文摘
Patients diagnosed with Ewing sarcoma family of tumors (ESFT) commonly require multimodality therapy including radiation therapy, in addition to surgery and chemotherapy, to achieve optimal outcomes. In most cases, a specific genetic defect with a translocation t(11:22)(q24;q12) corresponding to fusion between the EWS gene and an ETS proto-oncogene is present. The Intergroup Ewing Sarcoma Studies (IESS) Studies, the CESS studies, and the Euro-Ewing studies, among others, have helped to define the efficacy of chemotherapy agents, as well as the radiation therapy backbone for ESFT used today. The relative effectiveness of surgery, radiation therapy, and combinations of these local control modalities continue to be defined in specific clinical situations and body sites. Important late effects of radiation therapy may include pneumonitis, radiation cystitis, and second malignancy. Promising for the future may be new chemotherapeutic agents, improved radiation techniques including proton radiation therapy, and possibly myeloablative strategies.