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Since its first description in 1921, Ewing sarcoma has been the subject of several morphologic and genetic investigations. Currently, the overall survival for localized Ewing sarcoma is 65–70%. However, in patients presenting with metastatic disease, the overall survival is poor, being in the range of 20–30%. There are several unknown features of Ewing sarcoma, such as its cell of origin, genetic background, chemotherapy resistance, and abnormal presentation sites, among others. A better understanding of the molecular basis of the development of Ewing sarcoma is needed to help improve survival, especially in metastatic/resistance cases. In this chapter, we provide an overview of the features of metastatic Ewing sarcoma.
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