This article focuses on high-grade intramedullary osteosarcoma (also referred to as simply osteosarcoma), including its classic osteoblastic form and its fibroblastic and chondroblastic forms. Osteosarcoma is the most common malignant bone tumor.1, 2 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.
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.
Synonyms and related keywords: osteogenic sarcoma, osteoblastic osteosarcoma, chondroblastic osteosarcoma, fibroblastic osteosarcoma, multifocal osteosarcoma, high-grade intramedullary osteosarcoma, typical osteosarcoma, classic osteosarcoma, conventional osteosarcoma, variant osteosarcoma, primary osteosarcoma, synchronous osteosarcoma, metachronous osteosarcoma, unicameral bone cyst, osteofibrous dysplasia, Campanacci tumor, periosteal osteosarcoma, malignant bone cancer, bone cancer
Mehlman & Cripe 2008