Abstract
Familial adenomatosis polyposis (FAP) is one of two well described forms of hereditary colorectal cancer. The primary cause of death from this symdrome is colorectal caner which develops usually by the fifth decade of life. The overall survival of FAP was significantly improved by screening by genetic testing and endoscopy with prophylactic surgery. Almost all patients with FAP develop duodenal polyposis most of which occurs in periampullary. The second leading cause of death in FAP is duodenal adenocarcinoma. Between 5% and 10% of FAP patients die from duodenal cancer, usually periampullary in origin. Therefore, I will discuss the clinicopathological features, management and prevention of duodenal neoplasma in patients with FAP.
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