A Case of Intra-abdominal Desmoid Tumor after Total Colectomy in a Patient with Familial Adenomatous Polyposis / 대한소화기내시경학회지

ABSTRACT

Familial adenomatous polyposis (FAP) arises from germline mutations of the adenomatous polyposis coli (APC) gene. FAP is characterized by the occurrence of hundreds to thousands of adenomas throughout the colorectum, and there is nearly a 100% risk of colorectal cancer. In addition to polyposis coli, patients with FAP can develop a variety of extracolonic manifestations. Recent advances in screening and surgery have reduced the colon cancer occurrence and death in FAP patients, leaving desmoid tumors as a leading cause of their morbidity and mortality. Treatment of desmoid tumors is generally considered to be challenging for both the doctor and the patient. We report here on an 18 year old man with resectable intra-abdominal desmoid tumor that developed after total colectomy due to FAP and we include a review of the relevant literature.