Furukawa, Toru    Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine    Position   Visiting Professor
Article types	Review article
Language	English
Peer review	Peer reviewed
Presence of invitation	Invited paper
Title	Familial pancreatic cancer: Concept, management and issues.
Journal	Formal name：World Journal of Gastroenterology Abbreviation：World J Gastroenterol ISSN code：2219-2840/10079327
Domestic / Foregin	Foregin
Volume, Issue, Page	23(6),pp.935-948
Author and coauthor	Matsubayashi Hiroyuki†*, Takaori Kyoichi, Morizane Chigusa, Maguchi Hiroyuki, Mizuma Masamichi, Takahashi Hideaki, Wada Keita, Hosoi Hiroko, Yachida Shinichi, Suzuki Masami, Usui Risa, Furukawa Toru, Furuse Junji, Sato Takamitsu, Ueno Makoto, Kiyozumi Yoshimi, Hijioka Susumu, Mizuno Nobumasa, Terashima Takeshi, Mizumoto Masaki, Kodama Yuzo, Torishima Masako, Kawaguchi Takahisa, Ashida Reiko, Kitano Masayuki, Hanada Keiji, Furukawa Masayuki, Kawabe Ken, Majima Yoshiyuki, Shimosegawa Toru
Publication date	2017/02
Summary	Familial pancreatic cancer (FPC) is broadly defined as two first-degree-relatives with pancreatic cancer (PC) and accounts for 4%-10% of PC. Several genetic syndromes, including Peutz-Jeghers syndrome, hereditary pancreatitis, hereditary breast-ovarian cancer syndrome (HBOC), Lynch syndrome, and familial adenomatous polyposis (FAP), also have increased risks of PC, but the narrowest definition of FPC excludes these known syndromes. When compared with other familial tumors, proven genetic alterations are limited to a small proportion (< 20%) and the familial aggregation is usually modest. However, an ethnic deviation (Ashkenazi Jewish > Caucasian) and a younger onset are common also in FPC. In European countries, "anticipation" is reported in FPC families, as with other hereditary syndromes; a trend toward younger age and worse prognosis is recognized in the late years. The resected pancreases of FPC kindred often show multiple pancreatic intraepithelial neoplasia (PanIN) foci, with various K-ras mutations, similar to colorectal polyposis seen in the FAP patients. As with HBOC patients, a patient who is a BRCA mutation carrier with unresectable pancreatic cancer (accounting for 0%-19% of FPC patients) demonstrated better outcome following platinum and Poly (ADP-ribose) polymerase inhibitor treatment. Western countries have established FPC registries since the 1990s and several surveillance projects for high-risk individuals are now ongoing to detect early PCs. Improvement in lifestyle habits, including non-smoking, is recommended for individuals at risk. In Japan, the FPC study group was initiated in 2013 and the Japanese FPC registry was established in 2014 by the Japan Pancreas Society.
DOI	10.3748/wjg.v23.i6.935
PMID	28246467