License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Ewing sarcoma (ES) is the second most common type of primary bone malignancy in children and young adults. Survival rates for localized ES have improved to upwards of 70 % with aggressive chemotherapy and local control. On the other hand, there has been little improvement in survival rates for patients with metastatic or recurrent ES. Herein we review the different current therapeutic approaches available, including the different upfront and salvage chemotherapy regimens, the role for stem cell transplantation, and potential use of immunotherapy. 1. Background Ewing sarcoma (ES) is the second most common type of primary bone malignancy in children and young adults, and age of onset is most often in the second decade, with a slight male predominance . The ES family of tumors is a group of small round blue cell neoplasms of neuroectodermal origin, which includes classical ES, primitive neuroectodermal tumors (PNETs), and Askin tumors of the chest wall
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