Summary
Definition
History and exam
Key diagnostic factors
- profuse watery diarrhea
Other diagnostic factors
- age around 50 years
- headache
- weight loss
- poor skin turgor
- dry mucus membranes
- muscle weakness
- muscle cramps
- flushing
- hepatomegaly
- history of multiple endocrine neoplasia type 1 (MEN1)
Risk factors
- multiple endocrine neoplasia type 1 (MEN1)
- age around 50 years
Diagnostic tests
1st tests to order
- serum potassium
- serum bicarbonate
- serum calcium
- serum glucose
- vasoactive intestinal peptide (VIP) radioimmunoassay
- liver function tests
Tests 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
- somatostatin receptor scintigraphy
- endoscopic ultrasound
- abdominal ultrasound
- operative exploration
- intraoperative ultrasound
Treatment algorithm
initial presentation of symptomatic patient
localized disease
metastatic disease: surgical candidate
metastatic disease: nonsurgical 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
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.
Christopher Wolfgang, MD, PhD, FACS
Assistant Professor of Surgery and Oncology
Cameron Division of Surgical Oncology
The Sol Goldman Pancreatic Cancer Research Center
Johns Hopkins University
Baltimore
MD
Disclosures
CW 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
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
Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Full text Abstract
Falconi M, Eriksson B, Kaltsas G, et al; Vienna Consensus Conference participants. 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
Pavel M, O'Toole D, Costa F, et al; Vienna Consensus Conference participants. ENETS consensus guidelines update for the management of distant metastatic disease of intestinal, pancreatic, bronchial neuroendocrine neoplasms (NEN) and NEN of unknown primary site. Neuroendocrinology. 2016;103(2):172-85. 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 diarrhea
- 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
Diarrhea in adults
Diarrhea in children
More Patient information
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