EPITHELIAL NEOPLASIA AND INTESTINAL MUCOS AL GANGLIONEUROMATOSIS WITHOUT MULTIPLE ENDOCRINE NEOPLASIA

The characteristic feature of intestinal ganglioneuromatosis is hyperp lasia of ganglionic cells, mainly in the Meissner and Auerbach plexuse s. Intestinal ganglioneuromatosis is one of the main components of mul tiple endocrine neoplasia Type IIb in which histological studies discl ose diffuse, transmural involvement of the gut wall. In contrast, when intestinal ganglioneuromatosis occurs in other disorders, such as Von Recklinghausen, disease and epithelial carcinomas, histologic anomali es are confined to the gut mucosa. In a 32 year old female with a hist ory of multinodular thyroid goiter, mucosal ganglioneuromatosis of the colon was found upon examination of endoscopic biopsy specimens. Othe r manifestations included vascular malformations, pancreatic lipomatos is, and bilateral ductal adenocarcinoma of the breasts. This case cann ot be classified as Nem Type IIb because of the absence of endocrine d isease.