Li F P, Williams W R, Gimbrere K, Flamant F, Green D M, Meadows A T
Clinical Studies Section, National Cancer Institute, Bethesda, Maryland.
Pediatrics. 1988 Jan;81(1):147-9.
Heritability of the unilateral-sporadic (non-familial) form of Wilms tumor was examined in the offspring of 96 long-term survivors of the neoplasm. No Wilms tumor has developed in any of the 179 offspring of these patients. The maximum likelihood estimate of a hereditary Wilms tumor in our patients is zero and the corresponding 95% upper confidence limit ranges between 0.06 and 0.11, depending on penetrance. Among their offspring, the 95% upper bound of the risk of Wilms tumor is 0.02. These figures can be applied in genetic counseling of other survivors of unilateral-sporadic Wilms tumor.
在96名该肿瘤长期存活者的后代中，对单侧散发（非家族性）形式的肾母细胞瘤的遗传度进行了研究。这些患者的179名后代中均未发生肾母细胞瘤。我们患者中遗传性肾母细胞瘤的最大似然估计值为零，根据外显率，相应的95%置信上限在0.06至0.11之间。在他们的后代中，肾母细胞瘤风险的95%上限为0.02。这些数据可应用于其他单侧散发肾母细胞瘤存活者的遗传咨询。
