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Zollinger–Ellison Syndrome: Diagnosis and Management

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Management of Pancreatic Neuroendocrine Tumors

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Abstract

Zollinger–Ellison syndrome (ZES) is a clinical entity originally described as the triad of gastric acid hypersecretion, peptic ulcer disease, and the presence of a non-beta islet cell tumor of the pancreas. The signs and symptoms of this syndrome are caused by the release of the gastric acid-driving hormone gastrin from a tumor of the gastrointestinal tract. The diagnosis of ZES requires the documentation of an elevated fasting serum gastrin level in the setting of gastric acid hypersecretion, most easily identified by a low gastric pH. The management of ZES falls into two categories: the control of gastric acid hypersecretion and its clinical manifestations through the use of proton-pump inhibitors and the management of the tumor itself. The treatment involves a multimodal approach that is comprised of surgery, radiation, chemotherapy, and, more recently, the use of targeted peptide therapy depending on the tumor stage.

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Abbreviations

BAO:

Basal acid output

CT:

Computed tomography

EGD:

Esophagogastroduodenoscopy

FSG:

Fasting serum gastrin

GERD:

Gastroesophageal reflux disease

H2-R:

H2-Receptor

MAO:

Maximal acid output

MEN-1:

Multiple endocrine neoplasia type 1

PET:

Positron emission tomography

PPI:

Proton-pump inhibitor

PRRT:

Peptide receptor radionuclide therapy

PUD:

Peptic ulcer disease

SRS:

Somatostatin receptor scintigraphy

SST:

Secretin stimulation testing

ZES:

Zollinger–Ellison syndrome

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Gastroenterology, University of California, San Francisco, 513 Parnassus Avenue, Room S-357, San Francisco, CA, 94143, USA

    Maneesh H. Singh M.D.

  2. Department of Internal Medicine, Perelman School of Medicine at the University of Pennsylvania, 9th floor Penn Tower, 1 Convention Ave, Philadelphia, PA, 19104, USA

    David C. Metz M.D.

  3. Division of Gastroenterology, Perelman School of Medicine at the University of Pennsylvania, 9th floor Penn Tower, 1 Convention Ave, Philadelphia, PA, 19104, USA

    David C. Metz M.D.

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  1. Maneesh H. Singh M.D.
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  2. David C. Metz M.D.
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Correspondence to David C. Metz M.D. .

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Editors and Affiliations

  1. VA Greater Los Angeles Healthcare System, David Geffen School of Medicine at UCLA VA Greater Los Angeles Healthcare System, Los Angeles, California, USA

    Joseph R. Pisegna

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Singh, M.H., Metz, D.C. (2015). Zollinger–Ellison Syndrome: Diagnosis and Management. In: Pisegna, J. (eds) Management of Pancreatic Neuroendocrine Tumors. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1798-3_4

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