Bone Tumors, Primary Malignant
A rare type of bone cancer, primary malignant bone tumors (sarcomas of the bone) constitute less than 1 % of all malignant tumors. Most malignant bone tumors are secondary, caused by seeding from a primary site. Primary malignant bone tumors are more common in males, especially in children and adolescents, although some types do occur in people between ages 35 and 60.
The tumors may originate in osseous or nonosseous tissue. Osseous bone tumors arise from the bony structure itself and include osteogenic sarcoma (the most common), parosteal osteogenic sarcoma, chondrosarcoma, and malignant giant cell tumor. Together they make up 60% of all malignant bone tumors. Nonosseous tumors arise from hematopoietic, vascular, and neural tissues and include Ewing 's sarcoma, fibrosarcoma, and chordoma. Osteogenic and Ewing's sarcomas are the most common bone tumors in childhood.
CausesAlthough some cases of osteosarcoma are associated with genetic abnormalities (Li-Fraumeni, retinoblastoma, Rothmund-Thomson) or exposure to carcinogens (such as ingested radium in watch dial painters), most cases have no immediately apparent cause. Ewing 's sarcoma cells demonstrate a characteristic translocation of genetic material from chromosome 22 to chromosome 11. Additional theories point to heredity, trauma, and excessive radiation therapy
Signs and symptoms
Patients may present with persistent pain, swelling, or tenderness of a bone. They may have unexplained fracture of one or more bones, sometimes without noticeable trauma.
A biopsy (by incision or by aspiration) is essential for confirming primary malignant bone tumors. Bone X-rays and radioisotope bone and computed tomography (CT) scans show tumor size. Serum alkaline phosphatase is usually elevated in patients with sarcoma.
CLINICAL TIP Bone X-rays, CT scans, and magnetic resonance imaging are all useful in assessing tumor size. Bone scans and CT scans of the lungs are important in checking for metastatic disease.
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