Histologically the tumor consists of high nuclear grade, clear cell type, renal cell carcinoma. In review of 20 cases of pediatric RCC, median age was 11.8 years and 10 patients died of distant metastases within a year of diagnosis. Only 10 patients were disease-free at a median of 4 years. Most RCC is associated with a terminal deletion of the short arm of chromosome 3. Papillary carcinomas are associated with trisomy 7 and 17.
Smoking is a predisposing factor.
These changes are reversible following nephrectomy. Seventy percent of RCC is of the clear cell variety. Caution is advised. Clear cell carcinoma tends to be of low grade. Murphy, W., et al.