Iwama Takeo, Tamura Kazuo, Morita Takayuki, Hirai Takashi, Hasegawa Hirotoshi, Koizumi Koichi, Shirouzu Kazuo, Sugihara Kenichi, Yamamura Takehira, Muto Tetsuichiro, Utsunomiya Joji
Department of Surgery, Kyoundo Hospital, Sasaki Institute, 1-8 Kanda-Surugadai, Chiyoda-ku, 101-0062, Tokyo, Japan.
Int J Clin Oncol. 2004 Aug;9(4):308-16. doi: 10.1007/s10147-004-0414-4.
The clinical situation of familial adenomatous polyposis (FAP) in Japan has changed in the period since the last analysis of data of the Japanese Polyposis Center. To reevaluate our data and elucidate the changes we analyzed the records of the 1390 FAP patients in 900 families registered with the Polyposis Committee of the Japanese Society for Cancer of the Colon and Rectum. In the 13-year period 1990-2003, clinical differences between men and women with FAP diminished. The postoperative prognosis was substantially better in patients without advanced colorectal cancer (stage > or = T2) than in those with early cancer or no cancer. Mean age at death improved from 42.5 years in the period before 1990 to 46.0 years, and it was a result of a decreased proportion of deaths from colorectal cancer. The distribution of colorectal cancer in FAP patients was similar to that in the general population. Desmoid tumors accounted for about 10% of deaths in the recent 13 years (1990-2003). The cumulative risk of rectal cancer in the preserved rectum was 12% at 10 years and 23% at 15 years. The registry system in Japan revealed a new clinical situation in FAP patients, and the findings of this study will be useful to improve the prognosis of patients with FAP.
自日本息肉病中心上次进行数据分析以来，日本家族性腺瘤性息肉病（FAP）的临床情况发生了变化。为了重新评估我们的数据并阐明这些变化，我们分析了日本结直肠癌学会息肉病委员会登记的900个家庭中1390例FAP患者的记录。在1990年至2003年的13年期间，FAP男性和女性之间的临床差异减小。无进展期结直肠癌（分期≥T2）患者的术后预后明显好于早期癌症或无癌症患者。平均死亡年龄从1990年以前的42.5岁提高到46.0岁，这是结直肠癌死亡比例下降的结果。FAP患者中结直肠癌的分布与普通人群相似。在最近13年（1990 - 2003年），硬纤维瘤约占死亡人数的10%。保留直肠中直肠癌的累积风险在10年时为12%，在15年时为23%。日本的登记系统揭示了FAP患者的新临床情况，本研究结果将有助于改善FAP患者的预后。
