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Neuroendocrine Tumors NeuroendocrineNeuroendocrineNeuroendocrine TumorsTumors Tumors AA A ComprehensiveComprehensive Comprehensive GuideGuide Guide toto to DiagnosisDiagnosis Diagnosis andand and ManagementManagement Management 5th5th EditionEdition 20122012 31/08/12 3:01 AM 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 NPI# 1013917020 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 U.S. Department of Justice (DEA) Registration Number RI0119722 ©2012 Inter Science Institute. Format, text and presentation of this publication may not be reproduced for commercial purposes without written permission of Inter Science Institute. Inter Science Institute, Inc. 944 W. Hyde Park Boulevard Inglewood, CA 90302 (800) 255-2873 (310) 677-3322 Fax: (310) 677-2846 www.interscienceinstitute.com email: [email protected] Cover design: Tullula G. Mamikunian, B.S. 341002c_1&4-S_LINK.indd 2 NeuroendocrinE TumorS A Comprehensive Guide to Diagnosis and Management Fifth Edition Aaron I. Vinik Eugene A. Woltering Thomas M. O’Dorisio Vay Liang W. Go Gregg Mamikunian The fifth edition of the NETs handbook is dedicated to Dr. Maria Tellez Since Dr. Maria Tellez’s arrival at Inter Science in 2003, she was chosen twice as “Employee of the Year” in 2007 and 2011. Her dedication, devotion and performance under the most demanding circumstances are beyond description. No words can describe my respect and admiration for her enormous scientific talents and immense personal integrity and humility. How fortunate for Inter Science and its employees and associates to have the privilege of knowing and working with her. Thank you Maria. Sincerely, Gregg August 27, 2012 Inter Science Institute’s G.I. Council 2002-2012 Chairman Eugene A. Woltering, M.D., F.A.C.S. The James D. Rives Professor of Surgery and Neurosciences Chief of the Sections of Surgical Endocrinology & Oncology Director of Surgery Research The Louisiana State University Health Sciences Center New Orleans, LA 70112 Executive Members Aaron I. Vinik, M.D., Ph.D., FCP., MACP Professor of Medicine, Pathology and Neurobiology Director of Strelitz Diabetes Research Center & Neuroendocrine Unit Eastern Virginia Medical School Norfolk, VA 23510 Thomas M. O’Dorisio, M.D. Professor of Medicine Director of Neuroendocrine Tumor Program Clinical Attending, Holden Comprehensive Cancer Center University of Iowa College of Medicine Iowa City, IA 52242 Vay Liang W. (Bill) Go, M.D. Distinguished Professor of Medicine David Geffen School of Medicine at UCLA University of California at Los Angeles Los Angeles, CA 90095 Gregg Mamikunian, M.S. Chief Executive Officer Inter Science Institute, Inc. iv Preface In 1971, Inter Science Institute (ISI) established its first commercial GI laboratory, and six years later published the first GI handbook that listed critical GI peptides of clinical significance. The commercial availability of such tests was groundbreaking at that time. A few years later, more than twenty cases of Zollinger-Ellison Syndrome were documented surgically as a result of ISI’s gastrin analyses. As a result of the chemical evidence of these hormone concentrations, many pancreatic, adrenal, ovarian, renal, and gastric tumors were determined at leading medical institutions. It is no accident that the field of neuroendocrine laboratory procedures at ISI began to expand its menu, that was first available in 1994, to develop specific, additional biomarkers to assist in the diagnosis and treatment of neuroendocrine patients. In 2002, a decade ago, ISI established its second GI Council with the vision of presenting the medical community with advances in GI, pancreatic and regulatory peptides. Since then, we have published a dozen peer-reviewed scientific articles as to the utility, significance, and predictability of these biomarkers to provide diagnosis in healthy and disease states. The history of any major discovery, not only in medical science, and whether accidental or intentional, has been amply directed towards the benefit of all mankind. It would be foolhardy for a university, government laboratory, or private research laboratory to lay claim as guarantors and guardians of such lofty ideals and goals. When any one of such entity waves the banner of exclusivity, it diminishes the noble pursuit of scientific inquiry and achievement. Fortunately, history can recite endlessly the great achievements, discoveries, and breakthroughs that have emerged through the collaborative efforts of educational, governmental and commercial entities. ISI will, as it has in the past, expand its reach to academic and commercial institutions both in the US and overseas in the furtherance of neuroendocrine advances for the benefit of the patient. Gregg Mamikunian Chief Executive Officer August 12, 2012 v Acknowledgments to the Fifth Edition A special appreciation and thank you to Dr. Aaron I. Vinik for his valuable time afforded in bridging the scientific content and clinical applications in this current edition. The authors of the fifth edition of the neuroendocrine tumors handbook sincerely thank M. Sue O’Dorisio, M.D., Ph.D. for her contribution updating Chapter 2 of the handbook. Our thanks to Debra C. Godsey, R.N. for updating the ICD-9 codes throughout the various chapters. A special and great thank you to Mia S. Tepper, MBA for the months of diligent attention to every detail in reviewing and editing this manuscript. The fifth edition does reflect the dedication of the G.I. Council members’ ever-ready guidance to ensure a handbook that reflects their commitment to excellence in providing current and credible scientific information to the physician, and the ultimate beneficiary ---- the patient. To this end, my personal and sincere thanks to each of the Council members, Drs. Vinik, O’Dorisio, Go and its chairman, Dr. Woltering for a decade of selfless service, guidance and encouragement. Thank you, Gregg Mamikunian Inter Science Institute 2012 vi 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 scientific 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 scientific matrix and its accretion into a clinically useful and diagnostically relevant format must be applauded. Gregg Mamikunian Inter Science Institute 2009 Reprinted from Inter Science Institute’s Neuroendocrine Tumors: A Comprehensive Guide to Diagnosis and Management handbook, 2009. vii 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
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