Diarrhée chronique par ganglioneuroblastome (GNB) sécrétant du VIP chez l'enfant. A propos d'un cas avec revue de la littérature.

A literature review was conducted in relation to a case of chronic diarrhea associated with a VIP (vasoactive intestinal polypeptide) producing ganglioneuroblastoma (GNB), in an 18-month old female baby. This is a rare entity characterized by premonitory, persisting diarrhea, causing fluid and electrolyte changes typical of the WDHA syndrome, associating watery diarrhea, hypokalemia, and achlorhydia. Elevated VIP plasma levels are an indication for an echographic and/or CT-scan search for the causal secreting tumor. Although the prognosis of this condition seems favorable, the recommended treatment is surgery. The VIP substance represents an excellent biological monitoring marker. Ganglioneuroblastomas are tumors of the sympathetic nervous system, which, according to Pearse's cell and embryologic theory (1966), have to be linked to the APUD system tumors (paraneuromas). VIP-producing forms are rare in children, and only 29 case studies have been compiled in the literature since 1970, when the VIP substance was discovered. The case reported in this study illustrates the diagnostic problems raised by such lesions, and allows us to confirm VIP's imputability for the occurrence of the chronic diarrhea condition in this child.