Vasoactive intestinal peptide secreting tumors of childhood
S. J. Kaplan, C. T. Holbrook, H. G. McDaniel, W. L. Buntain and W. M. Crist
A 2-year-old boy with failure to thrive, watery diarrhea, abdominal
distention, hypokalemia, metabolic acidosis, and episodes of hypertension
and sweating was found to have a calcified right lower quadrant mass. Blood
levels of vasoactive intestinal peptide (VIP) and norepinephrine (NE) were
elevated. Presurgical management with phenoxybenzamine hydrochloride and
metyrosine was associated with an absence of expected postoperative
hypotension, and resection of a benign ganglioneuroma resulted in prompt
relief of all symptoms and return to normal of VIP and NE levels. Evidence
supports the theory that VIP is the substance responsible for the diarrhea
that accompanies some neural crest tumors.
