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    Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Osteosarcoma and Malignant Fibrous Histiocytoma (MFH) of Bone

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    Osteosarcoma occurs predominantly in adolescents and young adults. Review of data from the Surveillance, Epidemiology, and End Results program of the National Cancer Institute resulted in an estimate of 4.4 cases per 1 million new cases of osteosarcoma each year in people aged 0 to 24 years.[3] The U.S. Census Bureau estimates that there will be 110 million people in this age range in 2010, resulting in an incidence of roughly 450 cases per year in children and young adults younger than 25 years. Osteosarcoma accounts for approximately 5% of childhood tumors. In children and adolescents, more than 50% of these tumors arise from the long bones around the knee. Osteosarcoma can rarely be observed in soft tissue or visceral organs. There appears to be no difference in presenting symptoms, tumor location, and outcome for younger patients (<12 years) compared with adolescents.[4,5] Two trials conducted in the 1980s were designed to determine whether chemotherapy altered the natural history of osteosarcoma after surgical removal of the primary tumor. The outcome of patients in these trials who were treated with surgical removal of the primary tumor recapitulated the historical experience before 1970; more than half of these patients developed metastases within 6 months of diagnosis, and overall, approximately 90% developed recurrent disease within 2 years of diagnosis.[6] Overall survival for patients treated with surgery alone was statistically inferior.[7] The natural history of osteosarcoma has not changed over time, and fewer than 20% of patients with localized resectable primary tumors treated with surgery alone can be expected to survive free of relapse.[6,8]; [9][Level of evidence: 1iiA]

    Prognostic Factors

    Pretreatment factors that influence outcome include the following:[10]

    • Primary tumor site.
    • Size of the primary tumor.
    • Presence of clinically detectable metastatic disease.

    After administration of preoperative chemotherapy, factors that influence outcome include the following:

    • Surgical resectability.
    • Degree of tumor necrosis.

    In general, prognostic factors in osteosarcoma have not been helpful in identifying patients who might benefit from treatment intensification or who might require less therapy while maintaining an excellent outcome.

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