- 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.
Last updated: May 08, 2013