When should neuroendocrine tumors be suspected as a cause of chronic secretory diarrhea

When should neuroendocrine tumors be suspected as a cause of chronic secretory diarrhea? 

Neuroendocrine tumors are uncommon causes of chronic secretory diarrhea. For example, one VIPoma might be expected per 10 million people per year.

The below table lists these tumors and their markers. Because of the rarity of these tumors as a cause for chronic diarrhea, other causes of secretory diarrhea should be considered first.

If tumor is visualized by CT scan or if systemic symptoms (e.g., flushing) are present, evaluation for neuroendocrine tumors may have a better yield. Blanket testing for tumor-associated peptides is likely to yield many more false-positives than true-positives and therefore can be very misleading.

Neuroendocrine Tumors Causing Chronic Diarrhea and Their Markers

Typical SymptomsTumorMediator and Tumor Marker
GastrinomaZöllinger-Ellison syndrome: pancreatic or duodenal tumor, peptic ulcer, steatorrhea, diarrheaGastrin
VIPomaVerner-Morrison syndrome: watery diarrhea, hypokalemia, achlorhydria, flushingVasoactive intestinal polypeptide
Medullary thyroid carcinomaThyroid mass, hypermotilityCalcitonin, prostaglandins
PheochromocytomaAdrenal mass, hypertensionVasoactive intestinal polypeptide, norepinephrine, epinephrine
CarcinoidFlushing, wheezing, right-sided cardiac valvular diseaseSerotonin, kinins
SomatostatinomaNonketotic diabetes mellitus, steatorrhea, diabetes, gallstonesSomatostatin
GlucagonomaSkin rash (migratory necrotizing erythema), mild diabetesGlucagon
MastocytosisFlushing, dermatographism, nausea, vomiting, abdominal painHistamine

VIPoma, Vasoactive intestinal polypeptide tumor.


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