Rare entities, making up .18% of all new cancers.
3000 new cases per year in the U.S.
Characteristically are tumors of children and young adults.
Osteosarcoma, Ewing’s sarcoma, are the most common lesions which occur during childhood and adolescence.
Fibrosarcomas, chondrosarcomas, and malignant fibrous histiocytomas are less common.
Osteosarcomas and Ewing sarcoma incidences peak in second decade of life.
Chondrosarcomas are heterogenous and occur in patients older than 50 years.
90% of chondrosarcomas are conventional low-to-intermediate grade with indolent behavior and low metastatic potential.
Only 5-10% of conventional chondrosarcomas are grade 3 lesions, with metastatic potential.
Chemotherapy plays a major role in the treatment of osteosarcomas, except for tumors of the jaw, for which surgery is a critical determinant of success.
With the addition of chemotherapy perioperatively for osteogenic sarcoma the five-year overall survival has improved 60-70% for localized disease.
Chemotherapy alone can result in durable remissions, but the disease eventually relapses in patients who do not undergo resection.
Chemotherapy for osteogenic sarcoma relies on for active drugs-doxorubicin, cisplatin, high-dose methotrexate, and ifosfamide.
Treatment of high grade localized osteosarcoma of the extremity consists of neoadjuvant chemotherapy followed by limb sparing surgical resection and subsequent adjuvant chemotherapy.
In the above studies long-term overall survival rates are greater than 60% and limb salvage is greater than 80% of patients.