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Davies, D.R., et al. (1995) Sever Gardner Syndrome in Families with Mutations Restricted to a Specific Region of the APC Gene. Am J of Hum Genet, 57, 1151-1158.

has been cited by the following article:

• TITLE: Prevention and Management of Periampullary Tumors in Familial Adenomatous Polyposis

  AUTHORS: Naoki Hashimoto

  KEYWORDS: Familial Adenomatous Polyposis (FAP), Duodenal Adenocarcinoma, Spigelman Classfication, Pancreas-Preserving Total Duodenectomy (PPTD)

  JOURNAL NAME: Open Access Library Journal, Vol.3 No.1, January 22, 2016

  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.