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

다국어 초록 (Multilingual 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-year-old man with desmoid tumor and duodenal adenoma developing after total colectomy with ileostomy due fo FAP, and liferafures were reviewed.