Purpose of review: Pediatric renal tumors account for 7% of new cancer diagnoses in children. Here, we will review results from recently completed clinical trials informing the current standard of care and discuss targeted and immune therapies being explored for the treatment of high risk or relapsed/refractory pediatric renal malignancies.
Recent findings: Cooperative group trials have continued to make improvements in the care of children with pediatric tumors. In particular, trials that standardize treatment of rare cancers (e.g., bilateral Wilms tumor) have improved outcomes significantly. We have seen improvements in event free and overall survival in recently completed clinical trials for many pediatric renal tumors. Still, there are subsets of rarer cancers where outcomes remain poor and new therapeutic strategies are needed. Future trials aim to balance treatment toxicity with treatment efficacy for those with excellent outcomes while identifying novel therapeutics for those with poor outcomes.
Trial registration: ClinicalTrials.gov NCT03155620.
Keywords: 1q Gain; AREN0321; AREN0532; AREN0533; AREN0534; Clear-cell sarcoma of the kidney; LOH 1p/16q; Malignant rhabdoid tumor; Mesoblastic nephromas; Pediatric renal tumors; Wilms tumor.