Desmoid Tumor and Duodenal Adenoma in a Patient with Familial Adenomatous Polyposis: A Case Report / 대한소화기내시경학회지

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 adolescence 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 tumors of the abdominal cavity, and duodenal adenomas and carcinomas are the most serious ones. Desmoid tumors and duodenal carcinomas are major causes 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 to FAP, and literatures were reviewed.