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泌尿系统癌症的家族风险:临床咨询数据。

Familial risk of urological cancers: data for clinical counseling.

作者信息

Hemminki Kari, Li Xinjun, Czene Kamila

机构信息

Division of Molecular Genetic Epidemiology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 580, 69120 Heidelberg, Germany.

出版信息

World J Urol. 2004 Feb;21(6):377-81. doi: 10.1007/s00345-003-0375-7. Epub 2003 Nov 13.

Abstract

Familial risks for cancer are important for clinical counseling and understanding cancer etiology. The nationwide Swedish Family-Cancer Database includes all Swedes born in 1932 and later (0 to 68-year-old offspring) with their parents, totaling over 10.2 million individuals. Urological cancer cases were retrieved from the Swedish Cancer Registry up to year 2000. Standardized incidence ratios (SIR) and 95% confidence limits (CI) were calculated for age-specific familial risk in offspring by an exact proband status. The familial risks for offspring cancer were increased at all urological sites from concordant cancer in the parent and in a sibling proband. The highest SIRs by parent were for testicular and prostate cancer (4.26 and 2.45). When a sibling was affected, even kidney cancer (4.74) showed a high SIR. For kidney cancers, and also for prostate and testicular cancers, the SIRs were higher among siblings than among offspring and parents, which may indicate the involvement of recessive effects. Family members of patients with prostate cancer or von Hippel Lindau disease can expect organized clinical counseling, but family members of patients with other urological cancers are probably not counseled. Guidelines for clinical counseling or action level should be developed for all urological cancers because of the established familial risks. Urological cancers also offer a challenge to molecular geneticists attempting to identify the susceptibility genes underlying the familial clustering.

摘要

癌症的家族风险对于临床咨询和理解癌症病因非常重要。瑞典全国性的家庭癌症数据库涵盖了所有1932年及以后出生的瑞典人(0至68岁的后代)及其父母,总计超过1020万人。截至2000年,泌尿系统癌症病例从瑞典癌症登记处获取。通过精确的先证者状态计算后代特定年龄家族风险的标准化发病率(SIR)和95%置信区间(CI)。父母和同胞先证者中存在一致癌症时,后代患癌症的家族风险在所有泌尿系统部位均增加。父母引发的最高SIRs见于睾丸癌和前列腺癌(分别为4.26和2.45)。当同胞患病时,甚至肾癌(4.74)也显示出高SIR。对于肾癌以及前列腺癌和睾丸癌,同胞中的SIRs高于后代和父母,这可能表明存在隐性效应。前列腺癌或冯·希佩尔-林道病患者的家庭成员可获得有组织的临床咨询,但其他泌尿系统癌症患者的家庭成员可能未得到咨询。鉴于已确定的家族风险,应为所有泌尿系统癌症制定临床咨询或行动水平指南。泌尿系统癌症也给试图识别家族聚集背后易感基因的分子遗传学家带来了挑战。

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