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Despite significant progress with the use of intensive multiagent chemotherapy and local control measures, a significant proportion of patients die of disease progression. Chemotherapy dose intensification and autologous hematopoietic stem cell transplant (HSCT) have been explored by many institutions without obvious benefit in high\u2010risk patients. Our current understanding in the biology and treatment of ESFT suggests that a more rational approach to the development of risk\u2010adapted therapy should be undertaken.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>We performed a review of the most relevant data regarding the current status in the treatment of ESFT. The results of the major American and European cooperative groups were analyzed, including the treatment strategies used and the prognostic factors identified for both localized and metastatic ESFT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The intensification of alkylating agents and topoisomerase\u2010II inhibitors is feasible and has resulted in some survival improvement for selected patients. This benefit seems to be restricted to patients with localized disease, and a proportion of survivors are at risk of developing treatment\u2010related hematologic malignancies. Nevertheless, these advances have resulted in a re\u2010definition of prognostic factors, which may help to define risk groups based on tumor load parameters as well as biologic factors (type of fusion transcript and histologic response to chemotherapy). Patients with advanced metastatic disease may benefit from HSCT. New strategies such as immunotherapy and the use of biologic modifiers may have a role in the treatment of ESFT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Future treatment for ESFT should consider risk\u2010adapted strategies and the inclusion of newer therapies such as biologic modifiers for the minimal residual disease. A modified risk\u2010adapted therapy is proposed. 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