Pastore G, Carli M, Lemerle J, Tournade M F, Voute P A, Rey A, Burgers J M, Zucker J M, Burger D, de Kraker J
International Society of Pediatric Oncology, Torino, Italy.
Med Pediatr Oncol. 1988;16(1):7-11. doi: 10.1002/mpo.2950160103.
This descriptive epidemiology study of 1,040 children with Wilms' tumor (WT) registered in the International Society of Paediatric Oncology (SIOP) clinical trials confirms the findings reported by the National Wilms' Tumor Study. The male:female rate was 0.89:1. The mean age at diagnosis of the 43 bilateral cases was significantly younger than children with unilateral renal involvement (32.4 vs 45 months). However, the mean ages of diagnosis for unilateral multicentric and for unicentric WT were very similar. On the other hand, the mean age at diagnosis of children with sporadic aniridia and hypospadias was younger than the mean age of patients with or without other congenital malformations. Thus aniridia as well as hypospadias could be indices of the first mutation, according to the Knudson and Stron hypothesis. WT was reported in two members of each of five families. However, these familial cases were comparable in terms of demographic and clinical features to the nonfamilial ones. These data suggest that the heritable fraction of WT is relatively small and that genetic and environmental factors interact in the development of WT.
这项对国际儿科肿瘤学会（SIOP）临床试验中登记的1040例肾母细胞瘤（WT）患儿进行的描述性流行病学研究证实了国家肾母细胞瘤研究报告的结果。男女比例为0.89:1。43例双侧病例的诊断平均年龄明显低于单侧肾脏受累患儿（32.4个月对45个月）。然而，单侧多中心和单中心WT的诊断平均年龄非常相似。另一方面，散发性无虹膜和尿道下裂患儿的诊断平均年龄低于有或无其他先天性畸形患者的平均年龄。因此，根据Knudson和Stron假说，无虹膜以及尿道下裂可能是首次突变的指标。五个家族中每个家族都有两名成员患WT。然而，这些家族性病例在人口统计学和临床特征方面与非家族性病例相当。这些数据表明，WT的遗传比例相对较小，并且遗传和环境因素在WT的发生发展中相互作用。
