<unk>  syndrome consists of adenomatous polyps of the gastrointestinal tract,<unk>  fibromas, desmoid tumors, osteomas, epidermoid cysts, lipomas, dental abnormalities, and periampullary carcinomas. The incidence of the syndrome is 1:14,025 with an equal sex distribution. It is determined by the autosomal dominant familial polyposis coli gene (APC) on chromosome 5.<unk>  syndrome can be identified based on oral findings, including multiple impacted and supernumerary teeth, multiple jaw osteomas that give a "cotton-wool" appearance to the jaws, as well as multiple odontomas, congenital hypertrophy of the retinal pigment epithelium (CHRPE), in addition to multiple adenomatous polyps of the colon.<unk>  syndrome is also associated with familial adenomatous polyposis and may manifest as aggressive fibromatosis (desmoid tumors) of the retroperitoneum. Desmoid tumors arise most frequently from the aponeurosis of the rectus abdominal muscle of multiparous women. The extra-abdominal form is rare and desmoids of the breast may arise in the mammary gland or may occur as an extension of a lesion arising from the muscles of the chest wall. The incidence of mammary desmoid tumors is less than 0.2% of primary breast neoplasms. In<unk> , the incidence ranges from 4% to 17%. Desmoid tumors associated with<unk>  have been shown to have an alteration of the β-catenin pathway and over express β-catenin.
Gardner's syndrome
Meige's syndrome
Wissler's syndrome
Zieve's syndrome
Satchmo's syndrome
Löffler's syndrome
Grinspan's syndrome
Meigs's syndrome