Osteosarcoma

Definition

Osteosarcoma is a malignant bone tumor that usually develops during the period of rapid growth which occurs in adolescence, as a teenager matures into an adult.

Alternative Names

Causes, incidence, and risk factors

Osteosarcoma is the most common malignant bone tumor in youth. The average age at diagnosis is 15. Boys and girls have a similar incidence of this tumor until late adolescence, at which time boys are more commonly affected.

The cause is not known. In some cases, osteosarcoma runs in families and at least one gene has been linked to increased risk. This gene is also associated with familial retinoblastoma, a cancer of the eye which occurs in children. It appears to be a defective tumor inhibitor gene.

Osteosarcoma tends to occur in the bones of the thigh (distal end, near the knee), upper arm (proximal end, near the shoulder), and shin (proximal end, near the knee). This cancer occurs most commonly in larger bones and in the area of bone with fastest growth rate. Osteosarcoma can occur in any bone, however.

The most common symptom initially is pain. As the tumor grows, there may be visible swelling and limitation of motion. Tumors in the legs cause limping, while those in the arms cause pain on lifting. Swelling over the tumor may be warm and slightly reddened.

Symptoms

• Bone pain
• Tenderness, swelling, or redness at the site of the bone pain
• The first sign may be a bone fracture. The tumor may cause weakness in the affected area of the bone. A fracture at the site of the tumor is called a "pathological fracture," which occurs after what often seems like a routine movement. For example, a young athlete who breaks an arm during a routine throw should be tested to ensure there is no underlying bone problem, such as a tumor or cyst.

Signs and tests

• X-ray of the affected area
• CT scan of the affected area
• Blood tests
• CT of the chest to see if the cancer has spread to the lungs
• Open biopsy (at time of surgery for diagnosis)
• Bone scan to see if the cancer has spread to other bones

Treatment

Treatment usually starts after a biopsy of the tumor. Before a major surgery to remove the tumor, chemotherapy is usually given. This is strong medication, injected intravenously (into the vein) to make the tumor shrink. Chemotherapy also treats any cancer cells which may have spread to other parts of the body. Surgery is then used to remove any remaining tumor, while attempting to spare the affected limb. In most cases, surgery can remove the tumor while sparing the affected limb (this is called limb-salvage surgery).Rarely more radical surgery (such as amputation) may be necessary for a permanent cure.

• High dose methotrexate with leucovorin
• Doxorubicin (Adriamycin)
• Cisplatin
• Carboplatin (Paraplatin)
• Cyclophosphamide (Cytoxan)
• Ifosfamide (Ifex)

Support Groups

Association of Cancer Online Resources

www.acor.org

Cure Search (formerly the National Childhood Cancer Foundation)

www.curesearch.org

Expectations (prognosis)

In the absence of pulmonary metastasis (spread of the tumor to the lungs), long-term survival is likely to reach 70%. If the cancer has spread to other parts of the body, there is still a good chance of cure with effective treatment.

Complications

• Limb removal
• Lung metastases
• Effects of chemotherapy vary for each person and depend on the specific chemotherapy given

Calling your health care provider

Call your health care provider if bone pain, tenderness, or swelling occur.