Management of primary Ewing sarcoma of the kidney with inferior vena cava (IVC) tumor thrombosis

Ewing sarcoma (ES) is an entity which belongs to a spectrum of neoplastic diseases called the Ewing sarcoma family of tumors (EFT). EFTs of the kidney represent less than 1% of all renal tumors. Herein, we presented a case of primary renal ES with tumor thrombosis up to vena cava who underwent radical nephrectomy and IVC tumor thrombectomy followed by adjuvant chemotherapy. Histopathology showed that the tumor composed of small uniform, dark, round cells arranged in sheets, and rosettoid pattern. The diagnosis of ESFT was confirmed by detecting EWS/FLI-1 fusion gene using reverse transcription polymerase chain reaction (RT-PCR).