Olson J M, Breslow N E, Barce J
Department of Biostatistics, University of Washington, Seattle 98195.
Am J Med Genet. 1993 Aug 1;47(1):91-4. doi: 10.1002/ajmg.1320470119.
We interviewed families of 71 patients registered in the National Wilms Tumor Study and identified as having a twin sibling. Questions concerning zygosity and the occurrence of congenital anomalies and other forms of cancer in the twins were asked. Of the 71 twin pairs, 35 were dizygotic, 31 were monozygotic, and 5 were of unknown zygosity. The only pair concordant for Wilms tumor was dizygotic, leading to a heritability estimate of zero. In a monozygotic pair, one twin was diagnosed with Wilms tumor and the other with medulloblastoma. The estimated relative risk of Wilms tumor and childhood cancer in the co-twin was 250 times and 10 times the population rate, respectively. Four discordant pairs had a family history of Wilms tumor, suggesting that the penetrance of the condition is not complete. Because of the small sample size, caution should be exercised in the interpretation of these results.
我们采访了在国家肾母细胞瘤研究中登记的71名患者的家属，这些患者被确定有一个双胞胎兄弟姐妹。我们询问了有关双胞胎的合子性以及先天性异常和其他癌症形式的发生情况。在这71对双胞胎中，35对是异卵双生，31对是同卵双生，5对合子性未知。唯一一对患肾母细胞瘤一致的双胞胎是异卵双生，导致遗传度估计为零。在一对同卵双胞胎中，一个被诊断患有肾母细胞瘤，另一个患有髓母细胞瘤。双胞胎中患肾母细胞瘤和儿童期癌症的估计相对风险分别是人群发病率的250倍和10倍。四对不一致的双胞胎有肾母细胞瘤家族史，这表明该病的外显率不完全。由于样本量小，对这些结果的解释应谨慎。
