Retrospective Analysis and Clinical Implications of Pediatric Renal Cell Carcinoma: A Single-Center Study

Background: Pediatric Renal Cell Carcinoma (RCC) accounts for ~2% of renal tumors in pediatrics and differs from adult RCC. Translocation-associated RCC driven by TFE3 or TFEB is the most common pathological type of pediatric RCC. One study has suggested that cancer survivors have a higher risk for another malignancy and especially translocation-associated RCC could be developed after cytotoxic chemotherapy. However, research in the field is still insufficient. This study analyzed and summarized the clinical data of pediatric RCC. Methods: Pediatric patients aged 0-18 years who received histological diagnosis of RCC from 1998 to 2025 were identified at Samsung Medical Center, one of the tertiary hospitals located in Seoul, South Korea. Demographic data, clinical features, therapeutic outcomes and mortality were retrospectively analyzed. Results: Twenty-two patients met inclusion criteria. The mean age at diagnosis was 13 years; the male-to-female ratio was 1:1. Initial manifestations were gross hematuria 50%, abdominal pain 27.3%, incidental radiological findings 18.2% and palpable mass 13.6%. Comorbid conditions were present in 22.7% (5/22): neuroblastoma (n = 2), T-cell acute lymphoblastic leukemia, focal segmental glomerulosclerosis and liver cirrhosis (each n = 1). All five had received nephrotoxic agents, yet none showed disease progression or died. All patients underwent primary surgical resection; therapies for relapse included resection surgery, targeted agents, radiofrequency ablation or cryoablation as clinically indicated. The mortality rate was 22.7% (5/22). Patients without metastatic disease or recurrence all survived. In contrast, metastasis at diagnosis (9%, n = 2) conferred 100% mortality. Additionally, 18.2% (n = 4) experienced recurrence after primary surgical treatment, and their mortality rate was 50%. Lastly, 4.5% (n = 1) had both metastasis and recurrence, with 100% mortality. Metastasis or recurrence was asymptomatic and usually detected radiologically. Conclusion: Metastatic or recurrent disease is the principal determinant of mortality in pediatric RCC, whereas underlying comorbidities and prior nephrotoxic therapy were not associated with worse outcomes in this cohort. Larger studies are warranted to clarify the relationship between nephrotoxic exposure and RCC development and to optimize risk-adapted management strategies.