Common chemotherapy regimens for Ewing sarcoma

  I. Overview Ewing sarcoma is a malignant tumor of small round cell proliferation in bone. Recent cytomolecular genetic studies have shown that Ewing’s sarcoma is one of the following groups of tumors, including primary neuroectodermal tumors, peripheral neuroepithelial tumors, Askin’s tumor of the chest wall and Ewing’s sarcoma outside the bone.  II. Chemotherapy regimen 1. VACA regimen.  2.Risk classification based regimen-CESS86 regimen.  Ewing’s sarcoma is a highly malignant tumor of bone, originating from the neuroepithelium within the bone marrow. As with osteosarcoma, Ewing sarcoma is prone to early metastasis and has a long-term survival rate of 5%-10% without chemotherapy. Ewing sarcoma is still treated with neoadjuvant chemotherapy, which follows the steps of chemotherapy, surgery, and then chemotherapy, but because the tumor is sensitive to radiotherapy, radiotherapy is often given before and after surgery in parallel with chemotherapy. The efficiency of chemotherapy alone is around 30%. The survival rate of Ewing sarcoma can reach more than 70% with multi-drug combination and integrated radiotherapy and surgery.  The traditional chemotherapy regimens are VAC and VACA regimens. Since cyclophosphamide and actinomycin D both have myelosuppressive effects and vincristine is mainly neurotoxic, there is a trend to replace CTX by IFO. After clinical observation, ADM has a significant effect in the treatment of Ewing sarcoma, which can significantly improve the 5-year survival rate. In terms of dosing modality, high-dose intermittent chemotherapy was significantly more effective than low-dose continuous chemotherapy. The size of the tumor is also related to the prognosis. Recent studies have shown that IFO and Vp-16 can be more than 60% effective in Ewing sarcoma. The conventional VAC and VACA regimens, as well as chemotherapy regimens for SSG, are listed below.  In cases of poor histologic response and recurrent metastases, Marfalan has better clinical results. Increasing the dose of drugs in the regimen combined with systemic radiotherapy, using bone marrow and stem cell rescue is expected to improve the 5-year survival rate. The effect of biological treatments such as transfer factor, interferon, and interleukin-2 is uncertain.