Renal Ewing sarcoma with extensive neuroectodermal differentiation: Case report and literature review.

Abstract

Ewing sarcoma is part of the Ewing sarcoma family of tumors, the renal localization being very rare. Less than 200 cases were reported in the Medline database. Currently there is no universally accepted treatment regimens for Ewing sarcoma with extensive neuroectodermal differentiation because most data we possess is based on case reports and case series. Case summary: A 33-year-old patient presented at the Emergency Department with right lumbar pain following a mild trauma and an episode of macroscopic hematuria. Physical examination confirmed hematuria and flank pain and a palpable flank mass was identified. MRI showed a cystic lesion of the upper pole of the right kidney of 127/110/123 mm. After prior agreement of the multidisciplinary team, a 3D laparoscopic right radical nephrectomy was done. The histopathological diagnosis revealed a Ewing sarcoma with extensive neuroectodermal differentiation staged as pT3N1M0L1V2R0. The patient underwent chemotherapy, but the disease progressed. Currently, one year after the patient’s diagnosis, the disease is progressing despite the chemotherapy and now the patient is candidate to receive third line chemotherapy but the general status was not changed. Conclusions: As renal localization of Ewing sarcoma with extensive neuroectodermal differentiation is extremely rare, multimodal treatment strategies need to be established by a trasndisciplinary team. Despite its aggressive biological behavior, a proper therapeutic management might prolonge the patient’s lives.