Abstract

The Ewings family of tumours comprises a spectrum of malignancies of primitive neuroectodermal cells: embryonic cells that migrate from the neural crest. 1Primary kidney Ewings sarcoma is a rare neoplastic disease representing less than 1% of renal tumors and is characterized by highly aggressive biological behavior. We reported a case of an asymptomatic, 53 year old man with an incidental lesion in the right kidney found in a routine renal ultrasound. Computed tomography showed a solid nodule with 67 x 65 mm, occupying the lower pole of the right kidney with renal vein, inferior vena cava and right auricular invasion. The patient underwent a right radical nephrectomy and Inferior vena cava and atrial tumor thrombectomy with cardiopulmonary bypass and deep hypothermic circulatory arrest. Pathologic characteristics and immunohistochemical analysis confirmed the diagnosis of Ewings Sarcoma. Surgery and early post‐operative were free of complications. The patient underwent chemotherapy and died 21 months after the surgery of relapse of the disease.