フルカワ　トオル   Furukawa Tooru   古川　徹    所属   医学部 医学科（東京女子医科大学病院）    職種   客員教授
論文種別	総説
言語種別	英語
査読の有無	査読あり
招待の有無	招待あり
表題	Familial pancreatic cancer: Concept, management and issues.
掲載誌名	正式名：World Journal of Gastroenterology 略　 称：World J Gastroenterol ISSNコード：2219-2840/10079327
掲載区分	国外
巻・号・頁	23(6),pp.935-948
著者・共著者	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
発行年月	2017/02
概要	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