VIPoma

VIPomas are rare functional neuroendocrine tumours that usually arise from within the pancreas.

VIPoma is characterised by profuse watery diarrhoea, hypokalaemia, metabolic acidosis, and hypochlorhydria or achlorhydria, in the presence of elevated serum vasoactive intestinal peptide (VIP).

VIPomas are highly malignant. Patients usually present with regional lymph node or liver involvement at diagnosis.

Surgical resection is the standard treatment for VIPoma.

Symptoms may be controlled by hydration, electrolyte repletion, administration of somatostatin analogues, and/or surgical debulking if metastatic.

VIPomas are rare functional neuroendocrine tumours that are highly malignant (usually with regional lymph node or liver involvement at diagnosis). They are usually solitary tumours. In most cases (>90%) they arise from within the pancreas. The remaining 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 Dec;17(4):389-400. http://www.ncbi.nlm.nih.gov/pubmed/10089056?tool=bestpractice.com

VIPomas secrete an active hormone called vasoactive intestinal peptide (VIP), which results in a clinical syndrome characterised by profuse watery diarrhoea, hypokalaemia, metabolic acidosis, and hypochlorhydria or achlorhydria.