Upper gastrointestinal cancer risk in familial adenomatous polyposis.

Familial adenomatous polyposis has a known risk of colorectal carcinoma. Less well recognized, however, is the association with duodenal polyps and their subsequent malignant degeneration. While frequent endoscopic surveillance is acceptable, a case of early malignant transformation is presented, and the argument for prophylactic preemptive pancreaticoduodenectomy is made.