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Last reviewed: 24 Apr 2025
Last updated: 22 May 2025

Summary

Definition

History and exam

Key diagnostic factors

  • refractory or recurrent peptic ulcer disease
  • diarrhea

Other diagnostic factors

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

Risk factors

  • multiple endocrine neoplasia type 1 (MEN1)

Diagnostic tests

1st tests to order

  • fasting serum gastrin
  • gastric pH

Tests 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)

Treatment algorithm

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

Disclosures

PP declares that he has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

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.Full text  Abstract

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

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.Full text  Abstract

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.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available here.

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