Introduction

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 15 years of age and it is the most common renal malignancy in patients between the ages of 15 and 21 years.

There is increasing data which demonstrate that pediatric RCC is a distinct disease process from adult RCC [1][2][3][4][5]. 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 papillary subtype compared to adults. Genetic translocations are seen very commonly in pediatric RCC leading to their description as translocation carcinomas. Lymph node metastases appear to be of less clinical significance in childhood cases as opposed to adult RCC. Despite these differences, the treatment remains largely the same as both adult and pediatric RCC do not respond well 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