Primary renal sarcoma with inferior vena cava thrombus presenting with tumor rupture.

Primary renal sarcoma represents approximately one per cent of all primary tumors of the kidney in adults. We report an unusual case of primary renal sarcoma with inferior vena cava (IVC) thrombus. This patient presented with hemorrhagic shock due to tumor rupture. Emergent radical nephrectomy and vena caval thrombectomy were performed. Histological examination proved a clear cell sarcoma.