramen or canal ➢ Enlarged enhancing nerve ➢ Obliteration of fat at skull base foramen T1 MR shows maxillary mass with hard palate low SI on left Perineural spread along vidian nerve

Esthesioneuroblastoma AKA Olfactory Neuroblastoma • • • • • •

Tumor of neural crest origin Accounts for 2% of sinonasal malignancies Arises in nasal cavity near cribriform plate Age range 3 to elderly Nasal cavity mass, erosion of cribriform plate, often with intracranial extension Peripheral intracranial tumoral cysts

Figure 5-19-30

Esthesioneuroblastoma [Figure 5-19-30]

Intracranial extension from esthesioneuroblastoma Paranasal Sinuses

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Neuroradiology

Summary • • •

•

Development Anatomy, especially for endoscopic sinus surgery (ESS) Infection ➢ Acute ➢ Chronic ➢ Complications Neoplasms ➢ Benign, including tumor-like lesions ➢ Malignant

Neuroradiology

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Paranasal Sinuses

The Sella and Parasellar Region Patricia A. Hudgins, MD Recommended Imaging Techniques [Figures 5-20-1 and 5-20-2] •

•

MR Imaging ➢ Multiplanar: ❖ Sagittal & Coronal ➢ Small FOV 16-18 ➢ 3mm ➢ T1WI, T2WI ➢ Post T1WI +/- FS ➢ Dynamic enhanced Dynamic Imaging ➢ Microadenomas ➢ 3 – 4 slices ➢ T1 FSE, Turbo SE ➢ Image continuously after contrast (10s) ➢ Increases sensitivity

Normal sella, sphenoid sinus, pituitary gland, infundibulum, and suprasellar region on graphic and post-gado. T1-WI (courtesy of Amirsys, Inc.)

Pituitary: Normal Anatomy •

•

Figure 5-20-1

Figure 5-20-2

Anterior Lobe ➢ Lateral ❖ PRL (10%-30%) ❖ GH (50%) ➢ Midline ❖ ACTH (10%-30%) ❖ TSH (5%) ❖ FSH/LH (10%) ➢ Location of adenomas parallels the distribution Posterior Lobe ➢ Infundibulum ➢ Pituicytes (glial) ➢ Axons ➢ Vasopressin (ADH) ➢ Oxytocin

Normal adult pituitary gland on sag T1-WI and cor T2-WI

Figure 5-20-3

Pituitary: Normal Anatomy •

Posterior Lobe ➢ Posterior Pituitary Bright Spot (PPBS) ➢ High SI on T1 ➢ Doesn’t suppress on fat sat

Parasellar Region: Normal Anatomy [Figure 5-20-3] •

Parasellar Structures ➢ Cavernous Sinus ➢ Cranial Nerves ❖ III, IV, V1, V2, VI ➢ Cavernous ICA ➢ Optic Chiasm ➢ Hypothalamus ➢ Sphenoid Sinus

Sella and Parasellar Region

Normal coronal graphic through the sella, including the pituitary gland, suprasellar cistern, infundibulum, and cavernous sinuses (courtesy of Amirsys, Inc.)

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Neuroradiology

Parasellar Region: Normal Anatomy •

Figure 5-20-4

Parasellar Structures ➢ Optic Chiasm ➢ Hypothalamus ❖ Tuber cinereum ❖ Mamillary bodies ➢ Sphenoid Sinus

Parasellar Region: Normal Anatomy •

[Figure 5-20-4]

Bony Structures ➢ Planum sphenoidale ➢ Tuberculum sellae ➢ Sella turcica ➢ Dorsum sellae

Parasellar Region: Normal Anatomy Sella and Parasellar Pathology •

Differential Diagnoses ➢ Intrasellar ➢ Suprasellar ➢ Infundibular

Skull base bony structures around the sella, including the planum sphenoidale, tuberculum sellae, sella turcica, and dorsum sellae (courtesy of Amirsys, Inc.)

Intrasellar Pathology •

•

•

Nonneoplastic Lesions ➢ Hyperplasia (physiologic, end organ failure) ➢ Cysts (RCC, pars intermedia cyst) ➢ Lymphocytic hypophysitis Primary Neoplasms ➢ Pituitary adenoma (Most common) ➢ Craniopharyngioma (Only 5% purely intrasellar) ➢ Meningioma (Purely intrasellar rare) ➢ Pituitary carcinoma (Extremely rare) Metastasis (1%)

Pituitary Neoplasms •

Adenoma ➢ Prolactinoma 30% ➢ Null cell 25% ➢ GH 20% ➢ ACTH 10% ➢ FSH/LH 10% ➢ PRL-GH 5% ➢ Mixed, TSH 1%-5% ➢ Incidental pituitary lesions are common (17%)

Figure 5-20-5

Graphic and post-gado T1-WI of small right microadenoma (courtesy of Amirsys, Inc.)

Sella: Pathology [Figure 5-20-5] •

Pituitary Microadenoma ➢ 10 mm or less ➢ 10%-20% of autopsies ➢ Micro >>> Macro ➢ Convex margin ➢ Stalk deviation ➢ Sella floor thin

Neuroradiology

1249 1251

Sella and Parasellar Region

Pituitary Microadenoma

Figure 5-20-6

Sella: Pathology • •

Pituitary Microadenoma Dynamic Imaging ➢ Increases sensitivity (10% seen only on dynamic MR) ➢ Enhances slower than normal gland

Physiologic Hypertrophy •

•

Maximum normal height Pituitary hypertrophy in a patient with ➢ 6 mm infants and children hypothyroidism ➢ 8 mm males, postmenopausal females ➢ 10 mm young women (convex superiorly) ➢ 12 mm late pregnancy, postpartum females (convex superiorly) Abnormal hypertrophy ➢ End-organ failure (esp. hypothyroid) Figure 5-20-7 ➢ Neuroendocrine tumor (rare)

Pituitary Gland Hypertrophy [Figure 5-20-6] Sella: Rathke Cleft Cyst •

Clinical ➢ Intrasellar 40% ➢ Suprasellar extent 60% ➢ 3mm – 3cm ➢ Most incidental ➢ Symptomatic ➢ Pituitary dysfunction ➢ Visual change, HA

Intrasellar Rathke cleft cyst, hyperintense on T1 non-contrast MR, with small intra-cyst nodule seen on T2-WI

Rathke Cleft Cyst: CT • • • •

75% hypodense 25% iso/hyperdense Ca++ rare May be difficult to differentiate from other benign cysts or craniopharyngiomas

Rathke Cleft Cyst: MR Imaging Features • Signal varies - cyst content ➢ 50%-60% T1 hyperintense ➢ 30%-40% follow CSF • 75% intracystic nodule • +/- rim enhancement

Rathke Cleft Cyst [Figure 5-20-7] Lymphocytic hypophysitis • • • • • • •

During pregnancy or shortly after delivery F >>> M Pituitary insufficiency H/A & visual changes Amenorrhea or inability to lactate Diffuse enlargement of adenohypophysis May mimic hyperplasia or adenoma

Sella and Parasellar Region

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Neuroradiology

Lymphocytic hypophysitis - Pathology • • •

Diffuse infiltration of the adenohypophysis by lymphocytes & plasma cells ? Autoimmune Infundibuloneurohypophysitis ➢ Affects infundibulum & neurohypophysis ➢ Thickened pituitary stalk ➢ Diabetes insipidus

Lymphocytic hypophysitis Other Intrasellar Masses Uncommon • • • •

Craniopharyngioma (5% intrasellar) Metastasis (1% of sellar masses) Aneurysm (medially-projecting from cavernous ICA) Meningioma (rare purely intrasellar)

Suprasellar Masses: Five Most Common •

75% of all sellar/parasellar masses ➢ Pituitary macroadenoma (35%-50%) ➢ Approximately 10% each ❖ Meningioma ❖ Aneurysm ❖ Craniopharyngioma ❖ Astrocytoma (hypothalamic-chiasmatic)

Figure 5-20-8

Suprasellar Differential Diagnosis •

•

Adult Lesions ➢ Pituitary Macroadenoma ➢ Meningioma ➢ Aneurysm Pediatric Lesions ➢ Craniopharyngioma ➢ Chiasmatic / hypothalamic Glioma ➢ Hypothalamic Hamartoma

Pituitary Macroadenoma [Figure 5-20-8] •

Clinical / Pathologic ➢ Most common suprasellar mass (50%) ➢ 10% of intracranial tumors ➢ Snowman shape ➢ Compressive symptoms ➢ Rare in prepubescent children, adolescent males

Sella and suprasellar macroadenoma with narrowing where lesion extends through the diaphragma sellae (courtesy of Amirsys, Inc.)

Suprasellar: Pathology •

Macroadenoma ➢ > 10mm ➢ Enlarged sella turcica ➢ Sellar/suprasellar ➢ MR test of choice ➢ Robust enhancement ➢ ? Cav. sinus invasion, mass effect on chiasm

Pituitary Adenoma •

Prolactinoma ➢ 30% of adenomas ➢ Female >> Males ➢ Galactorrhea ➢ Amenorrhea ➢ Serum PRL > 150ng/mL ➢ If > 1000ng/mL, cavernous sinus invasion

Neuroradiology

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Sella and Parasellar Region

Pituitary Macroadenoma: CT •

CECT ➢ Enlarged sella turcica ➢ Moderate to strong enhancement ➢ May be heterogeneous (cysts, hemorrhage)

Figure 5-20-9

Pituitary Macroadenoma: MR [Figure 5-20-9] Imaging Features • Isointense GM: T1, T2WI • May have hemorrhage, cystic components • Figure-eight, snowman • Robust but heterogeneous enhancement • Determining cavernous sinus invasion difficult

Pituitary Macroadenoma: MR Cavernous Sinus Invasion • • • •

More aggressive Can’t be resected > 2/3 surrounds ICA ICA venous sulcus compartment filled

Invasive Pituitary Macroadenoma [Figure 5-20-10] • • •

Pituitary macroadenoma with right cavernous sinus invasion

May extend inferiorly into sphenoid sinus & skull base Must differentiate from skull base primary tumor Will involve bony sella turcica & pituitary gland

Figure 5-20-10

Invasive macroadenoma (courtesy of Amirsys, Inc.)

Pituitary Apoplexy 1. 2. • • • •

Hemorrhage into tumor Pituitary gland infarction Acute onset Headache, visual changes, vomiting Usually hemorrhagic, may be non-hem May be life threatening

Figure 5-20-11

Pituitary Apoplexy [Figure 5-20-11] Suprasellar: Pathology •

Meningioma ➢ 2nd most common (adults) ➢ 15% of meningiomas ❖ Tuberculum sellae ❖ Clinoid processes ❖ Cavernous sinus ➢ Look for pituitary gland distinct from mass ➢ Sag images helpful

Sella and Parasellar Region

Pituitary apoplexy can be hemorrhagic or not

1252 1254

Neuroradiology

Suprasellar: Meningioma [Figures 5-20-12 and 5-20-13]

Figure 5-20-12

Suprasellar: Aneurysm • • •

Third most common lesion in adults Noncalcified suprasellar mass Must differentiate from other suprasellar masses ➢ Macroadenoma ➢ Meningioma

Suprasellar: Aneurysm - CT • •

Noncalcified central suprasellar mass Can be difficult to distinguish from adenoma, meningioma

Suprasellar meningioma on T2 and post-contrast T1 coronal images

Parasellar: Aneurysm - MRI [Figure 5-20-14] • •

Figure 5-20-13

Flow void or complex mass separate from pituitary Phase artifact

Figure 5-20-14

Suprasellar meningioma with typical extension along planum sphenoidale

Figure 5-20-15

Left parasellar ICA aneurysm, with typical flow void

Suprasellar: Aneurysm Suprasellar Mass: Adult [Figure 5-20-15] •

• •

Invasive macroadenoma vs. meningioma

Macroadenoma ➢ Pituitary is mass ➢ Enhancement Meningioma ➢ Pit separate ➢ Marked C+ ➢ Dural tail Aneurysm ➢ Pit separate ➢ Flow void ➢ Complex SI

Figure 5-20-16

Suprasellar: Craniopharyngioma [Figure 5-20-16] •

Clinical ➢ Most common suprasellar mass in children ❖ 5-15 yrs ❖ 50-60 yrs ➢ Visual changes ➢ Endocrine dysfunction ➢ Mass effect ➢ H/A, N, V, papilledema

Neuroradiology

Graphic of craniopharyngioma, depicting complex sellar and suprasellar mass (courtesy of Amirsys, Inc.) 1253 1255

Sella and Parasellar Region

Suprasellar: Craniopharyngioma •

Figure 5-20-17

Pathology ➢ Adamantinomatous ❖ Classic ❖ “Crank-case oil” in cysts ➢ Papillary (Adults) ➢ 70% suprasellar with small sellar component ➢ 5% purely intrasellar

Craniopharyngioma: CT •

•

NECT scan ➢ Adamantinomatous ❖ 90% Ca++ (rim) ❖ 90% Cystic ➢ May enlarge sella ➢ Papillary type ❖ 50% Ca++ ❖ Majority solid CECT scan ➢ 90% enhance ➢ Solid ➢ Nodular ➢ Rim

Complex multiloculated sellar and suprasellar craniopharyngioma

Craniopharyngioma: MR • • • • •

Variable signal Often heterogeneous Ca++ difficult to detect Nodular & rim enhancement Occasionally optic tract hyperintensity on T2WI – mass effect

Craniopharyngioma: MR [Figure 5-20-17] Chiasmatic-hypothalamic glioma •

Clinical ➢ Second most common suprasellar mass in children ➢ Often large at presentation ➢ H/A, visual, endocrine abnormalities common ➢ M=F ➢ 15%-30% have NF-I

Chiasmatic-hypothalamic glioma •

Pathology ➢ 30% of all pilocytic astrocytomas occur in chiasm or hypothalamus ➢ 75% Pilocytic astrocytoma ➢ 25% Low-grade fibrillary ➢ Long-term survival (90% at 5 yrs, 75% at 10 yrs)

Figure 5-20-18

Chiasmatic-hypothalamic glioma - MR • • • • •

Variable signal Iso-, hypointense on T1WI Hyperintense on T2WI Variable enhancement Spread along optic tracts common

Chiasmatic-hypothalamic glioma [Figure 5-20-18] T2 and post contrast T1 axial images show a nonenhancing hypothalamic/chiasmic glioma Sella and Parasellar Region

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Neuroradiology

Hypothalamic Hamartoma [Figure 5-20-19] •

Figure 5-20-19

Clinical ➢ Precocious puberty ❖ Usually < 2yrs ➢ Gelastic seizures ➢ M>F ➢ Pallister-Hall ❖ Facial anomalies ❖ Polydactyly ❖ Imperforate anus

Hypothalamic Hamartoma •

Graphic and post contrast T1 sagittal image through hypothalamic hamartoma (courtesy of Amirsys, Inc.)

Pathology ➢ Hamartoma of tuber cinereum ➢ Congenital nonneoplastic heterotopia ➢ Between infundibular stalk, mamillary bodies

Figure 5-20-20

Hypothalamic Hamartoma : MR • • • • • •

Signal follows GM Isointense on T1WI May be slightly T2 hyperintense Pedunculated or sessile May project into 3rd ventricle Do not enhance

Hypothalamic Hamartoma

Comparison of suprasellar pediatric lesions

Suprasellar Mass: Child [Figure 5-20-20] •

• •

Cranio ➢ Complex mass ➢ 90% cystic ➢ 90% calcified Astrocytoma ➢ Chiasm/Hypoth ➢ T2 hyperintense ➢ Variable C+ Hamartoma ➢ Hypothalamus ➢ GM signal ➢ No C+

Infundibulum Differential Diagnosis •

•

Figure 5-20-21

Lesions ➢ Germinoma ➢ Langerhans cell histiocytosis (LCH) ➢ Sarcoid ➢ Lymphoma, Metastasis Rare Lesions ➢ Hypophysitis ➢ Pituicytoma

Infundibular: Germinoma [Figure 5-20-21] •

Clinical ➢ Suprasellar region is 2nd most common site ➢ M = F suprasellar ➢ 90% present < 20 yrs ➢ Endocrine dysfunction ❖ Diabetes insipidus ❖ Panhypopituitarism ➢ Very radiosensitive ➢ Up to 90% 10 survival

Neuroradiology

Graphic of infundibular lesion (courtesy of Amirsys, Inc.)

1255 1257

Sella and Parasellar Region

Germinoma •

Pathology ➢ Pineal most common ➢ Pineal + suprasellar 10% ➢ Similar to seminoma ➢ 2/3 of germ cell tumors are germinoma

Germinoma: Imaging •

CT & MR ➢ Combined lesion typical but may affect only infundibular stalk ➢ May be hyperdense (CT) ➢ Isointense T1WI ➢ Hyper- to isointense T2WI ➢ Enhances homogeneously ➢ CSF dissemination common

Germinoma: MR Figure 5-20-22

Langerhans Cell Histiocytosis •

Clinical ➢ First decade ➢ M>F ➢ Diabetes insipidus ➢ High signal of neurohypophysis is commonly absent ➢ Thickening of stalk ➢ Formerly Histiocytosis X

Langerhans Cell Histiocytosis [Figure 5-20-22] Sarcoid •

Clinical ➢ Chronic, multisystem, inflammatory disease ➢ Noncaseating granulomas ➢ Neurologic findings 5% ➢ Diabetes insipidus or hormone deficiency ➢ Steroid responsive Coronal and sagittal images show typical stalk thickening and enhancement of LCH

Sarcoid Lymphoma [Figure 5-20-23] •

Clinical ➢ NHL (B-cell) ➢ 90% supratentorial ➢ Pituitary gland, hypothalamus, stalk ➢ 6th - 7th decade ➢ AIDS: 4th decade

Figure 5-20-23

Lymphoma •

Imaging ➢ Pituitary gland, hypothalamus, stalk ➢ Hyperdense on CT ➢ T1 Iso- to hypointense ➢ T2 hypointense ➢ Homogeneous enhancement

Sella and Parasellar Region

Lymphoma

1256 1258

Neuroradiology

Metastasis • • • •

1% of sellar/parasellar masses Usually occurs with known primary Can involve third ventricle, hypothalamus, infundibular stalk May be both supra-, intrasellar

Figure 5-20-24

Metastasis: Pituitary Gland Metastasis:Infundibulum Infundibular Mass: Adult •

•

Sarcoid ➢ Systemic dz ➢ Thickened stalk ➢ Enhancement Lymphoma ➢ +/- Systemic dz ➢ Stalk or gland ➢ Enhancement

Differential diagnosis of infundibular mass in a child.

Infundibular Mass: Child [Figure 5-20-24] •

• •

LCH ➢ Thickened stalk ➢ “Bright spot” gone ➢ Enhancement Germinoma ➢ Stalk +/- pineal ➢ T2 hyperintense ➢ CSF spread Meningitis ➢ Meningeal ➢ Diffuse ➢ Enhancement

Presentation Summary •

• •

Intrasellar Mass ➢ Microadenoma, Rathke cleft cyst Suprasellar Mass ➢ Craniopharyngioma, Macroadenoma, Meningioma, Aneurysm Infundibular Lesion ➢ Germinoma, LCH ➢ Granulomatous disease, LH

References 1. 2. 3. 4. 5. 6. 7.

Bonneville JF, Cattin A, Racle A, et al: Dynamic CT of the laterosellar extradural venous spaces. AJNR 1989; 10: 535-542 Cottier J-P, Destrieux C, Brunereau L, Bertrand P, Moreau L, Jan M, Herbreteau D. Cavernous sinus invasion by pituitary adenoma: MR imaging. Radiology 2000; 215:463-469 Elster AD, Chen MYM, Williams DW III, et al: Pituitary gland: MR imaging of physiologic hypertrophy in adolescence. Radiology 1990;174: 681-685 Elster AD, Sanders TG, Vines FS, et al: Size and shape of the pituitary gland during pregnancy and post partum: measurement with MR imaging. Radiology 1991; 181 :531-535 Elster AD. Modern imaging of the pituitary. Radiology 1993; 187: 1-14 FitzPatrick M, Tartaglino LM, Hollander MD, Zimmerman RA, Flanders AE. Imaging of sellar and parasellar pathology. Radiol Clin North Am 1999;37:101-121 Sato N, Tanaka S, Tateno M, Ohya N, Takata K, Endo K. Origin of posterior pituitary high intensity on T1weighted magnetic resonance imaging: immunohistochemical, electron microscopic, and magnetic resonance studies of posterior pituitary lobe of dehydrated rabbits. Invest Radiol 1995; 30:567-571

Neuroradiology

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Congenital Spinal Anomalies Erin Simon Schwartz, MD Figure 5-21-1 Spinal Dysraphism [Figure 5-21-1] • •

Defect of closure of neural tube For defects of primary neurulation ➢ Involving tubulation of neural plate, separation from ectoderm, disjunction of superficial from neural ectoderm

Myelomeningocele [Figures 5-21-2 and 5-21-3] •

•

Failure of neurulation & placode elevation from expansion of SAS ➢ Placode protrudes through osseous & cutaneous defect Most LS, also more proximal, normal appearing distal cord Neural tube development. Neural plate and neural ➢ > = 4 sites initiating neurulation, site that fails crest derive from midline ectoderm, notochord and determines defect location somites arise from midline mesenchyme

Figure 5-21-2 Figure 5-21-3

Axial schematic of lumbar MMC with placode forming dorsal wall of expanded CSF space

Sagittal MR (22 weeks gestation) showing posterior osseous defect and neural tissue traversing expanded CSF space

Congenital Spinal Anomalies

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Neuroradiology

Myelocele (Myeloschisis) [Figure 5-21-4] • • •

Figure 5-21-4

Placode of OSD in plane with back Less common, embryologically similar Clinical signs & function similar

HemiMMC/Hemimyelocele • • •

Canal split, only one hemicord affected Rare, crucial to recognize as septum can tether cord --> decline in function after repair Present with markedly asymmetrical neurological abnormalities

Chiari II Malformation [Figure 5-21-5] • • •

Absence raises suspicion that “MMC” really terminal myelocystocele or lipoMMC From poor distention rhombencephalic vesicle, herniation CSF leakage Not assoc with CSD, may develop in TM

Axial lumbar myelocele with placode at level of skin defect

Figure 5-21-5

CSD with subcutaneous mass • • •

Lipoma with dorsal defect (lipoMMC/lipomyelocele(schisis)) Terminal myelocystocele Meningocele ➢ Cervical myelocystoceles & meningoceles extremely rare

Lipoma with Dorsal Defect • • •

Premature disjunction of cutaneous ectoderm from neuroectoderm allows mesenchyme to contact inner portion of neural tube As tube tries to close, mesenchyme --> fat, interferes with neurulation Lipomas contain ectodermal, mesodermal, endodermal elements = teratomas ➢ Grows in proportion to overall adipose, rarely with AVM, ▲ retether vs MMC

Lipomyelomeningocele [Figure 5-21-6] •

Lipoma outside canal, expanded SAS ➢ Placode deformed, with rotation toward lipoma & protrusion of meninges contralaterally ➢ Nerve roots deformed, short on side of lipoma (tether cord), elongated on side of meninges

Sagittal MR (22 weeks gestation) with small posterior fossa, hindbrain herniation, loss of supratentoral CSF spaces

Lipomyelocele (lipomyeloschesis) [Figure 5-21-7] •

Lipoma traverses defect to attach to placode within or along edge of canal

Figure 5-21-6

Figure 5-21-7

Axial lumbar lipomyelomeningocele with asymmetrical placode/lipoma interface outside canal Neuroradiology

Axial lumbar lipomyelocele with placode/lipoma interface along edge of canal 1259 1261

Congenital Spinal Anomalies

Meningocele [Figure 5-21-8] • • • •

Figure 5-21-8

Meningeal-lined CSF sac protruding through defect Cord does not enter sac, may be assoc with hypertrophy of filum or cord tethering ? From CSF pulsations Lateral assoc with NF 1, also post-trauma, connective tissue disorders

Terminal Myelocystocele [Figure 5-21-9] • • • • • •

Large, skin covered lumbosacral mass Dilatation of terminal ventricle Herniates through SB High assoc with caudal cell mass anomalies (GU, lower GI, abd wall) Cb herniation late Incontinent, extremely poor LE function

Figure 5-21-9

Thoracic meningocele

Figure 5-21-10

Schematic of terminal myelocystocele. The expanded CSF spaces are separate from the markedly dilated terminal ventricle.

CSD w/o Subcutaneous Mass • • • • • • •

Simple Dysraphic States Posterior spina bifida Intradural, intramedullary, filum terminale lipoma Persistent terminal ventricle Cutaneous stigmata: dimple, hemangioma, hair 4.8% nl neonates (74% simple dimple, No SB) Atypical dimples = high risk ➢ Larger than 5mm ➢ > 2.5cm cephalad to anus ➢ Hemangiomas, hairy patches, tails

Posterior Spina Bifida • • •

Most basic, commonly encountered L5 or S1, in isolation or with CSD when cord tethered Posterior arch of L5 can remain unfused until 5-6 yrs

Intraspinal Lipoma [Figure 5-21-10] • • •

Mesodermal cells contact primitive ependyma LS, any level Usually intradural, rarely entirely intramedullary

Congenital Spinal Anomalies

Intradural, extramedullary lipoma 1260 1262

Neuroradiology

Fibrolipomatous Infiltration of Filum [Figure 5-21-11] • •

Figure 5-21-11

Anomaly of secondary neurulation, from totipotential caudal cell mass Axial T1 most sens ➢ ?Small amount of fat anatomical variant vs any fat or thickening (>2mm) = tether

Tight Filum Terminale [Figure 5-21-12] • • •

Impaired retrogressive diff --> short, hypertrophic filum Conus low, assoc with SB, scoliosis, ± dermal sinus Axial T2 for detection

Figure 5-21-12

Fibrous thickening of filum terminale seen as hypointense on axial T2

Fat deposition within filum terminale

Figure 5-21-13

Persistent Terminal Ventricle [Figures 5-21-13 and •

5-21-14]

• • • •

Incomplete regression of TV of secondary neurulation, continuity with central canal ? small cavity PTV vs terminal myelocystocele (--> severe manifestation from inability of CSF to escape) Common, transient finding until 5 yrs Identical to CSF, in conus or conus-filum transition Anatomic variant, no clinical sig, but > 4-5mm can -> pain & neuro signs

Complex Dysraphic States Disruption during gastrulation --> notochordal derangement • Dorsal enteric fistula • Neurenteric cysts • Split cord malformation • Dorsal dermal sinus • Caudal regression syndrome • Segmental spinal dysgenesis

Development of distal spine from caudal cell mass via retrogressive differentiation

Figure 5-21-14

Gastrulation [Figure 5-21-15] • •

Cells migrate towards primitive streak, through primitive groove --> endoderm & mesoderm Prospective notochordal cells in cranial margin of Hensen node become notochordal process

Dorsal Enteric Fistula • • • •

Most multifaceted, failure of notochordal integration Persistence of neurenteric canal --> cleft from bowel to dorsal skin surface, Terminal ventricle on through vertebral column & spinal canal axial ultrasound through Usually lumbar conus medullaris High assoc with other CNS & non-CNS anomalies (renal, GI, CDH, pulmonary)

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Neurenteric Cyst [Figure 5-21-16] • • • • •

Figure 5-21-15

From partial regression of neurenteric canal Lined by secretory epith, contents iso to CSF or proteinaceous Intraspinal, ventral to T cord, anywhere Assoc with dysplastic vert > GI, resp anom Present late teens, compressive signs & sx

Split Cord Malformation [Figure 5-21-17] • • • • • • • • • •

Diastematomyelia (splitting) & diplomyelia (duplication), not radiologically distinguishable Type I: less common, osseous septum dividing two dural tubes & hemicords, assoc vertebral anomalies Type II: more common, hemicords in single dural tube ± fibrous septum Each hemicord contains a central canal, dorsal horn & ventral horn, each --> 1 nerve root Can be asymmetrical, smaller easily missed Septum --> tethering, ?SCM at CTJ under-recognized Migration of prospective notochordal Despite lack of s/sx, ~ 75% abnl voiding cells during gastrulation Cutaneous stigmata (Type I), F > M Present: scoliosis, ULE weak, wasting, tether Figure 5-21-16 8%-45% of OSD, separate from site of non-neurulation --> rarity of hemiMMC

Dorsal Dermal Sinus [Figures 5-21-18 and 5-21-19] • • • • • •

Epith-lined tube from dorsal skin to cord/coverings Focal nondisjunction neuroectoderm & cutaneous ectoderm Risk for bacterial infxn Epithelium secretes squamous debris, can exude cheesy material P/w mass or recurrent infxn ◗ 10% intraspinal dermoid

Figure 5-21-18

Thoracic osseous anomalies seen with neurenteric cyst

Figure 5-21-19

Figure 5-21-17

Axial lumbar myelocele with placode at level of skin defect

Scoliosis and hairy patches indicating underlying split cord malformation

Axial lumbar myelocele with placode at level of skin defect Congenital Spinal Anomalies

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Caudal Regression Syndrome [Figure 5-21-20] • • •

•

Figure 5-21-20

Spectrum from coccygeal/LS hypogenesis --> sirenomelia Infants of diabetic mothers, 1 in 7500 live births Assoc with: ➢ OEIS (omphalocele, exstrophy, imperforate anus, spinal anomalies) ➢ VACTERL (vertebral, renal, cardiac, limb anomalies with anorectal atresia & TEF) ➢ Currarino triad (sacral hypogenesis, anorectal malformations, presacral teratoma or meningocele) Level determines type & severity ➢ Type I: < =S1, even mid-T, cord terminates high, blunted tip & deformation of cauda common, ant & post separation of roots ➢ Type II: > = S2, less severe, distal-most conus absent, tethered by tight filum, lipoma, or CSD with subQ mass ➢ Mild CRS: only tip of conus absent, cord not tethered, may be missed

Segmental Spinal Dysgenesis • • •

Focal segment of lumbar or thoracic spine agenetic-markedly hypogenetic, cord segmentally disrupted, distal canal unaffected Distal cord large, focal kyphosis --> early presentation Anomalous lower extremities, incontinence ? Within CRS, morphology depends on level of notochordal disruption Distal --> CRS, Proximal --> SSD ➢ Frequency of CRS (11:1), indicates higher degree of susceptibility of the caudal cell mass to derangement

Blunted conus and absent distal sacrum/coccyx of caudal regression syndrome

References 1. 2. 3. 4.

Barkovich AJ. Pediatric Neuroimaging. 4th Ed. Lippincott, Williams & Wilkins, Philadelphia 2000. Dias MS, Partington M. Embryology of myelomeningocele and anencephaly. Neurosurg Focus 2004; 16:E1. Ellison D, Love S, Chimelli L, Harding BN, Lowe J, Vinters HV. Neuropathology: A Reference Text of CNS Pathology. 1st ed. Mosby International Ltd, London 1998. Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology 2000; 42:471-491.

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Congenital Spinal Anomalies

Imaging of the Suprahyoid Neck: Superficial, Parapaharyngeal and Carotid Spaces Wendy R. K. Smoker MS, MD, FACR Figure 5-22-1

Cervical Fascia [Figure 5-6-1] •

•

Superficial Cervical Fascia ➢ Fat-filled layer of connective tissue that completely surrounds the neck and permits the skin to glide easily over deeper structures Deep Cervical Fascia ➢ Superficial Layer (Investing Fascia) ➢ Middle Layer (Visceral or Pre-tracheal Fascia) ➢ Deep Layer (Perivertebral Fascia)

Fascial Spaces of the Suprahyoid Neck • • • • • • • • •

Superficial Space Parapharyngeal Space Carotid Space Masticator Space Parotid Space Pharyngeal Mucosal Space Retropharyngeal/Danger Space Perivertebral Space Posterior Cervical Space

Superficial Space-Contents • • • • • • •

Sternocleidomastoid muscle Trapezius muscle Platysma muscle Lymph nodes Blood vessels/EJV Hair follicles Fat

Hemangioma

Figure 5-22-2

Superficial Space-Pathology •

• • •

•

Hair follicles ➢ Sebacceous cyst Blood vessels: ➢ EJV thrombosis ➢ Hemangiomas/vascular malformations Lymph nodes ➢ Reactive/suppurative adenopathy; ➢ Nodal metastases Fat ➢ Lipoma/liposarcoma Pseudomass ➢ Fibromatosis coli

Hemangiomas [Figures 5-22-1 and 5-22-2] • • • • • •

Most common head and neck TUMOR of infancy and childhood Rarely present at birth but manifest in early infancy, grow slowly, and involute by adolescence 80% are isolated lesions Females > males Large facial hemangioma: Isointense on T1WI, Isointense on T1WI, hyperintense on T2WI hyperintense on T2WI, and intense enhancement +Enhancement

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Lymphatic Malformations [Figures 5-22-3 and 5-22-4] • • • •

Figure 5-22-3

Arise from sequestrations of the primitive embryonic yolk sac Classified according to lymphatic vessel size (capillary, cavernous, cystic) Largest is the cystic variety, previously termed cystic hygroma. Typically multiseptated Fluid-fluid levels within the lesions are almost pathognomonic

Madelung’s Disease • • • • •

Almost exclusively a disease of middle aged alcoholic males 50 years of age Appearance of lesions is preceded by 10 years of heavy drinking Non encapsulated fatty masses SIGHT DIAGNOSIS

A large lymphatic malformation with multiple septations

Liposarcoma • • • •

Slowly enlarging, painless, non-ulcerated mass Middle-aged onset Most arise de novo; frequently arise from the stroma rather than the submucosal or subcutaneous fat WHO classification recognizes 5 categories of liposarcomas: ➢ Well differentiated (adipocytic, sclerosing, and inflammatory subtypes) ➢ Dedifferentiated ➢ Myxoid ➢ Round cell ➢ Pleomorphic

Figure 5-22-4

Fibromatosis Coli [Figure 5-22-5] (Stenocleidomastoid Tumor of Infancy) • • • •

•

Diffuse SCM enlargement Most common type of congenital torticollis Fibrocollagenous infiltration-Cause????? Typical course: ➢ Not detected at birth ➢ Palpable neck mass at 2-4 weeks of age ➢ Increases in size for a few weeks (growth phase) ➢ Most recede spontaneously at 4-8 months of age This is a LEAVE ALONE lesion

Parapharyngeal Space (PPS) • • •

2 year-old child with a large, mixed, venolymphatic malformation

Figure 5-22-5

[Figure 5-22-6]

Is an “in-between” space lying between other fascially-defined spaces. Not fascially-defined itself. Few lesions actually arise within this space but typically originate from surrounding spaces and produce characteristic encroachment on the PPS fat. Can thereby define the space of origin. The “parapharyngeal space” can be considered as consisting of two compartments: ➢ Prestyloid = Parapharyngeal Space (PPS) ➢ Retrostyloid = Carotid Space (CS)

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Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces

Parapharyngeal Space - Contents • • • • • •

Figure 5-22-6

Fat Branches of the mandibular nerve (V3) Internal maxillary artery branches Ascending pharyngeal artery Pharyngeal venous plexus Ectopic salivary gland tissue

Parapharyngeal Space - Pathology •

[Figures 5-22-7 to 5-22-11]

• •

• •

Pseudomass ➢ Asymmetric pterygoid plexus of veins Congenital/Vascular ➢ Atypical second BCC, hemangioma, The normal “in-between” location of the PPS, lymphangioma, aneurysm colored in the diagram. T1W MR images Inflammatory optimally demonstrate the symmetric, “fat➢ Cellulitis/abscess filled” PPS spaces. Benign Tumor ➢ Pleomorphic adenoma from ectopic salivary gland rests, neurogenic tumor, lipoma Malignant Tumor ➢ MECa and ACCa from ectopic salivary gland rests, direct spread from malignancies in surrounding spaces, liposarcoma, distant mets (rare)

Figure 5-22-7

Figure 5-22-9

Small PPS hemangioma (arrows) demonstrates calcification on the CT image. The lesion is iso-intense on T1WI, hyperintense on T2WI, and exhibits intense enhancement

Figure 5-22-8 Small PPS lymphangioma (arrows) is isointense on T1WIs and demonstrates a fluid-fluid level on the T2WI

PPS odontogenic abscess (arrows) in a 17 year-old male, status-post left third molar removal 5 days ago, now with facial pain and swelling Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces

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Figure 5-22-10

Figure 5-22-11

Pleomorphic adenoma, well-centered within the PPS, is clearly separated from the deep lobe of the parotid gland (arrows)

Predominantly PPS lipoma. Since the PPS is not fascially-defined, intrinsic pathology is free to extend along fascial planes, as can be seen with this lipoma

Carotid Space - Contents • • • • •

Internal carotid artery Cranial nerves (IX-XII) Sympathetic Chain Internal jugular vein Deep cervical (internal jugular) lymph node chain

Carotid Space - Pathology •

• •

• • •

Pseudomass ➢ Ectatic CCA or ICA, asymmetric IJV (can mimic a vascular tumor) Congenital ➢ Second branchial cleft cysts Inflammatory ➢ Cellulitis/abscess, adenopathy Vascular Lesions ➢ IJV thrombosis or thrombophlebitis, ICA thrombosis, aneurysm, or dissection Benign Tumor ➢ Paragangliomas (jugular, vagal, carotid body), nerve sheath tumors, meningioma (from jugular foramen) Malignant Tumor ➢ SCCa nodal metastases, direct invasion by SCCa, NHL, other nodal mets

Figure 5-22-12

Second Branchial Cleft Cyst [Figure 5-22-12] •

•

•

The most common of the branchial cleft cysts ➢ 66%-75% in children “Classic location” at, or just caudal to, the angle of mandible (but may present in a variety of locations): ➢ Posterior to the submandibular gland ➢ Anterior to the SCM ➢ Lateral to the carotid space ➢ Occasionally see a “beak” with the cyst pointing medially between the ECA and ICA (track) Often enlarge with URIs and become painful if infected

ICA Dissection with Pseudoaneurysm [Figure 5-22-13]

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Classic displacements produced by second branchial cleft cysts: Anterior displacement of the submandibular gland (SMG), medial displacement of the carotid space (CS) structures, and posterior displacement of the sternocleidomastoid muscle (SCM)

Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces

Paragangliomas • • • • • • • •

Figure 5-22-13

Highly vascular tumors arising from non-chromaffin cells of neural crest origin Usually asymptomatic from endocrine standpoint but rare catecholamine-secreting lesions do occur No resemblance to true glomus tumors found in skin and superficial soft tissues so term “glomus tumor” should be avoided Familial and non-familial patterns exist with synchronous lesions seen in 5-8% of non-familial cases and up to 25% of familial cases Highly vascular “Salt and Pepper” appearance of larger lesions on MR suggestive in correct locations Both regional and distant (lung, liver) metastatic disease are reported in 10-15% of cases. Etiology is unclear-probably due to hypoxic stimuli Four common locations: ➢ Middle ear cavity-tympanicum paragangliomas ➢ Jugular foramen-jugular paragangliomas ➢ High carotid space-vagal paraganliomas ➢ Carotid bifurcation-carotid body paragangliomas

Jugular Paragangliomas [Figure 5-22-14] • • • • • •

Hemorrhage in the wall of the vessel is bestdemonstrated on non-contrast T1WI (arrow). The angiogram demonstrates the dissection to be maximal just below the base of the skull and shows a pseudoaneurysm at the level of approximately C1 (arrow)

Arise in adventitia of IJV, from Arnold’s nerve (IX) and Jacobson’s nerve (X) Represent the most common tumor found in the jugular foramen Permeative erosive changes with amputation of the jugular spine demonstrated on CT Multicentric in 5% of sporadic cases and up to 25% in familial cases Sx: Pulsatile tinnitus; IX-XI cranial neuropathy +/- XII Malignant with mets in approximately 3%

Figure 5-22-15

Meningioma [Figure 5-22-15] Figure 5-22-14

High carotid space meningioma (arrows) is isointense on T1WI, mixed intensity on T2WI, and markedly enhances. Calcification demonstrated on CT mitigates against consideration of a paraganglioma

A right jugular paraganglioma extends inferiorly into the high carotid space (arrows). Prominent flow-voids are seen on the T1WIs. The lesion erodes the jugular tubercle and fills the hypoglossal canal, the normal counterparts labeled on the left (arrows). Note late chronic denervation atrophy of the right hemitongue Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces

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Vagale Paragangliomas [Figure 5-22-16] • • • • •

Figure 5-22-16

Arise from paraganglia located around nodose ganglion, the more caudal of the two vagal ganglia Situated just below skull base, lower than typical jugulare lesions and higher than typical carotid body tumors Usually lie entirely within carotid space (post-styloid parapharyngeal space) As vagus nerve lies dorsal to ICA, these tumors usually displace ICA anteriorly Approximately 10% incidence of malignancy

Meningioma [Figure 5-22-17]

Figure 5-22-17 Large vagal paraganglioma displaces the ICA anteriorly with the ECA (arrows). Large flow voids are seen. The MRA optimally demonstrates the anterior vascular displacements (arrows)

Enhancing lesion in the high carotid space, which displaces the ICA anteriorly (arrows), suggests a vagal paraganglioma. However, identification of associated calcification and sclerosis (arrows) of the jugular tubercle, and lack of destruction, makes the diagnosis of meningioma

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Suprahyoid Neck: Superficial, Parapharyngeal, Carotid Spaces

Figure 5-22-18 Carotid space vagal schwannoma displaces the ICA anteriorly (arrows). The lesion is very homogeneous and exhibits no flow voids

Figure 5-22-19 Carotid Body Paragangliomas •

[Figures 5-22-19 and 5-22-20]

• • • •

Arise from paraganglia located in the “crotch” of the carotid bifucation-most common location Pathognomonic finding is “splaying” of the ECA and ICA and “filling” the bifucation Multiple in 5%-14% of sporadic cases and up to 33% in familial cases Sx: Only 8% of these lesions are large enough to present as carotid space mass; may have X and/or XII neuropathy Malignant in 10%-15% of cases

Classic displacements associated with carotid body paraganglioma with splaying of the ECA and ICA (arrows)

Figure 5-22-20

Patient with large ipsilateral vagal and jugular paragangliomas

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References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19.

Ablin DS, et al. Ultrasound and MR imaging of fibromatosis colli (sternomastoid tumor of infancy). Pediatr Radiol. 28(4):230-233, 1998. Ahuja A, et al. Madelung disease: Distribution of cervical fat and preoperative findings at sonography. MR, and CT. AJNR Am J Neuroradiol. 19(4):707-710, 1998. Alkadhi H, et al. Evaluation of topography and vascularization of cervical paragangliomas by magnetic resonance imaging and color duplex sonography. Neuroradiology. 44(1):83-90, 2002. Bancroft LW, et al. Imaging characteristics of spindle cell lipoma. AJR Am J Roentgenol. 181(5):1251-1254, 2003. Bousson V, et al. Dissections of the internal carotid artery: Three-dimensional time-of-flight MR angiography and MR imaging features. AJR Am J Roentgenol. 173(1):139-143, 1999. Eldevik OP, et al. Imaging findings in schwannomas of the jugular foramen. AJNR Am J Neuroradiol. 21(6):1139-1144, 2000. Fruin ME, et al. The carotid space in the suprahyoid neck. Seminars Ultrasound CT MR 11:504-510, 1990. Gilbert MR, et al. Meningioma of the jugular foramen: Glomus jugulare mimic and surgical challenge. Laryngoscope. 114(1):25-32, 2004. Gilmer-Hill HS, et al. Neurogenic tumors of the cervical vagus nerve: Report of four cases and review of the literature. Neurosurgery. 46(6):1498-1503, 2000. Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005. Heis HA, et al. Carotid body tumors. Int Surg. 88(4):226-230, 2003. Koeller KK, et al. Congenital cystic masses of the neck: Radiologic-pathologic correlation. RadioGraphics. 19(1):121-146, 1999. Macdonald AJ, et al. Primary jugular foramen meningiomas: Imaging appearance and differentiating features. AJR Am J Roentgenol. 182(2):373-377, 2004. Mafee MF, et al. Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and simulating lesions: Role of MR Imaging. Radiol Clin North Am. 38(5):1059-1076, 2000. Nadgir RN, et al. Simultaneous bilateral internal carotid and vertebral artery dissection following chiropractic manipulation: Case report and review of the literature. Neuroradiology. 45(5):311-314, 2003. Noujaim SE, et al. Paraganglioma of the temporal bone: Role of magnetic resonance imaging versus computed tomography. Top Magn Reson Imaging. 11(2):108-122, 2000. Rao AB, et al. From the archives of the AFIP. Paragangliomas of the head and neck: Radiologic-pathologic correlation. Armed Forces Institute of Pathology. RadioGraphics. 19(6):1605-1632, 1999. Sharma S, et al. Fibromatosis colli in infants: A cytologic study of eight cases. Acta Cytol. 47(3):359-362, 2003. Snitzer EL, et al. Magnetic resonance imaging appearance of fibromatosis colli. Magn Reson Imaging. 15(7):869-871, 1997.

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Imaging of the Suprahyoid Neck: Masticator and Parotid Spaces Wendy R. K. Smoker MS, MD, FACR

Figure 5-22-21

Masticator Space - Contents •

[Figure 5-22-21]

• • •

Muscles of Mastication ➢ Lateral and medial pterygoid muscles ➢ Masseter muscle ➢ Temporalis muscle Inferior alveolar nerve (branch of V3) Inferior alveolar artery and vein Ramus and posterior body of the mandible

Masticator Space Normal Anatomy Coronal Plane [Figure 5-22-22]

Figure 5-22-22 Normal anatomy of the masticator space. Axial images best demonstrate muscles within this space. The coronal and parasagittal images demonstrate the mandibular division of the trigeminal nerve (V3) (arrow) and are best to assess for perineural tumor

Figure 5-22-23

The fascia attaches to the skull base MEDIAL to the foramen ovale putting the foramen within this fascial compartment. The cortical margins of the foramen ovale are arrowed. V3 is well-visualized traversing the foramen

There is volume loss and complete fatty replacement of the muscles innervated by V3, including those innervated by the mylohyoid branch (mylohyoid and anterior digastric muscles). The responsible meningioma is indicated by the arrow Suprahyoid Neck: Masticator and Parotid Spaces

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Masticator Space - Pathology •

Figure 5-22-24

[Figures 5-22-23 to 5-22-32]

• •

•

•

•

Pseudotumors ➢ Denervation atrophy patterns ➢ “Benign” masseteric hypertrophy ➢ Accessory parotid tissue Congenital Lesions ➢ Hemangiomas/Lymphangiomas Vascular ➢ Aneurysm Inflammatory/Infection ➢ Odontogenic abscess, with or without, osteo is most common Benign masseteric hypertrophy Benign Neoplasms Note benign-appearing enlargement ➢ Lipomas of the right masseter muscle. Not ➢ Neurogenic tumors infrequently, this is perceived as a ➢ Aggressive fibromatosis (Desmoid) parotid mass ➢ Hemangiopericytomas Malignant Neoplasms ➢ Malignant schwannomas Figure 5-22-26 ➢ Non-Hodgkin Lymphoma ➢ Chondrosarcoma ➢ Osteogenic sarcoma ➢ SCCa spread from contiguous spaces ➢ Osseous mets to mandible

(Long Parotid Tails and) Accessory Parotid Tissue • • •

[Figure 5-22-25]

•

Encountered in approximately 20% of the population May be unilateral or bilateral Has separate ductal system draining into Stensen’s duct Neoplastic involvement is uncommon, but when it does occur, 50% of lesions are malignant (as oppossed to main parotid gland in which majority of tumors are benign) Masseter hemangioma (arrows): Isointense on T1WI, hyperintense on T2WI and exhibits enhancement

Figure 5-22-25

Figure 5-22-27

The right parotid tail is covering the entirety of the masseter muscle (arrows). Patient complained of a right cheek mass Severe masticator space infection spreading up along the temporalis muscle (arrows on coronal image) with medial pyerygoid muscle abscess (arrows on axial images). The elderly male had suffered a fall, complained of very severe headaches, and was suspected of having a SDH. PMH indicated he had a dental procedure 2 months prior to his fall! Neuroradiology

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Suprahyoid Neck: Masticator and Parotid Spaces

Masticator Space Infections •

Figure 5-22-28

[Figure 5-22-27]

• • • •

Most common cause is seeding from infected tooth following dental manipulation In patients s/p zygomatic arch wiring for treatment of facial fractures, evaluate the suprazygomatic MS Check bone windows for mandibular osteomyelitis Check all spaces for pus pockets Generally, one drain is necessary for each fascial space involved

Benign Neoplasms • • •

[Figure 5-22-28]

Neurogenic Tumors Aggressive Fibromatosis (Extraabdominal Desmoid) Lipomas

Aggressive Fibromatosis [Figures 5-22-29] • • • • • •

Extra-abdominal desmoid tumor of fibrous origin More aggressive than their abdominal counterparts-infiltrate muscles and encase vessels and nerves High recurrence rate after incomplete resection Isointense to muscle on T1WI Variable T2 signal depending on amount of fibrous tissue and collagen Enhance after contrast

Figure 5-22-29

Massive enlargement of the right foramen ovale (arrows) produced by a large V3 schwannoma traversing the foramen

Aggressive fibromatosis. CT demonstrates loss of the fat planes within the left masticator space and bowing of the posterolateral wall of the maxillary sinus (arrow). The lesion (predominantly replacing the lateral pyerygoid muscle) is isointense on T1WI, very hypointense on T2WI (reflecting the very fibrous content) and demonstrates mild enhancement (arrows)

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Malignant Neoplasms • • • • • •

[Figures 5-22-30 to 5-22-32]

Malignant schwannoma Non-Hodgkin lymphoma Chondrosarcoma Osteogenic sarcoma SCCa spread from contiguous spaces Metastases (usually mandibular)

Figure 5-22-30

Non-Hodgkin lymphoma. There is loss of the fat planes within the masticator space, including enlargement and infiltration of the temporalis muscle in the suprazygomatic masticator space (arrow). The right pterygopalatine fossa is enlarged indicating V2 perineural extension. Involvement of the extraconal orbit and extra-axial middle cranial fossa are evident. Involvement of the lateral pterygoid and temporalis muscles are evident (arrows) and extension along V2 and V3 is noted (open arrows)

Figure 5-22-32

Figure 5-22-31

T1 WI

Chondrosarcoma - This lesion replaces fat at the entrance to the inferior alveolar canal, widens the foramen, and replaces the normal fatty marrow (colored arrows). The normal fat at the entrance to the inferior alveolar canal on the left is indicated by the white arrows

Neuroradiology

Mandibular breast metastasis There is almost complete destruction of the right mandible in this 42 yearold woman presenting with trismus. This was the INITIAL manifestation of her breast carcinoma 1277

Suprahyoid Neck: Masticator and Parotid Spaces

Parotid Space - Contents •

Figure 5-22-33

[Figures 5-22-33 and 5-22-34]

• • • •

Parotid gland and duct (Stenson’s duct) coursing through buccal space Facial nerve (VII): Creates a surgical plane that divides parotid into superficial and deep lobes; Not usually identifiable on either CT or MR; Courses immediately lateral to retromandibular vein Retromandibular vein (lateral to ECA) External carotid artery Intraparotid lymph nodes: 20-30 nodes; first order drainage for adj scalp, EAC, and deep face

Parotid Space - Pathology [Figure 5-22-36] •

• • • •

Pseudomass Stylomandibular tunnel - Superficial ➢ Long parotid tails, accessory parotid glands, parotid agenesis parotid space pathology may push Congenital through and enlarge the ➢ First branchial cleft cyst; hemangiomas; lymphangiomas stylomandibular tunnel (double Inflammatory / Infection headed arrow) and compress the ➢ Abscess or cellulitis; benign lymphoepithelial lesions; reactive PPS fat from a posterolateral adenopathy; sialoliths; sialectasis (including autoimmune) direction (shaded arrow) Benign Neoplasm ➢ Pleomorphic adenoma; Warthin’s tumor; oncocytoma; lipoma; Figure 5-22-34 VII neurogenic tumor Malignant Neoplasm ➢ Mucoepidermoid CA; adenoid cystic CA; NHL; acinic cell CA; malignant myxoid tumor; metastases (SCCa, melanoma, NHL)

1st Branchial Cleft Cysts [Figure 5-22-35] • •

Account for 8% of BCCs Typical History: Middle aged female with h/o multiple parotid abscesses unresponsive to drainage and antibiotics (Otorrhea if cyst connects to bony-cartilaginous junction of EAC) • If there is an external sinus, it is typically found in the skin at angle of mandible • Will image as cyst superficial to, within, or deep to parotid gland. Normal anatomy: Dots=parotid Wall thickness varies with degree of inflammation glands; Parotid ducts (short arrows) • CT is preferred to MR as it better defines cystic nature of lesion in traverse the buccal space fat and some cases pierce the buccinator muscles (long arrows) to enter the vestibule of the Benign Lymphoepithelial Lesions [Figure 5-22-37] oral cavity opposite the second • Old term of benign lymphoepithelial cysts replaced as both solid maxillary molar and cystic lesions occur • •

Typical history: Bilateral parotid swelling associated with cervical adenopathy, usually in a patient seropositive for HIV virus (parotid lesions may occur prior to seroconversion and may be the initial presentation) Imaging features: Bilateral parotid enlargement associated with intragladular cystic and solid lesions. May see associated cervical adenopathy

Figure 5-22-35

A well-circumscribed, first branchial cleft cyst involves the parotid tail (arrows). Other cystic lesions cannot be differentiated

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Figure 5-22-36 Figure 5-22-37

A large hemangioma (arrows) replaces much of the left parotid gland, isointense on T1WI, hyperintense on T2WI, and exhibiting marked enhancement

Lymphoepithelial lesions - There are multiple cystic and solid lesions in the parotid glands bilaterally in this 27 year-old HIV positive

Sjogren’s Syndrome [Figure 5-22-38] •

• • •

Clinical triad: ➢ Enlarged salivary glands with xerostomia ➢ Enlarged lacrimal glands with keratoconjunctivitis sicca ➢ Connective tissue disease (RA most common) Increased risk of developing a lymphoma, often aggressive biologically Sialography: Punctate, globular, cavitary, and destructive lesions can all be seen CT/MR: Enlarged glands with honeycomb appearance; Some cysts may be quite large and indistinguishable from LEL of AIDS by imaging alone

Figure 5-22-38

Pleomorphic Adenoma [Figures 5-22-39 and 5-22-40] • • • • • •

Most common benign parotid tumor (80%); if left Sjogren’s syndrome - The non-contrast CT scan untreated, est. 25% will undergo malignant demonstrates cystic enlargement of the parotid degeneration glands with specks of calcification identified, Path: Mixture of epithelial and myoepithelial cells suggestive of an infectious process. The MR Typical patient: >50 years of age with slow-growing images reveal multiple, various-sized cysts in the lump in the cheek superficial and deep lobes of both glands Sharply marginated; round, oval, or lobulated CT: Variable enhancement, infrequent dystrophic calcification, and internal pockets of low density when large due to “mucoid Figure 5-22-39 matrix” MR: Very hyperintense on T2WI; may have internal pockets of greater hyperintensity if “mucoid matrix”

Classic superficial lobe pleomorphic adenoma Neuroradiology

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Figure 5-22-40

Figure 5-22-41 Large deep lobe pleomorphic adenoma herniates through and widens the right stylomandibular tunnel (arrows)

Warthin Tumor:

Warthin’s Tumor Large cystic/solid right parotid superficial mass in a 65 year-old man. The heterogeneity would suggest (Papillary Cystadenoma Lymphomatosum) • • • •

a lesion other than a pleomorphic adenoma

[Figure 5-22-41]

• • •

Second most common benign tumor of the parotid space 80% male, greater than 50 years of age Usually slow-growing mass in region of parotid tail Arise from ectopic salivary gland rests within intraparotid lymph nodes Limited to PAROTID GLAND ONLY Bilateral in 10%-15% of patients Imaging: Well-circumscribed, usually 3-4 cm ➢ Complex mixture of solid and cystic components ➢ Appears more complex than typical PA

Figure 5-22-42

Oncocytoma • • • • •

Occurs exclusively in adults over 50 years of age; No sex predilection; about 1% of parotid tumors Oncocytes are large cells with granular eosinophilic cytoplasm that may be found in groups, normally Multiple facial nerve neurofibromas (arrows) in a within the parotid gland. patient with NF2. Also note the ipsilateral V2 An ONCOCYTOMA describes a solid tumor neurofibroma in the right retromaxillary fat, bowing composed entirely of oncocytes the posterolateral maxillary wall Imaging features are essentially identical to those of a pleomorphic adenoma May be multiple

Figure 5-22-43

Malignant Tumors • • • • • • • •

The smaller the salivary gland, the higher the chance a mass is malignant Sublingual mass-70% malignancy Submandibular mass-60% malignancy Parotid mass-20% malignancy Mucoepidermoid Carcinoma-more than 80% occur in parotid glands; most common malignant salivary gland tumor in most series Adenoid Cystic Carcinoma-2%-6% of parotid tumors Acinic Cell Carcinoma-10%-30% of parotid tumors Low-grade superficial parotid lobe mucoepidermoid Adenocarcinoma-rare in major salivary carcinoma. The lesion is somewhat complex and exhibits glands indistinct margins laterally (arrows) leading away from the diagnosis of a benign neoplasm

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Mucoepidermoid Carcinoma [Figure 5-22-43] • • • • •

Figure 5-22-44

Most common malignant lesion in parotid gland Most common between 35 and 60 years of age but may be found at any age and is most common malignant tumor in persons under 20 years of age. Slight predilection for women Clinical: Rock hard mass with associated pain and itching over the course of the facial nerve. Imaging features depend on grade: ➢ Low grade: Benign appearance; cannot distinguish from pleomophic adenoma ➢ Higher grade: Infiltrating, indistinct margins; look for PNT along VII!!! ➢ Low to intermediate signal on T1 and T2WI

References 1.

2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13.

VII Perineural Tumor - This patient has recurrent adenoid cystic carcinoma of the parotid gland. Curtin HD. Detection of perineural spread: Fat Note perineural extension of tumor along the facial suppression versus no fat suppression. AJNR Am J nerve (VII) up through the stylomastoid foramen Neuroradiol. 25(1):1-3, 2004. (arrow on coronal) to extend to the geniculate Go JL, et al. The trigeminal nerve. Semin Ultrasound CT ganglion (arrow on axial) MR. 22(6):502-520, 2001. Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005. Holliday RA, et al. Benign lymphoepithelial parotid cysts and hyperplastic cervical adenopathy in AIDS risk patients: A new CT appearance. Radiology. 168:439-441, 1998. Izumi M, et al. MR imaging of the parotid gland in Sjogren’s syndrome: A proposal for new diagnostic criteria. AJR. 166:1483-1487, 1996. Marsot-Dupuch K, et al. Mandibular nerve: MR versus CT about 10 proved unusual tumors. Neuroradiology. 32:492-496, 1990. Palacios E, et al. Benign asymmetric hypertrophy of the masticator muscles. Near Nose Throat J. 79(12):915, 2000. Russo CP, et al. MR appearance of trigeminal and hypoglossal motor denervation. AJNR Am J Neuroradiol. 18(7):1375-1383, 1997. Shah GV. MR imaging of salivary glands. Magn Reson Imaging Clin N Am. 19(4):631-662, 2002. Triglia JM, et al. First branchial cleft anomalies: A study of 39 cases and a review of the literature. Arch Otolaryngol Head Neck Surg. 124(3):291-295, 1998. Tryhus MR, et al. The normal and diseased masticator space. Semin Ultrasound CT MR. 11:476-485, 1990. Williams LS, et al. MR imaging of the trigeminal ganglion, nerve, and the perineural vascular plexus: Normal appearance and variants with correlation to cadaver specimens. AJNR Am J Neuroradiol. 24(7):1317-1323, 2003. Yonetsu K, et al. Deep facial infections of odontogenic origin: CT assessment of pathways of space involvement. AJNR. 19:123-128, 1998.

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Imaging of the Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity Wendy R. K. Smoker MS, MD, FACR Pharyngeal Mucosal Space •

Figure 5-22-45

[Figures 5-22-45 and 5-22-46]

•

Extends from skull base to hyoid bone and includes the nasopharynx, oropharynx, and hypopharynx The mucosa lining the upper aerodigestive tract

Pharyngeal Mucosal Space - Contents • • • • • •

Mucosa Waldeyer’s ring of lymphatic tissue Minor salivary glands (esp. in soft palate) Superior and middle pharyngeal constrictor muscles Cartilaginous (distal) end of eustachian tube (torus tubarius) (nasopharynx) Levator palatini muscle (nasopharynx)

Pharyngeal Mucosal Space - Pathology •

• • •

•

Pseudomass ➢ Lymphoid hyperplasia Congenital ➢ Tornwaldt cyst Infection / Inflammatory ➢ Post-inflammatory cysts (retention cysts); cellulitis/abscess Extension of the nasopharynx Benign Neoplasms ➢ Pleomorphic adenoma Malignant Neoplasms ➢ NPSCCa; non-Hodgkin lymphoma; minor salivary gland neoplasms (mucoepidermoid Ca, adenoid cystic Ca…)

Figure 5-22-46

A) Arrows indicate cartilaginous ends of eustachian tubes; B) short arrows=medial pterygoid muscles; long arrows=PPS fat; C) long arrows on PPS fat; short arrows indicate air in lateral pharyngeal recesses

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Nasopharyngeal Carcinoma (NPSCCa) [Figures 5-22-47 and 5-22-48] • • • • • •

Figure 5-22-47

Centered in lateral pharyngeal recess Invasion of levator palatini results in eustachian tube dysfunction and serous otitis media with CHL (check mastoids!) Perivascular spread (via foramen lacerum) and perineural extension (mainly V3) are common so skull base must be carefully assessed Nodal metastases present in 90% at presentation (retropharyngeal, level II, and level V-first) Distant metastases-uncommon at presentation (<10%) (bone, lung, and liver) Strong relationship with Epstein-Barr virus (EBV)

PMS Non-Hodgkin Lymphoma (NHL) • •

[Figure 5-22-49]

• • • • • •

Tends to remain localized and grow slowly NHL in H&N from nasopharyngeal lymphoid tissue in CT shows NPSCCa of left lateral recess (arrows) 35% of cases (versus palatine tonsil lymphoma in with ipsilateral foramen ovale enlargement (arrow). 50% and lingual tonsil lymphoma in 15%) MR also demonstrates the mass (arrow) with 50% have associated adenopathy at presentation enhancing perineural tumor extending through the 20% may have GI tract NHL involvement foramen (arrow) AIDS and Sjogren’s Syndrome predispose Usually >50 years; M:F = 1.5:1 Figure 5-22-48 Bulky mass filling nasopharynx T1WI-isointense, T2WI-hyperintense, enhance

48 year-old male with bilateral IX-XII palsies

Figure 5-22-49

Very large NPSCCA replaces the entire basiocciput (distal clivus), occipital condyles, jugular tubercles, and portions of the C1 lateral masses (seen on the coronal images). Coronal images also demonstrate extensive bilateral adenopathy

This homogeneous, bulky, NHL mass fills the nasopharynx but exhibits no infiltrtion of adjacent spaces and no skull base involvement

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Oropharynx

Figure 5-22-50

[Figure 5-22-50]

Oral PMS Anatomy Oro - PMS Pathology •

[Figure 5-22-51]

• •

•

•

Congenital ➢ Thyroglossal duct cyst; lingual thyroid Infection / Inflammatory ➢ Post-inflammatory cysts (retention cysts); cellulitis/abscess (tonsillar) Benign Neoplasms ➢ Pleomorphic adenoma Malignant Neoplasms ➢ SCCa (base of tongue and faucial pillars); non-Hodgkin lymphoma; minor salivary gland malignancies (especially soft palate) Pseudomass ➢ Lymphoid hyperplasia (lingual tonsil) Extension of the oropharynx

Thyroglossal Duct Cysts • • • • • • • •

[Figure 5-22-52]

Most common non-odontogenic extracranial head and neck cyst 20% are suprahyoid in location Well-circumscribed 2-4 cm Occasionally septated Capsular enhancement Smooth margins DDx: submental node

Figure 5-22-51

Figure 5-22-52

Oropharyngeal Mucosal Space Anatomy A) Arrows indicate tonsillar pillars; B) Long arrows indicate tonsillar pillars; short arrows indicate fat in PPS; C) Long arrows indicate tonsillar pillars; short arrow indicates soft palate; D) Arrows indicate regions of glossotonsillar sulci

Foramen cecum thyroglossal duct cyst (arrows). Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity

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Lingual Thyroid Gland • • • • • •

Figure 5-22-53

[Figure 5-22-53]

• •

Failure of thyroid descent from foramen cecum Accounts for 90% of ectopic thyroid High female predominance (7:1) Only functioning thyroid tissue in 70-80% CT: Hyperdense with avid enhancement MR: Iso-to hyperintense to muscle on both T1- and T2WI with strong enhancement Look in lower neck to confirm lack of gland in normal location Subject to typical “thyroid pathology”

Figure 5-22-54

Lingual thyroid gland. Note absence of thyroid in normal location in lower neck

Figure 5-22-55

Bilateral tonsillar abscesses

Figure 5-22-56

Right tonsillar SCCa with an ipsilateral level 2A node

Large base of tongue SCCa Neuroradiology

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Oral Cavity Normal Anatomy

Figure 5-22-57

[Figures 5-22-57 to 5-22-59]

Figure 5-22-58

The location of the oral cavity. The oral cavity is separated from the oropharynx by the circumvallate papillae, the soft palate complex, and the anterior tonsillar pillars

Figure 5-22-59 T1 WI

A: Sagittal MR. Black arrows=intrinsic muscles of the tongue; white arrows=genioglossus muscles; dotted arrows=geniohyoid muscles

T1 WI

B. Coronal MR. Vertical white arrows=sublingual spaces; horizontal black/white arrows=submandibular spaces; vertical black/white arrows=platysma muscles

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Oral Cavity Pathology •

Figure 5-22-60

[Figure 5-22-60]

• • •

Congenital Lesions ➢ Hemangiomas/vascular malformations; dermoids/epidermoids Inflammatory Lesions ➢ Cellulitis/abscesses; sialoliths; ranulas Benign Neoplasms ➢ Pleomorphic adenomas; aggressive fibromatosis Malignant Neoplasms ➢ SCCa (floor of mouth, oral tongue…); minor salivary gland neoplasms (SMG, SLG)

Dermoid Cysts •

[Figure 5-22-61]

• • • • • •

Refers to dermoid. epidermoid, and teratoid lesions Most uncommon of the congenital lesions Multiple oral cavity venous malformations in a child with Blue Rubber Bleb Nevus Syndrome Sublingual and submandibular locations Low density/intensity, unilocular, well-circumscribed Figure 5-22-61 Cyst wall enhances with contrast Individual fat globules=dermoid In absence of fat, cannot DDx epidermoid from dermoid

Ranulas •

[Figures 5-22-62 and 5-22-63]

• • • • •

Mucoceles/mucous retention cysts of the floor of the mouth Secondary to trauma or obstruction of sublingual gland/ducts Thin-walled; unilocular; non-enhancing Two varieties: Simple ranula-in SLS (true) Plunging ranula-in SMS (pseudocyst) Dermoid cysts in 2 patients with classic “bag of marbles” appearance

Figure 5-22-62

Figure 5-22-63

Simple ranula in a 30 year-old female with obstructive sleep apnea Neuroradiology

Plunging ranulas in 3 different patients 1287

Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity

Pleomorphic Adenomas •

Figure 5-22-64

[Figure 5-22-64]

• • • • • •

Most common benign glandular tumor--majority in parotid gland 8% in submandibular gland; 0.5% in sublingual gland Well demarcated; homogeneous when small Heterogeneous (cystic changes necrosis, hemorrhage) Hypo-isointense on T1WI Hyperintense on T2WI Homo/heterogeneous enhancement

Exostoses • • • • •

[Figure 5-22-65]

•

Dense cortical bone w/ or w/o cancellous bone Incidental unless they preclude proper denture fitting Occasionally very large and interfere with swallowing Torus palatinus Torus mandibularis ➢ interna / externa Torus maxillaris ➢ interna / externa

Sublingual gland pleomorphic adenoma

Figure 5-22-65

Figure 5-22-66

This T3N2b oral tongue SCCa (double arrows) invades the ipsilateral mylohyoid muscle (dots) to extend to the floor of the mouth. An ipsilateral node is indicated by the single arrow

Arrows indicate torus palatinus, bilateral mandibular tori interna, and bilateral maxillary externa tori Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity

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Figure 5-22-67

Left floor of mouth SCCa (arrows) extends to block the submandibular gland ducts bilaterally (arrows)

References 1.

2. 3. 4. 5. 6. 7. 7. 8.

Fischbein NJ, et al. Clinical utility of positron emission tomography with 18F-fluorodeoxyglucose in detecting residual/recurrent squamous cell carcinoma of the head and neck. AJNR Am J Neuroradiol. 19(7):1189-1196, 1998. Harnsberger et al. Diagnostic Imaging: Head and Neck. Amirsys Publishers, 2005. King AD, et al. In vivo proton MR spectroscopy of primary and nodal nasopharyngeal carcinoma. AJNR AM J Neuroradiol. 25(3):484-490, 2004. Mukherji SK, et al. Squamous cell carcinoma of the oropharynx and oral cavity: How imaging makes a difference. Semin Ultrasound CT. 19:463-475, 1998. Roh JL, et al. Nasopharyngeal carcinoma with skull base invasion: A necessity of staging subdivision. Am J Otolaryngol. 25(1):26-32, 2004. Sigal R, et al. CT and MR imaging of squamous cell carcinoma of the tongue and floor of the mouth. RadioGraphics. 16:787-810, 1996. Smoker WRK, et al. Computed tomography of the nasopharynx and related spaces. Seminars Ultrasound CT MR. 7:107-130, 1986. Smoker WRK. The Oral Cavity in Head and Neck Imaging (4th ed) Som and Curtin, eds. Mosby Year Book Publishers. pp 1377-1464, 2002. Weber AL, et al. Malignant tumors of the oral cavity and oropharynx: Clinical, pathologic, and radiologic evaluation. Neuroimaging Clin N Am. 13(3):443-464, 2003.

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Spine: Degenerative Disease and Infections Wendy R. K. Smoker MS, MD, FACR Figure 5-23-1 Low Back Pain Annual Costs • • •

250,000 operations/year 18-56 billion dollars/year 85% of costs are due to recurrent or chronic disability

Degenerative Disc Disease Lumbar Nomenclature [Figures 5-23-1 and 5-23-2] • • • •

Normal Bulge (symmetric, asymmetric) Annular tear/fissure Herniation (focal or broad-based) ➢ Protrusion ➢ Extrusion ➢ Extrusion with free fragment

Annular Tear/Fissure [Figure 5-23-3] • • • • •

Typically seen with degenerated discs but even seen in 25% of patients < 20 years of age Disc protrusions/extrusions often associated Primary failure of the annulus – all layers involved Present with back/radicular pain MR: 1-2 mm band of increased T2 signal (High intensity zone-HIZ) ➢ linear area of contrast enhancement (96%) ➢ not as sensitive as discography

Figure 5-23-3

Disc bulges Upper: Symmetric Lower: Asymmetric (>50% of circumference)

Figure 5-23-2

Multilevel disc bulges

Note high intensity zone on these T2WIs (arrows)

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Disc herniations and types

[Figures 5-23-4 and 5-23-5]

Figure 5-23-4

Figure 5-23-5

Focal: <25% of circumference

Broad-based: > 25% but < 50% of circumference

Protrusion

Extrusion

Protrusion

Extrusion

Extrusion with migration

L4-5 Disc Extrusion with Migration [Figure 5-23-6]

Degenerative changes typically induce secondary changes in the adjacent vertebral bodies (degenerative discovertebral changes) (Modic Changes)

Figure 5-23-6

This extruded disc retains a connection to the parent L4-5 disc as it migrates down behind the L5 vertebral body (arrows)

Type I

Vascularized Marrow (edema)

Low T1WI High T2WI

Type II

Proliferation of endplate fatty marrow

High T1WI High T2WI

Type III

Low T1WI Dense bone devoid of marrow (sclerosis) Low T2WI

Neuroradiology

plus sequestered fragment

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Spine: Degenerative Disease and Infections

Lumbar Spinal Canal and Foraminal Stenosis

Figure 5-23-7

Lumbar Facet Arthropathy [Figures 5-23-7 and 5-23-8] Figure 5-23-8

Multilevel degenerative disease with severe right L1-2 foraminal stenosis from a combination of disc bulge, facet arthropathy, and ligamentum flavum buckling

Facet Joint Synovial Cysts [Figure 5-23-9] • • • • • • • •

Note the nerve root exits Intraspinal juxta-articular synovial cysts are uncommon; associated with facet superiorly within the arthopathy neural foramen, just Most in lumbar spine, especially L4-5 under the pedicle Slight female preponderance; mean age 58 years (arrow). Note the normal Origin: DJD? Trauma?? “keyhole” configuration No specific symptom history; waxing and waning symptoms as these increase of the neural foramen and decrease in size CT: Cystic lesion w/ or w/o calcified rim adjacent to degenerated facet MR: Iso- or slightly hyperintense on SE sequences Figure 5-23-9 DDx: Ganglion cyst (does not communicate with joint)

Failed Back Surgery Syndrome (FBSS) [Figure 5-23-10] • • •

10%-40% of patients Intractable pain with variable incapacitation Differential considerations include: ➢ Recurrent/residual disc herniation ➢ Post-op infection ➢ Second level disease ➢ Facet disease ➢ Arachnoiditis ➢ Neuritis ➢ Epidural fibrosis (“Scar”) ➢ Miscellaneous

Spine: Degenerative Disease and Infections

Synovial cyst produces compression on the thecal sac from a posterolateral location (arrows). Compression of the nerve roots is best appreciated on the MR myelogram (right image)

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FBSS: Type I Arachnoiditis [Figure 5-23-11]

Figure 5-23-10

Figure 5-23-11

FBSS: Recurrent Disc. A large recurrent disc (arrows) demonstrates mild enhancement but the surrounding epidural fibrosis enhances to a much greater degree

Type I arachnoiditis. Nerve roots are clumped centrally within the thecal sac (arrows)

FBSS: Type II Arachnoiditis [Figure 5-23-12] Figure 5-23-12

Type II arachnoiditis. Nerve roots are “plastered” to the margins of the thecal sac producing an “empty” appearance (large arrows). The more proximal nerve roots are seen as distinct entities (small arrows)

Cervical Spinal Canal Stenosis Cervical Spinal Canal Stenosis [Figures 5-23-13 and 5-23-14] Figure 5-23-13

Congenital cervical spinal canal stenosis may be produced by either pedicle or lamina hypoplasia. The underlying dimensions of the canal should be assessed on every study as even mild degenerative disease can be significant in the face of underlying congenital stenosis

Neuroradiology

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Ossification of the Posterior Longitudinal Ligament (OPLL) • • • • •

Figure 5-23-14 C2

[Figures 5-23-15 and 5-23-16]

Begins with calcification followed by frank ossification of the posterior longitudinal ligament in upper C-spine and progresses into upper T-spine Can see on plain films in 0.12% of asymptomatic North Americans and 20%-30% of symptomatic patients Most easily seen on CT On MR, thick band of decreased signal on T1 and T2WI with mass effect on thecal sac and cord Associated with DISH

Figure 5-23-15

C4-5

C5-6

Acquired cervical spinal canal stenosis. Disc disease, coupled with ligamentum flavum buckling, produces severe canal stenosis at multiple levels (dark arrows) with associated pathologic signal in the spinal cord (white arrows), most severe at C5-6

T1WI

OPLL and DISH Thick hypointense signal is identified between the spinal cord and posterior vertebral bodies on MR (arrows). CT confirms the diagnosis of OPLL severely compromising the spinal canal diameter. DISH is present at lower levels

Figure 5-23-16

Ossification of Ligamentum Flavum Marked thecal sac and cord compression produced by OLF Spine: Degenerative Disease and Infections

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Infections

Figure 5-23-17

Pyogenic Osteomyelitis •

[Figures 5-23-17]

• • •

• • • •

Ill-defined T1-hypointense vertebral marrow with loss of adjacent endplate definition on both sides of infected disc; hyperintense on T2WI; enhances Lumbar-48%; Thoracic-35%; Cervical6.5% Paraspinal and/or epidural involvement in 75% S aureus most common; E coli if gram negative; salmonella in sickle cell Source: GU, GI, lung, cardiac, cutaneous/mucous-seeds vascularized subchondral bone Disc first site of involvement in children (vascularity) Bimodal: Pediatric patients and 6th-70th decade; also IV drug abusers and those with HIV Pain, tenderness, and fever

Pyogenic osteomyelitis. Note paraspinal and epidural involvement (arrows).

Figure 5-23-18 Post Gd

Tuberculous Osteomyelitis • •

[Figure 5-23-18]

• • • • •

AKA: Pott’s disease Typical: Gibbus vertebrae with relatively intact disc and paraspinal abscess Abscesses dissect over considerable length Mid-thoracic or thoracolumbar most common Inoculum in anterior vertebral body; spreads under anterior longitudinal ligament; spares discs due to absence of proteolytic enzymes M=F; presents in 50s; fever infrequent Concomitant pulmonary TB in 10%

T2 WI

Tuberculous osteomyelitis. Note gibbus deformity and extension under the anterior longitudinal ligament (arrows)

Epidural Phlegmon / Abscess •

[Figures 5-23-19 and 5-23-20]

• • • • • •

S aureus most common (57-73%), then TB (25%) Predisposing conditions: IV drug users, immunocompromised states, DM, CRF, alcoholism… Anterior from adjacent discitis or osteomyelitis Posterior from GU, GI, lung, cardiac… Direct inoculation from penetrating trauma, surgery…. Peaks in 50s and 70s Lumbar EA may mimic herniated disc

Figure 5-23-19

Epidural Phlegmon. A definite abscess cavity is not defined but abnormal enhancing soft tissue fills the posterior epidural space (arrows)

Neuroradiology

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Spine: Degenerative Disease and Infections

Miscellaneous

Figure 5-23-20

Brachial Plexus Traction Injury • •

[Figure 5-23-21]

•

•

AKA: Traumatic meningocele Avulsion of root(s) of brachial plexus invariably from traumatic injuries: (Adults-ATVs, motorcycles…; infants: complicated deliveries) Location critical to planning and prognosis: ➢ Pre-gang (central to dorsal root ganglion)-worse prognosis ➢ Post-gang (peripheral to dorsal root ganglion)-better prognosis Demonstration of “rootless” meningoceles are diagnostic

Idiopathic Transdural Cord Herniation •

Epidural and paraspinal abscesses. The paraspinal abscess is confirmed on the DWIs. The etiology was a septic right facet joint

[Figure 5-23-22]

• • • •

• •

Typical presentation is Brown-Sequard Syndrome Clinical deficits tend to be progressive unless treated Surgical reduction of the herniated cord can lead to improvement in the motor deficit Typical patient is an adult, affected location is the upper and mid-thoracic level (T2-T7) ??Cause?? ➢ Cong weakness of ventral dura? ➢ Damage by disc hernitation? ➢ Abnormal adhesions of cord to dura? ➢ Large root sleeve diverticulum? Interruption of the usually smooth ventral margin of the cord over a short segment (pulled anteriorly) Primary differential: Is cord is being displaced by a posterior mass (arachnoid cyst?) or is cord tethered ventrally?

Figure 5-23-21

Brachial Plexus Traction Injury (8 year-old boy s/p ATV accident with “dead” right arm). Multiple traumatic meningoceles are demonstrated (arrows)

Figure 5-23-22

Idiopathic transdural spinal cord herniation (arrows)

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References 1. 2. 3. 4. 5. 6. 7. 8.

9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20.

Akman S, et al. “Magnetic resonance imaging of tuberculous spondylitis”. Orthopedics. 26(1):69-73, 2003. Ben Hamouda K, et al. “Thoracic myelopathy caused by ossification of the ligamentum flavum: A report of 18 cases”. J Neurosurg. 99(2 Suppl):157-161, 2003. Boos N, et al. “Classification of age-related changes in lumbar intervertebral discs: 2002 Volvo Award in basic science”. Spine. 27(23):2631-2644, 2002. Carragee EJ. “The clinical use of magnetic resonance imaging in pyogenic vertebral osteomyelitis”. Spine. 22(7):780-785, 1997. Cinotti G, et al. “Stenosis of lumbar intervertebral foramen: Anatomic study on predisposing factors”. Spine. 27(3):223-229, 2002. Consensus statement on nomenclature and classification of lumbar disc pathology by NASS, ASSR, and ASNR. 2001. Dix JE, et al. “Spontaneous thoracic spinal cord herniation through an anterior dural defect”. AJNR Am J Neuroradiol. 19(7):1345-1348, 1998. Doi K, et al. “Cervical nerve root avulsion in brachial plexus injuries: Magnetic resonance imaging classification and comparison with myelography and computerized tomography myelography”. J Neurosurg. 96(3 Suppl):277284, 2002. Eastwood JD, et al. “Diffusion-weighted imaging in a patient with vertebral and epidural abscesses”. AJNR Am J Neuroradiol. 23(3):496-498, 2002. Fassett DR, et al. “Spinal epidural lipomatosis: A review of its causes and recommendations for treatment”. Neurosurg Focus. 16(4):Article 11, 2004. Geers C, et al. “Polygonal deformation of the dural sac in lumbar epidural lipomatosis: Anatomic explanation by the presence of meningovertebral ligaments”. AJNR Am J Neuroradiol. 24(7):1276-1282, 2003. Modic MT, et al. “Degenerative disk disease: Assessment of changes in vertebral body marrow with MR imaging”. Radiology. 166(1 Pt 1):193-199, 1988. Munter FM, et al. “Serial MR imaging of annular tears in lumbar intervertebral disks”. AJNR Am J Neuroradiol. 23(7):1105-1109, 2002. Ross JS, et al. “Assessment of extradural degenerative disease with Gd-DTPA-enhanced MR imaging: Correlation with surgical and pathologic findings”. AJNR Am J Neuroradiol. 10(6):1243-1249, 1989. Ross JS, et al. “Association between peridural scar and recurrent radicular pain after lumbar discectomy: Magnetic resonance evaluation”. Neurosurgery. 38:855-861, 1996. Ross JS, et al. “MR imaging of lumbar arachnoiditis”. AJR. 1987;149:1025-1032. Sasaoka R, et al. “Idiopathic spinal cord herniation in the thoracic spine as a cause of intractable leg pain: Case report and review of the literature”. J Spinal Disord Tech. 16(3):288-294, 2003. Van Goethem JW, et al. “Review article: MRI of the postoperative lumbar spine”. Neuroradiology. 44(9):723239, 2002. Wang MY, et al. “Intradural spinal arachnoid cysts in adults”. Surg Neurol. 60(1):49-55; discussion 55-56, 2003. Watters MR, et al. “Transdural spinal cord herniation: Imaging and clinical spectra”. AJNR Am J Neuroradiol. 19(7):1337-1344, 1998.

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Spinal Tumors, Cysts, and Mimics Wendy R. K. Smoker MS, MD, FACR First Things First!! - Localize the Lesion!! [Figure 5-24-1]

Figure 5-24-1

Extradural

Extramedullary Intramedullary Intradual Localizing a spinal lesion to the appropriate “compartment” (extradural, extramedullary-intradural, or intramedullary) allows a tailored differential diagnosis •

Common Intramedullary Lesions

•

Non-neoplastic: ➢ Acute trauma (contusion, edema…) ➢ Syringohydromyelia ❖ Syrinx-cavity in cord NOT lined by ependyma ❖ Hydromyelia-dilatation of the central canal lined by ependyma ➢ Demyelinating disease (MS, ADEM…) Neoplastic ➢ Ependymoma ➢ Astrocytoma

THERE IS NO SPECIFIC IMAGING PATTERN THAT RELIABLY PERMITS DIFFERENTIATION BETWEEN EPENDYMOMAS AND ASTROCYTOMAS Uncommon •

•

Non-neoplastic Lesions ➢ Acute cord ischemia/stroke ➢ Myelitis (Post viral ADEM, etc) Neoplastic Lesions ➢ Hemangioblastoma ➢ Astrocytoma (anaplastic and GBMs))

Spinal Tumors, Cysts, and Mimics

Rare • •

Non-neoplastic Lesions ➢ Vascular lesions (cavernomas, AVM, etc) ➢ Infections (sarcoid, TB, Lyme disease…) Neoplastic Lesions ➢ Metastases ➢ Lipoma ➢ Subependymoma ➢ Oligodendroglioma ➢ Ganglioma ➢ Paraganglioma

1296 1298

Neuroradiology

•

Ependymomas [Figure 5-24-2] • • • • • • •

Figure 5-24-2

Most common intramedullary tumor in adults- 60% Mean age 43 years; females predominate (slightly) Arise from ependymal cells lining central canal Slow-growing; canal expansion is typical 56% cervical; 28% thoracic; 16% lumbar 75% are isointense on T1WI; 100% hyperintense on T2WI Cystic degeneration and hemorrhage are common Hemosiderin deposition-common at periphery Heterogeneous enhancement in 65%

Ependymoma - Note lesion isointensity on T1WIs and relatively homogeneous enhancement on the Post-Gd images. • This lesion exhibits unusual hypointensity on the T2WI, most likely due to the prominent amount of hemosiderin noted on histology. Also note the associated areas of cystic Astrocytomas [Figure 5-24-3] • Second most common IM tumor overall; degeneration, indicated by the arrows. Significant cord edema most common tumor in children in best appreciated as marked increased signal intensity on the • Cervical=thoracic; M=F; mean age 21 T2WI years Figure 5-24-3 • Typically pilocytic and diffuse fibrillary types (low grade); anaplastic astrocytomas and GBMs are rare • May extend to involve the entire cord • Cyst formation is common; Syrinxabove or below tumor • Iso- to hypointense on T1WI; Hyperintense on T2WI • Hemorrhage is LESS COMMON than for ependymomas • Virtually 100% enhance • • •

Hemangioblastoma [Figure 5-24-4]

• • • •

Uncommon; 1%-5% of cord tumors Peak age between 30 and 40 years One third associated with VHL syndrome 85% intramedullary or combined intramedullary/ extramedullaryintradural 50% thoracic; 40% cervical; 80% are solitary Isointense on T1WI; hyperintense on T2WI; strong enhancement; may see flow voids, etc. On angio, highly vascular mass; dense prolonged tumor stain, prominent draining veins

Neuroradiology

Ependymoma - Lesion demonstrates isointensity on T1WIs, hyperintensity on T2WIs, and manifests only very faint enhancement on post-Gd images (somewhat unusual). A large signal void is present on the T2WIs, possibly representing an area of hemosiderin/calcification. Significant edema is also best appreciated on the T2WIs

1297 1299

Spinal Tumors, Cysts, and Mimics

• • •

Intramedullary Metastases [Figure 5-24-5]

•

Figure 5-24-4

Intramedullary metastases are rare 1%-3% of all intramedullary tumors No specific imaging characteristics to clearly distinguish from other intramedullary lesions Breast and lung most common Also lymphoma, leukemia, and malignant melanoma

Non-Neoplastic Intramedullary Pathology Cavernoma

[Figure 5-24-6]

Acute Disseminated Encephalomyelitis [Figure 5-24-7] Figure 5-24-5

Classic appearance of an intramedullary hemangioblastoma with T1 isointensity, T2 hyperintensity, and marked enhancement with numerous flow-voids, best seen on the T2 and post-Gd images (arrows)

Figure 5-24-6

Classic cavernoma appearance with central slight T1 hyperintensity and heterogeneous T2 hyperintensity (popcorn appearance). Note the rim of hemosiderin which “blooms” on the T2WI. The finding of multiple areas of intracranial hemosiderin on the gradient echo (GRE) image further solidifies the diagnosis. (Courtesy M Modic)

Figure 5-24-7

Intramedullary Metastasis (79 year-old female with lung carcinoma and brain metastases) - Note subtle enlargement of the spinal cord without significant signal change on the T1WI, hypointensity of the lesions on the T2WI, and lesion enhancement. The smaller lesion (arrow) would be almost impossible to detect without contrast Spinal Tumors, Cysts, and Mimics

1298 1300

Acute Disseminated Encephalomyelitis (15 yearold male with headache, lethargy, and nuchal rigidity) - Note significant cord expansion with slight T1 hypointensity, marked T2 hyperintensity, and very patchy enhancement. This boy had a URI two weeks prior to onset of symptoms Neuroradiology

Multiple Sclerosis

[Figure 5-24-8]

Extramedullary-Intradural Lesions • • • • •

Common

• • • • • • •

Neurogenic Neoplasms ➢ Neurofibromas ➢ Schwannomas Meningiomas Myxopapillary Ependymomas Arachnoiditis

Uncommon Lipomas Arachnoid Cysts Epidermoids/Dermoids Drop metastases AVM Infection Paragangliomas

Figure 5-24-8

Nerve Sheath Tumors • •

• • • •

Most common extramedullary-intradural tumors (70%-75%). 15% extradural; 15% dumbell Schwannomas slightly more common than neurofibromas Neurofibromas: M=F; 20-30 years. No true capsule ➢ Localized, diffuse, or plexiform Schwannomas: M=F; 30-60 years. Encapsulated ➢ 40% cystic changes; 10% hemorrhage; target sign Neural foraminal enlargement; pedicle thinning Isointense on T1WI; Hyperintense on T2WI; Enhance Multiple in neurofibromatosis

Neurofibroma

vs.

[Figures 5-24-9 to 5-24-11]

• • • • •

Neurofibroma Schwann cells Fibroblasts Acellular material Infiltrating Resect parent nerve

• • • • •

Multiple Sclerosis - Note absence of significant spinal cord expansion. The MS plaques are not Schwannoma appreciated on the T1WI, only faintly suspected on the T2WI, but very well seen on the STIR sequence. Faint enhancement of one, probably Schwannoma more active, plaque can be seen on the post Schwann cell neoplasm contrast sequence (arrow) Secondary vascular Figure 5-24-9 changes Mostly cellular Encapsulated Nerve sparing surgery

Figure 5-24-10 T1WI

T2WI

Post Gd

Schwannoma at the level of the conus. Sagittal images nicely demonstrate the classic subarachnoid cap at the inferior margin of the lesion with widening of the subarachnoid space. Note hypointensity on the T1WI and hyperintensity on the T2WI. The post-contrast images demonstrate a nice “target sign.” The lesion occupies most of the spinal canal and significantly compresses the spinal cord, best seen on the axial post contrast image (arrow) Neuroradiology

Classic dumbbell neurofibroma at C2-3 demonstrates isointensity on T1WIs, hyperintensity on the T2WI and manifests very significant enhancement on the coronal post-Gd image

1299 1301

Spinal Tumors, Cysts, and Mimics

• • • • • • • •

Meningiomas

•

Figure 5-24-11

[Figures 5-24-12 and 5-24-13]

Second most common spinal tumor (25%) 90% intradural; 10% extradural or dumbell Females > males at 4:1; Primarily 5th-6th decade Thoracic (80%) > Cervical (16%) > Lumbar (4%) More often anterior in cervical region Below C7, more common posterior to cord 85% intradural; usually single unless NF 2 Iso on T1WI; iso or hyperintense on T2WI; hypointense if Ca++ Marked homogeneous enhancement

Figure 5-24-12 A large schwannoma is seen arising from the S1 nerve root (arrows), isointense on T1WI, heterogeneously hypointense on T2WI, and primarily peripheral enhancement following contrast

Figure 5-24-13

Meningioma (C Spine-Ventral) - The sagittal images clearly localize this lesion as extramedullary-intradural by demonstrating a nice subarachnoid cap (widening of the subarachnoid space-arrows). The lesion severely compresses the spinal cord, best appreciated on the axial T2WI image (outlined). The lesion is essentially isointense to cord on T1 and T2WIs

• • •

Myxopapillary Ependymoma

• • • • • •

[Figure 5-24-14]

Meningioma (T Spine-Dorsal) - Note lesion 27%-30% of all ependymomas; 90% of filum tumors isointensity on T1 and T2WIs and homogeneous Genetically different from intracranial ependymomas enhancement. The subarachnoid cap is best Occur exclusively in conus and filum terminale-from appreciated at the superior margin of the tumor on ependymal cells in filum the T2WI Males:females = 2:1; peak between 30 and 40 years Figure 5-24-14 Slow growing and may fill entire lumbar spinal canal Vertebral scalloping; canal enlargement Highly vascular—hemorrhage is common Iso on T1WI; hyper on T2WI; hypointense margin if hemosiderin Intense enhancement in 100%

“Other” Extramedullary-Intradural Neoplastic Lesions

A large myxopapillary ependymoma fills and expands the lumbar spinal canal, scalloping the lower vertebrae. Note isointensity on the T1WI, hyperintensity on theT2WI and marked enhancement. The small superior component (arrow) was suspected on the T2WI but only confirmed on the post-contrast image Spinal Tumors, Cysts, and Mimics

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Neuroradiology

Hemangioblastoma • •

Lipomas • • •

Figure 5-24-15

[Figure 5-24-15]

[Figure 5-24-16]

Originate from fat cells in subpial region Vertebral and dermal anomalies are NOT associated with these lipomas 25% are diagnosed within the first 5 years of life No sex predilection Excessive weight gain and pregnancy may predispose

All Post-Gd

Figure 5-24-16

Small hemangioblastoma in a patient with von Hipple Lindau syndrome. The enlarged feeding vessels (arrows) are well-demonstrated and may initially suggest a diagnosis of AVF

Figure 5-24-17

Large cervical lipoma has caused marked widening of the spinal canal and severe spinal cord compression. The lesion manifests fat signal intensity on the T1 and T2WIs. The intraoperative photograph shows fat bulging through the dura

Type III Meningeal Cysts (Arachnoid Cysts) • •

[Figure 5-24-17]

• • • • • • • • •

Intradural arachnoid cysts are rare Unclear etiology: ?congenital; ?hemorrhage; ?inflammation Thoracic spine is most common location 80% arise near septum posticum and located posterior to cord Most communicate with subarachnoid space CTM: compressed cord displaced anteriorly MR: signal intensity of CSF so may not be able to identify unless cord is displaced/deformed

Dermoid Cysts [Figure 5-24-18]

• •

Congenital (100%) Symptomatic before age 20; M=F 80% in lumbosacral or cauda Hypointense areas-? Water content from sweat gland secretions Fat hyperintensity on T1WI A large dorsal arachnoid cyst has produced May cause chemical meningitis if rupture with marked expansion of the spinal canal with severe cholesteol crystals discharged into CSF pedicle thinning and cord compression. The compressed spinal cord is outlined on the softtissue windows. The lesion exhibits fluid signal intensity on the T1 and T2WIs. Note severe cord compression (arrows)

Neuroradiology

1301 1303

Spinal Tumors, Cysts, and Mimics

Figure 5-24-18

Figure 5-24-19

T1WI

Implantation Epidermoid Cyst (7 year-old female-had LP as an infant) - The lesion is slightly hyperintense on the T1WI, very hyperintense on the T2WI (such that the margins cannot be separated from the surrounding CSF), and does not enhance. A tract from the prior LP is not identified Lumbar dermoid cyst. The complex nature of the lesion with identification of fat signal (high) on the T1WI should suggest the diagnosis. (Courtesy M Modic) • • •

Epidermoid Cysts

• • • •

[Figure 5-24-19]

Less than 1% of spinal tumors Congenital-60%; Aquired-40% Upper thoracic-17%; lower thoracic26%; lumbosacral-22%; cauda equina35% Strong association with lumbar puncture in neonatal period (implantation epidermoid) Iso or slightly hyperintense on T1WI; hyper on T2WI Mild rim enhancement DWI can DDx from arachnoid cysts and other lesions

Drop Metastases •

Figure 5-24-20

Drop Metastases (25 year-old woman with breast CA and radicular symptoms) - Numerous enhancing lesions on the cauda equina are identified

[Figures 5-24-20 and 5-24-21]

•

Figure 5-24-21

CNS Primary Tumors ➢ Astrocytomas ➢ Medulloblastomas ➢ Pineal cell tumors ➢ Ependymomas ➢ Germ cell tumors Non-CNS Primary Tumors ➢ Breast ➢ Lung ➢ Lymphoma ➢ Melanoma ➢ Pituitary

Diffuse Mantle Cell Lymphoma. Note marrow signal in the thoracic spine is diffusely abnormal (darker that that of the intervertebral discs). There is infiltration of virtually all of the nerve roots of the cauda equina such that they are not visualized as separate entities on the sagittal image. Enlargement of individual roots is best appreciated on the axial images Spinal Tumors, Cysts, and Mimics

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Neuroradiology

Extradural Lesions Common • • • • •

Uncommon

Epidural Metastases Herniated discs, etc Degenerative lesions (osteophytes, ligament infolding…) Lymphoma Infection (discitis, etc…)

Epidural Hematoma

• • • • • • • • • •

Epidural Abscess Arachnoid cysts Lipomas Primary vertebral tumors-benign Primary vertebral tumors-malignant Paget’s disease Epidural hematoma Epidural lipomatosis Extramedullary hematopoesis Angiolipomas

[Figure 5-24-22]

Epidural Lipomatosis •

[Figures 5-24-23 and 5-24-24]

• • •

Most often associated with chronic steroid use (exogenous or endogenous Cushing’s syndrome) Also associated with rapid weight gain and obesity Thoracic spine most common Myelopathic symptoms predominate

Figure 5-24-22

Figure 5-24-23

Three year-old girl with 2 day h/o neck pain and UE/LE weakness - A large epidural hematoma (from C2 to T5) is producing severe spinal cord compression. A fluid-fluid level is indicated by the arrows. (Courtesy A Gean)

Figure 5-24-24

Epidural Lipomatosis - Severe accumulation of dorsal epidural fat in the thoracic region is producing severe cord compression Epidural lipomatosis is producing severe compression of the cauda equina in the lumbar spine

Neuroradiology

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Spinal Tumors, Cysts, and Mimics

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18.

Arslanoglu A, et al. “MR imaging characteristics of pilomyxoid astrocytomas”. AJNR Am J Neuroradiol. 24(4):1906-1908, 2003. Chu BC, et al. “MR findings in spinal hemangioblastoma: Correlation with symptoms and with angiographic and surgical findings”. AJNR Am J Neuroradiol. 22(1):206-217, 2001. Cohen-Gadol AA, et al. “Spinal meningiomas in patients younger than 50 years of age: A 21-year experience”. J Neurosurg. 98(3 Suppl):258-263, 2003. Conti P, et al. “Spinal neurinomas: Retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature”. Surg Neurol. 61(1):34-43; discussion 44, 2004. Fujiwara F, et al. “Intradural spinal lipomas not associated with spinal dysraphysm: A report of four cases”. Neurosurgery. 37(6):1212-1215, 1995. Garg RK. “Acute disseminated encephalomyelitis”. Postgrad Med J. 79(927):11-17, 2003. Hickman SJ, et al. “Imaging of the spine in multiple sclerosis”. Neuroimaging Clin N Am. 10(4):689-704, viii, 2000. Khong PL, et al. “Childhood acute disseminated encephalomyelitis: The role of brain and spinal cord MRI”. Pediatr Radiol. 32(1):59-66, 2002. Kikuchi K, et al. “The utility of diffusion-weighted imaging with navigator-echo technique for the diagnosis of spinal epidermoid cysts”. AJNR Am J Neuroradiol. 21(6):1164-1166, 2000. Koeller KK, et al. “Neoplasms of the spinal cord and filum terminale: Radiologic-pathologic correlation”. RadioGraphics. 20:1721-1749, 2000. Murphey MD, et al. “Imaging of musculoskeletal neurogenic tumors: Radiologic-pathologic correlation”. RadioGraphics. 19:1253-1280, 1999. Potgieter S, et al. “Epidermoid tumours associated with lumbar punctures performed in early neonatal life”. Dev Med Child Neurol. 40(4):266-269, 1998. Simon JH. “Brain and spinal cord atrophy in multiple sclerosis”. Neuroimaging Clin N Am. 10(4):753-770, 2000. Sun B, et al. “MRI features of intramedullary spinal cord ependymomas”. J Neuroimaging. 13(4):346-351, 2003. Thakkar SD, et al. “Spinal tumours in neurofibromatosis type I: An MRI study of frequency, multiplicity and variety”. Neuroradiology. 41(9):625-629, 1999. Wanebo JE, et al. “The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease”. J Neurosurg. 98(1):82-94, 2003. Wang MY, et al. “Intradural spinal arachnoid cysts in adults”. Surg Neurol. 60(1):49-55; discussion 55-56, 2003. Yamada CY, et al. “Myxopapillary ependymoma of the filum terminale”. AJR Am J Roentgenol. 168(2):366, 1997.

Spinal Tumors, Cysts, and Mimics

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Neuroradiology

Congenital Abnormalities of the Brain Wendy R. K. Smoker MS, MD, FACR Figure 5-25-1

Timing of Events •

• •

Disorders of Dorsal Induction ➢ (3-4 weeks) Disorders of Ventral Induction ➢ (5-10 weeks) Disorders of Neuronal Proliferation ➢ (2-5 months)

Disorders of Dorsal Induction (Insult at 3-4 weeks) •

•

Cranioschesis ➢ Anencephaly ➢ Cephaloceles Chiari Malformations ➢ Chiari I ➢ Chiari II (Arnold Chiari Malformation) ➢ Chiari III ➢ Chiari IV (Cerebellar hypoplasia)

Anencephaly • • • • • •

Symmetric absence of calvaria, cerebral hemispheres, diencephalon Replaced by flat amorphous vascular- neural mass Facial structures and orbits present Most common CNS malf (1:1000) Invariably fatal (aborted/stillborn) Implications for future pregnancies: ➢ 1 in 50 (2%) risk in next child ➢ if 2 prior pg with CNS mal, risk is 1 in 10)

Cephaloceles •

• • • • •

Skull base (enchondral bone) ➢ Failure of neural tube closure or failure of ossification centers to unite Large cephalocele containing brain, Calvarium (membraneous bone) ventricles, and midline fat. Note ➢ Defective bone induction, pressure erosion, or failure of neural absence of the corpus callosum and tube closure an open-lip schizencephalic cleft Occipital (Arrow) ➢ 80%-90%; F>M; assoc with neural tube defects; most common in Caucasian NAs and Europeans Frontal Sincipital ➢ Nasofrontal ➢ Nasoethmoidal-Most common in SE Asia; M>F ➢ Nasoorbital Basal ➢ Transethmoidal, transsphenoidal, sphenomaxillary, sphenoorbital

Frontal Lipoencephalocystocele [Figure 5-25-1]

Neuroradiology

1305 1307

Congenital Abnormalities of the Brain

Chiari I Malformation • • •

Figure 5-25-2

Probably due to occipital bone dysplasia and small posterior fossa Caudal displacement of “pegged” cerebellar tonsils into upper cervical spinal canal Associated with: ➢ Syringohydromyelia (25%-50%) ➢ CVJ anomalies in up to 50% ➢ Associated with many brainstem/lower cranial nerve sxs (hearing loss, vertigo, abnormal gag/swallowing, etc.)

Chiari I Malformation [Figure 5-25-2] •

• •

Tonsillar ectopia ➢ number of millimeters the tonsillar tips extend below the foramen magnum (basion to opisthion) ❖ < 3mm – Normal ❖ 3-5mm – “Low-lying” ❖ >5 mm is quoted to be 100% specific and 92% sensitive for Chiari I Difference with age ➢ tonsils regress with age ➢ between 5-15 years, even 6mm may be OK if asymptomatic Up to 50% have osseous CVJ anomalies ➢ Basiocciput hypoplasia (short clivus) ➢ Platybasia ➢ Atlanto-occipital non-segmentation ➢ Non-segmentation of C2-C3 ➢ Klippel-Feil deformity

Chiari I Malformation. Note protrusion of cerebellar tonsils (arrow) below the plane of the foramen magnum (dotted line) and cervical syringohydromyelic cavity. The fourth ventricle (dot) is normal in location and configuration

Chiari II Malformation - (Arnold Chiari Malformation) • •

Downward displacement of the cerebellar tonsils, inferior cerebellar vermis, fourth ventricle, and medulla into the upper cervical spinal canal Very commonly associated with a lumbar myelomeningocele

Chiari II Malformation - Imaging findings •

Figure 5-25-3

[Figures 5-25-3 to 5-25-5]

• • • • • • •

Luckenschadel skull-universal at birth ➢ Typically associated with Chiari II ➢ An ossification disturbance of the membraneous skull ➢ Is NOT the result of hydrocephalus ➢ Disappears by approximately 6 months of age Macrocephaly with colpocephaly Large massa intermedia Falx and tentorium hypoplasias Beaking of the tectum “Triple peak” appearance of pons/medulla Low torcula (shallow posterior fossa) Syringohydromyelia (50%)

Luckenschadel Skull-plain and CT Congenital Abnormalities of the Brain

1306 1308

Neuroradiology

Figure 5-25-4

Figure 5-25-5

A Chiari II Malformation-sagittal. Note tectal beaking, caudal displacement of cerebellar tonsils to C4, slit-like, caudally-elongated fourth ventricle, vertical straight sinus (small posterior fossa), and concavity of the clivus

Chiari III Malformation •

[Figure 5-25-6]

Herniation of cerebellum +/- brainstem, fourth ventricle, and upper cord into a low occipital or high cervical encephalocele

Figure 5-25-6

Chiari III Malformation

B

Chiari IV Malformation •

[Figure 5-25-7]

A severe cerebellar hypoplasia with essentially a CSF-filled posterior fossa • Very small brainstem—pons most severely affected Chiari IV Malformation Figure 5-25-7

C Chiari II Malformation-axial. Note tectal beaking in A (arrow); “triplepeak” appearance in B (arrows); and tentorial hypoplasia in C (arrows)

Neuroradiology

1307 1309

Congenital Abnormalities of the Brain

Disorders of Ventral Induction (Insult at 5-10 weeks) •

• •

Figure 5-25-8

Holoprosencephaly ➢ Alobar, semilobar, lobar ➢ Septo-optic dysplasia (DeMorsier’s syndrome) Cerebral hemiatrophy (Dyke-Davidoff-Masson syndrome) Posterior fossa malformations ➢ Dandy Walker malformation and variants ➢ Joubert’s syndrome ➢ Rhombencephalosynapsis

Alobar Holoprosencephaly [Figure 5-25-8] • • • • • • •

Alobar holoprosencephaly

Most severe form-no diverticulation Midline facial deformities (cycloplegia, cebocephaly, hypertelorism, hypotelorism, cleft anomalies…) Microcephaly with monoventricle (absent septum pellucidum) DDx: Hydranencephaly Horseshoe-shaped forebrain Fusion of basal ganglia and thalami Absence of: Corpus callosum, falx/interhemispheric fissure, olfactory tracts and bulbs Azygos anterior cerebral artery

Figure 5-25-9

Alobar Holoprosencephaly - DDx: Hydranencephaly •

[Figure 5-25-9]

• •

CSF replaces cerebrum supplied by the internal carotid arteries bilaterally (ICA occlusions occurring between 3 and 6 months in utero) Cerebellum, brainstem, and thalami are not involved as they are supplied by the posterior circulation Associated with: TORCH infections, maternal syphillis, abdominal trauma, radiation

Semilobar Holoprosencephaly [Figure 5-25-10] • • • • • • •

Small brain with monoventricle (absent septum pellucidum) Falx may be present Fusion of basal ganglia and thalami Corpus callosum may be absent or incomplete Some rudimentary sulcation may be present Olfactory bulbs and tracts are absent Azygos anterior cerebral artery

Hydranencephaly. Note normal, non-fused thalami and presence of falx anteriorly which distinguish this from alobar holoproencephaly

Lobar Holoprosencephaly [Figure 5-25-11] • • • • • •

LEAST severe form Normal size brain Monoventricle (absence of the septum pellucidum) Usually no facial deformities Lack of cleavage is usually subtle and frontal CORONAL IMAGING IS BEST TO DETECT

Septo-Optic Dysplasia (DeMorsier’s Syndrome) [Figure 5-25-12] • • • • •

Partial or complete absence of the septum pellucidum Hypothalamic / pituitary axis abnormalities in 66% Absent fornix and corpus callosum dysgenesis Schizencephaly in approximately 50% Diminutive optic nerves and chiasm

Congenital Abnormalities of the Brain

1308 1310

Neuroradiology

Figure 5-25-10

Figure 5-25-11

Lobar holoprosencephaly

Figure 5-25-12

Semilobar holoprosencephaly

Septo-optic dysplasia Neuroradiology

1309 1311

Congenital Abnormalities of the Brain

Cerebral Hemiatrophy (Dyke-Davidoff-Masson Syndrome) [Figure 5-25-13] • • •

Figure 5-25-13

Clinical: Hemiparesis, mental retardation, seizures Small cerebral hemisphere from in utero ischemia or infarction after infection or trauma Compensatory ipsilateral: ➢ Lateral ventricular enlargement ➢ Calvarial thickening ➢ Enlarged paranasal sinuses ➢ Enlarged mastoid air cells

Posterior Fossa Malformations •

• •

Dandy-Walker Complex ➢ Dandy-Walker malformation ➢ Dandy-Walker variant ➢ Mega cisterna magna Joubert’s syndrome Rhombencephalosynapsis

Dandy-Walker Malformation [Figure 5-25-14] • • •

• • • •

Hypoplasia of the cerebellar hemispheres, hypoplasia or aplasia of the inferior cerebellar vermis, and marked enlargement of the fourth ventricle Etiology unclear but probably NOT failure of development of fourth ventricular foranima Prominent imaging features include: ➢ Enlarged posterior fossa—macrocephaly ➢ Torcular-lambdoid inversion ➢ Absence of the falx cerebelli ➢ Hydrocephalus (91% at diagnosis) ➢ Other brain anomalies are common 66% have associated anomalies including corpus callosal hypogenesis(30%) Polymicrogyria/ heterotopia(5%-10%), Occipital cephaloceles (16%), syringohydromyelia Association with various syndromes – Fetal Alcohol, TORCH, Aicardi , Klippel Feil….. Cerebral hemiatrophy. Small left cerebral hemisphere. Note left mastoid air cells and frontal sinus are larger than right counterparts. The left middle cranial fossa is also diminutive

Figure 5-25-14

Note hydrocephalus, torcular-lambdoid inversion with large posterior fossa cyst, and severe vermian hypoplasia Congenital Abnormalities of the Brain

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Neuroradiology

Joubert’s Syndrome [Figure 5-25-15] • • • • •

Figure 5-25-15

Rare syndrome of vermian aplasia with brainstem hypoplasia Autosomal recessive inheritance Variable symptoms: Ataxia, mental retardation, pendular nystagmus,…. “Molar-tooth sign” from prominent superior cerebellar peduncles “Bat-wing” shape to fourth ventricle

Joubert’s Syndrome. Note vermian hypoplasia and the classic “molar tooth” appearance of the brainstem, best appreciated on the axial images (Courtesy M Castillo)

Rhombencephalosynapsis [Figure 5-25-16] • • • • •

Congenital fusion of cerebellar hemispheres, dentate nuclei, and superior cerebellar peduncles with vermian agenesis “Keyhole” configuration of fourth ventricle Absent septum pellucidum +/- SOD or holoprosencephaly Often hydrocephalus (aqueduct stenosis) Clinical: Variable depending upon other anomalies

Figure 5-25-16

Disorders of Neuronal Proliferation (Insult at 2-4 months) • • • • • •

Microcephaly (usually intrauterine ischemia) Generalized Megalencephaly Unilateral Megalencephaly (hemimegalencephaly) Hydranencephaly Neurocutaneous Disorders Aqueduct Anomalies (stenosis, etc.)

Unilateral Megalencephaly (Hemimegalencephaly) [Figure 5-25-17] • • • •

Clinical: Intractable siezures, mental retardation, hemiplegia, developmental delay Cerebral hemisphere may appear so anomalous as to appear unrecognizable Association with Linear Sebaceous Nevus syndrome Multiple migration and sulcation anomalies ➢ Polymicrogyria/Agyria ➢ Grey matter heterotopias

Figure 5-25-17

Rhombencephalosynapsis 2 year-old male with ataxia and developmental delay

Hemimegalencephaly. Note enlargement of the right hemisphere with diffuse cortical thickening in a pachygyric appearance with abnormal sulcation. The ipsilateral lateral ventricle is markedly enlarged Neuroradiology

1311 1313

Congenital Abnormalities of the Brain

Disorders of Neuronal Proliferation (Insult at 2-5 months) • • •

• •

Figure 5-25-18

Lissencephaly (agyria, pachygyria) Non-lissencephalic cortical dysplasias (microgyria and polymicrogyria) Schizencephaly ➢ Type I (closed-lip); Type II (open-lip) Grey matter heterotopias Callosal abnormalities ➢ Complete/partial agenesis ➢ Pericallosal lipomas

Lissencephaly [Figure 5-25-18] • • • • •

“Smooth Brain” Interruption during last phase of migration (11-26 weeks) Usually severe disabilities, developmental delay, seizures… Lissencephaly Type I (Classic) Lissencephaly Type II (Cobblestone)

Lissencephaly Type I • • •

• •

Arrested neuronal migration Also termed agyria-pachygyria complex Thick gray and thin white matter ➢ Agyria: Parietal-occipital ➢ Pachygyria (“incomplete lissencephaly”): frontal and temporal “Figure 8” or hourglass configuration Assoc with Miller-Dieker syndrome ➢ Large deletion gene on chromosome 17p13.3

Lissencephaly Type II • • •

Classic lissencephaly

Also termed “Cobblestone” lissencephaly Due to neuronal overmigration Associations include: ➢ Walker-Warburg Syndrome ➢ Fukuyama congenital muscular dystrophy

Figure 5-25-19

Non-Lissencephalic Cortical Dysplasias Microgyria/Polymicrogyria • • • •

Innumerable, small cerebral convolutions with thickened cortex and abnormal cortical histology Cortex around Sylvian fissure commonly involved DDx: Stenogyria (packed gyri) associated with the Chiari II malformation Typically associated with other migrational disorders

Schizencephaly (Cleft Brain) Type I (Closed-Lip (Fused)) [Figure 5-25-19] • • • • •

Cleft with fusion of opposing grey matter layers (no intervening CSF) extending from cortex to ventricle Fusion of ventricular ependyma and pia covering the brain (pialependymal seam) Ventricular diverticulum (“tit”) at base of cleft is very useful for identification of closed cleft May have near-normal mentation +/- seizures and spasticity; bilateral=worse prognosis 35%-50% have septo-optic dysplasia

Closed-lip schizencephaly. Note gray matter lining the cleft and a ventricular “tit” at base of the seam (arrow). Patient also has septooptic dysplasia—note absence of the septum pellucidum Congenital Abnormalities of the Brain

1312 1314

Neuroradiology

Schizencephaly Type II (Open-Lip (Separated)) [Figure 5-25-20] • • • • •

Figure 5-25-20

CSF interposed between grey matter lining the cleft IF NO GREY MATTER INTERPOSED, CONSIDER PORENCEPHALY Usually severe mental retardation, seizures, hypotonia, spasticity, blindness, inability to walk or speak Absent septum pellucidum (80%-90%) Genetic counseling is required as there is a 5%-20% incidence of brain abnormalities in siblings

Grey Matter Heterotopias [Figures 5-25-21 to 5-25-23] • • • •

Arrest of migrating neurons between ventricle and pial surface of the brain (looks like GM on all imaging sequences) Seizures and mental retardation are common Associated with schizencephaly, callosal agenesis, agyria, hemimegalencephaly… Three varieties ➢ Nodular/periventricular-subependymal (nodules of GM indent lateral ventricles; irregular ventricular walls) ➢ Focal or diffuse subcortical (clumps of GM within the WM) ➢ Band (circumferential and symmetric) ❖ Most severe form with worst prognosis Open-lip schizencephaly in two ❖ Neurons fail to reach their destination. different patients. Note absence of ❖ Confluent band of gray matter between lateral ventricle septum pellucidum in the patient with and cortex separated from both by layer of white matter bilateral schizencephaly

Figure 5-25-21

Figure 5-25-23 Nodular/periven tricular heterotopia— note signal intensity of the periventicular nodules is identical to that of cortical grey matter

Figure 5-25-22

A Neuroradiology

Band heterotopia with the classic “double cortex” sign

Bilateral subcortical nodular heterotopia (A and C-arrows) with complete callosal dysgenesis noted in B. Also note closed-lip schizencephaly in A (open arrow)

B

C 1313 1315

Congenital Abnormalities of the Brain

Callosal Dysgenesis [Figures 5-25-24 and 5-25-25] •

Figure 5-25-24

Corpus callosum forms anterior to posterior: ➢ Genu ---> Body ---> Splenium --->Rostrum Complete Agenesis ➢ Clinical: Siezures, developmental delay, microcephaly ➢ Imaging: ❖ Absent corpus callosum ❖ Elevated third ventricle ❖ Separated lateral ventricles ❖ Colpocephaly Partial Dysgenesis ➢ Acquired: Anterior CC is affected ➢ Developmental: Posterior CC is affected Lipomas ➢ Associated with ACC in 40% ➢ Located in interhemispheric fissure ➢ Often encase the pericallosal arteries

•

• •

A

Figure 5-25-25

B A

C

B Partial callosal dysgenesis: (A) The splenium is absent. (B) Note gyri separating the occipital horns in a position normally occupied by the splenium on the axial image Callosal dysgenesis (complete). Note radially arranged gyri converging toward the third ventricle in A; parallel, separated lateral venticles in B; Probst bundles (arrows); vertical orientation to hippocampi in C; and colpocephaly in D Congenital Abnormalities of the Brain

1314 1316

D Neuroradiology

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17.

Altman NR, Naidich TP, Braffman BH. Posteri or fossa malformations. AJNR Am J Neuroradiol 13:691-724, 1992 Barkovich AJ, Chung SH, Norman D. MR of neuronal migration anomalies. AJNR Am J Neuroradiol 8:10091017, 1987 Barkovich AJ, Chung SH. Unilateral megalencephaly: Correlation of MR imaging and pathologic characteristics. AJNR Am J Neuroradiol 11:523-531, 1990 Barkovich AJ, Jackson DE, Jr., Boyer RS. Band heterotopias: A newly recognized neuronal migration anomaly. AJNR Am J Neuroradiol 171:455-458, 1989 Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJNR Am J Neuroradiol 10:997-988, 1989 Barkovich AJ, Kjos BO. Grey matter heterotopias: MR characteristics and correlation with developmental and neurological manifestations. Radiology 182:493-499, 1992 Barkovich AJ, Kjos BO. Non-lissencephalic cortical dysplasia: Correlation of imaging findings with clinical deficits. AJNR Am J Neuroradiol 13:95-103, 1992 Barkovich AJ, Kjos BO. Schizencephaly: Correlation of clinical findings with MR characteristics. AJNR Am J Neuroradiol 13:85-94, 1992 Barkovich AJ, Norman D. MR imaging of schizencephaly. AJNR Am J Neuroradiol 9:297-302, 1988 Barkovich AJ, Norman D. Anomalies of the corpus callosum. AJNR Am J Neuroradiol 9:493-501, 1988 Barkovich AJ. Subcortical heterotopia: A distinct clinicoradiologic entity. AJNR Am J Neuroradiol 17:1315-1322, 1996 Byrd S, Osborn R, Bohan T, Naidich T. The CT and MR evaluation of migration disorders of the brain, II: Scizencephaly, heterotopia, and polymicrogyria. Pediatr Radiol 19:219-222, 1989 Catilo M. Bouldin TW, Scatliff JH, Suzuki K. Radiologic-pathologic correlation: Alobar holoprosencephaly. AJNR Am J Neuroradiol 14:1151-1156, 1993 Fitz CR. Holoprosencephaly and related entities. Neuroradiol 25:225-238, 1983 Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA. Cephaloceles and related malformations. AJNR Am J Neuroradiol 13:655-690, 1992 Osenbach RK, Menezes AH. Diagnosis and management of the Dandy-Walker malformation: 30 years of experience. Pediatr Neurosurg 18:179-189, 1992 Truit CL, Barkovich AJ, Shanahan R, Marlado TV. MR imaging of rhombencephalosynapsis: Report of three cases and review of the literature. AJNR Am J Neuroradiol 12:957-965, 1991

Neuroradiology

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Congenital Abnormalities of the Brain

Neuroradiology Seminar 1: Discussion of Unknown Cases Kelly K. Koeller, MD, FACR

History •

6-year-old girl with 10 days of vomiting

Pediatric Posterior Fossa Tumors • • • •

Medulloblastoma (PNET):1/3 Juvenile pilocytic astrocytoma (JPA): 1/3 Brain stem glioma: 1/6 Ependymoma: 1/6

Medulloblastoma or JPA? • • • •

Use non-contrast CT Medulloblastoma: hyperdense JPA: iso- or hypodense Most reliable imaging feature to distinguish between these tumors

Medulloblastoma • •

• • •

Most common (?) childhood brain tumor Childhood: 75% < 15 y/o, 50% < 10 y/o ➢ Peak 4-8 y/o; second peak 15-35 y/o Vermis into fourth ventricle Cysts, calcification, hemorrhage rare CT: 90-95% homogeneous, slightly hyperdense on NCCT, uniform enhancement

Medulloblastoma •

• •

MR ➢ Hypointense on T1WI ➢ Hyperintense on T2WI Cerebellopontine angle involvement rare CSF spread: 20-25% at time of diagnosis ➢ Check spine post-gad after brain MRI

Neuroradiology Seminar 1

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Neuroradiology

History •

46-year-old male with marked short-term memory loss and bizarre behavior over 9 month period

Sellar Masses: “SATCHMO”

• • • • • • •

Sellar tumor, Sarcoid Aneurysm, Arachnoid cyst Teratoma Craniopharyngioma Hypothalamic glioma, Histiocytosis, Hamartoma of tuber cinereum Meningioma Optic glioma

Craniopharyngioma • • •

Arises from squamous epithelial remnants 50% <20 y/o; second peak: middle age Location ➢ 70%: both intrasellar and suprasellar ➢ 20%: intrasellar only ➢ 10%: purely suprasellar

Craniopharyngioma •

• •

CT: “typical” = cystic with enhancing rim ➢ Partially calcified enhancing mural nodule MR: hyper or hypointense on T1WI; hyperintense on T2WI ➢ Appearance does not correlate well with chemical composition of contents Enhancement of rim: more common than in Rathke’s cleft cyst

History •

30-year-old woman (12 weeks pregnant) with headaches, nausea, and vomiting. Her obstetrician saw papilledema on fundoscopic exam and asked for an imaging study.

Neuroradiology

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Neuroradiology Seminar 1

Hemangioblastoma • • •

• • •

Young, middle-aged adults 10-20% in von Hippel-Lindau (often multiple) Cerebellar hemispheres: most common ➢ Cervical spinal cord: #2 location Cystic with mural nodule: 60% ➢ Solid 40% Calcification very rare Nodule enhances intensely

History •

62-year-old female with increasingly severe headaches

Pineal Region Masses • •

•

Germ Cell Tumors (60%) Pineal parenchymal tumors (14%) ➢ Pineocytoma ➢ Pineoblastoma Others ➢ Pineal cyst, arachnoid cyst, lipoma ➢ Vein of Galen AV fistula ➢ Glioma ➢ Meningioma

Tentorial Meningioma • • • • •

Most common primary non-glial intracranial neoplasm (16% of all brain tumors) Females > males (2:1) Multiple 6-9% Rare in children unless neurofibromatosis Arise from meningothelial arachnoid villi and possibly dural fibroblasts or pial cells

History •

35 year-old African-American male with ataxia and headaches

Neuroradiology Seminar 1

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Neuroradiology

Differential Diagnosis • • • • • •

Germinoma Lymphoma Optic nerve glioma Metastasis Tuberculosis Sarcoidosis

Sarcoidosis • • • • •

Etiology: unknown Worldwide prevalence United States: more common in African-Americans and women Peak age: 20-40 years old Multi-organ disease ➢ CNS: 5% of cases

Sarcoidosis – Clinical • • • • •

Adenopathy Skin rash Ocular abnormalities Elevated angiotensin converting enzyme Diagnosis: biopsy of skin or nodes

Sarcoidosis – Imaging •

•

4 forms ➢ Parenchymal mass ➢ Periventricular ➢ Leptomeningeal ➢ Mixed Enhances intensely ➢ Combination of parenchymal and leptomeningeal enhancement: clue to diagnosis

Sarcoidosis – Imaging • • • • •

Hyperintense on T2-weighted images Parenchymal: may mimic glioma Dural: may mimic meningioma Hydrocephalus Lesions diminish with steroid therapy

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Neuroradiology Seminar 1

Neuroradiology Seminar 2: Discussion of Unknown Cases Kelly K. Koeller, MD, FACR History •

11-year-old male with sudden onset of headache and vomiting at school, followed 5 days later by onset of left hemiplegia and weakness

Ring-enhancing Masses “MAGICAL DR”

• • • • • • • •

Metastasis Abscess Glioblastoma Multiforme Infarct (subacute) Contusion AIDS: ➢ Toxoplasmosis ➢ Lymphoma (usually immunocompromised) Demyelinating disease Resolving hematoma, Radiation necrosis

Glioblastoma Multiforme •

• •

Most common primary CNS neoplasm overall ➢ 12-15% of all primary tumors ❖ 50% of all astrocytomas ➢ Most common supratentorial neoplasm in adults Peak age: 45-70 years old; children: 10% Shorter clinical duration (usually less than 3 months)

Glioblastoma Multiforme • • •

Heterogeneous hemispheric mass with abundant vasogenic edema Most develop from pre-existing astrocytomas Subcortical white matter: frontal-temporal predilection

Neuroradiology Seminar 2

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Glioblastoma Multiforme •

•

WHO Grade IV ➢ Mitotic activity ➢ Pleomorphism ➢ Hemorrhage: common ➢ Endothelial proliferation and/or necrosis Subarachnoid seeding: 2-5%

Glioblastoma Multiforme •

• •

Heterogeneous mass ➢ Necrosis and hemorrhage common ➢ Calcification: rare Enhancement: >90% ➢ Ring-enhancement: central necrosis “Butterfly” pattern: corpus callosum extension

History •

70-year-old male with recurrent basal cell carcinoma and squamous cell carcinoma. Had radiation therapy 18 months prior to this study.

Radiation Necrosis vs. Tumor • •

Conventional MR: difficult Advanced imaging ➢ MR spectroscopy: lactate, acetate, and succinate peaks without choline peak ➢ DWI: restricted water diffusion ➢ PET/SPECT: hypometabolic

History •

42-year-old male with 2-day history of left upper extremity and shoulder weakness.

Neuroradiology

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Neuroradiology Seminar 2

Lymphoma • • • •

Increasing prevalence in immunocompromised and immunocompetent populations Dismal prognosis: some survivors at 4 years from time of diagnosis B-cell lymphoma: perivascular spaces Periventricular or leptomeningeal ➢ Primary: brain parenchyma ➢ Secondary: dura

Lymphoma • • •

Non-contrast CT: hyperdense mass Long TR images: hypointense Enhancement virtually always but may be heterogeneous

Lymphoma in AIDS • • • •

Necrosis more common than in immunocompetent hosts CT: hypodense T2WI: hyperintense Ring enhancement

Lymphoma vs. Toxoplasmosis •

•

Anti-toxoplasma therapy for 3 weeks ➢ Smaller: toxo ➢ No change: probable lymphoma PET / SPECT-Thallium ➢ Lymphoma: hypermetabolic

History • •

41-year-old male. History withheld.

Contusion •

• • •

Rough inner table of skull ➢ Anterior cranial fossa (frontal) ➢ Middle cranial fossa (temporal) “Bowl of Jell-O” model Spectrum: contusion —-> hematoma ➢ Perivascular space —-> parenchyma Variable clinical disability

Neuroradiology Seminar 2

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Neuroradiology

History •

7-year-old female with optic neuritis

Multiple Sclerosis • • • • •

Clinical diagnosis Etiology remains unknown Cooler climate predilection Children: very rare especially before puberty Optic neuritis ➢ Retrobulbar pain, central loss of vision, Marcus-Gunn pupil ➢ Strong affinity for females and MS

Multiple Sclerosis: MR • •

• • •

T1WI: Hypointense T2WI: Hyperintense ➢ Frequently shows lesions that are clinically unsuspected Active plaques enhance Chronic plaques: no enhancement MR often shows more disease than predicted clinically

Neuroradiology

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Neuroradiology Seminar 2

Pediatric Radiology

Childhood Urinary Tract Infection Ellen Chung, LTC, MC Urinary Tract Infection • • • •

Most common disorder of the urinary tract in children Second most common infection in children More common in girls after first 3-6 mo – short urethra Diagnosis – bag vs. clean-catch vs. catheter ➢ Bag specimen only valuable if negative

UTI • • • •

E. coli responsible for vast majority of cases High recurrence rate Infants and young children are less likely to have specific symptoms Lower versus upper tract infection – fever, systemic illness

Imaging Studies •

Ultrasound ➢ Infant and young child kidneys versus adult ❖ Growth ❖ Fetal lobulation ❖ Increased cortical echogenicity <1 yo ❖ Hyopechoic pyramids ➢ Hydronephrosis vs. splaying of renal sinus fat ➢ Renal size ➢ Scarring, cortical thinning ➢ Anomalies ➢ Bladder – filling-defects, diverticula, wall thickening, PVR ➢ Lateral position of ureteral orifice

Ultrasound: Limitations • • • •

US is NOT a screening exam Less sensitive than VCUG/RNC for diagnosis of VUR Less sensitive than CECT and DMSA for acute pyelonephritis Less sensitive than DMSA for renal scar

Congenital Anomalies • •

•

Figure 6-1-1

Renal agenesis Renal ectopia ➢ Simple ➢ Crossed Renal fusion ➢ Horseshoe ➢ Lump or cake

Renal Agenesis [Figure 6-1-1] • • • • •

1 in 1000 live births Ureter and ipsilateral hemitrigone are absent Medial positioning of colonic flexure Look in pelvis for ectopic kidney If observed kidney is large, it is the only functioning kidney Medial positioning of the hepatic flexure, which almost touches the spine, indicating absence of right kidney from right renal fossa. Differential includes renal agenesis, renal ectopia, or nephrectomy

Pediatric Radiology

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Childhood Urinary Tract Infection

Renal Agenesis [Figures 6-1-2 and 6-1-3] • •

Figure 6-1-2

Ipsilateral adrenal is elongated and may mimic kidney on US in newborn 25% have associated anomalies ➢ Mullerian duct duplication – ipsilateral obstruction common ➢ Seminal vesicle cyst – ipsilateral

Renal Ectopia and Fusion • • • • •

Failure to separate into two blastemas or In the absence of the kidney, the adrenal gland (arrow) is to migrate from pelvis in utero elongated, and may be mistaken for a small kidney. The Most common ectopia is pelvic finding is bilateral in this patient, who has associated Most common type of fusion is pulmonary hypoplasia with bilateral pneumothorax. Bilateral renal agenesis is incompatible with life horseshoe Anomalous kidney is often small, dysmorphic and malrotated Figure 6-1-4 Increased risk of trauma, stones, infection, renovascular hypertension and possibly tumors

Horseshoe Kidney [Figure 6-1-4] • • • • • • • •

1 in 400-600 live births Higher incidence in Turner syndrome Low position with abnormal axis Usually malrotated Midline parenchymal isthmus well seen on US in young children Multiple renal arteries and veins Ureters cross isthmus Associated with UPJO

Figure 6-1-3

Two cervices (arrows) and two widely separated uterine horns (arrow heads) associated with right renal agenesis

Renal Ectopia [Figures 6-1-5 and 6-1-6] • • • •

Most are small and dysmorphic Collecting system is superficial and renal sinus echo complex is absent or eccentric May be mistaken for a mass Blood supply from iliac arteries

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Ultrasound of the kidneys shows illdefined lower poles on the longitudinal images and a midline parenchymal connection anterior to the aorta on transverse imaging. The midline parenchymal isthmus is well seen in babies and toddlers with horseshoe kidney. The kidneys are abnormally echogenic in this patient with renal dysfunction

Pediatric Radiology

Imaging Studies •

Figure 6-1-5

VCUG ➢ Gold standard exam for reflux and urethral abnormalities ➢ Versus cystosonography ➢ Patient and parental preparation ➢ Male voiding image ➢ Early-filling and oblique Empty left renal fossa with pelvic kidney behind the bladder and directly in front of the spine. Note the abnormally small size, somewhat dysmorphic views appearance, and the eccentric renal sinus echo complex ➢ Cyclic study in young infants - more sensitive

Figure 6-1-6

Indications for VCUG • • •

• • •

Febrile UTI Abnormal ultrasound Patient < 6 yo and ➢ First UTI male or recurrent UTI female ➢ First degree relative with VUR (RNC) ➢ Solitary functioning kidney Any patient with neurogenic bladder Follow-up of patient with VUR (RNC) Post-op to confirm success

Imaging Studies •

Radionuclide cystogram ➢ Lower radiation dose ❖ Female gonads ➢ Continuous imaging ➢ Lack of spatial resolution ❖ Ureteral insertion ❖ Male urethra ❖ Grade I VUR

Crossed fused renal ectopia on excretory urogram. Note orthotopic insertions of left and right ureters

Imaging Studies •

Renal cortical scintigraphy ➢ DMSA or glucoheptonate ➢ More sensitive than ultrasound for renal scarring and pyelonephritis ➢ Indications ❖ Recurrent breakthrough infections ❖ Suspected acute pyelonephritis but equivocal laboratory or imaging findings

Imaging Studies •

CT ➢ Disadvantage of ionizing radiation and need for intravenous contrast compared with US ➢ More sensitive than US for acute pyelonephritis

Imaging Studies •

MR ➢ Gd-enhanced MR may be more sensitive than renal scintigraphy in acute pyelo ➢ Sensitive for renal scarring ➢ Less available ➢ Costly ➢ Need for sedation

Pediatric Radiology

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Childhood Urinary Tract Infection

Imaging Studies •

Figure 6-1-7

Diuretic Renography ➢ Obstructive versus nonobstructive dilatation when VCUG shows no reflux ➢ Differential function

VUR Vesicoureteric Reflux •

•

•

Primary abnormality related to length and angle of submucosal course of distal ureter Versus secondary – bladder outlet obstruction, neurogenic bladder, bladder diverticulum Up to 50% of children with UTI

Internationally standardized VCUG reflux grading system. Illustration by Heike Blum, MFA

Figure 6-1-8

Vesicoureteric Reflux • • • • •

Normal US in 75% Hydronephrosis – especially if changing Mild pelviectasis does not predict VUR Renal scar, cortical thinning, or lack of growth Urothelial thickening

VCUG Reflux Grading System [Figure 6-1-7] What to Look for in Addition to VUR • • • • •

Ureteral insertion Bladder filling defect Axis of collecting system Coexisting obstruction Intrarenal reflux

VCUG showing ureter inserting into a Hutch diverticulum. This finding is an indication for surgery

Abnormal Ureteral Insertion • •

Into or near diverticulum – secondary rather than primary VUR Ectopic insertion – too close to bladder neck

Bladder Diverticula [Figure 6-1-8] • • • •

Usually secondary to urethral obstruction and neurogenic bladder Congenital diverticula also occur – usually solitary Herniation of urothelium through defect in muscular wall of bladder Hutch diverticulum – at UVJ, associated with VUR

Figure 6-1-9

Ectopic Ureter [Figure 6-1-9] • • • • •

Lower than normal 3-4 x more frequent in females – usually upper pole of duplex system Girls may present with lifelong, day and night incontinence Left image from a VCUG showing an ectopic right In males, the ectopic ureter inserts above the ureter inserting into the urethra below the external sphincter in this girl with lifelong day and night external sphincter incontinence. Right image shows the ectopic The associated kidney may be dysplastic or atrophic, ureter is an upper pole ureter of a duplex system, especially upper pole of a duplex which is usually the case in girls with ectopic ureter; however, this is a very unusual image, because ectopic upper pole ureters do not reflux. This image was obtained because the catheter preferentially selected the upper pole ureter rather than the bladder when it was placed in the urethra

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Pediatric Radiology

Ureterocele [Figure 6-1-10] • • • • • •

Figure 6-1-10

Dilation of the distal ureter, usually caused by stenosis of the UVJ, with invagination into the bladder Ectopic vs simple In females, usually ectopic Can be so large as to cause obstruction of bladder outlet or contralateral UVJ Cobra-head or spring onion appearance With bladder filling, can evert into distal ureter and mimic a diverticulum

Abnormal Axis of Intrarenal Collecting System [Figure 6-1-11] • •

Normally a line drawn through the upper- and lowermost calcyces points to the opposite shoulder. Causes of abnormal axis ➢ Duplicated collecting system ➢ Malrotation with or without ectopia/fusion anomaly ➢ Mass, especially neuroblastoma

Ureteropelvic duplication [Figures 6-1-12 and 6-1-13] • • • • • •

Common 1 in 160 Spectrum from bifid pelvis to complete duplication 50% bilateral Complete duplication associated with increased incidence of UTI, VUR, scarring and obstruction Upper pole ureter is ectopic and may be obstructed Lower pole moiety may have VUR or UPJ obstruction

Everting ureterocele. On upper left early filling view, the ureterocele is easily identified as a round filling defect. It becomes progressively smaller the increased bladder filling until, in the lower right image, it everts into the distal ureter, mimicking a Hutch diverticulum

Figure 6-1-11

Figure 6-1-12

The kidney on the left demonstrates a normal axis, directed toward the opposite shoulder. The kidney on the right shows the axis directed toward the ipsilateral shoulder. This is the drooping lily sign of a duplex kidney

Figure 6-1-13

There is grade II VUR on the right and a straight renal axis due to duplication. There is grade V VUR on the left and a drooping lily sign. Note the filling defect in the left side of the bladder. This is due to the urine-filled ureterocele of the obstructed upper pole. The course of the lower pole ureter is unusual because it is intertwined with the dilated upper pole ureter. The upper pole ureter is not seen on the VCUG, because it is obstructed and does not reflux Pediatric Radiology

The dilated upper pole ureter and its ureterocele are seen on ultrasound 1331 1333

Childhood Urinary Tract Infection

Coexisting Obstruction [Figure 6-1-14] • • •

Figure 6-1-14

Not associated with each other, but both common The presence of contrast in upper tract due to reflux allows one to diagnose the obstruction 3 signs ➢ Hesitation ➢ Dilution ➢ Delayed drainage

Intrarenal Reflux • • •

Important factor in the pathogenesis of renal scarring More commonly occurs at the poles Indication for surgery

Vesicoureteric Reflux [Figures 6-1-15 to 6-1-17] •

•

Relationship between VUR and renal scarring controversy ➢ Can reflux of sterile urine cause scarring? ➢ Does asymptomatic bacteriuria require tx? 3 Conditions are necessary for renal scarring to occur ➢ UTI ➢ VUR ➢ Intrarenal reflux VCUG showing the 3 signs of VUR with coexisting obstruction. First, the obstruction works in both directions, so the contrast hesitates to go past the obstruction. Second, behind the obstruction is a lot of trapped, unopacified urine. When the contrast gets past the obstruction, it is diluted by the unopacified urine. Third, there is delay in the drainage of the contrast that got past the obstruction

Figure 6-1-15

Figure 6-1-16

Illustration and gross specimen of the simple papilla. In the simple, unfused papilla with its pyramidal shape, the collecting ducts empty onto the papillary surface at an oblique angle. As the surrounding calyx becomes distended with urine, these slit-like openings tend to close off

In the compound papilla, there is distortion of the surface, which is greatest where there is the most fusion. As shown in this diagram and gross specimen, at these flatter, distorted surfaces, the openings of the collecting ducts are more rounded. When the calyx gets full, these cannot close, and urine in the calyx can flow retrograde, which is intrarenal reflux

Figure 6-1-17

The blush of renal parenchyma seen in the VCUG of this patient with VUR shows the typical brush or fan like configuration of the papillary ducts and collecting tubules. This is macroscopic intrarenal reflux

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Pediatric Radiology

Acute Pyeloneprhitis • •

Figure 6-1-18

May be hematogenous, but usually an ascending infection Barriers to infection ➢ Perineal resistance – which is overcome by ❖ Vaginal reflux, labial adhesions ❖ Uncircumcised male ➢ Frequent voiding ➢ Functioning UVJ ➢ Papillary/calyceal structure – resistance to intrarenal reflux

Acute Pyeloneprhitis - Clinical •

Infants usually present with fever, but may present with nonspecific symptoms • Older children present with fever, flank pain but may call it abdominal pain Pathologic appearance of acute pyelonephritis. • If straight forward clinical picture, no imaging needed The gross image on the left shows that the surface of the involved parenchyma is pale compared to in acute setting the surrounding normal parenchyma. Note the sharp demarcation between normal and abnormal. Acute Pyelonephritis - Pathology [Figure 6-1-18] There is a patchy or lobar distribution. On the cut • Gross Path – full thickness, loss of C-M specimen on the right, the medial upper pole is normal. In the lateral upper pole and mid portion, differentiation, enlargement, sharp demarcation, the infection involves a full-thickness wedge from urothelial thickening the papilla to the surface of the kidney. The tissue • Histo – tubulointerstitial nephritis is expanded. There is disruption of the corticomedullary differentiation and a striated Acute Pyelonephritis - Imaging [Figure 6-1-19]] appearance • If fails to respond to therapy, US vs. CT to evaluate for complication • CT/US/Nuc - triangular, peripheral focus of decreased flow, decreased corticomedullary differentiation • Diffuse or focal enlargement – can appear mass-like • VCUG indicated – may perform while hospitalized

Figure 6-1-19

US demonstrating focal enlargement with decrease flow mimicking a mass. The more sensitive enhanced CT shows more diffuse triangular and striated areas of decreased enhancement typical of acute pyelonephritis

Complicated Upper Tract Infection • •

Renal or perinephric abscess Pyonephrosis – must be treated urgently

Reflux Nephropathy • • • •

Renal scarring – chronic pyelonephritis – post-infectious nephropathy Related to bacterial infection, VUR, and intrarenal reflux Usually at poles – especially upper Scarring can be prevented or limited if early diagnosis of upper tract infection

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Childhood Urinary Tract Infection

Reflux Nephropathy [Figure 6-1-20] • • • • • •

Figure 6-1-20

Destruction centered in medulla Full thickness - indentation overlying dilated calyx (versus fetal lobulation) Echogenic focal scar Small kidney, no growth Compensatory hypertrophy between scars – can appear masslike Almost all cases of severe scarring have VUR

Vesicoureteric Reflux - Treatment • • • •

Medical with annual follow-up US, RNC and urine culture Surgical reimplantation Endoscopic subureteric injection Surgery is considered for ➢ Failure to resolve or worsening ➢ Scarring or growth failure ➢ High grade VUR ➢ Intrarenal reflux ➢ Frequent breakthrough infections

Upper pole cortical thinning due to reflux nephropathy. Note that the upper pole calyx extends almost to Neurogenic Bladder the interface between the kidney and • Failure of detrusor muscle and internal and external sphincters to the liver function in concert to hold and release urine • Emptying or filling phase dysfunction • Causes – spinal dysraphysm, caudal regression, paraplegia, presacral teratoma, anterior sacral meningocele • Suspect in patients with recurrent UTI and constipation • Lower motor neuron lesion – large, smooth-walled bladder • Upper motor neuron lesion – small, trabeculated bladder

Secondary VUR

Neurogenic Bladder - Imaging • • • • •

Trabeculated, thick-walled or large and atonic Taller than wide Funnel-shaped bladder neck Large post-void residual Look for spinal dysraphism

Bladder Augmentation • • • •

Used to treat small, noncompliant bladders If small bowel is used, gut signature and peristalsis are seen on US Complication of bladder rupture Alternative - autologous bladder cells grown in tissue culture

Antenatal Pelvicaliectasis • •

Prevalence of prenatal sonography has changed the natural history of some causes of neonatal hydronephrosis Common causes ➢ VUR ➢ UPJO ➢ Obstructed upper pole of duplex system ➢ PUV

Antenatal Pelvicaliectasis - Work-Up • • • • •

Perform postnatal ultrasound after DOL 4 or 5 VCUG if mod-severe hydro In male with bilateral severe hydro, perform VCUG before discharge Repeat at 6 weeks if normal or mild If VCUG negative, perform diuretic renography or excretory urogram

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Posterior Urethral Valves • • •

Figure 6-1-21

Most common cause of urethral obstruction in the male infant Usually present in infancy (first UTI male) or prenatally diagnosed Less commonly delayed presentation with failure to thrive, incontinence, or hypertension

Posterior Urethral Valves - Imaging [Figures 6-1-21 and 6-1-22] •

• • •

3 types ➢ Type 1 - folds attach below the veru montanum ➢ Type 2 – folds attach above the veru ➢ Type 3 – diaphragm with central opening Trabeculated or thick-walled bladder Dilated posterior urethra – perineal window US Bilateral hydronephrosis – not a constant finding but bilateral hydro in a male infant is PUV until proven otherwise

Posterior Urethral Valves [Figure 6-1-23] • • •

•

In utero obstruction causes renal dysplasia (dysgenesis) Prognosis related to degree of renal dysplasia Factors that protect one or both kidneys ➢ Large bladder ➢ Bladder or calyceal diverticula ➢ Unilateral VUR or no VUR (50%) ➢ Urinary ascites No VUR ◗ “valve bladder”

Figure 6-1-22

Figure 6-1-23

Same patient showing posterior urethral valve and dilated posterior urethra

Ultrasound of a patient with PUV showing irregular, thickened bladder wall, patulous UVJ, and hydronephrosis. Note also the cysts and poor corticomedullary differentiation due to associated renal dysplasia

Prune Belly Syndrome • •

•

Trabeculated bladder in 1 month-old male with bilateral prenatal hydronephrosis. Note the irregularity of the wall despite the bladder being full

AKA Eagle-Barrett or Triad syndrome Triad ➢ Hypoplastic or absent abdominal wall musculature ➢ Cryptorchidism ➢ Urinary tract anomalies Almost exclusively males

Prune Belly Syndrome – 2 Types •

Severe – ➢ Complete urethral obstruction (bladder like PUV) ➢ Renal dysplasia and pulmonary hypoplasia ➢ Associated anomalies of GI tract, genital tract, CHD ➢ Death in first year of life

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•

Mild-Moderate ➢ Functional abnormality of bladder emptying – floppy, dilated bladder ➢ Urachal remnant ➢ Mild to markedly dilated renal pelvis and ureters ➢ Ureters mimic small intestine ➢ +/- pulmonary hypoplasia ➢ Long-term survival

Figure 6-1-24

Prune Belly Syndrome [Figures 6-1-24 and 6-1-25] • •

Dilated ureter, bladder and urethra with patchy foci of Young infant with Eagle-Barrett deficient musculature Syndrome. Note dilated flaccid bladder, reflux into tortuous ureters Other findings – dilated posterior urethra, megalourethra, urethral diverticula, dilated prostatic with the appearance of small bowel loops, urachal remnant at dome of utricle bladder and dilated posterior urethra without valve

Other Causes of Hydronephrosis

Ureteropelvic Junction Obstruction • • • • •

Most common cause of upper tract obstruction Previously presented with flank pain, mass, UTI or hematuria with mild trauma Now commonly prenatally diagnosed Dilated pelvis and calices – no dilated ureter Ddx: extrarenal pelvis

Figure 6-1-25

UPJO •

Associations ➢ Increased risk of abnormality of contralateral kidney – most common is UPJO ➢ Renal dysplasia ➢ VUR ➢ UVJ obstruction ➢ Lower pole moiety of duplex kidney ➢ Horseshoe kidney

UPJO - Treatment • • • •

Mild – mod obstruction is followed and treated if it worsens Severe obstruction in young children is treated with dysmembered pyeloplasty In adults and adolescents alternative treatment is endopyelotomy Often remain dilated after repair

Figure 6-1-26

UPJO [Figures 6-1-26 and 6-1-27] •

Radiograph of same patient showing flaccid abdominal wall musculature. Also the infant is intubated with small lungs, bell-shaped thorax and medial right pneumothorax due to associated pulmonary hypoplasia

Intrinsic versus extrinsic ➢ Intrinsic – fibrosis, stricture, valve/fold, etc ➢ Extrinsic – crossing vessel ❖ Intermittent symptoms and findings ❖ Dietl’s crisis ➢ Prenatally diagnosed – 10-15% extrinsic ➢ Diagnosed due to symptoms - 50% extrinsic CT of patient with UPJO obtained when the patient presented with severe abdominal pain and peritoneal signs following a MVC. Note the dilated pelvis and calyces, and the urine in the retroperitoneum due to UPJ rupture. Note also the large crossing vein that was found to be the cause of the UPJO at surgery. Prior to this accident the patient previously complained of chronic abdominal pain and was treated for lactose intolerance and had an appendectomy for a normal appendix

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Primary Megaureter [Figure 6-1-28] • • • • • •

Figure 6-1-27

Functional obstruction analogous to Hirschprung disease of the GI tract Short aperistaltic segment of distal ureter Proximal ureter dilates – aperistaltic segment fixed and narrowed More common in boys and on the left Predisposed to UTI and stone formation Surgically repaired only if severe or symptomatic

Congenital Megacalyces • • • •

Nonobstructive enlargement of calyces and hypoplasia of the medullary pyramids Benign nonprogressive condition Can coexist with megaureter Occasional stone formation, hematuria

Complications of endopyelotomy performed in a patient with UPJO due to crossing vessel. The upper left image shows the origin of the left main • Large bladder without obstruction • Large ureteral orifices with free reflux and voiding into renal artery. The lower right image shows the origin of an accessory renal artery to the lower markedly dilated ureters pole, which on the lower left image is seen to cross the stent in the ureter (arrow). This artery Summary was cut in the procedure causing the perirenal hematoma seen well in the lower two images. • VUR may be primary or secondary to bladder outlet obstruction/ neurogenic bladder or abnormal ureteral Note also the infarction of the anterior lower pole in the upper right image, due to spasm of the cut insertion artery • Primary VUR is familial and resolves by age 6, but secondary VUR requires surgical intervention • In primary VUR the VCUG appears normal except for reflux Figure 6-1-28 • US is insensitive and is not a screening exam • On US, look for scarring and lack of growth • RNC best for sibling screen, girls, and follow-up • VCUG best for symptomatic patients and boys • Surgical options are surgical reimplantation or subureteric injection • Bilateral hydro in an infant male is due to PUV until proven otherwise • Not all boys with valves have reflux and hydronephrosis • In utero obstruction causes renal dysplasia • UPJO is associated with abnormality of Primary megaureter. Excretory urogram shows dilation of the other kidney left ureter proximal to a short, fixed, relatively narrow segment • UPJO diagnosed in utero or on of ureter near the UVJ screening US is caused by intrinsic abnormality in 85% of patients • UPJO diagnosed due to symptoms is caused by extrinsic compression (crossing vessel) in 50% of patients • Extrinsic compression causes transient sx and findings • Endopyelotomy is contraindicated in patients with crossing vessels

Congenital Megacystis-Megaureter

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References Texts 1. Kirks DR, ed. Practical Pediatric Imaging, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998. 2. Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002. 3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams & Wilkins, 2004. Journal Articles 1. American Academy of Pediatrics Committee on Quality Improvement Subcomittee on Urinary Tract Infection. Practice parameter: the diagnosis, treatment and evaluation of the initial urinary tract infection in febrile infant and young children. Pediatrics 1999;103:842-852. 2. Berrocal T, Gaya F, Arjonilla A. Vesicoureteral reflux: diagnosis and grading with echo-enhanced cystosonography versus voiding cystourethrography. Radiology 2001;221:359-365. 3. Blane CE, DiPietro MA, Strouse PJ, et al. Pediatric renal pelvic fullness: an ultrasonographic dilemma. J Urol 2003;170:201-203. 4. Blane CE, DiPietro MA, Zerin JM, et al. Renal sonography is not a reliable screening examination for vesicoureteral reflux. J Urol 1993;150:752-755. 5. Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the era of sonography. AJR Am J Roentgenol 1987;148:959-963 6. Daneman A, Alton DJ. Radiographic manifestations of renal anomalies. Radiol Clin North Am 1991;29:351-363. 7. Davey MS, Zerin JM, Reilly C, et al. Mild renal pelvic dilation is not predictive of vesicoureteral reflux in children. Pediatr Radiol 1997;27:908-911. 8. Donnelly LF, Gylys-Morin VM, Wacksman J. Unilateral vesicoureteral reflux: association with protected renal function in patients with posterior urethral valves. AJR Am J Roentgenol 1997;168:823-836. 9. Eggli KD, Eggli D. Color Doppler sonography in pyelonephritis. Pediatr Radiol 1992;22:422-425. 10. Elder JS, Peters CA, Arant BS Jr, et al. Pediatric vesicoureteral reflux guidelines panel summary report of primary vesicoureteral reflux in children. J Urol 1997;157:1846-1851 11. Fernbach SK, Feinstein KA, Schmidt MB. Pediatric voiding cystourethrography: a pictoral guide. RadioGraphics 2000;20:155-168. 12. Gross GW, Lebowitz RL. Infection does not cause reflux. AJR Am J Roentgenol 1981;137:929. 13. Hoffer FA, Lebowitz RL. Intermittent hydronephrosis: a unique feature of ureteropelvic junction obstruction caused by a crossing renal vessel. Radiology 1985;156:655-658. 14. Lavocat MP, Granjon D, Allard D, et al. Imaging of pyelonephritis. Pediatr Radiol 1997;27:159-165. 15. Lebowitz RL, Blickman JG. The coexistence of ureteropelvic junction obstruction and reflux AJR Am J Roentgenol 1983;140:231-238. 16. Lebowitz RL, Olbing H, Parkkulainen KV, et al. International system of radiographic grading of vesicoureteral reflux. International Reflux Study in Children. Pediatr Radiol 1985;15:105-109. 17. Lonergan GJ, Pennington DJ, Morrison JC, et al. Childhood pyelonephritis: comparison of Gadolinium- enhanced MR imaging and renal cortical scintigraphy for diagnosis. Radiology 1998; 207:377-384. 18. Mentzel JJ, Vogt S, Patzer L, et al. Contrast enhanced sonography of vesicoureterorenal reflux in children: preliminary results. AJR Am J Roentgenol 1999;173:737-740. 19. Orellana P, Baquedano P, Rangarajan V. Relationship between acute pyelonephritis, renal scarring and vesicoureteral reflux. Results of a coordinated research project. Pediatr Nephrol 2004;19:1122-1126. 20. Paltiel HJ, Mulkern RV, Perez-Atayde A. Effect of chronic low-pressure sterile vesicoureteric reflux on renal growth and function in a porcine model: a radiologic and pathologic study. Radiology 2000;217:507-515. 21. Paltiel HJ, Rupich RC, Kiruluta HG. Enhanced detection of vesicoureteral reflux in infants and children with use of cyclic voiding cystourethrography. Radiology 1992;184:753-755. 22. Rooks VJ, Lebowitz RL. Extrinsic ureteropelvic junction obstruction from a crossing renal vessel: demography and imaging. Pediatr Radiol 2001;31:120-124. 23. Sargent MA. What is the normal prevalence of VUR? Pediatr Radiol 2000; 30:87-593. 24. Van den Abbeele AD, Treves ST, Lebowitz RL, et al. Vesicoureteral reflux in asymptomatic siblings of patients with known reflux: radionuclide cystography. Pediatrics 1997;79:147-153. 25. Walsh G, Dubbins PA. Antenatal renal pelvis dilatation: a predictor of vesicoureteral reflux? AJR Am J Roentgenol 1996;167:887-890.

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Neonatal GI Tract Obstruction Ellen Chung, LTC, MC Figure 6-2-1

Objectives •

•

To become familiar with the diagnosis of the major causes of proximal and distal GI obstruction in the neonatal period (first 24 to 48 hours) To develop an approach to the evaluation of the obstructed neonate including a method of performing diagnostic and therapeutic enema

Neonatal Intestinal Obstruction - General Principles • • • • •

Any obstruction distal to the ampulla of Vater causes biliary emesis Perforation may be present without free air Loop immediately proximal to atresia often disproportionately dilated Start evaluation with plain radiographs Small and large bowel cannot be differentiated on plain film

Neonatal Intestinal Obstruction •

•

High ➢ Proximal to mid ileum ➢ Few dilated loops ➢ UGI or no further imaging Low ➢ Distal ileum, colon ➢ Many dilated loops ➢ Contrast enema

5 types of esophageal atresia/distal fistula

ESOPHAGUS

Figure 6-2-2

Esophageal Atresia • • •

•

Error in differentiation of the foregut into trachea and esophagus Spectrum from esophagotrachea to H-type fistula without esophageal atresia Presentation – feeding intolerance, regurgitation, choking, aspiration, increased oral secretions, symptoms in first 24h of life in 85 - 95% Half have other anomalies - VACTERL

Esophageal Atresia [Figures 6-2-1 and 6-2-2] • • • • • •

Junction of upper and middle 1/3’s Fistula 0.5 to 1.0 cm above carina in 89% 5 types Length of the gap - longest without fistula Atresia with distal fistula is most common H-type – present later due to chronic aspiration, actually Nshaped

Most common type is esophageal atresia with distal fistula

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Esophageal Atresia - Imaging [Figures 6-2-3 and 6-2-4] •

•

Esophageal Atresia - Imaging • • •

Figure 6-2-3

Prenatal ➢ Polyhydramnios ➢ +/- no stomach bubble ➢ +/- dilated fluid-filled proximal pouch Neonatal ➢ Anterior tracheal displacement on lateral CXR ➢ Distal bowel gas (85%) ➢ Gasless (8%) ➢ Dilated air-filled pouch ➢ OG tube coiled in pouch ➢ UGI usually not indicated – use air

Cross-sectional imaging: 3D CT, virtual bronchoscopy Document side of arch for surgical planning Ddx: pharyngeal perforation – pneumomediastinum, pleural effusion

EA/distal TEF. AP radiograph shows dilated, airfilled proximal pouch. Lateral view shows NG coiling in pouch and anterior displacement of the trachea

Esophageal Atresia – Post-op •

•

Complications ➢ Anastomotic leak ➢ Anastomotic stricture ➢ Recurrent fistula ➢ Reflux esophagitis/stricture Expected findings ➢ Disordered motility below anastomosis ➢ GER ➢ Tracheomalacia

Figure 6-2-4

VACTERL • • • • • •

Non-random association of anomalies No one patient has all High perinatal mortality > 60% 46% of patients with TEF Recurrence risk (offspring) 2-3% Recurrence risk (siblings) ➢ TEF or EA < 1% ➢ Other VACTERL lesions 1.2%

Ddx: Pharyngeal Perforation • • •

Traumatic delivery Traumatic intubation Nasogastric tube placement

STOMACH Gastric Atresia/Antral Web • • •

Very rare Atresia presents near birth Web is usually perforated so present in childhood with recurrent nonbilious emesis

Esophagram showing H-type fistula with filling of tracheobronchial tree

Microgastria • • • •

Rare Isolated vs. associated anomalies, especially asplenia Small tubular midline stomach and dilated distal esophagus Duodenal bulb may also dilate

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DUODENUM

Figure 6-2-5

Normal Rotation of Midgut Loop [Figure 6-2-5] • • • •

GI tract straight short tube Midgut divided by SMA 270 degrees counterclockwise rotation 6th week Normal mesentery is broad based

Malrotation/Malfixation - Pathogenesis [Figures 6-2-6 and 6-2-7]

• • •

All types referred to as malrotation Malfixation - short root of mesentery predisposes to midgut volvulus Ladd bands – ➢ Attempts to secure the bowel ➢ Most common site is from high medial cecum across 2nd-3rd portion of duodenum to porta hepatis ➢ May cause obstruction

Figure 6-2-6

Normal intestinal rotation and fixation showing long root of the mesentery (line)

Figure 6-2-7

Illustrations showing malrotation and midgut volvulus (center)

Midgut Volvulus - Presentation • • •

Prenatal ➢ Necrosis of bowel and multiple atresias First month of life – most patients ➢ Bilious emesis, occasional bloody stool Older child ➢ Chronic recurrent abdominal pain, failure to thrive, diarrhea, malabsorption ➢ Volvulus can occur at any age

Most common location of Ladd band

Figure 6-2-8

Midgut Volvulus - Pathology [Figure 6-2-8] • •

Volvulus impedes venous and lymphatic return leading to bowel wall edema If prolonged, arterial obstruction and bowel infarction

Malrotation – Plain Radiograph [Figure 6-2-9] • • • • • •

Evaluation of emesis/obstruction begins with plain film Classic - partial obstruction of duodenum 2nd -3rd portion --> malro until proved otherwise May mimic gastric outlet obstruction Ileus or distal SBO Gasless abdomen Normal Chronic midgut volvulus

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Malrotation – UGI • • • •

Figure 6-2-9

Duodenal jejunal junction – diagnostic Normal position on AP is on or to the left of the left pedicle of L1 Normal position on lateral is posterior/retroperitoneal AP must be perfectly positioned

Malrotation – UGI [Figures 6-2-10 and 6-2-11] • • •

Jejunum in RUQ DJJ may be displaced by distended bowel, masses and enlarged organs If DJJ is equivocal, empty stomach with NG tube or complete small bowel follow-through

Figure 6-2-10

Plain radiograph of a newborn with bilious emesis. High small bowel obstruction is noted, with all air-filled loops on the right

Normal position of the DJJ on frontal view (left image) on or to the left of the left pedicle of L1. Lateral view from an UGI (right image) showing the normal retroperitoneal location of the duodenum

Figure 6-2-11

Midgut Volvulus – UGI [Figure 6-2-12] • • •

Duodenal obstruction Beak Corkscrew appearance of duodenum and jejunum

Malrotation – Contrast Enema • • • • • •

No longer part of work-up Cecum and DJJ rotate independently Cecum normal in 20% of patients with malrotation so enema is no longer used in the work-up High mobile cecum is a common normal variant – 15% Entire colon may be to left of midline in malrotation More often cecum is high and medial Malrotation. Duodenal jejunal junction low and to the right of midline

Figure 6-2-12

Lateral view showing obstruction of the 2nd – 3rd portion of the duodenum and corkscrew appearance of the jejunum diagnostic of malrotation Neonatal GI Tract Obstruction

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Malrotation – Ultrasound/CT [Figures 6-2-13 and 6-2-14] • • • • • •

Figure 6-2-13

Dilated proximal bowel, wall thickening, ascites Inversion of SMA/SMV relationship1 Normally the SMV is to the right of the SMA 33% surgically proven malrotation have normal relationship 8 of 9 with SMA/SMV inversion had malrotation Below the portal confluence, look for the swirl sign

1Zerin JM, DiPietro MA. Superior mesenteric vascular anatomy as

ultrasound in patients with surgically proved malrotation of the midgut. Radiology 1992; 183:693-4 Ultrasound images showing inversion of the SMV/SMA relationship. The Figure 6-2-14 SMV is usually larger and the SMA is usually surrounded by echogenic fat, but he identity of each vessel must be confirmed with Doppler or by connecting SMV to the portal vein

Midgut volvulus in 4 yo with acute onset of emesis. Inversion of SMA/SMV relationship (lower image) and swirl sign (upper image) of volvulus. SMV (arrow) is to the left of the SMA

Malrotation - Treatment •

Ladd procedure ➢ Reduce midgut volvulus ➢ Lyse bands ➢ Place in orientation of nonrotation – all small bowel on the right and all colon on the left ➢ Inversion appendectomy ➢ 95% have no recurrence

Malrotation – Associated Congenital Anomalies • • • • •

Omphalocele Gastroschisis Diaphragmatic hernia Bowel atresia/stenosis Heterotaxy – not surgically repaired

Duodenal Atresia/Stenosis/Web • • • •

Atresia much more common than stenosis 3-6 weeks gestation – failure of canalization of solid tube of foregut Annular pancreas in 20% May have preduodenal portal vein

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Duodenal Atresia - Clinical • • •

Figure 6-2-15

30% have Down’s syndrome Associated with other atresias, biliary anomalies, CHD, VACTERL Almost always just distal to ampulla of Vater --> bilious emesis

Duodenal Atresia - Imaging [Figures 6-2-15 to 6-2-18] • • • • •

Polyhydramnios Double bubble Windsock deformity How do we know it is not midgut volvulus? Dilation of duodenal bulb indicates it is a chronic condition Double bubble is diagnostic, but if obstruction is not complete, an UGI is indicated

Figure 6-2-16

Double bubble is diagnostic of duodenal atresia. vein

Figure 6-2-17

Prenatal ultrasound for polyhydramnios shows fluid-filled double bubble. This finding should prompt chromosomal analysis for possible Trisomy 21

Figure 6-2-18

Annular pancreas. Upper GI shows circumferential narrowing of second portion of duodenum

Caution: Incomplete obstruction mandates an UGI. Double bubble appearance with distal gas caused by midgut volvulus with beak appearance on upper GI Neonatal GI Tract Obstruction

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JEJUNUM

Figure 6-2-19

Jejunal Atresia and Stenosis • •

Atresia more common than stenosis Ischemic injury to developing gut ➢ Primary vascular accident – more common ➢ Secondary to mechanical obstruction, e.g. in utero volvulus

Jejunal Atresia - Presentation • • •

Bilious emesis first hours of life Distended epigastrium with scaphoid lower abdomen +/- failure to pass meconium

Intraoperative photograph demonstrating jejunal atresia

Atresia/Stenosis - Pathology [Figure 6-2-19] • • • •

Most common sites are proximal jejunum and distal ileum 20% multiple 5 types Two types inherited ➢ Apple-peel (Christmas tree) ➢ Syndrome of multiple intestinal atresias and intraluminal calcification

Figure 6-2-20

Jejunal Atresia - Imaging [Figure 6-2-20] • • • • •

Triple bubble Dilated loop of bowel proximal to atresia is disproportionately dilated with bulbous end Proximal bowel may be fluid-filled and mimic a mass UGI is rarely indicated Surgeon may request BE to evaluate for additional distal atresia

Proximal Neonatal Intestinal Obstruction - Ddx • • • • • •

Esophageal atresia Gastric atresia/web Malrotation/midgut volvulus Duodenal atresia Jejunal atresia All surgical – UGI only to determine who needs to go emergently

Low Intestinal Obstruction • •

•

Plain film ➢ Multiple dilated loops of bowel Contrast enema ➢ Dilute ionic water-soluble contrast (cystography) ➢ Low osmolar nonionic contrast Microcolon ➢ Unused colon of small caliber, <1 cm

ILEUM

Triple bubble sign of jejunal atresia. Upper GI is not necessary

Figure 6-2-21

Ileal Atresia [Figure 6-2-21] • • •

Primary vascular accident or secondary to mechanical obstruction (in utero volvulus) Plain film - low obstruction Contrast enema – ➢ Microcolon ➢ Abrupt cut off of contrast column at atresia ➢ No filling of dilated ileum

Plain radiograph (left) shows a large number of dilated tubular loops of bowel. Contrast enema (right) reveals a microcolon Pediatric Radiology

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Meconium Peritonitis [Figures 6-2-22 and 6-2-23] • • • • •

Figure 6-2-23

In utero bowel perforation with leakage of meconium Linear, clumps or at periphery of pseudocyst, or generalized Scrotal Perforation may have sealed Intraluminal – severe stasis, anorectal or cloacal malformation

Figure 6-2-22

Newborn with scrotal swelling due to calcifications in both scrotal sacs from meconium peritonitis

Figure 6-2-24

Generalized meconium peritonitis. Note calcifications around liver and spleen. Perforation was due to ileal atresia

Meconium Ileus [Figure 6-2-24] • • •

Inspissated abnormal meconium in the distal ileum and colon Almost all have cystic fibrosis - presenting feature of CF in 5-20% 50% complicated - volvulus, perforation, atresia or peritonitis

Meconium ileus. Illustration of pellets of inspissated meconium causing distal small bowel obstruction. Intraoperative photograph shows thick, viscous meconium in dilated small bowel with microcolon distal to the obstruction

Meconium Ileus – Plain Film [Figure 6-2-25] •

•

Classic ➢ Distal obstruction ➢ Bubbly appearance in RLQ, ➢ Variation in caliber of loops ➢ Paucity of air-fluid levels Complicated ➢ Peritoneal calcifications ➢ Bowel wall edema ➢ +/- free air

Figure 6-2-25

Meconium Ileus - Contrast Enema [Figure 6-2-26] • • • •

Microcolon (smallest) Distal 10 - 30 cm of ileum small in caliber but still larger than colon Multiple round filling defects Contrast eventually fills dilated ileum proximal to obstruction

Meconium ileus. Supine radiograph shows many dilated, unfolded loops of bowel and classic soap-bubble lucencies in the right lower quadrant (arrow) Neonatal GI Tract Obstruction

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Meconium Ileus - Treatment • •

•

Figure 6-2-26

1968 Noblett – nonoperative therapy - Gastrograffin enema Treatment enema - half-strength Gastrograffin or full urographic contrast ➢ 1-2 enemas per day - several attempts ➢ 50-80% success rate ➢ <2% perforation rate Hydrostatic reduction failure =>complicated meconium ileus => surgical intervention

Berdon Syndrome • • • • • • • •

Megacystis-microcolon-intestinal hypoperistalsis syndrome Functional small bowel obstruction Malrotation common Transient microcolon and dilated SB loops Large unobstructed bladder, also dilated ureters and pelvicalyceal system 4:1 F:M Associated GU and cardiac anomalies Poor prognosis for long term survival

COLON/RECTUM Colonic Atresia • • • • •

Meconium ileus. Contrast enema shows a microcolon and pellet-like filling defects in the distal small bowel

Figure 6-2-27

Rare (stenosis even rarer) Diaphragm, web, fibrous cord, gap Intrauterine vascular accident Plain film - disproportionately dilated, bulbous loop of proximal colon Contrast enema - microcolon distal to atresia

Functional Immaturity of the Colon • • • •

1956 Clatworthy - “meconium plug syndrome” 1974 Davis -“small left colon syndrome” 1975 Lequesne and Reilly – small left colon could occur with or without meconium plug 1977 Berdon - "functional immaturity” – above are overlapping entities in a spectrum of functional neonatal intestinal obstruction

Functional Immaturity of the Colon • • •

Abnormal intestinal motility in the left colon May have a meconium plug – effect rather than cause of obstruction Risk factors ➢ Infant of diabetic mother ➢ Mother treated with magnesium sulfate

Functional immaturity of the colon. Contrast enema showing abrupt transition to small caliber left colon at the splenic flexure

Figure 6-2-28

Functional Immaturity - Contrast Enema [Figures 6-2-27 and 6-2-28]

• • • •

Narrow descending and rectosigmoid colon with abrupt transition to distended colon at splenic flexure Passage of plug classic but not common Passage of lots of meconium - nonspecific Clinical improvement after enema in hours or days

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Differentiation from Hirschprung Disease Functional Immaturity Location of Always at splenic transition zone flexure Quality of transition Abrupt zone Caliber of left colon Small

Hirschprung Disease Uncommon at splenic flexure Gradual

Distensibility of rectum

Non distensible

Distensible

Normal

Figure 6-2-29

Hirschprung Disease • • • •

Functional obstruction of colon due to absence of intramural ganglion cells of myenteric plexus Failure of distal intestine to relax Etiology - ? arrest of craniocaudal migration of neuroblasts in the distal colon in 12th week 1 in 5000 live births

Hirschsprung Disease - Presentation • • • • • •

80% present in 1st 6 weeks of life Term newborns Boys:Girls 3-4:1 for short segment Equal sex distribution for total colonic 1/3 develop NEC-like enterocolitis in first month of life May present with neonatal appendicitis

Hirschprung - Location • • •

•

Hirschprung disease. Gross specimen in center shows transition zone with narrow distal sigmoid and rectum. Histology specimen from aganglionic segment (left) shows hypertrophied neural bundles. Histology specimen from proximal colon shows normal ganglion cells

Ultrashort segment – we don’t see Short segment – rectosigmoid (73%) Intermediate - long segment ➢ Descending colon (14%) ➢ Proximal colon (10%) Total colonic – familial (1-3%)

Figure 6-2-30

Hirschprung - Pathology [Figure 6-2-29] •

Superficial suction biopsy ➢ Absent ganglion cells ➢ Hypertrophied submucosal nerve bundles

Hirschsprung – Plain Films [Figure 6-2-30] • • • • •

Distal obstruction Paucity of rectal gas Prone cross table lateral may show transition zone Pneumatosis of bowel proximal to aganglionic segment possible 5% present with pneumoperitoneum – usually total colonic

Hirschsprung – Contrast Enema [Figure 6-2-31] • • • • • • • •

Hirschprung disease. Plain No balloon catheters in the rectum radiograph shows distal bowel Lateral view best obstruction and paucity of gas in rectum Only enough contrast to diagnose Low rectosigmoid index (normally > 1) Irregular contractions of aganglionic segment Early evacuation film may help 24 hour delayed film – retention and lack of movement to the left, but not specific Initial enema may be normal in neonates

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Total Colonic Aganglionosis [Figure 6-2-32] • • • • •

Figure 6-2-31

Microcolon Normal caliber colon Dilated colon Small bowel transition zone Family history

Associated Anomalies • • • • •

3%-5% Down syndrome Esophageal dysmotility syndromes Malrotation Ileal and colonic atresia Neurocristopathies ➢ Neuroblastoma ➢ Ondine curse – central hypoventilation and congenital neuroblastoma

Hirschprung disease. Lateral image from contrast enema showing abnormally low recto sigmoid ratio and saw tooth contractions in aganglionic segment

Imperforate Anus • •

• •

Anorectal malformation – abnormal separation of GU tract from hindgut High vs. low - levator sling development determines surgical approach and prognosis Low – fistula to perineum High – fistula to ➢ Boys – post urethra, calcified meconium, air in bladder ➢ Girls – vagina or vestibule

Figure 6-2-32

Imperforate Anus [Figure 6-2-33] •

•

Associated anomalies ➢ VACTERL ➢ GU ➢ L-S spine ➢ Dysraphism ➢ Tethered cord ➢ Currarino triad – imperforate anus, sacral defect, presacral mass Post-op incontinence => MRI

Twin boys with total aganglionosis, twin (A) manifesting a microcolon; twin (B) with normal colon caliber & small bowel transition zone (arrow)

Figure 6-2-33

Neonatal Low Intestinal Obstruction Ddx • • • • • •

Ileal atresia Meconium ileus Colonic atresia Functional immaturity of the colon Hirschprung disease Imperforate anus

Neonatal Bowel Obstruction Always remember, and please don’t ever forget . . . • • • • • •

Start with plain film All complete high obstructions are surgical. Usually no further imaging required. UGI for incomplete high obstruction or normal film Contrast enema for low obstruction Differentiates surgical from medical causes Therapeutic in medical cases Sacral defect associated with history of imperforate anus

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References 1. 2. 3. 4. 5. 6.

Buonomo C. Neonatal Gastrointestinal Emergencies. Radiologic Clinics of North America 1997;35: 845-864 Cohen MD. Choosing Contrast Media for the Evaluation of the Gastrointestinal Tract of Neonates and Infants. Radiology 1987;162:447-56 Kao SC, et al. Nonoperative treatment of simple meconium ileus: a survey of the Society for Pediatric Radiology. Pediatr Radiol 1995;25: 97-100 Kirks DR, et al. Practical Pediatric Imaging. 1998 Kirks DR. Emergency Pediatric Radiology. American Roentgen Ray Society. 95th Annual Meeting April, 1995 Long FR, Kramer SS, Markowitz RI, Taylor GE. Radiographic patterns of intestinal malrotation in children. RadioGraphics 1996;16:547-556.

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Acute GI Disorders of Infants and Children Ellen Chung, LTC, MC Necrotizing Enterocolitis – Pathogenesis • • •

Hypoxemia ◗ ischemic bowel ◗ bacterial invasion Feeding plays a role – hyperosmolar, early Risk factors ➢ Prematurity ➢ Term with CHD ➢ Hirschprung disease ➢ UAC

Figure 6-3-1

NEC – Clinical Features [Figure 6-3-1] • • • • • • •

Onset 3-6 DOL Abdominal distension Vomiting Metabolic acidosis Temperature lability Hypotension Apnea/bradycardia

Necrotizing Enterocolitis – Pathology [Figure 6-3-2] • • • • •

Ileum and colon most common Coagulative and hemorrhagic necrosis Dilated, friable bowel Submucosal and subserosal gas bubbles Complications – perforation, sepsis, stricture

Distended and discolored abdomen in a neonate with severe NEC

NEC – Radiographic Findings [Figure 6-3-3] • •

Normal intestinal gas pattern of a neonate – uniform polygonal lucencies throughout the abdomen NEC – nonspecific plain film findings ➢ Distended loops ➢ “Sentinel loop” sign ➢ Wall edema ➢ Ascites

Figure 6-3-2

Figure 6-3-3

Gross specimen of ileum showing submucosal and subserosal pneumatosis in NEC

Normal neonatal bowel gas pattern

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NEC – Radiographic Findings •

•

Figure 6-3-4

[Figures 6-3-4 to 6-3-6]

NEC – Specific plain film findings ➢ Pneumatosis intestinalis ❖ Subserosal – curvilinear ❖ Submucosal – bubbly, looks like stool ➢ Portal venous gas Indication for surgical intervention ➢ Free air

Figure 6-3-5

NEC with diffuse pneumatosis and pneumoperitoneum below the liver tip on left lateral decubitus view (right image).

Figure 6-3-6

Branching portal venous air

NEC – Other Imaging [Figure 6-3-7] •

•

US ➢ Thickened bowel wall ➢ Mural gas ➢ Portal venous gas - mobile Contrast enema ➢ Contraindicated acutely ➢ Late strictures - 20%

Free air around the falciform ligament (arrow). The American football sign

STOMACH Hypertrophic Pyloric Stenosis • • • • • •

Acquired hypertrophy of antropyloric circular muscle Etiology unknown Common – 1 in 500 live births in the US Males more often affected – 5:1 First born Family history – 5%

Figure 6-3-7

HPS – Clinical Features • • • • • •

Present at 2-9 weeks of age Rare after 3 mo Uncommon in preemies Progressive nonbilious projectile vomiting Dehydration Failure to thrive

HPS – Pathology [Figures 6-3-8 and 6-3-9] • •

Neurons supplying the circular muscle layer lack nitric oxide synthetase activity Circular muscle layer undergoes hypertrophy and elongation

Colonic stricture due to prior NEC

HPS – Imaging [Figures 6-3-10 and 6-3-11] •

•

Plain film ➢ +/- dilated stomach with little distal gas ➢ Gastric hyperperistalsis Ultrasound – diagnostic

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➢ ➢ ➢ ➢

Long pyloric channel (>17 mm) with thick muscular wall (> 3 mm) Measure sonolucent part of one wall May have fluid/debris filled stomach No passage of fluid through pylorus

Figure 6-3-8

Figure 6-3-9

Normal pylorus on left and hypertrophic pyloric stenosis on right

Figure 6-3-11 Gross specimen of hypertrophic pyloric stenosis

Figure 6-3-10

AP radiograph shows markedly dilated stomach with deep indentations due to hyperperistalsis and little distal bowel gas

HPS – Imaging •

Upper image shows hypertrophic pyloric stenosis with thickened sonolucent muscle and marked elongation compared to normal (lower image)

Figure 6-3-12

Ultrasound ➢ Borderline measurements ◗ follow-up in 1 or 2 days ➢ Pyloric US is only good for HPS - if US negative, look for other causes of vomiting ❖ SMA/SMV inversion ❖ Hydronephrosis ➢ Pitfalls: overdistended stomach, coapted antrum

HPS – Imaging [Figure 6-3-12] •

•

UGI ➢ No longer used unless post-op with persistent symptoms ➢ Multiple signs – string, double string, beak, tit, shoulder and mushroom Ddx: pylorospasm, antral gastritis

UGI in HPS showing string sign (A), beak sign (B), shoulder sign (C), and tit sign (D) Pediatric Radiology

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HPS – Treatment • • •

Figure 6-3-13

Hydration and electrolyte replacement Pyloromyotomy – U.S. Nonoperative treatment – U.K. and Scandinavia

Gastrostomy Tubes [Figure 6-3-13] • • •

Usually in neurologically impaired child Use water-soluble contrast, not too hyperosmolar Trouble shooting ➢ Tube not in track or not in stomach ➢ Obstruction due to balloon migration into gastric outlet ➢ GJ tubes in babies may cause intussusception/curtain-rodding

Bezoar [Figure 6-3-14] • • • •

How to do a G-tube check. Frontal view suggests the tube is properly positioned, but lateral view tangential to the tube track, shows the tube is in the track and not in the stomach

Lactobezoar – too concentrated formula Trichobezoar – young and emotionally disturbed children Can embolize Can be treated endoscopically unless embolic

Figure 6-3-14

SMALL BOWEL Duodenal Hematoma •

• •

Etiology – blunt abdominal trauma ➢ Handlebar ➢ Seat belt ➢ Inflicted trauma Associated injury to other organs, especially pancreas Usually present with vomiting, pain, less commonly mass, jaundice

Embolic trichobezoar mimicking malrotation as emboli connected to gastric bezoar by hair that straightened the bowel

Duodenal Hematoma – Imaging • •

US - mixed echogenicity mass which becomes more hypoechoic as it liquefies UGI ➢ Intramural mass causes curved impression on duodenum ➢ Widened, separated folds ◗ stack of coins

NAT-Visceral Injury • • • • •

Seen at all ages Usually blunt Delay in seeking treatment 20%-50% mortality Proximal SB hematoma, distal SB perforation

Differential of Intramural Hemorrhage • •

•

Blunt abdominal trauma Coagulation disorder ➢ Hemophilia ➢ Blood dyscrasia with pancytopenia ➢ Henoch-Schonlein purpura Ischemia

Henoch-Schönlein Purpura • • • •

Idiopathic anaphylactoid reaction with diffuse vasculitis In the small bowel it causes intramural hemorrhage Jejunum most frequently involved Enteroenteric intussusception common

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HSP – Clinical Features • • • • • •

Figure 6-3-15

Characteristic purpuric rash of LE’s and buttocks Abdominal pain – may precede rash by days Arthritis Nephritis Vasculitis Acute scrotum

HSP - Pathology [Figure 6-3-16] • • •

Gross – focal mucosal hemorrhage, edema, and Ultrasound and CT of HSP showing markedly thickened wall and echogenic/enhancing erosions thickened mucosa of the dilated small bowel Histo - acute leukoclastic vasculitis of small vessels in the submucosa or deep lamina propria Ddx: SLE, enterohemorrhagic strains of E. Coli (0157:H7 which causes hemolytic uremic syndrome)

HSP – Imaging Findings [Figures 6-3-15 and 6-3-16] • • • • •

Thickening of SB wall Focal areas of dilatation alternating with stenosis Separation of bowel loops Submucosal masses Enteroenteric intussusception

Figure 6-3-16

Inguinal Hernia • • • • •

Most common cause of intestinal obstruction in young infants Usually a clinical diagnosis Incarceration or strangulation can cause bowel obstruction Most male - 90% Bowel, fat, fluid, ovaries can herniate

Gross images showing the dilated markedly thickened small bowel and classic purpuric rash

Inguinal Hernia - Imaging [Figure 6-3-17] • •

Plain film – look for air in scrotum or thickened inguinal fold Ultrasound ➢ Bowel or fat in the inguinal canal or scrotum ➢ Color Doppler to evaluate for flow to incarcerated bowel

Figure 6-3-17

Intussusception - Clinical [Figure 6-3-18] • • • • • •

90% ileocolic or ileoileocolic 90% due to lymphoid hyperplasia – seasonal (winter and spring) 6 mo to 3.5 years (peak 5-9 mo) Outside that age range consider lead points Colicky pain, vomiting, bloody stools, lethargy, palpable mass RLQ 10% recurrence rate

Figure 6-3-18

Young infant with distal bowel obstruction with air over the right inguinal canal

Ileocolic (left) and ileoileocolic (right) intussusception Pediatric Radiology

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Intussusception - Pathology • • •

Figure 6-3-19

Invagination of one segment of bowel into another Edema, congestion, coagulative and hemorrhagic necrosis Prominent Peyer patches

Intussusception - Pathology •

8-10% pathologic lead points ➢ Meckel diverticulum if younger ➢ Lymphoma if older ➢ Polyp ➢ Enteric duplication cyst ➢ Appendix ➢ Henoch-Schonlein purpura

Intussusception - Imaging [Figures 6-3-19 to 6-3-21] •

•

Plain film ➢ Left lateral decubitus films ➢ No air in cecum or filling defect ➢ Air crescent sign ➢ SBO possible – especially in infant US – graded compression ➢ Pseudokidney/donut/target sign ➢ Doppler flow to wall ◗ viable ➢ Ascites – nonspecific

Intussusception. Crescent of air surrounds intussusceptum in the transverse colon

Figure 6-3-20

Intussusception Reduction •

•

Contraindications ➢ Free air/peritoneal signs ➢ Septic shock ➢ Hx >24 hours Preparation ➢ Surgical consult – capable surgeon present ➢ IV – antibiotics ➢ Someone to monitor patient ➢ 16-G Angiocath to treat tension pneumoperitoneum

US in 10 mo with intussusception showing donut (left) and pseudokidney signs. Echogenic material within the intussuscepiens is mesenteric fat

Intussusception Reduction • • • • • • •

Figure 6-3-21

Air up to 120 mm Hg1 or Water soluble contrast 3 attempts, 3 minutes each Largest tip possible Squeeze buttocks when at IC valve If losing air, apply forward pressure to tip Endpoint is reduction of soft tissue mass AND rush of air into the SB

1Shiels WE, Maves CK, Hedlund G, Kirks DR. Air enema

for diagnosis and reduction of intussusception: clinical experience and pressure correlates. Radiology 1991;181:169-172

CT of colonic intussusception due to polyp. Note fat and vessels inside the intussuscepiens

Intussusception Reduction [Figure 6-3-22] • • • • •

Air slightly more effective than water soluble contrast Perforations with air are smaller1 Perforations usually occur near IC valve and are associated with necrotic bowel “Perforation” is probably the uncovering of a perforation that was already there If concerned for recurrence, go straight to enema

1Shiels WE, Kirks DR, Keller GL, et al. Colonic perforation by air and liquid

enemas: comparison study in young pigs. AJR 1993;160:931-935.

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Meckel Diverticulum • • • • • •

Figure 6-3-22

Omphalomesenteric/vitelline duct remnant Most common congenital anomaly of the GI tract 2% of population 45% present under age 2y Within 2 ft of IC valve 2% complications – intussusception, obstruction, hemorrhage, diverticulitis, perforation

Meckel Diverticulum - Presentation • • • •

Most commonly become symptomatic in first years of life Bleeding due to ulceration caused by secretion of acid by ectopic gastric mucosa Obstruction due to inverted Meckel diverticulum serving as a lead point for intussusception Vitelline band can serve as a fulcrum for volvulus

Meckel Diverticulum - Pathology [Figure 6-3-23] • • • • •

1-5 cm long Therapeutic air contrast enema in older child with Antimesenteric border intussusception due to lymphoma. Watch as Most discovered incidentally are lined by small bowel mass moves from hepatic flexure to cecum. The epithelium procedure is not successful until the mass is Those that present with symptoms are more likely to completely reduced and air rushes into the ileum contain ectopic gastric mucosa - 15-25% as shown in the lower right image of another patient Can be giant

Meckel Diverticulum - Imaging [Figures 6-3-24 and 6-3-25] •

•

•

Plain film – ➢ May be visible if giant – mottled air collection ➢ May be filled with air or entherolith Ultrasound ➢ Gut signature – resembles normal bowel ➢ When inflamed, mimics appendicitis Tc99mPertechnetate scan positive if contains gastric mucosa

Figure 6-3-23

Figure 6-3-24

Meckel diverticulum

Figure 6-3-25

Lethargic 4 mo with small bowel obstruction. Ultrasound shows fluid-filled lead point due to Meckel diverticulum

Meckel diverticulum on small bowel follow-through

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Figure 6-3-26

Figure 6-3-27

Longitudinal (left) and transverse images showing intussusception with large fluid-filled lead point, a duplication cyst

Intraoperative image showing ileocolic intussusception and sectioned resected specimen showing duplication cyst lead point

Enteric Duplication Cyst • • • •

Developmental tubular or cystic structures adjacent to the GI tract Share wall and blood supply with adjacent bowel Usually round and do not communicate with bowel lumen Occasionally tubular, communicating with GI tract at one end and blind-ending at the other

Duplication – Clinical Features • •

Thoracic – respiratory symptoms, incidental finding Abdominal – obstruction, mass, pain, GI bleeding, incidental on prenatal ultrasound

Duplication Cyst - Location • • • • • •

Ileum - 40% Thorax (posterior mediastinum) - 20 Jejunum - 10 Stomach - 10 Colon - 10 Multiple - 5

Duplication Cyst - Pathology [Figure 6-3-27] • • • • •

Often share muscularis layer with adjacent bowel (intramural) Mesenteric border Filled with mucoid material Histologically, recapitulate normal GI tract May contain ectopic gastric mucosa (20%) or pancreatic tissue

Duplication Cyst - Imaging [Figures 6-3-26 and 6-3-28] • •

Plain film – as soft tissue mass if large Ultrasound ➢ Preferred study ➢ “Rim” sign – gut signature in the wall ➢ Peristalsis ➢ May contain debris

Figure 6-3-28

Crohn Disease • • • •

¼ present in childhood Abdominal pain, diarrhea, hematochezia, weight loss CT for abscess, UGI/SBFT for diagnosis Ddx: TB, yersinia, pseudomembranous colitis, lymphoma

Appendicitis • • •

Children more often have atypical presentation Children have a higher rate of negative laparotomy and of perforation than adults Rare in infants

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Duplication cyst (longitudinal and transverse) showing round fluid-filled cavity surrounded by a wall with gut signature. Note echogenic rim of mucosa

Pediatric Radiology

Appendicitis • • • •

Appendicolith 10-15% US – blind-ending, uncompressible, > 6mm CT – ? IV, oral, rectal contrast – young children have no fat Pitfalls ➢ Perforated appendix may be decompressed ➢ Tip appendicitis

COLON/RECTUM Neutropenic Enterocolitis • • • •

AKA typhlitis Necrotizing enterocolitis often affecting the right colon in patients with neutropenia Pathologically similar to NEC in premature infants and pseudomembranous colitis Transmural – risk of perforation

Neutropenic Enterocolitis - Clinical •

• •

Usually leukemic children on chemotherapy, but also those with aplastic anemia or immunosuppression for organ transplantation, and AIDS Fever, nausea, vomiting, diarrhea, abdominal tenderness Mortality has decreased from 80% to less than 20% due to early recognition and treatment

Figure 6-3-29

Neutropenic Enterocolitis - Imaging • •

•

All imaging findings are nonspecific – clinical setting most helpful Plain films ➢ No gas in RLQ ➢ Dilated ascending colon ➢ Pneumatosis Ultrasound ➢ Thickened bowel wall and thickened mucosa ➢ Dilated, fluid-filled loops ➢ No ascites

Neutropenic Enterocolitis - Imaging [Figure 6-3-29] • •

•

Contrast enema – contraindicated CT ➢ Transmural wall thickening ➢ Infiltration of the surrounding fat ➢ Can exclude other causes of RLQ pain Ddx: pseudomembranous colitis, leukemic infiltration, intramural hemorrhage, ischemic colitis

Neutropenic enterocolitis. KUB shows lack of bowel gas on the right and markedly thickened haustra in the right transverse colon. Note also the dual lumen catheter overlying the abdomen, a clue to the underlying diagnosis of leukemia

References Texts 1. Donnelly LF. Fundementals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001. 2. Kuhn JP, Slovis TL, Haller JO, eds. Caffey’s Pediatric Diagnostic Imaging. Philadelphia: Mosby, 2004 3. Stringer DA, Babyn PS, eds. Pediatric Gastrointestinal Imaging and Intervention. Hamilton: B.C. Decker Inc., 2000 4. Stocker JT, Dehner LP, eds. Pediatric Pathology. Philadelphia: Lippincott Williams & Wilkins, 2002 5. Swischuk LE. Imaging of the Newborn, Infant, and Young Child. Philadelphia: Lippincott, 2004 Journal Articles 1. Berrocal T, Lamas M, Gutieerez J, et al. Congenital anomalies of the small intestine, colon and rectum. RadioGraphics 1999:19:1219-1236 2. Blumhagen JD, Maclin L, Krauter D, et al. Sonographic diagnosis of hypertrophic pyloric stenosis. AJR 1988;150:1367-1370 3. Buonomo C. Neonatal Gastrointestinal Emergencies. Radiology Clinics of North America 1997; 35: 845-864

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4. 5. 6. 7. 8. 9.

Kleinman PK, Brill PW, Winchester P. Resolving duodenal-jejunal hematoma in abused children. Radiology 1986;160:747-750. O’Keeffe FN, Stansberry SD, Swischuk LE, Hayden CK, Jr. Antropyloric muscle thickness at US in infants: what is normal? Radiology 1991;178:827-830 Merritt CR, Goldsmith JP, Sharp MJ. Sonographic detection of portal venous gas in infants with necrotizing enterocolitis. AJR 1984;143:1059-1062. Segal SR, Sherman NH, Rosenberg HK, et al. Ultrasonographic features of gastrointestinal duplications. J ultrasound Med 1994; 13:863-870. Sivit CJ, Taylor GA, Newman KD, et al. Safety-belt injuries in children with lap-belt ecchymosis: CT findings in 61 patients. AJR 1991;157:111-114. Sloas MM, Flynn PM, Kaste SC, Patrick CC. Typhlitis in children with cancer: a 30 year experience. Clin Infect Dis 1993;17:484-90

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Diseases Affecting the Pediatric Airway Ellen Chung, LTC, MC Figure 6-4-2

Figure 6-4-1

Normal AP views of the airway. The left image shows the vocal cords open and the right image shows the vocal cords coapted (arrows). Note also the false cords (block arrows). Between the true and false cords is the laryngeal ventricle. The true cords create shoulders on the subglottic trachea resembling those of a wine bottle

Normal sagittal MR of an adult and lateral soft tissue neck radiograph of a child showing normal upper airway structures. 1.Adenoids (none in adult) 2. Vallecula 3. Epiglottis 4. Aryepiglottic folds 5. Subglottic trachea 6. Retropharyngeal soft tissues

Figure 6-4-3

ACUTE UPPER AIRWAY OBSTRUCTION Viral Croup [Figures 6-4-3 and 6-4-4]

• • • • • • •

Laryngotracheobronchitis Characteristic barking, brassy cough w/ inspiratory stridor Most frequent cause of stridor 6 mo - 3 yo Self-limited disease-7 days Parainfluenza Subglottic edema 5-10 mm below cords Less than 1% need intubation

Figure 6-4-4

Viral croup. Note the hypopharyngeal overdistension and subglottic tracheal narrowing and indistinctness (block arrow). The most important finding on this film is the normal pinkie-like epiglottis (arrow)

Viral croup, AP view, showing subglottic narrowing and loss of the normal “shoulders” of the subglottic trachea Pediatric Radiology

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Figure 6-4-5 Epiglottitis [Figure 6-4-5] • • • • • •

Life-threatening supraglottic inflammatory disease Clinically appear toxic Typically 3-6 yo Haemophilus influenzae, Group A strep Do not make uncomfortable Edema of epiglottis and aryepiglottic folds

Epiglottitis DDX • • • • • • • •

Caustic ingestion or thermal injury Angioneurotic edema Radiation Sarcoidosis Hemorrhage Abscess Epithelial cyst Omega epiglottis

Retropharyngeal Cellulitis [Figures 6-4-6 • • • • • •

Epiglottitis with epiglottis shaped like a thumb, thickened aryepiglottic folds, and loss of the normal concavity of the aryepiglottic folds (arrow) to 6-4-8]

Most common in children between 6 to 12 months Fever, stiff or wry neck, dysphagia Straightening or reversal of normal cervical lordosis Thickening of the prevertebral soft tissues May cause airway compression, vasospasm or venous thrombosis (Lemierre syndrome) May extend into mediastinum or rupture into airway

Figure 6-4-7

Figure 6-4-6

AP and lateral films demonstrate a normal epiglottis and aeryepiglottic folds with thickened prevertebral soft tissues and reversal of the normal lordosis of the cervical spine

Figure 6-4-8

Adapted from Rivera and Young, unpublished material, 1992. A (solid line) represents the thickness of the prevertebral soft tissues anterior to the intervertebral disc space C2-3. B (dashed line) represents the AP diameter of the base of C2 Diseases Affecting The Pediatric Airway

Retropharyngeal cellulitis. Contrast enhanced axial CT shows marked prevertebral soft tissue swelling and ill defined hypodensity without ring enhancement

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Figure 6-4-9 Retropharyngeal Cellulitis - DDX •

•

Adenopathy ➢ Leukemia/lymphoma ➢ Kawasaki ➢ Langerhans cell histocytosis ➢ Metastatic disease Edema ➢ SVC syndrome ➢ Angioneurotic edema

Parapharyngeal Abscess [Figure 6-4-9] • • •

Plain film cannot distinguish between cellulitis and abscess CT skull base to aortic arch Low density center surrounded by an enhancing rim characteristic of an abscess • Drainable pus vs. focal cellulitis – delayed imaging helps Axial CT of the neck demonstrates a • Ddx: necrotic nodes low density ring enhancing lesion in the left parapharyngeal space Membranous Tracheitis consistent with an abscess • AKA Membranous croup, bacterial tracheitis • Uncommon, life-threatening • Exudative plaques form along tracheal walls like those seen in diphtheria • Irregular tracheal wall, asymmetric subglottic narrowing, loss of definition of the wall

ACUTE LOWER AIRWAY OBSTRUCTION Reactive Airways Disease • • • •

Very common with increasing incidence Obstruction due to bronchospasm May have other associated allergic disorders Precipitating factors ➢ Infections, especially RSV ➢ Weather changes ➢ GER ➢ Aspirin or NSAIDs

Reactive Airways Disease • •

Chest radiograph – normal to hyperinflated with mild peribronchial cuffing and parahilar increased markings Complications ➢ Atelectasis ➢ Air trapping ➢ Pneumonia ➢ Air block phenomena

Aspirated Foreign Body – Clinical • • • • • • •

Choking Loss of phonation Cough Wheezing Asymmetric or decreased breath sounds Hemoptysis Recurrent or persistent pneumonia

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Aspirated Foreign Body – Radiology [Figure 6-4-10] • • • • •

• •

Figure 6-4-10

Maintain a high index of suspicion in crawling infants and toddlers If unwitnessed, may present with chronic symptoms suggesting pneumonia The vast majority are not radiopaque Most commonly lodge in the bronchus If initial radiographs are normal (up to 1/3 of patients), do additional views to look for air trapping – lack of change of volume of the affected lung 18 month old with normal PA and lateral chest Atelectasis and air trapping radiographs but high clinical suspicion of aspirated Suspect in children <3 yo with pneumonia that fails foreign body. Right lung fails to collapse with to clear after 2 weeks or with air block phenomena dependent positioning due to tortilla chip in the right mainstem bronchus

Aspirated Foreign Body • •

Treatment – bronchoscopic removal Complications ➢ Pneumothorax ➢ Pneumomediastinum ➢ Chronic/recurrent pneumonia ➢ Bronchiectasis

Figure 6-4-11

Hydrocarbon aspiration • • • • •

Hydrocarbons – furniture polish, gasoline, kerosene, lighter fluid Aspirated due to low viscosity and surface tension Severe chemical pneumonitis with destruction of surfactant Radiographic abnormalities develop within 24h Pneumatoceles may develop

Upper esophageal foreign body • • •

•

Can be a cause of stridor Accompanied by dysphagia, but this symptom may go unnoticed Coins in esophagus are seen en face on PA chest, whereas coins Axial CT in bone window showing in trachea are seen in tangent on PA chest due to posterior gap in right bony choanal atresia (arrow). the cartilage rings of the trachea Note the thickening of the posterior UGI helpful for radiolucent esophageal foreign bodies. Start with midline vomer and medial deviation of the lateral wall of the nasal cavity water soluble contrast.

CHRONIC CONDITIONS AFFECTING THE AIRWAY Nasal Cavity: Paranasal Sinuses Figure 6-4-12

Choanal Atresia [Figure 6-4-11] • • • • • •

Bony or membranous septum at posterior nasal septum If bilateral, will present early as neonates are obligate nasal breathers CT with very thin cuts following nasal suction +/- topical decongestants 90% are bony Thickening of the vomer and medial bowing of the lateral wall of the nasal cavity Air-fluid level Axial CT showing piriform aperture stenosis (arrows). Note the hour-glass configuration of the nasal cavity

CHARGE Association • • • • • •

Coloboma Heart defect Atresia of the choana Retardation of growth and developmental delay Genital hypoplasia Ear deformities and deafness

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Pyriform Aperture stenosis [Figure 6-4-12] • • • • •

Figure 6-4-13

AKA “inlet” stenosis Cyanosis with feeds Width less than 11 mm considered abnormal Also well evaluated with CT Associated with abnormalities of pituitary gland and dentition and craniofacial syndromes

Congenital Midline Nasal Mass • • •

Frontoethmoidal cephalocele Nasal glial heterotopia Nasal dermoid

Nasal dermoid (arrow).Note midline bony efect, seen best on CT

Congenital Midline Nasal Mass • •

CT shows bony defect – 1- 1.5 mm w/ sagittal reformats MR shows intracranial extension and associated brain abnormalities ➢ Cephalocele – dysgenesis of CC,interhem cyst/lipoma, cortical dysplasia, craniofacial dysraphism ➢ Dermoid – intracranial dermoid/epidermoid ➢ Nasal glial heterotopia – no intracranial extension

Figure 6-4-14

Nasal Dermoid • • • •

Midline, usually round and cystic May have hypertelorism or nasal pit May occur anywhere along dermal sinus tract Tract may communicate with the cranial contents

Dermoid bony defect [Figure 6-4-13] Cephalocele [Figure 6-4-14] • • • •

Meninges or meninges and brain herniate into the nasal cavity through a defect in the cribiform plate or an open suture Anterior cephaloceles – sincipital (roof of nose) or basal (skull base) Sincipital – nasofrontal, nasoethmoidal, or naso-orbital Nasal obstruction, rhinorrhea, epistaxis

Sagittal T2-weighted MRI showing sphenoethmoidal encephalocele with herniation of meninges and optic chiasm

Nasal Glial Heterotopia • • •

AKA nasoglioma Related to cephalocele No communication with intracranial contents

Juvenile Angiofibroma • • • •

Highly vascular, locally invasive, histologically benign Adolescent boys w/ nasal obstruction, sinusitis and epistaxis Originates in sphenopalatine foramen, nasopharynx or posterior nasal cavity Spreads early into pterygopalatine fossa, infratemporal fossa, middle cranial fossa, orbit or sphenoid sinus

Figure 6-4-15

Juvenile Angiofibroma [Figure 6-4-15] •

• • •

CT - sharply marginated mass with homogeneous enhancement; anterior bowing of the posterior wall of the maxillary sinus MR - iso- to hypointense to muscle on T1 and iso- to hyperintense on T2 Juvenile angiofibroma. CT on left shows anterior MR differentiates tumor from secretions in obstructed bowing of posterior wall of maxillary sinus due to sinus mass in pterygopalatine fissure. T1-weighted Prominent tumor vessels may be seen as flow voids contrast enhanced MR shows enhancing mass in left nasal cavity and ethmoid air cells with – internal maxillary artery nonenhancing trapped secretions in the maxillary sinus

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Sinus Mass Differential • • • • • •

Figure 6-4-16

Fungal sinus infection Mucocele Rhabdomyosarcoma Esthesioneuroblastoma Lymphoma Melanoma

Esthesioneuroblastoma [Figure 6-4-16] • • • • •

AKA olfactory neuroblastoma Very rare tumor originating from neural crest cells in the olfactory groove Olfactory neuroblastoma Extensive destruction Extends intracranially

Rhabdomyosarcoma • • • • • • • • • • • •

3rd most common childhood primary malignancy of the head and neck Esthesioneuroblastoma on CT and coronal Gd40% arise in the head and neck enhanced T1W MRI showing mass in nasal cavity Orbit and nasopharynx most common with invasion of the right frontal lobe with Also paranasal sinuses and middle ear surrounding edema Usually embryonal cell subtype May spread intracranially via skull base foramina CT-iso to brain and enhances uniformly MR- T1 iso to muscle, T2 hyperintense and heterogeneous MR shows intracranial extension through fissures and foramina well CT shows bony erosion well Enhancement may make extracranial portions of the tumor less conspicuous on MR Fat saturate post-gadolinium sequences of orbits and face

Nasal polyps • • •

Uncommon in children except those with cystic fibrosis Can also be associated with recurrent infection and allergies In CF can be so large and chronic as to widen the nasal passages

Antrochoanal Polyp [Figure 6-4-17] • • • • • •

Polyp originating in the maxillary sinus Protrudes through an opening into the nasopharynx Unilateral nasal congestion is the most common symptom May present as large mass in oropharynx Ipsilateral maxillary sinus is opacified Incomplete resection associated with high recurrence rate

Palatine Tonsil and Adenoid Enlargement • • • • • • • • •

Adenoids are lymphoid tissue in the posterior nasopharynx Absent at birth Absence after 6 months suggests immune deficiency May cause snoring, hypoxemia, respiratory acidosis, pulmonary hypertension May obstruct Eustachian tubes Adenoidal pillow normally smooth Physiologic enlargement maximum age 3-5 years Significant enlargement obliterates nasopharyngeal airway Mononucleosis (EBV,CMV) rare <3, mean 14 yo

Figure 6-4-17

Antrochoanal polyp extending from right maxillary sinus into nasopharynx Diseases Affecting The Pediatric Airway

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Oral Cavity Hypopharynx

Figure 6-4-18

Macroglossia • • • •

Down’s syndrome Hypothyroidism Beckwith-Wiedemann syndrome Storage diseases

Mandibular Hypoplasia •

•

Hemifacial microsomia – unilateral ➢ Ipsilateral TMJ hypoplasia ➢ Congenital ear defects “First arch” syndromes ➢ Pierre Robin sequence ➢ Goldenhar syndrome ➢ Weyer mandibulofacial dysostosis ➢ Treacher Collins mandibulofacial dysostosis ➢ Trisomies ➢ Airway obstruction by normal sized tongue

Left image is an IV contrast-enhanced CT showing left parotitis. Compared to the normal right parotid gland, the left is enlarged, enhancing and has small hypodense foci consistent with intragland abscesses. The image on the right shows a large calcification in the duct of the right submandibular gland

Inflammatory disease of the salivary glands [Figure 6-4-18]

• • • • • • • • • •

Acute infection is viral or bacterial Bacterial sialadenitis is most common in parotid gland Bacterial infection usually due to decrease in flow of secretions Predisposing conditions include sialolithiasis Can occur in dehydrated newborns On CT the affected gland is enlarged and hyperdense Intragland abscess may be seen Most calcified duct stones are visible on noncontrast CT US can be used to evaluate salivary glands Obstruction of a sublingual duct or accessory duct is a ranula

Figure 6-4-19

Inflammatory disease of the salivary glands •

Chronic disease ➢ Recurrent bacterial infection ➢ Granulomatous disease ➢ Autoimmune disease – Sjogren syndrome ➢ Lymphoepithelial cysts in HIV disease

Hypopharyngeal cyst • • • • •

Epiglottic or aryepiglottic folds Retention cysts or lymphatic malformations Inspiratory stridor or choking during feeding Present in infancy or early childhood Treated with marsupialization

Vallecular cyst (arrow). Bright on T2WI (top) and nonenhancing on post-Gd T1WI (bottom)

Vallecular Cyst [Figure 6-4-19] • •

Caused by obstruction of submucosal mucous and serous glands between the epiglottis and base of tongue If large can cause SOB, hoarseness, dysphagia, failure to thrive, acute airway obstruction

Tongue Base Mass • • • •

Hemangioma Enlarged lingual tonsils Thyroglossal duct cyst Lingual thyroid

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Lingual Thyroid [Figure 6-4-20] • •

Figure 6-4-20

Document orthotopic thyroid whenever evaluating a near midline tongue-base or neck mass Ectopic thyroid most commonly near foramen cecum

Subglottic Trachea Neck Childhood Neck Neoplasms • • • • • •

Neuroblastoma Neurofibroma/schwannoma Lymphoma Hemangioma Lymphatic malformation/lymphangioma Teratoma

Subglottic Hemangioma •

• • • •

Rare cause of stridor in infants, but most common subglottic soft tissue mass causing upper airway obstruction Noisy breathing at 6-12 months 50% have cutaneous hemangiomas Asymmetric narrowing of subglottic trachea Ddx: subglottic cyst, papilloma, cervical ectopic thymic tissue, tracheal granuloma

Hemangioma • • • • •

Benign vascular tumor of infancy – high flow lesion Most common tumor of infancy Usually absent at birth and appears in first few months Early proliferative phase, later involutional phase May involve skin and have characteristic appearance

Lingual thyroid. Left CT image shows homogeneous intensely enhancing mass at base of tongue. Lower right CT image shows no thyroid gland in the base of the neck. Upper right image is a pertechnetate scan showing no uptake above the sternal notch and most uptake at the base of tongue

Figure 6-4-21

Hemangioma [Figure 6-4-21] • • • •

May occur in the liver and cause high output heart failure Very well marginated, intensely enhancing with large feeding vessels If it does not look like a hemangioma, it is not a hemangioma Only require treatment if affect airway or vision or cause intractable heart failure

Hemangioma on contrast enhanced T1-weighted MR image in the posterior neck. The mass very well defined and homogeneous except for the multiple vascular flow voids. Compare to well marginated, lobulated surface of the resected tumor

Hemangioma •

•

PHACE syndrome ➢ Posterior fossa abnormalities ➢ Hemangioma of the face ➢ Arterial abnormalities ➢ Coarc/cardiac defects ➢ Eye abnormalities Kassabach-Merritt syndrome ➢ Thrombocytopenia and consumptive coagulopathy associated with vascular tumor

Figure 6-4-22

Congenital teratoma of anterior neck. MR and CT show complex appearance characteristic of a mature teratoma with cystic and solid components. Note deviation of the trachea Diseases Affecting The Pediatric Airway

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Teratoma [Figure 6-4-22] • • • •

Arise from pleuripotential cells 20% malignant Neck – large anterior mass in neonate, respiratory distress and dysphagia Heterogeneous – cyst, calcification, fat

Lymphoma • • • • • •

Common malignant tumor in the neck Both Hodgkin's and NHL NHL more frequently involves extranodal sites, including tonsils and adenoids Immunodeficiency predisposes Enlarged lymph nodes and conglomerate masses of nodes On ultrasound the nodes may be sonolucent

Lymphoma • • •

On CT, isointense to muscle On MR, isointense to muscle on T1WI and hyperintense on T2WI Burkitt lymphoma – mass originating in jaw

Neuroblastoma • • • • •

Primary or metastatic in neck Neck or thoracic primary has better prognosis than intra-abdominal primary Calcification common US may show increased blood flow May involve skull or extend intracranially

Figure 6-4-23

Fibromatosis Colli [Figure 6-4-23] • • • • •

Focal thickening or mass of sternocleidomastoid muscle associated with torticollis Noted at or shortly after birth Histologically – atrophy and partial replacement of muscle with fibrous tissue US preferred – shows continuity of mass with SCM Resolves with physiotherapy

Larynx and Trachea - Intrathoracic Airways Laryngomalacia • • • • •

Most common laryngeal abnormality of the neonate Early inspiratory stridor Worsens at rest - unusual Laryngeal collapse during inspiration with hypopharyngeal overdistension seen on airway fluoroscopy Usually resolves by age 1 year

US of sternocleidomastoid muscle showing fusiform enlargement characteristic of fibromatosis colli

Laryngotracheal cleft • • • • • •

AKA persistent esophagotrachea Extreme form of failure of separation of trachea from foregut Spectrum from posterior laryngeal cleft to common tube May have abnormal cry or mutism Symptoms mimic esophageal atresia Esophagram shows massive aspiration

Tracheomalacia • • • • • • •

Collapse of the trachea with expiration Delayed development of cartilage Focal or generalized Recurrent infections and stridor Compressed in AP diameter Associated with esophageal atresia and vascular ring Also common in Down’s syndrome

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Tracheal Stenosis [Figure 6-4-24] • •

•

Figure 6-4-24

Acquired – traumatic intubation Congenital ➢ Subglottic smooth, circumferential narrowing – looks like croup ➢ Intact cartilage rings ➢ Associated with pulmonary sling, TE fistula, pulmonary atresia or hypoplasia Biphasic stridor

Tracheobronchomegaly • • • • • •

AKA Mounier-Kuhn Dilation of airways in inspiration Due to congenital deficiency of elastic tissue 3rd-5th decade Dilated trachea and central bronchi with diverticulosis of trachea Perihilar bullae

Tracheal bronchus [Figure 6-4-25] • • • • •

RUL bronchus arises directly from trachea 1% of the population May supply whole RUL or a supernumerary segment Persistent RUL pneumonia, atelectasis or air trapping Associated with TEF, tracheal stenosis, pulmonary sling, Down syndrome

Bronchogram showing long segment tracheal stenosis in a patient status post surgical correction of pulmonary sling. Note that the trachea is of smaller caliber than either mainstem bronchus

Figure 6-4-25

Bronchial Atresia [Figure 6-4-26] • • • • •

Lobar or segmental luminal fibrosis of bronchus Recurrent infections, dyspnea or asx UL and RML most frequent Involved lung is fluid then air-filled with air trapping Tubular soft tissue density – trapped mucus just distal to atresia

Laryngeal- Tracheopapillomatosis • • • • • • •

Tracheal bronchus. Postmortem bronchogram Most common laryngeal tumors in infancy and specimen showing small bronchus to Human papilloma virus implicated supernumerary right upper lobe arising directly 2/3 pts less than 4 yo from the trachea Dx made on endoscopy with nodules on vocal cords Figure 6-4-26 Transbronchial spread < 5%, related to surgical procedures Pulmonary solid lesions with cavitation Poor prognosis with pulmonary involvement

Bronchial atresia with abrupt cut off of bronchus dilated and filled with a mucous plug (arrow). Note also the adjacent air trapping Diseases Affecting The Pediatric Airway

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References Texts 1. Ball WS Jr. Pediatric Neuroradiology. Philadelphia: Lippincott-Raven, 1997. 2. Barkovich AJ. Pediatric Neuroimaging. 3rd ed. Philadelphia: Lippincott-Raven, 2000. 3. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001. 4. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998. Journal Articles 1. Capitanio MA and Kirkpatrick JA. Upper respiratory tract obstruction in infants and children. Radiol Clin North Am 1968;6:265 2. Chinwuba C, Wallman J and Strand R. nasal obstruction: CT assessment. Radiology 1986;159:503 3. Dunbar JS. Upper respiratory tract obstruction in infants and children. AJR Am J Roentgenol 1970;109:227-246. 4. John SD, Swischuk LE. Stridor and upper airway obstruction in infants and children. RadioGraphics 1992;12:625643. 5. Panicek DM, et al. The continuum of pulmonary developmental abnormalities. RadioGraphics 1987;7:747.

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Vascular Rings and Slings Ellen Chung, LTC, MC Figure 6-5-1

Vascular Rings • • • • • •

Double aortic arch Right arch with aberrant LSCA Pulmonary sling Left arch with aberrant RSCA Anomalous innominate artery Descending aorta-carina compression

Clinical Presentation •

•

Respiratory symptoms – tight complete rings ➢ Stridor ➢ Recurrent respiratory difficulties ➢ Apnea Feeding difficulties ➢ Choking with feeds ➢ Failure to thrive ➢ Solid food dysphagia

Early in fetal development the primitive aorta develops as a ventral tube which separates into two ventral aortae. Two dorsal aortae fuse to form a single vessel supplying the lower body. As the pharyngeal pouches Evaluation of Stridor/Dysphagia develop at the rostrum of the embryo, • PA and lateral chest and high kV airway films – determine side of so too do paired pharyngeal arterial arch arches, numbering 6 (but no 5th arch in humans) between the ventral and • Esophagram with airway fluoroscopy dorsal aortae. These arches go on to ➢ MRI/MRA or CTA if vascular ring suspected fuse, partially regress and fully ➢ Otherwise, CT regress to form the pulmonary arteries and the mature left-sided Embryology of the Normal Left Aortic Arch aortic arch and its branches [Figures 6-5-1 to 6-5-4]

• • •

• •

•

Arches 1 and 2 regress Arch 3 --> common and proximal internal carotid arteries Arch 4 ➢ Right --> regresses (portion of right subclavian) ➢ Left --> persists as LEFT AORTIC ARCH Arch 5 – rudimentary in humans Arch 6 – pulmonary arteries and ducti arteriosi ➢ Right regresses ➢ Left becomes ligamentum arteriosum Dorsal aortae ➢ Right regresses – part of RSCA ➢ Left becomes descending aorta

Figure 6-5-2

In the development of the normal left aortic arch, the gap or complete regression occurs in the 8th segment of the right dorsal aorta. The normal arch has 3 branches – the brachiocephalic (innominate), the left common carotid, and the left subclavian arteries

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Figure 6-5-3 Edwards postulated the existence of a double arch with bilateral ducti arteriosi in the embryo. This double arch can be represented by this ring. The T in the center represents the pulmonary arteries. The black lines connecting the main pulmonary arteries to the aorta represent the bilateral ducti arteriosi. The anterior center of the ring (dotted circle) represents the ventral or ascending aorta branching into bilateral arches which join to form the descending aorta in the posterior center of the ring (dotted circle). Each arch gives rise to its own carotid and subclavian artery. The anterior solid circles represent the bilateral carotid arteries, and the posterior solid circles are the bilateral subclavian arteries. The portion of the ring between the carotid and subclavian arteries is formed from the 4th pharyngeal arch. The portion of the ring dorsal to the subclavian artery represents the contribution of the 8th segment of the dorsal aorta. These are the most common sites of gaps or complete regression of a portion of the ring that give rise to the different types of aortic arches

Figure 6-5-4

Figure 6-5-5

Diagram representing the normal regression of the 8th segment of the right dorsal aorta and right ductus arteriosus in development of the normal left arch. The right subclavian artery is separated from the descending aorta and arises in common with the right common carotid artery from the ascending aorta

In the development of the aberrant right subclavian artery, there is early obliteration of the right 4th arch, and the 8th segment of the right dorsal aorta persists, so the right subclavian artery maintains its connection to the descending aorta, becoming the last branch from the aortic arch (arrow). Thus, there are four, rather than the normal three, branches of the aortic arch and there is no brachiocephalic (innominate) artery. The descending aorta is on the left, so the aberrant RSCA then crosses behind the esophagus to get to the right side

Aberrant RSCA [Figures 6-5-5 to 6-5-8] • • • • •

Asymptomatic in children – normal variant Due to early complete obliteration of right 4th arch and persistence of 8th segment of the right dorsal aorta Left arch RSCA originates distal to LSCA Posterior impression on the esophagus

Figure 6-5-6

The gap or obliteration occurs in the right 4th arch. The aortic arch is on the left

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Figure 6-5-7

Figure 6-5-8

When the RSCA crosses from left to right a posterior impression is created on the esophagus, as seen on the lateral view (left image). In the AP view (right image), a normal left aortic arch is seen as well as an oblique impression on the contrast-filled esophagus extending from the left arch to the right shoulder

The oblique course of the aberrant right subclavian artery creates a sloped contour of the aortic knob (arrow) that may be visible in older children and adults

Determining Side of Arch [Figures 6-5-9 and 6-5-10] • • •

Figure 6-5-10

Tracheal deviation, buckling or impression – the most reliable sign indicating the side of the aortic arch Asymmetric density of pedicles Descending aorta can be on right or left with right arch

Figure 6-5-9

PA chest radiograph showing rightsided impression on the trachea due to right aortic arch

Normal tracheal buckling away from the left arch on an expiratory chest radiograph (arrow)

Right Arch •

•

Branching patterns ➢ Aberrant LSCA ➢ Mirror image ➢ Isolated LSCA – congenital subclavian steal High association with congenital heart disease

Mirror Image Right Arch [Figures 6-5-11 and 6-5-12] • • • •

Association with CHD 98% 25% of patients with tetralogy of Fallot have mirror image right arch 35% of patients with truncus arteriosus have mirror image right arch 90% of patients with mirror image right arch have tet

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Figure 6-5-11

Figure 6-5-12

Patient with right-sided arch and descending aorta and enlarged heart with upturned apex, a classic plain film diagnosis of Tetralogy of Fallot

Figure 6-5-13 Diagram showing the mirror-image branching right arch from the front. The ductus is on the left (arrow) so this is not a ring. A rare variant with ductus arteriosus from proximal descending aorta coursing behind esophagus to left pulmonary artery, the so-called retro esophageal ductus, is a true ring

Aberrant LSCA [Figure 6-5-13] • • •

• • • • • •

Mirror image of aberrant RSCA Left ductus arteriosus completes the ring Symptomatic patients typically have a a tight ductus or large diverticulum of Kommerell (dilation of origin of aberrant artery) This type needs to be distinguished from double arch with MRI or angiography Association with congenital heart disease 5-12% Dysphagia lusoria Posterior impression on the esophagus Right-sided impression on the trachea Ddx: double arch

Diagram showing development of right arch aberrant left subclavian artery which is the mirror image of left arch aberrant right subclavian artery (shown on the left). The right image shows early obliteration of the left 4th pharyngeal arch, separating the left subclavian artery from the left common carotid artery. Persistence of the 8th segment of the left dorsal aorta maintains continuity of the left subclavian artery with the descending aorta. The left ductus completes the ring

Right arch with aberrant LSCA and left ductus [Figure 6-5-14]

Figure 6-5-14

This diagram shows the right arch with aberrant left subclavian artery viewed from anterior. Note the four branches of the aortic arch, the last of which is the left subclavian artery. The left ductus (arrow) completes the ring, so that the trachea and esophagus are completely surrounded by aorta and pulmonary arteries

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Right arch aberrant LSCA with posterior impression on esophagus [Figures 6-5-15 to 6-5-18]

Figure 6-5-16

Figure 6-5-15

CT of same patient showing right arch and posterior course of the aberrant left subclavian artery (arrow) Esophagram shows posterior impression on the esophagus at the level of the aortic arch. When the patient is in the AP projection the right arch is identified. If there were a left arch, this patient would have an asymptomatic normal variant. With the right arch, the diagnosis is a complete vascular ring

Cervical aortic arch • • • • •

Figure 6-5-18

Arch above the clavicle – not a specific arch anomaly May have pulsatile mass in supraclavicular fossa 80% are right arches Half are symptomatic rings Most common variant - right arch that descends on the right then crosses to left behind esophagus and gives off left subclavian artery and left ductus

Double Aortic Arch [Figures 6-5-19 to 6-5-21]

• • • • • •

Figure 6-5-17

Persistence of both left and right fourth arches Arteriogram showing a Most common symptomatic vascular right arch with aberrant ring LSCA and a Right is usually larger, higher and diverticulum of posterior Kommerell (arrow), or enlargement of the Anterior and bilateral lateral origin of the aberrant impressions on the trachea subclavian artery Posterior and bilateral lateral impressions on esophagus Treatment is to ligate the nondominant arch

This sagittal MR image in a 4 month old with recurrent respiratory infections, shows the aberrant subclavian artery in cross section posterior to the trachea (arrow). Normally there is no large artery behind the trachea

Figure 6-5-19

Figure 6-5-20

Esophagram in same patient. The double arch causes bilateral lateral impressions on the esophagus as seen on the AP view (left image). On the lateral view (right image), there is a prominent posterior impression caused by the joining arches. Thus, there are 3 impressions on the esophagus

Vascular Rings and Slings

PA chest radiograph in a patient with double aortic arch, showing a higher larger right sided aortic impression on the trachea and a smaller, lower left sided impression (arrow). The left impression is often difficult to discern, so double aortic arch is in the differential of right aortic arch seen on plain film

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Pulmonary Sling [Figures 6-5-22 and 6-5-23] • • • • • • •

•

Figure 6-5-21

AKA anomalous pulmonary artery Left PA originates from right Anterior impression on esophagus at level of carina Posterior impression on trachea Ductus passes from origin of RPA to aorta forming a complete ring around the trachea only Compression of bronchus intermedius by anomalous artery Associated tracheal abnormalities ➢ Tracheomalacia ➢ Complete tracheal rings ➢ T-shaped trachea Other associated anomalies – abnormal pulmonary lobation, bronchus suis, CHD

Figure 6-5-22 3-D MRA of a double aortic arch, viewed from posterior

Figure 6-5-23

Left image is a lateral view from an esophagram showing posterior impression on the trachea and anterior impression on the esophagus at the level of the hila. Black blood MRI image shows the left pulmonary artery originating from the right and coursing behind the trachea to get to the left lung

Illustration of pulmonary sling viewed from anterior with ascending aorta cut away to show the left pulmonary artery (arrow) originating from the right, then passing between the trachea and esophagus to get to the left side

Innominate Artery Compression Syndrome • • • •

Normally the innominate artery passes in front of the trachea just below the thoracic inlet In infants it arises more to the left than in adults and there is also thymus in this region, so it may cause symptomatic compression of the trachea Increased incidence of symptomatic compression in patients with dilated esophagus Compression decreases with advancing age

Midline Aorta Carina Compression Syndrome •

Midline course of descending aorta or abnormal position of the carina allows aorta to compress carina or mainstem bronchus

Other CV Abnormalities That Can Compress the Airway Congenital Heart Disease • • •

Tetralogy of Fallot with absent pulmonary valve Large left to right shunts Massive cardiomegaly

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Absent Pulmonary Valve Syndrome [Figure 6-5-24] • • • •

Figure 6-5-24

Variant of Tetralogy of Fallot Severe pulmonic regurgitation Aneurysmal dilatation of proximal left and right pulmonary arteries Compression of adjacent bronchi

PA and lateral chest radiographs in patient Tetralogy of Fallot with absent pulmonary valves. Note the massive enlargement of the pulmonary arteries, hyperinflation of the lungs due to bilateral bronchial compression and associated right aortic arch References Texts 1. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001. 2. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998. 3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams & Wilkins, 2004. Journal Articles 1. Berdon WE and Baker DH. Vascular anomalies and the infant lung: rings, slings and other things. Semin Roentgenol 1972;7:39-63. 2. Berdon WE. Rings, slings and other things: vascular compression of the infant trachea updated from the midcentury to the millennium—the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology 2000;216:624-632. 3. Bisset GS III et. Al. Vascular rings: MR imaging. AJR Am J Roentgenol 1987;149:251 4. Donnelly LF, Bisset GS 3rd , McDermott B. Anomalous midline location of the descending aorta: a cause of compression of the carina and left mainstem bronchus in infants. AJR AM J Roentgenology 1995; 164:705-707. 5. Donnelly LF, Strife JL, Bisset GS III. The spectrum of extrinsic lower airway compression in children: MR imaging. AJR Am J Roentgenol 1997;168:59-62 6. Kussman BD, Geva T, McGowan FX. Cardiovascular causes of airway compression. Paediatr Anaesth 2004;14:6072. 7. Newman R, Meza MP, Tobin RB, et al. Left pulmonary artery sling: diagnosis and delineation of associated tracheobronchial anomalies with MR. Pediatr Radiol 1996;26:661-668 8. Pickhardt PJ, Siegel MJ, Gutierrez FR. Vascular rings in symptomatic children: frequency of chest radiographic findings. Radiology 1997;205:581-582 9. Shuford WH, Sybers RG, Edwards FK. The three types of right aortic arch. AJR 1970;109:67-74

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Cystic Renal Disease of Childhood Ellen Chung, LTC, MC Terminology • • •

Cyst Polycystic kidney disease – ARPKD and ADPKD only Multicystic kidney

Old Classification1 • • • •

Type I, infantile polycystic kidney disease Type II, multicystic dysplastic kidney Type III, adult polycystic kidney Type IV, cortical cysts associated with massive hydronephrosis

1 Osthanondh V, Potter EL. Pathogenesis of polycystic kidneys: historical survey.

Arch Pathol 1964;77:459

Genetically-based Classification2 •

•

Nongenetic ➢ Multicystic dysplasia ➢ Multilocular cyst (tumor) ➢ Simple cyst/calyceal diverticulum ➢ Medullary sponge kidney ➢ Acquired cystic disease Genetic ➢ Autosomal recessive polycystic kidneys ➢ Autosomal dominant polycystic kidneys ➢ Juvenile nephronophthisis (AR)-medullary cystic disease (AD) complex ➢ Cysts associated with multiple malformation syndromes

2Glassberg KI, Stephens FC, Lebowitz RL, et al. Renal dysgenesis and cystic

disease of the kidney: a report of the Committee on Terminology, Nomenclature and Classification, Section on Urology, American Academy of Pediatrics. J Urol 1987, Oct; 138:1085

Simple Renal Cyst • • • • •

Uncommon in children Usually solitary Found with increasing frequency due to US screening in patients with UTI Arise in renal cortex Do not communicate with collecting system

Simple Renal Cyst • • • •

Observed on PNUS ◗ screen for malformation syndrome US criteria for simple cyst ◗ no further imaging unless recurrent symptoms of infection Otherwise ◗ CT to exclude tumor No treatment unless symptomatic or obstructing collecting system

Calyceal Diverticulum • • • • • •

Cyst that communicates with collecting system Need contrast study to distinguish from cyst Urine stasis leads to infection and stone formation Look for stone in tic on US, KUB, or non-con CT Delayed images show contrast-filling of the cyst and a neck Treatment – surgical ablation if symptomatic

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Medullary Sponge Kidney • • • • • •

Figure 6-6-1

Congenital focal dilation of collecting tubules usually presenting in adulthood Associated with stones or infection but may be found incidentally Usually bilateral Medullary nephrocalcinosis – radially aligned Streaky linear densities in involved pyramids US may show calcifications before plain film

Acquired Renal Cysts • • •

AIDS Hemo- and peritoneal dialysis ➢ Increase in number and size with length time on dialysis Complications – intracyst or subcapsular or perinephric hemorrhage

Multicystic Dysplastic Kidney • • • • •

Most common form of cystic renal disease in infants and children One of the most common causes of renal mass in first week of life Rarely bilateral Due to early severe in utero obstruction Extreme end of spectrum of UPJO

Classic MCKD gross specimen showing nonreniform shape, cysts of multiple sizes and no identifiable renal parenchyma

Multicystic Dysplastic Kidney • • •

Risk of abnormality of contralateral kidney 20-50% - UPJO, VUR Negligible renal function Nodular blastemal elements in 3-5% - risk of Wilms tumor

MCDK - Presentation • • •

Figure 6-6-2

PNUS Neonate with abdominal mass Incidentally in older child – mimics agenesis

MCDK – Gross Pathology [Figures 6-6-1 to 6-6-3] • • • • • • •

Macrocysts of variable size Randomly distributed Cysts do not communicate Hydronephrotic variant – large central cyst Rarely segmental – upper pole of duplex or lower crossed fused ectopic No identifiable normal parenchyma Associated atresia of ureter or infundibulopelvis

Hydronephrotic variant of MCDK with reniform shape and patent central pelvis

Figure 6-6-3

Stillborn baby with bilateral MCDK. Plain radiograph shows small, bellshaped thorax with airless lungs and bulging flanks. Autopsy specimen (viewed from posterior) reveals bilateral enlarged kidneys which are much larger than the hypoplastic lung Cystic Renal Disease of Childhood

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MCDK - Histopathology [Figure 6-6-4] • • • •

Figure 6-6-4

Histologic hallmark – presence of immature dysplastic-appearing tubules surrounded by collarettes of PAS-staining condensed mesenchyme Cysts of varying size formed by dilated, dysplastic tubules Cysts can occur in any part of the nephron High nuclear to cytoplasmic ratio ◗ dysplasia

MCDK - Imaging [Figures 6-6-5 and 6-6-6] • • • • • • •

Large mass with cysts of varying sizes scattered throughout Cysts do not communicate Hydronephrotic type – large central cyst but no identifiable parenchyma No identifiable cortex or medulla Nonreniform shape Nuclear renogram – no significant excretion VCUG – 25% VUR

Figure 6-6-5 Photomicrograph of dysplastic kidney showing primitive ducts surrounded by mesenchymal collarettes

Figure 6-6-6

Prenatal ultrasound showing right MCDK

MCDK - DDx •

•

Multilocular cystic kidney ➢ A tumor ➢ Cysts within the intervening septa Severe hydronephrosis ➢ UPJO ➢ Nuclear renogram ➢ Contralateral kidney also affected

Renal ultrasound showing normal right kidney and left MCDK. Note noncommunicating cysts and lack of visible normal renal parenchyma

MCDK – Course and Prognosis [Figures 6-6-7 and 6-6-8]

• • • •

Natural history of a true MCDK is to resolve Formerly these were all removed due to rare reports of nephroblastoma1 Now followed to resolution If they do not resolve, surgical removal is indicated to prevent complications of infection and neoplasm

Figure 6-6-7

1Strife JL, Souza AS, Kirks DR, Strife CF, Gelfand MJ, Wacksman J.

Multicystic dysplastic kidney in children: US follow-up. Radiology. 1993 Mar;186(3):785-8.

Autosomal Recessive Polycystic Kidney Disease (ARPKD) • • • • •

Kidneys and liver – ectasia and fibrosis Kidneys – ectasia of the collecting tubules Delayed CT diagnosis of left MCDK Liver – biliary duct ectasia and periportal fibrosis which has regressed to a partially calcified nubbin Latter develops in early childhood Degrees of renal and liver involvement are inversely proportional and determine age of presentation and prognosis

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ARPKD – Spectrum of Presentation Blyth and Ockenden Clinical Classification1

Figure 6-6-8

[Figures 6-6-9 and 6-6-10]

• • • •

•

“Perinatal” – 90% of tubules involved, bilateral nephromegaly, Potter syndrome, death in first week “Neonatal” – 60%, present in first month, death by 1 year “Infantile” – 25%, present at 3-6 mo “Juvenile” – 10%, presentation in first decade ➢ Portal hypertension ➢ Incidental finding on US Presentation in second decade with renal failure

1Blyth H, Ockenden BG. Polycystic disease of kidney and liver

CT showing Wilms tumor which arose in a left MCDK

presenting in childhood. J Med Genet. 1971 Sep;8(3):257-84.

Figure 6-6-9

Figure 6-6-10

ARPKD Autopsy

ARPKD – Pathology - Kidneys [Figure 6-6-11] • • • • • • •

Large kidneys “Cysts” are dilated collecting tubules predominantly in the medulla Dilated (1-2mm) tubules arranged in a fan-shape Cortex relatively spared No dysplasia On cut section, cortex and medulla unrecognizable Few coalescent macrocysts

ARPKD – Pathology- Liver [Figures 6-6-12 and 6-6-13] • • • •

All associated with congenital hepatic fibrosis = ductal plate Gross image showing Potter facies malformation Dilation of interlobular bile ducts associated with a variable amount of portal fibrosis (Caroli syndrome) All portal areas are expanded and contain dilated ducts at the periphery with blood vessels in the middle Sinusoidal portal hypertension

ARPKD – Plain Film • • • •

Bilateral flank masses in newborns Small, bell-shaped thorax Pneumothorax Older children may have slightly enlarged kidneys, hepatosplenomegaly, and/or ascites

Figure 6-6-11

ARPKD - Ultrasound [Figure 6-6-14] •

• • •

Large kidneys with increased echogenicity in the medulla due to multiple accoustic interfaces of dilated, ectatic ducts May mimic nephrocalcinosis Compressed, spared cortex may form relatively dark rim Poor delineation of cortex, medulla, sinus

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ARPKD. From left to right cut gross specimen, photomicrograph and diagram show dilated collecting tubules in the medulla Pediatric Radiology

Figure 6-6-12 Formation of the intrahepatic biliary radicals begins with a single layer of primitive ductal plate surrounding the portal vein. Some insult can cause congenital hepatic fibrosis by promoting fibroblast proliferation.

Figure 6-6-13

Figure 6-6-14

Gross specimen showing ectatic biliary ducts of Caroli disease

ARPKD - Ultrasound [Figure 6-6-15] • • •

High frequency linear transducer may resolve tubular structures in fan-like array or tiny cysts Occasional macrocysts Those who present as children have milder renal findings – normal or mild nephromegaly, +/- increased echogenicity of medulla, loss of corticomedullary differentiation

Ultrasound - Liver [Figure 6-6-16] • • •

ARPKD. Coronal ultrasound shows both kidneys to be markedly enlarged and echogenic centrally with a relatively sonolucent rim of compressed cortex

Increased echogenicity +/- ductal ectasia in older children Portal radicals surrounded by bile ducts Splenomegaly, varices, and ascites may also be seen in older children

Figure 6-6-16

Figure 6-6-15

12 yo diagnosed in infancy with ARPKD, now preop for liver transplant. Ultrasound on left shows markedly dilated biliary ducts with hepatic artery and portal vein branches in the center, creating a target appearance. CT shows same target appearance of biliary ducts as well as cysts in the renal medulla and splenomegaly due to portal hypertension

ARPKD. High frequency linear transducer ultrasound images show echogenic, markedly enlarged kidneys in which tiny cysts can be resolved

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ARPKD - Other Imaging [Figures 6-6-17 to 6-6-19] • • • • • •

Imaging beyond US is rarely necessary Spoke-wheel or striated appearance of nephrogram Prolonged nephrographic phase Calyces compressed, separated and distorted In older children, mild tubular ectasia similar to medullary sponge kidney MR - few cortical cysts in about half of patients

Figure 6-6-18 Figure 6-6-17

ARPKD. CT of infant showing markedly enlarged kidneys with striated delayed nephrograms due to compression of parenchyma by radially oriented dilated fluid-filled collecting ducts. The kidneys are of density similar to water due to the unopacified urine in the dilated ducts. Note rim of enhancing cortex (arrow). Also note left kidney hypodense macrocyst (block arrow)

Excretory urogram of ARPKD showing bilateral massively enlarged kidneys with striated nephrograms and distorted collecting systems

ARPKD - Prognosis • • •

Infantile – poor prognosis due to renal insufficiency and pulmonary hypoplasia Outcome in childhood is better than previously thought Early recognition and management are important

Figure 6-6-19

Differential Diagnosis Large Echogenic Kidneys in Neonate • •

Glomerulocystic disease ADPKD

Glomerulocystic Disease • • •

Rare, sporadic or heritable (AD) May be found in some patients with malformative syndromes Occasionally found in children with family history of ADPKD

Glomerulocystic Disease Clinical Features • •

Present early with renal failure and palpable abdominal masses Renal function normal but deteriorates with age

ARPKD with biliary duct ectasia. CT of the kidneys shows delay of transit of contrast into dilated, urine-filled collecting tubule Cystic Renal Disease of Childhood

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Glomerulocystic Disease Pathology [Figure 6-6-20] • •

Figure 6-6-20

Cystic dilation of Bowman capsule and proximal convoluted tubule Periportal fibrosis, bile duct hyperplasia and hepatic cysts may also be found

Glomerulocystic Disease Ultrasound • • •

Echogenic normal-sized to enlarged kidneys Poor corticomedullary differentiation Tiny cysts may be seen in cortex (vs. ARPKD)

Autosomal Dominant Polycystic Kidney Disease (ADPKD) • • • • •

Much more common than AR Three genetic loci – PKD1-3 Family screening routine Spontaneous mutations frequent Cysts become larger and more numerous with age

Autosomal Dominant Polycystic Kidney Disease (ADPKD) • • •

Glomerulocystic disease in a patient with Zellweger or cerebrohepatorenal syndrome. Gross and low magnification images show small cortical cysts

Cysts not usually seen in 1st and 2nd decades but can be seen in neonates screened for positive family history Affects multiple organs – kidneys, liver, pancreas, spleen, seminal vesicles, ovaries Association with occult intracranial aneurysms – screen in adulthood

ADPKD - Presentation • • •

Present in 4th-5th decade with hypertension and renal failure Does not present in childhood but may be found incidentally or secondary to flank pain due to bleeding into cyst Rarely presents in infancy – minimal cysts in enlarged, echogenic kidneys

ADPKD - Pathology • • • • •

Enlarged but reniform kidneys Cysts of varying size scattered throughout the kidney (cortex and medulla) Usually bilateral but may be asymmetric or even unilateral Abnormality of the ampullary and interstitial portions of the collecting tubules and nephrons Hepatic fibrosis is rare

Figure 6-6-21

ADPKD - Ultrasound [Figure 6-6-21] • • • • •

Infantile presentation – small, spherical cysts on high resolution US (vs. fan-like, tubular appearance in AR) Older children - cysts of varying size in cortex and medulla Can be unilateral at presentation Normal size or slightly enlarged Look for cysts in liver and pancreas

ADPKD - Prognosis • •

Most develop renal failure in 4th-5th decade Presentation in infancy – more severe renal cystic disease, more hypertension, more rapid progression to renal failure than adult relatives

Medullary Cystic Disease Complex • • • • •

Juvenile nephronophthisis – AR, presents in first decade Medullary cystic disease – AD, presents in 3rd decade Polydypsia, polyuria, salt wasting, severe anemia Progressive renal failure Growth retardation

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Neonatal ADPKD. Kidneys are echogenic but high resolution ultrasound helps to distinguish small cysts (arrows) (vs. radially aligned, tubular cysts in ARPKD) Cystic Renal Disease of Childhood

Medullary Cystic Disease Complex Pathology

Figure 6-6-22

[Figure 6-6-22]

• • • •

<1.5 cm cysts that increase in size and number with age Cysts in medulla or subcortical region -70% of patients Secondary glomerulosclerosis – small, fibrotic kidneys Associated retinitis pigmentosa, hepatic fibrosis, skeletal defects and CNS abnormalities in familial juvenile form

Medullary Cystic Disease Complex Imaging [Figure 6-6-23]

• •

Small echogenic kidneys Multiple small medullary or corticomedullary cysts – may not be present when very young

Cysts Associated with Syndromes • • •

Zellweger - cerebrohepatorenal syndrome – AR, lethal peroxisomal deficiency Meckel-Gruber – AR, posterior encephalocele, polydactyly, cystic kidneys, congenital hepatic fibrosis Beckwith-Wiedemann syndrome – macroglossia, visceromegaly and omphalocele, Wilms tumor, also medullary cysts in 13-19%

Cysts Associated with Syndromes • • • • • •

Turner Down’s Orofaciodigital Jeune, short rib polydactyly TS – cortical cysts 33-50% VHL – multifocal cystic adenocarcinomas in 45% after 3rd decade

Medullary cystic disease. Sectioned gross specimen shows cysts in the medulla and corticomedullary junction

Figure 6-6-23

Summary • • • • • •

ARPKD and MCKD are most common in perinatal period Classic MCDK is managed conservatively Renal dysplasia is caused by in utero obstruction VCUG indicated in MCDK to exclude contralateral VUR (solitary functioning kidney) ARPKD is a spectrum of renal and hepatic disease ARPKD and ADPKD can both be diagnosed in infants as well as adolescents/adults

References

12 yo girl with medullary cystic disease

Texts 1. Kuhn JP, Slovis TL, Haller JO, eds. Caffey's Pediatric Diagnostic Imaging, 10th Ed. Philadelphia: Mosby, 2004. 2. Hartman DS. Renal cystic disease. AFIP Atlas of Radiologic-Pathologic Correlation. Fascicle I. Philadelphia: WB Saunders; 1989:1-5. 3. Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002. 4. Stocker T, Dehner L, ed. Pediatric Pathology, 2nd ed. Philadelphia: Lippincott-Williams & Wilkins, 2002. 5. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams & Wilkins, 2004. Journals 1. Blane CE, Barr M, DiPietro MA, Sedman AB, Bloom DA. 2. Blickman JG, Bramson RT, Herrin JT. Autosomal recessive polycystic kidney disease: long-term sonographic findings in patients surviving the neonatal period. AJR Am J Roentgenol. 1995 May;164(5):1247-50. 3. Corrales JG, Elder JS. Segmental multicystic kidney and ipsilateral duplication anomalies. J Urol. 1996 Apr;155(4):1398-401. 4. Diard F, Le Dosseur P, Cadier L, Calabet A, Bondonny JM. Multicystic dysplasia in the upper component of the complete duplex kidney. Pediatr Radiol. 1984;14(5):310-3.

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5. 6. 7. 8. 9.

Evans WP, Sumner TE, Lorentz WB Jr, Resnick MI. Association of crossed fused renal ectopia and multicystic kidney. J Urol. 1979 Dec;122(6):821-2. Narchi H. Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 2005 Feb;90(2):147-9. Oddone M, Marino C, Sergi C, Occhi M, Negri F, Kotitza Z, et al. Wilms' tumor arising in a multicystic kidney. Pediatr Radiol. 1994;24(4):236-8. Renal obstructive dysplasia: ultrasound diagnosis and therapeutic implications. Pediatr Radiol. 1991;21(4):274-7. Traubici J, Daneman A. High-resolution renal sonography in children with autosomal recessive polycystic kidney disease. AJR Am J Roentgenol. 2005 May;184(5):1630-3.

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Pediatric Renal Tumors: Infancy & Young Children Marilyn J. Siegel, MD Objectives • • •

Describe a variety of renal masses in infants and children Recognize the imaging features of these masses Understand unique clinical and pathologic features of these tumors

Clues to Assessing Renal Tumors •

•

Imaging: ➢ Dominant tissue composition ❖ Soft tissue ❖ Fluid (cystic) ❖ Fat Other clues: ➢ Patient age ➢ Pattern of metastases

Soft Tissue Masses •

•

< 5 years of age ➢ Nephroblastomatosis ➢ Rhabdoid tumor ➢ Clear cell sarcoma ➢ Ossifying renal tumor of infancy ➢ Mesoblastic nephroma > 5 years of age ➢ Renal cell cancer ➢ Lymphoma

Wilms Tumor: Epidemiology • • •

• •

85% of renal masses 6%-7% of all childhood cancers Approximately 500 cases/year ➢ 6/1,000,000 children Mean age at diagnosis = 3 yrs ➢ 90% < 7 yrs Sex (M:F) equal

Risk Factors • •

• •

Race: Afro-Americans > Caucasians > Asians Familial predisposition (1%) ➢ Autosomal dominant Aniridia Deletion of tumor suppressor genes on short arm of chromosome 11 ➢ 11p13 locus (WT1 gene) ➢ 11p15 locus (WT2 gene)

Risk Factors: Congenital Syndromes • • • •

WAGR syndrome (Wilms tumor, aniridia, genital abnormalities, retardation) (WT1 gene) Beckwith-Wiedemann syndrome & hemihypertrophy (WT2 gene) Drash syndrome (nephritis & male pseudohermaphrodism) WT 1 gene Trisomy 18

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Wilms Tumor: Gross Features [Figure 6-7-1] •

• • • •

Figure 6-7-1

Large cortical mass ➢ (400 to 500 gm) Hemorrhage & necrosis (90%) Pseudocapsule Spares collecting system Calcification & fat <10%

Favorable Histology (85%) [Figure 6-7-2] •

•

Triphasic composition ➢ Metanephric blastema ➢ Immature stroma ➢ Tubular elements Good prognosis if triphasic

“Unfavorable” Histology (15%) [Figure 6-7-3] •

•

Anaplastic changes ➢ Nuclear enlargement ❖ > 3x size ➢ Hyperchromatic nuclei ➢ Atypical mitotic figures Implies poor prognosis & resistance to conventional therapy

Wilms tumor, gross pathology

Figure 6-7-2

Figure 6-7-3

Wilms tumor. Classic triphasic histology showing immature blastema, tubules/glomeruli, and stroma Wilms tumor. Atypical histology showing anaplastic nuclear changes

Wilms’ Tumor: Clinical Features • •

Mean age: 3-36 mos (range, 6 mos-4 yr) Symptoms ➢ Mass: 90% ➢ Pain, fever, hematuria: 30% ➢ Hypertension: 75% - 90% ➢ Aniridia, hemihypertrophy ➢ Budd-Chiari syndrome

Wilms’ Tumor: US Findings • • •

• •

Well-defined margins Solid, intrarenal mass Tumor matrix ➢ Homogeneous: 50% ➢ Heterogeneous : 50% IVC thrombus (5%-10%) Relatively avascular

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Wilms’ Tumor

Figure 6-7-4

Wilms’ Tumor [Figure 6-7-4]

Wilms’ Tumor: Caval Thrombus US

Wilms tumor, ultrasound. Two gray-scale sonograms showing well-defined, echogenic mass (M) in lower pole of right kidney. Doppler sonogram (right image) showing flow in surrounding parenchyma. Tumor is avascular

Wilms’ Tumor US CT • •

Sensitivity (%) 100 100

Accuracy (%) 25 > 95

US performed to confirm presence of a mass and its location CT to determine tumor extent

Figure 6-7-5

Wilms’ Tumor •

• • •

Contrast enhanced CT ➢ low density, intrarenal mass ➢ rim of compressed parenchyma Little enhancement Central necrosis 75% May contain fat or calcifications

Wilms Tumor •

Exophytic mass, pseudocapsule

Wilms tumor, CT. Transverse and coronal CT reformation showing large mass extending exophytically from lower pole of right kidney

Wilms’ Tumor Wilms Tumor [Figure 6-7-5] Calcified Wilms Tumor (< 5%) [Figure 6-7-6]

Figure 6-7-6

Role of CT • •

To confirm presence of tumor and assess tumor extent Important diagnostic questions: ➢ Tumor thrombus (5%-10%) ➢ Contralateral tumor (5%-10%) ➢ Hepatic or lung metastases

Wilms Tumor: Venous Extension • • • •

Renal or caval extension: 5-10% of cases Often clinically silent Calcified Wilms tumor May prolapse through tricuspid valve Tumor thrombus in heart or hepatic segment of IVC alters surgical approach ➢ Cardiopulmonary bypass

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Wilms Tumor with IVC Extension [Figure 6-7-7]

Figure 6-7-7

Tumor Thrombus Tumor Thrombus - Bilateral Wilms’ Tumors [Figure 6-7-8]

Bilateral Wilms’ Tumor • • •

5%-10% of patients at diagnosis (synchronous disease) Younger mean age: 27 mos Dominant renal mass & small contralateral tumor or bilateral large masses

Wilms’ Tumor: Bilateral Tumors

Wilms tumor with inferior vena caval extension of tumor

Wilms’ Tumor

Figure 6-7-8

Wilms’ Tumor: Metastases •

• •

About 10% have metastases at diagnosis ➢ Lung: 85%-90% of all mets ➢ Liver: 10%-15% Plain radiographs have FN rate of 729% when CT positive CT is study of choice for distant staging

Wilms’ Tumor [Figure 6-7-9]

Bilateral Wilms tumor with caval invasion (arrow)

Wilms’ Tumor: Magnetic Resonance Imaging • • • •

Figure 6-7-9

Dark on T1-weighted images Bright on fat-suppressed images Contrast enhances Tumor thrombus seen as a luminal defect

Wilms Tumor: MRI Wilms Tumor Staging: NWTS-5 • • • • •

I. Limited to kidney, completely excised II. Extracapsular extension, but completely removed III. Residual tumor confined to abdomen IV. Hematogenous mets V. Synchronous bilateral tumors

Wilms Tumor: Treatment • • • •

En bloc resection of affected kidney Wilms tumor, lung metastases Excisional biopsy of nodes and lung nodules Postop chemotherapy Preoperative chemotherapy for invasive disease or bilateral Wilms, then surgery

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4-Year Relapse Free Survival • • • • •

Stage I: 91% Stage II: 88% Stage III: 79% Stage IV: 78% Stage V: 70%

Screening High Risk Patients (BWS, WAGR syndromes) • • •

US every 3 months until age 6 or 7 Hypothesis is that tumor will be detected at a lower stage Risk of developing Wilms tumor increases if nephromegaly at birth

Figure 6-7-10

Other Soft Tissue Tumors - Nephroblastomatosis • •

Defined as "the presence of nephrogenic rests or nephrogenic blastema beyond 36 weeks gestation” Important because it is a precursor to Wilms tumor

Nephrogenic Rests: Location •

•

2 types by location ➢ Perilobar ➢ Peripheral cortex or columns of Bertin Intralobar ➢ Deep cortex ➢ Greater risk of Wilms tumor

Nephrogenic Rests [Figure 6-7-10] Nephroblastomatosis: Imaging • • •

Findings vary with “burden” Small lesions may be inapparent Larger lesions ➢ Multifocal cortical nodules & masses ➢ Nephromegaly with confluent solid peripheral rind

Nephroblastomatosis: Imaging • • •

Nephrogenic rests. Pathology. Small cortical rests in peripheral cortex (arrowheads) and larger Wilms tumor in deep cortex

US: Hypo-, iso-, or hyperechoic masses or diffuse renal enlargement CT: Poorly enhancing low attenuation confluent subcortical rind or peripheral nodules MRI: Low signal on T1, iso- or slightly increased signal on T2

Diffuse Nephroblastomatosis [Figure 6-7-11]

Figure 6-7-11

Diffuse Nephroblastomatosis •

Clue is peripheral tissue rind

Diffuse Nephroblastomatosis

Diffuse nephroblastomatosis. Confluent peripheral soft tissue tumor rind, which may involve both cortex and medulla or the subcapsular space

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Nephroblastomatosis:Cortical Nodules [Figure 6-7-12]

Figure 6-7-12

Focal nephroblastomatosis. Longitudinal sonogram and contrast-enhanced CT and MR showing small peripheral nodules. The peripheral distribution is the clue to the diagnosis

Nephroblastomatosis: Cortical Nodule

Figure 6-7-13

Nephroblastomatosis: Treatment • • • •

Controversial Chemotherapy in some centers Close imaging surveillance for enlarging masses in others Renal sparing surgery

Rhabdoid Tumor of Kidney • • • •

2% of childhood renal neoplasms Arises from renal medulla Mean age 16 months (90% cases < 3yrs) Synchronous CNS lesions (10%) ➢ Metastases ➢ Primary neuroectodermal tumor, typically posterior fossa

Rhabdoid Tumor: Pathology [Figure 6-7-13] •

•

Pathology ➢ Large mass (<300 gm) ➢ Infiltrating Histology ➢ Mononuclear cells ➢ Eccentric nuclei and eosinophilic cytoplasm

Rhabdoid Tumor of Kidney: Radiologic features • • •

Heterogeneous soft tissue mass Calcification: 66% Peripheral low density crescent: 70% ➢ Characteristic but non-diagnostic ❖ Mesoblastic nephroma

Rhabdoid tumor. Gross pathology showing large intrarenal mass arising in medulla and infiltrating parenchyma. Histology, mononuclear cells with eccentric nuclei

Rhabdoid Tumor •

Clue: peripheral low density collection

Figure 6-7-14

Bilateral Rhabdoid Tumors [Figure 6-7-14]

Rhabdoid tumor. Two CT scans showing a large necrotic mass in the left kidney with a peripheral low density crescent (arrow). Also noted is a solid mass in the right kidney and multiple hepatic metastases Pediatric Radiology

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Rhabdoid Tumor with PNET Figure 6-7-15 Rhabdoid Tumor: Outcome • •

•

Poor prognosis Highly aggressive tumor ➢ mets 80% at diagnosis ➢ lung, liver, brain, nodes 20% survival @ 18 months

Clear Cell Sarcoma (Bone metastasizing renal tumor of childhood) • • • •

4% of pediatric renal neoplasms Peak incidence 2nd year of life Arises from medulla Aggressive tumor ➢ Mets to bone (40%-75%), lung, nodes Clear cell sarcoma. Solid mass with infiltrating margins

Clear Cell Sarcoma: Pathology [Figure 6-7-15] •

•

Pathology ➢ Solid, white surface ➢ Infiltrating margins Histology ➢ Clear cytoplasm ➢ Vascular stroma

Figure 6-7-16

Clear Cell Sarcoma: Imaging Features [Figure 6-7-16] • •

• •

Heterogeneous intrarenal mass Cystic changes (70%) ➢ dilated tubules ➢ mucoid substance Indistinct margins Bone metastases

Clear Cell Sarcoma Clear Cell Sarcoma •

Clear cell sarcoma. Large infiltrating mass in the left kidney with cystic areas

Cystic Change

Clear Cell Sarcoma: Outcome • •

Treatment is nephrectomy & chemotherapy Survival rates 60%-70%

Figure 6-7-17

Mesoblastic Nephroma • •

• • •

5% of all renal tumors Diagnosis in neonatal period ➢ mean age, 2 months Incidental mass May be diagnosed in utero Other findings ➢ hypertension (renin) ➢ hypercalcemia (parathormone)

Mesoblastic Nephroma: Path [Figure 6-7-17] • • •

Firm, rubbery, yellow-gray Unencapsulated Hemorrhage & necrosis uncommon

Renal Tumors

Mesoblastic nephroma, gross pathology. Solid rubbery mass with minimal hemorrhage. The tumor replaces most of the kidney

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Mesoblastic Nephroma: Histology • • •

Figure 6-7-18

Classic pattern: mature spindle cells Cellular pattern: increased mitoses, potentially more aggressive May entrap glomeruli & tubules

Mesoblastic Nephroma: Imaging • • •

Homogeneous mass Cystic changes rare Well circumscribed

Mesoblastic Nephroma • •

Mimics Wilms tumor Clue: patient age

Mesoblastic Nephroma [Figure 6-7-18] Mesoblastic Nephroma: Therapy and Prognosis • • •

•

Most behave in benign fashion Cured by nephrectomy Metastases & recurrence very rare ➢ Associated with atypical cellular histology Overall prognosis excellent

Mesoblastic nephroma. Longitudinal sonogram and CT showing soft tissue mass replacing renal parenchyma. Histologic specimen showing mature spindle cells. Gross specimen showing classic solid tumor without necrosis or hemorrhage

Figure 6-7-19

Ossifying Renal Tumor of Infancy • • • • •

Very rare!! < 20 cases reported Age range 6 days-14 months Most patients < 4 months Small, 2-3 cm diameter Typically present with hematuria

Cancer 1980; 45: 609-612

Ossifying Renal Tumor [Figure 6-7-19] • • • •

Arises in medulla Ill-defined margins Extends into collecting system Obstructs collecting system

Ossifying Renal Tumor of Infancy •

Plump, ovoid spindle cells with associated osteoid & bone production

Ossifying renal tumor, gross pathology. Medullary mass involving collecting system with associated hydronephrosis

Ossifying Renal Tumor of Infancy: Imaging Features • •

Central solid mass causing hydronephrosis Calcification (ossification) 80% ➢ May mimic a staghorn calculus

Figure 6-7-20

Ossifying Renal Tumor of Infancy [Figure 6-7-20]

Ossifying renal tumor of infancy. Noncontrast scan (left panel) showing soft tissue mass with calcification. Enhanced CT scan (right panel) showing a central tumor (T) with associated hydronephrosis Pediatric Radiology

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Ossifying Renal Tumor of Infancy:Treatment & Prognosis • • •

Managed surgically Apparently benign No reported cases of malignant spread or recurrence to date

Cystic Masses •

•

< 5 years of age ➢ Multilocular cystic nephroma ➢ Multicystic dysplastic kidney > 5 years of age ➢ Simple renal cysts (rare)

Figure 6-7-21

Multilocular Cystic Renal Tumor: Clinical Findings •

• •

Biphasic age distribution ➢ Boys 3 months to 2 years ➢ Women > 40 years Usually asymptomatic mass Pain & hematuria from ureteral prolapse of cyst

Multilocular Cystic Renal Tumor [Figure 6-7-21] • • •

Composed of cysts & septa Encapsulated Mean diameter 7 to 10 cm

Multilocular cystic renal tumor. Gross pathology showing multiple fluidfilled locules with surrounding septations

Multilocular Cystic Renal Tumor Histologic Features •

Two grossly identical but histologically distinct lesions ➢ Cystic nephroma (CN) ➢ Cystic partially differentiated nephroblastoma (CPDN)

Figure 6-7-22

Cystic nephroma (CN) [Figure 6-7-22] • • •

Peak age, 18 months Cysts 2 to 5mm Mature renal elements in septa ➢ No blastema

Cystic Partially Differentiated Nephroblastoma (CPDN) [Figure 6-7-23] • • •

Mean age, 12 months Cysts, 2 to 3 mm Immature elements in septa ➢ Usually blastema

Multilocular Cystic Renal Tumors:Imaging • • • •

Cystic nephroma, histology. Fibrous septa with differentiated tubules in septa

Figure 6-7-23

Cystic, fluid-filled mass Water density, signal intensity Variable thickness septations Septations enhance, but not fluid contents

Multilocular Cystic Renal Tumor (CN) •

Septa enhance

Multilocular Cystic Renal Tumor Cystic partiallly differentiated nephroblastoma, histology. Immature blastemal cells in septa

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Multilocular Cystic Renal Tumor [Figure 6-7-24]

Figure 6-7-24

Multilocular Cystic Renal Tumor: Therapy & Prognosis • •

Both cystic and partially differentiated forms usually cured by complete resection Partially differentiated tumor has potential for aggressive behavior & recurrence ➢ warrants follow-up imaging

Multilocular Cystic Renal Tumor: Differential Diagnosis •

•

Multicystic dysplastic kidney ➢ Non-functioning ➢ Entire kidney involved ➢ Conservative treatment, not surgery Usually found in neonates

Multilocular cystic renal tumor. CT and gross path (left panel) showing multicystic mass with septations. T1-weighted (middle panel) and gadolinium contrast enhanced image (right panel) also showing a multicystic mass

Figure 6-7-25

Multicystic Dysplastic Kidney [Figure 6-7-25]

Multicystic Dysplastic Kidney -CT Fat-containing Masses:Angiomyolipoma • • • •

Any age, but more often after 5 years Associated with tuberous sclerosis 80% of tuberous sclerosis patients have angiomyolipomas Imaging: ➢ Bilateral fatty, renal masses ➢ Solitary lesions, very rare

Multicystic dysplastic kidney. Gross path and longitudinal sonogram showing multiple cysts of variable size with no normal renal tissue

Figure 6-7-26

Angiomyolipoma: Imaging [Figure 6-7-26] Tumors of Older Children - Renal Cell Cancer • • •

•

< 2% of all renal cell neoplasms Mean age, 9 years Clinical findings ➢ flank pain, hematuria, mass Imaging findings: ➢ solid renal mass ➢ average diameter, 4 cm

Renal Cell Cancer: Pathology - Classic Clear Cell Cancer • •

Non-specific mass with hemorrhage and necrosis Tumor cells with clear cytoplasm, arranged in nests

Angiomyolipomas, tuberous sclerosis. Transverse sonogram showing highly echogenic kidney. CT, multiple fatty tissue masses. The presence of fat is the clue to the diagnosis

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Renal Cell Cancer •

Figure 6-7-27

Clue: small lesion, older pt age

Renal Cell Cancer [Figure 6-7-27] Renal Cell Cancer: Outcome •

•

Metastases at diagnosis: 30% ➢ 75% lung ➢ other sites- lymph nodes, bone, liver Prognosis ➢ regional spread: 75% survival ➢ vascular invasion or metastases: 0 to 10% survival

Renal cell cancer, 10 year old boy. Two contrast-enhanced CT scans showing a solid intrarenal mass with associated perinephric hemorrhage

Lymphoma • • • •

Secondary involvement from direct extension or hematogenous spread Non-Hodgkin >> Hodgkin disease Occurs late in course of disease Usually asymptomatic

Figure 6-7-28

Lymphoma: Imaging Patterns • • • •

Multiple or solitary nodules (80%) Diffuse infiltration Direct invasion Perinephric involvement

Renal Lymphoma: Nodules [Figure 6-7-28]

Summary: Lesions you need to know •

•

< 5 years of age ➢ Nephroblastomatosis ➢ Rhabdoid tumor ➢ Clear cell sarcoma ➢ Ossifying renal tumor of infancy ➢ Mesoblastic nephroma > 5 years of age ➢ Renal cell cancer ➢ Lymphoma

Lymphoma, multiple appearances. Multiple nodules, top left image. Solitary nodule, lower left image. Perinephric tumor, top right image. Diffuse infiltration, bottom right image.

References 1. 2. 3. 4. 5. 6. 7. 8.

Siegel MJ. Urinary Tract. In: Siegel MJ, ed. Pediatric Sonography, 3rd ed. Lippincott Williams & Wilkins. Philadelphia. 2002; 385-473. Siegel MJ. The Kidney. In: Siegel MJ, ed. Pediatric Body CT. Philadelphia, Lippincott Williams & Wilkins, 1999; 226-252. Siegel MJ. MRI of the pediatric abdomen. MRI Clin North Am 1995; 3:161-182. Geller E, Smergel E, Lowry P. Renal neoplasms of childhood. Rad Clin North Am 1997; 35:1391-1413. Green DM, Coppes MJ, Breslow NE, et al. Wilms tumor. In: Pizzo PA, Poplack DG, (eds). Principles and Practice of Pediatric Oncology, 3rd ed. New York. Lippincott-Raven. 1997; 733-759. Lowe LH, Isuani BH, Heller RM, et al. Pediatric renal masses: Wilms tumor and beyond. RadioGraphics 2000; 20:1585-1603. Navoy JE, Royal SA, Vaid YN, Mroczek-Musulman EC. Wilms’ tumor: unusual manifestations. Pediatr Radiol 1995; 25:S76-S DeBaun MR, Siegel MJ, Choyke PL. Nephromegaly in infancy and early childhood: a risk factor for Wilms tumor in Beckwith-Wiedemann syndrome. J Pediatr 1998; 132:401-404.

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9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19.

Lonergan GJ, Martinez-Leon MI, Agrons GA, Montemarano H, Suarez ES. Nephrogenic rest, nephroblastomatosis, and associated lesions of the kidney. Radiographics 1998; 18:947-968. Rohrschneider WK, Weirich A, Rieden K, Darge K, Troger J, Graf N. US, CT, and MR imaging characteristics of nephroblastomatosis. Pediatr Radiol 1998; 28:435-443 Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: a comparative study of 21 cases. AJR 1997; 168:447-45 Chung CJ, Lorenzo R, Rayder S, Schemankewitz E, Guy CD, Cutting J, Munden M. Rhabdoid tumors of the kidney in children: CT Findings. AJR 1995; 164:697-700. Kabala JE, Shield J, Duncan A. Renal cell carcinoma in childhood. Pediatr Radiol 1992; 22:203-205. Davidson AJ, Choyke PL, Hartman DS, Davis CJ, Jr. Renal medullary carcinoma associated with sickle cell trait: radiology findings. Radiology 1995; 195:83-85 Chepuri NB, Strouse PJ, Yanik GA. CT of renal lymphoma in children. AJR 2003; 180:419-431. Hugosson C, Mahr MA, Sabbah R. Primary unilateral renal lymphoblastic lymphoma. Pediatr Radiol 1997; 27:23-25 Wooten SL, Rowen SJ, Griscom NT. Congenital mesoblastic nephroma. RadioGraphics 1991; 11:719-721 Agrons GA, Wagner BJ, Davidson AJ, Suarez ES. Multilocular cystic renal tumor in children: radiologicpathologic correlation. RadioGraphics 1995; 16:653-669. Hopkins JK, Giles HW, Wyatt-Ashmead J, Bigler SA. Cystic nephroma. Radiographics 2004; 24:589-593

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Pediatric Adrenal Masses Marilyn J. Siegel, MD Objectives • • •

Discuss differential diagnoses of adrenal masses in neonates and children Describe imaging features of common adrenal masses in a pediatric population Describe pitfalls in diagnosis

Adrenal Masses of Childhood: Differential Considerations •

•

Neoplastic ➢ Medullary tumors ➢ Adrenocortical neoplasms ➢ Metastases Non-neoplastic ➢ Hemorrhage ➢ Congenital hyperplasia ➢ Storage disorders

Figure 6-8-1

Adrenal Medullary Tumors •

Neoplastic tumors ➢ Neuroblastic tumors ❖ Neuroblastoma ❖ Ganglioneuroblastoma ❖ Ganglioneuroma ➢ Pheochromocytoma

Neuroblastic Tumors: Histogenesis • • •

Arise from neural crest tissue Involve adrenal medulla or anywhere in sympathetic chain Spectrum of differentiation and biologic behavior

Spectrum of neuroblastic tumors, histology. Neuroblastoma (NB) (left panel), characterized by immature small blue cells. Ganglioneuroblastoma (GNB) (middle panel) containing neuroblasts and mature cells (gangliocytes). Ganglioneuroma (GN) (right panel) composed of mature gangliocytes and mature stroma

Neuroblastic Tumors: Spectrum of Histology •

• •

Neuroblastoma (NB): immature cells ➢ Small round blue cells Ganglioneuroblastoma (GNB): both neuroblasts & mature cells (gangliocytes) Ganglioneuroma (GN): mature gangliocytes & mature stroma

Neuroblastic Tumors: Histology [Figure 6-8-1] Neuroblastic Tumors: Spectrum of Catecholamine Production •

• • •

Less mature tumors more active than mature tumors ➢ 90% of all tumors produce catecholamines Vanillylmandelic acid (VMA) - metabolite of epinephrine & norepinephrine Homovanillic acid (HVA) - metabolite of dopamine Vasoactive intestinal peptide (VIP) - elaborated by ganglion cells

Neuroblastoma: Epidemiology • • • • • •

Most common extracranial solid neoplasm of childhood 2nd most common abdominal malignancy (after Wilms tumor) 10% of pediatric cancers 500-525 new cases/yr in the US Mean age ~ 2 yrs. 75% < 5 yrs.

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Neuroblastoma: Symptoms • •

Figure 6-8-2

Symptoms are age dependent Infants < 6 mos: ➢ Skin nodules ❖ blueberry muffin syndrome ➢ Hepatomegaly (liver mets) ❖ Pepper syndrome ➢ Respiratory compromise ❖ from massive liver ➢ Abdominal mass is small & may not be palpable

Blueberry Muffin Syndrome Neuroblastoma: Symptoms • • •

Infant > 6 months Palpable abdominal mass 75% have systemic symptoms ➢ bone pain ➢ hepatomegaly ➢ paraplegia ➢ opsoclonus, ataxia ➢ diarrhea (VIP production) Distribution of neuroblastoma

Neuroblastoma: Location [Figure 6-8-2] • • • • •

Abdomen: 75% Thorax: 20% Neck: 1%-5% Pelvis: 2%-3% Unknown primary: 1% ➢ Present with mets

Figure 6-8-3

Neuroblastoma: Pathology [Figure 6-8-3] • • •

Mean size 6-8 cm Hemorrhage and necrosis common Path-deep red to gray-white to tan appearance

Neuroblastoma: Histology •

Small blue cell tumors with occasional cluster of cells arranged in rosettes

Neuroblastoma: Genetic Associations •

•

N-myc oncogene ➢ Located on distal end of chromosome 2p ➢ Multiple copies (n-myc amplification) associated with aggressive tumor behavior Deletion of short arm of chromosome p1 ➢ More aggressive tumor behavior

Neuroblastoma, gross pathology. Large solid extrarenal mass, not encapsulated

Role of Imaging • • •

Identification of primary tumor Determination of extent of local disease Detection of distant metastases

Abdominal Neuroblastoma: Spectrum of Imaging Features Neuroblastoma: Approach to Diagnosis • • •

Screening abdominal US (if palpable mass) Chest X-ray CT &/or MRI following abnormal US or X-ray

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• •

MIBG Bone marrow aspirate/biopsy

Figure 6-8-4

Neuroblastoma: Imaging Spectrum •

•

Typical findings ➢ Infants (> 6 mos) & children ➢ Predominantly solid mass Atypical findings ➢ Neonates ➢ Predominantly cystic

Neuroblastoma: Typical US Findings • • • • •

Extrarenal mass Irregular margins Heterogeneous 90% Homogeneous 10% Mixed pattern reflects high cellularity, dystrophic calcification and necrosis

Neuroblastoma: US [Figure 6-8-4] • •

Typical appearance infants & children Predominantly solid mass

Neuroblastoma: Typical CT Findings • • • • • •

Neuroblastoma. Typical appearance in infants and children. Solid extrarenal mass with small cystic areas and/or calcification

Extrarenal mass Smooth or irregular margins Density less than adjacent tissues No definable capsule Midline extension Calcifications 85%

Figure 6-8-5

Neuroblastoma [Figure 6-8-5] • •

25% of cases ipsilateral No midline extension

Neuroblastoma [Figure 6-8-6] •

75% of cases-midline extension

Neuroblastoma: Atypical Findings • •

• •

Neonates Predominantly cystic tumor ➢ degenerative change or microcysts Appearance non-specific, mimics hematoma Diagnosis requires evidence of metastatic disease, + VMA analysis or serial US

Neuroblastoma. Two CT images showing a well-defined, right adrenal mass containing calcification and displacing the kidney inferiorly. The mass is localized to the right abdomen and does not cross the midline

Figure 6-8-6

Neuroblastoma. Three CT scans showing a large left adrenal mass with irregular margins and areas of necrosis. The tumor crosses the midline, encases vessels, and displaces the left kidney laterally

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Neonatal Neuroblastoma [Figure 6-8-7]

Figure 6-8-7

Neuroblastoma: MRI •

• •

T1-weighted images: ➢ low signal intensity (black) Fat-suppressed images: ➢ high signal intensity (bright) Contrast enhances

Neuroblastoma: MRI [Figure 6-8-8] Local Extension: Diagnostic Questions • • • •

Midline extension (> 30%) (see Fig. 6) Vascular encasement (>30%) Liver metastases (5-10%) Intraspinal extension (15%)

Midline Extension: Vascular Encasement [Figure 6-8-9]

Neonatal neuroblastoma. Predominantly cystic mass arising in the right adrenal gland, reflecting necrosis, hemorrhage or intrinsic cystic changes

Spinal Invasion [Figure 6-8-10] Cervicothoracic Neuroblastoma: Imaging Features Figure 6-8-10 Figure 6-8-8

Neuroblastoma. T1-weighted axial image (left panel) showing a low signal intensity mass with high signal intensity areas representing hemorrhage. T2-weighted image (middle panel) showing a high signal intensity mass. Coronal gadolinium-enhanced image (left panel) showing an enhancing suprarenal mass

Figure 6-8-9

Neuroblastoma. Transverse CT scan (top panel) and sagittal STIR (bottom panel) images showing intraspinal tumor extension (arrows) Neuroblastoma. T2-weighted axial (left panel) and coronal STIR images (right panel) showing large left adrenal mass, which crosses the midline and encases the aorta, renal vessels and superior mesenteric artery Pediatric Radiology

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Thoracic Neuroblastoma [Figure 6-8-11]

Figure 6-8-11

Thoracic Neuroblastoma • • • • • •

Posterior mediastinum Fusiform shape Soft tissue density Calcifications (50%) Extends over several interspaces Osseous erosions (rib/vertebra)

Thoracic Neuroblastoma [Figure 6-8-12] Thoracic neuroblastoma. Posterior mediastinal mass

Figure 6-8-12

Figure 6-8-13

Pelvic neuroblastoma. Transverse and sagittal CT scans show a large presacral pelvic mass (M) which invades the spinal canal (arrows)

Thoracic neuroblastoma. Transverse, coronal and sagittal CT images showing a right paraspinal mass with a fusiform shape extending over several vertebral body levels

Thoracic Neuroblastoma: MRI - Intraspinal Extension Pelvic Neuroblastoma [Figure 6-8-13] Neuroblastoma Metastases: Age Dependent Pattern •

•

< 6 months ➢ Liver (usually diffuse) ➢ Skin ➢ Bone marrow > 6 months ➢ Cortical bone & bone marrow ➢ Liver (solitary or diffuse) ➢ Lymph nodes

Figure 6-8-14

Hepatic Metastases (5%-10%) • •

[Figure 6-8-14]

Infant with diffuse liver mets Pepper syndrome Neuroblastoma. Transverse hepatic sonogram showing diffuse parenchymal heterogeneity. Transverse CT showing diffuse hepatic metastases and a small right primary tumor (M).

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Hepatic Metastases Distant Metastases: Skeleton Imaging Studies (sensitivity) • •

• • • •

Bone scintigraphy: 90% Metaiodobenzylguanidine (MIBG) ➢ I-123 MIBG: 95% In-111 pentetreotide Total body MRI: > 95% FDG PET: 100% X-ray: 35%-70%

Figure 6-8-15

Sites for Skeletal Metastases • • • • •

Metaphyses of long bones Calvarium-dura Ribs & vertebral bodies Flat bones I.e., the sites of red marrow

Neuroblastoma, bone metastases. Plain radiographs (left panel) showing lytic metaphyseal lesions. Bone scintigraphy (right panel) showing increased radionuclide activity in metaphyseal ends of the long bones

Neuroblastoma: Radionuclide Imaging Sensitivity • •

•

Primary tumor 35-90% Skeletal metastases ➢ Radionuclide 90% ➢ X-ray 35-70% ❖ lytic or permeative Metastases are usually asymmetric and metaphyseal in location

Skeletal Metastases: Bone Scintigraphy [Figure 6-8-15] Split Suture Sign - Dural Metastases MIBG Scintigraphy [Figure 6-8-16] • • • •

Figure 6-8-16

Norepinephrine analogue I-123 MIBG Sens: 80% to 95% Sens >> bone scan

Skeletal Metastases • •

MRI FDG-PET

Staging •

• •

• • •

Neuroblastoma. MIBG scintigraphy. Increased activity in right adrenal mass and in skeleton

Stage 1: ➢ Localized tumor confined to area of origin; complete excision Stage 2A: ➢ Unilateral tumor with incomplete excision; ipsilateral nodes negative microscopically. Stage 2B: ➢ Unilateral tumor complete or incomplete excision; positive ipsilateral nodes Stage 3: ➢ Tumor across midline; or unilateral tumor with contralateral node involvement; or midline tumor with bilateral lymph node involvement Stage 4: ➢ Spread to distant lymph nodes, bone, bone marrow, liver, or other organs Stage 4S: ➢ Unilateral primary tumor with spread limited to liver, skin and/or bone marrow. ➢ Limited to infants < 1 year of age.

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Neuroblastoma: Stage Distribution [Figure 6-8-17]

Figure 6-8-17

Neuroblastoma: Treatment • •

•

Resectable disease: surgery Unresectable disease: ➢ chemotherapy Radiation therapy for tumor that does not regress with chemotherapy

Neuroblastoma: Overall Survival • • • • •

Stage 1: 94% Stage 2: 90% Stage 3: 64% Stage 4: 24% Stage 4S: 75%

Distribution of stages of neuroblastoma. Majority of patients have advanced disease at time of diagnosis

Favorable Outcome: Associated Factors • • • • •

Low stage Young patient age (< 1 yr) No N-myc amplification No chromosome 1p Stroma-rich

Neuroblastoma: Outcome- 3-year survival • • •

•

Stage & age of patient at diagnosis most important predictors of outcome Stages 1, 2, & 4S: 75%-90% Children < 1 year ➢ Stage 3 disease: 80%-90% ➢ Stage 4 disease: 60%-75% Children > 1 year ➢ Stage 3: 50% ➢ Stage 4: 15%

Figure 6-8-18

Differential Diagnosis •

• • •

Other neuroblastic tumors ➢ Ganglioneuroblastoma ➢ Ganglioneuroma Pheochromocytoma Adrenocortical tumors Metastases

Other neuroblastic tumors. Ganglioneuroblastoma (left panel). Ganglioneuroma (right panel). The appearance overlaps that of neuroblastoma

Other Neuroblastic Tumors [Figure 6-8-18] •

•

Ganglioneuroblastoma ➢ Neuroblasts & ganglion cells Ganglioneuroma ➢ Mature ganglion cells

Neurofibroma Pheochromocytoma • • •

•

Mean age: 11 yr, (range 6-18 yr) 10% malignant Bilateral 20%: associated with: ➢ MEN-IIA & IIB ➢ von Hippel-Lindau syndrome ➢ von Recklinghausen syndrome (NF1) Clinical ➢ Paroxysmal hypertension, headaches, diaphoresis

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Pheochromocytoma: Locations •

•

Figure 6-8-19

97% abdominopelvic ➢ Adrenal (90%) ➢ Paraganglioma (10%) ❖ Organ of Zuckerkandl ❖ Retroperitoneum ❖ Pelvis Thorax (rare site)

Pheochromocytoma: Gross Path [Figure 6-8-19] • • • •

Rounded solid mass 3-5 cm Hemorrhage &necrosis common Rarely purely cystic

Pheochromocytoma: Imaging • • • • •

Cystic changes, hemorrhage common US: Solid circumscribed mass with variable heterogeneity CT: Heterogenous mass (when large), enhances MR imaging: high signal T2-WT image MIBG avid

Pheochromocytoma [Figure 6-8-20]

Pheochromoctyoma. Gross pathology showing a round cystic mass with hemorrhagic contents

Adrenocortical Tumors • • • • •

Rare in children Adrenal carcinoma most common Mean patient age: 9 years Hormonally active producing virilization, less often Cushing syndrome Adenomas rare

Figure 6-8-20

Adrenocortical Cancer: Path & Imaging [Figure 6-8-21] • • • • • •

Large solid mass (mean, 6 cm) Well circumscribed Lobulated surface Cystic areas of hemorrhage and necrosis common Calcification 20% Mets to liver, lung, bone

Adrenocortical Cancer [Figure 6-8-22]

Pheochromocytoma. Longitudinal sonogram showing an echogenic suprarenal mass (M). Coronal T2-weighted image showing a high signal intensity mass (M) with central necrosis

Figure 6-8-21

Adrenocrotical cancer. Cut section shows a solid mass with areas of hemorrhage and necrosis Pediatric Radiology

Figure 6-8-22

Adrenal cancer. Longitudinal sonogram (left panel) showing a solid adrenal mass. Coronal T1-weighted image (middle panel) showing a large, left suprarenal mass with necrosis. T2-weighted image (right panel) showing a high-signal intensity mass with areas of necrosis 1407 1409

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Adrenal Metastases • • •

Figure 6-8-23

Rare in children Lymphomatous involvement may occur Nonspecific uniform solid adrenal mass

Adrenal Masses of Childhood: Differential Considerations •

•

Neoplastic ➢ Medullary tumors ➢ Adrenocortical neoplasms ➢ Metastases Non-neoplastic—neonatal diseases ➢ Hemorrhage ➢ Congenital hyperplasia ➢ Storage disorders

First you need to know------US of Normal Adrenal • • • • •

[Figure 6-8-23]

Echogenic medulla Hypoechoic cortex Inverted V or Y shape Mean length, 15 mm Mean width, 3 mm

Adrenal Hemorrhage • • •

• •

Most common neonatal adrenal mass Result of passive venous engorgement during delivery Predisposing conditions: ➢ birth trauma, hypoxia, IDM, coagulopathy, renal vein thrombosis, sepsis Does not cause adrenal insufficiency R > L (3-4:1), may be bilateral

Normal adrenal gland. Upper panel: V shaped adrenal gland. Lower panel Y-shaped adrenal gland. Note: hypoechoic cortex, echogenic medulla

Figure 6-8-24

Adrenal Hematoma •

Clinical findings ➢ Abdominal mass ➢ Anemia ➢ Jaundice

Adrenal Hematoma: US •

• • •

Appearance varies with age of hematoma ➢ Day1-2: echogenic or complex mass (fibrin,debris) ➢ Day 3 to 2 wks: complex (liquefaction) ➢ Later: cystic (hypo- or anechoic) Shrinkage within 1 to 2 weeks May calcify as early as 1 week Associated caval thrombus

Adrenal Hematoma [Figures 6-8-24 and 6-8-25]

Adrenal Masses

Adrenal hematoma. Longitudinal sonograms on day 1 (left upper panel), on day 12 (left lower panel), at 6 weeks (right upper panel) and at 2 months (right lower panel) showing changes in echogenicity and near complete resolution of a right adrenal hematoma

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Differential Diagnosis: Neuroblastoma •

• •

Figure 6-8-25

Neuroblastoma does not liquefy ➢ (but may spontaneously regress) Neuroblastoma makes catecholamine byproducts US surveillance to document resolution of hemorrhage (may take 6 to 8 weeks)

Adrenal Hematoma •

Calcified hematomas

Congenital Adrenal Hyperplasia • • • •

Adrenal hematoma. Left panel. Day 3, complex suprarenal mass. Right panel, day 10, nearly complete involution

Autosomal recessive enzymatic deficiency 21-hydroxylase deficiency most common (>90%) Androgen overproduction ➢ virilism in girls ➢ premature masculization in boys ➢ advanced somatic development in both sexes Aldosterone underproduction ➢ salt wasting crisis

Figure 6-8-26

Congenital Adrenal Hyperplasia: Pathology [Figure 6-8-26] • • •

Bilateral adrenal enlargement with cerebriform appearance Mean length > 20 mm Mean width > 4 mm

Congenital Adrenal Hyperplasia: Imaging • • •

Congenital adrenal hyperplasia. Gross section showing large bilateral adrenal glands with cerebriform pattern

Adrenal enlargement Wavy contour Cerebriform appearance

Woman Disease • • •

Figure 6-8-27

Rare inborn lysosomal acid lipase deficiency Cholesterol esters accumulate in all organs Presents in infancy, death in 1 year

Woman Disease: Imaging • • • •

Marked adrenomegaly Preserved adreniform contour Punctate or coarse calcifications Hepatosplenomegaly

Wolman Disease [Figure 6-8-27] Adrenal Tumor-Neonate: Hemorrhage or Neuroblastoma? •

• •

•

Enlarged adrenal gland? ➢ Both Cystic and/or solid? ➢ Both Complete involution? ➢ Hemorrhage Liquefies? ➢ Hemorrhage

Pediatric Radiology

Wolman disease. Plain radiographs (upper panels) showing enlarged, calcified adrenal glands. Longitudinal sonogram (left lower panel) showing a suprarenal mass with marked shadowing. CT (right lower panel) showing bilateral calcified adrenal glands. 1409 1411

Adrenal Masses

Adrenal Tumor-Neonate: Hyperplasia or Wolman Disease •

• •

•

•

Figure 6-8-28

Enlarged adrenal gland? ➢ Both Solid? ➢ Both Cerebriform pattern? ➢ Hyperplasia Calcifies? ➢ Wolman Hormonally active? ➢ Hyperplasia

The Spectrum: Neonate [Figure 6-8-28]

Adrenal Tumor-Infant & Older Child: Neuroblastoma, Cancer, Pheo •

• •

•

•

Solid mass? ➢ All Cystic changes? ➢ All Bone mets? ➢ Neuroblastoma Virilization? ➢ Adrenal cancer Paroxysmal hypertension ➢ Pheochromocytoma

Neonatal adrenal lesions. Neuroblastoma (upper left panel). Wolman (lower left panel). Hemorrhage (upper right panel). Congenital adrenal hyperplasia (lower right panel)

Spectrum of Adrenal Lesions: US - Infant & Older Child [Figure 6-8-29]

Spectrum of Adrenal Lesions: CT - Infant & Older Child Figure 6-8-29

[Figure 6-8-30]

The Need to Know Adrenal Masses •

•

Neoplastic ➢ Medullary tumors ❖ Neuroblastic ❖ Pheochromocytoma ➢ Adrenocortical cancer Non-neoplastic ➢ Hemorrhage ➢ Congenital hyperplasia ➢ Wolman disease

Adrenal lesions infant and older children, ultrasound. Neuroblastoma (left panel). Pheochromoctyoma (middle panel). Cancer (right panel)

Figure 6-8-30

Adrenal lesions infant and older children, CT. Neuroblastoma (left panel). Pheochromoctyoma (middle panel). Cancer (right panel)

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References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17.

Siegel MJ. Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ (ed), Pediatric Sonography, 3rd. Philadelphia. Lippincott Williams & Wilkins. 2002; 475-527. Siegel MJ. Adrenal glands, pancreas and other retroperitoneal structures. In: Siegel MJ (ed), Pediatric Body CT. Philadelphia. Lippincott-Williams & Wilkins. 1999; 253-286. Westra SJ, Zaninovic AC, Hall TR, Kangarloo H, Boechat MI. Imaging of the adrenal gland in children. RadioGraphics 1994; 14:1323-1340. Abramson SJ. Adrenal neoplasms in children. Radiol Clin North Am 1997; 35:1415-1453. Brodeur GM, Maris JM. Neuroblastoma. In: Devita VT, Hellman S, Rosenberg SA, eds. Cancer Principles and Practice of Oncology. Lippincott Williams & Wilkins. Philadelphia. 2001; 895-933. Lonnergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma and ganglioneuroma: radiologic-pathologic correlation. Radiographics 2002; 22:911-934. Berdon W, Ruzal-Shapiro C, Abramson S. The diagnosis of abdominal neuroblastoma: Relative roles of ultrasonography, CT and MR. Urol Radiol 1992; 14:252-262 Cassady C, Winter WD. Bilateral cystic neuroblastoma: imaging features and differential diagnoses. Pediatr Radiol 1997; 27:758-759. Teoh SK, Whitman GJ, Chew FS. Neonatal neuroblastoma. AJR 1997; 168:54. Meyer JA, Harty MP, Khademian Z. Imaging of neuroblastoma and Wilms’ tumor. Magn Reson Imaging Clin 2002; 10:275-302. Siegel MJ. MR imaging of pediatric abdominal neoplasms. MRI Clin North Am 2000; 8:837-851 Gelfand M J. Meta-iodobenzylguanidine in children. Semin Nucl Med 1993; 23: 231-242 Shulkin BL, Wieland DM, Baro ME, et al. PET hydroxyephedrine imaging of neuroblastoma. J Nuc Med 1996; 37:16-21. Argons GA, Lonergan GJ, Dickey GD, Perez-Monte JE. Adrenocortical neoplasms in children: radiologicpathologic correlation. Radiographics1999; 19:989-1008. Riberio J, Ribeiro RC, Fletcher BD. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 2000; 30:45-51. Sivit CJ, Hung W, Taylor GA, Catena LM, Brown-Jones C, Kushner DC. Sonography in neonatal congenital adrenal hyperplasia. AJR 1991; 156:141–143. Özmen MN, Aygün N, Kiliç I, Kuran L, Yalçin B, Besim A. Wolman’s disease: ultrasonographic and computed tomographic findings. Pediatr Radiol 1992; 22:541–542.

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Pediatric Pelvic Masses Marilyn J. Siegel, MD • • •

Figure 6-9-1

Objectives Review normal anatomy Discuss causes of pelvic masses Describe imaging features of common and some uncommon pelvic lesions

“To recognize the abnormal, one must first know the normal” J. Caffey • •

Ovarian volume Uterine volume

• • • • •

Neonate (1day-3 mos) Infant (4-12 mos) Early childhood (2-8 yrs) Late childhood (9-14 yrs) Pubertal

Normal Maturation: Growth

Age vs. Ovarian Volume

•

1.1 cm3 0.7 cm3 0.8-1.1 cm3 2.2-4.2 cm3 9.8 cm3

Ovarian Maturation: Morphology

•

Prepubertal ovary ➢ Usually homogeneous ➢ But may be heterogeneous due to presence of primordial follicles (< 1cm) Pubertal ovary ➢ Usually heterogeneous ➢ Reflects presence of primordial & functional follicles (1- 3cm)

• •

Presence of cysts is normal in infants & children Do not confuse for pathology

• • •

10 follicles stimulated each cycle 1 becomes dominant Grows to 15 to 30 mm

Ovarian Morphology: Need to Know

Normal prepubertal ovary. Two 2year-old girls. Left panel: The ovary is relatively homogeneous. Right panel: Longitudinal sonogram showing multiple small follicles, measuring less than 9 mm in diameter. B = bladder

Figure 6-9-2

Normal Maturation

Normal Prepubertal Ovary: US [Figure 6-9-1]

Normal Pubertal Ovary: US [Figure 6-9-2]

Normal pubertal ovaries. Left panel: Longitudinal sonogram on day 10 of the menstrual cycle shows multiple follicles less than 10 mm in diameter (calibers=right ovary). Right panel: Longitudinal sonogram on day 20 of the menstrual cycle shows a dominant follicle (arrow), measuring 18 mm in length. This likely represents a corpus luteum cyst

Pediatric Pelvis Masses

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Normal Ovary: CT and MR [Figure 6-9-3]

Figure 6-9-3

•

Uterine Morphology: Maturation Prepubertal uterus ➢ tubular shape ➢ fundus equal to cervix ➢ endometrial canal seen in neonates, not in infants and children • Pubertal uterus Normal ovaries. Left panel: CT scan of a 6 year old girl. The ➢ fundus elongates and thickens ovaries are not well seen. Middle panel: CT scan of a 13-year➢ fundus larger than cervix old girl shows homogeneous right ovary (arrow) and a small ➢ endometrial canal again present developing follicle, measuring 2.5 cm diameter, in the left ovary. Right panel. Fat-saturated T2 weighted MR of an adolescent Age vs. Uterine AP Diameter girl showing multiple high signal intensity follicles in both • Neonate (1day-3 mos) 2.1 cm ovaries (arrowheads) • Infant (4-12 mos) 0.8 cm • • •

Early childhood (2-8 yrs) Late childhood (9-14 yrs) Pubertal

0.7 cm 1.4 cm 1.6-3 cm

Figure 6-9-4

Normal Uterus: US [Figure 6-9-4] Normal Uterus: CT and MR [Figure 6-9-5]

•

Pelvic Mass Lesions •

•

Anterior Pelvis ➢ Ovarian ➢ Bladder and lower genital tract Posterior Pelvis (Presacral) ➢ Neurogenic tumors ➢ Teratomas

Pelvic Masses: Imaging Approach • • •

Normal uterine morphology. Left panel: Neonatal uterus. Longitudinal sonogram shows a tubular uterus (arrowheads) with prominence of the uterine fundus and a thin, hyperechoic endometrial stripe. Middle panel: A 2-year-old girl. The uterus is small and tubular with no differentiation between fundus and cervix and no recognizable endometrial stripe. Right panel. Sonogram showing a pear-shaped uterus with a fundus that is larger than the cervix. The endometrial stripe (calipers) is again visualized and varies in thickness with the phase of the menstrual cycle

US is screening examination of choice for most clinically suspected masses CT is study of choice for evaluation of gynecologic, bladder and prostate lesions shown on sonography MRI usually reserved for presacral masses

Ovarian Masses

•

•

Ovarian Tumors: First Key Point •

Figure 6-9-5

Cystic ➢ Follicular cysts ➢ Paraovarian cysts ➢ Cystic neoplasms ❖ Teratoma, cystadenoma Solid ➢ Malignant germ cell tumors ➢ Sex cord-stromal tumors

Epithelial tumors are extremely rare in the 1st two decades ➢ Put them low on the list THINK SIMPLE CYSTS, GERM CELL TUMORS, OR STROMAL TUMORS

Pediatric Radiology

Normal uterus. Left panel: CT scan of a 5-year-old girl. The uterus (arrow) is seen as a small oval soft tissue structure. Middle panel: Normal pubertal uterus. CT scan of a 15-yearold girl shows an oval uterine fundus that demonstrates zonal differentiation-- higher attenuation myometrium and endometrium and the lower attenuation endometrial canal. Right panel: T2-weighted image from a 12-year-old girl shows normal zonal anatomy--endometrial complex (e), junctional zone (arrow), and outer myometrium (m)

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Pediatric Pelvis Masses

• • •

Follicular Cysts

Figure 6-9-6

Most common ovarian mass Over stimulated follicle Imaging findings ➢ fluid-filled lesion ➢ unilocular ➢ thin-walled ➢ avascular ➢ 3 to 8 cm

Functional Ovarian Cyst: US [Figure 6-9-6]

Functional Ovarian Cyst CT/MR [Figure 6-9-7]

•

Hemorrhagic Ovarian Cyst: US

• •

Functional ovarian cysts, US. Left panel: Sonogram showing a 6-cm anechoic cyst (C) with imperceptible walls and sound transmission. Right panel: Color Doppler sonogram of another patient with an ovarian cyst (C). The cystic contents show no color signal. Minimal flow is noted in the adjacent parenchyma

US Findings ➢ Complex mass (>85%) ❖ Septations, fluid-debris level ➢ Hyperechoic mass (<15%) (early) ➢ Acoustic transmission Doppler: avascular Painful masses

Figure 6-9-7

Hemorrhagic Ovarian Cysts: Spectrum of US Features [Figure 6-9-8]

Hemorrhagic Cysts: CT/ MRI •

[Figure 6-9-9]

•

CT ➢ High density ➢ “fluid-fluid” level MR ➢ Mixed signal ➢ “fluid-fluid” level

Functional ovarian cysts, CT and MR. CT (left panel) showing a 6 cm low attenuation cyst (C) arising from the right ovary displacing the bladder (B) to the left. Fat-saturated T2weighted image (right panel) showing a high signal intensity, right ovarian cyst, measuring 5 cm in diameter. The normal zonal anatomy of the uterus--endometrial complex, inner junctional zone, and outer myometrium-- is noted

Figure 6-9-8

Figure 6-9-9

Hemorrhagic ovarian cysts. Different adolescent patients. Left panel: Early hemorrhage. A hyperechoic cyst (arrow) with acoustic enhancement. Ut = uterus. Middle panel: Late hemorrhage. A complex cyst (C), measuring 5 cm in diameter, with internal echoes and septations. Right panel: A complex mass (arrows) with fluid-debris level

Hemorrhagic ovarian cyst, CT/ MRI. Upper image: CT showing high attenuation right ovarian cyst (arrow). Lower image: T2-weight fat-saturated MR showing mixed signal intensity cyst (arrows) with a blood-fluid level (arrowhead). U = uterus Pediatric Pelvis Masses

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• •

Follicular & Hemorrhagic Cysts: More Points •

Most cysts regress within 2 cycles If the cyst remains for > 3 cycles, likely not functional ➢ Suspect paraovarian cyst or neoplasm Cysts > 6 cm should be followed

Hemorrhagic Ovarian Cyst: Serial US to document resolution • • •

DDX: Follicular Cysts

• •

Paraovarian cysts Teratoma Cystadenoma

Figure 6-9-10

Paraovarian Cysts

• • •

Arise in broad ligament or fallopian tube Imaging: ➢ round or oval ➢ fluid-filled No cyclic changes Do not regress Can make diagnosis if cyst is separate from ovary

Paraovarian Cysts (Fallopian Tube) Paraovarian Cyst [Figure 6-9-10] •

Paraovarian cyst. Left panel: Longitudinal sonogram showing a large cyst adjacent to a normal right ovary (arrow). BL = bladder. Right panel: Fat-saturated T2-weighted axial MR image showing a high signal intensity cyst separate from both ovaries

Cystic Neoplasms: Ovarian Teratoma • • • • •

Most common ovarian tumor ➢ 95% of ovarian neoplasms 90% benign 75% asymptomatic ➢ 25% pain due to torsion Bilateral 8% to 15% Large, mean diameter 15 cm Usually adolescent girls

Figure 6-9-11

Mature Teratoma: Pathology •

[Figure 6-9-11]

•

•

Gross ➢ Cystic mass ➢ Foci of fat, Ca++, bone Histology ➢ Respiratory, GI elements ➢ Sebaceous glands, hair skin ➢ Muscle, cartilage ➢ No malignant elements

Ovarian teratoma, pathology. Cut section showing cystic teratoma containing small nidus of calcification posteriorly (arrow). Histologic section showing sebacous glands and skin

Cyst Ovarian Teratoma: Imaging

• •

90% predominantly cystic ➢ >>50% fluid contents ➢ Minimal soft tissue ❖ mural nodule ❖ septations ➢ Foci of fat & Ca++ 10% contain only fat & hair contents Avascular

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Cystic Ovarian Teratoma: US [Figure 6-9-12]

Figure 6-9-12

Multiple Benign Teratomas: CT [Figure 6-9-13]

Teratoma 15 yo girl with abdominal distention Ovarian Teratoma: MRI • • • • •

Ovarian Cystadenoma

• • • •

Epithelial tumor < 5% neoplasms, benign Mucinous >> serous 4 to 20 cm diameter Epithelial linings ➢ Simple (serous type) ➢ Columnar, mucinous (mucinous type)

Mature teratomas, US. Different patients. Left panel: Longitudinal sonogram showing a predominantly cystic mass with a an echogenic mural nodule (dermoid plug) (arrow). B=bladder; UT=uterus. Right panel: Sonogram showing a complex cystic mass containing large echogenic mural nodule anteriorly (arrow) with acoustic shadowing

Figure 6-9-13

Cystadenoma: Imaging [Figure 6-9-14] Usually large Cystic Multilocular with septations Sometimes unilocular ➢ Particularly serous type

Serous Cystadenoma • • •

Ovarian Malignancies

• • • •

Germ cell tumors (85%) Stromal tumors (15%) Epithelial tumors

Malignant Germ Cell Tumors 10% of germ cell tumors Immature: neural (brain) tissue Malignant: frank malignant elements Predominantly soft tissue (>50%) plus fat and calcium

Mature ovarian teratomas, CT. Spectrum of appearances

Malignant Ovarian Tumors: Path [Figure 6-9-15]

Figure 6-9-15

Figure 6-9-14

Mucinous cystadenoma. Longitudinal sonogram showing a multilocular mass. CT showing a cystic mass with enhancing septations

Pediatric Pelvis Masses

Malignant ovarian tumors, pathology. Left panel, dysgerminoma. Right panel, malignant teratoma. Malignant tumors typically have lobulated surfaces and contain predominantly solid elements with areas of necrosis and hemorrhage

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• •

Malignant Germ Cell Neoplasms: Clinical & Imaging

• •

Pelvic or abdominal masses Imaging findings suggestive of malignancy: ➢ solid or complex mass ❖ > 50% soft tissue elements ➢ thick, irregular walls Nonspecific findings: > 10 cm size, Ca++ Metastasize to lymph nodes and liver ➢ (rarely to omentum or mesentery)

Dysgerminoma [Figure 6-9-16]

Figure 6-9-16

Dysgerminomas. Left panel: Longitudinal sonogram showing a large predominantly solid mass with hypoechoic areas representing necrosis. Middle and right panels. Different patients. Contrast-enhanced CT scans showing a large mostly solid mass with cystic components

Malignant Teratoma Clue: Predominantly Solid Mass

Figure 6-9-17

Endodermal Sinus Tumor (Yolk Sac Cancer) • • •

Sex Cord-Stromal Tumors (15%)

•

Prepubertal girls Granulosa theca cell & Sertoli-Leydig Hormonally active ➢ Estrogens (Granulosa-Theca) ➢ Androgens (Sertoli-Leydig) Can be malignant or benign ➢ Spread to peritoneum and liver

Sex cord stromal tumors, pathology. Left panel: granulosa theca cell tumor. Right panel: SertoliLeydig tumor. Both tumors appear as large, predominantly solid mass with varying size areas of necrosis

Sex Cord-Stromal Tumors: Pathology [Figure 6-9-17]

•

Figure 6-9-18

Sex Cord-Stromal Tumors: Imaging • •

Granulosa cell tumors ➢ mixed solid-cystic mass with thick, irregular septations Sertoli-Leydig tumors ➢ homogeneous solid mass or mixed solid-cystic mass Metastases, although rare, are to peritoneal surfaces and liver

Granulosa-theca Cell Tumor [Figure 6-9-18]

Sertoli-Leydig cell tumor [Figure 6-9-19]

Granulosa-theca cell tumor. 5 year-old girl with breast development and vaginal bleeding. Transverse sonogram showing a heterogenous solid right ovarian mass with cystic areas. CT showing a low attenuation mass with irregular septations

Figure 6-9-19

Setoli-Leydig cell tumor. Young girl with virilization. Two transverse CT scans showing a heterogeneous mass with solid and cystic (necrotic) areas. The appearance mimics malignant germ cell tumors, but the diagnostic clue is hormonal activity Pediatric Radiology

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• • •

Ovarian Cancer Rare lesion in pediatric population Epithelial origin Imaging ➢ Solid mass ➢ Often necrotic ➢ Smaller in size than germ cell tumors ➢ Spreads to mesentery and omentum

Ovarian Cancer (<5% of tumors) •

Which Ovarian Tumor? • •

•

•

•

Cystic with mural nodules? ➢ Teratoma Cystic with septations? ➢ Cystadenoma Solid with cystic elements? ➢ Malignant germ cell tumors, stromal tumors Older age (pubertal)? ➢ Germ cell tumors, Cystadenoma Younger age? ➢ Stromal tumors

Figure 6-9-20

Uterine Masses •

•

Cystic ➢ hydrocolpos Solid ➢ rhabdomyosarcoma

Hydrocolpos • •

Vaginal obstruction ➢ due to stenosis or membrane Result is pelvic/abdominal mass Imaging findings ➢ Fluid-filled midline mass ➢ Well defined walls ➢ Internal debris or blood ❖ (hemato- or hematometrocolpos)

Hydrocolpos-hematocolpos, US. Left panel. Neonate. Longitudinal sonogram showing dilated fluid-filled vagina (V) outlining the cervix (C). B = bladder. Right panel. Hematocolpos. Longitudinal sonogram showing a dilated vagina (V) with low level central echoes, representing blood. Uterus (U) is normal

Hydrocolpos—Hematocolpos [Figure 6-9-20] Hydrocolpos: CT & MRI [Figure 6-9-21] • •

Rhabdomyosarcoma • •

Most common pelvic malignancy Bimodal age distribution: ➢ 2 to 6 and 14 to 16 years Embryonal cell type Sites: ➢ Head/Neck 38% ➢ GU 21% ➢ Extremity 18%

Figure 6-9-21

Hydrocolpos, CT and MR. CT showing a dilated fluid-filled vagina (V). Axial T2-weighted MRI showing high-signal intensity fluid in the vaginal canal (V). B = bladder Pediatric Pelvis Masses

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• • •

Rhabdomyosarcoma: Female Pelvis

•

Figure 6-9-22

Arises in vagina Vaginal bleeding Imaging findings ➢ Soft tissue mass ➢ Enlarged pelvic nodes Mets to liver, lung, node and bone

Vaginal Rhabdomyosarcoma [Figure 6-9-22] Rhabdomyosarcoma: Male Bladder & Prostate •

•

Bladder ➢ Trigone or bladder base ➢ Polypoid mass Prostate gland ➢ Solid mass ➢ Deforms base of bladder ➢ Elongates prostatic urethra

Vaginal rhabdomyosarcoma. Sagittal T1-weighted and fat-suppressed T2-weighted images showing a soft tissue mass filling the vaginal canal (arrow). (Case courtesy of Mary E. McCarville, MD, Memphis, TN)

Prostatic Rhabdomyosarcoma [Figure 6-9-23] Rhabdomyosarcoma: Prostate Bladder Rhabdomyosarcoma • • • • •

Bladder Masses: Differential Diagnosis

Figure 6-9-23

Rhabdomyosarcoma Pheochromocytoma Neurofibroma Transitional cell Leiomyosarcoma

Which Lower Genital Tract Tumor? •

•

•

YOU DON’T NEED CLUES ➢ Rhabdomyosarcoma Solid, infiltrative? ➢ Rhabdomyosarcoma

Presacral Masses •

• •

Cystic ➢ benign teratoma ➢ meningocele Solid ➢ malignant teratoma ➢ neuroblastoma

Prostatic rhabdomyosarcoma. CT scans showing an enlarged prostate gland (P) The planes between the mass and right obturator internus muscles are obliterated. Invasion of pelvic sidewalls confirmed at surgery

Sacrococcygeal Teratoma • • • •

CA++: Malignancy:

60% 10% newborn 90 > 2 months Location: 45 % external 45% external & internal 10% presacral Bony defect: very low frequency Arise from coccyx Do not invade spinal canal

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Sacrococcygeal Teratoma •

Sacrococcygeal Teratoma: Imaging • •

Figure 6-9-24

Benign teratomas ➢ Contain predominantly fluid or fat ➢ Sometimes Ca++ Malignant teratomas ➢ Predominantly solid Fed by sacrococcygeal and iliac arteries

Benign Sacrococcygeal Teratoma Sacrococcygeal Teratoma Benign SC Teratoma [Figure 6-9-24] •

Anterior Meningocele • •

Herniation of spinal contents through a congenital defect in a vertebral body Scimitar shaped sacrum CT/MR ➢ absent sacral elements ➢ tethered cord

Anterior Meningocele •

Benign sacrococcygeal teratoma. T1-weighted MR (left panel) showing a predominantly fat-containing mass with small fluidfilled nodules. Fat-saturated T2-weighted MR (right panel). The fatty tissue has decreased in signal intensity. Fluid components are bright. The tumor arises from the coccyx (arrow)

Presacral Masses •

Figure 6-9-25

Cystic ➢ benign teratoma ➢ meningocele Solid ➢ malignant teratoma ➢ neuroblastoma

Malignant SC Teratoma [Figure 6-9-25] Neuroblastoma [Figure 6-9-26] •

Which Presacral Tumor? • •

•

Cystic Mass, normal spine? ➢ Teratoma Cystic mass, abnormal spine? ➢ Anterior meningocele Solid with normal spine ➢ Malignant teratoma Solid with spinal canal invasion ➢ Neuroblastoma

Malignant sacrococcygeal teratoma. Transverse CT scan (left panel), T1-weighted MR (middle panel) and T2-weighted MR (right panel) showing a large presacral mass with a predominance of soft-tissue components

Figure 6-9-26

Presacral neuroblastoma. CT showing a soft-tissue mass (M) anterior to the sacrum and posterior to the bladder (BL). The tumor has invaded the right obturator fossa (arrow). Sagittal STIR image showing a presacral soft-tissue mass (M) extending into the spinal canal (arrowheads) and displacing the bladder superiorly. F = foley catheter Pediatric Pelvis Masses

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•

Lateral Pelvic Masses •

• •

Adenopathy ➢ Lymphoma Neurofibroma

Neurofibromas • •

•

Nerve sheath tumors Often plexiform in the pelvis ➢ multiple or interlacing masses Affect about 5%-15% of people with NF1 Imaging ➢ Multiple soft tissue masses ➢ Target sign T2 MR ➢ Enlarged sacral foramen

Plexiform Neurofibromatosis Clue: enlarged sacral foramen

Neurofibromatosis: T2 MR Lymphadenopathy • • • • • • • • • •

Top 10 Pelvic Lesions: What you need to know Functional ovarian cyst Ovarian teratoma Malignant ovarian tumors Sex cord stromal tumors Hydrocolpos Rhabdomyosarcoma Sacrococcygeal teratoma Anterior meninogocele Presacral neuroblastoma Neurofibromatosis (NF1)

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13.

Garel L, Dubois J, Grignon A, Filiatrault D, Van Vliet G. US of the pediatric female pelvis: a clinical perspective. Radiographics 2001; 21:1393-1407. Siegel MJ. Female pelvis. In: Siegel MJ, ed. Pediatric Sonography, 3rd ed. Philadelphia. Lippincott Wiliams & Wilkins. States LJ, Bellah RD. Imaging of the pediatric female pelvis. Semin Roentgenol 1996; 31:312-329. Boechat IN. MR imaging of the pediatric pelvis. MRI Clin North Am. 1996; 4:679-697. Rigsby CK, Siegel MJ. CT appearance of pediatric ovaries and uterus. J Comput Assist Tomogr 1994; 18:72-76. Siegel MJ. Pelvic organs and soft tissues. In: Siegel MJ, ed. Pediatric Body CT. Philadelphia, Lippincott Williams and Wilkins, 1999, pp 287-311. Siegel MJ. Magnetic resonance imaging of the adolescent female pelvis. Mag Reson Imaging Clin North Am 2002; 10:303-324. Baltarowich OH. Female pelvic organ measurements. In: Goldberg BB, Kurtz AB, eds. Atlas of Ultrasound Measurements. Chicago. Year Book Medical Publishers. 1990; 190-242. Cohen HL, Eisenberg P, Mandel F, Haller JO. Ovarian cysts are common in premenarchal girls: a sonographic study of 101 children 2-12 years old. AJR 1992; 159:89-91. Siegel MJ. Pelvic tumors. Radiol Clin North Am 1997; 35:1455-1475. Fried AN, Kenney CM III, Stigers KB, Kacki MH, Buckley SL . Benign pelvic masses: sonographic spectrum. RadioGraphics 1996; 16:321-334 Kim JS, Woo SK, Suh SJ, Morettin LB. Sonographic diagnosis of paraovarian cysts: value of detecting a separate ipsilateral ovary. AJR 1995; 164:1441-1444 Jabra AA, Fishman EK, Taylor GA. Primary ovarian tumors in the pediatric patient: CT evaluation. Clin Imaging 1993;17:199-203.

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14. Quillin SP, Siegel MJ. CT features of benign and malignant teratomas in children. J Comput Assist Tomogr 1992; 16:722-726. 15. Castleberry RP, Cushing B, Perlman E, Hawkins EP. Germ cell tumors. In: Pizzo PA, Poplack DG, et al. Principles and Practice of Pediatric Oncology. Philadelphia: Lippincott Raven 1997; 921-945. 16. Outwater EK, Wagner BJ, Mannion C, McLarney JK, Kim B. Sex-cord-stroma and steroid cell tumors of the ovary. Radiographics 1998; 18:1523-1546. 17. Blask ARN, Sanders RC, Gearhart JP. Obstructed uterovaginal anomalies: demonstration with sonography. Part I neonates and infants. Radiology 1991; 179:79-83. 18. Blask ARN, Sanders RC, Rock JA. Obstructed uterovaginal anomalies: demonstration with sonography. Part II teenagers. Radiology 1991; 179:84-88 19. Argons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Genitourinary rhabdomyosarcoma in children: radiologic-pathologic correlation. RadioGraphics 1997; 17:919-937. 20. Fletcher BD, Kaste SC. Magnetic resonance imaging for diagnosis and follow-up of genitourinary, pelvic, and perineal rhabdomyosarcoma. Urol Radiol 1992; 14:262-272. 21. Argons GA, Wagner BJ, Lonergan GJ, Dickey GE, Kaufman MS. Genitourinary rhabdomyosarcoma in children: radiologic-pathologic correlation. RadioGraphics 1997; 17:919-937.

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Bone Marrow Imaging Marilyn J. Siegel, MD Lecture Objectives • • • •

Review normal bone marrow anatomy Discuss MRI marrow techniques Describe features of normal marrow on MRI Recognize causes of pathologic marrow on MRI

• • • •

We all have it It shows up on every MRI It is an important site of pathology We need to know the normal before we can recognize the abnormal

Why study bone marrow?

M. Siegel, “Stating the Obvious” •

Why Study Bone Marrow? It Has Clinical Applications • •

MRI has become the imaging modality of choice for evaluating marrow changes. MRI can help characterize the composition of marrow or marrow process in question MRI provides excellent anatomic detail of surrounding structures, boundaries of disease

PART I: Bone Marrow Constituents • • •

Bone Marrow: 3 Components

•

Osseous Matrix Red Marrow Yellow Marrow

Figure 6-10-1

Marrow Components

•

Osseous matrix ➢ (aka cancellous or spongy bone) ➢ Provides support for cellular components

Marrow Components •

Red Marrow ➢ Cellular, active or myeloid marrow ➢ Composed of red & white blood cells & platelets Yellow Marrow ➢ Inactive or fatty marrow ➢ Composed of fat cells

Normal marrow constituents. Histologic section showing hematopoietic and yellow marrow

The Major Marrow Constituents: Histology [Figure 6-10-1] Red and Yellow Marrow: Different Chemical Composition

Red

Yellow

Water

40

15

Fat

40

80

• MRI appearance of marrow reflects the relative fractions of red & yellow marrow

Protein 20

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Bone Marrow Imaging

•

Bone Marrow Vascularity •

Red ➢ Rich sinusoidal vascular supply ➢ Favors metastases, infection Yellow ➢ Sparse vascular supply ➢ Favors infarction

PART II: MRI Techniques • •

PART II: Technique • •

Depends on clinical indication Dedicated MRI of limited body part is used in the evaluation of localized pain or to follow a focal lesion Whole body MRI is used for staging, restaging and surveillance

Basic Pulse Sequences

•

For imaging pathology, these are your main sequences: ➢ Spin echo T1 ➢ STIR or T2 with fat suppression

Whole-body MRI

•

Technique ➢ Vertex to toes ➢ Coronal plane ❖ Sagittal plane Table moves 4-6 times ➢ Total imaging time ❖ ~ 7-20 minutes

Lauenstein, Radiology 2004;233:139 • • • •

Whole-body MRI: Results

•

51 patients. 43 with metastases Reference standards: CT and bone scan All brain, lung, liver metastases > 6 mm seen Small lung metastases missed ➢ did not change therapeutic strategies Whole-body MR imaging on per-patient basis -- 100% sensitivity & specificity values

Lauenstein, Radiology 2004;233:139

Part III: MR Features of Normal Marrow • • •

Normal Marrow: T1

• • • •

This is your “Fat Finder” sequence Yellow marrow will be bright due to abundant fat composition Red marrow contains much less fat, and will be intermediate in signal. (But enough fat not to be dark!!!!)

Normal Marrow: Fat Suppression

•

STIR & T2 with fat saturation Both sequences exhibit T2 weighting, while suppressing fat signal Red Marrow remains intermediate Yellow marrow is dark, as fat is suppressed

Normal Marrow Imaging: T2 •

T2 weighted imaging causes both red and yellow marrow to be intermediate in intensity, making them look similar Not a widely used sequence

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•

Part III: MRI Features of Normal Marrow

•

•

Figure 6-10-2

Red Marrow ➢ T1-weighted: dark (> muscle < fat) ➢ STIR/FS: mildly bright (>muscle < fat) Yellow Marrow ➢ T1-weighted: bright (= fat) ➢ STIR/FS : black (< muscle)

What About Gadolinium? • • • •

Used if lesion is identified on T1 or fat suppressed images Increases lesion conspicuity Worthwhile to remember that normal adult red & yellow marrow does not enhance Red marrow in neonate enhances Pathologic lesions enhance

Normal red marrow. T1-weighted (left panel) and fat-saturated T2-weighted (right panel) images. Hematopoietic marrow in the pelvis and femora exhibits a signal intensity equal to that of muscle

Figure 6-10-3

Normal Red Marrow Signal: Neonate • •

In neonate, marrow has minimal lipid content Red marrow = muscle

•

Red > muscle

[Figure 6-10-2]

Red Marrow Signal: Older Child [Figure 6-10-3] Normal Yellow Marrow Signal [Figure 6-10-4]

• •

Review: Red/Yellow Signal Intensity (T1)

• •

Yellow > red > muscle Unossified cartilage is equal to muscle

Review: Red/Yellow Signal Intensity (STIR) Red > muscle > yellow Unossified cartilage is bright on T2, due to its “watery” content

Red marrow signal intensity. Red marrow has signal intensity slightly higher than that of muscle on T1-WT and STIR images, reflecting greater fraction of lipids. Yellow epiphyseal marrow has signal intensity identical to that of subcutaneous fat on both sequences

MR Normal Marrow: Variations in Distribution • • • •

Conversion of red to yellow marrow occurs during growth and development and has a predictable and orderly pattern You need to know this to avoid mistakes in diagnosis

Figure 6-10-4

Patterns of Marrow Distribution Neonate: virtually all red marrow Shortly after birth red-to-yellow marrow conversion begins ➢ Overall: extremities to axial ➢ In a given bone: epiphysis/apophysis ? diaphysis --> metaphysis

Marrow Conversion Appendicular to Axial Conversion Custer. J Lab Clin Med 1932 • •

Long Bone Conversion

Normal yellow marrow. T1-weighted image (left panel) and fat-saturated T2-weighted image (Right panel). Yellow marrow has a signal intensity similar to subcutaneous fat on both sequences

Distal to proximal in individual bones Epiphysis to diaphysis to metaphysis

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• •

Marrow Distribution [Figure 6-10-5] Adult distribution by age 25 years From nearly 100% red marrow at birth to 40% red marrow in adulthood

Figure 6-10-5

Vogler JB III. Radiology 1998; 168:679-693. •

Variations in Marrow Distribution • •

A common EXCEPTION to the rule that red marrow converts to yellow marrow is that the epiphyses & apophyses completely convert to yellow marrow once they ossify Controversial whether they ever contain red marrow

Variations in Marrow Distribution Another exception is that red marrow can persist in the proximal humeral and femoral epiphyses of adults

• •

Epiphysis: subchondral, curvilinear focus Other patterns are likely abnormal

• • •

Yellow marrow around basivertebral vein common in children Other patterns common in adults Islands of fat can mimic metastases

Variations in Red Marrow Distribution

Bone Marrow Variations: Vertebral Marrow [Figure 6-10-6]

Ricci C. Radiology 1990;177:83-88.

Normal Vertebral Marrow: MRI • • • •

Summary Key Points: Criteria for Normal Marrow

• • • •

Shows expected signal intensities on all image sequences Shows expected distribution in the skeleton for patient age Is symmetric % of fatty marrow increases with age

PART IV: Marrow Pathology

• • • •

Reconversion Replacement Depletion Vascular – mediated lesions ➢ Edema ➢ Ischemia

Adult marrow distribution. Yellow marrow predominates in the appendicular skeleton. Red marrow is found mainly in the skull, flat bones, spine, proximal metaphyses of both the humeri and femora

Marrow Reconversion Opposite of conversion Results when increased demand for hematopoiesis Generally symmetric Causes: ➢ Chronic anemias (sickle cell, thalassemia) ➢ Increased O2 needs (altitude, athletes, smokers) ➢ Granulocyte colony stimulating factor ➢ Cyanotic heart disease

•

Axial skeleton responds first, followed by extremities

• •

Reverse order from normal conversion process! Proximal to Distal!!

Figure 6-10-6

Reconversion Marrow Reconversion

Bone Marrow Imaging

Vertebral marrow, red-yellow marrow distribution. Yellow marrow is located near basivertebral vein in neonates and young children (upper right diagram). Other patterns predominate in adolescents and adults 1426 1428

Pediatric Radiology

• •

Increased red cells No fat cells to generate signal

• • •

T1-WT images: dark signal intensity Red = or slightly > muscle STIR/Fat sat T2 images: slightly bright

Marrow Reconversion: Histology [Figure 6-10-7]

Figure 6-10-7

Marrow Reconversion: Sickle cell anemia

Reconversion: Sickle Cell Anemia [Figure 6-10-8] Reconversion: Thalassemia • • •

Granulocyte Colony Stimulating Factor Stimulates marrow hyperplasia Focal abnormality (mimics tumor) Clues to diagnosis ➢ onset 2 to 8 weeks after treatment ➢ increased white blood cell count ➢ affects metaphysis and diaphysis

G-CSF • • •

Marrow Replacement or Infiltration [Figure 6-10-9]

• • • •

Implantation of cells in marrow that do not normally exist there Follows red marrow distribution Causes ➢ Neoplastic (lymphoma, leukemia, mets) ➢ Inflammatory (osteomyelitis) ➢ Myeloproliferative (fibrosis) ➢ Lipidoses

Marrow reconversion. Histologic section showing predominance of red cell elements

Figure 6-10-8

Marrow Replacement T1 WT: dark STIR/Fat sat: very bright Can be diffuse or focal Predominates in red marrow (axial skeleton)

Diffuse Replacement Disorders: Leukemia [Figure 6-10-10]

Marrow reconversion. Two different patients. Coronal T1weighted image of the knees shows diffuse low signal intensity red marrow in the distal femoral and proximal tibial metaphyses. Scattered high signal intensity foci represent islands of yellow marrow and/or infarcts

Figure 6-10-10 Figure 6-10-9

Marrow replacement. The normal hematopoietic elements have been replaced by neoplastic cells, characteristic of chronic lymphocytic leukemia Pediatric Radiology

Marrow replacement by leukemia. Coronal T1-weighted image (left panel) shows diffusely low signal intensity marrow throughout the pelvis and femora. Fat-saturated T2weighted image (right panel). Replaced marrow has a signal intensity much brighter than that of muscle 1427 1429

Bone Marrow Imaging

Diffuse Replacement Disorders: Metastatic Disease • •

Focal Replacement: Metastases Metastases prefer red marrow Preferred sites: ➢ Vertebrae - 69% ➢ Pelvis - 41% ➢ Femur - 25% ➢ Skull - 14%

Vertebral Metastases: Breast Cancer •

Review: Hyperplasia or Tumor?

•

Distinguishing criteria: ➢ Red marrow: orderly distribution ❖ Tumor: random distribution ➢ Red marrow: usually symmetric bilaterally ❖ Tumor: usually asymmetric ➢ Red marrow: minimally bright on STIR/FS ❖ Tumor: extremely bright

Hyperplasia or Tumor? Fat Suppressed Images •

Normal red marrow ➢ SI: = or slightly > muscle Tumor infiltration ➢ SI: >>>>> muscle

Figure 6-10-11

• •

Acute compression due to osteopenia? Malignant compression fracture?

• • • • • •

Abnormal signal intensity May involve pedicle, posterior element or entire vertebral body Convex posterior cortex Epidural mass Paraspinal soft tissue mass Marrow enhancement post gadolinium

Pitfall: Compression Fractures

Malignant Compression Fracture

Pathologic Fracture •

Osteoporotic Fracture • • • • •

Partial body involvement ➢ Usually involves end plate Signal of spared marrow is normal Thoracolumbar junction Clustering of abnormalities Thin paraspinal soft tissue mass Improves in 6 to 8 weeks

Compression fractures Pathologic and Osteoporotic Fractures [Figure 6-10-11] • • •

Blastic and fibrotic lesions Low signal on T1 and fat -suppressed images Often heterogeneous

• • •

Osteomyelitis Myeloproliferative disorders Lipidoses

Variations in Appearance of Metastases

Vertebral fractures. Pathologic fracture (left panel). Gadoliniumenhanced T1-weighted MR shows abnormal signal intensity in entire vertebral body, convex posterior cortex, and small epidural mass. Osteoporotic fracture (right panel). T1weighted MR shows partial body involvement (upper end plates), normal marrow signal, and clustering of abnormalities at thoracolumbar junction

Other Infiltrative Processes with Low T1 and High T2 Signal

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Pediatric Radiology

•

Osteomyelitis [Figure 6-10-12] • • •

Figure 6-10-12

Infection produces increased cellularity and water content in marrow (edema) T1: dark signal FS: bright signal Adjacent ST changes ➢ edema

Vogler JB III. Radiology 1998; 168:679-693 • •

Osteomyelitis [Figure 6-10-13] T1: DARKER >> muscle. Water, cells replace marrow FST2: BRIGHTER >>muscle

Figure 6-10-13

Osteomyelitis. Inflammatory cells (I) have replaced normal marrow elements. T=supporting trabeculae

Figure 6-10-14 Osteomyelitis. Coronal T1-weighted image (left panel) showing low signal intensity area in distal femoral metaphysis. Fat-saturated T2weighted image (right panel) showing increased signal intensity

Osteomyelitis: MR • •

Myelofibrosis [Figure 6-10-14] •

Myelofibrosis. Marrow space contains abundant fibrotic tissue and sparse red cells

Marrow replacement by fibrosis Sequela of treated malignancy ➢ Rarer as primary disorder Results in lowering of signal intensity

Figure 6-10-15

Myelofibrosis [Figure 6-10-15] •

Mimics of Fibrosis • • • •

Gaucher disease ➢ Marrow replacement by glucocerebroside-laden cells Hemosiderin deposition ➢ Marrow replacement by iron ➢ Usually from transfusion therapy Decreased T1 and T2-weighted signal Slightly increased signal on STIR

Gaucher Disease • •

Due to deficiency of the enzyme acid betaglucocerebrosidase, which helps to break down glucosyl ceramide Follows distribution of red marrow Proximal to distal

Pediatric Radiology

Primary myelofibrosis. Coronal T1-weighted (left panel) and T2-weighted (right panel) MR images show low signal intensity marrow in the ilia, femora and vertebral bodies

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Bone Marrow Imaging

Gaucher Disease [Figure 6-10-16] •

Figure 6-10-16

Hemosiderin [Figure 6-10-17] Low SI on all sequences (black)

Marrow Depletion Fatty Replacement [Figure 6-10-18] • •

• •

Absence of red marrow Causes ➢ chemotherapy ➢ radiation therapy ➢ aplastic anemia Marrow has signal of fat

Radiation & Chemotherapy

• •

Gaucher disease. Decreased signal intensity on T1-weighted Typical changes images (left panel) and on STIR (right panel) images ➢ 1-2 days: edema ➢ 7-14 days: fatty replacement ➢ 3-4 wks: regenerating red marrow May see irreversible changes (fat or fibrosis) with high doses

Figure 6-10-17

Myeloid Depletion: MRI •

Signal follows fat ➢ high T1 ➢ dark with fat sat Usually see sharp cut off lines at radiation port

Andrews. Radiographics 2000 •

Myeloid Depletion [Figure 6-10-19] Follows signal intensity of fat

Figure 6-10-19

Hemosiderin deposition. Patient with sickle cell anemia who received transfusional therapy. T1-weighted (left panel) and T2-weighted (right panel) MR images showing diffusely low marrow signal intensity. Scattered high foci area represent residual fat and/or edema due to infarcts

Figure 6-10-18

Myeloid depletion following radiation treatment. Sagittal T1-weighted image shows diffuse high signal intensity fatty marrow in multiple vertebral bodies, which were included in the radiation port

Bone Marrow Imaging

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Marrow depletion. Marrow elements have been replaced by fatty tissue

Pediatric Radiology

• •

Myeloid Depletion

Figure 6-10-20

Pre-treatment Post-treatment

Vascular Mediated Disorders: Edema • • • • •

Results in increased extracellular water Signal changes are those of water T1: low signal STIR/FS: bright signal Regionally limited

Vascular Mediated Disorders: Ischemia (infarct) • • •

Marrow edema. Multiple causes. Bone bruise, left panel. Fracture, middle panel. Transient osteoporosis, right panel

Result of death of yellow & red marrow elements Signal changes represent balance of cell death and repair Regionally limited ➢ Subarticular ➢ Likes yellow marrow

Ischemia & Edema: Causes Not all individually detailed in this lecture Ischemia Steroids Sickle cell SLE Gaucher Pancreatitis Radiation •

Edema Trauma Stress facture Transient osteoporosis Regional migratory osteoporosis Reflex sympathetic dystrophy

CLUE: These favor yellow marrow

Marrow Edema (STIR): Common Causes [Figure 6-10-20] Marrow Ischemia: Avascular Necrosis [Figure 6-10-21] Figure 6-10-21 Bone Marrow Disorders

Hyperplasia

T1

STIR/FS

Dark

Intermediate

Replacement Dark

Bright

Fibrosis

Dark

Dark

Depletion

Bright

Dark

Vascular Mediated

Dark

Bright

Avascular necrosis.T1-weighted (left panel) and fat-saturated T2-weighted (right panel) MR images showing low signal intensity epiphyseal lesion. Ischemic injuries favor yellow marrow

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Bone Marrow Imaging

References 1. 2. 3. 4. 5. 6.

7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26.

Siegel MJ. MR imaging of paediatric haematologic bone marrow disease. J Hong Kong Coll Radiol 2000; 3:3850. Steiner RM, Mitchell DG, Rao VM, Schweitzer ME. Magnetic resonance imaging of diffuse bone marrow disease. Radiol Clin North Am 1993; 31:383-409. Vande Berg BC, Malghem J, Lecouvet FE, Maldague B. Magnetic resonance imaging of the normal bone marrow. Skeletal Radiol 1998;27:471-483 Vanel D, Dromain C, Tordivon A. MRI of bone marrow disorders. Eur Radiol 2000; 10:224-229. Vogler JB III, Murphy WA. Bone marrow imaging. Radiology 1998; 168:679-693. Eustace S, Tello R, DeCarvalho V, et al. A comparison of whole body turboSTIR MR imaging and planar 99mTcmethylene diphosphonate scintigraphy in the examination of patients with suspected skeletal metastases. AJR 1997; 169:1655-1661. Lauenstein TC, Goedhe SC, Herborn CU, et al. Three-dimensional volumetric interpolated breath-hold MR imaging for whole-body tumor staging in less than 15 minutes: a feasibility study. AJR 2002 Aug;179(2):445-9. Lauenstein TC, Goedhe SC, Herborn CU, et al. Whole-body MR imaging: evaluation of patients for metastases. Radiology 2004; 233:139-148. Mazumdar A, Siegel M, Narra V, Luchtman-Jones L. Whole-body fast inversion recovery MR imaging of small cell neoplasms in pediatric patients: a pilot study. AJR 2002; 179:1261-1266. O’Connell MJ, Hargaden G, Powell T, Eustace SJ. Whole-body short tau inversion recover MR imaging with a moving table top. AJR 2002; 179: 866-868. Padhani A, Husband J. Bone. In: Husband JES, Reznek RH, eds. Imaging in Oncology. Isis Medical Media. Oxford 1998; pgs 765-786. Kricun ME. Red-yellow marrow conversion: its effect on the location of some solitary bone lesions. Skeletal Radiol 1985; 14:10-19. Custer RP, Ahlfeldt FE. Studies of the structure and function of bone marrow: variations in cellularity in various bones with advancing years of life and their relative response to stimuli. J Lab Clin Med 1932; 17:960-962. Moore DG, Dawson KL. Red and yellow marrow in the femur: age-related changes in the appearance at MR imaging. Radiology 1990; 175:219-223. Ricci C, Cova M, Kang YS et al. Normal age-related patterns of cellular and fatty bone marrow distribution in the axial skeleton: MR imaging study. Radiology 1990; 177:83-88. Taccone A, Oddone M, Dell’ Acqua A, Occhi M, Ciccone MA. MRI “road-map” of normal age-related bone marrow. Pediatr Radiol 1995; 25:596-606. Mirowitz SA. Hematopoietic bone marrow within the proximal humeral epiphysis in normal adults: investigation with MR imaging. Radiology 1993; 188:689-93. Fletcher BD, Wall JE, Hanna SL. Effect of hematopoietic growth factors on MR images of bone marrow in children undergoing chemotherapy. Radiology 1993; 189:745-751. Ryan SP, Weinberger E, White KS, et al. MR imaging of bone marrow in children with osteosarcoma: effect of granulocyte colony-stimulating factor. AJR 1995; 165:915-920. Shellock FG, Morris E, Deutsch AL, et al. Hematopoietic bone marrow hyperplasia: high prevalence on MR images of the knee in asymptomatic marathon runners. AJR 1992; 335-338. Baker LL, et al. Benign versus pathologic compression fractures of vertebral bodies: assessment with conventional spin-echo, chemical shift, and STIR MR imaging. Radiology 1990; 174:495 Blomlie V, Rofstad EK, Skjonsberg A, Tverå, Lien HH. Female pelvic bone marrow: serial MR imaging before, during, and after radiation therapy. Radiology 1995; 194:537-543. Otake S, Mayr N, Ueda T et al. Radiation-induced changes in the MR signal and contrast enhancement of lumbosacral vertebrae: do changes occur only inside the radiation therapy field? Radiology 2002; 222:179-183. Stevens SK, Moore SG, Kaplan I. Early and late radiation changes in the spine: magnetic resonance imaging. AJR 1990; 154:745-750. Hermann G, Shapiro RS, Abdelwahab IF, Grabowksi G. MR imaging in adults with Gaucher disease type I: evaluation of marrow involvement and disease activity. Skeletal Radiol 1993; 22:247-251. Levin TL, Sheth SS, Hurlet A, Comerci SC, Ruzal-Shapiro, Piomelli S, Berdon WE. MR marrow signs of iron overload in transfusion-dependent patients with sickle cell disease. Pediatr Radiol 1995; 25:614-619.

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Congenital Lung Malformations Marilyn J. Siegel, MD • • •

Learning Objectives

•

Review clinical & pathologic features of common lung anomalies Describe imaging appearances of the common congenital anomalies Emphasize imaging differential diagnosis

Congenital Lung Anomalies

•

• •

Normal vascularity ➢ Lobar emphysema ➢ Cystic adenomatoid malformation ➢ Bronchogenic cyst ➢ Parenchymal agenesis, hypoplasia Abnormal vascularity ➢ Scimitar syndrome ➢ Sequestration ➢ Arteriovenous malformation

Figure 6-11-1

Congenital Lobar Emphysema

• • •

Misnomer; true emphysema not present Congenital lobar emphysema. Gross section showing an Infantile lobar “emphysema” = overinflated lobe that fails to deflate post resection. Histology “overinflation” showing alveolar overdistention Progressive lobar air-trapping from bronchial obstruction; ➢ deficiency of bronchial cartilage ➢ intraluminal web Onset: 50% 1st week, 90% < 6 months Neonates--dyspnea, cyanosis, cough Later-asymptomatic, wheezing

• • •

Sponge-like mass, fails to deflate on resection Compressed normal lung deflates & reexpands Histo- Alveolar distention 5-10X normal

•

Congenital Lobar Emphysema: Pathologic Features [Figure 6-11-1]

• • • •

Figure 6-11-2

Lobar Emphysema: Imaging

• •

Lobar hyperinflation Atelectatic adjacent lung Initially opaque if retained lung fluid Mass effect: mediastinal shift, attenuated vessels Lobar predilection: ➢ LUL (50%), RML (24%), RUL (18%)

Lobar Emphysema [Figure 6-11-2] Neonate with respiratory distress

•

2-week-old boy with mild dyspnea

• •

Usually diagnosed in neonates & infants 20% diagnosed in adolescents & adults

Companion Case Congenital Lobar Emphysema

Pediatric Radiology

Congenital lobar emphysema. Chest radiograph showing large hyperlucent left upper lobe with atelectasis of adjacent lung. CT showing hyperinflated left upper lobe with attenuated vascularity

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Congenital Lung Malformations

Lobar Emphysema

Figure 6-11-3

•

DDX: Swyer-James Syndrome Bronchiolitis obliterans ➢ viral infection in childhood ** • Imaging findings ➢ unilateral hyperlucent (low attenuation) lung ➢ small or normal size ** ➢ bronchiectasis ** ➢ air-trapping on expiration **Helps to differentiate from CLE

•

Swyer-James Syndrome

Swyer-James syndrome. Inspiration CT (left panel) and expiration CT (right panel) showing a low attenuation right lung with air trapping on expiration. Also noted is bronchiectasis

10-year-old boy, cough

Swyer-James Syndrome [Figure 6-11-3] • • • •

Cystic Adenomatoid Malformation

•

25% of congenital lung lesions Result of abnormal proliferation of bronchioles Normal arterial supply & venous drainage Communicates with bronchial tree

Figure 6-11-4

CCAM: Clinical • • •

70%-90% symptomatic as neonates ➢ Cyanosis, grunting, tachypnea Rare in older children & adults ➢ Presents as pneumonia or recurrent infection May be antenatal diagnosis

CCAM: Histologic Types Stocker classification ➢ Type I: (50%) Large cyst(s) (> 2 cm) ➢ Type II: (40%) Multiple cysts (< 2 cm) ➢ Type III: (10%) Microcysts on cut-section

Stocker JT. Hum Pathol 1977; 8:155-171 •

CCAM: Imaging • •

•

•

Air-filled mass, mediastinal shift ➢ Except may be opaque initially if fluid-filled Type I ➢ Multicystic lesion, with dominant cyst(s) ➢ Air fluid levels Type II ➢ Heterogeneous, uniform small cysts Type III ➢ Large, homogeneous, solid ➢ Resembles consolidation

Type I CCAM: Large Cysts [Figure 6-11-4] Neonate with dyspnea

Congenital Lung Malformations

Cystic adenomatoid malformation, Type I. Chest radiograph and CT showing large cystic lesion in the right middle lobe. Gross pathologic section also shows a large cystic mass

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Pediatric Radiology

Type II CCAM: Small Cysts [Figure 6-11-5]

Figure 6-11-5

CCAM [Figure 6-11-6] Figure 6-11-6

Cystic adenomatoid malformation, Type II. Chest radiograph showing a lucent area in the left lower lobe. Two CT scans and gross pathologic section showing a cystic mass with multiple small cysts

Cystic adenomatoid malformation. Chest radiograph on day 1 showing an opaque right upper lobe mass. Follow-up radiographs on days 3 and 7 show a more cystic appearing mass. CT showing a multicystic mass in the right upper lobe • •

CCAM Antenatal US Diagnosis

• •

Polyhydramnios Fetal hydrops ➢ Ascites ➢ Anasarca ➢ Placental edema Solid or cystic lung mass High perinatal mortality

• • •

10% diagnosed in adolescents & adults Infection common May mimic infiltrate or mass on imaging studies

•

Adolescent with cough

Spectrum of CCAM

Infected CCAM Figure 6-11-7

Infected CCAM [Figure 6-11-7] •

Bronchogenic Cyst • • •

Failure of lung bud to incorporate into primitive lung Lung (30%), mediastinum (70%) Asymptomatic & incidental finding Symptomatic from mass effect

Infected cystic adenomatoid malformation. Chest radiograph showing an air-space opacity with a cystic component superiorly. Transverse CT showing multiple cysts with thick walls and some air-fluid levels Pediatric Radiology

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Congenital Lung Malformations

•

Bronchogenic Cyst: Path [Figure 6-11-8]

•

• •

Figure 6-11-8

Gross ➢ Separate from lung ➢ Round or ovoid ➢ Clear or turbid contents Histo ➢ Lined by respiratory epithelium ➢ Bronchial glands, cartilage, smooth muscle in wall

Pulmonary Bronchogenic Cyst: Imaging • • •

Smooth, rounded, unilocular mass Fluid-filled, usually serous fluid ➢ Sometimes protein or mucin Air or air-fluid levels if infected No mediastinal shift Non-enhancing

•

7-day old girl, wheezing

Bronchogenic Cyst •

Bronchogenic cyst, pathology. Gross specimen showing a fluid-filled lung mass. Histologic section showing respiratory epithelium lining the cyst wall

Bronchogenic Cyst [Figure 6-11-9] 7- year-old girl with cough

Figure 6-11-9

Figure 6-11-10

Bronchogenic cyst. Transverse and coronal CT scans showing a well defined, smoothly marginated mass containing only air in the right upper lobe •

Septations and multiple cysts favor CAM

• • •

Lobar emphysema CCAM Bronchogenic cyst

Bronchogenic cyst. Chest radiograph showing a right paratracheal mass. Transverse CT scans showing a water attenuation mass with imperceptible margins in the right paratracheal area

DDX: Cystic Adenomatoid Malformation Review

•

Figure 6-11-11

Mediastinal Bronchogenic Cysts Same characteristics as pulmonary cysts ➢ Round, solitary, air or fluid filled

Mediastinal Bronchogenic Cyst [Figure 6-11-10]

Bronchogenic Cyst: MRI Enteric cyst. Transverse CT (left panel) showing a water attenuation mass in the lower mediastinum. Gross section (middle image) showing a cystic mass. Histologic section (right panel) showing gastrointestinal lining

Differential Diagnosis: Enteric Cysts [Figure 6-11-11]

Congenital Lung Malformations

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Pediatric Radiology

Enteric Foregut Cyst •

Figure 6-11-12

Pulmonary Underdevelopment • • •

Agenesis: Complete absence of lung tissue, artery, & small or absent bronchus Hypoplasia: Small lung & bronchus (artery may or may not develop)

Lung Agenesis [Figure 6-11-12] Small bronchus No lung or PA

Companion Case: Lung Agenesis •

Arrested Pulmonary Development

•

Pulmonary hypoplasia ➢ Small lung (hypoplasia) ➢ Small bronchus ➢ Absent or small pulmonary artery ➢ Mediastinal shift to side of hypoplasia

Pulmonary Hypoplasia [Figure 6-11-13] 2-month-old boy, mild dyspnea

Figure 6-11-13

Lung agenesis. Chest radiograph in a neonate showing an opacified hemithorax. CT scan showing absence of the right lung and pulmonary artery and a small rudimentary bronchus (arrow) Pulmonary hypoplasia. Frontal chest radiograph in a 2 month old boy showing a small right lung and mediastinal shift to the right. Lateral radiograph showing a posterior sternal stripe, representing fibrofatty tissue (arrow)

Figure 6-11-14

Pulmonary Hypoplasia [Figure 6-11-14] •

Pulmonary Hypoplasia Small lung & bronchus No PA

Congenital Anomalies with Abnormal Vasculature •

• •

• •

Hypoplasia with anomalous venous return ➢ Scimitar syndrome Pulmonary sequestration Arteriovenous malformation

Hypogenetic Lung Syndrome •

Lung hypoplasia with PAPVR PAPVR into IVC, portal vein/ hepatic vein, or right atrium Often asymptomatic and discovered during evaluation of another anomaly

Pediatric Radiology

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Pulmonary hypoplasia. Transverse chest CT scans showing a small right lung, main right bronchus, absent right pulmonary artery and mediastinal shift to the right

Congenital Lung Malformations

• • • • • •

Hypogenetic Lung Syndrome: Imaging Features

Figure 6-11-15

Usually right lung Anomalous pulmonary venous return Lung hypoplasia Hypoplasia of Rt pulmonary artery Dextrocardia May have abnormal systemic arterial supply to lung

Hypogenetic Lung Syndrome [Figure 6-11-15] •

Pulmonary Sequestration • •

•

No normal connection with bronchial tree or pulmonary arteries Systemic blood supply 2 types ➢ Intralobar (acquired) ➢ Extralobar (congenital)

Pulmonary Sequestration ELS ➢ Own pleura ➢ Neonate ➢ 90% left ➢ Solid ➢ Syst. arterial supply ➢ Syst.venous drainage ➢ Assoc. anomalies

ILS Shared pleura Children & adults 90% left Cystic or solid Syst. arterial supply Pulm. venous drainage +/- assoc. anomalies

Hypogenetic lung syndrome. Transverse CT scan showing a small right lung and mediastinal shift towards the right. Also noted a left aortic arch with anomalous right subclavian artery. Coronal multiplanar (middle panel) and 3D volume rendered image (viewed posteriorly) showing an anomalous vein (arrow) draining the right lower lobe

Figure 6-11-16

•

Sequestration: Vascular Findings ILS ➢ Arterial supply ❖ Thoracic aorta (70%) ❖ Other-abdominal aorta, intercostal ➢ Venous drainage Pulmonary (95%), systemic (5%) • ELS ➢ Arterial supply ❖ Abdominal aorta (80%) ❖ Other—thoracic aorta, pulmonary artery ➢ Venous drainage ❖ Systemic (80%), pulmonary (20%)

• •

Intralobar sequestration, pathology. Cut section shows abnormal inflamed parenchyma

Pulmonary Sequestration

•

Intralobar Extralobar

Figure 6-11-17

Intralobar Sequestration: Path [Figure 6-11-16] •

•

Gross ➢ Edge may abruptly abut normal lung or blend diffusely Microscopic ➢ Chronic inflammation ➢ Bronchopneumonia

Extralobar Sequestration: Path [Figure 6-11-17] •

Separate from normal lung ➢ Pyramidal, rounded, ovoid Extralobar sequestration. Gross specimen ➢ Resembles normal lung resembles normal lung Histologic section shows Microscopic dilated bronchioles, alveolar ducts, and alveoli. ➢ Dilated bronchioles, alveoli & subpleural Also noted is a well-formed bronchus near one lymphatics edge of the lesion ➢ Well-formed bronchus near edge of lesion (50%)

Congenital Lung Malformations

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Pediatric Radiology

•

Sequestration: Clinical

•

•

Figure 6-11-18

ELS ➢ Often asymptomatic ➢ Dyspnea, cyanosis occasionally (10%) ➢ Associated anomalies ❖ CHD, diaphragmatic hernia, CCAM ILS ➢ Symptomatic ➢ Cough, recurrent pneumonia

Sequestration: US Features

•

• • •

Gray-scale ➢ Echogenic mass ❖ Supradiaphragmatic-likely ILS ❖ Subdiaphragmatic (suprarenal)-likely ELS Doppler US ➢ Feeding artery off aorta ➢ Draining vein usually not identified

Intralobar sequestration. Chest radiograph showing a left lobe air space opacity. Sonogram showing a supradiaphragmatic echogenic mass with a feeding artery in the left lower lobe

Figure 6-11-20

Sequestration: CT Features Anomalous feeding artery Draining vein to pulmonary or systemic veins Parenchymal findings ➢ ILS: ❖ Infiltrate ❖ Abscess ❖ Focal emphysema ➢ ELS ❖ Triangular/round solid mass

Intralobar Sequestration: Infiltrate [Figure 6-11-18] Intralobar Sequestration: Infiltrate & Emphysema [Figure 6-11-19]

Figure 6-11-19

Intralobar sequestration. Transverse CT showing a cystic mass with an enhancing wall in the left lower lobe, representing an abscess. 3D reconstruction (viewed posteriorly) showing an anomalous artery (arrow) from the distal thoracic aorta supplying the sequestered lung

Intralobar sequestration. Transverse CT at lung windows (left panel) showing a left lower lobe infiltrate with surrounding emphysema. Transverse contrast enhanced CT (right panel) showing an opacified anomalous artery (arrow) from the distal thoracic aorta supplying the sequestered lung

Intralobar Sequestration: Drainage via PV to Left Atrium •

Intralobar Sequestration Abscess Formation 6-year-old girl, fever and cough

Intralobar sequestration: Abscess Formation [Figure 6-11-20]

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Congenital Lung Malformations

Extralobar Sequestration: Triangular Mass

Figure 6-11-21

[Figure 6-11-21]

Extralobar Sequestration: Round Lower Lobe Mass [Figure 6-11-22] Extralobar Sequestration: Systemic Drainage •

Extralobar Sequestration Solid, well-defined LLL mass

Extralobar sequestration. Chest radiograph showing a triangular mass (arrow) at the left lung base. Transverse sonogram showing an heterogeneous mass (arrows) which was below the left hemidiaphragm

Sequestration: MRI ELS with CCAM (40%) • •

Summary

•

ILS ELS

Figure 6-11-22

Arteriovenous Malformation • •

80% Hereditary telangiectasia (OWR) ➢ 15% sporadic ➢ 5% cardiac surgeries (Glenn or Fontan) Symptomatic in older patients (cyanosis, polycythemia, dyspnea) 80%-90% are simple AVMs ➢ single feeding and draining vessel ➢ commonly lower lobes Extralobar sequestration. Coronal CT reformation (left panel) and 3D reconstruction (right panel) showing two anomalous feeding arteries entering a solid left lower lobe sequestration

Arteriovenous Malformation •

Pulmonary AVM Simple architecture

Simple Pulmonary AVM Figure 6-11-23

Multiple AVMs [Figure 6-11-23] • •

Congenital Lung Anomalies ABNORMAL LUNG - NORMAL VASCULATURE NORMAL LUNG - ABNORMAL VASCULATURE

Multiple pulmonary arteriovenous malformations. Two transverse CT scans and 3D reconstructions showing multiple pulmonary arteriovenous malformations (arrows)

Congenital Lung Malformations

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References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

Siegel MJ. Lung, pleural and chest wall. In: Pediatric Body CT. Philadelphia, Lippincott Williams & Wilkins. 1999;101-.140 Kim WS, Lee KS, Kim IO, et al. Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation. AJR 1997; 168:47Rosado-de-Christenson JL, Stocker JT. Congenital cystic adenomatoid malformation. J Comput Assist Tomogr 1989; 13:612-616 Shackelford GD, Siegel MJ. CT appearance of cystic adenomatoid malformation. J Comput Assist Tomogr 1989; 13:612-616. Felker RE, Tonkin IL. Imaging of pulmonary sequestration. AJR 1990; 154:241-249 Frazier AA, Rosado de Christenson ML, Stocker JT et al. Intralobar sequestration: radiologic-pathologic correlation. RadioGraphics 1997; 17:725-745. Ko SF, Ng SH, Lee TY, et al. Noninvasive imaging of bronchopulmonary sequestration. AJR 2000; 175:10051012 Lee E, Siegel MJ, Sierra LM, Foglia RP. Evaluation of angioarchitecture of pulmonary sequestration in pediatric patients using multidetector CT angiography. AJR 2004; 183:183-188. Rosado-de-Christenson ML, Frazier AA, Stocker JT, Templeton PA. Extralobar sequestration: radiologicpathologic correlation. From the archives of the AFIP. RadioGraphics 1993; 13:425-441. Konen E, Raviv-Zilka L, Cohen RA, et al. Congenital pulmonary venolobar syndrome: Spectrum of helical CT findings with emphasis on computerized reformatting. RadioGraphics 2003; 23:1175-1184 Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. RadioGraphics 1994; 14:349-369. Remy J, Remy-Jardin M, Giraud F, et al. Angioarchitecture of pulmonary arteriovenous malformations: clinical utility of three-dimensional helical CT. Radiology 1994; 191:657-664. Hoffman LV, Kuszyk BS, Mitchell SE, et al. Angioarchitecture of pulmonary arteriovenous malformation: characterization using volume-rendered 3D CT angiography. Cardiovasc Intervent Radiol 2000; 23: 165-170. Rotondo A, Scialpi M, Scapati C. Pulmonary arteriovenous malformation: evaluation by MR angiography. AJR 1997; 168: 847-849.

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Lung Diseases in Neonates Marilyn J. Siegel, MD Neonatal Respiratory Distress: Spectrum of Disorders • • •

Surgical Disease Congenital Heart Disease Medical Disease

• • •

Discuss lung diseases in preterm and term neonates Review treatment complications Understand important differential findings

[Figure 6-12-1]

Figure 6-12-1

Objectives

•

Neonatal Medical Lung Diseases

•

WHAT YOU WILL SEE: ➢ Respiratory distress syndrome ➢ Retained lung fluid ➢ Meconium aspiration ➢ Neonatal pneumonia WHAT YOU MAY SEE: ➢ Chylothorax ➢ Congenital surfactant protein B deficiency

Medical Lung Diseases: Premature Neonate • • • •

Neonatal respiratory distress, spectrum of causes

Premature Births < 37 weeks ~500,000 preterm deliveries/year 12% of all live births Lung disease most common problem

CDC National Vital Statistics Reports, Vol. 52, No. 10, Dec 17, 2003 • •

Respiratory Distress Syndrome (RDS) • • •

Same as hyaline membrane disease (HMD) & surfactant deficiency disease 1% of pregnancies, typically prematures ➢ 26-34 weeks Post-term infants of diabetic mothers More frequent and severe in males More common in whites than blacks

• • • •

Surfactant deficiency Leads to alveolar collapse Increased capillary permeability Hyaline membrane formation

Figure 6-12-2

RDS/HMD: Pathogenesis

• •

What are Hyaline Membranes? [Figure 6-12-2] Line terminal & respiratory bronchioles Contain ➢ Necrotic alveolar cells ➢ Plasma transudate ➢ Aspirated squames ➢ Fibrin

Lung Diseases in Neonates

Hyaline membranes. Eosinophilic hyaline membranes surrounded by collapsed air spaces

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• • •

Mature airspace Surfactant deficiency Bronchial collapse & atelectasis

• • •

Firm atelectatic lungs (“hepatization”) Alveolar edema +/- focal hemorrhage

• • • • •

Small lung volumes Diffuse bilateral granular opacities Air bronchograms Loss of vascular definition No pleural effusion

RDS/HMD: Pathogenesis

RDS/HMD: Gross Pathology

RDS/HMD: Imaging Findings Figure 6-12-3

RDS/HMD [Figure 6-12-3] • •

RDS/HMD: Classic Clinical Approach

• •

Confirm suspected diagnosis with radiographs Positive pressure ventilator therapy ➢ Increases risk of air leak ➢ Increases risk of BPD Respiratory distress syndrome, untreated. Low lung volumes with diffuse bilateral granular opacities

RDS/HMS: Other Treatment Options

• •

Maternal (in utero) steroid administration Exogenous surfactant

Exogenous Surfactant Therapy: Clinical Impact

• • • • •

Slow liquid bolus into airway Desired effect is decreased: ➢ Oxygen requirement ➢ Mortality from RDS ➢ Air leak

Surfactant Therapy:Imaging Findings

Figure 6-12-4

Uniform improvement in granularity at 24-48 hrs (38%) Asymmetric improvement (35%) Small cystic lucencies (17%) No improvement (10%) Focal hemorrhage or hemorrhagic pulmonary edema (1%-2%)

Pediatr Radiol 1997; 27:26-31

Surfactant Therapy [Figure 6-12-4] •

Complications of Treatment

• • • •

Related to respirator ➢ Air leaks ➢ Bronchopulmonary dysplasia

Chest radiograph (left image) showing RDS, pretreatment. Chest radiograph (right image) showing RDS, 24 hours after surfactant therapy

Air Leak: Pathogenesis Results from positive pressure ventilation of noncompliant lungs Rupture at bronchiolar-alveolar junction Gas dissects into interlobular septa and septal lymphatics (interstitial air) May enter pleural space, mediastinum, pericardium, peritoneum, venous system

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Lung Diseases in Neonates

Pulmonary Interstitial Emphysema [Figure 6-12-5]

Figure 6-12-5

Acute Pulmonary Interstitial Emphysema: Imaging Findings • • • • • •

Tubular & cystic lucencies Focal or diffuse Unilateral or bilateral Pulmonary overexpansion Mediastinal shift (unilateral PIE) Small cardiac silhouette (diffuse bilateral PIE)

Pulmonary Interstitial Emphysema [Figure 6-12-6] RDS/Pulmonary Interstitial Emphysema • •

Persistent Interstitial Pulmonary Emphysema [Figure 6-12-7] • • •

PIE lasting > 1 week Associated with pseudocysts ➢ Aggregate thick-walled cysts Right parahilar location May resolve completely or may need resection Mimic solid mass when fluid-filled

Air leak, pulmonary interstitial emphysema. Gas in interlobular septa

Williams, et al. AJR 1988; 150:885-887 • •

Persistent PIE: CT Findings

•

Figure 6-12-6

17 preterm neonates Hyperexpanded complex cystic masses developed from typical PIE (mean, 13 days) 53% underwent resection

Donnelly LF, et al. AJR 2003; 180:1129

Persistent PIE with Air Leak “Pseudocysts” [Figure 6-12-8] Figure 6-12-8

Figure 6-12-7

Acute pulmonary interstitial emphysema. Chest radiograph showing hyperinflated lungs with cystic lucencies bilaterally. Pathologic section showing interstitial air

Persistent pulmonary interstitial emphysema. Chest radiograph showing cysts in right parahilar region. CT showing complex multicystic mass in the right lower lobe

Persistent interstitial pulmonary emphysema. Gross specimen. Mass-like aggregate of thickwalled cysts Lung Diseases in Neonates

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Air Leak “Pseudocysts” • • •

Figure 6-12-9

Air Leak: Pneumothorax [Figure 6-12-9]

• • •

May be subtle in infants Collects anteriorly in supine patients Look for “deep sulcus” sign and asymmetric lucent lung Mediastinal shift with tension pneumothorax

Air Leak: Pneumomediastinum • •

Most are clinically insignificant Usually does not dissect into neck because of persistence of sternopericardial ligament Thymus elevation (“spinnaker sail sign”) Gas beneath heart ➢ “Continuous diaphragm sign”

Left panel. Two different patients. Chest radiographs in both patients show a tension pneumothorax with mediastinal shift and hyperexpansion of the affected hemithorax. Left image, also note underlying changes of hyaline membrane disease. Right image, also note underlying pulmonary interstitial emphysema

Pneumomediastinum [Figure 6-12-10] • • •

Air Leak: Pneumopericardium [Figure 6-12-11] Gas delimited superiorly by pericardial reflection about great vessels No thymic displacement May require needle compression of tamponade

Figure 6-12-10

Pneumopericardium Air Leak: Pneumopericardium & PTX •

Air Leak: Systemic Gas Embolism •

Pathogenesis uncertain ➢ Alveolar-venous fistula? ➢ Lymphatic gas entering right heart? Virtually 100% fatal

Pneumomediastinum. Elevation of thymic lobes on frontal radiograph (spinnaker sail sign). Substernal air seen on lateral radiograph

Bronchopulmonary Dysplasia: Clinical Diagnosis • • • • •

> 3 days positive pressure ventilation + 02 during 1st 2 weeks of life Respiratory distress lasting > 28 days Require supplemental 02 > 28 days to maintain Pa02 > 50 mm Hg Characteristic radiologic findings

Figure 6-12-11

Bronchopulmonary Dysplasia: Risk Factors

• •

Stress factors ➢ Barotrauma ➢ Oxygen cytotoxicity (free radicles) ➢ Infection Pulmonary edema (related to PDA) Host Factors ➢ Genetics (family history of atopy & asthma) ➢ Endogenous low steroids

Bronchopulmonary Dysplasia: Basic Pathologic Features •

•

Pneumopericardium. Air extends to level of great vessels. Also noted is underlying pulmonary interstitial emphysema

Tracheal & bronchial injury ➢ Mucosal ulceration & necrosis Pulmonary arterial injury ➢ Intimal & adventitial thickening ➢ Hypertensive vascular disease ❖ Bronchiolar & alveolar injury ➢ Cell necrosis & septal edema

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Lung Diseases in Neonates

•

Reparative phase ➢ Alveolar septal fibrosis

Figure 6-12-12

Stocker JT. Hum Pathol 1986; 17:943

Bronchopulmonary Dysplasia [Figure 6-12-12] • • • • •

I: 0-4 days granular opacities of RDS II: 4-10 days “increased opacities” III: 10-30 days “Bubbly Lungs” IV: > 30 days disordered aeration All 4 stages rarely observed

• • •

“Bubbly” lungs develop at end of 2nd postnatal week < 1000 gram newborns Lower respiratory tract infection

Classic Stages of BPD:Imaging Findings

Variant in Stages of BPD“Precocious BPD”

Crouse DT et al. Clin Infect Dis 1993; 17: S 122-130

Classic BPD: “Bubbly Lungs” [Figure 6-12-13] •

Figure 6-12-13

Bronchopulmonary Dysplasia

•

Bronchopulmonary dysplasia. Gross path specimen (left ipanel) showing fibrotic lungs. Histologic specimen (right panel) showing dilated air spaces and septa thickened by a combination of edema, inflammatory cells and fibroblasts

HRCT ➢ Emphysema ➢ Cystic or bullous changes ➢ Septal lines No zonal predominance

Bronchopulmonary Dysplasia [Figure 6-12-14]

BPD: Classic Temporal Course BPD: Chronic Course: Disorganized Aeration • • • •

Bronchopulmonary dysplasia. Hyperinflation and bubbly cystic lungs

Figure 6-12-14

BPD: Prognosis

• •

Pulmonary function slowly improves Radiographs normalize in most by 3 years PFTs remain abnormal for years Refractory pulmonary hypertension = poor prognosis & lung transplant

Terminology: Need to recognize • •

BPD is a chronic insult Any insult that increases the need for mechanical ventilation can lead to chronic injury Mechanical ventilation increases risk of lung damage! Will have the same appearance as BPD

Bronchopulmonary dysplasia. CT showing emphysematous changes, septal lines extending to pleural surface and bullous formation

Medical Lung Diseases: Term Neonate • • • • •

Other Neonatal Lung Diseases Retained fetal lung fluid Aspiration syndromes Pneumonia (term & premature neonates) Surfactant B protein deficiency Chylothorax

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•

Retained Fetal Lung Fluid or Transient Tachypnea of Newborn • •

Figure 6-12-15

Associated with C-section, maternal sedation, maternal diabetes Lung fluid normally removed by capillary and lymphatic resorption & retrograde tracheal flow Lack of compression by birth canal (C-section) & decreased maternal & neonatal beta-adrenergic responsiveness may alter clearance

Retained Fetal Lung Retained Fetal Lung Fluid: Radiographic Findings • • • • •

Retained fetal lung fluid. Frontal chest radiograph showing reticular opacities and small right pleural effusion. Lateral radiograph showing fissural fluid

Increased lung volumes Reticular opacities Fissural fluid Small pleural effusions Clearing in 24 to 48 hours

Figure 6-12-16

Retained Fetal Lung Fluid [Figures 6-12-15 and 6-12-16]

• • •

Meconium Aspiration Syndrome

• •

In utero defecation due to fetal distress Usually > 34 weeks gestation 10%-15% of pregnancies have meconium stained amniotic fluid 5% of meconium stained neonates develop meconium aspiration syndrome 30%-50% require mechanical ventilation

Retained fetal lung fluid. Frontal chest radiograph day 1 (left panel) showing reticular opacities. Chest radiograph day 2 (right panel) showing interval clearing of interstitial fluid

Figure 6-12-17

Meconium Aspiration Syndrome: Pathologic Features •

• • •

Acellular debris ➢ Squamous epithelial cells Tenacious green-yellow meconium plugs in airways Pneumonitis Hemorrhagic edema

medic.med.uth.tmc.edu/ •

Meconium Aspiration: Pathophysiology

•

• • •

Primary ➢ Mechanical Obstruction ➢ Chemical inflammation ➢ Surfactant inactivation Secondary ➢ Air trapping ➢ Air leak ➢ Atelectasis

Meconium aspiration. Hyperinflated lungs and coarse interstitial opacities

Figure 6-12-18

Meconium Aspiration: Imaging [Figure 6-12-17] Hyperinflation Coarse patchy opacities Air leak (25%-40%) ➢ Pneumomediastinum ➢ Pneumothorax ➢ Pleural effusion (rare)

Meconium aspiration. Hyperinflated lungs with large pneumothoraces and pneumomediastinum

Meconium Aspiration [Figure 6-12-18]

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Lung Diseases in Neonates

• • • • • •

Antibiotics Surfactant Mechanical ventilation Inhaled nitric oxide (iNO) May require ECMO 94% overall survival

• • •

0.5% of term infants Frequently associated with neonatal sepsis Clinical and radiographic challenging diagnosis

Meconium Aspiration: Treatment Figure 6-12-19

Neonatal Pneumonia

•

Neonatal Pneumonia: Etiology • • • •

In utero (hematogenous) infection ➢ CMV, syphilis, listeriosis Ascending infection (PPROM) ➢ Group B beta hemolytic strep ➢ E. coli During delivery ➢ Strept ➢ Chlamydia trachomatis Postnatal infection ➢ Bacterial

Neonatal pneumonia. Coarse interstitial opacities, increased lung volumes

Figure 6-12-20

Neonatal Pneumonia: Path •

Air spaces contain neutrophils & squamous epithelial cells without fibrin Air spaces surrounded by thin cellular septa

• • • • •

Nonspecific, need to correlate clinically Diffuse granularity (may mimic RDS) Patchy and streaky opacities (may mimic TTN or meconium) Pleural effusion (65%) Lung volumes usually normal, but may be increased

Neonatal Pneumonia: Radiologic Findings

Neonatal pneumonia. Patchy confluent opacities. Normal lung volumes

Neonatal Pneumonia [Figures 6-12-19 and 6-12-20] Figure 6-12-21

Review & Quiz Time TTN, Pneumonia or Meconium? Concede that lung findings are nonspecific: Look for the clues!! [Figure 6-12-21] •

Meconium, pneumonia, TTN? • •

•

•

Hyperinflation? ➢ Meconium, TTN, pneumonia Air leak? ➢ Meconium aspiration Normal aeration? ➢ Pneumonia Patchy confluent opacities? ➢ Pneumonia Fissural fluid? ➢ TTN Clues to diagnosing neonatal lung disease

Lung Diseases in Neonates

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•

OR Stated Another Way • • •

Meconium aspiration ➢ Reticular opacities, increased lung volumes, effusion rare (<10%) Neonatal pneumonia ➢ Reticular or confluent opacities, normal or increased aeration, effusions common (65%) TTN ➢ Reticular opacities, increased lung volumes, pleural or fissural fluid (90%)

For Comparison Respiratory distress syndrome ➢ Decreased long volumes ➢ Granular lung disease ➢ NO effusions in uncomplicated disease!!

Medical Lung Diseases: Presenting in 1st week • •

Chylothorax • • •

Rupture of thoracic duct Usually due to birth trauma ➢ Rarely congenital anomaly Full term infants ➢ 70% symptomatic in 1st week Right > left pleural effusions ➢ Rarely bilateral

Chylothorax • • •

Thoracentesis usually yields cloudy fluid because of the high lipid content of chyle Treatment includes thoracentesis, chest tube drainage, and feedings of medium-chain triglycerides Most lymphatic ruptures seal with combined chest tube and dietary treatment

Chylothorax Differential diagnosis includes causes of nonchylous pleural effusions ➢ Wet-lung disease ➢ Hydrops fetalis ➢ Turner’s syndrome ➢ Pulmonary vein obstruction ➢ Congestive heart failure ➢ Esophageal rupture

Chylothorax [Figure 6-12-22] • • •

Alveolar Proteinosis

• •

Due to congenital surfactant protein B deficiency Autosomal recessive PAS-positive abnormal surfactant lipids & proteins accumulate in alveoli & macrophages Diagnosis confirmed by alveolar lavage & peripheral blood DNA analysis Treatment - lung transplantation

Figure 6-12-22

Pediatr Radiol 2001; 31: 327-331

Chylothorax. Day 1 (left image), large right pleural effusion. Day 7 (middle image), decreased fluid following thoracenteses. One month (right image), resolution, following thoracentesis and feedings of medium chain triglycerides Pediatric Radiology

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Lung Diseases in Neonates

• •

Surfactant B Deficiency: Imaging Features

•

• •

Figure 6-12-23

Mimics RDS, but in term infant Chest ➢ Low lung volumes ➢ Hazy lungs CT ➢ Ground-glass opacities & septal lines ➢ “Crazy paving”

Surfactant B Deficiency [Figure 6-12-23] Mimics HMD Crazy paving

Neonatal Medical Diseases: The Big Four

Surfactant B deficiency. Chest radiograph showing low volume, hazy lungs. CT showing ground-glass opacities and thickened septal lines

[Figure 6-12-24]

Figure 6-12-24

The top 4 neonatal medical diseases

References 1. 2. 3. 4. 5. 6. 7.

Center for Disease Control and Prevention: National Center for Disease Statistics. National Vital Statistics Reports, Vol. 52, No. 10, Dec 17, 2003. Web: www.cdc.gov/nchs Dinger J, Schwarze R, Rupprecht E. Radiological changes after therapeutic use of surfactant in infants with respiratory distress syndrome. Pediatr Radiol 1997; 27:26-31. Donnelly LF, Lucaya J, Ozelame V, et al. CT findings and temporal course of persistent pulmonary interstitial emphysema in neonates: a multiinstitutional study. AJR Am J Roentgenol 2003; 180:1129-1133. Medic: medical education information. University of Texas - Houston, Department of Pathology and Laboratory Medicine. http://medic.med.uth.tmc.edu/ Newman B, Kuhn JP, Kramer SS, Carcillo JA. Congenital surfactant protein B deficiency--emphasis on imaging. Pediatr Radiol 2001; 31:327-331. Stocker JT. Pathologic features of long-standing "healed" bronchopulmonary dysplasia: a study of 28 3- to 40month-old infants. Hum Pathol 1986; 17:943-961. Williams DW, Merten DF, Effmann EL, Scatliff JH. Ventilator-induced pulmonary pseudocysts in preterm neonates. AJR Am J Roentgenol 1988; 150:885-887.

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Pediatric Cardiac Imaging Part I: Vascular Anomalies Marilyn J. Siegel, MD • •

Lecture Outline

•

Review MRA and CTA techniques Discuss CT and MR appearances of common thoracic vascular anomalies

MR Angiography: Basic Technique • •

Black blood sequence: Single shot FSE with half fourier reconstruction (RARE, HASTE) ➢ Evaluation of airway, vessel lumen Bright blood sequence: Steady-state free precession (FIESTA, FISP, true FISP) ➢ Evaluation of shunts/jets due to dephasing associated with turbulent flow Contrast-enhanced MR angiography ➢ Evaluation of stenoses, bronchial collaterals, anomalous pulmonary veins

CEMRA depicts blood flow from PA to systemic arteries to systemic veins • • • • • • •

CT Angiography: Basic Protocol

•

PE protocol Thin collimation (< 1mm) Fast table speed Reconstruct 1 to 2 mm intervals (3D) Low mAs and kVp Bolus tracking for scan initiation Trigger @ 100-120 HU

Position of the ROI: Over the Area of Interest

•

Aorta and surgical shunts ➢ Ascending aorta Pulmonary artery ➢ Main PA or branches Pulmonary veins: LA

• • •

Multiplanar MIPs Volume Rendering

•

Reconstructions Increase diagnostic accuracy

•

Which One is Best? MRA or CTA • • •

•

NO BEST ANSWER ➢ It depends on what you need to know Both accurate for anatomic detail MR >CT for functional information CT>MR for showing stents and calcifications

Applications for Thoracic MRA & CTA

•

Congenital vascular anomalies ➢ Aorta ➢ Pulmonary vessels ➢ Vena cava Systemic diseases ➢ Marfan disease ➢ Kawasaki disease

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Vascular Anomalies

•

Aortic Arch Anomalies

Figure 6-13-1

Symptomatic lesions ➢ Vascular rings ❖ Right arch with aberrant Lt SCA ❖ Double arch Anomalous innominate artery • Asymptomatic lesions ➢ Left arch with aberrant Rt SCA ➢ Cervical arch

• • • •

Vascular RingsDouble Aortic Arch

•

Right arch is dominant Left arch may be patent or atretic Complete ring Clue: 2 arches ➢ 4 artery sign (no brachiocephalic artery)

Double Arch

Double aortic arch. Transverse CT scans showing two arches. The right arch is larger and more superior than the left. The two arches unite posterior to the esophagus and a single aorta descends on the left

6 month old boy with cough

Double Arch: Patent Left Limb [Figure 6-13-1]

Figure 6-13-2

CTA: Double Aortic Arch Double Arch Infant with wheezing MRA: Double Arch Double Arch Atretic Segment [Figure 6-13-2] •

Double Arch Atretic Segment Look at the Airway Double aortic arch with hypoplastic left segment (arrow)

Right Aortic Arch: Aberrant left subclavian artery • • •

True ring-completed by ligamentum d. arteriosum Left SCA is last vessel off aorta Encircles trachea & esophagus

Figure 6-13-3

Right aortic arch Right Arch Aberrant Left SCA •

6 wk old boy with CHD

• •

GRE MR CE MRA

Right Arch Aberrant Left SCA [Figure 6-13-3] MRA: Right arch/aberrant LT SCA

•

Mirror Image Right Arch Mirror image branching ➢ Asymptomatic ➢ Not a true vascular ring ➢ Congenital heart disease (98%) ❖ Tetralogy of Fallot ❖ Truncus arteriosus

Vascular Anomalies

Right aortic arch with aberrant left subclavian artery, CT The aberrant subclavian artery courses behind the esophagus and trachea. LCCA=left common carotid artery; RCCA=right common carotid artery; RSA=Right subclavian artery; LSA=left subclavian artery

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•

Mirror Image Right Arch [Figure 6-13-4] •

Figure 6-13-4

Tetralogy of Fallot

Innominate Artery Compression •

Anterior tracheal compression by the right innominate artery Symptoms--respiratory obstruction, repeated infection, stridor and on occasion respiratory arrest

Mirror image right aortic arch. Transverse CT scans showing a right sided aortic arch without a posterior crossing vessel

Innominate Artery Compression [Figure 6-13-5]

• • •

Pulmonary Arterial Anomalies

• • •

Agenesis (interruption) of main PA Pulmonary sling Ductus arteriosus

Interrupted Pulmonary Artery

•

Rt or Lt PA is congenitally absent Interrupted 1 cm beyond origin Leads to increased systemic blood flow (collateral circulation) to affected hemithorax Affected lung absent or hypoplastic ➢ Growth dependent on collateral supply

Figure 6-13-5

Interrupted Pulmonary Artery Hypoplastic Lung [Figure 6-13-6] •

Interrupted Pulmonary Artery Bronchial collaterals

Absent Pulmonary Artery Absence of lung •

Pulmonary Sling •

Created by anomalous course of left PA ➢ Arises from right pulmonary artery & crosses between trachea & esophagus Usually symptomatic in children, dyspnea

Figure 6-13-6

Innominate artery compression. Transverse and sagittal reconstruction CT showing anterior compression of the trachea by the right innominate artery (arrow)

Interrupted pulmonary artery. Transverse CT showing the right pulmonary artery (arrow) abruptly interrupted about 1 cm beyond its origin. Right lung is hypoplastic. Coronal reformation CT showing fibrofatty tissue filling a small right hemithorax

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Vascular Anomalies

Pulmonary Sling 3 month old girl [Figure 6-13-7]

Figure 6-13-7

Pulmonary Sling MRA: Pulmonary Sling •

Patent Ductus Arteriosus •

Tubular connection between proximal descending aorta and left pulmonary artery Isolated lesion or associated with other anomalies

Patent Ductus Arteriosus [Figure 6-13-8] •

Ductus Calcified ductus

Figure 6-13-8 Pulmonary sling. Chest radiograph (upper panel) showing tracheal compression. CTA (upper right panel) showing the left pulmonary artery (LPA) arising from the right pulmonary artery (RPA) and crossing behind the trachea to reach the left hilum. 3D rendering of the airway (lower panel) confirming right-sided tracheal compression

Figure 6-13-9

Patent ductus arteriosus, CTA. Multiple transverse CT scans showing the patent ductus (arrow) connecting the proximal descending aorta and pulmonary artery

Patent Ductus: MRI [Figure 6-13-9] •

Pulmonary Venous Anomalies Anomalous return ➢ Anomalous drainage to systemic veins ➢ Anomalous drainage to left atrium Patent ductus arteriosus, MRA. Transverse MR images and 3D sagittal reconstruction showing the patent ductus (arrow) connecting the proximal descending aorta and pulmonary artery

Partial Anomalous Return •

Partial Anomalous Venous Return •

All PAPVR are LT to RT shunts but the shunt is usually clinically insignificant Some present with pulmonary hypertension

Vascular Anomalies

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RUL Anomalous Return to SVC

Figure 6-13-10

MRA: Anomalous RUL pulmonary vein [Figure 6-13-10]

•

RUL PAPVR 90% have venosum ASD

RLL Anomalous Return Transverse MR (left image) showing anomalous right upper lobe pulmonary vein (arrow) draining into the superior vena cava. T1-weighted image (right image) showing associated sinus venosus atrial septal defect (arrow)

• •

MIP CTA GRE MR

• • • •

Hypogenetic lung syndrome, pulmonary venolobar syndrome Vein draining RLL enters IVC, portal vein/ hepatic vein, or right atrium Hypoplastic lung Small pulmonary artery

• •

10 year old girl Pneumonia suspected

RLL Anomalous Return [Figure 6-13-11]

Scimitar Syndrome

Figure 6-13-11

Scimitar syndrome

Scimitar syndrome Scimitar Syndrome [Figure 6-13-12] Figure 6-13-12 Right lower lobe anomalous pulmonary venous return. Coronal CT showing right lower lobe vein draining into the inferior vena cava

Scimitar syndrome. Anomalous return of the right lower lobe is associated with a hypoplastic right lung

LUL Anomalous Return [Figure 6-13-13] Figure 6-13-13

Anomalous left upper lobe pulmonary venous return. Transverse CT images showing the left upper lobe pulmonary vein (arrow) coursing next to the aorta and passing adjacent to the left pulmonary artery. There is no vessel noted at the level of the coronary sinus which helps to differentiate this condition from left superior vena cava Pediatric Radiology

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Vascular Anomalies

LUL Anomalous Return

Figure 6-13-14

Anomalous LUL Vein [Figure 6-13-14] •

Systemic Venous Anomalies

• • • • •

Systemic ➢ Persistent left superior vena cava ➢ Interrupted inferior vena cava

Left (Double) Superior Vena Cava 0.5% of general population 5% CHD patients Drains subclavian vein Empties into coronary sinus Small Rt SVC, 90% of cases Anomalous left upper lobe venous return. Axial MR images showing the left upper lobe pulmonary vein (arrow) coursing adjacent to the aorta and pulmonary artery. Coronal contrast enhanced MRA (right panel) showing the anomalous left upper lobe vein (arrow) draining into the brachiocephalic vein. Also noted is anomalous drainage of the right upper lobe vein (arrowhead) into the superior vena cava

Left Superior Vena Cava [Figures 6-13-15 and 6-13-16]

Figure 6-13-15

Figure 6-13-16

Left superior vena cava. Transverse images showing an enhancing left sided cava (arrow) and a smaller right superior vena cava. The left superior vena cava courses past the aortic arch and left pulmonary hilum to enter into a dilated coronary sinus

Differential diagnosis Left paramediastinal structures • •

Left superior intercostal vein Anomalous LUL venous return

Another Lt-sided Vessel Superior Lt Intercostal Vein •

Superior intercostal vein ➢ Drains left 2nd-4th intercostal spaces ➢ Opens into brachinocephalic vein ➢ Joins accessory hemiazygous vein ➢ Aortic nipple shadow on CXR

Left superior vena cava. Coronal and sagittal multiplanar CT reconstructions showing left superior vena cava (arrows) draining into the coronary sinus

Left Superior Intercostal Vein [Figures 6-13-17 and 6-13-18]

Vascular Anomalies

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Figure 6-13-17

Figure 6-13-18

Left superior intercostal vein creating aortic nipple shadow (arrow) •

Summary: Left Paramediastinal Structures • •

Left superior vena cava ➢ Subclavian vein to coronary sinus Anomalous LUL pulmonary vein ➢ Left pulmonary hilum to BCV Left superior intercostal vein ➢ BCV to accessory hemiazygos vein

Left superior intercostal vein. The vein (arrow) courses adjacent to the aortic arch and connects with the accessory hemiazygous vein inferiorly

Figure 6-13-19

Review: Paramediastinal Veins [Figure 6-13-19] Differential diagnosis Right paramediastinal structures •

•

Azygous vein ➢ joins hemiazygos ➢ ascends on right ➢ drains into SVC Superior intercostal vein ➢ joins azygos vein ➢ drains 2nd-4th intercostal veins

Summary: Left image, left superior vena cava. Middle image, anomalous left upper lobe pulmonary venous return. Right image, superior left intercostal vein

Figure 6-13-20

Right Azygous System Azygous Vein [Figure 6-13-20] Right Superior Intercostal Vein [Figure 6-13-21]

Figure 6-13-21

Normal azygous vein (arrow) draining into superior vena cava (SVC). Right superior intercostal vein. CT scans showing the right superior intercostal vein (arrow) draining into the azygous vein Pediatric Radiology

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Vascular Anomalies

• •

Azygos Continuation IVC •

Figure 6-13-22

Absence of infrahepatic segment of IVC Blood from lower half of body returns to heart via dilated azygous and hemiazygous veins Isolated condition or associated with CHD

Azygos Continuation IVC [Figure 6-13-22] Dilated Azygous Vein: Differential Diagnoses • • • • • • •

Azygous continuation of the inferior vena cava. CT scans showing dilated azygous and hemiazygous veins and absence of the infrahepatic inferior vena cava

Azygous continuation IVC Congestive heart failure Constrictve pericarditis Acquired obstruction SVC or IVC Pericardial effusion Tricuspid insufficiency

Figure 6-13-23

Acquired Vascular Lesions •

• • •

Aortic aneurysm & dissection ➢ Marfan syndrome Coronary artery aneurysms ➢ Kawasaki disease

Kawasaki Disease

• •

Aka Mucocutaneous lymph node syndrome Unknown etiology Clinical features ➢ fever ➢ rash ➢ conjunctivitis ➢ erythema of lips & buccal mucosa ➢ myocarditis ➢ coronary artery aneurysms

Coronary artery aneurysm, Kawasaki disease. CT of a neonate (left panel) showing dilated left anterior descending and circumflex arteries (arrows) . CT scan of an adolescent girl (right panel) showing calcified aneurysm (arrow) of the left coronary artery

Coronary Artery Aneurysms Arise in proximal part of arteries Fusiform, saccular, or cylindrical

Coronary Artery Aneurysms-Kawasaki [Figure 6-13-23] •

Marfan Disease • •

•

Inherited connective tissue disorder transmitted as an autosomal dominant trait Localized to a mutation in chromosome 15 Cardinal features ➢ tall stature ➢ slender limbs and fingers ➢ ectopia lentis ➢ pectus excavatum ➢ scoliosis

Common Cardiovascular Findings • •

Aortic-root dilatation ➢ involves sinuses of Valsalva ➢ prevalence 70%-80% Aortic dissection ➢ Ascending >> descending aorta Mitral valve prolapse (55%-69%)

Vascular Anomalies

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• •

Marfan Disease Aortic Aneurysm & Dissection 15 year old boy with Marfan’s syndrome Dilated aortic root

Figure 6-13-24

Marfan Disease: Dissection Marfan Disease [Figure 6-13-24] • • • • • • • • • •

Review: Top 10 Vascular Lesions Double aortic arch Right arch with anomalous SCA Absent pulmonary artery Pulmonary sling Patent ductus arteriosus Anomalous pulmonary venous return Double SVC Interrupted IVC Aortic aneurysm & dissection--Marfan Coronary artery aneurysm-- Kawasaki

Marfan disease, aortic aneurysm and dissection. CT showing a dilated ascending aorta (A, top left image) and a dissection of the descending aorta (arrows)

References 1. 2.

3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18.

Katz M, Konen E, Rozenman, et al. Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies J Comput Assist Tomogr 1995; 19:564-568. Lee Ed, Siegel MJ, Hildebolt CF, Gutierrez FR, Bhalla S, Fallah JH. Multidetector CT Evaluation of Pediatric Thoracic Aortic Anomalies: Comparison of Axial, Multiplanar, and Three-Dimensional Images. AJR 2004; 182:777-784 Lawler LP, Fishman EK. Multi-detector row CT of thoracic disease with emphasis on 3D volume rendering and CT angiography. RadioGraphics 2001; 21: 1257-1273 Remy-Jardin M, Remy J, Mayo JR, Muller NL. Thoracic aorta. In: CT Angiography of the Chest. Lippincott Williams & Wilkins. Philadelphia. 2001; 29-50. Czum JM, Corse WR, Ho VB. MR angiography of the thoracic aorta. Magn Reson Clin N Am 2005; 13:41-64. Choe YH, Kim YM, Han BK, Park KG, Lee HJ. MR Imaging In the morphologic diagnosis of congenital heart disease. RadioGraphics 1997; 17:403-422. Ho VS, Corse WR, Hood MN, Rowedder AM. MRA of the thoracic vessels. Semin Ultrasound CT MR 204:192216. Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and adult patients. AJR 2003; 180:973-980. Gup HW, Park I-S, Ko JK, et al. CT of congenital heart disease: normal anatomy and typical pathologic conditions. Radiographics 2003; 23: S147-165. Hopkins KL, Patrick LE, Simoneaux SF, et al. Pediatric great vessel anomalies: initial clinical experience with spiral CT angiography. Radiology 1996; 200:811-815. Stella VB, Toutouzas P. Patent arterial duct and aortopulmonary window. In: Gatzoulis MA, Wevbb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 247-252. Morgan-Hughes GJ, Marshall AJ, Roobottom C. Morphologic assessment of patent ductus arteriosus in adults using retrospectively ECG-gated multidetector CT. AJR 2003; 181:749-754. Mahnken AH, Wildberger JE, Spuntrup E, et al. Unilateral absence of the left pulmonary artery associated with coronary-to-bronchial artery anastomosis. J Thorac Imaging 2000; 15:187-190; Gupta H, Mayo-Smith WW, Mainiero MB, Dupuy DE, Abbott GF. Helical CT of pulmonary vascular abnormalities. AJR 2002; 178: 487-492. Park HS, Im JG, Jung JW, et al. Anomalous left pulmonary artery with complete cartilaginous ring. J Comput Assist Tomogr 1997; 21:478-480. Zwetsch B, Wicky S, Meuli R et al. Three-dimensional image reconstruction of partial anomalous pulmonary venous return to the superior vena cava. Chest 1995; 108:1743-1735, Dillon EH, Camputaro C. Partial anomalous pulmonary venous drainage of the left upper lobe vs duplication of the superior vena cava: distinction based on CT findings. AJR 1993;160: 375-379. Van Praagh S, Carrera ME, Sanders S, Mayer JE, Van Praagh R. Partial or total direct pulmonary venous drainage to the right atrium due to malposition of septum primum. Chest 1995; 107:1488-1498.

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19. Woodring JH, Howard TS, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics 1994; 14:349-369. 20. Remy-Jardin M, Remy J, Mayo JR, Muller NL. Superior vena cava syndromes. In: CT Angiography of the Chest. Lippincott Williams & Wilkins. Philadelphia. 2001; 130-139 21. White CS, Blaffa JM, Haney PH, Pace ME, Campbell AB. MR imaging of congenital anomalies of the thoracic veins. RadioGaphics 1997; 17:595-608. 22. Bass JE, Redqine MD, Kramer LA, Huynh PT, Harris JH. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 2000; 20:639-652. 23. Yamada I, Nakagawa T, Himeno Y, Numano F, Shibuya H. Takayasu arteritis.: evaluation of the thoracic aorta with CT angiography. Radiology 1998; 209:103-109

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Pediatric Cardiac Imaging Part II: Congenital Heart Disease Marilyn J. Siegel, MD • •

Lecture Outline

• •

Review CT & MRI techniques Describe CT and MR appearances of common congenital heart diseases ➢ Neonates and infants ➢ Older children & adolescents Discuss treatment options

Techniques: Cardiac CTA/MRA

• • • • •

MRA: Same as mediastinal vascular lesions (see part I), but add cine sequence ➢ Black blood: delineation of anatomic structures and vessel lumens ➢ White blood: evaluation of shunts/jets as turbulent flow causes dephasing ➢ Gd for vessels: vessel stenoses ➢ Cine: function & flow dynamics

CT Angiography: Basic Protocol

•

Use a PE protocol Thin collimation (< 1mm) Fast table speed Low mAs and kVp Bolus tracking for scan initiation ➢ Trigger @ 100-120 HU

Pediatric Cardiac Imaging: What you need to know Neonates & Infants: ➢ Top Ten Diagnoses ➢ CT & MRI Features

Top 10 Congenital Heart Diseases Neonates & Infants SHUNTS VSD ASD PDA AV Canal

CYANOTIC HEART LESIONS Tet of Fallot TGV TAPVR* Tricuspid atresia* Truncus * Adds to 11 because TA &TAPVR have similar frequency • • • • •

OBSTRUCTIVE Coarctation HLHS

I. The Shunt Lesions

•

Atrial septal defect Ventricular septal defect Patent ductus arteriosus Atrioventricular canal (chest x-ray: cardiomegaly & increased vascularity

Atrial Septal Defects • • •

Sinus venosus (10%) ➢ Level of SVC ➢ associated with PAPVR Secundum (60%) ➢ Level of fossa ovalis Primum (30%) Lower atrial septum ➢ Part of AV canal defect

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Sinus Venosus ASD [Figure 6-14-1]

Figure 6-14-1

Sinus Venosus ASD: MRI • •

Secundum ASD Large PAs Mid septal defect

Secundum ASD [Figure 6-14-2] •

Ostium Primum ASD •

•

Low septal defect Sinus venosus ASD. CT scan at level of superior ➢ Just above AV valves vena cava (left panel) shows anomalous right Two types upper lobe vein (arrow) draining into superior vena ➢ Partial: involves atrial septum & mitral valve cava. CT scan more caudal (right panel) shows ➢ Complete: involves atrial & ventricular septums & sinus venosus ASD (arrow) both AV valves ❖ Aka AV canal Figure 6-14-2

Ostium Primum ASD: CT/MR •

Partial primum ASD ➢ Low ASD + mitral insufficiency AV canal ➢ Low ASD, high VSD + common AV valve

Complete Atrioventricular Septal Defect [Figure 6-14-3] ASD: Overview [Figure 6-14-4] •

ASD Repair •

•

Occluder devices ➢ Small secundum lesions ➢ Amplatzer or CardioSeal Surgical repair ➢ Large Secundum ASD ➢ Sinus venosus ➢ Primum ASD

Secundum ASD (arrow). Atrial septal defect (arrow) is at the level of fossa ovalis and aortic valve. The right atrium is enlarged

Figure 6-14-3

Repaired ASD Occluder device for small secundum ASD

Figure 6-14-4

Complete atrioventricular septal defect (aka AV canal and endocardial cushion defect). CT scan and MRI show a low atrial septal defect (ASD) and high ventricular septal defect (VSD). In this lesion, there is a common atrioventricular valve

Summary. Sinus venosus ASD (left panel). Secundum ASD (middle panel). Primum ASD (right panel)

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Occluder Devices [Figure 6-14-5] •

Figure 6-14-5

Repaired ASD

• •

Septal patch for large secundum ASD & venosus & primum ASD

Ventricular Septal Defects Most common CHD Locations ➢ Sub-aortic (80%) ❖ (perimembranous) ➢ Intramuscular) ➢ Sub-pulmonic ➢ RV inlet (associated with AV canal)

Amplatzer occluder for smaller ASD. CT scans showing two disks connected by a short neck in the location of the atrial septum. RA=right atrium, LA=left atrium, PA=dilated pulmonary artery

Approximate Location of VSDs Subaortic VSD [Figure 6-14-6]

Figure 6-14-6

Muscular VSD [Figure 6-14-7] Subpulmonic ( supracristal) VSD [Figure 6-14-8]

• • • • •

VSD Subaortic Most Common Muscular Multiple RV Inlet With canal Subpulmonic TOF

• • •

Small lesions may close spontaneously 30% to 40% Small lesions that fail to close are occluded with septal occluder device Large lesions closed with patch graft

• •

Tubular connection between proximal descending aorta and left PA Isolated lesion or associated with other anomalies

Overview VSD [Figure 6-14-9]

Subaortic (perimembranous) ASD (arrow). CT and MR showing the ventricular septal defect at the level of the aortic valve (A)

VSD-Treatment

Figure 6-14-7

Patent Ductus Arteriosus

Figure 6-14-8

Intramuscular VSD (arrow). The defect is within the interventricular septum

Figure 6-14-9

Subpulmonic VSD. MR showing the septal defect (arrow) at the level of the right ventricular outlet in the supracristal area Pediatric Radiology

Summary. Subaortic VSD (far left panel). Muscular VSD (middle left panel). Ostium primum (inlet) VSD (middle right panel).Subpulmonic VSD (far right panel) 1465

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PDA [Figure 6-14-10] • • •

Figure 6-14-10

II. More Top 10 Obstructive Lesions

•

Coarctation of the aorta Hypoplastic left heart (X-ray: cardiomegaly & edema)

Obstructive Lesions Aortic Coarctation

•

•

Post-ductal ➢ Distal to left SCA ➢ Normal diameter arch ➢ Collaterals common Pre-ductal ➢ Above left SCA ➢ Hypoplastic arch

Post-Ductal Coarctation-CT Clues: dilated ascending aorta, post-stenotic dilatation & collaterals

Post-ductal Coarctation-CTA [Figure 6-14-11] MR: Postductal Coarctation •

Patent ductus arteriosus. Sagittal MRA showing patent ductus between aorta and left pulmonary artery

Figure 6-14-11

Preductal Coarctation-CT [Figure 6-14-12]

• •

Pre-ductal ➢ Above left SCA ➢ Arch hypoplasia ➢ Collaterals uncommon

Coarctation Repair Resection & end-to-end anastomosis Stents, angioplasty, patch aortoplasty

After balloon dilatation •

Complications Coarctation Repair (5-30%) Complications ➢ Re-stenosis ➢ Stent fracture ➢ Pseudo-aneurysm ❖ 5% to 12% angioplasty ❖ 33% patch aortoplasty

Figure 6-14-12

Stent Restenosis Post-op Complication: Pseudoaneurysm • •

Hypoplastic left heart syndrome Presents as CHF in neonate Classic findings ➢ Small or absent LV ➢ Hypoplastic ascending aorta ➢ Hypoplasia aortic & mitral valves ➢ ASD & PDA Preductal coarctation, neonate with heart failure. Sagittal CT showing coarctation (arrow) above origin of left subclavian artery Congenital Heart Disease

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Postductal coarctation, neonate. Transverse CT (upper left image) shows a small caliber descending aorta (arrow). Sagittal CT (middle and right images) show the level of obstruction (arrow) just below origin of left subclavian artery Pediatric Radiology

Hypoplastic Left Heart [Figure 6-14-13] Part III. Common Cyanotic Diseases SHUNTS VSD ASD PDA AV Canal

•

OBSTRUCTIVE Coarctation HLHS

CYANOTIC HEART LESIONS Tet of Fallot Tricuspid Atresia TGV Truncus TAPVR

Figure 6-14-13

Cyanotic CHD •

•

Indicates that unoxygenated venous blood is reaching aorta Causes: ➢ Right heart obstruction with Rt to Lt shunting via a septation defect ❖ TOF, tricuspid atresia ➢ Mixing of pulmonary & systemic blood due to incomplete separation Hypoplastic left heart syndrome. Transverse CT (left image) of chambers showing small left ventricle (arrow). CT (right image) at a more ❖ TGV, Truncus, TAPVR proximal level shows small ascending aorta (arrow)

Tetralogy of Fallot: Clues: 4 findings

•

Figure 6-14-14

The Tetrad: ➢ Subaortic VSD ➢ Infundibular pulmonic stenosis ➢ Overriding aorta ➢ Right ventricular hypertrophy

Tetralogy of Fallot: CTA Membranous VSD, RVH, infundibular PS, overriding aorta

Tetralogy of Fallot-MR [Figure 6-14-14] •

Surgical Repair TOF [Figure 6-14-15] Initial surgery is palliative ➢ Blalock-taussig shunt ➢ Subclavian artery to PA

Tetralogy of Fallot, MRI. Transverse MR (left panel) shows perimembranous VSD (arrow) and right ventricular hypertrophy (RVH). Sagittal MR (middle panel) shows narrowed pulmonary outflow tract (arrow). (PA=normal size main pulmonary artery). Sagittal MR (right panel) shows aorta (Ao) overriding right and left ventricles, VSD (arrow), and right ventricular hypertrophy

Figure 6-14-15

Blalock-Taussig shunt for palliation of tetralogy of Fallot. 3D volume rendered CT showing subclavian artery (SCA) to pulmonary artery (PA) anastomosis (arrow) Pediatric Radiology

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•

Surgical Repair TOF [Figure 6-14-16]

Figure 6-14-16

Definitive repair ➢ enlargement of PA via patch graft of pulmonary valve annulus or outflow tract ➢ closure of VSD

• • • •

Fatty bar between RA & RV Hypoplastic RV Large RA VSD

•

RAE, fatty tricuspid valve, small RV

Tricuspid Atresia [Figure 6-14-17]

Tricuspid Atresia •

Tricuspid Atresia: Surgical Repair [Figure 6-14-18] •

•

Definitive repair of tetralogy of Fallot. Transverse CT (left image) at level of pulmonary artery shows a normal caliber main pulmonary artery with adjacent graft material. CT at level of ventricles (right image) shows closure of VSD

Glen shunt ➢ SVC to PA Fontan ➢ RA to PA

Figure 6-14-17

Total Cavopulmonary Fontan [Figure 6-14-19] Cavopulmonary Fontan ➢ Conduit between SVC & IVC which is joined to main PA

Lesions with Increased Flow D-Transposition of Great Vessels • • • • •

Ventriculoarterial discordance Aorta arises from RV Pulmonary artery from LV Circuits are in parallel ASD, VSD, PDA common Tricuspid atresia classic features. CT showing atretic valve replaced by fatty tissue (arrow), right atrial (RA) enlargement, and right ventricle hypoplasia

Figure 6-14-18 Figure 6-14-19

Surgical repairs for tricuspid atresia. Glenn shunt (left panel) is a superior vena cava (S) to pulmonary artery anastamosis. Fontan proceudre (right panel) is a right atrium (RA) to main pulmonary artery anastomosis. Note the markedly dilated right atrium. (Left panel, Glenn shunt, reprinted from Core Curriculum, Siegel M, Coley B 2005).

Congenital Heart Disease

Total cavopulmonary shunt. Superior vena cava (SVC) and inferior vena cava (IVC) joined via conduit (C), which is anastomosed to pulmonary artery (PA) 1468

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D-Loop Transposition [Figure 6-14-20] • •

Figure 6-14-20

Surgical Repair: Atrial Switch •

Intra-atrial baffle Blood from SVC and IVC enters superior chamber & directed to LV and PA Blood from pulmonary veins enters inferior chamber & directed to RV & Aorta

Atrial Switch [Figure 6-14-21] • •

Mustard Procedure

• • •

Systemic venous baffle Pulmonary venous baffle D-Transposition of great vessels. Sagittal MRA (left panel) showing aorta arising from right Current procedure of choice ventricle (RV) and pulmonary artery from left Great artery switch ventricle (LV). Transverse MRI and CT showing Aorta and PA sectioned above valves & reconnected aorta (A) anterior and to right of pulmonary artery to proper ventricles (PA) Coronary arteries reimplanted

Jantene Procedure

•

Arterial Switch: Jatene Procedure [Figure 6-14-22] • •

Figure 6-14-21

Total Anomalous PV Return

• •

Pulmonary veins drain to RA (not LA) 4 types ➢ I. supracardiac (55%) ➢ II. cardiac (coronary sinus) (30%) ➢ III. infracardiac (to portal vein or IVC) (12%) ➢ IV. two of the above (3%) ASD essential for survival Repair: reimplant

•

Veins reanastomosed to LA

• • • •

I. Single PA arises from truncus (80%) II. R and L PA arise from posterior truncus III. R and L PA arise from sides of truncus. IV. R and L PA arise from descending aorta

Total Anomalous Return: Surgery Truncus Arteriosus Atrial switch, mustard procedure. Coronal CT (upper image) shows systemic baffle directing blood from right heart to left heart where it exits into pulmonary arteries. Transverse CT (lower image) shows systemic baffle and also pulmonary venous (PV) baffle which directs blood from pulmonary veins into right ventricle where it exits into aorta

Figure 6-14-22

Jatene procedure, arterial switch. Pulmonary artery (PA) lies anterior and to right of aorta (A)

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Truncus Arteriosus Type I [Figure 6-14-23]

Figure 6-14-23

Type IV Truncus (pseudotruncus) [Figure 6-14-24]

•

Truncus: Surgical Repair •

Pulmonary arteries detached from common artery (truncus arteriosus) and connected to RV using a conduit. VSD is closed with a patch

Additional Lesions: Older Children & Adolescents • • • • •

Truncus arteriosus. Type I A single pulmonary artery arises from the truncus (T)

Aortic stenosis Pulmonic stenosis

Figure 6-14-24

Aortic Stenosis Valvular >> sub- or supravalvular Usually due to bicuspid valve CT/MR: dilated ascending aorta ➢ Bicuspid valve

Aortic Stenosis-CT [Figure 6-14-25] •

Ca++ uncommon before 4th decade

• •

Thickened, domed leaflets during systole Thick wall left ventricle

Bicuspid Aortic Valve Truncus arteriosus type IV. Oblique MRA showing both pulmonary arteries arise from the descending aorta. Transverse MR showing an associated VSD (arrow)

AS and Bicuspid Valve-MR

• • • •

Figure 6-14-25

Aortic Stenosis: Treatment

•

Balloon dilatation Valvotomy Valve replacement Ross procedure ➢ aortic valve replaced with patient’s pulmonary valve ➢ pulmonary valve replaced with cadaveric valve

Valvular Pulmonic Stenosis

•

Valvular stenosis most common (95%) ➢ 90% due to commissural fusion ➢ 10% due to a dysplastic valve ❖ thickened, but non-fused commissures Aortic stenosis. Transverse and coronal CT showing dilated ascending aorta

Pulmonary Artery Stenosis [Figure 6-14-26] CT/MR ➢ Dilated main PA ➢ Dilated Lt PA ➢ Rt ventricular hypertrophy

Figure 6-14-26

Pulmonic valve stenosis. CT showing dilated main (M) & left (L) pulmonary arteries Congenital Heart Disease

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• • •

Pulmonic Stenosis: Repair

•

Surgical valvotomy Percutaneous balloon valvuloplasty Less successful in patients with valvular dysplasia

Summary Diagnosis of CHD depends on knowledge of the anatomic abnormality, the clinical findings & understanding of imaging findings

References 1. 2. 3. 4. 5. 6. 7. 8. 9.

10. 11. 12. 13. 14. 15. 16. 17. 18.

19.

Amplatz K, Moller JH Radiology of Congenital Heart Disease Mosby –Year Book Inc 1993 Boxt LM. MR imaging of congenital heart disease. Magn Reson Imaging Clin North America;1996:4:327-359 Choe YH, Kim Ym, Han BK, Park KG, Lee HJ. MR imaging in the morphologic diagnosis of congenital heart disease. RadioGraphics 1997; 17:403-422. Poustchi-Amin M, Gutierrez FR, Brown JJ, et al. How to plan and perform a cardiac MR imaging examination. Radiol Clin North Am 2004; 42:497-514 Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of congenital heart disease in pediatric and adult patients. AJR 2003; 180:973-980. Goo HW, Park I-S, Ko J-K, et al. CT of congenital heart disease: normal anatomy and typical pathologic conditions. Radiographics 2003; 23: S147-165 Gutierrez FR, Canter CE, Mirowitz SA: MR appearance of congenital heart defects. In: Gutierrez FR, Brown JJ, Mirowitz SA (eds.): Cardiovascular magnetic resonance Imaging. St. Louis: Mosby Year Book, 1992;72-83. Higgins CB: Congenital heart disease. In: Higgins CB, Hricak H, Helms CA (eds.): Magnetic Resonance Imaging of the body. 3rd ed. Philadelphia: Lippincott-Raven, 1997; 461-518. Lee E, Siegel MJ, Guttierez F, Hildebolt CF, Bhalla S, Fallah JH. Multidetector CT evaluation of thoracic aortic anomalies in pediatric patients and young adults: comparison of thoracic axial, multiplanar, and 3D images. AJR 2004; 182:777-78410. Kaemmerer H. Aortic coarctation and interrupted aortic arch. In: Gatzoulis MA, Wevbb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 253-264. Becker C, Soppa C, Fink U et al. Spiral CT angiography and 3D reconstruction in patients with aortic coarctation. Eur Radiol 1997; 7:1473-1477. Roest AA, Helbing WA, van der Wall EE. Postoperative evaluation of congenital heart disease by magnetic resonance imaging. J Magn Res Imag 1999; 10:656-666. Donnelly LF, et al. MR imaging of cono-truncal abnormalities. AJR 1996; 166:925-928. Connelly M. Common arterial trunk. In: Gatzoulis MA, Webb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 265-271. Jacobs ML. Congenital heart surgery nomenclature and database project: truncus arteriosus. Ann Thorac Surg 2000; 69:S50-S55. Kim TH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR 2000; 175: 1381-1386. Bardo DM, et al. Hypoplastic left heart syndrome. Radiographic 2001; 21:705-717. Mavroudis C, Backer CL, Deal BJ. Venous shunts and the Fontan circulation in adult congenital heart disease. In: Gatzoulis MA, Wevbb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 79-83. Feeedom RM, Li J, Yoo S-J. The complications following the Fontan operation. In: Gatzoulis MA, Wevbb GD, Daubeney PEF. Adult Congenital Heart Disease. Churchill Livingstone. Edinburgh 2003; 85-91.

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Congenital Heart Disease Gael J. Lonergan, MD • • • •

Topics Assessing the chest film Acyanotic CHD with increased PBF Cyanotic CHD with increased PBF Cyanotic CHD with decreased PBF Increased PBF ACYANOTIC VSD ASD PDA ECD AP Window

Increased PBF CYANOTIC TGV TAPVR Truncus Tric Atresia Single Chamber HLHS

Decreased PBF CYANOTIC Tet of Fallot Ebstein Tric Atresia Pulm Atresia

• •

Assessing the Chest Film Pulmonary blood flow (PBF) – KEY!!! Cardiomediastinal silhouette ➢ Configuration ➢ Size • Aortic arch side • Situs

• •

Pulmonary Blood Flow Normal Increased (arterial overcirculation and venous congestion) ➢ Requires 2 : 1 (pulmonary : systemic) shunt to see increased PBF @ CXR • Decreased • Systemic collaterals (AKA bronchial or major aorticopulmonary collaterals) •

Pulmonary Blood Flow Indicators • • •

•

Gestalt – too big, seen too far in periphery (look at hilar vessels – most reliable and most technique - independent) Main pulmonary artery Interlobar artery diameter = supraaortic tracheal diameter (+/– 2 mm) Bronchovascular couplet: diameter artery = diameter bronchus in upper zones in “normal” patients (less than 2:1 shunt) Hepatic window – if vessels too large here or seen on end, there is increased PBF

Figure 6-15-1

•

Chamber Assessment [Figure 6-15-1] RA: not reliably assessed by plain film & infrequently a diagnostic discriminator • RV: retrosternal space >1/3 filled • LA: upper 1/2 of posterior cardiac border • LV: lower 1/2 of posterior cardiac border Does either or both: • Touch spine? • Extend beyond posterior tracheal line?

RV touches lower 1/3 of sternum; LA is upper 1/2 and LV is lower 1/2 of posterior cardiac border

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•

Cardiomediastinal Silhouette Size Cardiothoracic ratio: ➢ Infant < .65 ➢ Adult < .50 • Infant cardiomegaly: ➢ Apex touches lateral chest wall on AP ➢ Posterior border touches spine on lateral •

Cardiomediastinal Silhouette Configuration Acyanotic CHD: usually normal shape ➢ Cardiomegaly common • Cyanotic CHD: more often bizarre ➢ Absence of thymus - think cyanotic disease (stressed infant, agenesis) • •

Acyanotic CHD with Increased PBF Increased blood volume in lungs Can only occur in 1 of 2 ways: ➢ Passive congestion: impeded pulmonary venous return ❖ Example: congestive heart failure ➢ overcirculation: too much blood delivered to lungs ❖ Example: ventricular septal defect

Figure 6-15-2

Acyanotic CHD with Increased PBF – shunts •

[Figures 6-15-2 and 6-15-3]

Unifying theme: ➢ Pressure difference R v. L • Blood will preferentially flow thru an opening from high pressure L to lower pressure R • Blood is recirculated thru pulmonary bed (result: increased PBF)

Acyanotic CHD with Increased PBF ( L to R shunts – INTRACARDIAC) • • • • •

Ventricular septal defect (VSD) Atrial septal defect (ASD) Patent ductus arteriosus (PDA) Endocardial cushion defect (ECD) Aorticopulmonary window (APW) Pressures in infant cardiac chambers

Acyanotic CHD with Increased PBF (L to R shunts – EXTRACARDIAC) • • •

Vein of Galen aneurysm Hepatic hemangioendothelioma Peripheral AV fistula

• • • • • • •

Hypoxic cardiac injury (birth asphyxia, drowning) Anomalous origin left coronary artery Sepsis Endocardial fibroelastosis Glycogen storage disease (Pompe) Viral myocarditis Adriamycin toxicity

Figure 6-15-3

Acyanotic CHD with Increased PBF(FAILURE)

•

Caveat In infants, all forms of increased PBF may lead to pulmonary edema • Cannot reliably distinguishing arterial overcirculation from passive congestion • Consider the 3 causes of increased PBF: ➢ Intracardiac shunts ➢ Extracardiac shunts ➢ Cardiac failure VSD: blood flows from LV to RV Pediatric Radiology

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•

Ventricular Septal Defect (VSD) [Figure 6-15-3] Most common CHD (25%)

• • • • •

VSD: Hemodynamics Blood flows from LV to RV Volume overload in RV RV enlarges and hypertrophies Blood in RV goes to lungs, LA, LV. When it arrives in LV, it “decompresses” into RV ➢ LV remains normal • 4 basic types ➢ Membranous 80% ➢ Muscular (often multiple) 10% ➢ Supracristal 5% ➢ Inflow / AV canal type 5% ➢ Size matters, not location! • •

Radiology [Figures 6-15-4 and 6-15-5] Large heart RV and LA large

•

VSD: Presentation Usually in 2nd month of life ➢ Pulmonary vascular resistance has dropped sufficiently to allow increased shunting • Presentation ➢ Frequent pneumonias ➢ Failure to thrive ➢ CHF

Figure 6-15-4

•

VSD: Natural History Small: close spontaneously by 3 years ➢ Muscular ingrowth ➢ By 2 months of age: follow v. repair • Large: repair via trans-atrial approach ➢ Goretex patch ➢ Pericardial patch

VSD: cardiomegaly, large RV, and large LA

• •

VSD All need to be closed Large: ➢ Significant L to R shunting ➢ Less turbulence ➢ Pulmonary HTN, Eisenmenger syndrome • Small: ➢ Less L to R shunting ➢ More turbulence ➢ Higher risk of bacterial endocarditis • • • • •

Figure 6-15-5

Atrial Septal Defect (ASD) [Figures 6-15-6 a and b] Incidence 10% 3 : 1 female : male Most common shunt to present after the age of 3 Size determines shunt Locations ➢ Ostium secundum (fossa ovalis) 60% ❖ Patent foramen ovale ➢ Ostium primum (part of cushion) 30% ➢ Sinus venosus (just below SVC, usually with partially anomalous pulm. venous return from RUL)

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MRI of VSD

Pediatric Radiology

• • •

ASD: Hemodynamics

•

Figure 6-15-6a

Blood shunts LA to RA to RV to lungs to LA RV & RA enlarge in response to increased volume L heart: no increased volume (blood from lungs returning to LA decompresses into RA) ➢ LA, LV, and aorta are normal

Radiology [Figures 6-15-7 and 6-15-8] RV large; no L-sided chamber enlargement

Figure 6-15-6b ASD locations: uppermost is sinus venosus, middle is ostium secundum, lowest is ostium primum

ASD

Figure 6-15-7

•

ASD: Complications Pulmonary HTN & Eisenmenger syndrome ➢ 30% with mod-large ASD develop HTN • Atrial arrhythmias (volume overload) • Paradoxical emboli • •

ASD

ASD: R to L Shunting [Figure 6-15-9] “Paradoxical” emboli R to L shunting may occur: ➢ Pregnancy (decreased SVR) ➢ Diving,valsalva, cough ➢ COPD, pulmonary embolus

•

Figure 6-15-8

Paradoxical Embolus Ischemic stroke: no cause in 35-40% ➢ “Cryptogenic stroke” ➢ Paradoxical embolus from ASD? • Pts with cryptogenic ischemic stroke and PFO, on anticoagulant therapy: ➢ 1.2% risk/year of TIA ➢ 3.4% risk/year of TIA or stroke

Figure 6-15-9

ASD at MR

R to L shunting across septal defect

Pediatric Radiology

1473 1475

Congenital Heart Disease

• •

ASD

Figure 6-15-10

Repair all between 2-5 years Repair methods: ➢ Amplatzer occluder (secundum only) ❖ Repair mortality <1% ➢ Suture (small, any location) ➢ Patch (any location) ❖ Goretex ❖ Pericardium

• •

Patent Ductus Arteriosus (PDA) [Figure 6-15-10] Incidence 8% In utero, allows fetal blood returning from placenta to bypass underdeveloped lungs • 2:1 F:M • Birth: ➢ Lungs aerate, fluid leaves ➢ Pulmonary pressure drops ➢ Ductus closes by 15 hours ➢ All RV blood to lungs ➢ Normal PBF • Ductus may remain open ➢ High pulmonary pressures ❖ Prematurity ❖ Meconium aspiration ❖ Pulm hypoplasia (diaph hernia,oligo, ARPKD) ➢ Idiopathic

Patent ductus arteriosus

•

PDA: shunt Shunt direction determined by: ➢ Pulmonary vs systemic pressure • In otherwise normal infants, it will be L to R • • • •

Shunt is from Ao to PA LA, LV, Ao: increased volume L heart enlarges RV: increased pressure from afterload; hypertrophy

• •

LA, LV, Ao arch large Some RVH

Hemodynamics

Radiology [Figure 6-15-11]

• • •

PDA

• •

Anatomically closed by 3 weeks If open @6 months, certainly will remain Flow depends on: ➢ Width ➢ Length ➢ Shape

Figure 6-15-11

Patent Ductus Arteriosus L to R shunt: pulmonary HTN Bacterial endocarditis ➢ Turbulent flow ➢ Continuous flow ➢ 0.5%/year cumulative risk ➢ SBE prophylaxis until repair

PDA: increased PBF, enlargement of RV, LA, LV

Congenital Heart Disease

1474 1476

Pediatric Radiology

•

Patent Ductus Arteriosus

Figure 6-15-12

Treatment: ➢ Small: coils ➢ Medium to large: ❖ Mesh occluder ➢ Neonatal ❖ Clip (any size ductus) ❖ Prostaglandin inhibitors: only for stable neonates ❖ Arterial access limited by small size • Tiny, incidental PDA: Rx controversial • SBE prophylaxis if residual shunt

2 3 Spectrum of cushion defects:

1

Endocardial Cushion Defect (ECD) / Atrioventricular Canal (AV Canal) [Figure 6-15-12]

1-Normal 2-Moderate ECD: ASD & cleft AV valve leaflets 3-Severe ECD: ASD, VSD, common AV valve

• •

Incidence 4% Spectrum of lesions & severity: ➢ Ostium primum ASD ➢ + / - common anterior and posterior mitral and tricuspid valve leaflets ➢ + / - inlet VSD (most severe) • Presentation/radiographic appearance depends on severity ➢ Variable cardiomegaly and increased PBF • 40% have Down syndrome (look for 11 or 13 rib pairs, 6 sternal ossification centers) ➢ Most common cardiac lesion in Down syndrome

Figure 6-15-13

ECD: increased PBF, RV and LA enlargement

•

Radiology [Figures 6-15-13 and 6-15-14]

Figure 6-15-14

Chamber enlargement varies with nature of ECD ➢ Cardiomegaly & increased PBF • “Gooseneck” deformity at angio from LV outflow obstruction • •

Aorticopulmonary Window (APW) [Figures 6-15-15 and 6-15-16] Rare Communication between ascending aorta and pulmonary trunk or RPA • Large L to R shunt • Predominantly L chamber enlargement ➢ Similar to PDA

Figure 6-15-15

Figure 6-15-16

Aorticopulmonary window

Pediatric Radiology

ECD: gooseneck deformity of LV outflow tract

AP window: increased PBF, RV, LA, & LV enlargement 1475 1477

Congenital Heart Disease

•

Eisenmenger Physiology [Figures 6-15-17 and 6-15-18] • • • • •

L to R shunting creates increased volume in pulmonary arteries; results in increased pressure Pulmonary arteries respond by undergoing hypertrophy & endothelial thickening, creating pulmonary hypertension (a vicious cycle) if not corrected (may begin in utero) Pulmonary pressure may become higher than systemic pressure Shunt switches from L to R to R to L Deoxygenated blood from R heart shunts to L heart and systemic circulation Cyanosis results (“cyanosis tardive”)

Figure 6-15-17

Normal (left) and hypertensive (right) pulmonary artery, showing endoluminal narrowing

•

Radiology [Figures 6-15-19 and 6-15-20] “Pruned” appearance of PBF on CXR: ➢ Dilated central arteries ➢ Diminutive peripheral arteries • Irreversible course of events • Goal of surgery is to prevent this dreaded complication

Figure 6-15-18 1

2

Figure 6-15-19

Eisenmenger syndrome in VSD: 1-VSD initially shunts L to R 2-With onset of pulmonary HTN, blood begins to shunt R to L

Figure 6-15-20

ASD with Eisenmenger syndrome: large hilar arteries, diminutive peripheral vessels • •

Cyanotic CHD [Figures 6-15-21 and 6-15-22] Cyanosis: Hb saturation < 85% Deoxygenated Hb (blood from R heart) has entered systemic (L heart) circulation • This can only happen if there is . . . ➢ R to L shunt (from R-sided outlet obstruction, such as Tet of Fallot, Eisenmenger syndrome) ➢ Mixing of R & L sided blood in systemic circulation (eg: truncus arteriosus)-”admixture” lesion

Figure 6-15-21

Pulmonary artery banding reduces PBF, reducing risk of Eisenmenger syndrome

Deoxygenated blood from R side enters L side, leading to cyanosis Congenital Heart Disease

1476 1478

Pediatric Radiology

•

Admixture Lesions [Figure 6-15-23] • •

Figure 6-15-22

Mixing of R & L circulations, across a large VSD, ASD, PDA, or a “single chamber” where there usually are 2 Admixed blood flows to pulmonary circulation These are the “cyanotic with increased PBF” lesions

Admixture Lesions “5T’s” and H • • • • •

Transposition of the great vessels Total anom. pulmonary venous return Truncus arteriosus Tricuspid atresia Single chamber ➢ single ventricle ➢ double outlet right ventricle ➢ common atrium) • Hypoplastic left heart syndrome

R to L shunt lesions shunt PBF to L side of heart; there is decreased PBF

Complete Transposition of Great Vessels (d-TGV, D loop TGV) [Figure 6-15-24] • • • • • • •

Admixture lesions allow intracardiac mixing of R and L circulations; blood preferentially flows to lower pressure pulmonary vascular bed, increased PBF results and patient is cyanotic

Incidence 8% Most common cyanotic CHD presenting in neonate ➢ TOF most common overall 2 : 1 male : female Classic TGV: ➢ Aorta arises from RV ➢ Pulm art from LV 2 closed circuits ➢ Incompatible with life w/o L-R communication All have ASD Half with VSD

Figure 6-15-23

•

Radiology [Figure 6-15-25] PA not border-forming on left ➢ Mediastinum appears narrow ➢ “Egg on a string” appearance • Vast majority with L aortic arch • Small to absent thymus (makes perception of narrow vascular pedicle possible) DORV: admixture lesion

Figure 6-15-24

Figure 6-15-25

D-TGV: cardiomegaly, narrow mediastinum, increased PBF

D (classic) transposition Pediatric Radiology

1477 1479

Congenital Heart Disease

•

Surgical Repair [Figures 6-15-26 and 6-15-27]

Figure 6-15-26

Figure 6-15-27

Arterial switch (Jatene) – switching aorta & PA to normal location • Atrial septostomy (palliative) - opens ASD to improve admixture ➢ Balloon septostomy = Rashkind • Atrial switch/baffle (Mustard, Senning) systemic blood returns to LA, pulmonary returns to RA

“D” and “L” Designations • •

[Figures 6-15-28 and 6-15-29]

Refers to aortic or ventricular position “D” may refer to: ➢ Aortic position (D is ABNORMAL) ➢ Ventricular looping & position (D is NORMAL) • d-TGV = D loop TGV = complete TGV • “L” designation may refer to ➢ Aorta to left and anterior to pulmonary artery (not normal, but close) ➢ Ventricular looping & position (L is ABNORMAL)

Jatene switch repair of TGV

Figure 6-15-28

Atrial baffle (Mustard procedure) palliation of TGV

Figure 6-15-29

Congenitally Corrected TGV (L-TGV or L loop TGV) • • • •

• • •

Ventricular inversion key abnormality ➢ There is great vessel transposition, too, but... Blood circulatory pattern normal Radiograph: NORMAL High incidence associated anomalies (responsible for morbidity) ➢ Ebstein-like changes in tricuspid valve ➢ VSD ➢ Pulmonary stenosis/atresia AV valves (tricuspid & mitral) go with the D (classic) transposition of inverted ventricles Ao & PA Coronary arteries are anatomically inverted as well ➢ RV (inverted LV) supplied by two coronary arteries ➢ LV (inverted RV) supplied by one Increased mortality: ➢ Coronary artery disease (single vessel) ➢ Associated anomalies

L-TGV (transposition of ventricles)

Figure 6-15-30

Total Anomalous Pulmonary Venous Return (TAPVR) [Figure • • • •

6-15-30]

Incidence 2 % PBF returns to RA Admixes with systemic return in RA MUST have communication with L heart ➢ All have ASD ➢ Admixed blood: R to L flow

TAPVR type 2 with return of PBF to RA

Congenital Heart Disease

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Pediatric Radiology

Darling’s Classification of TAPVR : Type 1 (supracardiac) • •

Figure 6-15-31

[Figure 6-15-31]

55% Pulmonary veins drain cephalad into L SVC (aka left vertical vein), into left BCV, then into SVC (occasionally azygos) • “Snowman” heart ➢ Almost half are obstructive (from bronchial compression, vein stenosis) • • •

Type 2 (cardiac) [Figure 6-15-32] 30% Pulmonary veins drain into coronary sinus or directly into RA Non-specific appearance (like any L to R shunt). Rarely obstructive

Type 3 (infracardiac) • • • •

[Figure 6-15-33]

• • • • •

• •

12% Common pulmonary vein descends thru esophageal hiatus Drains into portal vein/ductus venosus, hepatic vein, or IVC Pulmonary venous return is always obstructed because: ➢ Long course of vein ➢ Passage through diaphragm ➢ Return through hepatic parenchyma when draining into PV/ductus venosus (most common) Pulmonary edema Heart size is NORMAL ➢ Lungs act as capacitor for obstructed pulmonary venous return Distinctive appearance: normal size heart with pulmonary edema Type 4 = combination lesion of some the above (5%) Partial APVR (clinically insignificant) [Figure 6-15-34] ➢ Most commonly is return of RUL pulmonary vein directly into SVC ➢ Scimitar syndrome is PAPVR of hypoplastic RML, RLL; drains to IVC

Type 1 (supracardiac) TAPVR; PBF returns to SVC

Figure 6-15-32

TAPVR: Variable Physiology Increased PBF Obstruction occurs in all type 3 and in 25 – 40% of type 1 (from bronchial compression or intrahepatic drainage), which creates pulmonary edema

Figure 6-15-33

Figure 6-15-34

Type 3 (infracardiac) TAPVR; PBF returns to portal vein, hepatic vein, or IVC Pediatric Radiology

Type 2 TAPVR; PBF returns to RA

Partial APVR; PBF from 1-2 lobes returns to RA 1479 1481

Congenital Heart Disease

• •

TAPVR 1: Radiology [Figures 6-15-35 and 6-15-36] • • •

Figure 6-15-35

Cardiomegaly and increased PBF “Snowman” heart from prominent left vertical vein and distended SVC Superior mediastinum looks wide and round

TAPVR 2: Radiology [Figure 6-15-37] Non-specific Looks like many L to R shunts (large heart with increased PBF)

Figure 6-15-37 TAPVR 1: “Snowman” heart & increased PBF

Figure 6-15-36

TAPVR 2: increased PBF, non-specific appearance •

TAPVR 3: Radiology [Figures 6-15-38 and 6-15-39] Always obstructive ➢ Pulmonary edema • Heart size normal (it is not seeing increased volume) • Normal size heart + pulmonary edema = TAPVR III

TAPVR 1: MRI of L SVC and large R SVC

Figure 6-13-39

Figure 6-15-38

TAPVR 3: normal size heart + pulmonary edema + increased PBF • •

Surgical Repair Depends on type Aim is to patch-graft pulmonary vein to LA or create pulmonary venous conduit to LA

Congenital Heart Disease

1480 1482

Type 3 (infracardiac) TAPVR: pulmonary venous return to portal vein

Pediatric Radiology

• • • • • • •

Truncus Arteriosus [Figures 6-15-40 ] 1% Failure of septation of truncal artery into Ao & PA Single great artery arises from heart, via single valve Gives rise to Ao, PA, and coronary arteries Truncal artery overrides a VSD (may have ASD and/or PDA too) R aortic arch in 35% Collett & Edwards classification (anatomic) ➢ Type 1: PA arises as single main PA ➢ Type 2: PAs arise separately but close ➢ Type 3: PAs arise independently, with widely spaced origins ➢ Type 4: “PA” arise from descending Aorta (aka pseudotruncus) ❖ Really bronchial arteries

Figure 6-15-40

Type 1 truncus arteriosus: single artery arises from RV & LV • • • •

Type 2 truncus

Type 3 truncus

Pseudotruncus (bronchial collaterals arise from descending Ao)

Radiology [Figures 6-15-41 and 6-15-42]

•

R aortic arch + increased PBF + cyanosis: truncus arteriosus likely Always have large heart Resembles TGV Type 2 & 3 give “hilar comma” or “hilar waterfall” sign from vertical course of pulmonary arteries descending to pulmonary hilum

Figure 6-15-41

Repair [Figure 6-15-43] Rastelli: RV to PA Conduit

Figure 6-15-43 Truncus with R Ao arch

Figure 6-15-42

Truncus arteriosus: cardiomegaly + increased PBF

Rastelli repair Pediatric Radiology

1481 1483

Congenital Heart Disease

• •

Tricuspid Atresia [Figure 6-15-44]

Figure 6-15-44

1.5% Very variable ➢ Appearance ➢ Physiology ➢ Associated defects • Blood flows RA to LA (across ASD) to LV to RV (across VSD) and out PA; admixture occurs in LA RV rudimentary • Classified into 2 types: [Figure 6-15-45] ➢ With TGV (25%) (increased PBF) ➢ Without TGV (75%) (decreased PBF) • Therefore, the great vessel served by RV is usually underperfused. This would be the: ➢ Pulmonary artery in normally related great vessels (therefore PBF is decreased) ➢ Aorta in TGV (therefore PBF is increased) • •

Radiology [Figure 6-15-46] •

Figure 6-15-45

Non-specific Increased or decreased PBF depending on transposition Difficult dx to exclude!

Tricuspid atresia with normally related Ao & PA (decreased PBF)

Figure 6-15-46

Surgical Repair •

[Figure 6-15-47]

Correction with Fontan (conduit between RA and main PA and closure of VSD)

Single Ventricle/DORV/ Common Atrium •

[Figure 6-15-48]

•

All have single dominant chamber (where normally there are 2 separate L and R chambers) into which all blood flows Tricuspid atresia with transposition Admixture occurs in this of Ao & PA (increased PBF) single chamber

Tricuspid atresia with TGV: cardiomegaly + increased PBF

Figure 6-15-47

Figure 6-15-48

Single chamber lesions: single ventricle, double outlet right ventricle, & common atrium

Congenital Heart Disease

1482 1484

Fontan repair of tricuspid atresia

Pediatric Radiology

•

Single Ventricle [Figures 6-15-49]

• •

Figure 6-15-49

All blood returns to the atria and into single ventricle (admixture occurs here), and is pumped from this into the great vessels May have increased or decreased PBF depending on outflow tract obstructions Radiographic appearance hence very variable

Double Outlet Right Ventricle • • •

[Figure 6-15-50a]

Origin of both great vessels from RV Single ventricle Usually with VSD Admixture occurs in RV and admixed blood is pumped to systemic and pulmonary circulations

Single Ventricle vs DORV •

[Figure 6-15-50b]

Single ventricle: cardiomegaly + increased PBF

Figure 6-15-50b

Figure 6-15-50a

To the patient, not much physiologic difference • Both have: ➢ Admixture of circulations in dominant ventricle ➢ A functional single ventricle that pumps admixed blood to both pulmonary and systemic circulations •

Common Atrium [Figures 6-15-51] Essentially huge ASD permitting significant admixture between RA & LA ➢ No gradient L-R • Very uncommon lesion • Large heart with increased PBF (nonspecific)

Double outlet right ventricle DORV: cardiomegaly + increased PBF

Figure 6-15-51

Figure 6-15-52

Common atrium (similar appearance to large ASD) • • • • • • • •

Hypoplastic Left Heart Syndrome: HLHS [Figure 6-13-52] 8% Most common cause cardiac death 1st week of life Hypoplastic LV, AoV, proximal Ao, LA, MV (degrees variable) L-sided outflow tract obstruction Variable severity Systemic perfusion of aorta entirely thru PDA @ pulmonary pressures RV failure rapidly ensues, especially as ductus closes in 24 – 48 hours Infants are dusky (poorly perfused with admixed blood) ➢ They are too poorly perfused to appear cyanotic • Dusky infant in failure in first 48 hrs of life = HLHS Pediatric Radiology

1483 1485

Hypoplastic left heart syndrome Congenital Heart Disease

• • •

Radiology [Figure 6-15-53]

• •

Figure 6-15-53

Typically very large heart Failure One of very few conditions that cause failure in first 24 hours of life

Surgical Repair Cardiac transplant Norwood (3 stage repair) ➢ Stage 1: (birth) ❖ Conduit RV to Ao root ❖ Divide PA from RV and ductus ❖ Perfuse PA via BT shunt ➢ Stage 2: (6 mos) HLHS: cardiomegaly + pulmonary ❖ Construct Glenn shunt (SVC to PA) to reduce RV volume edema load ➢ Stage 3: (18 mos) Figure 6-15-54 ❖ Extracardiac Fontan. Direct blood from IVC & SVC thru RA to PA

Conduit RV to Aorta: newborn [Figure 6-15-54] Blalock-Taussig Shunt: newborn [Figure 6-15-55] Bidirectional Glenn Shunt: 4-6 mos [Figure 6-15-56] Extracardiac Fontan: 2 years [Figure 6-15-57] Fontan: 2+ years [Figure 6-15-58]

Figure 6-15-56

Figure 6-15-55

Rastelli repair for HLHS: conduit from RV to Aorta

Figure 6-15-57

BT shunt: subclavian artery to ipsilateral PA

Figure 6-15-58

Glenn shunt: SVC to R PA (bidirectional = perfuses R and L PA)

Extracardiac Fontan: SVC and IVC grafted to PA (bypasses RA & RV)

Fontan: RA to PA graft (adds atrial kick to perfusion pressure) Congenital Heart Disease

1484 1486

Pediatric Radiology

Cyanotic CHD with Decreased PBF – R to L Shunt Lesions • •

[Figure 6-15-59]

•

Patients with decreased PBF are ALWAYS cyanotic Blood shunts right to left (bypasses lungs so is not oxygenated); there is decreased PBF

Figure 6-13-59

R to L Shunts

• • • •

2 common features: ➢ Opening between R and L sides of heart (allows R to L shunting) ➢ R-sided outflow tract obstruction (pulmonary stenosis/ atresia, tricuspid stenosis/atresia)

R to L Shunt Lesions “TET P” Tetralogy of Fallot Ebstein malformation Tricuspid atresia Pulmonary atresia

• • •

Tetralogy of Fallot [Figure 6-15-59] Incidence 9% Most common cyanotic CHD Associated with Down Syndrome ➢ ECD most common cardiac lesion in Down syndrome • Tetrad: ➢ VSD (usually large) ➢ Infundibular pulmonary stenosis ➢ Overriding aorta ➢ R ventricular hypertrophy •

Tetralogy of Fallot

Figure 6-15-60

Associated Anomalies • • • •

R aortic arch in 25% (usually mirror image branching) ➢ R arch also seen in 35–40% Truncus (most highly associated) ➢ But TOF much more common Pulmonary valvular stenosis (90%) Peripheral pulmonary stenoses (75%) ASD (10%) (w/ VSD=pentalogy of Fallot) Enlarged systemic collateral arteries

• •

Physiology Large VSD means pressure LV = RV Severity of pulmonary stenosis dictates amount of R to L shunt ➢ Mild stenosis = little/ no shunting = “pink tet”) • Tet spells – paroxysmal dyspnea progressing to cyanosis and unconsciousness (unknown etiology) • Squatting: patient squats to increase systemic resistance, decrease R to L shunting, and improve PBF. Especially important when exercising / playing (SVR decreases with exercise, so R to L shunting worsens) • •

Tetralogy: coeur en sabot heart

Figure 6-15-61

Radiology [Figures 6-13-60 and 6-13-61] •

Boot-shaped heart (“coeur en sabot” or heart in a boot) Concave PA segment – gives unusually straight (horizontal) upper L cardiac border The more severe the pulmonary outflow obstruction, the more “classic” the X-ray appearance Tetralogy: angiogram of large VSD, overriding Ao, pulmonary stenosis

Pediatric Radiology

1485 1487

Congenital Heart Disease

•

Surgical Repair [Figure 6-15-62]

Figure 6-15-62

Corrective: ➢ Patch VSD ➢ Ao isolated to LV ➢ Widen infundibulum, ➢ Repair other anomalies

Waterston [Figure 6-15-63] Potts [Figure 6-15-64] Figure 6-15-63

Figure 6-15-64

Tetralogy corrective repair

Figure 6-15-65

Waterston palliative shunt • •

Potts palliative shunt

Surgical Complications [Figure 6-15-65] RV outflow tract aneurysms Obstruction of a shunt ➢ Look for asymmetry of PBF ➢ Change from baseline

•

Ebstein Malformation [Figure 6-15-66] 2 tricuspid valve leaflets displaced into RV ➢ RV functionally small ➢ “Atrialization” of RV ➢ RV outflow tract obstructive • Tricuspid valve insufficiency • ASD shunts R to L • RA is large Figure ➢ Cardiomegaly • •

RV outflow tract aneurysm, S/P Tet repair

6-15-66

Figure 6-15-67

Radiology [Figure 6-15-67] Cardiomegaly (RA dilation) Decreased PBF

Ebstein malformation Congenital Heart Disease

1486 1488

Ebstein malformation: large heart, decreased PBF Pediatric Radiology

• •

Tricuspid Atresia [Figure 6-15-68] • • • •

Figure 6-15-68

Incidence 1.5% 75% of TA have decreased PBF (they do NOT have associated TGV) ASD shunts R to L Small VSD PA arises from diminutive RV PBF decreased

2

1

• •

Radiology [Figure 6-15-69] Mild cardiomegaly Increased or decreased PBF ➢ Depends on whether PA or Ao arises from RV • Very variable • •

Tricuspid atresia: 1-With transposition (increased PBF) 2-With normal great vessels (decreased PBF)

Surgical Repair [Figure 6-15-70] Palliative L to R conduits Fontan: ➢ Conduit from RA to PA ➢ RV usually closed off

Figure 6-15-69

Figure 6-15-70

•

Single Ventricle Physiology Common disorders: ➢ Tricuspid atresia ➢ Hypoplastic left heart ➢ Pulmonary atresia ➢ DORV • Increased or decreased pulmonary vascularity ➢ Determined by R outflow obstruction (eg.: pulmonary stenosis)

Single Ventricle Protocol All: single ventricle serves Ao

Tricuspid atresia with normally related vessels: decreased PBF Fontan palliation of tricuspid atresia

•

Step 1 (newborn): adjust PBF ➢ BT shunt for decreased PBF ➢ Banding, other Rx for increased PBF • Step 2 (4-6 mos): take volume load off heart ➢ Bidirectional Glenn shunt • Step 3 (2 yrs): ➢ IVC and SVC serve PA ❖ Via RA = Fontan ❖ Directly = extracardiac Fontan • • • • •

Incidence 1% Pulmonary atresia with VSD = tet physiology Similar to tricuspid atresia physiologically Lungs perfused via PDA “Ductal dependent” lesion

• •

Appearance variable Usually large heart

Figure 6-15-71

Pulmonary Atresia with Intact Ventricular Septum [Figure 6-15-71]

Radiology [Figure 6-15-72]

Pediatric Radiology

Pulmonary atresia with intact ventricular septum

1487 1489

Congenital Heart Disease

Top 10 CHD 1. VSD 2. ASD 3. Tetralogy of Fallot 4. PDA 5. TGV 6. Hypopl. L Heart 7. Coarctation 8. AV Canal Defects 9. TAPVR 9. Tricuspid Atresia 10.Truncus

25% 10 9 8 8 8 5 4 2 1.5 1

Syndromes & Cardiac Lesions Down DiGeorge Ellis-van Creveld Holt-Oram Noonan Rubella Turner Williams

ECD, tetralogy, VSD Interrup. Ao arch, truncus ASD, common atrium ASD Pulmonary stenosis Peripheral PS, PDA Coarctation Ao Supravalv. Ao stenosis

References Textbooks 1. Gedgaudas E. Cardiovascular radiology. Philadelphia, Pa: WB Saunders, 1985. 2. Tonkin, ILD. Pediatric cardiovascular imaging. Philadelphia, Pa: WB Saunders, 1992. 3. Amplatz K, Moller JH. Radiology of congenital heart disease. St Louis, Mo: Mosby, 1993. Journal Articles 1. Alexiou C, Mahmoud H, Al-Khaddour A, et al. Outcome after repair of tetralogy of Fallot in the first year of life. Ann Thorac Surg. 2001;71:494-500 2. Bichell DP, Geva T, Bacha EA, Mayer JE, Jonas RA, del Nido PJ. Minimal access approach for the repair of atrial septal defect: the initial 135 patients. Ann Thorac Surg. 2000;70:115-8. 3. Coussement AM, Gooding CA. Objective radiographic assessment of pulmonary vascularity in children. Radiology 1973;109:649-654. 4. El-Najdawi EK, Driscoll DJ, Puga FJ, et al. Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg. 2000;119:880-889. 5. Fisher RG, Moodie DS, Sterba R, et al. Patent ductus arteriosus in adults - long term follow-up: nonsurgical versus surgical treatment. J Am Coll Card. 1986;8:280-284. 6. Harvey JR, Teague SM, Anderson JL, et al. Clinically silent atrial septal defects with evidence for cerebral embolization. Ann Intern Med. 1986;105:695-687. 7. Jacobs ML, Pourmoghadam KK. The hemi-Fontan operation. Semin Thorac Cardiovasc Surg, 2003;6:90-97. 8. Kreutzer C, De Vive J, Oppido G, et al. Twenty-five-year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg. 2000 Aug;120:211-223. 9. Murphy JG, Gersh BJ, McGoon MD, et al. Long term outcome after surgical repair of isolated atrial septal defect. N Engl J Med 1990;323:1645-1650. 10. Ohye RG, Bove EL. Advances in congenital heart surgery. Curr Opin Pediatr. 2001;13(5):473-481. 11. Thibeault DW, Emmanouilides GX, Nelson RJ, et al. Patent ductus arteriosus complicating the respiratory distress syndrome in preterm infants. J Pediatr 1975; 86:120-126. 12. Williams DL, Gelijns AC, Moskowitz AJ, et al. Hypoplastic left heart syndrome: valuing the survival. J Thorac Cardiovasc Surg. 2000;119(4 Pt 1):720-731.

Congenital Heart Disease

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Forensic Radiology of Child Abuse Gael J. Lonergan, MD • • • •

Figure 6-16-1

Scope of the problem1: U.S. Data for the year 2000 3 million cases reported 879,000 substantiated cases ~1200 fatalities (U.S.) in the year 2000 from abuse 1.22% of all children in US are abused

1Child Maltreatment 2000: Reports from the States to the National Child Abuse

and Neglect Data System. In: US Department of Health and Human Services Children’s Bureau (online). Available at: http://www.calib.com/nccanch/prevmnth/scope/ncands/cfm • • • • • •

Common radiographic findings in abuse Long bone fx (shaft & metaphyseal) Rib fx Skull fx Subdural & subarachnoid hemorrhage Cerebral edema Visceral injury

•

Long bone shaft fracture [Figure 6-16-1] MOST common fracture in abuse (?4x more common than metaph. fx?) when all ages considered ➢ Infants: metaphyseal, rib, & skull fx more common1,2 • Not specific for abuse . . . • Except in the very young ➢ Shaft fx (esp spiral) in a non-walking infant is suggestive of abuse w/o convincing & verifiable history • Most common sites: femur, humerus

Inflicted femoral shaft fracture

Figure 6-16-2

1Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Inflicted skeletal injury: a postmortem radiologic-histopathologic study in 31 infants. AJR 1995; 165:647-650.

2Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and nonaccidental injury in children: a comparative study. Br Med J 1986; 293:100-102.

Spiral Fracture [Figure 6-16-2]

Figure 6-16-3

Developmental Milestones 4 mos 5–6 mos 8–9 mos 15 mos 18 mos 24 mos 36 mos

raises head 90º rolls over sits alone walks alone climbs stairs runs well alternates feet up stairs

• •

Metaphyseal Fracture1 [Figure 6-16-3] High specificity for abuse Also known as “corner” and “buckethandle” fractures • Most common in lower extremity ➢ Knee ➢ Ankle • Fracture through most immature bone ➢ Primary spongiosa (metaphysis)

Spiral humeral fracture

1Kleinman PK, Marks, SC, et al. AJR 1986;

146:895-905

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•

Shaking mechanism [Figure 6-16-4] • • • • •

Figure 6-16-4

From rapid, forceful acceleration and deceleration: ➢ Shearing forces oriented perpendicular to long axis of bone Child may be held around chest (or by limb) & shaken violently Microfracture thru primary spongiosa Thicker collar of metaphyseal bone at periphery Periosteum usually normal except with extensive injury

Radiology [Figure 6-16-5] • •

Subtlest radiographic finding: metaphyseal lucency (non-specific, seen in leukemia, stress) “Corner” fracture and “bucket-handle” fracture are different projections of the same fracture Appearance depends on shape of metaphysis itself & projection of fracture

• •

Healing of metaphyseal fractures

Callus unusual – difficult to date Ηeal quickly (10 days to several wks) ➢ prompt radiography is ESSENTIAL • The younger the infant, the quicker the healing • Usually no deformity or sequela except in the unusual case of a hip metaphyseal fracture (may cause coxa vara) • •

Shaking mechanism

Rib fracture [Figures 6-16-4, 6-16-6 and 6-16-7 • • • •

Fracture anywhere on rib Posterior fracture: high specificity for abuse ➢ All locations: very suspicious Occur with chest compression, typically during violent shaking Seen in 35 – 60% of abused infants (<18 mos)1,2 Lateral rib fracture: AP compression “folds” rib laterally, causing fx Posterior rib fracture: posterior compression levers rib end over transverse process ➢ Fx most pronounced at ventral cortex ➢ Posterior compression/impact necessary for fracture to occur

Figure 6-16-5

1 Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Lateral and frontal views of metaphyseal fracture Inflicted skeletal injury: a postmortem radiologic-histopathologic study in 31 infants. AJR 1995; 165:647-650. 2Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-accidental injury in children: a comparative study. Br Med J 1986; 293:100-102.

Figure 6-16-6 Figure 6-16-7

Cross sectional diagram of chest squeeze by adult hands

Multiple bilateral rib fractures Forensic Radiology of Child Abuse

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Visualization of posterior rib fractures1

Figure 6-16-8

•

[Figures 6-16-8 and 6-16-10]

High miss rate acutely unless oblique views or bone scan performed • Production of callus (best at 7-14 days) aids visualization • To increase detection: ➢ Skeletal technique chest film (NOT chest radiograph technique) ➢ Bone scintigraphy ➢ Chest CT ➢ Oblique chest films (bone technique) ✧ Consider as part of original skel survey ➢ Follow-up films in > 7 days

Posterior rib fractures without evidence of healing

1Kleinman PK, Marks SC, Adams VI, Blackbourne BD. Factors affecting

Figure 6-16-9

visualization of posterior rib fractures in abused infants. AJR 1988; 150:635-638. •

Rib fracture & CPR •

•

Posterior compression NOT a feature of CPR Experimental studies fail to reproduce posterior fractures with CPR or report their occurrence1,2,3 Rib fractures (anterior and lateral) rarely seen after CPR in normally mineralized bones of infants & young children

Occult posterior rib fractures seen at scintigraphy

Figure 6-16-10

1Kleinman PK, Schlesinger AE. Mechanical factors associated with posterior rib fractures: laboratory and case studies. Pediatr Radiol 1997; 27:87-91. 2Feldman KW, Brewer DK. Child abuse, cardiopulmonary resuscitation, and rib fractures. Pediatrics 1984; 73:339-342. 3Spevak MR, Kleinman PK, Belanger PL, Primack C, Richmond JM. Cardiopulmonary resuscitation and rib fractures in infants. A postmortem radiologic-pathologic study. JAMA 1994; 272:617-618.

Healing of fractures1 • • • •

No callus fx < 14 days old Callus fx > 7 days old Caveat: these are general estimates and should not be thought of as fixed time frames, as healing & callus formation are a continuum The younger the child, the faster the healing

CT of healing left posterior rib fracture

1O’Connor JF, Cohen J., in: Kleinman PK Diagnostic Imaging of Child Abuse, 2nd

ed. Mosby, 1998 168–177. •

Figure 6-16-11

Spine injury [Figure 6-16-11] Abuse (typically shaking) results in: ➢ Compression & superior endplate fxs at

thoracolumbar junction from hyperflexion1 ➢ Avulsion injury to interspinous ligament and spinous process cartilage (hyperflexion mechanism)2 • Spinous process fractures are high specificity, though unusual 1Kleinman PK, Marks SC. Vertebral body fractures in child abuse. Radiologic-histopathologic correlates. Invest Radiol 1992; 27:715-722. 2Swischuk LE. Spine and spinal cord trauma in the battered child syndrome. Radiology 1969; 92:733-738. Pediatric Radiology

1491 1493

Vertebral compression fractures Forensic Radiology of Child Abuse

• •

More sensitive than plain films for rib fractures Probably slightly more sensitive for detection of some other fractures

• • • • •

Higher radiation dose Insensitive for skull fxs (always need supplementary plain films of skull) Less sensitive than plain films for the detection of metaphyseal & vertebral fx Can’t determine age or type of fracture More technically & professionally demanding & more costly

Scintigraphy: Advantages

Scintigraphy: Limitations [Figure 6-16-12]

•

Figure 6-16-12

Evaluation of skeletal injury • • • •

Skeletal survey with high-detail film (mammography film or high-detail extremity film ideal) At least 2 views of any abnormality Do all of above even if post-mortem! consider chest CT, scintigraphy, or repeat CXR to visualize missed posterior rib fxs Specimen radiography of abnormalities in postmortem child with subsequent dissection & dating

• • • • • • • • • • • •

AP thorax AP humeri AP forearms Oblique hands AP feet AP femora AP & LAT tibiae AP & LAT skull AP pelvis LAT C-spine LAT thorax LAT L-spine

• • • • •

Metaphyseal fracture Posterior rib fracture Spinous process fracture Sternal fracture Scapular fracture

• • • • • •

Multiple fractures, especially bilateral Fractures of different ages Epiphyseal separation Vertebral body fracture & subluxation Digital fractures Complex skull fractures

• • •

Clavicular fracture Long bone shaft fracture Linear skull fracture

The skeletal survey (all: bone technique)

Skull fracture missed at scintigraphy

High specificity

Moderate specificity

Common, but low specificity

•

Cranial injury Leading cause morbidity & mortality ➢ Mortality peaks at 6 months • Mechanisms: ➢ Shaking ➢ Direct blow ➢ Strangulation / suffocation • Shaking alone is sufficient to cause fatal CNS injury (shearing forces) Forensic Radiology of Child Abuse

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•

In the first year of life1: ➢ 64% of all head injuries needing admission or with positive CT findings are inflicted (excluding simple skull fracture) ➢ 95% of all serious head injuries are inflicted

Figure 6-16-13

1Billmire ME, Myers PA. Serious head injury in infants: accident or abuse? Pediatrics 1985; 75:340-342.

•

Specific injuries [Figures 6-16-13 and 6-16-14] Edema ➢ Most common, but non-specific as to mechanism (blow, strangulation, post-traumatic apnea, etc) • Shear injury at grey-white junction and in large WM tracks (c. callosum) • Cortical contusion, laceration • SAH & SDH (when interhemispheric, very worrisome for abuse)1 1Zimmerman RA, Bilaniuk LT, Bruce D, Schut L, Uzzell B, Goldberg HI. Computed tomography of craniocerebral injury in the abused child. Radiology 1979; 130:687-690.

Left cerebral edema and SDH

Figure 6-16-14

Interhemispheric extra-axial hemorrhage • • •

[Figures 6-16-15 to 6-16-17]

Blood adjacent to falx, usually asymmetric & posterior Difficult to distinguish SAH from SDH here From violent shaking: hemispheres impact falx, on rebound bridging veins to sagittal sinuses are torn • May be associated with posterior convexity and tentorial hematomas • Interhemispheric extraaxial hemorrhage may be difficult to distinguish from normal falx (caveat: normal falx may appear strikingly bright in presence of global cerebral edema) ➢ Thicker than expected ➢ Irregular thickness ➢ Asymmetric thickness ➢ Extension into a posterior convexity SDH

Figure 6-16-15

Left frontal white matter tear

Figure 6-16-16

Diagram of SDH

Figure 6-16-17

Interhemispheric hemorrhage

Interhemispheric and left tentorial extraaxial hemorrhage Pediatric Radiology

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Forensic Radiology of Child Abuse

The reversal sign1,2 [Figure 6-16-18] AKA the bright cerebellum sign • •

Figure 6-16-18

Diffuse cortical and subcortical WM edema Basal ganglia, thalami, brainstem, & cerebellum retain normal attenuation so appear bright Dismal prognosis: hemispheric injury may go on to multicystic encephalomalacia, atrophy

•

1Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball WS, Jr.

Reversal sign on CT: effect of anoxic/ischemic cerebral injury in children. 1990; 154:361-368. 2Harwood-Nash DC. Abuse to the pediatric central nervous system. AJNR 1992; 13:569-575. •

The reversal sign Basal ganglia, thalami, brainstem, & cerebellum increased relative attenuation may be due to ➢ Neovascularity ➢ Neuronal preservation ➢ Vascular engorgement ➢ Mineralized neurons ➢ Petechial hemorrhages

Cerebral edema, parenchymal hemorrhage, and extraaxial hemorrhage

• •

Cerebral edema Not specific for abuse May be seen in: ➢ Drowning ➢ Non-abusive head trauma • When abuse-related, may be from: ➢ Direct brain injury ➢ Strangulation ➢ Venous pressure from chest compression ➢ Post-traumatic apnea

Sequellae • Acute • Polytrauma

CT

• Delayed presentation • Differing ages of injuries/blood collections • Normal or equivocal CT with high suspicion (better than CT for shear and SDH) • Sequela

MRI

Dating of intracranial blood1 1Bradley WG, Radiology 1993 189: 15

Stage

Form

T1 MR

T2 MR

hyperacute (<12-24 hrs) oxy Hb

iso-low

high (slightly)

acute (1-3 days)

iso-low

low

deoxy Hb

early subacute (3-7 days) intracell met Hb high

low

late subacute (1-2 wks)

extracell met Hb high

high

chronic (>2 wks)

ferritin, hemosiderin, approaches CSF with time

Forensic Radiology of Child Abuse

iso >>>> low high in center low in rim

1494 1496

Pediatric Radiology

• •

Hemorrhage dating by MR [Figure 6-16-19]

Figure 6-16-19

An inexact science Affected by ➢ Technical aspects MR scanner, sequences ➢ Concentration of Hb ➢ Relative concentrations of degradation products (a continuum) ➢ Arachnoid tear with leak of CSF into SDH

Rebleeding in a Chronic SDH • • • •

Outer membrane itself shown to bleed frequently1 Elevated tissue plasminogen activator (TPA) found in chronic SDH fluid1 Little (if anything) in the literature to support a single, significant rebleed as cause of rapidly enlarging SDH2 No documented cases of significant rebleed occurring in safe environment

Right SDH

Figure 6-16-20

1Chen JC, Levy ML. Causes, epidemiology, and risk factors of chronic subdural hematoma. Neurosurg Clin N Am 2000; 11:399-406. 2Block RW. Child abuse--controversies and imposters. Curr Probl Pediatr 1999; 29:249-272.

Sinus Thrombosis & Infarct [Figure 6-16-20] Cord injury •

In 9 of 11 abuse fatality autopsies in infants1,2 ➢ Subarachnoid ➢ Subdural ➢ Epidural ➢ 4 of 6 with ventral cord contusion • Probably overestimates real incidence 1Hadley MN, Sonntag VK, Rekate HL, Murphy A. The infant whiplash-shake injury syndrome: a clinical and pathological study. Neurosurgery 1989; 24:536-540. 2Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in

Right transverse sinus thrombosis

abused infants. Child Abuse Negl 1997; 21:199-205.

Figure 6-16-21

•

Skull fracture [Figure 6-16-21] Overall, poorly correlated with CNS injury (linear fx most common in NAT) ➢ 10% of all abuse cases have skull fx • Non-specific (altho common) unless: ➢ Stellate / eggshell ➢ Multiple ➢ Diastatic (>3 mm) ➢ Occipital ➢ Inconsistent history (“rolled off changing table”) •

Skull fracture from falls & stairs Falls: ➢ In 529 falls from heights up to 150 cm, 4 skull, 4 clavicle, 1 Diastatic eggshell skull fractures humerus (1.7% incidence) fractures and no significant neurologic injuries occurred ➢ Conclusion: “household” falls rarely associated with fx, almost never with any intracranial injury1,2,3 ➢ Stairs: more injurious, though significant injuries usually single (not multiple body parts)4,5

1Helfer RE, Slovis TL, Black M. Injuries resulting when small children fall out of bed. Pediatrics 1977; 60:533-535. Pediatric Radiology

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2Nimityongskul P, Anderson LD. The likelihood of injuries when children fall out of bed. J Pediatr Orthop 1987; 7:184-186. 3Lyons TJ, Oates RK. Falling out of bed: a relatively benign occurrence. Pediatrics 1993; 92:125-127. 4Joffe M, Ludwig S. Stairway injuries in children. Pediatrics 1988; 82:457-461. 5Chiaviello CT, Christoph RA, Bond GR. Stairway-related injuries in children. Pediatrics 1994; 94:679-681.

• •

Visceral Injury Seen in all ages of abused children Usually blunt trauma (punch or kick to abdomen, rapid deceleration after being thrown) • 50% mortality rate of clinically apparent visceral injury ➢ Delay in seeking treatment pivotal! • Estimated to account for 12% of all abuse fatalities ➢ 2-4% of all abusive injuries • •

Small Intestine [Figure 6-16-22]

• • • •

Hematoma (prox SB), perforation (distal SB) Intramural hematoma duod & proximal jejunum most common abdominal injury ➢ 90% in duod & prox jejunum, most near ligament of Treitz ➢ ? is bowel most tethered most vulnerable? Pt presents with pain, vomiting Mural, asymmetric mass on UGI Coiled spring appearance of heaped-up proximal mucosa +/- free air if perforation ➢ Sepsis

Bowel Injury • •

Figure 6-16-22

Duodenal hematoma

Pancreas •

Probably compressed against spine with blunt trauma Abuse is a common cause of pancreatitis (trauma is most common cause in children) May develop pseudocysts

• • •

Ascites Decreased pancreatic echogenicity at US, ductal dilation Peripancreatic inflammation

• • • •

Liver: contusion, laceration Kidney: contusion, laceration, fx Bladder: rupture Adrenal: hemorrhage

Figure 6-16-23

Pancreatic Injury Radiography [Figures 6-16-23 and 6-16-24]

Other abdominal injuries [Figure 6-1-25] Pancreatic laceration

Figure 6-16-24

Figure 6-16-25

Pancreatitis Forensic Radiology of Child Abuse

Liver contusion 1496 1498

Pediatric Radiology

• •

Hypoperfusion complex

Figure 6-16-26

Severe abdominal injury resulting in hemodynamic instability Imaging: ➢ Small caliber Ao and IVC (intravascular volume depletion) ➢ Fluid-filled bowel with enhancing wall (concentrated contrast) ➢ Ascites ➢ Intensely enhancing kidneys

Shock Bowel (Hypoperfusion Complex) [Figure 6-16-26] • •

Differential diagnosis of abuse injuries • • • • • • • • •

• • • •

Birth trauma: clavicle & humerus fxs Normal variant: periosteal new bone in infants 2–8 mos of life Hypoperfusion complex (single layer, smooth, bilaterally symmetric) Osteogenesis imperfecta: fxs, periosteal rxn (should also see osteopenia, +/– wormian bones, blue sclera) Rickets: widened, irregular physes with metaphyseal irregularity (look for osteopenia) Scurvy: white metaphyseal line of Frankel with sub-metaphyseal lucency, spurs, periostitis (look for osteopenia, Wimberger ring around epiphyses) Vit A intoxication: periosteal rxn (pronounced periostitis in ulnae & tubular bones of hands & feet) Caffey’s (presumed viral illness of infancy rarely seen today): periostitis mandible, clavicle, ulna, irritable, febrile child < 6 mos old Syphilis: metaphyseal lucencies, periostitis (destructive metaphyseal osteomyelitis lesions), + serology Leukemia: metaphyseal lucency (see lucent bands symmetrically, osteopenia) Menke’s kinky hair: metaphyseal spurring, flared anterior rib ends (no true metaphyseal fx, findings bilateral & symmetric, characteristic hair) Remember to consider: ➢ congenital insensitivity to pain ➢ spinal dysraphism

Child abuse We as radiologists are uniquely able to diagnose abuse We may be the 1st to recognize abuse Radiographic findings in abuse are among the most specific & diagnostic in medicine Our findings may be PIVOTAL to investigation & prosecution

References 1. 2. 3. 4.

Billmire ME, Myers PA. Serious head injury in infants: accident or abuse? Pediatrics 1985; 75:340-342. Block RW. Child abuse--controversies and imposters. Curr Probl Pediatr 1999; 29:249-272. Bradley WG. MR appearance of hemorrhage in the brain. Radiology 1993 189: 15-26 Chen JC, Levy ML. Causes, epidemiology, and risk factors of chronic subdural hematoma. Neurosurg Clin N Am 2000; 11:399-406. 5. Chiaviello CT, Christoph RA, Bond GR. Stairway-related injuries in children. Pediatrics 1994; 94:679-681. 6. Child Maltreatment 2000: Reports from the States to the National Child Abuse and Neglect Data System. In: US Department of Health and Human Services Children's Bureau (online). Available at: http://www.calib.com/nccanch/prevmnth/scope/ncands/cfm 7. Feldman KW, Brewer DK. Child abuse, cardiopulmonary resuscitation, and rib fractures. Pediatrics 1984; 73:339342. 8. Feldman KW, Weinberger E, Milstein JM, Fligner CL. Cervical spine MRI in abused infants. Child Abuse Negl 1997; 21:199-205. 9. Hadley MN, Sonntag VK, Rekate HL, Murphy A. The infant whiplash-shake injury syndrome: a clinical and pathological study. Neurosurgery 1989; 24:536-540. 10. Han BK, Towbin RB, De Courten-Myers G, McLaurin RL, Ball WS, Jr. Reversal sign on CT: effect of anoxic/ischemic cerebral injury in children. 1990; 154:361-368. 11. Harwood-Nash DC. Abuse to the pediatric central nervous system. AJNR 1992; 13:569-575. 12. Helfer RE, Slovis TL, Black M. Injuries resulting when small children fall out of bed. Pediatrics 1977; 60:533-535. Pediatric Radiology

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13. Joffe M, Ludwig S. Stairway injuries in children. Pediatrics 1988; 82:457-461. 14. Kleinman PK, Marks SC, Adams VI, Blackbourne BD. Factors affecting visualization of posterior rib fractures in abused infants. AJR 1988; 150:635-638. 15. Kleinman PK, Marks SC, Blackbourne B. The metaphyseal lesion in abused infants: a radiologic-histopathologic study. AJR 1986; 146:895-905. 16. Kleinman PK, Marks SC, Jr., Richmond JM, Blackbourne BD. Inflicted skeletal injury: a postmortem radiologichistopathologic study in 31 infants. AJR 1995; 165:647-650. 17. Kleinman PK, Marks SC. Vertebral body fractures in child abuse. Radiologic-histopathologic correlates. Invest Radiol 1992; 27:715-722. 18. Kleinman PK, Schlesinger AE. Mechanical factors associated with posterior rib fractures: laboratory and case studies. Pediatr Radiol 1997; 27:87-91. 19. Lyons TJ, Oates RK. Falling out of bed: a relatively benign occurrence. Pediatrics 1993; 92:125-127. 20. Nimityongskul P, Anderson LD. The likelihood of injuries when children fall out of bed. J Pediatr Orthop 1987; 7:184-186. 21. O’Connor JF, Cohen J., in: Kleinman PK Diagnostic Imaging of Child Abuse, 2nd ed. Mosby, 1998 168-177. 22. Spevak MR, Kleinman PK, Belanger PL, Primack C, Richmond JM. Cardiopulmonary resuscitation and rib fractures in infants. A postmortem radiologic-pathologic study. JAMA 1994; 272:617-618. 23. Swischuk LE. Spine and spinal cord trauma in the battered child syndrome. Radiology 1969; 92:733-738. 24. Worlock P, Stower M, Barbor P. Patterns of fractures in accidental and non-accidental injury in children: a comparative study. Br Med J 1986; 293:100-102. 25. Zimmerman RA, Bilaniuk LT, Bruce D, Schut L, Uzzell B, Goldberg HI. Computed tomography of craniocerebral injury in the abused child. Radiology 1979; 130:687-690.

Forensic Radiology of Child Abuse

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Neonatal Brain: Radiologic Evaluation Dorothy I Bulas, MD Learning Objectives • • •

Review the differences in cranial anatomy of the preterm and term infant Review hemorrhagic (IVH) & nonhemorrhagic (PVL) injuries due to partial asphyxia. Understand radiologic work up of hypoxic ischemic injury of the neonate including neurosonographic techniques (transmastoid view, Doppler)

Figure 6-17-1

Sonographic Technique • • •

Anterior fontanelle ➢ Coronal and sagittal planes Axial views ➢ Posterior and mastoid fontanelle Doppler ➢ MCA/ACA resistive index ➢ Color Doppler of fluid collections

Posterior Fontanelle [Figure 6-17-1] Mastoid Fontanelle Scanning • •

Ultrasound image via the posterior fontanelle demonstrates the occipital horn filled with choroid

24/200 patients with PF abnormality Mastoid view: ➢ Improved visualization (96%) ➢ Increased diagnostic confidence (75%) ➢ Only technique to show abnormality (46%)

Figure 6-17-2

Luna & Goldstein, AJR 2000; 174

Transmastoid view -Trapped fourth ventricle [Figure 6-17-2]

Sagittal Sinus Thrombosis [Figure 6-17-3] Resistive Index – MCA or ACA Peak Systole – End Diastole •

• • •

Peak Systole ➢ Minimize affect of angulation ❖ Age dependent values ❖ Term infants 0.7 + 7% ❖ By age 2 0.5+ 15% An increase in diastolic flow results in decrease RI A decrease in diastolic flow results in increase RI As ICP increases above mean arterial pressure, diastolic flow reverses w/ RI > 1.0.

Transmastoid view demonstrates lateral and fourth ventriculomegaly

Figure 6-17-3

Normal Anatomy: Premature Brain •

•

Preterm cerebral vessels penetrate from meninges to periventricular walls ➢ single vessel walls Term cerebral vessels-watershed at cortex ➢ smooth muscle AA, collagen VV

Nelson et al AJNR 1991:215 Longitudinal ultrasound via the anterior fontanelle demonstrates irregular flow of the sagittal sinus consistent with sagittal sinus thrombosis Pediatric Radiology

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Neonatal Brain

Neonatal Hypoxic-Ischemic Injury • • •

Figure 6-17-4

Injuries are the result of partial or global asphyxia due to various causes: intrauterine, sepsis, apneic episodes Partial asphyxia: IVH, PVL Global asphyxia: multifocal ischemic necrosis

Injury patterns: Term infant [Figure 6-17-4] •

•

Prolonged Partial Asphyxia ➢ Watershed infarction (ACA-MCA-PCA; “parasagittal”) - cortex / white matter Profound asphyxia: ➢ Basal ganglia, ➢ Rolandic cortex

Germinal Matrix • • • • •

Origin of neuronal/glial development. Rich arterial supply perforators of ACA, MCA, PCA. Drain to deep venous system. Regression posterior to anterior b/w 24-28 wks Involutes by 32 wks

Axial CT demonstrates diffuse cortical infarction in a term infant with severe hypoxic ischemic injury

Germinal Matrix Hemorrhage • • • •

Most common origin of preterm hemorrhages 20% of preterms <1500 gms Venous origin

Germinal Matrix Hemorrhage • • •

Capillaries and sinusoids in caudothalamic groove with poor stromal support Vessels converge to form draining veins Theory- increased flow vv rupture at convergence points.

Nakamura et al Mod Path 1991;475

Germinal Matrix Hemorrhage: FACTORS • •

Asphyxia-abolishes autoregulationCBF varies with SBP > injury to endothelium of germinal matrix vessels. Hypoxia- decrease myocardial energy reserve > circulatory failure > hypotension > cerebral ischemia > venous hypertension

Funato M, Munksgard 1994:456; Milligan D Lancet 1980:26:896

Germinal Matrix Hemorrhage •

•

O2 delivery reduced w/ hemorrhage. ➢ Switch from aerobic to anaerobic metabolism ➢ Increase lactate formation. Blood ruptures into ventricles ➢ Dilatation, ➢ Disrupt ependymal lining

Figure 6-17-5

Modified Papile Classification • • • •

Grade I Subependymal hemorrhage Grade II Intraventricular blood w/ no/min ventricular dilatation Grade III Intraventricular blood w/ prominent ventricular dilatation Grade IV/PHI Parenchymal hemorrhage associated with IVH

Sagittal US demonstrates a Grade 1 subependymal hemorrhage

Grade I IVH [Figure 6-17-5]

Neonatal Brain

1500 1502

Pediatric Radiology

Grade II IVH w/out dilatation [Figure 6-17-6]

Figure 6-17-6

Grade II IVH w/ distended ventricle [Figure 6-17-7] Grade III - IVH w/ distended ventricle [Figure 6-17-8] Periventricular Hemorrhagic Infarction [Figure 6-17-9] Figure 6-17-9 Coronal and sagittal US images demonstrate Grade II intraventricular hemorrhage with blood extending into the ventricle

Figure 6-17-7

Coronal sonogram demonstrates a heterogeneous region in the right parietal periventricular white matter consistent with a periventricular hemorrhagic infarction and ventriculomegaly

Coronal US and axial CT images of Grade III intraventricular hemorrhage with blood filling and dilated the ventricles

Periventricular Hemorrhagic Infarction (PHI/ Grade IV IVH) • • •

Figure 6-17-8

15% with IVH dev PHI Same side as IVH, after IVH has occurred Usually unilateral or asymmetric

Periventricular Hemorrhagic Infarction (PHI/ Grade IV IVH) •

Hemorrhagic necrosis in PVWM dorsal/lat of lateral ventricle where medullary veins confluent.

Periventricular Hemorrhagic Infarction • •

PET scans demonstrate parenchymal ischemia beyond boundaries of hemorrhage Further injury may be due to accumulation of metabolic toxins due to impaired blood flow

Volpe JJ. Neurology of the Newborn 1987

Periventricular Hemorrhagic Infarction Sonography Time

Pathology

Early

hemorrhagic infarcts PVWM surrounding ischemia Subacute cystic cavities diminished myelin Chronic

porencephalic cyst ventricular dilatation gliosis

Pediatric Radiology

Coronal sonogram demonstrate dilated ventricles filled with hemorrhage consistent with an evolving grade III intraventricular hemorrhage

US findings echogenic areas mass effect retraction of clot, porencephalic cyst ventricular debri/dilat porencephalic cyst ventricular dilatation atrophy 1501 1503

Neonatal Brain

IVH: Sonography • • •

•

Figure 6-17-10

US very useful -lesions well visualized deep in the brain Difficult to make diagnosis clinically Screening recommendations ➢ Preterms <32 wks ➢ Birth wt <1.5 kg ?When- majority of bleeds occur 3-4 days after birth. ➢ First screening US 4-7 days after birth

IVH ? CT/MRI • • •

Difficult transporting unstable preterms CT may miss bleed after several days MRI - Hemosiderin staining can be seen up to 1 yr ➢ May demonstrate additional abnormalies ➢ Focal WM loss ➢ Diffuse hypoxic-ischemic changes

Pulsed Doppler demonstrates no flow during diastole with resistive index of 1

IVH/PVH: Follow up [Figure 6-17-10] •

Doppler useful to differentiate HC /atrophy ➢ RI >.8 sign of increased ICP.

IVH/Periventricular Hemorrhagic Infarction: Outcome • • • • •

Normal US Grade 1 Grade 2 Grade 3 PHI

90% nl neurologic outcome 90% nl neurologic outcome 85% nl neurologic outcome 50-70% nl outcome, 8% mortality 90-100% major motor deficit, 64% dec cognitive function, 60% mortality

IVH: Outcome •

• •

3 mechanisms for poor outcome ➢ 1. Damage from ventricular dilatation-may be reversible if shunted ➢ 2. Shunt related complications: infection, sepsis, obstruction, seizures. ➢ 3. Hypoxic ischemic injury-white matter edema, gliosis, axonal swelling Spastic hemipareses, intellectual deficits. Prognosis parallels size of parenchymal echodensity

Periventricular Leukomalacia: Etiology •

•

• • • •

•

?Arterial ischemia? ➢ Size of zones decrease with gestational age ❖ Older infants w/PVL - strong hx of hypotension ❖ Very premature infant-no hx of hypotension ?Cytokine response? ➢ Maternal chorioamniotitis assoc. w/ PVL ❖ ?fluctuation in cerebral blood flow ❖ ?Inflammatory cytokine response to infection Prevalence in preterms before 25% ➢ Now reported as low as 7%. Yet reports of increasing survival of very low weight preterms with increase rate of CP ?are we accurately identifying PVL sonographically? Size of zones decrease w/ gestational age ➢ Older infants w/PVL - strong hx of hypotension ➢ Very premature infant-no hx of hypotension Resultant injury of PVL > than border zones ➢ ?Intrinsic vulnerability of periventricular glial cells and intrinsic metabolic properties. ➢ O2 reduced >lactic acid accumulation

Neonatal Brain

1502 1504

Pediatric Radiology

•

Glial cells are differentiating to astrocytes & oligodendroglia with active myelination. Intense metabolic activity w/ high O2 demandsvulnerable to hypoxia [Figure 6-17-11]

Figure 6-17-11

Periventricular Leukomalacia:Sonography • • •

Infarction occurs in deep cerebral WM Bilateral symmetric US not sensitive in detecting PVL ➢ Difficult to distinguish from anisotropic effect of periventricular halo ➢ Echodensities resolve and can be missed

Periventricular Leukomalacia:Sonography Time

Pathology

US Findings

Acute 1-3 days

focal necrosis petechial hemor

patchy areas of inc echogenicity PVWM

Subacute 1-2 wks 2-3 wks

diminished myelin cystic cavities

decreasing echogenicity may be normal small cysts “Swiss cheese”

Chronic

cysts disappear gliosis

cysts disappear vent dilatation

Coronal sonogram demonstrates multiple cysts in the periventricular white matter consistent with periventricular leukomalacia

Periventricular Leukomalacia • • •

Less severe PVL-diminished myelin result in dilated ventricles. Severe PVL will cavitate Cysts eventually resolve -gliotic scarring

Figure 6-17-12

Periventricular Leukomalacia:CT [Figures 6-17-12 and 6-17-13]

• • •

Difficult transporting premature infants Due to high water content, difficult differentiating acute PVL from nl preterm brain Chronic: CT useful extent of lesions, atrophy ➢ Irregular lateral ventricles ➢ Prominent sulci ➢ Subcortical gray matter abuts ventricles ➢ Small cysts missed

Figure 6-17-13

Axial CT image demonstrates diffuse periventricular and cortical edema with intraventricular hemorrhage

Axial image demonstrates ventricular dilatation with irregular walls and marked loss of periventricular white matter Pediatric Radiology

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Neonatal Brain

Periventricular Leukomalacia: MRI

Figure 6-17-14

[Figure 6-17-14]

• •

•

Acute/subacute: dec. sig T1, inc. sig T2 in PVWM High water content makes edema difficult to detect on T1, due to delayed myelination, high signal on T2 apparent. Periatrial gliosis; involves the subependymal periatrial white matter (tapedum) Distal corpus callosum (pre-splenium) thinned

Periventricular Leukomalacia:Outcome • •

•

Outcome correlates with cystic cavitation PVWM traversed by fibers of motor cortex results in spastic diplegia ➢ Lower> Upper extremities Infants with larger lesions often delayed

Nonhemorrhagic Infarction • • •

Rare, term infants 90% major handicaps Perinatal asphyxia ➢ Emboli ➢ Congenital Heart disease ➢ Meningitis ➢ Polycythemis

Axial T2w image demonstrates high signal in the periventricular white matter

Profound Asphyxia • •

Energy requirements related to state of myelination. Most metabolically active and mature regions with most advanced Figure 6-17-15 ➢ myelination, ➢ perfusion ➢ glucose uptake are regions that suffer the most damage • Complete arrest - injury determined by ➢ metabolic maturity of brain ➢ myelination ➢ autoregulation ➢ watershed pattern ➢ excitatory neurotransmitter release Coronal US demonstrates diffuse increased ➢ severity and duration of event echogenicity of the cerebral cortex with slit ventricles Profound Asphyxia: Sonography Anterior cerebral artery Resistive Index < .5 • Diffuse hypoxic ischemic encephalopathy often consistent with arterial vasodilatation due to loss superimposed on IVH/PVL of sutoregulation

Profound Asphyxia in Preterms • • • •

Preterm have low O2 demand and immature cardiopulmonary control Neuronal injury can occur in ventral pons, inferior olivary nuclei, subiculum of hippocampus Unlike term infant, less involvement of basal ganglia, thalami, brainstem PVWM injury dominating

Profound Asphyxia in Term Infants •

Involvement of basal ganglia, brainstem ➢ hippocampus ➢ posterior and medial lentiform nuclei ➢ lateral thalami ➢ cortical gyri

Profound asphyxia Term: Sonography [Figure 6-17-15] •

Acute - increased echogenicity ➢ small vents/sulci ➢ Doppler - Loss of autoregulation RI < .6

Neonatal Brain

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Pediatric Radiology

Figure 6-17-16

Profound asphyxia: CT [Figure 6-17-16] • •

Acute-low attenuation cortex, basal ganglia, periventricular white matter, laminar necrosis Chronic-diffuse atrophy prominent extraaxial fluid spaces and sulci

Profound asphyxia: MRI findings in Preterm Infants •

•

PVWM changes dominate ➢ T1,T2 shortening in peritrigonal WM ➢ T1,T2 prolongation in cerebral WM Chronic - atrophy ➢ Relative sparing of cerebral cortex ➢ Hippocampal and brainstem atrophy ➢ Thin corpus collosum

Profound asphyxia: MRI findings in term infants •

Short T, at times short T2 relaxation times ➢ Basal ganglia ➢ Hippocampus ➢ Posterior and medial lentiform nuclei ➢ Lateral thalami ➢ Cortical gyri

Axial CT images demonstrate low attenuation of the cortex, basal ganglia as well as periventricular white matter.in this term infant following profound asphyxia

Figure 6-17-17

Cerebellar Infarction [Figure 6-17-17] • • • • • • •

Cerebellum felt to be less vulnerable to anoxic damage - sparing during hypoxic ischemic episodes May not be rare in preterms Due to echogenicity of CBL, infarction and hemorrhage easily missed US Most common watershed distribution - between the superior cerebellar artery and the PICA Can be lobar /holohemispheric; +/- hemorrhagic Most in early premature (28-32) with hx of hypotension

Mercuri Ped Rad 1997;27:139 Tsuru Acta Neuropath 1995:90;400

US- Posterior Fossa Hemorrhages [Figures 6-17-18 and 6-17-19] • • •

Coronal MRI demonstrates right cerebellar infarction in a child with a history of prematurity

Easy to miss Close evaluation of sagittal view Additional views ➢ transmastoid view ➢ posterior fontanelle

Figure 6-17-19

Figure 6-17-18

Lateral image of the skull demonstrates where the transducer is placed for imaging via the mastoid .B. Axial sonogram demonstrates a heterogenous lesion in the posterior fossa consistent with a posterior fossa hemorrhage

Sagittal midline image of a normal brain clearly demonstrates the vermis (arrow). B. Sagittal midline image demonstrates a heterogeneous region in the posterior fossa with no visualization of the vermis in an infant with a posterior fossa hemorrhage

Pediatric Radiology

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Neonatal Brain

Conclusions • • • •

Neurosonology is an integral part of care in the neonate. Type of hypoxic injuries vary with gestational age US sensitive for IVH screening, less sensitive for identifying PVL, infarcts and posterior fossa hemorrhages Flexibility in technique – Doppler, transmastoid view- important in identifying subtle anomalies

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

Barkovich AJ, Sargent SK: Profound asphyxia in the premature infant: imaging findings AJNR 1995;16;1837. Benson JE et al. Intracranial Neonatal Neurosonography: An Update. Ultrasound Quarterly 2002: 18;89 Boo NY et al. Early cranial US changes as predictors of outcome during first yr of life. J Ped Child Healthy 2000;36:363 Bulas DI, Taylor GA, Fitz CR, Revenis ME, Glass P, Ingram JD. Posterior fossa intracranial hemorrhage in infants treated with extracorporeal membrane oxygenation: Sonographic findings. AJR 1991; 156:571. Bulas DI. Vezina G: Anoxic injury in the Preterm infant Radiologic evaluation Radiologic Clinics of North America, Vol 37, Nov 1999:1147. Bulas DI. TCD: Practical Applications in Pediatrics. Applied Radiology 1999, April 7-15. Chadduck WM, Duong DH Kast JM et al: Pediatric cerebellar hemorrhage. Child Nerv Syst 1995;110:579. Perlman JM, Rollins N: Surveillance protocol for the detection of intracranial abnormalities in premature neonates. Arch Pediatr Adol Med 2000;154:822. Rumack CM et al. Timing and course of neonatal intracranial hemorrhage using US. Radiology 1985:154:101 Rumack C, Drose J. Neonatal and Infant Brain Imaging ed Rumack et al. Diagnostic Ultrasound. Elsevier Mosby 2005 Taylor GA. Recent advances in neonatal cranial ultrasound and Doppler techniques. Clin Perinataol 1997;24:677 Seibert JJ et al. Use of power Doppler in pediatric neurosonography: a pictorial essay. Radiographics 1998;18:879 Volpe JJ: Neurobiology of periventricular leukomalacia in the premature infant Pediatr Res 2001;50:553-562 Vohr B, Allan WC, Scott DT et al: Early onset IVH in preterm neonates: Incidence of neurodevelopmental handicap. Semin Perinatol 1999;23:212.

Neonatal Brain

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Pediatric Radiology

Pediatric Liver Tumors William E. Shiels II, DO • • • • •

Pediatric Liver Tumors: GOALS

• • •

Types and presentations Pathologic features Clinical features Imaging Therapeutic implications/correlation

Pediatric Liver Tumors: ABC’s of Liver Tumors Age of patient Biologic imaging features Chemistry-blood ➢ Alpha-fetoprotein ➢ Endothelial growth factor

• • • • •

Benign Epithelial Tumors Benign Mesenchymal Tumors Malignant Epithelial Tumors Malignant Mesenchymal Tumors Metastases

• • •

Focal Nodular Hyperplasia Hepatocellular Adenoma Hepatic cysts

• • •

Mesenchymal Hamartoma Hemangioendothelioma Hemangioma

• • •

Hepatoblastoma Hepatocellular Carcinoma Fibrolamellar Carcinoma

• • •

Undifferentiated embryonal sarcoma Embyronal Rhabdomyosarcoma Angiosarcoma

• • • • •

Neuroblastoma Burkitt’s Lymphoma Sarcomas Wilms’ tumor Other

Categorization

Benign Epithelial Tumors

Benign Mesenchymal Tumors

Malignant Epithelial Tumors

Malignant Mesenchymal Tumors

Metastases

• •

Mesenchymal Hamartoma Mesenchymal tissue, disorganized bile ducts, hepatocytes, fluid filled spaces Developmental disturbance: ➢ Portal/biliary obstruction, lymphangiomatous tissue

Pediatric Radiology

1509

Pediatric Liver Tumors

• • • • •

Mesenchymal Hamartoma: Pathology [Figures 6-16-1 and 6-16-2]

Figure 6-18-1

Multicystic, variable size: 2–25cm 15–30% pedunculated 80% right lobe, slow growing- fluid accumulation Sharply demarcated, lobulated Mesenchymal stroma, hepatocytes, cysts: ➢ Biliary/lymphangiomatous origin (Portal tract)

Figure 6-18-2

Hepatic mesenchymal hamartoma with smoothly marginated nonaggressive cysts within a fibrous stroma

Figure 6-18-3 Photomicrograph with fibrous stroma and cysts predominantly lined by flat vascular endothelium from the lympatic component of the hamartoma • •

Mesenchymal Hamartoma: Clinical Enlarging abdominal mass Child < 2 yrs old ➢ Can Dx in utero • Otherwise asymptomatic • Negative alpha-fetoprotein

Sonography with smooth walled cysts with thin septations, similar to those seen in lymphatic malformations

Mesenchymal Hamartoma: Imaging • • •

[Figures 6-18-3 to 6-18-6]

• •

Figure 6-18-5

Hepatomegaly, abdominal mass US: Multilocular cysts, hypoechoic Less frequent-solid mass with few cysts Rare to find hemorrhage, Ca +2 MR: Cysts-low T1, high T2 signal

Figure 6-18-4

CT with few macrocysts in the mesenchymal hamartoma

Figure 6-18-6

Sonography demonstrating microcystic component in the hamartoma

Cystic mesenchymal hamartoma with single macrocyst with normal adjacent liver

Pediatric Liver Tumors

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Pediatric Radiology

• • • •

SURGICAL Enucleation, partial hepatectomy Incision/drainage of cysts Marsupialize large cyst(s)

• • •

Age: Young (less than 3y/o) Biologic imaging: Cystic Chemistry: Normal alpha-fetoprotein

• • • • • •

Most common hepatic tumor- first year of life Usually Dx in first few months of life Female predominance 1.5:1 Growing mass, clinically symptomatic Vascular, high flow, mass-stroma History of involution in first year, if survivor

Mesenchymal Hamartoma: Treatment

Figure 6-18-7

Mesenchymal Hamartoma

Infantile Hemangioendothelioma Large vascular spaces in hemangioendothelioma with normal adjacent liver

Figure 6-18-8

Infantile Hemangioendothelioma: Pathology • • • • •

[Figures 6-18-7 and 6-18-8]

• • • • • •

Solitary, may be multicentric 0.2–15 cm diameter May be well demarcated, no capsule Dilated vascular spaces- anastomosing,+ stroma Central necrosis/fibrosis, hemorrhage, Ca2+ ➢ Evidence of regression

Infantile Hemangioendothelioma: Clinical Infant, usually less than 2 months Abdominal mass, CHF, Kasabach/Merritt, DIC Occasionally asymptomatic hepatomegaly Massive hemoperitoneum Multiple cutaneous angiomas-40% Normal alpha-fetoprotein (positive EGF)

Infantile Hemangioendothelioma: Imaging

Top micrograph with small “tight” vascular spaces in a hemamgioendothelioma from a child with no heart failure. Bottom specimen from a child with high output failure from left-to-right shunting in the hemangioendothelioma; note the large vascular spaces associated with arteriovenous anastomoses

• •

[Figures 6-18-9 to 6-18-15]

Hepatomegaly, abdominal mass US: Heterogeneous, speckled Ca2+ ➢ High flow, venous/arterial: A-V shunts ➢ Aortic caliber decrease-after celiac artery • CT: Hypodense, 40% Ca2+, periph. enhancement • MR: Low T1, High T2 signal, +/– hemorrhage • Angio: Vascular, A-V shunting, large celiac A.

Figure 6-18-9

Flow-failure chest radiographic image from left-to-right shunt at the level of the liver in patient with hepatic hemangioendothelioma

Pediatric Radiology

Figure 6-18-10

Large vascular spaces in hemangioendothelioma prior to color flow and duplex Doppler interrogation

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Pediatric Liver Tumors

Figure 6-18-11

Figure 6-18-12

Decrease in abdominal aortic caliber from left-to-right shunting of blood at the level of the celiac artery in patient with hemanioendothelioma

Figure 6-18-13 Diffuse hepatic involvement of hemangioendothelioma

Figure 6-18-14 Multiple discrete liver hemangiomas in patient who is clinically asymptomatic

Figure 6-18-15

Draping peripheral feeding vessels in liver hemangioma

Dynamic CT with centripetal contrast enhancement of liver multiple hemangiomas Pediatric Liver Tumors

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Pediatric Radiology

• •

Infantile Hemangioendothelioma: Treatment

Figure 6-18-16

Spontaneous involution in 12–18 months CHF- embolotherapy with interferon ➢ Interferon-alpha 1, 2a, 2b response in weeks • Embolize hepatic artery- coils, balloons PRN ➢ A-V shunts ➢ Particles (large) where possible • May couple with surgery PRN • •

Hemangioendothelioma Age: Young (Infants) Biologic imaging: ➢ Vascular-High flow state ➢ Solid stroma • Chemistry: Normal alpha-fetoprotein ➢ Positive endothelial growth factor

Photomicrograph of hepatoblastoma demonstrating cords and nests of malignant cells

Figure 6-18-17

• •

Hepatoblastoma Most common primary liver tumor in childhood Third most common abdominal malignancy ➢ After neuroblastoma, Wilms’ tumor • May be familial • Associations: Trisomy 18, Beckwith-Wiedemann, hemihypertropy, familial polyposis, exposure- metals, petroleum products, paints, oral contraceptives, Fetal Alcohol Syndrome • • • • • •

Hepatoblastoma: Pathology- Gross Solitary 80%, right lobe predominance May be lobulated 5–20 cm Nodular with fibrous bands throughout Fleshy; +/– hemorrhage, necrosis, Ca2+ Rare diffuse infiltration, adjacent liver-normal

Slide left with right hepatic lobe nodular solid hepatoblastoma in an otherwise normal liver. Slide right with same tumor demonstrating the typical nodular appearance in the gross specimen

•

Hepatoblastoma: Pathology-Histologic [Figure 6-18-16]

Figure 6-18-18

Epithelial ➢ Fetal 30%, Pink-cytoplasm, sparse mitoses ➢ Embryonal 20%- blastemic appearance (blue-H&E) ➢ Macrotrabecular- cords of tumor cells- 3% ➢ Small cell- 3% • Mixed epithelial-mesenchymal- 45%; Teratoid ➢ osteoid differentiation, also muscle, cartilage • AFP stain positive • • • • • •

Hepatoblastoma: Clinical Abdominal mass, hepatomegaly Anemia Child 90% < 5 yrs old, 65–70% under 2 yrs Males> females 2:1 Positive alpha-fetoprotein No prior history of liver disease

Hepatoblastoma: Imaging • • • • •

[Figures 6-18-17 to 6-18-19]

Hepatomegaly, abdominal mass, 10–15% Ca2+ US: Heterogeneous, predominantly solid CT: Heterogeneous, lobulated MR: Low T1, High T2 Angio: Tumor neovascularity

Pediatric Radiology

Unenhanced CT with focus of calcification in a teratoid variety of hepatoblastoma. Heterogeneous enhancement of same tumor involving left and right hepatic lobes

1511 1513

Pediatric Liver Tumors

• •

Hepatoblastoma: Treatment [Figure 6-18-20]

Figure 6-18-19

Pre-op chemotherapy ( 50%…90%) Surgical: Resectable 90%; 35% mortality ➢ Fetal best prognosis • Vascular definition- multisegmentectomy • Mets: Pulmonary, periaortic nodes, brain • Chemoembolization/RF after surgery PRN

Hepatoblastoma • • •

Heptoblastoma with solid nature in CT and gross specimen. Cystic change is irregular due to central necrosis of the solid tumor

Age: Young (infants, young children) Biologic imaging features: Solid Chemistry: Positive alpha-fetoprotein

Figure 6-18-20

• •

Hepatocellular Carcinoma #2 primary liver malignancy in childhood Often underlying liver disease/cirrhosis ➢ Tyrosinemia, alpha-1-antitrypsin deficiency, glycogen storage dz, biliary atresia, chronic hepatitis (HBsAg +), Fanconi anemia, methotrexate induced hepatic fibrosis • May be primary • • • • • • •

Multinodular, diffuse; less common solitary 2–25 cm Hemorrhage, cysts scattered with nodules Fibrous/cirrhotic background Adjacent liver abnormal Cords and nest- malignant hepatocytes Fibrolamellar variant: favorable prognosis

• • • • • •

Abdominal mass, hepatomegaly Abdominal pain Child > 4 yrs old, 12–14 yrs mean age Male predominance > 2:1 Positive alpha-fetoprotein + prior history of liver disease

Hepatocellular Carcinoma: Pathology [Figure 6-18-21]

Chemoembolization of residual tumor focus following surgery in patient who is not a liver transplantation candidate

Hepatocellular Carcinoma: Clinical

Figure 6-18-21

Hepatocellular Carcinoma: Imaging • • •

[Figures 6-18-22 and 6-18-23]

Hepatomegaly, abdominal mass US: Heterogeneous, mostly solid, hypoechoic CT: Hypodense/isodense ➢ Heterogeneous enhancement • MR: Low T1, High T2 (esp Fat Sat FSE), ➢ PV invasion • Similar appearance to aggressive hepatoblastoma

Nodular nature(cords and nests of tumor cells) of solid hepatocellular carcinoma

Figure 6-18-23

Figure 6-18-22

Nodular appearance of solid epithelial tumor (hepatocellular carcinoma) in 14 year old patient, with positive AFP

Pediatric Liver Tumors

1512 1514

Nests of inhomogeneously enhancing HCC Pediatric Radiology

• •

Hepatocellular Carcinoma: Treatment [Figures 6-18-24 to 6-18-26]

Figure 6-18-24

Pre-op chemotherapy Surgical: Resectable 20%, >75% mortality ➢ Fibrolamellar- best prognosis • Vascular definition- multisegmentectomy • Chemoembolization/RF after surgery PRN • • •

Hepatocellular Carcinoma

• • • • • •

Age of patient: Teens Biologic imaging: Solid, multifocal Chemistry-blood ➢ Positive alpha-fetoprotein

Undifferentiated Embryonal Sarcoma

RF ablation of focal hepatoma (left image) with needle seen during US guided RF ablation (right image).

#4 liver malignancy in childhood AKA malignant mesenchymoma Highly aggressive neoplasm Most children 6–10 yrs old, M=F Abdominal mass, fever, wt loss Normal alpha-fetoprotein

Figure 6-18-25

Undifferentiated Embryonal Sarcoma: Pathology • • • • • • •

Solid, globular, areas of necrosis/hemorrhage 2–30 cm Right lobe dominance (75%) Fibrous pseudocapsule, occasional-pedunculated Spindle/stellate shaped sarcomatous cells Myxoid background Local recurrence and metastasis Left slide with small echogenic focus of microbubbles form at the tip of needle (arrow) in the earliest phase of coagulation necrosis. The small focus of microbubbles grows to become a large area of echogenic necrotic tumor (right)

Undifferentiated Embryonal Sarcoma: Imaging [Figures 6-18-27 to 6-18-30] • • • • •

Hepatomegaly, abdominal mass US: Complex mass CT: Hypodense-heterogeneous enhancement MR: Low T1, High T2 Angiography: Hypovascular

Figure 6-18-26

Figure 6-18-27

Figure 6-18-28

Focal scar 1 year following RF tumor ablation

Complex US appearance of embryonal sarcoma in 11 year female

Demonstrable pseudocapsule anteriorly surrounding mesenchymal sarcoma Pediatric Radiology

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Pediatric Liver Tumors

Figure 6-18-29

Figure 6-18-30

MR with heterogeneous cystic and solid foci in embryonal sarcoma Multiple areas of cystic change in embryonal sarcoma in 10 y/o male • • • • •

Undifferentiated Embryonal Sarcoma: Treatment

• • •

Surgical: Resection Chemotherapy, XRT Poor prognosis Mean survival = 12 months Recurrence Dx 12–16 months after surgery

Figure 6-18-31

Undifferentiated Embryonal Sarcoma

• • • • • •

Age of patient: Adolescents/teens Biologic imaging: Mixed solid/cystic Chemistry-blood ➢ Negative alpha-fetoprotein

Liver Metastases [Figures 6-18-31 and 6-18-32]

• • •

Neuroblastoma- Stage 4 and 4s Burkitt’s lymphoma Wilms’ tumor Leukemia (AML) Sarcomas Other malignancies

Studded appearance of stage 4S neuroblastoma diffusely involving the liver

ABC’s of Pediatric Liver Tumors

Figure 6-18-32

Age of patient Biologic imaging features Chemistry-blood ➢ Alpha fetoprotein ➢ Endothelial growth factor

Pediatric Liver Tumors: Summary Summary chart of classic features of common pediatric liver tumors Tumor

Age

Characteristics

Inf. Hemangio

< 1 yr

Mes Hamartoma

< 2 yr

Hepatoblastoma HCC Emb Rhabdo UES Metastases

< 3 yr > 4 yr < 5 yr > 6 yr any

Solid, Ca+2, vasc, AFP (-), involutes with interferon Cystic > solid, AFP (-) Ca+2, solid, vasc, AFP(+) solitary

Pediatric Liver Tumors

Solid, vasc, AFP (+), multifocal Solid > cystic, mild vasc, AFP(-) Cystic > solid, AFP (-) Solid or cystic

1514 1516

Typical appearance of Burkitt Lymphoma involving both the liver and the posterior gastric wall in patient with fatty liver

Pediatric Radiology

References 1. 2. 3. 4.

Von Schweinitz, D. Management of liver tumors in childhood. Semin Ped Surg 2006; 15(1):17-24 Stocker JT. Hepatic tumors in children. Clin Liver Dis. 2001 Feb;5(1):259-81, viii-ix. von Schweinitz D. Neonatal liver tumours. Semin Neonatol. 2003 Oct;8(5):403-10. Tiao GM, Bobey N, Allen S, Nieves N, Alonso M, Bucuvalas J, Wells R, Ryckman F. The current management of hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation. J Pediatr. 2005 Feb;146(2):204-11. 5. Burrows PE, Dubois J, Kassarjian A. Pediatric hepatic vascular anomalies. Pediatr Radiol. 2001 Aug;31(8):533-45. 6. Dubois J, Hershon L, Carmant L, Belanger S, Leclerc JM, David M. Toxicity profile of interferon alfa-2b in children: A prospective evaluation. J Pediatr. 1999 Dec;135(6):782-5. 7. Dachman AH, Pakter RL, Ros PR, Fishman EK, Goodman ZD, Lichtenstein JE. Hepatoblastoma: radiologic-pathologic correlation in 50 cases. Radiology. 1987 Jul;164(1):15-9. 8. Gerber DA, Arcement C, Carr B, Towbin R, Mazariegos G, Reyes J. Use of intrahepatic chemotherapy to treat advanced pediatric hepatic malignancies. J Pediatr Gastroenterol Nutr. 2000 Feb;30(2):137-44. 9. Sun XY, Wu ZD, Liao XF, Yuan JY. Tumor angiogenesis and its clinical significance in pediatric malignant liver tumor. World J Gastroenterol. 2005 Feb 7;11(5):741-3. 10. Rhim H, Dodd GD 3rd, Chintapalli KN, Wood BJ, Dupuy DE, Hvizda JL, Sewell PE, Goldberg SN. Radiofrequency thermal ablation of abdominal tumors: lessons learned from complications. Radiographics. 2004 Jan-Feb;24(1):4152. 11. Iannitti DA, Dupuy DE, Mayo-Smith WW, Murphy B. Hepatic radiofrequency ablation. Arch Surg. 2002 Apr;137(4):422-6.

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Pediatric Liver Tumors

Pediatric Hip Sonography: Practical Radiologic Pathology William E. Shiels II, DO Figure 6-19-1

• •

Ultrasound of the Hip Developmental dysplasia The irritable hip ➢ Septic arthritis, toxic synovitis, ➢ Arthritis, LCP, hemophilia • US guided intervention • Practical points • • •

DDH - Risk Factors Family History: 12-36% Breech: up to 23% (esp female) Torticollis, metatarsus adductus, oligohydramnios

• •

DDH - Ultrasound Technique High frequency linear Static views (Graf) ➢ Coronal • Dynamic views (Harcke) ➢ Transverse and coronal ➢ Barlow maneuver • Standard minimum exam • • •

Coronal left hip sonography: Transducer in right hand, left hand pistons the left leg/hip

Figure 6-19-2

Dynamic Standard Minimum Exam [Figures 6-19-1 and 6-19-2] Static coronal image Static transverse image Transverse image with stress

Coronal Image [Figures 6-19-3 and 6-19-4] Transverse right hip sonography: Transducer in left hand, right hand pistons the right leg/hip

Figure 6-19-3

Figure 6-19-4

Coronal hip anatomy illustrates hyaline cartilage of the acetabular roof, femoral head, and triradiate cartilage; fibrocartilaginous acetabular labral tip Essential static coronal hip sonogram with Graf alpha angle. Note the critical horizontal orientation of the iliac body for accurate depiction of normal and pathologic anatomy

Pediatric Hip Sonography

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Pediatric Radiology

Static Transverse Image [Figure 6-19-5]

Figure 6-19-5

•

DDH - Coronal Rigidly standardized protocol ➢ Exam technique, interpretation ➢ Alpha, beta angles • Reproducible • Large European experience • • •

Normal - Graf I Physiologically Immature - Graf IIa Dysplasia - Graf IIb and above

• • • • •

Toxic synovitis Septic arthritis Arthritis - JRA, post-infectious Legg-Calve-Perthes Hemophilia

• • • •

Pain Limitation of motion Fever, WBC, ESR, CRP 50% with effusion

• • •

Direct visualization More sensitive than plain film Guided aspiration

DDH - Practical Grading

The Irritable Hip

Note femoral metaphysis in view in the transverse image obtained with the hip flexed. Femoral head deeply seated in the hip socket

The Irritable Hip

Hip Effusion - Ultrasound

Pediatric Radiology

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Pediatric Hip Sonography

• • • •

High frequency linear Supine, hip neutral Anterior, parallel femoral neck Comparison views

• • • •

Fluid distends joint capsule >2mm difference abnormal Fluid echogenicity unreliable Doppler flow unreliable

• • • • •

Fever Elevated WBC, ESR, CRP Symptoms more severe Staph, strep, H. flu Must obtain fluid for Dx

Hip Effusion - Ultrasound

Figure 6-19-6

Hip Effusion - Ultrasound

Septic Arthritis [Figures 6-19-6 and 6-19-7]

Essential Principles of US Guidance

Cursors delineate the effusion in the anterior hip joint space [Figure 6-19-8]

Figure 6-19-7

Figure 6-19-8

Small hip effusion in left image; right image with needle aspiration of the effusion

Critical principles of sonographic guidance; needle aligned in the center of the sound beam, straight longitudinal alignment of needle along the transducer face/sound beam short axis

References 1. Harcke HT, Grissom LE. Pediatric hip sonography. Diagnosis and differential diagnosis. Radiol Clin North Am. 1999 Jul;37(4):787-96. 2. Grissom LE, Harke HT. Developmental Dysplasia of the Pediatric Hip with Emphasis on Sonographic Evaluation. Semin Musculoskelet Radiol. 1999;3(4):359-370 3. ACR Practice Guideline for the Performance of the Ultrasound Examination for Detection of Developmental Dysplasia of the Hip. American College of Radiology Practice Standards/Guidelines 2004. American College of Radiology, Reston, VA.Headquarters Office: 1891 Preston White Dr, Reston, VA 20191, (703) 648-8900© 2004 American College of2004 American College of Radiology 4. Eich GF, Superti-Furga A, Umbricht FS, Willi UV. The painful hip: evaluation of criteria for clinical decisionmaking. Eur J Pediatr. 1999 Nov;158(11):923-8. 5. Buchmann RF, Jaramillo D. Imaging of articular disorders in children. Radiol Clin North Am. 2004 Jan;42(1):15168, vii. 6. Givon U, Liberman B, Schindler A, Blankstein A, Ganel A. Treatment of septic arthritis of the hip joint by repeated ultrasound-guided aspirations. J Pediatr Orthop. 2004 May-Jun;24(3):266-70.

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Pediatric Radiology

Pediatric Seminar 1: Pulmonary Infections Ellen Chung, LTC, MC CASE 1: 5 month with fever and cough

Normal thymus mimicking RUL pneumonia

Follow-up

Follow-up radiograph in one month shows expected decrease in size of the thymus and the thymic sail sign

Normal Thymus • • • • • •

Sail sign, wave sign, notch or sulcus No mass effect on trachea or vessels Homogeneous in density Commonly seen on CXR up to age 5 years then regresses in latter half of first decade Thymic rebound Masses can arise in thymus – leukemia/lymphoma, teratoma, thymolipoma, lymphatic malformation

Pediatric Radiology

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Pediatric Seminar 1: Pulmonary Infections

CASE 2: 12 yo with fever and cough Round Pneumonia • • • •

Unique to children – usually < 8 yo Usually posterior lower lobes Almost always bacterial – pneumococcus Exclude bone erosion – ddx: neoplasm

CT reveals a complex mass in the right posterior mediastinum with calcifications and extension through the chest wall and into the spinal canal. This is neuroblastoma

Tumors that look like pneumonia • • •

Neuroblastoma Chest wall masses Parenchymal mass ➢ Pleuropulmonary blastoma ➢ Plasma cell granuloma ➢ Cyst

In the differential of round pneumonia is mediastinal or chest wall mass. PA and lateral chest radiographs show a round mass in the region of the medial right lower lobe. Note the splaying of the ribs on the right adjacent to the mass. This is therefore a mass and not a pneumonia

Pulmonary Blastoma • • • • •

Arises from primitive mesenchymal blastema Histologically reminiscent of Wilms tumor Unlike other embryonal tumors, it is more commonly found in adults May present as solitary nodule or huge mass Heterogeneous, cystic areas

Plasma Cell Granuloma • • • • • • •

AKA inflammatory pseudotumor Localized proliferation of a variety of cells, mostly plasma cells Reactive, organizing pneumonia Patient often asx Chunky calcifications May be cystic May have spiculated margins or air bronchograms

Pediatric Seminar 1: Pulmonary Infections

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Pediatric Radiology

CASE 3: 17 mo with fever and cough without improvement after 1 week of oral abx

PA and lateral chest radiographs show dense opacification of the right lower lobe with silhouetting of the right hemidiaphram. Right lateral pleural thickening is also seen

Complicated Pneumonia • • • • •

Suspect in cases of near white out H. flu less than 2 y.o. Pneumococcus S. aureus CT vs. US ➢ Ultrasound may be better determining which fluid collections need drainage

Ultrasound of the right chest shows pleural fluid with septations and consolidated, airless lung that transmits sound. The hyperechoic foci with ringdown artifact represent round air collections

Multiple axial CT images show the pleural fluid adjacent to the right lower lobe consolidation. Note that the lung enhances and is not necrotic. The fluid-filled cavities with non-nondependent air collections are therefore most consistent with small abscesses rather than necrotic cavities

CASE 4: 17 yo with fever and cough, seizure disorder and DM

Round cavity in superior segment of right upper lobe. Clinical history suggests mild immunocompromise and possibility of aspiration

Pediatric Radiology

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Pediatric Seminar 1: Pulmonary Infections

Differential Diagnosis • • •

Pulmonary abscess Cavitary pulmonary necrosis Pneumatocele

Pulmonary Abscess • •

CT – fluid or air-filled cyst with enhancing, thick, irregular wall Both necrosis and small abscesses in children have good outcomes with antibiotics only

Pneumatoceles • • • • •

Thin walled cavity seen in the recovery phase usually of infection Staphylococcus, pneumococcus, tuberculosis Blunt chest trauma, hydrocarbon pneumonitis, Langerhans cell histiocytosis Bronchial obstruction leading to air trapping and alveolar rupture Pulmonary necrosis. Contrast this appearance to Pneumothorax or mediastinum case #3 with empyema and abscesses. Note decreased enhancement of surrounding parenchyma compared to more anterior parenchyma with preservation of enhancement of the visceral pleura (arrow)

CASE 5: Adult with history of pulmonary infection as a child

Left PA chest radiograph shows asymmetric density of lungs and smaller left PA compared to right. Right expiratory PA chest radiograph shows air trapping in left lung

Swyer-James Syndrome •

• •

Bronchiolitis obliterans ➢ Idiopathic, viral, toxic inhalation,drug reaction, collagen vascular dz, transplant, chronic aspiration. ➢ Adenovirus as child – unilateral hyperlucent lung Small hyperlucent lung, hypoplastic ipsilateral artery Reticular nodular pattern with hyperinflation, central bronchiectasis

Pediatric Seminar 1: Pulmonary Infections

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Pediatric Radiology

CASE 6: Adult with right chest pain

A round mass is seen in the right phrenicovertebral angle posterior to the right atrium

Differential Diagnosis • • • •

Bronchopulmonary foregut malformation CPAM Neurogenic tumor Sequestration

Sequestration •

• • • •

Area of pulmonary tissue that does not have a normal connection to the bronchial tree Systemic arterial supply Can present as an infant or young child or as a young adult Often a history of recurrent infections in the same lobe May contain air

Sequestration • •

LLL most common Multiloculated cystic or solid mass • Intralobar vs. extralobar CT reveals a predominantly cystic mass with focal nodular thickening ➢ Pleural investment along the posterior wall in the right posterior mediastinum. The lower ➢ Extalobar – own pleural right image reveals an additional simple cyst in the left superior investment mediastinum ➢ Extralobar – systemic venous drainage ➢ Extralobar – infants, associated anomalies ➢ Extralobar-congenital/intralobar-acquired • CT vs. MRI vs. US – define vascular supply and drainage

Arteriogram shows the blood supply to the right lower lobe sequestration is coming from the celiac axis below the diaphragm. Abdominal blood supply frequent, and alters the surgical approach. The superior mediastinal lesion was a foregut duplication cyst

Pediatric Radiology

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Pediatric Seminar 1: Pulmonary Infections

CASE 7: 9 yo with SOB

PA and lateral chest radiographs show bilateral lower lobe consolidation with silhouetting of both hemidiaphragms. Also note the large cardiac silhouette, absence of the splenic shadow, and cholecystectomy clips (unusual in children)

Acute Chest Syndrome • • • •

Fever, chest pain, shortness of breath Rib infarction with splinting and atelectasis versus infection Treated with oxygen, antibiotics and pain medication +/- plasma exchange Look for associated findings of SS ➢ CM ➢ Absent splenic shadow ➢ Evidence of gallbladder disease ➢ Skeletal findings

CASE 8: 5 yo with fever and SOB Pediatric TB – Clinical Findings • • • • • •

Asymptomatic Cough Fever Malaise / FTT Respiratory distress Lethargy

Pediatric TB – Radiologic Findings • • • • • •

Normal Focal infiltrate – Ghon lesion Unilateral hilar adenopathy – Ranke complex Paratracheal adenopathy Subcarinal adenopathy Calcified granulomatous nodes

Pediatric Seminar 1: Pulmonary Infections

Left image shows unilateral hilar and right paratracheal adenopathy. Right image also shows secondary RML atelectasis

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Pediatric Radiology

Pediatric Seminar 2: Skeletal Dysplasia Ellen Chung, LTC, MC Radiologic Approach •

•

Assess Proportion ➢ Rhizo-, meso-, or acromelia ➢ +/- platyspondyly Assess Components of Bone ➢ Epiphyses small or irregular ◗ epiphyseal dysplasia ➢ Metaphyses widened, flared, or irregular ◗ metaphyseal dysplasia ➢ Diaphyses widened or thickened ◗ diaphyseal dysplasia

Achondroplasia Group •

All have abnormalities of the same chromosomal locus and gene product, fibroblast growth factor receptor 3 (FGFR3) ➢ Thanatophoric dysplasia ➢ Achondroplasia ➢ Hypochondroplasia

Thanatophoric Dysplasia • • • • •

AD Probably the most common lethal bone dysplasia Skull - kleeblatschadel in type II Thorax - very short ribs and handlebar clavicles Spine – small flat vertebral bodies with round anterior ends, U or H-shaped on AP

Thanatophoric Dysplasia •

• •

Pelvis ➢ Small, flared iliac bones ➢ Very narrow sacrosciatic notches, flat dysplastic acetabula Extremities – telephone receiver Femora

Thanatophoric dysplasia. Note small flat vertebral bodies, very short ribs, and “telephone receiver” femora

Achondroplasia • • •

Most common nonlethal skeletal dysplasia AD, spontaneous mutation rate 8-% Skull ➢ Large with midface hypoplasia ➢ Small skull base and foramen magnum

Achondroplasia •

•

Spine ➢ Very short pedicles – risk of spinal canal stenosis ➢ Decrease in interpediculate distance – lumbar spine Pelvis ➢ Elephant-ear iliac wings ➢ Flat acetabular roofs ➢ Narrow sacrosciatic notches

Achondroplasia •

Extremities ➢ Rhizo- > meso- and acromelia ➢ Hands – brachydactyly with metaphyseal cupping of MC’s ➢ Knees – chevron and inverted chevron deformities ➢ Hips proximal femoral fade out and hemispheric capital femoral epiphyses

Pediatric Radiology

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Pediatric Seminar 2: Skeletal Dysplasia

Case 1 – 5 yo with short stature

Achondroplasia. AP view of the extremities show short widened bones with flared metaphyses and chevron deformities of the distal femora and proximal tibiae

Achondroplasia. AP view of the pelvis shows flared “elephant ear” iliac wings, shallow acetabular roof, narrowed sacrosciatic notches conferring a champagne-glass configuration to the pelvis, and narrowing of the interpediculate distance in the lower lumbar spine

Short Rib-Polydactyly Group •

•

Includes ➢ SRP I-IV - some with, some without polydactyly ➢ Asphyxiating thoracic dysplasia ➢ Chondroectodermal dysplasia Shortest ribs of all dysplasias

Short Rib-Polydactyly • • •

Thorax – shortest ribs, horizontal ribs Pelvis – small ilia, notched acetabula Extremities ➢ Micromelia ➢ Rolling pin-shaped or round-ended or spiked femora ➢ Ovoid tibiae ➢ Polydactyly in some types

Asphyxiating Thoracic Dysplasia (Jeune Syndrome) •

•

Mixed prognosis ➢ Some succumb early from respiratory compromise ➢ Others die later from progressive nephropathy Thorax ➢ Long and barrel-shaped ➢ Handlebar clavicles ➢ Short horizontal ribs with flared ant ends

Achondroplasia. AP views of the hands show brachydactyly with cupping of the metacarpal metaphyses

Asphyxiating Thoracic Dysplasia (Jeune Syndrome) • •

•

Spine – normal Pelvis ➢ Trident acetabular roof ➢ Flared iliac wings ➢ Narrowed SS notches Extremities – cone-shaped epiphyses in hands

Pediatric Seminar 2: Skeletal Dysplasia

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Pediatric Radiology

Case 2 – Newborn with severe respiratory distress

Asphyxiating thoracic dystrophy. Gross photo shows bilateral polydactyly of the hands and very small chest

Asphyxiating thoracic dystrophy

Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) •

• •

Nonskeletal findings important in diagnosis ➢ Hair, nail and teeth abnormalities ➢ Congenital heart disease Thorax – small with short ribs Pelvis ➢ Trident acetabula ➢ Small, flared iliac wings ➢ Narrowed SS notches

Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) •

Extremities ➢ Generalized shortening ➢ Exostosis of proximal medial tibia ➢ Post-axial polydactyly ➢ Capitate-hamate fusion ➢ Extra carpal bone ➢ Cone-shaped epiphyses

Case 3 – 6 year old with history of congenital heart disease

Chondroectodermal dysplasia. AP views of the hands show bilateral postaxial polydactyly and cone shaped epiphyses (arrows)

Pediatric Radiology

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Pediatric Seminar 2: Skeletal Dysplasia

Chondrodysplasia Punctata Group • •

•

All have epiphyseal stippling Rhizomelic ➢ AR, death in first year ➢ Spine – coronal clefts ➢ Symmetric bilateral shortening of femora Conradi-Hunermann ➢ X-linked dominant ➢ Asymmetric shortening of limbs ➢ Diffuse stippling of the spine

Case 4 – newborn boy with respiratory distress

Chondroectodermal dysplasia. AP view of pelvis and LE’s shows short long bones and short, flared iliac wings, with trident-shaped acetabular roofs

Chondrodysplasia punctata. Note diffuse stippled epiphyses and coronal clefts in the thoracic spine

Metaphyseal Chondrodysplasia Group • •

All have normal spine and wide irregular metaphyses Jansen-type ➢ Most severe ➢ Infantile presentation ➢ AD ➢ Extremities – extensive irregular, expanded metaphyses ➢ Hyperparathryroidism

Metaphyseal Chondrodysplasias • •

Schmid-type – mildest, metaphyseal flaring, especially around knees Shwachman-Diamond – AR ➢ Pancreatic insufficiency – malabsorption and lipomatosis of pancreas ➢ Cyclic neutropenia – recurrent infections

Metaphyseal Chondrodysplasias •

McKusick-type ➢ Cartilage-hair hypoplasia ➢ High frequency in the Amish and Finnish populations ➢ Hirschprung disease ➢ Immune deficiency and increased risk of malignancy, especially leukemia and lymphoma

Pediatric Seminar 2: Skeletal Dysplasia

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Pediatric Radiology

Case 5 – 12 yo boy with short stature and unusual hair McKusick-Type • • •

Spine – square vertebral bodies Extremities – flaring, cupping and fragmentation of metaphyses, especially at the knees Hands – shortening with metacarpal and phalangeal cupping and coning

Dysplasias with Prominent Membranous Bone Involvement •

Cleidocranial dysplasia ➢ AD, marked variability in expression Metaphyseal chondrodysplasia, McKusick type. ➢ Drooping narrow chest, hypermobile shoulders, Note widening and irregularity of metaphyses and dental anomalies about the knees ➢ Mild short stature ➢ Skull – wormian bones and wide, open anterior fontanelle

Dysplasias with Prominent Membranous Bone Involvement •

Cleidocranial dysplasia ➢ Thorax – hypoplasia or absence of clavicles, downward sloping ribs ➢ Spine – posterior wedging of vertebral bodies ➢ Pelvis - high, narrow iliac wings, absence or hypoplasia of pubic bones ➢ Extremities – tapered distal phalanges

Case 6 – Fretful 8 mo whose pediatrician thinks he has bilateral clavicular fractures

Cleidocranial dysplasia with wormian bones

Cleidocranial dysplasia. With hypoplastic clavicles with pseudarthroses

Cleidocranial dysplasia. Note absence of ossified pubic bones, narrowed sacrosciatic notches and narrow ilia

Pediatric Radiology

Cleidocranial dysplasia. Tapered distal phalanges

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Pediatric Seminar 2: Skeletal Dysplasia

Dysostosis Multiplex Group • • •

Mucopolysaccharidoses and mucolipidoses All AR All produce similar radiographic complex of findings

Hurler Syndrome • • •

•

Present in infancy or early childhood Skull – J-shaped sella Thorax ➢ Short thick clavicles ➢ Oar-shaped ribs Spine ➢ Gibbus deformity ➢ Inferior beaked T-L vertebral bodies

Hurler Syndrome • •

Pelvis – small flared iliac wings with inferior tapering and steep acetabular roofs Extremities ➢ Wide diaphyses of long bones and metacarpals ➢ Pointed proximal metacarpal poles

Case 7 – short 3 yo with unusual facial appearance

Dysostosis multiplex due to Hurler syndrome. AP chest shows thick clavicles and paddleshaped ribs. Lateral spine shows gibbus deformity at thoracolumbar junction and inferior beaking of vertebral bodies Hurler syndrome. AP pelvis shows flared iliac wings with inferior tapering and steep acetabular roofs

Hurler syndrome. Another patient with a J-shaped sella. Dental abnormalities are related to enlargement of the tongue Another patient with Hurler syndrome showing the pointed proximal poles of the metacarpals

Pediatric Seminar 2: Skeletal Dysplasia

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Pediatric Radiology

Morquio Syndrome • • • •

No J-shaped sella Vertebral beak is in the middle Ribs are widened but not oar-shaped Proximal metacarpal poles are rounded

Dysplasias with Decreased Density • •

Very large group of conditions that share an abnormality of type I collagen Osteogenesis Imperfecta Type II ➢ Invariably lethal Hurler syndrome. Second patient with coarsening of the soft tissues of the ➢ Skull – poor or absent ossification face including the tongue ➢ Thorax – small chest with beaded ribs

Osteogenesis Imperfecta •

•

Type II ➢ Spine – very poor ossification with collapse of vertebral bodies ➢ Extremities – accordion femora Other types ➢ Skull – more than 8-10 wormian bones, variable ossification ➢ Extremities – variable osteoporosis and fractures

Case 8 – 31-week fetus

Osteogenesis imperfecta. Prenatal ultrasound shows no shadowing by the skull. The near side of the brain is much too well visualized. Also the transducer is indenting the skull

Increased Bone Density •

Osteopetrosis (Albers-Schonberg Disease) ➢ Failure to resorb primary spongiosa ➢ Severe precocious type – AR ➢ Delayed type – AD ➢ Reduced bone marrow space ◗ anemia and extramedullary hematopoesis

Osteopetrosis – Radiographic Findings • • • •

Generalized increased bone density Skull – thick and dense especially at the base with foraminal narrowing Spine – “sandwich” or “picture-frame” vertebral bodies Extremities ➢ Widened metaphyses with dense bands ➢ Bone-within-bone appearance

Pediatric Radiology

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OI. No ossification of the membranous portions of the skull. Small chest with beaded ribs and accordioned long bones due to multiple in utero fractures

Pediatric Seminar 2: Skeletal Dysplasia

Osteopetrosis with diffusely dense bones, bone-within-bone appearance, and deformity due to pathologic femoral neck fractures Osteopetrosis. Acute femoral neck fracture on earlier film

Osteopetrosis with sandwich vertebrae

Pyknodysostosis • • • •

• •

AR, presents in infancy Micrognathia, short fingertips, fractures Generalized osteosclerosis Skull ➢ Wormian bones ➢ Marked delay in closure of sutures and fontanelles ➢ Obtuse mandibular angle Thorax – resorption of acromial ends of clavicles Extremities – resorption of phalangeal tufts

Case 9 – 17 year old with short stature

Pyknodysostosis. Skull shows persistent unfused sutures and very obtuse mandibular angle

Pyknodysostosis. Diffuse increased bone density and resorption of phalangeal tufts

Pediatric Seminar 2: Skeletal Dysplasia

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Pediatric Radiology

Pediatric Seminar 3: Cystic Fibrosis and Pulmonary Infections of the Immunocompromised Child Ellen Chung, LTC, MC Case 1 – 15 yo with fever and respiratory distress Cystic Fibrosis Epidemiology1 •

• • •

Most common lethal autosomal recessive disease in white populations (1:2500 live births) Rare in blacks (4% of cases) Very rare in Asians (0.2% of cases) Exceedingly rare in Native Americans (0.02% of cases)

1Cystic Fibrosis Foundation Registry 1990

Annual Data Report.

Cystic Fibrosis Epidemiology1 • Mean age at diagnosis 6 mo • 80% diagnosed by age 3 years • 10% of newly diagnosed are 18 years or older • 52.8% male • 11% of new diagnoses due to family history of CF

PA chest radiograph shows bilateral hyperinflation and right much greater than left linear and nodular opacity predominantly in the upper lobes. Waters view of the paranasal sinuses demonstrates complete opacification of the paranasal sinuses

1Cystic Fibrosis Foundation Registry. 2004 Annual Data Report.

Cystic Fibrosis CF Gene • • • • •

Long arm of chromosome 7 Encodes a large single chain protein, CF transmembrane conductance regulator (CFTR) CFTR forms cell membrane chloride channel 3 base pair deletion (delta F508 mutation) accounts for 70% CF cases Remaining cases due to over 1100 different mutations

Cystic Fibrosis Cellular Physiology • • • •

Normal CFTR: Epithelial chloride channel supplies luminal water by osmosis Abnormal CFTR: Decreased water flow produces viscous inspissated luminal secretions Exocrine duct obstruction Enhanced bacterial colonization

Cystic Fibrosis Exocrine Sites • • • • • • •

Bronchioles and small bronchi Pancreas Intestinal crypts Biliary ducts Vas deferens Cervix Sweat and salivary glands

Pediatric Radiology

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Pediatric Seminar 3: Cystic Fibrosis

Cystic Fibrosis Clinical Presentation • • • • • •

Neonatal intestinal obstruction in 16-20% Respiratory manifestations & pansinusitis Failure to thrive Malabsorption Unexplained hypochloremic acidosis Positive family history

Cystic Fibrosis Protean Expressions •

•

Pulmonary Manifestations ➢ Recurrent infection ➢ Pulmonary insufficiency Gastrointestinal Manifestations ➢ Pancreatic abnormalities ➢ Intestinal obstruction ➢ Nonobstructive bowel manifestations ➢ Hepatobiliary disease ➢ Gastrointestinal malignancy

Cystic Fibrosis Lung Disease • • • • • • •

Principal cause of morbidity and mortality Abnormal mucus obstructs terminal airways Decreased mucociliary transport Air trapping & increased dead space Colonization by S. Aureus, H. flu, Pseudomonas sp., atypical mycobacteria, Burkholderia cepacia complex Bronchiectasis Alveoli usually spared

Cystic Fibrosis Pathogenesis of Lung Disease • • • • •

Airway macrophages promote neutrophil (PMN) influx Elastase from autolyzed PMNs digests elastin, causing bronchiectasis and fibrosis Elastase is powerful mucus secretagogue Neutrophil death release high molecular weight DNA (Pulmozyme) Abnormal CFTR protein may bind pathogens (mucoid strain of Pseudomonas)

Cystic Fibrosis Pulmonary Complications • • • • • •

Pneumothorax Allergic bronchopulmonary aspergillosis Acute and chronic respiratory failure Hemoptysis from dilated bronchial arteries Pulmonary hypertension Cor pulmonale

Lung Disease in CF Pathologic Features • • • •

Bronchi filled with mucoid exudate laden with degenerating neutrophils Bronchial mucosa features increased goblet cells & focal metaplastic squamous epithelium Ciliary changes Bronchiectasis

Pediatric Seminar 3: Cystic Fibrosis

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Pediatric Radiology

Case 2 – 18 yo with respiratory distress

PA chest radiograph shows tunneled central venous catheter, bilateral linear and confluent opacities, and bilateral hilar enlargement

Lung Disease in CF Radiographic Features • • • • •

Increased lung volumes Bronchial impaction (“finger in glove” appearance) Hilar adenopathy Saccular bronchiectasis with upper lobe preponderance Thin-walled subpleural cysts

Case 3 - 14 yo with fever and cough Lung Disease in CF Cysts • • • • •

Cyst seen on CXR - saccular bronchiectasis vs abscess Less likely pneumatocele AFL may be seen in bronchiectasis or abscess Abscess rare in older children except with CF Subpleural blebs may be seen on HRCT

Case 4 – 13 yo with recurrent respiratory tract infections

PA radiograph of the chest shows a round mass with air fluid level in the right middle lobe

Pediatric Radiology

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Pediatric Seminar 3: Cystic Fibrosis

High resolution chest CT image on the left is in expiratory phase of respiration and shows mosaic pattern typical of small airways disease. Image on right shows dilated bronchi with thickened walls due to bronchiectasis

Lung Disease in CF - HRCT • • • • •

Currently CT is not part of the routine follow-up of CF patients. CXR and PFT are. HRCT is much more sensitive than CXR for bronchiectasis – 90% HRCT is much more sensitive for early and reversible changes of CF than CXR or PFT HRCT is becoming an outcome surrogate for CF Objective evaluation of HRCT is prerequisite

Case 5 – 14 yo with malabsorption

Axial IV contrast enhanced CT image shows complete replacement of the pancreas with fat

Exocrine Pancreatic Insufficiency • • • •

> 80% have clinical pancreatic insufficiency Insufficient lipolytic & proteolytic enzymes for normal digestion & absorption of nutrients Steatorrhea correlates with enzyme output < 10% of normal Delta 508 mutation – higher incidence of pancreatic insufficiency and earlier onset of lung disease and colonization with pseudomonas

“Pancreatic Sufficiency” • • • • • • •

10-15% of CF patients Do not require enzyme supplements Better nutritional status Older at diagnosis – later onset of lung disease Lower Pseudomonas colonization rates Better prognosis May convert to pancreatic insufficiency with age (genetically determined)

Cystic Fibrosis Endocrine Dysfunction • • • •

Glucose intolerance in 30-50% Diabetes mellitus develops in 1% of children & 13% of adults Screened annual starting at age 14 DM due to pancreatic fibrosis & other unknown factors

Pediatric Seminar 3: Cystic Fibrosis

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Pediatric Radiology

Cystic Fibrosis of the Pancreas Pathologic Features • • • • • • •

Proximal duct obstruction from inspissated pancreatic juice Acinar atrophy & inflammation Progressive interstitial fibrosis Fatty replacement Duct ectasia Micro- & macrocysts Calcification – punctate and diffuse or chunky

Cystic Fibrosis of the Pancreas Imaging Findings • • • •

Radiographs: Punctate pancreatic calcifications US: Small echogenic pancreas CT: Fat attenuation, +/- calcifications, small cysts, complete pancreatic replacement by macrocysts (rare) MRI: Variable signal intensity depending on amount of fat & fibrosis

Case 6 - 12 yo with failure to thrive

Sonogram on the left shows simple cyst anterior to the SMV and posterior to the left lobe of the liver. CT shows two simple cysts in the pancreas

Case 6 – Newborn with emesis and abdominal distension

KUB of infant shows many loops of dilated, unfolded bowel with soap bubble lucencies in the right lower quadrant

Pediatric Radiology

Contrast enema in the same patient showing a microcolon. The ileum is of larger caliber than the colon and shows multiple filling defects (arrow)

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Pediatric Seminar 3: Cystic Fibrosis

GI Manifestations of CF Intestinal Obstruction • • • • •

Meconium ileus Meconium plug syndrome Distal intestinal obstruction syndrome (“meconium ileus equivalent”) Intussusception Fibrosing colonopathy

GI Manifestations of CF Meconium Ileus • • • • •

Earliest clinical manifestation of CF 10-15% of CF patients present with meconium ileus All pts. with meconium ileus have CF Dysfunction of secretory intestinal epithelium plus panc enzyme insufficiency Distal small bowel obstruction from dessicated meconium pellets

Meconium Plug Syndrome • • • • •

Colonic obstruction in neonates Not a syndrome but a symptom 25% have CF The rest have functional immaturity of the colon or Hirshprung disease Contrast enema may relieve obstruction

Distal Intestinal Obstruction Syndrome (DIOS) • • • • • • • •

Formerly termed “meconium ileus equivalent” Reserve dx for patients with obstruction 10-15 % of CF pts (usually adolescents & adults) Results from fluid loss and poor compliance with pancreatic enzyme replacement May mimic appendicitis (appy uncommon in CF) Distal obstruction pattern on plain films Fecal mass in RLQ Enema may be therapeutic but usually treated medically

GI Manifestations of CF Rectal Prolapse • • • • •

Occurs in 20% of CF patients Presents in first years of life Resolves spontaneously by approx. 5 years Associated with bulky stools, diarrhea, or constipation Improved by pancreatic enzyme supplementation

GI Manifestations of CF Intussusception • • • •

Occurs in approx. 1% of pts. with CF Mean age of presentation = 10 years Usually ileocolic Lead points: adherent fecal residue, enlarged lymphoid follicles, chronically distended appendix, or DIOS

GI Manifestations of CF Fibrosing Colonopathy • • •

Contrast enema shows shortening and loss of haustration in ascending colon with short focal narrowing. Also there is a large round filling defect proximal to the narrowing. Colonscopy revealed the narrowing was a stricture and the filling defect was an inflammatory pseudopolyp

Usually right colon High-strength pancreatic enzyme supplementation compounded by high protease intake strongly implicated Submucosal fibrosis, fatty infiltration, mural thickening, haustral loss, shortening, stricture formation

Pediatric Seminar 3: Cystic Fibrosis

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Pediatric Radiology

Case 7 – 10 yo with abdominal pain

Air lucency is seen in the wall of the colon in the left upper quadrant on this CT image representing pneumatosis

Cystic Fibrosis Nonobstructive Bowel Manifestations • • • • • •

Thickened nodular mucosal folds in duodenum & small bowel “Jejunization” of colon Duodenal ulcer seen at autopsy in 10% Gastroesophageal reflux Barrett esophagus Pneumatosis intestinalis

Cystic Fibrosis Hepatobiliary Disease • • • • • •

Cholelithiasis (cholesterol stones) in 12-24% Microgallbladder at autopsy in 25% Atrophy or obstruction of cystic duct Distal CBD stricture Fatty liver Focal biliary cirrhosis and portal hypertension

Case 8 – 16 year old with hematemesis

Left images shows longitudinal linear filling defects in the esophagus indicating varices. Also note bronchial artery embolization coils. Axial image from a contrast enhanced CT shows fat density in the liver, cholecystectomy clips, and a large coronary vein in the left upper quadrant. (Patient underwent splenectomy as an infant.)

Focal Biliary Cirrhosis • • • • • •

Pathognomonic for CF Up to 40% of CF pt Attributed to thickened intrahepatic bile duct secretions Periductal inflammation, focal biliary fibrosis, & ductular proliferation Multinodular cirrhosis in 5 - 12% Portal hypertension and end-stage liver disease 1%

Pediatric Radiology

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Pediatric Seminar 3: Cystic Fibrosis

Radiologic Pathology 2006-2007 - Volume III - INDEX

1st Branchial Cleft Cysts 1278 Aberrant Internal Carotid Artery 1073 Aberrant LSCA 1377, 1454 Aberrant RSCA (Right Subclavian Artery) 1375 Abnormal Ureteral Insertion 1332 Abnormal Axis of Intrarenal Collecting System 1333 Abscess 1133 Abscess (Intracranial) 1234 Absent Internal Carotid Artery 1073 Absent Pulmonary Artery 1455 Absent Pulmonary Valve Syndrome 1380 Abuse injuries (Child) - Differential Diagnosis 1499 Accessory Parotid Tissue 1275 Achondroplasia 1527 Acinic Cell Carcinoma 1280 Acoustic Schwannoma 1079 Acquired Cholesteatoma 1076 Acquired Renal Cysts 1382 Active plaques (MS) 1038 Acute Chest Syndrome 1526 Acute disseminated encephalomyelitis 1040 Acute Disseminated Encephalomyelitis (ADEM) 1040 Acute GI Disorders (Infants and Children) 1353 Acute Meningitis 1232 Acute Pulmonary Interstitial Emphysema 1446 Acute Pyeloneprhitis 1335 Acyanotic CHD 1472 Acyanotic CHD with Increased PBF 1473 ADC (Aids Dementia Complex) 1237 Adenoid Cystic Carcinoma 1280 Adenoid Enlargement 1368 Adenoma 1251 Adenoma (Pleomorphic) 1279 Adenoma Sebaceum 1198 Admixture Lesions 1479 Adrenal Hematoma 1410 Hemorrhage 1410 Masses (Pediatric) 1402 Medullary Tumors 1402 Metastases 1410 Adrenocortical Cancer 1409 Adrenocortical Tumors 1409 Aggressive Fibromatosis 1276 AIDS Dementia Complex (ADC) 1237 AIDS related infections (Intracranial) 1231 Air Leak 1445 “Pseudocysts” 1447 Pneumomediastinum 1447 Pneumothorax 1447 Airway (Pediatric) 1363 Albers-Schonberg Disease 1533 Alcohol 1041 Allergic fungal sinusitis 1244 with polyps 1244 Alobar Holoprosencephaly 1310 Alveolar Proteinosis 1451 AMEN Differential Diagnosis 1079 Amyloid angiopathy 1039 Anencephaly 1307 Aneurysm (suprasellar) 1255 Aneurysms (Intracranial) 1210 Deconstructive Therapy 1218 Dissecting 1212

Saccular 1212 Angiomyolipoma 1201, 1399 ANGIOMYOMATOSIS vs. LYMPHANGIOMYOMATOSIS 1202 Annular Tear/Fissure 1290 Annulus of Zinn 1089 Anomalous innominate artery 1454 Antenatal Pelvicaliectasis 1336 Anterior chamber: aqueous humor 1088 Antoni A and B fibers 1079 Antral Web 1342 Antrochoanal polyp 1245, 1368 Anus (Imperforate) 1351 Aortic Arch 1378 Double 1378 Embryology 1374 Anomalies 1454 Aortic Coarctation 1466 Aortic Stenosis 1470 Aorticopulmonary Window 1477 Appendicitis 1360 Aqueous humor (Globe) 1088 Aqueous Protein Solution 1163 Arachnoid Cyst 1109 Arnold Chiari Malformation 1308 Arrested Pulmonary Development 1439 Arteriosclerosis 1039 Arteriosclerosis / venous collagenosis 1039 Arteriovenous Fistula (Orbit) 1100 Arteriovenous Malformation (Lung) 1442 Ash-Leaf Spots 1199 Asphyxia 1506 Asphyxiating Thoracic Dysplasia (Jeune Syndrome) 1528 Aspirated Foreign Body 1365 Aspiration syndromes 1448 Astrocyte Mutation 1142 Astrocytoma 1058, 1141, 1109, 1145 Circumscribed 1139 Diffuse 1142 Pilocytic 1139 Atresia Colonic 1349 Duodenal 1345 Esophageal 1341 Ileal 1347 Jejunal 1347 Tricuspid 1468 Atrial Septal Defect 1463, 1474 Atrial Switch 1469 Atrioventricular Canal 1477 Atrioventricular Septal Defect 1464 Atypical Teratoid / Rhabdoid Tumor 1053 Autosomal Dominant Polycystic Kidney Disease (ADPKD) 1387 Autosomal Recessive Polycystic Kidney Disease (ARPKD) 1383 AVM (Brain) 1222 AVM Grading (Intracranial) 1222 Azygos Continuation 1460 Azygous Vein 1459 Basal Ganglia Thalamus 1119 Beckwith-Wiedemann syndrome 1388 Benign Astrocytoma 1139, 1143 Benign Lymphoepithelial Lesions 1278 I1

Benign Sacrococcygeal Teratoma 1422 Benign sinus lesions 1247 Berdon Syndrome 1349 Bezoar 1356 Bezold abscess 1078 Biliary Cirrhosis (Cystic Fibrosis) 1541 Binswanger's 1039 Bladder Diverticula 1332 Blood Brain Barrier 1131 Blowout Orbital Trauma 1093 Blunt trauma 1498 Blyth and Ockenden Clinical Classification (ARPKD) 1384 Bone Marrow Components 1425 Bone Marrow Imaging (Pediatric) 1425 Bony Orbit 1088 Borden Classification (Dural AVF) 1225 Borrelia burgdorferi 1237 Bourneville Disease 1197 Brachial Plexus Traction Injury 1296 Brain (Congenital Abnormalities) 1307 Brain Tumor 1137 Branchial Cleft Cyst 1269, 1278 Bright cerebellum sign 1496 Bronchial Atresia 1372 Bronchogenic Cyst 1437 Bronchopulmonary Dysplasia 1447 Butterfly pattern 1046 Callosal Dysgenesis 1316 Calyceal Diverticulum 1381 Capillary Telangiectasia (Brain) 1229 Carbon monoxide poisoning 1041 Carcinoma (Choroid Plexus) 1151 Cardiac Imaging (Pediatric) 1453 Cardiomediastinal Silhouette Size 1473 Carotid Artery 1073 Carotid Body Paragangliomas 1272 Carotid Space 1269 Catecholamine Production 1402 Caudal Regression Syndrome 1265 Cavernous Angioma (Brain) 1228 Cavernous Hemangioma 1098 Cavernous Malformation (Brain) 1229 Cavernous Sinus Invasion (Pituitary Macroadenoma) 1254 Cellulitis (Orbit) 1101 Central Neurocytoma 1058, 1060, 1061 Central Posterior Fossa Lesion 1109 Cephaloceles 1307 Cerebellar Infarction 1507 Cerebellar Liponeurocytoma 1050 Cerebellopontine Angle Masses 1079 Cerebral edema 1496 Cerebral Hemiatrophy (Dyke-Davidoff-Masson Syndrome) 1312 Cerebral Infarction 1123 Cerebral Intraventricular Neoplasms 1058 Cerebral Neuroblastoma 1157 Cerebritis 1231 Ceruminoma 1079 Cervical aortic arch 1378 Cervical Fascia 1266 Chamber Assessment 1472 CHARGE Syndrome 1366 Chemotherapy 1042, 1155 Chiari I Malformation 1308 Chiari II Malformation - (Arnold Chiari Malformation) 1308 Chiari III Malformation 1309 Chiari IV Malformation 1309 Chiasmatic-hypothalamic glioma 1256

Child Abuse 1491 Differential Diagnosis 1499 Childhood Neck Neoplasms 1370 Choanal Atresia 1366 Cholesteatoma 1074, 1076 Cholesterol Granuloma (Cyst) 1083 Chondrodysplasia Punctata 1530 Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) 1529 Chondrosarcoma 1084 Chordoma 1158 Choriocarcinoma 1178 Chorioretinitis 1092 Choroid Plexus Carcinoma 1063, 1064 Metastasis 1065 Neoplasms 1151 Papilloma 1063 Tumors 1063 Choroidal Detachment 1090 Chylothorax 1448, 1451 Clear Cell Sarcoma (Kidney) 1396 Cleft Brain 1314 Clivus 1115 Cloquet’s canal 1091 Closed-Lip (Fused) 1314 CNS Bacterial Infections 1130 CNS infections (acquired) 1232 CNS Lyme Disease 1237 CNS Lymphoma 1045 CNS Neoplasms – Chromosome Loss of Heterozygosity 1192 CNS Neoplasms: Chromosomes 1184 CNS Tuberculosis 1235 Coats’ Disease 1092 Cochlea 1068 Colloid Cyst 1058, 1062, 1116, 1163 Colonic Aganglionosis (Total) 1351 Colonic Atresia 1349 Common Atrium 1485 Complete Atrioventricular Septal Defect 1464 Complete Labrynthine Aplasia 1072 Complete Transposition of Great Vessels 1479 Complicated Pneumonia 1523 Compression Fractures 1430 Conal Lesions 1094 Congenital Abnormalities of the Brain 1307 Adrenal Hyperplasia 1411 Anomalies (UTI) 1329 Cholesteatoma (Epidermoid) 1074 Dehiscence of Tegmen Tympani 1074 Heart Disease 1379, 1463, 1472 hyperplasia (Adrenal) 1410 Lobar Emphysema 1435 Lung Malformations 1435 Megacalyces 1339 Megacystis-Megaureter 1339 Midline Nasal Mass 1367 Spinal Anomalies 1260 Congenitally Corrected TGV 1480 Contrast Enema (Malrotation) 1344 Contrast Enhancement 1126 Contrast Enhancement- Abscess 1133 Contrast Enhancement: Hematoma 1134 Contusion (CNS) 1324 Convexity Extraaxial Differential 1124 Convexity Intraaxial 1123 I2

Cord Herniation (Idiopathic Transdural) 1296 Cord injury 1497 Coronary Artery Aneurysms-Kawasaki 1460 Cortical Tubers 1200 Cranial injury 1494 Cranial Nerve Enhancement 1129 Nerves 1107 Nerves III, IV, VI 1090 Vascular Development 1194 Craniopharyngioma 1114, 1253, 1255, 1319 Creutzfeldt-Jakob Disease 1235 Crohn Disease 1360 Crohn's 1037 Cryptococcus meningitis 1238 Cryptococcus neoformans 1238 CSF Dissemination 1156 Homeostasis 1152 Spread - Zuckerguss (Sugar Icing) 1157 CT Angiography: Basic Protocol 1453 Cyanotic CHD 1467, 1472, 1478 Cyanotic CHD with Decreased PBF 1487 Cyst (Neurenteric) 1264 Cystadenoma (Ovary) 1418 Cystic Adenomatoid Malformation 1436 Fibrosis 1535 CF Gene 1535 of the Pancreas 1539 Masses (Pediatric Renal Tumors) 1398 neoplasms (Ovary) 1415 Neoplasms: Ovarian Teratoma 1417 nephroma 1398 Partially Differentiated Nephroblastoma 1398 Renal Disease of Childhood 1381 Renal Tumor (Multilocular) 1398 Cysticercosis (Intracranial) 1236 Cysts of the CNS 1160 Dandy-Walker Malformation 1312 Darling’s Classification 1481 Dating of intracranial blood 1496 DAVF (Dural AVF) 1225 Dawson's fingers 1038 DBO’s MR Signal Abnormalities 1191 Deep and Periventricular 1121 Degenerative Disc Disease 1290 Degenerative Disease (Spine) 1290 Dehiscence of Tegmen Tympani 1074 Dehiscent jugular bulb 1073 DeMorsier’s Syndrome (Septo-Optic Dysplasia) 1310 Demyelinating Diseases 1037 Demyelination 1039, 1040, 1041 Imaging 1043 Dermal Sinus (Dorsal) 1264 DERMOID 1162 Dermoid / Epidermoid (Orbit) 1101 Dermoid Cysts 1287 Desmoplastic Infantile Ganglioglioma / Astrocytoma 1048 Differential diagnosis of abuse injuries 1499 Dilated Azygous Vein 1460 Dirty retrobulbar fat (Grave's Disease) 1094 Disc Disease 1290 Extrusion with Migration 1291 herniations and types 1291 Disorders of Neuronal Proliferation 1313, 1314

Distal Intestinal Obstruction Syndrome (DIOS) 1540 Dorsal Induction (Disorders of) 1307 Double Aortic Arch 1378 Double Arch 1454 Double Outlet Right Ventricle 1485 Double Ring sign 1085 Drug abuse 1041 Ductus Arteriosus 1456 Duodenal Atresia/Stenosis/Web 1345 Hematoma 1356 Duplication Cyst (GI tract location) 1360 Dural Tail 1131, 1169 Dyke-Davidoff-Masson Syndrome 1196, 1312 Dysembryoplastic Neuroepithelial Tumor 1051 Dysgenesis (Callosal) 1316 Dysgerminoma 1419 Dysostosis Multiplex 1532 Dysphagia 1374 Dysplasias with Prominent Membranous Bone Involvement 1531 Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) 1049 EAC cholesteatoma 1078 Ear 1068 Ebstein Malformation 1488 Echogenic Kidneys in Neonate 1386 Ectopic Ureter 1332 Eisenmenger Physiology 1478 Elevated Prolactin 1113 Ellis-van Creveld Syndrome 1529 Embryonal Sarcoma 1515 small cell tumor 1053 Emphysema (Pulmonary Interstitial) 1446 Encephalitis 1040, 1231 Encephalocele 1074 Endocardial Cushion Defect 1477 Endolymphatic Sac Tumor 1205 Enteric Duplication Cyst 1360 Enteric Fistula (Dorsal) 1263 Enterocolitis (Necrotizing) 1353 Enterocolitis (Neutropenic) 1361 Ependymitis granularis 1037 Ependymoma 1058, 1109, 1112, 1149 Epidermoid 1081, 1161 Epidermoid Inclusion Cyst 1109 Epidermoid vs. Arachnoid Cyst 1109 Epidural abscess 1231 Epidural Phlegmon / Abscess 1295 Epiglottitis 1364 Epstein-Barr virus (EBV) 1037 Esophageal Atresia 1341 Esthesioneuroblastoma 1248, 1368 Ethmoid sinus 1240 Exocrine Pancreatic Insufficiency 1538 Exostoses 1288 Exostosis (External Ear) 1078 External auditory canal (EAC) atresia 1071 External Ear Masses 1078 External Ear Neoplasms 1079 External Otitis 1078 Extraaxial Lesions 1106 Extraaxial Tumors 1158 Extraconal Lesions 1100 Extralobar Sequestration 1440 Extraocular muscles (EOM) 1089 Extrapontine myelinolysis 1041

I3

Facet Joint Synovial Cysts 1292 Facial Nerve Palsy 1081 Failed Back Surgery Syndrome (see also FBBS) 1292 Fallot 1487 FBSS (Failed Back Surgery Syndrome) 1293 Cervical Spinal Canal Stenosis 1293 Ossification (Posterior Longitudinal Ligament) 1294 Posterior Longitudinal Ligament (Ossification) 1294 Type I Arachnoiditis 1293 Type II Arachnoiditis 1293 Fibromatosis (Aggressive - Masticator Space) 1276 Fibromatosis Colli 1267, 1371 Fibrosing Colonopathy (Cystic Fibrosis) 1540 Fibrous Dysplasia (Paranasal Sinuses) 1246 Fibrous Histiocytoma (Orbit) 1099 Filum Terminale (Congenital Anomalies) 1263 Fissures of Santorini 1078 Focal Biliary Cirrhosis 1541 Follicular cysts 1415 Foreign Body 1365, 1366 Forensic Radiology of Child Abuse 1491 Fourth Ventricle 1109 Fractures (Healing) 1493 Fried egg appearance 1061 Frontal horn “capping” 1037 Frontal Lipoencephalocystocele 1307 Frontal sinus 1241 Functional Ovarian Cyst 1416 Fungal sinusitis 1102, 1245 Ganglioglioma / Gangliocytoma 1047 Ganglioneuroblastoma 1402, 1408 Ganglioneuroma 1402 Gastric Atresia 1342 Gastrostomy Tubes 1356 Gastrulation 1263 Gaucher Disease 1431 Germ Cell Tumors 1176, 1418 Germ Cell Tumors-AFIP Series 1177 Germinal Matrix 1502 Germinoma 1121, 1177 Germinoma (Infundibular) 1257 Ghost tumor 1046 GI Disorders (Acute - Infants and Chidren) 1353 GI Tract Obstruction 1341 Glioblastoma Multiforme 1119, 1147, 1322 Glioma 1109 Glioma (Chiasmatic-hypothalamic) 1256 Globe 1088 Globus pallidus 1191 Glomerulocystic Disease 1386 Glomus jugulare 1082 Glomus tympanicum 1082 Gradenigo’s syndrome 1078 Grading Problems in Gliomas 1140 Graf System (Pediatric Hip) 1519 Granulocyte Colony Stimulating Factor 1429 Granulosa-theca Cell Tumor 1419 Graves’ 1037 Graves’ Disease 1094 Great Vessels (Transposition) 1468 Grey Matter Heterotopias 1315 HAART 1239 Hamartoma 1510 Hamartoma (hypothalamic) 1257 Healing of fractures 1493 Healing of metaphyseal fractures 1492 Hemangioblastoma 1109, 1111, 1203, 1320 Hemangioblastomatosis 1202

Hemangioendothelioma 1511 Hemangioma 1370 Hemangiomas (suprahyoid Neck) 1266 Hemangiopericytoma 1173 Hemangiopericytoma vs. Meningioma 1173 Hematoma (Duodenal) 1356 Hemimegalencephaly 1313 Hemorrhage 1502 Adrenal 1410 Child Abuse 1495 Intracranial 1221 Hemorrhagic Cysts (Ovary) 1416 Hemorrhagic Infarction 1131 Hemorrhagic Ovarian Cysts 1416 Henoch-Schönlein Purpura 1356 Hepatobiliary Disease (Cystic Fibrosis) 1541 Hepatoblastoma 1513 Hepatocellular Carcinoma 1514 Hernia (Inguinal) 1357 heroin 1041 Herpes encephalitis 1234 Heterotopias (Gray Matter) 1315 High Jugular Bulb (“Megabulb”) 1073 Highly Active Antiretroviral TX (HAART) 1239 Hip Effusion 1519, 1520 Hip Sonography 1518 Hirschprung Disease 1350 HIV encephalitis 1040, 1041 HIV Leukoencephalopathy 1238 Holoprosencephaly (Alobar) 1310 Holoprosencephaly (Semilobar) 1310 Horseshoe kidney 1330 HSV Encephalitis 1131 Hurler Syndrome 1532 Hyaline Membranes 1444 Hydranencephaly 1310 Hydrocarbon aspiration 1366 Hydrocephalus 1181 Hydrocolpos 1420 Hydronephrosis 1338 Hyperostosis in Meningiomas 1171 Hypertrophic Pyloric Stenosis 1354 Hypogenetic Lung Syndrome 1439 Hypoperfusion complex (Child Abuse) 1499 Hypopharyngeal cyst 1369 Hypoplastic left heart syndrome 1466, 1485 Hypothalamic Hamartoma 1257 Hypothalamus 1115 Hypoxic-ischemic encephalopathy 1039 Iatrogenic Demyelinating Disorders: Chemotherapy 1042 Iatrogenic white matter degeneration 1037 Idiopathic Transdural Cord Herniation 1296 Ileal Atresia 1347 Ileus (Meconium) 1348 Imaging Studies (UTI) 1331 Immaturity of the Colon (Functional) 1349 Immune reconstitution syndrome 1239 Immunocompromised Child 1535 Immunocompromised Patient 1045 Imperforate Anus 1351 Incudostapedial disruption 1085 Infantile Hemangioendothelioma 1511 Infarction 1506, 1507 Infections (Intracranial) 1231 Infections (Spine) 1290, 1295 Inferior orbital fissure 1088 Inflammatory disease of the salivary glands 1369 Infratentorial 1106

I4

Inguinal Hernia 1357 Inner Ear Anomalies 1071 Innominate Artery Compression 1455 Innominate Artery Compression Syndrome 1379 Interhemispheric extra-axial hemorrhage 1495 Internal Auditory Canal 1107 Internal Carotid Artery 1073 Interrupted Pulmonary Artery 1455 Intestinal Obstruction (Neonatal) 1341 Intraaxial Lesions 1106 Intraconal Lesions 1097 Intracranial Aneurysms 1210 blood (Dating) 1496 Germ Cell Tumors 1176 Germinoma 1177 Infections 1231 Lipoma 1182 Vascular Malformations 1220 Intralobar Sequestration 1440 Intramural Hemorrhage (GI - Differential) 1356 Intrarenal Collecting System (Abnormal Axis) 1333 Intrarenal Reflux 1334 Intrasellar Pathology 1251 Intraventricular Lesions 1106 Intraventricular Meningioma 1065 Intussusception 1357 Cystic Fibrosis 1540 Reduction 1358 Inverted Papilloma (Paranasal Sinuses) 1247 Irritable Hip 1519 Ischemic Enhancement 1131 Jantene Procedure 1469 Jejunal Atresia 1347 Jeune Syndrome 1528 JNA (Juvenile Nasopharyngeal Angiofibroma) 1246 Joubert’s Syndrome 1313 Jugular bulb 1073 Jugular Diverticulum 1073 Foramen Masses 1083 Paragangliomas 1270 Jugulotympanic Paraganglioma 1082 Juvenile Angiofibroma 1367 Juvenile Nasopharyngeal Angiofibroma (JNA) 1246 Kawasaki disease 1460 Keratosis obturans 1078 Kernohan-Sayre (AFIP) Grading System 1137 Kidney (Medullary Sponge) 1382 Kidney (Multicystic Dysplastic) 1399 Kidney (Rhabdoid Tumor of) 1395 Labrynthine Aplasia 1072 Lacrimal Gland Lesions 1102 Lacrimal Sac Lesions 1103 Langerhans Cell Histiocytosis (Sella) 1258 Large Endolymphatic Duct and Sac (LEDS) 1072 Laryngeal- Tracheopapillomatosis 1372 Laryngomalacia 1371 Laryngotracheal cleft 1371 Left (Double) Superior Vena Cava 1458 Left paramediastinal structures (Differential Diagnosis) 1458 Left Superior Vena Cava 1458 Leukocoria 1090 Leukoencephalopathy (HIV) 1238 Lhermitte-Duclos Disease 1049 Lingual Thyroid 1370 Lingual Thyroid Gland 1285 Lipoencephalocystocele (Frontal) 1307

Lipoma (Intraspinal) 1262 Liposarcoma (Suprahyoid Neck) 1267 Lissencephaly 1314 Listeria Monocytogenes 1130 Liver Metastases 1516 Liver Tumors (Pediatric) 1509 Lobar emphysema 1435 Long bone shaft fracture 1491 Long Parotid Tails 1275 Low Back Pain 1290 Low Intestinal Obstruction 1347 Lumbar 1292 Facet Arthropathy 1292 Spinal Canal and Foraminal Stenosis 1292 Lung Agenesis 1439 Lung Disease(Cystic Fibrosis) 1537 Lung Diseases in Neonates 1444 Lyme disease 1231 Lymphangioma (Orbit) 1100 Lymphatic Malformations (SupraHyoid Neck) 1267 Lymphocytic hypophysitis 1252 Lymphoepithelial Lesions (Benign - Suprahyoid Neck) 1278 Lymphoma 1324, 1371 CNS 1045 Orbit) 1098 Pediatric Renal Tumors) 1400 Sella) 1258 in AIDS 1324 vs. Toxoplasmosis 1324 Macroglossia 1369 Madelung’s Disease 1267 Malfixation (Duodenum) 1343 Malignant Astrocytoma 1145 Compression Fracture 1430 Germ Cell Tumors 1418 Meningioma 1172 Sinus Lesions 1247 Malrotation – UGI 1344 Malrotation (Duodenum) 1343 Mandibular Hypoplasia 1369 Marburg 1039 Marchiafava-Bignami disease 1042 Marfan syndrome 1460 Marrow Components 1425 Conversion 1427 Depletion Fatty Replacement 1432 Distribution 1427 Reconversion 1428 Replacement or Infiltration 1429 Masses - Ring Enhancing (CNS) 1322 Masticator Space 1274 Mastoiditis 1078 Mature Teratoma (Ovary) 1417 Maxillary sinus 1241 McKusick 1531 Measles 1037 Meckel Diverticulum 1359 Meckel-Gruber Syndrome 1388 Meconium Aspiration Syndrome 1449 Meconium Ileus 1348 Cystic Fibrosis 1540 Meconium Peritonitis 1348 Meconium Plug Syndrome 1540 Mediastinal Bronchogenic Cysts 1438 Medullary Cystic Disease Complex 1387

I5

Medullary Sponge Kidney 1382 Medullary Tumors (Adrenal) 1402 Medulloblastoma 1109, 1154, 1318 Medulloblastoma - Desmoplastic 1157 Megabulb 1073 Megacalyces 1339 Megalencephaly (Unilateral) 1313 Megaureter 1339 Melanoma (Uveal) 1092 Membranous Tracheitis 1365 Meninges (Neoplasms) 1164 Meningioma 1065, 1081, 1108, 1125, 1164 MENINGIOMA *Imaging Features: CT vs. MR 1169 Angiography Transit Time 1170 Atypical Imaging 1172 Dural Tail 1131 Hyperostosis 1171 Vasogenic Edema 1167 Suprahyoid Neck 1270 Suprasellar 1254 Tentorial 1320 MR Imaging 1167 Meningitis 1231 Meningocele 1262 Mesenchymal Hamartoma 1509 Mesoblastic Nephroma 1396 Metabolic imaging 1043 Metaphyseal Chondrodysplasia 1530 Metaphyseal fracture 1491-1492 Metastasis (Pituitary and Infundibulum) 1259 Metastatic Lesions (Orbit) 1100 Methotrexate 1042 Michel’s deformity 1072 Microangiopathy 1039 Microgastria 1342 Microgyria 1314 Middle Ear 1068 Midgut Loop (Normal Rotation) 1343 Midgut Volvulus 1343 Migraine 1039 Mirror Image Right Arch 1376 Modic Changes 1291 Modified Papile Classification 1502 Brain (Neonatal) 1501 Mondini’s dysplasia 1072 Monosymptomatic demyelinating 1037 Morquio Syndrome 1533 MR Angiography: Basic Technique 1453 Mucocele 1246 Mucoepidermoid Carcinoma 1281 Mucopolysaccharides 1094 Multicystic Dysplastic Kidney 1382, 1399 Multilocular Cystic Renal Tumor 1398 Multiple sclerosis 1037, 1039, 1325 MR 1038 Mustard Procedure 1469 myasthenia gravis, 1037 myelinolysis 1041 myelitis 1040 Myelocystocele (Terminal) 1262 Myelofibrosis 1431 Myeloid Depletion: MRI 1432 Myelomeningocele 1260 Nasal Cycle 1128 Dermoid 1367

polyps 1368 Nasopharyngeal Carcinoma (NPSCCa) 1283 Neck Neoplasms (Child) 1370 Necrotizing Enterocolitis 1353 Necrotizing External Otitis 1078 Neonatal Brain 1501 GI Tract Obstruction 1341 Hypoxic-Ischemic Injury 1502 Low Intestinal Obstruction (Differential Diagnosis) 1351 Pneumonia 1450 Respiratory Distress 1444 Lung Diseases 1444 Neoplasms (Benign - Masticator Space) 1276 Neoplasms (Malignant - Masticator Space) 1277 Neoplasms of the Meninges 1164 Nephroblastoma (Cystic Partially Differentiated) 1398 Nephroblastomatosis 1394 Cortical Nodule 1395 Diffuse 1394 Nephrogenic Rests: Location 1394 Nephroma (mesoblastic) 1396 Nerve Sheath Tumors 1187 Neuroblastic Tumors 1402 Neuroblastoma 1371, 1402 Stage Distribution 1408 Neuroectodermal Tumor 1053 Neuroepithelial Tumors 1047 Neurofibroma vs. Schwannoma 1188 Neurofibromas (Pelvis) 1423 Neurofibromatosis 1185 Type 2 1191 Type 1 or von Recklinghausen Disease 1185 Neuromyelitis optica (Devic syndrome) 1039 Neuronal Proliferation (Disorders of) 1313, 1314 Neutropenic Enterocolitis 1361 Non-Astrocytic Gliomas 1149 Non-Glial Lesions 1158 Nonhemorrhagic Infarction 1506 Non-Hodgkin Lymphoma (NHL) -Pharyngeal Mucosal Space 1283 Non-Lissencephalic Cortical Dysplasias Microgyria/Polymicrogyria 1314 Normal Cranial Nerve Enhancement 1129 Enhancement 1128 Marrow (MR Features) 1426 Pineal Calcification 1175 Thymus 1521 Vertebral Marrow: MRI 1428 Norrie’s 1091 Obstruction (GI Tract - Neonatal) 1341 Olfactory Neuroblastoma 1248 Olidodendroglioma 1152 Oncocytoma (Suprahyoid Neck) 1280 Ophthalmic veins 1088 Opportunistic neoplasm 1045 Optic Nerve Glioma 1097 Sheath Meningioma 1097 Optic neuritis 1040 Oral Cavity Normal Anatomy 1286 Orbit 1088 Orbital Cellulitis 1101 fissures 1088 Lymphoma 1098

I6

septal system 1088 Trauma 1093 Varix 1099 Organic toxins 1041 Oropharynx 1284 Osmotic myelinolysis 1041, 1042 Ossicular Derangement 1085 Ossifying Renal Tumor of Infancy 1397 Osteogenesis Imperfecta 1533 Osteoma (Paranasal Sinuses) 1246 Osteomyelitis 1431 Osteomyelitis (Spine) 1295 Pyogenic 1295 Tuberculous 1295 Osteopetrosis (Albers-Schonberg Disease) 1533 Osteoporotic Fracture 1430 Ostiomeatal complex (Paranasal Sinuses) 1241 Ostium Primum ASD 1464 Otitis 1078 Otosclerosis 1085 Otospongiosis 1085 Outer Ear Anomalies 1071 Ovarian Cancer 1420 Cyst 1416 Cystadenoma 1418 Maturation 1414 Tumors 1415 Ovary (Prepubertal vs Postpubertal) 1414 Palatine Tonsil Enlargement 1368 Pancreas (Child Abuse) 1498 Pancreatic injury 1498 Papillary Cystadenoma Lymphomatosum 1280 Papillary Endolymphatic Sac Tumor 1083 Papilloma 1151 Papovavirus 1238 Paradoxical Embolus 1475 Paragangliomas 1082, 1270 Paranasal Sinuses 1240 Paraovarian cysts 1415, 1417 Parapharyngeal Abscess 1365 Parapharyngeal Space 1267, 1268 Parasellar Region 1250 Parinaud Syndrome 1175 Parotid Space 1278 Parotid Tail 1275 Partial Anomalous Venous Return (Pulmonary) 1456 Patent Ductus Arteriosus 1456, 1476 Patterns of Enhancement 1126 Patterns of Location 1106 Pediatric Adrenal Masses 1402 Airway 1363 Hip Sonography 1518 Liver Tumors 1509 Pelvic Masses 1414 Posterior Fossa Tumors 1318 Renal Tumors 1390 Tuberculosis 1526 Pelvic Masses (Pediatric) 1414 Pelvicaliectasis (Antenatal) 1336 Peritonitis (Meconium) 1348 Periventricular Hemorrhagic Infarction 1503 Sonography 1503 Periventricular Leukomalacia 1504 periventricular white matter 1039 Persistent hyaloid (Cloquet’s) canal 1091 Persistent Hyperplastic Primary Vitreous (PHPV) 1091

Persistent Interstitial Pulmonary Emphysema 1446 Persistent Stapedial Artery 1073 Petrous Apex (Differential Diagnosis) 1083 Phakomatoses 1184 Pharyngeal Mucosal Space 1282 Pharyngeal Perforation 1342 Pheochromocytoma 1408 Phlegmon / Abscess (Epidural) 1295 Pial A-V Fistula 1225 Pilocytic Astrocytoma 1110, 1134, 1139 Pilocytic Astrocytoma (Juvenile Pilocytic) 1141 Pineal Calcification 1175 Pineal Cyst 1121, 1181 Pineal Neoplasms Laboratory Tests 1179 Parenchyma 1180 Region Masses 1175, 1320 Region Neoplasms 1178 Pineal/Quadrigeminal Cistern Region 1121 Pinealomas 1121, 1175 Pineoblastoma 1121, 1180 Pineocytoma 1121, 1180 Pituitary 1250 Pituitary Adenoma 1113, 1253 Apoplexy 1254 Macroadenoma 1253 Neoplasms 1251 Plasma Cell Granuloma 1522 Pleomorphic Adenoma 1279, 1288 Pleomorphic Xanthoastrocytoma 1051, 1141 Plexiform Neurofibromatosis 1423 Pneumatoceles 1524 Pneumonia 1523 Pneumonia (term & premature neonates) 1448 Polycystic Kidney Disease (autosomal Recessive) 1383 Polymicrogyria 1314 Port Wine Stain 1193 Post fossa cysts 1109 Posterior chamber 1088 Posterior Fossa Malformations 1312 Tumors (Pediatric) 1318 Posterior Hyaloid Detachment 1090 Posterior Reversible Encephalopathy Syndrome (PRES) 1039, 1040 Posterior rib fractures (visualization) 1493 Posterior Urethral Valves 1337 Precocious Puberty 1175 Premature Births 1444 Premature Brain 1501 Prepubertal ovary 1414 Primary Megaureter 1339 Pringle’s Disease 1199 Profound Asphyxia 1506 Progressive multifocal leukoencephalopathy 1040 Progressive Multifocal Leukoencephalopathy (PML) 1041 Prolactin 1113 Prolactinoma 1251, 1253 Proteinosis (Alveolar) 1451 Proximal Neonatal Intestinal Obstruction 1347 Pseudotumor (Orbit) 1095 Pubertal ovary 1414 Pulmonary Abscess 1524 Arterial Anomalies 1455 Artery Stenosis 1470

I7

Atresia with Intact Ventricular Septum 1489 AVM 1442 Blastoma 1522 Blood Flow 1472 Bronchogenic Cyst 1438 Hypoplasia 1439 Infections 1521 Infections (Immunocompromised Child) 1535 Interstitial Emphysema 1446 Sequestration 1440 Sling 1379, 1455 Underdevelopment 1439 Venous Anomalies 1456 Pulsatile Tinnitus Lesions 1074 Pyeloneprhitis 1335 Pyknodysostosis 1534 Pyloric Stenosis 1354 Pyogenic Abscess (Intracranial) 1234 Pyogenic Osteomyelitis 1295 Pyriform Aperture stenosis 1367 Radiation 1155 Injury (Brain) 1043 Necrosis vs. Tumor (CNS) 1323 Ranulas 1287 Rathke Cleft Cyst 1252 Reactive Airways Disease 1365 Rebleeding 1497 Rectal Prolapse (Cystic Fibrosis) 1540 Rectus: medial, lateral, superior, inferior 1089 Red Marrow Signal 1427 Reflux Nephropathy 1335 Renal Agenesis 1329 Renal Cell Cancer 1399 Renal Cyst 1381 Renal Ectopia 1330 Renal Ectopia and Fusion 1330 Renal Tumors (Infancy and Young Children) 1390 Respiratory Distress (Neonatal) 1444 Respiratory Distress Syndrome (RDS) 1444 Retained fetal lung fluid 1448, 1449 Retina 1089 Retinal Detachment (RD) 1090 Retinoblastoma 1091 Gene 1091 Retinopathy of prematurity 1092 Retrobulbar (“Postseptal”) Space 1089 Retropharyngeal Cellulitis 1364 Reversal sign 1496 Rhabdoid Tumor 1054 Rhabdoid Tumor of Kidney 1395 Rhabdomyosarcoma 1368, 1420 Orbit 1101 Male Bladder & Prostate 1421 Rhabdomyosarcomatoid variant of Wilms tumor 1053 Rhombencephalosynapsis 1313 Rib fracture 1492 Rib Notching 1189 Right aortic arch 1454 Right Arch 1376 Right paramediastinal structures (Differential Diagnosis) 1459 Right to Left Shunts 1487 Ring Enhancing Mass 1132 Ring Lesion Features For Infection 1132 Ring Lesions Differential 1132 Ring-enhancing Masses (CNS) 1322 Risk Factors (subarachnoid Hemorrhage) 1211

Rotation of Midgut Loop 1343 Round Pneumonia 1522 Rules for Ring Enhancing Mass 1132 Sacrococcygeal Teratoma 1421 SAH (Subarachnoid Hemorrhage) 1210 Aneurysms (Intracranial) - Infectious 1218 Aneurysms (Intracranial) - Treatment Options 1218 Clinical Grading Scale 1213 CT 1214 CTA 1215 DSA 1215 induced Vasospasm 1214 Infectious Intracranial Aneurysms 1218 Lumbar puncture 1214 MRA 1215 MRI 1215 Outcomes 1213 Patterns 1213 Radiologic Grading Scale 1213 Risk Factors 1210 Screening 1217 Salivary glands (Inflammatory disease) 1369 Salt and pepper appearance 1082 Sarcoidosis (CNS) 1321 Sarcoidosis (Sella) 1258 SATCHMO 1319 SCCa (Squamous Cell Carcinoma) 1248 Schizencephaly 1314 Schwannoma 1107, 1188, 1192 Acoustic - Vestibular 1079 Orbit 1098 Scimitar Syndrome 1457 Sclera 1089 Scutum 1076 Second Branchial Cleft Cyst 1269 Secundum ASD 1464 Segmental Spinal Dysgenesis 1265 Sella 1250 Sella/Parasellar Region - Differential 1113 Sellar Masses: “SATCHMO” 1319 Semicircular Canals (SCC) 1068 Semilobar Holoprosencephaly 1310 Senescent White Matter Changes 1039 Senile Macular Degeneration 1090 Septic Arthritis (Hip) 1520 Septo-Optic Dysplasia (DeMorsier’s Syndrome) 1310 Sequestration 1525 Sertoli-Leydig cell tumor 1419 Sex Cord-Stromal Tumors 1419 Shaking mechanism 1492 Short Rib-Polydactyly 1528 Shunt Lesions 1463, 1487 Sickle Cell Anemia 1429 Simple Renal Cyst 1381 Single Ventricle 1484 Physiology 1489 Sinus Mass Differential 1368 Sinus Venosus ASD 1464 Sinuses (Paranasal) 1240 Sinusitis 1243 Sinusitis (Fungal) 1102 Sjogren’s Syndrome 1279 Skeletal Dysplasia 1527 Skeletal injury (evaluation) 1494 Skull fracture 1497 SLE 1037 Soap-bubble appearance 1051 I8

Spetzler-Martin Grading System (Intracranial Vascular Malformations) 1222 Sphenoid sinus 1242 Spina Bifida 1262 Spinal Anomalies (Congenital) 1260 Caudal Regression Syndrome 1265 Chiari II Malformation 1261 Complex Dysraphic States 1263 Dorsal Dermal Sinus 1264 Dorsal Enteric Fistula 1263 Fibrolipomatous Infiltration of Filum 1263 Gastrulation 1263 HemiMMC/Hemimyelocele 1261 Intraspinal Lipoma 1262 Lipoma with Dorsal Defect 1261 Lipomyelomeningocele 1261 Meningocele 1262 Myelocele (Myeloschisis) 1261 Myelomeningocele 1260 Neurenteric Cyst 1264 Persistent Terminal Ventricle 1263 Posterior Spina Bifida 1262 Segmental Spinal Dysgenesis 1265 Spinal Dysraphism 1260 Split Cord Malformation 1264 Terminal Myelocystocele 1262 Tight Filum Terminale 1263 Spinal Dysraphism 1260 Spine 1290 Spine injury 1493 Split Cord Malformation 1264 Squamous cell carcinoma 1248 Stapedial Artery 1073 Stridor 1363, 1374 Sturge-Weber Syndrome 1193 Subacute sclerosing panencephalitis 1040 Subarachnoid Hemorrhage 1210 Subdural empyema 1231 Subependymal Giant Cell Astrocytoma 1058, 1061 Subependymal Nodules 1200 subependymal veins 1039 Subependymoma 1058, 1059 Subepidermal Fibrosis 1199 Subglottic edema 1363 Subglottic Hemangioma 1370 Sugar Icing 1156 Superior and inferior ophthalmic veins 1088 Superior Left Intercostal Vein 1458 Superior orbital fissure 1088 Suprahyoid Neck 1266 Suprasellar Masses 1253 Supratentorial 1106 Supratentorial Primitive Neuroectodermal Tumor 1052 Surfactant 1445 Surfactant B protein deficiency 1448 Swyer-James Syndrome 1436, 1524 Systemic Gas Embolism 1447 Systemic Venous Anomalies 1458 Taenia solium 1236 Tegmen Tympani 1074 Temporal Bone Fracture 1084 Temporal Bone: Anatomy 1068 Congenital Lesions 1068 Infectious Lesions 1076 Neoplastic Lesions 1076

Temporomandibular joint (TMJ) anomalies 1071 Tenon’s capsule 1089 Tentorial Meningioma 1320 Teratoid Tumor 1053 Teratoma 1178, 1371 Ovary 1417 Sacrococcygeal 1421 vs. Dermoid (Pineal Region) 1178 Tetralogy of Fallot 1455, 1467, 1487 Thanatophoric Dysplasia 1527 Thiamin deficiency 1042 THIRD VENTRICLE 1116 Thoracic MRA & CTA 1453 Thymus (Pediatric - Normal) 1521 Thyroglossal Duct Cysts 1284 Thyroid Gland (Lingual) 1285 Thyroid Orbitopathy (Graves’ Disease) 1094 Time Density Curves 1126 Tinnitus 1074 Tongue Base Mass 1369 Top 10 Pelvic Lesions 1423 Total Anomalous Pulmonary Venous Return 1480 Total Anomalous PV Return 1469 Total Colonic Aganglionosis 1351 Toxic Demyelination 1041 Toxocara canis 1092 Toxocariasis 1092 Toxoplasmosis 1119 Intracranial 1238 Tracheal bronchus 1372 Tracheal Stenosis 1372 Tracheomalacia 1371 Transient Tachypnea of Newborn 1449 Transposition of Great Vessels 1468 Transverse Myelitis 1039 Tricuspid Atresia 1468, 1484, 1489 Trilateral Retinoblastoma 1180 Truncus Arteriosus 1469, 1483 Tuberculosis (Intracranial) 1231 Tuberculosis Pediatric 1526 Tuberous Sclerosis or Bourneville Disease 1197 Tumefactive Demyelination 1134 Tumor Blush 1171 UGI (Upper GI Tract - Malrotation) 1344 ulcerative colitis 1037 Uncommon Neuroepithelial Tumors 1045 Undifferentiated Embryonal Sarcoma 1515 Unilateral Megalencephaly (Hemimegalencephaly) 1313 Upper esophageal foreign body 1366 Ureterocele 1333 Ureteropelvic duplication 1333 Ureteropelvic Junction Obstruction 1338 Urethral Valves (Posterior) 1337 Urinary Tract Infection (Child) 1329 US Guidance 1520 Uterine Morphology: Maturation 1415 Uvea: choroid 1089 Uveal Melanoma 1092 Uveal Metastasis 1093 VACTERL 1342 Vagal paraganglioma 1082 Vagale Paragangliomas 1271 Vaginal Rhabdomyosarcoma 1421 Vallecular Cyst 1369 Valvular Pulmonic Stenosis 1470 Varix (Orbit) 1099 Vascular Anomalies (Pediatric Cardiac Imaging) 1453 Vascular Malformations (Intracranial) 1220

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Vascular Mediated Disorders (Bone Marrow) 1433 Edema 1433 Ischemia 1433 Ischemia & Edema: Causes (Bone Marrow) 1433 Vascular Rings and Slings 1374 Vascular White Matter Disease 1039 Vasculitis 1039 Vein Of Galen Malformation 1181 Venous Anomaly (Intracranial - Developmental) 1226 Venous Collagenosis 1039 Ventral Induction (Disorders of) 1310 Ventricular Septal Defect 1474 Ventricular Septal Defects 1465 Vesicoureteric Reflux 1332 Vestibular Schwannoma 1079, 1108 Vestibule 1068 Viral and Postviral Demyelination 1040 Viral Croup 1363 Virchow-Robin spaces 1037 Visceral injury 1498 Vitreous body 1088 Volvulus (Midgut) 1343 von Hippel-Linmdau Syndrome: NIH Classification 1202 von Recklinghausen Disease 1185 Warburg’s 1091 Warthin’s Tumor 1280 Wernicke encephalopathy 1042 White Matter Changes (Senescent) 1039 WHO 2000 Brain Tumor Classification 1137 Whole-body MRI 1426 Wilms Tumor 1390 Wishart Disease 1191 Wolman Disease 1411 Xanthoastrocytoma 1052 Xanthoastrocytoma (Pleomorphic) 1141 Yellow Marrow Signal 1427 Zellweger Syndrome 1388

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