Watery diarrhoea and ganglioneuroma with secretion of vasoactive intestinal peptide.

A young girl presenting with diarrhoea and stridor was found to have a thoracic ganglioneuroma. The tumour, which was partially resected, contained an abnormally high amount of vasoactive intestinal peptide (VIP) as measured by radioimmunoassay. This decreased after the operation. In addition, analysis of urine showed the presence of abnormal amounts of catecholamines and catechol metabolites. The findings suggest that the diarrhoea in the syndrome of ganglioneuroma and bowel dysfunction is due to an excess of VIP secreted by the tumour.