Pediatric and adolescent renal cell carcinoma

•We review the current body of literature pertaining to renal cell carcinoma (RCC) among pediatric populations.•Increasing evidence that pediatric RCC differs from adult RCC, and that these differences may be of clinical significance.•Increasing evidence that most cases of pediatric RCC, but only a small minority of adult RCC, are made of translocation type tumors.•Despite possible differences, pediatric RCC continues to be managed in much the same way as adult RCC.

ObjectiveRenal cell carcinoma (RCC) is an uncommon malignancy among children and adolescents. Because of this, there has been relatively sparse research and evidence on the topic. As the body of research regarding pediatric and adolescent RCC has developed in recent years, it has become increasingly clear that it demonstrates important differences from the much more common adult-type RCC. This review aims to examine and summarize the current literature, with a focus on the ways that pediatric and adolescent RCC differ from the adult disease, and to make recommendations for evaluation and management based on this evidence whenever possible.Methods and materialsA thorough search of all articles relating to pediatric and adolescent RCC has been undertaken using PubMed. The reference lists from all relevant articles have been further reviewed, to ensure inclusion of all pertinent literature.ConclusionThe most significant development in recent years has been the realization that most of the pediatric and adolescent RCC cases, but only a very small fraction of adult RCC cases, demonstrate “translocation tumor” pathology. It is likely that such differences may eventually explain many of the previous observations regarding differences in behavior of RCC among children and teenagers. At this point, however, the relevance of translocation pathology to clinical management remains unclear, and so most continue to treat these patients in much the same way as those with the more conventional tumor subtypes.