VIPoma

Rare neuroendocrine tumour typically of the pancreas and with high malignant potential.

Tumour secretes vasoactive intestinal peptide (VIP).

Clinical syndrome of watery diarrhoea, hypokalaemia, acidosis, hypochlorhydria, and hypercalcaemia.

Usually with regional lymph node or liver involvement at diagnosis.

Elevated VIP levels are diagnostic of VIPoma.

Surgical resection is the treatment of choice. Symptoms may be controlled by hydration, electrolyte repletion, administration of octreotide, and/or surgical debulking if metastatic.

A rare neuroendocrine tumour that is highly malignant (usually with regional lymph node or liver involvement at diagnosis) and arises from within the pancreas over 90% of the time. The remaining 10% of cases arise in extrapancreatic locations, such as the retroperitoneum or thorax.[1]Soga J, Yakuwa Y. Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases. J Exp Clin Cancer Res. 1998;17:389-400. http://www.ncbi.nlm.nih.gov/pubmed/10089056?tool=bestpractice.com It secretes an active hormone called vasoactive intestinal peptide (VIP), which results in a clinical syndrome characterised by watery diarrhoea, hypokalaemia, acidosis, hypochlorhydria, and hypercalcaemia.