ABSTRACT
Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissues arising predominantly in children and young adults. Current management of primary ES relies on a multimodality approach, coupling intensive cytotoxic drugs regimens with surgery and/or radiotherapy. The combination of primary site control and possible metastatic disease resulted in increased survival rates in localized disease, at the expense of substantial acute and long-term toxicity. Contrarily, the prognosis is still dismal in the metastatic setting, especially for patients with recurrent ES. Indeed, the lack of an effective treatment strategy after a first-line chemotherapy represents an unmet clinical need. The aim of this study is to examine the current treatment options and discuss potential future perspectives that could answer to the key issues in the management of ES.

IMPACT STATEMENT
This study reports the current management of Ewing sarcoma on a multimodal approach, coupling intensive cytotoxic drugs regimens with surgery and/or radiotherapy. It examines the current options and discuss potential future prospective.