Spinal Intramedullary Ewing Sarcoma with Meningeal Carcinomatosis: A Complete Response to Chemotherapy and Neuroaxis Irradiation. Case Report and Review of Literature

Abstract

Intramedullary Ewing sarcoma of the spinal cord is an extremely rare form of extraosseous Ewing sarcoma, with few cases reported in the literature. Given its rarity and the great difficulty in generating scientific evidence, there are no specific recommendations for its management, which is usually extrapolated from the guidelines for bone Ewing sarcomas. Aspects such as the preferred chemotherapy scheme and the indication of whole neuroaxis irradiation remain unanswered. We report the case of an 18-year-old patient with a spinal intramedullary Ewing sarcoma and a leptomeningeal relapse after surgery, who achieved a complete clinical and radiological response to chemotherapy with VDC/IE protocol and craniospinal irradiation.