Occult ganglioneuroma with diarrhea: localization by venous catecholamines

Neural crest tumors can be complicated by secretory diarrhea mediated by vasoactive intestinal peptide (VIP). An eight-month-old male with a several-month history of secretory diarrhea is described. Elevated urine vanillylmandelic acid (VMA), total urine catecholamines, and plasma VIP indicated that a neural crest tumor was responsible for his protracted diarrhea. An extensive search for the tumor including CT scans of his head, neck, thorax, abdomen, and pelvis was unrevealing. A selective vena caval catheterization showed elevated catecholamines in a sample obtained above the renal veins. Subsequent laparotomy disclosed a benign ganglioneuroma arising from the left adrenal; the diarrhea resolved after its removal. Selective venous sampling proved useful in establishing the tumor's location where other techniques had been unsuccessful.