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Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome.
Dig Dis Sci. 2017 09; 62(9):2258-2265.DD

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.

Authors+Show Affiliations

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA. amendel5@jhmi.edu.Division of Gastroenterology and Hepatology, Department of Internal Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

28776139

Citation

Mendelson, Aaron H., and Mark Donowitz. "Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome." Digestive Diseases and Sciences, vol. 62, no. 9, 2017, pp. 2258-2265.
Mendelson AH, Donowitz M. Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome. Dig Dis Sci. 2017;62(9):2258-2265.
Mendelson, A. H., & Donowitz, M. (2017). Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome. Digestive Diseases and Sciences, 62(9), 2258-2265. https://doi.org/10.1007/s10620-017-4695-7
Mendelson AH, Donowitz M. Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome. Dig Dis Sci. 2017;62(9):2258-2265. PubMed PMID: 28776139.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome. AU - Mendelson,Aaron H, AU - Donowitz,Mark, Y1 - 2017/08/03/ PY - 2017/05/04/received PY - 2017/07/26/accepted PY - 2017/8/5/pubmed PY - 2017/12/8/medline PY - 2017/8/5/entrez KW - 68Gallium positron emission tomography/computed tomography KW - Gastrinoma KW - Hypergastrinemia KW - Zollinger–Ellison syndrome SP - 2258 EP - 2265 JF - Digestive diseases and sciences JO - Dig. Dis. Sci. VL - 62 IS - 9 N2 - 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. SN - 1573-2568 UR - https://www.unboundmedicine.com/medline/citation/28776139/Catching_the_Zebra:_Clinical_Pearls_and_Pitfalls_for_the_Successful_Diagnosis_of_Zollinger_Ellison_Syndrome_ L2 - https://doi.org/10.1007/s10620-017-4695-7 DB - PRIME DP - Unbound Medicine ER -