Korean Journal of Gastrointestinal Endoscopy 2001;23(1): 32-35.
가족성 선종성 용종증 수술 후 발생한 유건종과 십이지장 선종 1 예 ( Desmoid Tumor and Duodenal Adenoma in a Patient with Familial Adenomatous Polyposis : A Case Report )
박병헌(Byeong Heon Park),김효종(Hyo Jong Kim),장영운(Young Woon Chang),김경진(Kyeong Jin Kim),이동근(Dong Keun Lee),동석호(Seok Ho Dong),김병호(Byung Ho Kim),이정일(Joung Il Lee),장린(Rin Chang)
Abstract
Familial adenomatous polyposis (FAP) is an autosomal dominantly inherited disorder due to germline mutation of the tumor suppressor adenomatous polyposis coli (APC) gene, Multiple large bowel polyps usually develop in adolescenoe or early adulthood with inevitable progression to colorectal carcinoma. It is well known that patients with FAP are at considerable risk of developing extracolonic manisfestations of the disease. Particularly, desmoid tunors of the abdominal cavity, and duodenal adenomas and carcinomas are the most serious ones. Desmoid tumors and duodenal carcinomas are major muses of death in those patients in whom a prophylactic (procto) colectomy has been performed. We report the case of a 38-yeaz-old man with desmoid tumor and duodenal adenoma developing after total colectomy with ileostomy due fo FAP, and 1iferafures were reviewed. (Korean J Gastroietest Endosc 2001;23:32-35)