Update on Management of Pediatric Renal Neoplasms

The treatment of pediatric renal tumors is nuanced and complex. This review highlights these nuances and aims to prepare the surgeon for treating these complex patients. Cooperative group study has improved survival for several pediatric renal tumors significantly, namely, Wilms tumor and clear cell sarcoma. There are several more rare tumors however, which have not achieved the same level of success and still require more study to find the optimal treatment regimen to achieve excellent overall survival. These tumors include rhabdoid tumor and renal medullary carcinoma. Several COG Wilms tumor protocols have recently closed and new ones are set to release soon. It is anticipated that these studies will continue to shift focus to minimizing adverse events/late effects by potentially eliminating or reducing chemotherapeutic exposures while maintaining excellent overall survival. Specifically, new discovery of molecular markers in Wilms tumor will play a significant role with respect to risk stratification. Already, loss of heterozygosity at both 1p and 16q prompts treatment intensification as they are associated with decreased survival. However, gain of 1q, independent of 1p/16q mutations, seems to be more common and even more tightly associated with poor outcomes, which will likely be a key factor involved with adjuvant therapy considerations. New treatment protocols for Wilms tumor are forthcoming and will include more molecular markers for risk stratification. Other COG studies on more rare renal tumors are underway to further improve survival for patients with these malignancies.