Therapy management in bone sarcoma

Abstract

SummaryBone sarcomas are rare, representing only 0.2% of all diagnosed cancers. Incidence is higher in children and adolescents, but bone sarcomas are still numerically outnumbered by benign bone tumors in this patient cohort. This article summarizes the management of treatment for bone sarcomas. Systemic therapy, surgical therapy, and radiotherapy are presented. Osteosarcoma, chondrosarcoma, and Ewing sarcoma are among the most frequently occurring bone sarcomas in all age groups. In recent decades, multimodal treatment of these rare entities has increased disease-free survival for these patients. As malignant primary bone tumors are rare cancers, and since management is complex, the standard of treatment should be performed by reference centers. If possible, wide en-bloc resection of the bone sarcoma should be performed. The role of radiotherapy in osteosarcoma and chondrosarcoma is limited, but Ewing sarcoma is a radiation-responsive tumor. In primary bone sarcomas, the efficacy of chemotherapy varies according to histological type. Prognosis is poor in patients with osteosarcoma or Ewing’s sarcoma if surgery without neoaqdjuvant chemotherapy is performed. Despite advances in surgical, medical, and radiation therapy, few significant positive changes in overall survival have been observed in patients with these diseases in recent decades.