Abstract
Bone sarcomas comprise less than 0.2% of all malignancies and are divided into several subtypes with varying sensitivities to systemic therapy. While the role of adjuvant chemotherapy in the management of soft-tissue sarcomas is controversial, perioperative chemotherapy has greatly improved the survival of nonmetastatic Ewing sarcoma and osteosarcoma. However, metastatic unresectable Ewing sarcoma and osteosarcoma are still associated with a poor prognosis. Giant cell tumor of bone and chordoma are refractory to conventional cytotoxic chemotherapy, but in recent years targeted therapy has been shown to be able to provide a degree of control of unresectable disease. Conventional chondrosarcoma remains refractory to systemic therapy, although the less common subtypes, dedifferentiated and mesenchymal chondrosarcomas, can be sensitive to chemotherapy but still have a poor prognosis.
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