Management of Adrenal Masses in Children and Adults

314 Pages·2017·11.78 MB·English

by  Electron Kebebew (eds.)

Management of
Adrenal Masses in
Children and Adults
Electron Kebebew
Editor
123
Management of Adrenal Masses
in Children and Adults
Electron Kebebew
Editor
Management of Adrenal
Masses in Children
and Adults
Editor
Electron Kebebew
Endocrine Oncology Branch
National Institutes of Health National
Cancer Institute
Bethesda , MD , USA
ISBN 978-3-319-44134-4 ISBN 978-3-319-44136-8 (eBook)
DOI 10.1007/978-3-319-44136-8
Library of Congress Control Number: 2016952804
© Springer International Publishing Switzerland 2017
This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or
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T he use of general descriptive names, registered names, trademarks, service marks, etc. in this
publication does not imply, even in the absence of a specifi c 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
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contained herein or for any errors or omissions that may have been made.
Printed on acid-free paper
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The registered company is Springer International Publishing AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
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.
Pref ace
A drenal neoplasms are common and are often discovered incidentally.
Virtually every medical provider will encounter a patient with an adrenal neo-
plasm 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 fi eld of adrenal neoplasms has been transformed due to signifi cant
developments in the last decade. There have been signifi cant advances in our
knowledge of adrenal neoplasms include the identifi cation of genetic changes
that result in these tumors, and genotype–phenotype associations that have
emerged and that have signifi cant 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 imag-
ing studies, advances in biochemical diagnostic tests to distinguish between
functioning and nonfunctioning tumors, and the application of advanced sur-
gical 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 fi eld, and will serve as a valuable resource for medical stu-
dents, 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
Contents
1 Epidemiology of Adrenal Neoplasms .......................................... 1
Pavel J. Nockel and Electron Kebebew
2 The Pathology of Adrenal Masses................................................ 11
Ranran Zhang and Ricardo V. Lloyd
3 Genetics of Benign Adrenocortical Tumors ................................ 31
Fady Hannah-Shmouni and Constantine A. Stratakis
4 Genetics of Adrenocortical Cancers ............................................ 55
Guillaume Assié and Jérôme Bertherat
5 Genetics of Pheochromocytoma and Paraganglioma ................ 85
Bruna Babic and Naris Nilubol
6 Imaging Modalities for Adrenocortical Tumors ......................... 105
Iuliana D. Bobanga and Christopher R. McHenry
7 Imaging Modalities for Pheochromocytoma
and Paraganglioma ....................................................................... 125
David Taïeb , Aoife Kilcoyne , Ingo Janssen , Katherine I. Wolf ,
Michael Austin Blake , and Karel Pacak
8 Primary Hyperaldosteronism ...................................................... 139
Saïd C. Azoury and Aarti Mathur
9 Cushing Syndrome: Presentation, Diagnosis,
and Treatment, Including Subclinical Cushing Syndrome ....... 159
Vladimir Neychev
10 Management of Pheochromocytoma and Paraganglioma ........ 179
Garima Gupta , Vitaly Kantorovich , and Karel Pacak
11 Congenital Adrenal Hyperplasia ................................................. 207
Ashwini Mallappa and Deborah P. Merke
12 Adrenocortical Carcinoma ........................................................... 225
Sara G. Creemers , Leo J. Hofl and , and Richard A. Feelders
13 Operative Techniques for Adrenal Neoplasms ........................... 245
Dhaval Patel
ix
x Contents
14 Therapies for Locally Advanced and Metastatic
Adrenocortical Carcinoma ........................................................... 257
Sarika N. Rao and Mouhammed Amir Habra
15 Management of Locally Advanced and Metastatic
Pheochromocytoma and Paraganglioma .................................... 277
Alejandro Roman-González , Paola Jiménez Vásquez ,
Samuel Mayer Hyde , Aaron C. Jessop , and Camilo Jimenez
Index ....................................................................................................... 301
Contributors
Guillaume Assié , M.D., Ph.D. INSERM U1016, CNRS UMR 8104 , Institut
Cochin , Paris , France
F aculté de Médecine Paris Descartes, U niversité Paris Descartes, P aris,
France
D epartment of Endocrinology, Referral Center for Rare Adrenal Diseases,
Assistance Publique Hôpitaux de Paris , Hôpital Cochin , Paris , France
Saïd C. Azoury , M.D. E ndocrine 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
F aculté de Médecine Paris Descartes, U niversité Paris Descartes, P aris,
France
D epartment 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. D ivision 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, E rasmus University Medical Center, R otterdam, T he
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
xii Contributors
Mouhammed Amir Habra , M.D. Department of Endocrine Neoplasia, and
Hormonal Disorders, T he 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. Hofl and , Ph.D. D ivision 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, T he 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. D epartment 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 ,
E unice 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