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.