Zollinger-Ellison syndrome

Last reviewed: 28 Dec 2022
Last updated: 16 Mar 2022



History and exam

Key diagnostic factors

  • presence of risk factors
  • refractory or recurrent peptic ulcer disease
  • diarrhoea
More key diagnostic factors

Other diagnostic factors

  • epigastric abdominal pain
  • gastro-oesophageal reflux disease
  • steatorrhoea
  • 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


localized disease: sporadic

localized disease: MEN1 syndrome

metastatic disease: predominantly hepatic

metastatic disease: extrahepatic



Joseph R. Pisegna, MD

Professor of Medicine


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



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


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




PD declares that he has no competing interests.

Petros Perros, BSc, MBBS, MD, FRCP

Consultant Endocrinologist

Freeman Hospital

Newcastle-Upon-Tyne Hospitals Trust



PP declares that he has no competing interests.

  • Differentials

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

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

    Peptic ulcers


    More Patient leaflets
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