New Diagnostic Test for Neural Tumors of Infancy: Increased Urinary Excretion of 3-Methoxy-4-hydroxymandelic Acid and Norepinephrine in Ganglioneuroma with Chronic Diarrhea

Abstract

Green et al. (Pediatrics, 23:951, 1959) reported the occurrence of chronic diarrhea with abdominal distension and wasting as presenting symptoms in three children who had either a ganglioneuroma or ganglioneuroblastoma. Since all symptoms abated after removal of the tumor, they postulated the secretion of a chemical substance by the tumor, affecting gastrointestinal motility or water reabsorption or both. We have made studies in an additional child, age 2½ years, who manifested a similar syndrome with a ganglioneuroma. The findings in this patient indicate that the ganglioneuroma was a functioning endocrine tumor, involved in the overproduction of 3-methoxy-4-hydroxymandelic acid, norepinephrine and perhaps two other compounds of unknown structure.