Catching the Zebra

Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger–Ellison Syndrome

Aaron H. Mendelson, Mark Donowitz

Research output: Contribution to journalReview article

Abstract

Zollinger–Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. While early recognition portends an excellent prognosis with >80% survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. Tumor localization is equally nuanced. Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results. Endoscopic ultrasound is another useful modality, particularly in patients with ZES in the setting of multiple endocrine neoplasia type 1. This review aims to provide clinicians with an overview of ZES with a focus on both clinical presentation and the proper utilization of the various biochemical and imaging tests available.

Original languageEnglish (US)
Pages (from-to)2258-2265
Number of pages8
JournalDigestive Diseases and Sciences
Volume62
Issue number9
DOIs
StatePublished - Sep 1 2017

Fingerprint

Equidae
Gastrins
Multiple Endocrine Neoplasia Type 1
Chromogranin A
Neoplasms
Somatostatin Receptors
Secretin
Octreotide
Somatostatin
Peptic Ulcer
Radionuclide Imaging
Diarrhea
Fasting
Stomach
Chronic Disease
Neoplasm Metastasis
Survival

Keywords

  • Gallium positron emission tomography/computed tomography
  • Gastrinoma
  • Hypergastrinemia
  • Zollinger–Ellison syndrome

ASJC Scopus subject areas

  • Physiology
  • Gastroenterology

Cite this

Catching the Zebra : Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger–Ellison Syndrome. / Mendelson, Aaron H.; Donowitz, Mark.

In: Digestive Diseases and Sciences, Vol. 62, No. 9, 01.09.2017, p. 2258-2265.

Research output: Contribution to journalReview article

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abstract = "Zollinger–Ellison syndrome (ZES) results from an ectopic gastrin-secreting tumor leading to peptic ulcer disease, reflux, and chronic diarrhea. While early recognition portends an excellent prognosis with >80{\%} survival at 15 years, symptoms are often nonspecific making the diagnosis difficult to establish. Diagnosis involves a series of tests, including fasting gastrin, gastric pH, chromogranin A, and secretin stimulation. Performing these tests in the correct sequence and at the proper time is essential to avoid inaccurate results. Tumor localization is equally nuanced. Although providers have classically used 111indium-radiolabeled octreotide with somatostatin receptor scintigraphy to evaluate tumor size and metastases, recent studies have shown superior results with newer imaging modalities. In particular, 68gallium (68Ga)-labeled somatostatin radiotracers (i.e., 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE) used with positron emission tomography/computed tomography can provide excellent results. Endoscopic ultrasound is another useful modality, particularly in patients with ZES in the setting of multiple endocrine neoplasia type 1. This review aims to provide clinicians with an overview of ZES with a focus on both clinical presentation and the proper utilization of the various biochemical and imaging tests available.",
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