Summary
Definition
History and exam
Key diagnostic factors
- profuse watery diarrhoea
Other diagnostic factors
- age around 50 years
- headache
- weight loss
- poor skin turgor
- dry mucous membranes
- muscle weakness
- muscle cramps
- gastrointestinal symptoms
- hyperglycaemia symptoms
- flushing
- hepatomegaly
- history of multiple endocrine neoplasia type 1 (MEN1)
Risk factors
- multiple endocrine neoplasia type 1 (MEN1)
- age around 50 years
Diagnostic investigations
1st investigations to order
- serum potassium
- serum bicarbonate
- serum calcium
- serum glucose
- vasoactive intestinal peptide (VIP) radioimmunoassay
- liver function tests
Investigations to consider
- arterial blood gas analysis
- gastric pH monitoring
- chromogranin A
- pancreatic polypeptide
- contrast-enhanced CT scan of abdomen
- contrast-enhanced MRI of abdomen
- somatostatin receptor PET-CT/MRI
- somatostatin receptor scintigraphy
- endoscopic ultrasound
- operative exploration
- intraoperative ultrasound
- biopsy
Treatment algorithm
initial presentation of symptomatic patient
localised disease
metastatic disease: surgical candidate
metastatic disease: non-surgical candidate
refractory disease
Contributors
Authors
Harish Lavu, MD, FACS
Professor of Surgery
Sidney Kimmel Medical College
Thomas Jefferson University
Philadelphia
PA
Disclosures
HL declares that he has no competing interests.
Charles J. Yeo, MD, FACS
The Samuel D. Gross Professor and Chair
Department of Surgery
Sidney Kimmel Medical College
Thomas Jefferson University
Philadelphia
PA
Disclosures
CJY declares that he has no competing interests.
Peer reviewers
Alexandria Phan, MD
Professor of Medicine
Froedtert Hospital and the Medical College of Wisconsin
Milwaukee
WI
Disclosures
AP declares that she has no competing interests.
Jonathan Strosberg, MD
Professor
Moffitt Cancer Center
Tampa
FL
Disclosures
JS has consulted for Novartis and serves on the Speakers’ Bureau for Ipsen.
Jaime Ruiz-Tovar, MD
Fellow of the Spanish Association of Surgeons (AEC)
Department of Surgery
University Hospital Ramon y Cajal
Madrid
Spain
Disclosures
JRT declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text
Hofland J, Falconi M, Christ E, et al. European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes. J Neuroendocrinol. 2023 Aug;35(8):e13318.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
Del Rivero J, Perez K, Kennedy EB, et al. Systemic therapy for tumor control in metastatic well-differentiated gastroenteropancreatic neuroendocrine tumors: ASCO guideline. J Clin Oncol. 2023 Nov 10;41(32):5049-67.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
- Infectious gastroenteritis
- Osmotic diarrhoea
- Ileal bile acid malabsorption
More DifferentialsGuidelines
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
More GuidelinesPatient information
Diarrhoea in children
More Patient information
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