Objective: To report two new cases of Ewing's sarcoma/primitive neuroectodermal tumor of the kidney, one of them with tumor thrombus in cava.
Method: Characterization of two new cases and literature review by PubMed search.
Results: We report the cases of two men diagnosed with primary renal Ewing's sarcoma, who have been treated with nephrectomy and adjuvant chemotherapy, being in complete remission to date.
Conclusion: Ewing's sarcoma / primitive neuroectodermal tumor of the kidney is a rare condition that mainly affects young adults. The natural history of these tumors is the evolution towards metastatic disease and death. Treatment is multimodal, combining surgery and chemotherapy. The role of radiotherapy is not well established.