Table Of ContentManagement
of Pancreatic
Neuroendocrine
Tumors
Joseph R. Pisegna
Editor
123
Management of Pancreatic Neuroendocrine Tumors
Joseph R. Pisegna
Editor
Management of Pancreatic
Neuroendocrine Tumors
Editor
Joseph R. Pisegna
David Geffen School of Medicine at UCLA
VA Greater Los Angeles Healthcare System
Los Angeles, CA , USA
ISBN 978-1-4939-1797-6 ISBN 978-1-4939-1798-3 (eBook)
DOI 10.1007/978-1-4939-1798-3
Springer New York Heidelberg Dordrecht London
Library of Congress Control Number: 2014951683
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Pref ace
N euroendocrine tumors (NETs) are becoming increasingly recognized by both
healthcare professionals and patients as being more common than previously recog-
nized. This increased awareness has resulted from increased educational efforts on
the part of several societies and patient-supported, nonprofi t organizations. We owe
much thanks to these educational efforts from dedicated health professionals,
patients, and advocates. An increase in federal funding and research support from
several nonprofi t groups such as Caring for Carcinoid Foundation, the North
American Neuroendocrine Tumor Society, and the European Neuroendocrine
Tumor Society have positively impacted the fi eld of research. Several pharmaceuti-
cal companies, including Novartis, Pfi zer, and Ipsen Pharmaceuticals Inc., have pro-
vided clinical research funding for new drugs to treat neuroendocrine tumors. As a
result of these combined efforts and as refl ected in this book, new imaging tech-
niques such as the DOTATATE-Gallium 68 and FDG-PET have improved our abil-
ity to detect small, previously unrecognized NETs. We have refi ned our endoscopic
imaging techniques to permit extirpation by endoscopic mucosal resection of small
intraluminal NETs such as gastric and rectal carcinoids. The pathology of NETs has
been refi ned which impacts the staging of disease through using molecular markers.
We now have a greater understanding of the genetics of NETs, an area which will
likely expand in the future. With these improved imaging and histopathological
techniques, our surgical colleagues have a greater awareness of tumor staging pre-
ceding a planned resection procedure. A greater understanding of the receptor and
signaling pathways of NETs has yielded directed chemotherapy and radiopharma-
ceuticals to treat regional or distant metastases. This book includes 12 chapters that
cover these important clinical areas of research and development and has been writ-
ten by experts in their respective fi elds. It is my hope that this will be the start of
increased awareness for junior investigators and seasoned clinicians to stimulate
improvements in research design and therapies which will ultimately translate to
improved clinical outcomes for patients with NETs.
Los Angeles, CA, USA Joseph R. Pisegna, M.D.
v
Contents
1 Pathology of Pancreatic Neuroendocrine Tumors ............................... 1
Nils Lambrecht
2 Inherited and Somatic Genetics of Pancreatic
Neuroendocrine Tumors ......................................................................... 9
Lauren Fishbein and Katherine L. Nathanson
3 Laboratory Assessment of NETs ........................................................... 33
Christos Toumpanakis
4 Zollinger–Ellison Syndrome: Diagnosis and Management ................. 41
Maneesh H. Singh and David C. Metz
5 Clinical Manifestations of Multiple Endocrine Neoplasia, Type 1 ..... 63
Susan Yuditskaya and Monica C. Skarulis
6 Gastric Carcinoids: Classification and Diagnosis ................................ 83
Kali Zhou and Wendy Ho
7 Endoscopic Approaches for Diagnosis of Pancreatic
Neuroendocrine Tumors ......................................................................... 95
Tarun Rustagi and James J. Farrell
8 Endoscopic Approaches to Treatment of Pancreatic
Neuroendocrine Neoplasms .................................................................... 111
Amit Raina and Vinay Chandrasekhara
9 Surgical Approaches to Pancreatic Neuroendocrine Tumors ............. 117
James X. Wu and F. Charles Brunicardi
10 Radiotherapy and Radiopharmaceuticals
for the Treatment of Pancreatic Neuroendocrine Tumors .................. 127
Lowell B. Anthony and Partha Sinha
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viii Contents
11 Nuclear Medicine Approaches to Treatment
of Neuroendocrine Tumors ..................................................................... 135
Ken Herrmann, Rudolf A. Werner, Christina Blümel,
and Martin S. Allen-Auerbach
12 Novel Targets for Future Medical Treatments ..................................... 145
Sandy T. Liu, Andrew E. Hendifar, and Edward M. Wolin
Index ................................................................................................................. 163
Contributors
Martin S. Allen-Auerbach Department of Molecular and Medical Pharmacology ,
UCLA Medical Center , Los Angeles , CA , USA
Lowell B. Anthony Division of Medical Oncology, Markey Cancer Center,
University of Kentucky , Lexington , KY , USA
Christina Blümel Department of Nuclear Medicine , Universitätsklinikum
Würzburg , Würzburg , Germany
F. Charles Brunicardi R onald Reagan UCLA Medical Center, Los Angeles, CA,
USA
UCLA Medical Center , Santa Monica , CA , USA
Vinay Chandrasekhara Gastroenterology Division , University of Pennsylvania
Health System , Philadelphia , PA , USA
James J. Farrell Yale Center for Pancreatic Disease , Yale University School of
Medicine , New Haven , CT , USA
Section of Digestive Diseases , Yale University School of Medicine , New Haven ,
CT , USA
Lauren Fishbein Division of Endocrinology, Diabetes and Metabolism,
Department of Medicine , Perelman School of Medicine at the University of
Pennsylvania , Philadelphia , PA , USA
Andrew E. Hendifar Department of Gastrointestinal Oncology , Samuel Oschin
Cancer Center, Cedars-Sinai Medical Center , Los Angeles , CA , USA
Ken Herrmann Department of Nuclear Medicine, Universitätsklinikum Würzburg ,
Würzburg , Germany
Wendy Ho Department of Medicine, Division of Digestive Diseases , UCLA ,
Los Angeles , CA , USA
ix