Renal cell carcinoma (RCC) is a predominantly adult cancer with a much lower incidence in children. Its incidence increases with advancing age and for pediatric cases the median age at diagnosis is nine years. The incidence approaches that of Wilms tumor at 13 years of age and it is the most common renal malignancy in adolescent and young adult patients [1].
There is increasing data which demonstrate that pediatric RCC is a distinct disease process from adult RCC [2][3][4][5][6]. Histologically, pediatric RCC often demonstrates heterogeneous features that do not allow it to be categorized into one of the typical adult subtypes. When it can be classified, a larger percentage of pediatric RCC are consistent with the translocation (TFE+) or papillary subtype compared to adults [7][8][9]. Genetic translocations are seen very commonly in pediatric RCC leading to their description as translocation carcinomas. Lymph node metastases appear to be of more common in childhood cases, even in small primary tumors, as opposed to adult RCC. Despite these differences, the treatment remains largely the same as both adult and pediatric RCC do not respond to chemotherapy or radiation. Complete surgical resection is the key element of treatment.
Content in this topic is referenced in SCORE Wilms Tumor, Renal Cell Carcinoma, and Hemihypertrophy overview
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