Preventative strategies for periampullary tumours in FAP

Abstract

Almost all patients with familial adenomatous polyposis develop duodenal polyps most of which occur in a cluster around the ampulla of Vater. Between 5 and 10% of FAP patients will die from upper gastrointestinal cancer, usually periampullary in origin. In an attempt to prevent cancer a screening programme has been developed using a well defined staging system to detect those patients most at risk of developing the disease. Treatment options are limited, with endoscopic clearance being contraindicated in most cases. The only certain method of preventing duodenal cancer is prophylactic radical surgery which has its own associated morbidity and mortality. Future developments may include new drug treatments or even gene therapy. Until then patients with FAP should all be considered for clinical trials as research continues.