Ganglioneuromatous polyp

The unique familial occurrence of polypoid intestinal ganglioneuromatosis of the large bowel is reported. The lesions affected a 38-year-old father and three children aged 14, 12, and 10 years. In these cases, extensive diffuse and polypoid proliferation of nerve fibers and ganglion cells within the colonic mucosa was associated with pronounced epithelial changes resembling juvenile polyposis. None of the patients had any clinical evidence of the mucosal neuroma syndrome or Von Recklinghausen's neurofibromatosis, conditions in which intestinal ganglioneuromatosis can occur...

A case of nonfamilial juvenile polyposis coli was associated ganglioneuromatous proliferation in the polyps is described. The ganglioneuromatous proliferation was characterized by clusters of mature ganglion cells and nerve fiber bundles in the lamina propria and submucosa of the juvenile polyps. The patient had no history of von Recklinghausen's disease or multiple endocrine neoplasia syndrome, type 2b. The implications of this peculiar finding are discussed and the literature is reviewed.