The Role of STAG2, CDKN2A, and TP53 Genes in Ewing Sarcoma: A Report of a Rare Case of Primary Ewing Sarcoma of the Kidney With Favorable Outcome

Background: Primary renal Ewing sarcoma (EWS) is a rare but aggressive pediatric malignancy with poor outcomes and limited knowledge of its behavior and biology. The use of molecular markers as prognostic factors and local control strategies with surgical resection and radiation is not well-described for renal EWS. Observation: We document a case of stage IV renal EWS with 4.5 years of durable remission. Conclusion: We hypothesize the favorable outcome may be associated with the lack of specific genomic signatures ( STAG2 , CDKN2A , and TP53 ) and the use of upfront surgical resection followed by radiation for local control.