Osteosarcoma is the most common malignant bone tumor. This disease is thought to arise from primitive mesenchymal bone-forming cells, and its histologic hallmark is the production of malignant osteoid. Other cell populations may also be present, as these types of cells may also arise from pluripotential mesenchymal cells, but any area of malignant bone in the lesion establishes the diagnosis as osteosarcoma. In the United States, the incidence of osteosarcoma is 400 cases per year (4.8 per million population < 20 years). The overall survival rate for patients diagnosed was 63% (59% for males; 70% for females).
The mainstay of therapy is surgical removal of the malignant lesion. Most often, limb-sparing (limb-preserving) procedures can be used to treat patients with this disease and, thus, preserve function. Chemotherapy is also required to treat micrometastatic disease, which is present but often not detectable in most patients (about 80%) at the time of diagnosis. Researchers are studying newer types of drugs that attack osteosarcoma cells in different ways: Immunotherapy drugs, Targeted therapy drugs: sorafenib pazopanib temsirolimus everolimus and Drugs that affect the bones.