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Zollinger-Ellison syndrome

Last reviewed: 23 Sep 2025
Last updated: 22 May 2025

Summary

Definition

History and exam

Key diagnostic factors

  • refractory or recurrent peptic ulcer disease
  • diarrhea
Full details

Other diagnostic factors

  • epigastric abdominal pain
  • gastroesophageal reflux disease
  • steatorrhea
  • gastrointestinal bleed
  • bone pain
Full details

Risk factors

  • multiple endocrine neoplasia type 1 (MEN1)
Full details

Diagnostic investigations

1st investigations to order

  • fasting serum gastrin
  • gastric pH
Full details

Investigations to consider

  • secretin stimulation test
  • calcium infusion test
  • glucagon test
  • abdominal CT scan
  • abdominal MRI scan
  • somatostatin receptor scintigraphy (SRS)
  • gallium(Ga)-68 DOTATATE PET
  • endoscopic ultrasound (EUS)
  • esophagogastroduodenoscopy (EGD)
  • chest CT
  • genetic testing for multiple endocrine neoplasia type 1 (MEN1)
Full details

Treatment algorithm

ACUTE

localized disease: sporadic

localized disease: MEN1

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. JRP has received a grant from Takeda Pharmaceuticals and is a speaker for Phathom Pharmaceuticals and AbbVie.

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.

Disclosures

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

Divulgaciones

PP declares that he has no competing interests.

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Referencias

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Artículos principales

Falconi M, Eriksson B, Kaltsas G, et al. ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology. 2016;103(2):153-71.Texto completo  Resumen

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Texto completo

Kunz PL, Reidy-Lagunes D, Anthony LB, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Texto completo  Resumen

Pavel M, Öberg K, Falconi M, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-60.Texto completo  Resumen

Artículos de referencia

Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
  • Diferenciales

    • Atrophic gastritis
    • Peptic ulcer disease from Helicobacter pylori infection
    • Idiopathic gastric hypersecretion
    Más Diferenciales
  • Guías de práctica clínica

    • NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
    • Clinical practice update on management of subepithelial lesions encountered during routine endoscopy: expert review
    Más Guías de práctica clínica
  • Folletos para el paciente

    Pancreatic cancer

    Peptic ulcers

    Más Folletos para el paciente
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