Neuroendocrine Tumors a Comprehensive Guide to Diagnosis and Management
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Neuroendocrine Tumors A Comprehensive Guide to Diagnosis and Management InterScience Institute InterScience Institute Gregg Mamikunian Aaron I. Vinik Thomas M. O’Dorisio Eugene A. Woltering Vay Liang W. Go Inter Science Institute Accreditations, Certifications, Licensers and Registries CLIA #05D0549406 Centers for Medicare and Medicaid (CMS) U.S. Department of Health and Human Services (DHHS) The College of American Pathologists (CAP) #2258701 State Licenses State of California Clinical License Number CLF 1801 Radioactive Materials License Numbers 1972-19, 3398-19 State of Florida Agency for Health Care Administration License Number L800013770 Maryland Department of Health and Mental Hygiene Medical Laboratory Permit Number 862 Pennsylvania Department of Health Lab ID Number 026479 Rhode Island Department of Health License Number 288 U.S. Federal Licenses Social Security Administration (SSA) Medicare Provider Number 05L0008967 Medi-Cal Provider Number ZZZ49545Z Food and Drug Administration (FDA) Registration Number 2050103 Nuclear Regulatory Commission (NRC) License Number 04-17814-01E Department of Justice (DEA) Registration Number RI0119722 Copyright © 2009 Inter Science Institute Inter Science Institute 944 W. Hyde Park Boulevard Inglewood, CA 90302 (800) 255-2873 (310) 677-3322 Fax: (310) 677-2846 www.interscienceinstitute.com email: [email protected] Format, text, and presentation of this publication may not be reproduced for commercial purposes without written permission of Inter Science Institute. Cover design: Tullula G. Mamikunian NeuroendocrinE TumorS A Comprehensive Guide to Diagnosis and Management Fourth Edition Gregg Mamikunian Aaron I. Vinik Thomas M. O’Dorisio Eugene A. Woltering Vay Liang W. Go Inter Science Institute GI Council Chairman Eugene A. Woltering, MD, FACS The James D. Rives Professor of Surgery and Neurosciences Chief of the Sections of Surgical Endocrinology and Oncology Director of Surgery Research The Louisiana State University Health Sciences Center New Orleans, Louisiana Executive Members Aaron I. Vinik, MD, PhD, FCP, MACP Professor of Medicine, Pathology and Neurobiology Director of Strelitz Diabetes Research Institute Eastern Virginia Medical School Norfolk, Virginia Vay Liang W. (Bill) Go, MD Professor of Medicine David Geffen School of Medicine at UCLA University of California at Los Angeles Los Angeles, California Thomas M. O’Dorisio, MD Professor of Medicine Director of Neuroendocrine Tumor Program Clinical Attending, Holden Comprehensive Cancer Center University of Iowa College of Medicine Iowa City, Iowa Gregg Mamikunian, MS Chief Executive Officer Inter Science Institute ii Preface Accretion “ . the wisdom that accrues with age . .” It is a fi tting tribute that the 4th edition of the Neuroendocrine Tumors handbook falls on the 40th anniversary of Inter Science Institute (ISI). The publication arising since the third edition is testimony to the progress of the science of Neuroendocrinology in the treatment of carcinoid tumors specifi cally and neuroendocrine sciences in general. The accretion of knowledge and its particular applications of these accreted test procedures have placed ISI at the forefront of providing the highly specialized tests and assays for usage by the medical establishment. This has been the simple mantra of ISI since its inception, “stay small but perform with distinction”. The accretion of leadership, performance, experience, commitment, and the delivery of accurate dependable analytical data, that correlates with the clinical manifestation, is the ultimate hallmark of ISI’s capabilities. Thus, we have arrived at this fortieth juxtaposition with a certain degree of pride and a greater degree of satisfaction. Clinical laboratory discipline is a dynamic science, and it must be nurtured by accruing talented scientists in the specifi c disciplines it serves. Consequently my high esteem is owed to the GI Council members who have provided ISI the catalyst to distinction in recommending test procedures that are novel and clinically signifi cant. Thus a debt of gratitude is owed to the Council members, who are embarking on their second fi ve-year terms, and have served tirelessly resulting in the authorship of the 3rd and 4th editions of the Neuroendocrine Tumors handbooks. Finally I can assure the Carcinoid community—patients, their physicians, clinicians and support groups—that ISI’s commitment, dedication, and support will not take recess as long as I am at the helm. Gregg Mamikunian Inter Science Institute 2009 iii Acknowledgments to the Fourth Edition The authors of the 4th edition of the Neuroendocrine Tumors handbook sincerely appreciate the contribution of M. Sue O’Dorisio, MD, PhD for the second chapter of the handbook. It is a great honor and privilege to have professor Sue O’Dorisio’s hitherto unpublished chapter appear in the ISI Neuroendocrine book. The GI Council welcomes professor Joy Ardill, MD, of Royal Victoria Hospital of Northern Ireland, (UK), for her scientifi c collaborative association with Inter Science Institute. The Council acknowledges Donald W. Richardson, MD of Eastern Virginia Medical School (EVMS) for his contribution on sections of pituitary tumors, Cushings and diabetes insipidus. Our thanks to Debbie Godsey for updating the ICD-9 codes throughout the chapters of the 3rd and 4th editions. Also, our thanks to Etta Vinik for her work on certain sections of the handbook. A special thanks to Mia Tepper for the months of diligent attention to details in reviewing the manuscript. The executive members of the council gratefully acknowledges the dedication, foresight and leadership of its chairman Eugene A. Woltering, MD. The enormous contributions of Arthur A. Vinik, MD, Thomas M. O’Dorisio, MD, and Vay Liang W. (Bill) Go, MD, encompassing the scientifi c matrix and its accretion into a clinically useful and diagnostically relevant format must be applauded. Gregg Mamikunian Inter Science Institute 2009 iv Acknowledgments to the Third Edition The GI Council of Inter Science Institute presents this comprehensive guide to diagnosis and management of neuroendocrine tumors to provide information and inspiration to all levels of clinicians, from novices to those professionally engaged in the field of neuroendocrine research, treatment, and analyses. This guidebook adds the new dimension of patient monitoring, not only through powerfully discriminating assays but through the recognition of clinical presentations and syndromes. This expertise is made possible by more than 150 years of cumulative experience of the advisory council. Since the publication of the first GI Handbook in 1977 up to the current edition of Neuroendocrine Tumors, Inter Science Institute has been at the forefront of bridging the gap between academic medicine and the availability of the most current tests for patient diagnosis. In the intervening three and a half decades, unparalleled progress has been made both in the diagnosis and treatment of gastrointestinal, pancreatic, and neuroendocrine tumors. This book is meant to be a beacon not only for listing tests but for all aspects of neuroendocrine tumors. Its publication represents a move from static text to the modern era of communication which allows for dynamic, continuously updating links to the ISI website, interscienceinstitute.com, as well as endotext.com as reference sources. Additionally, the book combines several references from the previous edition with an updated bibliography, in recognition of past contributions to the present. Special thanks to our dedicated reviewers of this publication, Etta J. Vinik and Mia S. Tepper. Finally, my appreciation and thanks to professors Vinik, Woltering, O’Dorisio, and Go for imparting their knowledge to the synergistic confluence that has given birth to this unique edition. Thank you, Arthur, Gene, O’Do, and Bill. Gregg Mamikunian Inter Science Institute 2006 Reprinted from Inter Science Institute’s Neuroendocrine Tumors: A Comprehensive Guide to Diagnosis and Management handbook 2006. v Acknowledgments to the Second Edition The current 1997 edition of the GI, Pancreatic Hormones, Related Peptides and Compounds® handbook presents comprehensive information for many rare procedures and tests that have been requested in the course of the past twenty-eight years. The handbook reflects the tremendous advances that have been made since 1977. The number of tests offered has increased six-fold in addition to increasing specificity, sensitivity of antibodies, and purity of the standards. The protocols dealing with challenges and provocative testing has been expanded with the latest information. The section on the physiology of the GI and Pancreatic Hormones has been updated as an adjunct to the various procedures in the handbook. Furthermore, the handbook covers a vast area of gastrointestinal, pancreatic, and other related procedures. Many of these procedures are clearly out of the realm of routine testing and request. On the other hand, quite a number of the procedures are indicators in the clinical confirmation of certain syndromes and disease states. Inter Science has witnessed the phenomenon over the years of the transformation of research-oriented procedures becoming useful, routine, and critical determining factors in the diagnosis and management of certain GI-related endocrinopathies. A special acknowledgment to Alan C. Kacena for his dedication and service of twenty-five years at Inter Science Institute and in bringing the current edition of the GI Hormones handbook into