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
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
Disclosures
PP declares that he has no competing interests.
References
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 to users with access to all of BMJ Best Practice.
Differentials
- Atrophic gastritis
- Peptic ulcer disease from Helicobacter pylori infection
- Idiopathic gastric hypersecretion
More DifferentialsGuidelines
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- Clinical practice update on management of subepithelial lesions encountered during routine endoscopy: expert review
More GuidelinesPatient information
Pancreatic cancer
Peptic ulcers
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