Mesenteric Fibromatosis in Gardner Syndrome

Gardner syndrome is an autosomal dominant disorder characterized by intestinal polyposis, osteomas of mandible and skull, epidermoid cysts, and mesenteric fibromatosis (desmoid tumor; shown here). Both copies of APC gene located on 5q must be functionally inactivated for the manifestations to occur. APC is a negative regulator of beta-catenin in WNT signaling pathway.