[Rectal ganglioneuromatosis and multiple type IIb endocrine neoplasia]

Intestinal tract ganglioneuromatosis was discovered in a 27-year old man through surgical biopsy specimens obtained after acute intestinal obstruction. The patient then developed type IIb endocrine neoplasia including a marfanoid morphotype, mucosal neuromatosis, metastatic medullary carcinoma of the thyroid and bilateral pheochromocytoma. The histological and clinical features, the pathophysiological mechanisms of intestinal ganglioneuromatosis and its place within the intricate group of neurocristopathies are discussed.