In local high-risk Ewing Sarcoma, treatment approaches differ between COG (Children's Oncology Group) and Euro Ewing 2012 protocols. In Euro-Ewing protocol, high-dose chemotherapy and autologous stem cell transplantation is performed after local therapy. COG protocol includes fourteen chemotherapy regimens and local therapy after the fourth chemotherapy regimen. Targeted agents added to standard chemotherapy and novel agents for maintenance are warranted (11)(12)(13)(14). Here, we discuss the time of local therapy in stem cell rescue, high-dose chemotherapy, and autologous stem cell transplantation in this aggressive tumor. Our fifteen-year-old patient had right pelvic Ewing sarcoma with a volume higher than 400 ml. After three chemotherapy regimens, the tumor was inoperable and radiotherapy was applied. Stem cell rescue is performed after the fourth chemotherapy regimen. High-dose chemotherapy and autologous stem cell transplantation are planned for relapse consolidation (17). There is no consensus about the indication of high-dose chemotherapy and autologous stem cell transplantation in high risc Ewing sarcoma with an initial tumor volume > 400 ml.
The mainstay treatment for recurrent metastatic osteosarcoma is surgical resection of all metastatic and recurrent lesions. Ifosfamide, gemcitabine-docetaxel, VOIT (vincristine-irinotecantemozolamide, bevacizumab) can be given in relapsed osteosarcoma. Radiotherapy is also applied in selected cases. The efficacy of tyrosine kinase inhibitors, pazopanib, and sorafenib, has been demonstrated in refractory osteosarcoma. In this case report, a case who underwent ten days radiotherapy and limb-sparing surgery in primary site recurrence, followed by ifosfamide-doxorubicin, high-dose methotrexate, and ifosfamide, received 6 cycles of VOIT after lung metastasectomy, could not tolerate pazopanib, and was treated with sorafenib as a maintenance treatment is presented. The case has been in remission for eight months after the last chemotherapy. Sorafenib can be safely given for one year for microscopic residual disease in remission-remitting patients with a high risk of relapse.
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