Zollinger-Ellison syndrome

Last reviewed: 2 Sep 2022
Last updated: 16 Mar 2022

Summary

Definition

History and exam

Key diagnostic factors

  • refractory or recurrent peptic ulcer disease
  • diarrhea
More key diagnostic factors

Other diagnostic factors

  • epigastric abdominal pain
  • gastroesophageal reflux disease
  • steatorrhea
  • gastrointestinal¬†bleed
  • bone pain
Other diagnostic factors

Risk factors

  • multiple endocrine neoplasia (MEN) type 1
More risk factors

Diagnostic investigations

1st investigations to order

  • fasting serum gastrin
  • acid output (basal and maximal)
  • secretin infusion test
  • calcium infusion test
More 1st investigations to order

Investigations to consider

  • upper gastrointestinal endoscopy
  • endoscopic ultrasound (EUS)
  • MRI liver
  • spiral CT liver
  • somatostatin receptor scintigraphy (SRS)
  • gallium(Ga)-68 DOTATATE
  • glucagon test
  • serum ghrelin
More investigations to consider

Treatment algorithm

ACUTE

localized disease: sporadic

localized disease: MEN1 syndrome

metastatic disease: predominantly hepatic

metastatic disease: extrahepatic

Contributors

Authors

Joseph R. Pisegna, MD

Professor of Medicine

Chief

Division of Gastroenterology, Hepatology, and Parenteral Nutrition

David Geffen School of Medicine at University of California

VA Greater Los Angeles Healthcare System

Department of Veterans Affairs

Los Angeles

CA

Disclosures

JRP is an author of a number of references cited in this topic.

Acknowledgements

Dr Joseph R. Pisegna would like to gratefully acknowledge Dr Deepinder Goyal, Dr Jihane Benhammou, Dr David Oh, and Dr Hank Wang, previous contributors to this topic. DG, JB, DO, and HW declare that they have no competing interests.

Peer reviewers

Peter Draganov, MD

Associate Professor of Medicine Division

Gastroenterology, Hepatology and Nutrition

University of Florida

Gainesville

FL

Disclosures

PD declares that he has no competing interests.

Petros Perros, BSc, MBBS, MD, FRCP

Consultant Endocrinologist

Freeman Hospital

Newcastle-Upon-Tyne Hospitals Trust

UK

Disclosures

PP declares that he has no competing interests.

  • Differentials

    • Atrophic gastritis
    • Peptic ulcer disease from Helicobacter pylori infection
    • Idiopathic gastric hypersecretion
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  • Guidelines

    • Informed consent for GI endoscopic procedures
    • Adverse events associated with EUS and EUS-guided procedures
    More Guidelines
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