Yu Kelly J, Hsu Wan-Lun, Chiang Chun-Ju, Cheng Yu-Juen, Pfeiffer Ruth M, Diehl Scott R, Goldstein Alisa M, Gravitt Patti E, Chen Chien-Jen, Hildesheim Allan
Division of Cancer Epidemiology and Genetics, National Cancer Institute, NIH, DHHS, Bethesda, MD 20852, USA.
Int J Cancer. 2009 Apr 1;124(7):1622-5. doi: 10.1002/ijc.24051.
Genetic and environmental factors have been implicated in the etiology of nasopharyngeal carcinoma (NPC), a tumor known to be closely associated with Epstein-Barr virus (EBV) infection. Studies have reported familial aggregation of NPC and have suggested the possible aggregation of NPC and other cancers. We evaluated familial aggregation of cancer in 358 high-risk families with two or more NPC cases enrolled in a NPC genetics study in Taiwan. Participants were linked to the Taiwan National Cancer Registry to identify incident cancers diagnosed after study enrollment (started in 1996) and before December 31, 2005, or death. In total, 2,870 individuals from the NPC Multiplex Family Study contributed 15,151 person-years over an average of 5.3 years of follow-up. One hundred ten incident cancers were identified. Multiple-primary standardized incidence ratios (MP-SIRs) were computed to evaluate overall cancer risk associated with infectious agents and with other tumors. The overall MP-SIR was 1.3 (95% CI: 1.1-1.6), which was largely explained by an excess in NPC (MP-SIR = 15; 95% CI: 10-23). Exclusion of incident NPC diagnoses led to an overall MP-SIR of 1.0 (95% CI: 0.83-1.3). Similarly, the observed excess risk of cancers associated with infectious agents (MP-SIR = 2.0; 95% CI: 1.5-2.6) was driven by the excess in NPC; exclusion of NPC cases led to a reduced MP-SIR that did not differ from 1.0. Analysis of the largest NPC multiplex family study to date confirms the presence of coaggregation of NPC within families in Taiwan but does not provide evidence for a broader familial syndrome involving NPC and other tumors.
遗传和环境因素被认为与鼻咽癌（NPC）的病因有关，鼻咽癌是一种已知与爱泼斯坦-巴尔病毒（EBV）感染密切相关的肿瘤。研究报告了鼻咽癌的家族聚集现象，并提示鼻咽癌与其他癌症可能存在聚集现象。我们评估了台湾一项鼻咽癌遗传学研究中358个有两例或更多鼻咽癌病例的高危家庭中癌症的家族聚集情况。研究参与者与台湾国家癌症登记处进行了关联，以确定在研究入组后（始于1996年）至2005年12月31日之前诊断出的新发癌症或死亡情况。总的来说，来自鼻咽癌多重家庭研究的2870名个体在平均5.3年的随访期间贡献了15151人年。共识别出110例新发癌症。计算了多原发标准化发病率比（MP-SIR），以评估与感染因子和其他肿瘤相关的总体癌症风险。总体MP-SIR为1.3（95%置信区间：1.1 - 1.6），这在很大程度上是由鼻咽癌的超额发病所致（MP-SIR = 15；95%置信区间：10 - 23）。排除新发鼻咽癌诊断后，总体MP-SIR为1.0（95%置信区间：0.83 - 1.3）。同样，观察到的与感染因子相关的癌症超额风险（MP-SIR = 2.0；95%置信区间：1.5 - 2.6）也是由鼻咽癌的超额发病驱动的；排除鼻咽癌病例后，MP-SIR降低，且与1.0无差异。对迄今为止最大规模的鼻咽癌多重家庭研究的分析证实了台湾家庭中鼻咽癌的共同聚集现象，但没有提供涉及鼻咽癌和其他肿瘤的更广泛家族综合征的证据。
