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
- gastrointestinal symptoms
- hyperglycemia symptoms
- 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/MRI
- somatostatin receptor scintigraphy
- endoscopic ultrasound
- operative exploration
- intraoperative ultrasound
- biopsy
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
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.
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 children
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