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Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution

Journal of Pediatric Urology Jul 04, 2020

Qureshi SS, Bhagat M, Verma K, et al. - Researchers investigated the incidence of non-Wilms renal tumors as well as the associated diagnostic challenges, therapies and, outcomes at the largest tertiary cancer center in India. In the pediatric oncology unit of Tata Memorial Hospital, 569 patients with renal tumors were reported between 2006 and 2019; of these, 109 (19%) patients had primary (n = 97) or recurrent (n = 12) non-Wilms renal tumors. Clear cell sarcoma (CCSK) (39.4%), renal cell carcinoma (RCC) (19.3%), malignant rhabdoid tumor (MRTK) (12.8%), Ewing's sarcoma (rES) (15.6%), synovial sarcoma (2%), and undifferentiated sarcoma (2%) were the histological high-risk group. Surgery alone results in excellent outcomes in low-risk tumors while the high-risk tumors were associated with a variable outcome. The worst survival was observed in correlation with MRTK and recurrent non-Wilms tumor. In this study, CCSK and RCC had favorable outcomes and MRTK was associated with worst outcomes. Higher incidence of treatment failure and unsatisfactory outcomes were observed for renal ES. An extremely poor outcome was reported among those with recurrent non-Wilms tumors and more alternative or innovative approaches are required for their treatment.

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