<prompt> Familial adenomatous polyposis is characterized by all exceptAutosomal recessive transmissionMore than 100 polyps in colorectal areaPolyps seen in duodenumExtra-intestinal manifestations are present  <response>: Familial adenomatous polyposis FAP Is a rare, inherited condition caused by a defect in the adenomatous polyposis coli APC gene - located in 5q 21 chromosome. FAP is autosomal dominant- 50 chance of inheritance in family members Has 100 adenomatous polyps 100 RISK of malignancy Polyp formation - by 15 yrs. 100 develop malignancy - by 40 yrs. TOC: Total proctocolectomy ileal pouch-anal anastomosis TPC IPAA MCC of death after TPC Peri-ampullary Ca polyps in duodenum - periampullary region Two variants of FAP Gardners syndrome Osteoma mandible Congenital hyperophy of the retinal pigmented epithelium CHRPE lesions Sebaceous cysts Benign lymphoid polyposis of ileum Desmoid tumors Supernumerary teeth Turcots syndrome: Brain tumors Medulloblastoma - mc associated with FAP Glioblastoma multiforme - mc associated with HNPCC 