Management of Adrenal Masses in Children and Adults
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1 Management of Adrenal Masses in Children and Adults
2 Electron Kebebew Editor Management of Adrenal Masses in Children and Adults
3 Editor Electron Kebebew Endocrine Oncology Branch National Institutes of Health National Cancer Institute Bethesda, MD, USA ISBN ISBN (ebook) DOI / Library of Congress Control Number: Springer International Publishing Switzerland 2017 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
4 I would like to dedicate this book to my wife Tida, and sons Ras and Ezana. I would also like to thank all my teachers, mentors, and colleagues for their support.
5 Preface Adrenal neoplasms are common and are often discovered incidentally. Virtually every medical provider will encounter a patient with an adrenal neoplasm during their training and clinical practice. Given the aging population, the number of patients found to have an adrenal neoplasm will continue to increase. The field of adrenal neoplasms has been transformed due to significant developments in the last decade. There have been significant advances in our knowledge of adrenal neoplasms include the identification of genetic changes that result in these tumors, and genotype phenotype associations that have emerged and that have significant clinical implications in the management of these tumors, including screening of at-risk family members. The type of treatment selected, improved high resolution anatomic and functional imaging studies, advances in biochemical diagnostic tests to distinguish between functioning and nonfunctioning tumors, and the application of advanced surgical technology and techniques have improved patient outcome. This unique textbook is focused on adrenal neoplasms and written by a multidisciplinary team of experts. It provides a comprehensive, state-of-the art view of this field, and will serve as a valuable resource for medical students, clinicians, and researchers. The book reviews new data about common genetic syndromes that impact management strategies, imaging, biochemical evaluation to optimize treatment, and new and emerging approaches to the treatment of adrenal neoplasms. We hope this textbook will serve as a useful resource for all providers dealing with, and interested in these common but challenging tumor. Bethesda, MD, USA Electron Kebebew vii
6 Contents 1 Epidemiology of Adrenal Neoplasms... 1 Pavel J. Nockel and Electron Kebebew 2 The Pathology of Adrenal Masses Ranran Zhang and Ricardo V. Lloyd 3 Genetics of Benign Adrenocortical Tumors Fady Hannah-Shmouni and Constantine A. Stratakis 4 Genetics of Adrenocortical Cancers Guillaume Assié and Jérôme Bertherat 5 Genetics of Pheochromocytoma and Paraganglioma Bruna Babic and Naris Nilubol 6 Imaging Modalities for Adrenocortical Tumors Iuliana D. Bobanga and Christopher R. McHenry 7 Imaging Modalities for Pheochromocytoma and Paraganglioma David Taïeb, Aoife Kilcoyne, Ingo Janssen, Katherine I. Wolf, Michael Austin Blake, and Karel Pacak 8 Primary Hyperaldosteronism Saïd C. Azoury and Aarti Mathur 9 Cushing Syndrome: Presentation, Diagnosis, and Treatment, Including Subclinical Cushing Syndrome Vladimir Neychev 10 Management of Pheochromocytoma and Paraganglioma Garima Gupta, Vitaly Kantorovich, and Karel Pacak 11 Congenital Adrenal Hyperplasia Ashwini Mallappa and Deborah P. Merke 12 Adrenocortical Carcinoma Sara G. Creemers, Leo J. Hofland, and Richard A. Feelders 13 Operative Techniques for Adrenal Neoplasms Dhaval Patel ix
7 x Contents 14 Therapies for Locally Advanced and Metastatic Adrenocortical Carcinoma Sarika N. Rao and Mouhammed Amir Habra 15 Management of Locally Advanced and Metastatic Pheochromocytoma and Paraganglioma Alejandro Roman-González, Paola Jiménez Vásquez, Samuel Mayer Hyde, Aaron C. Jessop, and Camilo Jimenez Index
8 Contributors Guillaume Assié, M.D., Ph.D. INSERM U1016, CNRS UMR 8104, Institut Cochin, Paris, France Faculté de Médecine Paris Descartes, Université Paris Descartes, Paris, France Department of Endocrinology, Referral Center for Rare Adrenal Diseases, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France Saïd C. Azoury, M.D. Endocrine Surgery Section, Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA Bruna Babic, M.D. Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Jérôme Bertherat, M.D., Ph.D. INSERM U1016, CNRS UMR 8104, Institut Cochin, Paris, France Faculté de Médecine Paris Descartes, Université Paris Descartes, Paris, France Department of Endocrinology, Referral Center for Rare Adrenal Diseases, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France Michael Austin Blake, M.B.B.C.H. Division of Abdominal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA Iuliana D. Bobanga, M.D. General Surgery, University Hospitals Case Medical Center, Cleveland, OH, USA Sara G. Creemers, B.Sc. Division of Endocrinology, Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands Richard A. Feelders, M.D., Ph.D. Division of Endocrinology, Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands Garima Gupta, M.D. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA xi
9 xii Contributors Mouhammed Amir Habra, M.D. Department of Endocrine Neoplasia, and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Fady Hannah-Shmouni, M.D. Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA Leo J. Hofland, Ph.D. Division of Endocrinology, Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands Samuel Mayer Hyde, M.M.Sc. Department of Surgical Oncology, Clinical Cancer Genetics Program, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Ingo Janssen, M.D. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health Clinical Center, Bethesda, MD, USA Aaron C. Jessop, M.D., M.B.A. Division of Diagnostic Imaging, Department of Nuclear Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Camilo Jimenez, M.D. Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Vitaly Kantorovich, M.D. Division of Endocrinology, Department of Medicine, University of Connecticut Health Center, Farmington, CT, USA Electron Kebebew, M.D. Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Aoife Kilcoyne, M.B.B.Ch. Division of Abdominal Imaging and Intervention, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA Ricardo V. Lloyd, M.D., Ph.D. Department of Pathology, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA Ashwini Mallappa, M.D. National Institutes of Health Clinical Center, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD, USA Aarti Mathur, M.D. Endocrine Surgery Section, Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA Christopher R. McHenry, M.D. Department of Surgery, MetroHealth Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA
10 Contributors xiii Deborah P. Merke, M.D., M.Sc. National Institutes of Health Clinical Center, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, MD, USA National Institutes of Health Clinical Center, Bethesda, MD, USA Vladimir Neychev, M.D., Ph.D. Departments of Surgery, University Multiprofile Hospital for Active Treatment Alexandrovska, Medical University of Sofia, Sofia, Bulgaria Naris Nilubol, M.D. Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Pavel J. Nockel, D.O. Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Karel Pacak, M.D., Ph.D., D.Sc. Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA Dhaval Patel, M.D. Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Sarika N. Rao, D.O. Department of Endocrine Neoplasia, and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Alejandro Román-González, M.D. Departments of Internal Medicine and Endocrinology, Hospital Universitario San Vicente Fundación, Universidad de Antioquia, Medellín, Colombia Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA Constantine A. Stratakis, M.D., D(Med)Sc. Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA David Taïeb, M.D., Ph.D. Biophysics and Nuclear Medicine, European Center for Research in Medical Imaging, La Timone University Hospital, Aix Marseille University, Marseille, France Paola Jiménez Vásquez, M.D. Department of Oncology, Hospital Militar Central, Hemato-oncologos asociados, Universidad Militar Nueva Granada, Bogota, Colombia Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
11 xiv Contributors Katherine I. Wolf, B.Sc. Section on Medical euroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health Clinical Center, Bethesda, MD, USA Ranran Zhang, M.D., Ph.D. Department of Pathology, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA
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