VIPoma: Mechanisms, clinical presentation, diagnosis and treatment (Review)

Abstract

Vasoactive intestinal peptide (VIP) secreting tumour (VIPoma) is a rare neuroendocrine tumour that most often originates from pancreatic islet cells and affects one in ten million individuals per year. In adults, it develops most commonly in the fortieth year of life with a sparse female predominance. Excessive VIP secretion induces refractory watery diarrhoea, hypokalemia and achlorhydria. Other symptoms include hyperglycemia (20-50%), hypercalcaemia (25-50%), hypochlorhydria (20-50%) and flushing (15-30%). VIP plasma levels are increased in almost all patients with VIPoma, and, together with profusing secretory diarrhoea, it is sufficient to establish the diagnosis. Moreover, the majority of VIPomas are malignant or have already metastasised at the time of diagnosis. The treatment of the neoplasm comprises medical management and surgery. While surgery remains the gold standard of treatment, peptide receptor radionuclide therapy represents one of the most effective and well-tolerated treatment options. The average survival rate of patients with VIPoma is 96 months. The objective of this review was to summarise all features of pancreatic VIPoma, as well as present novel treatment approaches for this rare neoplasm.