Figure 1.

Current and future EWS treatment options. Current treatment of EWS typically employs VAC+IE or VIDE regimens. Local control includes surgery and/or radiation therapy. With this regimen, patients with only local disease have about 70% disease-free survival (EFS). However, patients with EWS who have metastatic disease or who have recurrence have <20% EFS. Second-line relapse regimens as shown below often provide temporary benefit. New agents against IGF1R and /or mTOR are currently available. Future options include innovative targeted therapies. V: vincristine; D: doxorubicin; C: cyclophosphamide; I: ifosphamide/mesna; E: etoposide; IGF1R insulin-like growth factor 1 receptor inhibitor; mTOR: mammalian target of rapamycin inhibitor.