Systemic Treatment of Ewing Sarcoma: Current Options and Future Perspectives

Abstract Ewing sarcoma (ES) is an uncommon malignant neoplasm, mostly affecting young adults and adolescents. Surgical excision, irradiation, and combinations of multiple chemotherapeutic agents are currently used as a multimodal strategy for the treatment of local and oligometastatic disease. Although ES usually responds to the primary treatment, relapsed and primarily refractory disease remains a difficult therapeutic challenge. The growing understanding of cancer biology and the subsequent development of new therapeutic strategies have been put at the service of research in recurrent and refractory ES, generating a great number of ongoing studies with compounds that could find superior clinical outcomes in the years to come. This review gathers the current available information on the treatment and clinical investigation of ES and aims to be a point of support for future research.