林奇综合征日本家族中一级亲属的癌症死亡原因

Causes of Cancer Death Among First-Degree Relatives in Japanese Families with Lynch Syndrome.

作者信息

Tanakaya Kohji, Yamaguchi Tatsuro, Ishikawa Hideki, Hinoi Takao, Furukawa Yoichi, Hirata Keiji, Saida Yoshihisa, Shimokawa Mototsugu, Arai Masami, Matsubara Nagahide, Tomita Naohiro, Tamura Kazuo, Sugano Kokichi, Ishioka Chikashi, Yoshida Teruhiko, Ishida Hideyuki, Watanabe Toshiaki, Sugihara Kenichi

机构信息

Department of Surgery, Iwakuni Clinical Center, Iwakuni, Japan.

Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan.

出版信息

Anticancer Res. 2016 Apr;36(4):1985-9.

PMID:27069191

Abstract

AIM

To elucidate the causes of cancer death in Japanese families with Lynch syndrome (LS).

METHODS

The distributions of cancer deaths in 485 individuals from 67 families with LS (35, 30, and two families with MutL homologue 1 (MLH1), MSH2, and MSH6 gene mutations, respectively), obtained from the Registry of the Japanese Society for Cancer of the Colon and Rectum were analyzed.

RESULTS

Among 98 cancer deaths of first-degree relatives of unknown mutation status, 53%, 19%, 13% (among females), 7% (among females) and 5% were due to colorectal, gastric, uterine, ovarian, and hepatobiliary cancer, respectively. The proportion of deaths from extra-colonic cancer was significantly higher in families with MSH2 mutation than in those with MLH1 mutation (p=0.003).

CONCLUSION

In addition to colonic and uterine cancer, management and surveillance targeting gastric, ovarian and hepatobiliary cancer are considered important for Japanese families with LS. Extra-colonic cancer in families with MSH2 mutation might require for more intensive surveillance.

摘要