VIPoma

VIPomas are rare functional neuroendocrine tumours typically originating within the pancreas.

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

The clinical syndrome associated with VIPomas is sometimes referred to as WDHA syndrome (watery diarrhoea, hypokalaemia, and achlorhydria), Verner-Morrison syndrome, or pancreatic cholera.

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

Surgical resection is the standard treatment.

Symptoms are managed by hydration, electrolyte repletion, somatostatin analogues, and/or surgical debulking if metastatic.

VIPomas are rare functional neuroendocrine tumours, highly malignant, often presenting with regional lymph node or liver involvement at diagnosis. They are usually solitary tumours, with over 90% originating in 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 VIP, causing a clinical syndrome characterised by profuse watery diarrhoea, hypokalaemia, metabolic acidosis, and hypochlorhydria or achlorhydria.