作者: Nathalie Gaspar , Douglas S. Hawkins , Uta Dirksen , Ian J. Lewis , Stefano Ferrari
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摘要: Ewing sarcoma (ES) is an aggressive of bone and soft tissue occurring at any age with a peak incidence in adolescents young adults. The treatment ES relies on multidisciplinary approach, coupling risk-adapted intensive neoadjuvant adjuvant chemotherapies surgery and/or radiotherapy for control the primary site possible metastatic disease. optimization multimodality therapeutic strategies has resulted from efforts several national international groups Europe North America cooperation between pediatric medical oncologists. Successive first-line trials addressed efficacy various cyclic combinations drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, dactinomycin identified prognostic factors now used to tailor therapies. role high-dose chemotherapy still debated. Current 5-year overall survival patients localized disease 65% 75%. Patients metastases have < 30%, except those isolated pulmonary metastasis (approximately 50%). recurrence dismal prognosis. many insights into biology EWS-FLI1 protein initiation progression remain be translated novel strategies. options future approaches will discussed.