Breslow N, Beckwith J B, Ciol M, Sharples K
Department of Biostatistics, University of Washington, Seattle 98195.
Cancer Res. 1988 Mar 15;48(6):1653-7.
Median ages at diagnosis were 36.5 and 42.5 mo for 1523 males and 1678 females with unilateral Wilms' tumor registered between October 1969 and December 1985; they were 23.5 mo and 30.5 mo for 100 males and 141 females with bilateral disease. The median age for multicentric, unilateral cases was intermediate between the bilateral and unicentric medians. Patients with hemihypertrophy in addition to their Wilms' tumor had a typical age distribution, whereas those with aniridia or characteristic genitourinary anomalies were substantially younger. Patients with perilobar nephroblastomatosis had a median age of 35.5 mo and those with intralobar nephroblastomatosis, a median age of 18.5 mo. Most of the bilateral disease occurred in the presence of one or both of these precursor lesions. These findings suggest heterogeneity in the pathogenesis of Wilms' tumor, having implications for genetic counselling, and call into question certain aspects of Knudson and Strong's two-stage mutational model for the origin of Wilms' tumor.
1969年10月至1985年12月登记的1523例单侧肾母细胞瘤男性患者和1678例女性患者的诊断中位年龄分别为36.5个月和42.5个月；100例双侧肾母细胞瘤男性患者和141例女性患者的诊断中位年龄分别为23.5个月和30.5个月。多中心单侧病例的中位年龄介于双侧和单中心病例的中位年龄之间。除肾母细胞瘤外还患有半侧肥大的患者具有典型的年龄分布，而患有无虹膜或特征性泌尿生殖系统异常的患者则明显年轻。叶旁肾母细胞瘤患者的中位年龄为35.5个月，叶内肾母细胞瘤患者的中位年龄为18.5个月。大多数双侧肾母细胞瘤病例是在存在一种或两种这些前驱病变的情况下发生的。这些发现提示肾母细胞瘤发病机制存在异质性，对遗传咨询有影响，并对Knudson和Strong提出的肾母细胞瘤起源的两阶段突变模型的某些方面提出了质疑。
