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Gastrinoma

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Surgical Endocrinopathies

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Abstract

Since the initial report of Zollinger–Ellison syndrome (ZES) in 1955, there have been numerous insights into physiology of gastric acid secretion, pathogenesis, and molecular biology of gastrinoma as well as natural history of the disease. As a result of these advances, effective gastric antisecretory agents have become the treatment of choice for gastric acid hypersecretion. The aim of surgery in patients with ZES has changed from gastric acid control to prevention of tumor progression with curative intent in patients with sporadic and localized ZES. Improved understanding of the molecular pathogenesis of pancreatic neuroendocrine tumors (NETs) has resulted in several new diagnostic and treatment options for patients with advance disease, such as tyrosine kinase inhibitor, mammalian target of rapamycin (mTOR) inhibitor, Gallium-68 DOTATATE, and somatostatin peptide receptor radionuclide therapy. Moreover, the classification, tumor grading, and staging system specific for gastrointestinal and pancreatic NETs have been recently developed to improve patient risk stratification. This chapter includes a brief history of ZES and provides a comprehensive review of clinical presentation, current approach to diagnose ZES and localize gastrinoma, molecular pathogenesis, treatments of sporadic gastrinoma and multiple endocrine neoplasia type 1 (MEN1)-associated ZES, and metastatic disease, as well as current classification, staging, and prognosis.

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Authors and Affiliations

  1. Endocrine Oncology Branch, National Cancer Institute, The National Institutes of Health, 10 Center Drive, Building 10, Room 4-5932, 20817, Bethesda, MD, USA

    Naris Nilubol MD

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  1. Naris Nilubol MD
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Correspondence to Naris Nilubol MD .

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  1. Divisions of General Surgery and Surgical Oncology, Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada

    Janice L. Pasieka

  2. Department of Surgery, Columbia University Medical Center, New York, New York, USA

    James A. Lee

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Nilubol, N. (2015). Gastrinoma. In: Pasieka, J., Lee, J. (eds) Surgical Endocrinopathies. Springer, Cham. https://doi.org/10.1007/978-3-319-13662-2_47

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