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.

Causes

Although 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.

Diagnosis

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.

Treatment

Excision of the tumor along with a 3" (7.6 cm) margin is the treatment of choice. It may be combined with preoperative chemotherapy.
In some patients, radical surgery (such as hemipelvectomy or interscapulothoracic amputation) is necessary. However, surgical resection of the tumor (often with preoperative and postoperative chemotherapy) has saved limbs from amputation.
Intensive chemotherapy includes administration of doxorubicin, vincristine, cyclophosphamide, cisplatin, and dacarbazine. Chemotherapy may be infused intraarterially into the long bones of the legs.
Radiation therapy is used to prevent local recurrences of radiosensitive tumors and may be given either before or after surgery.

Disclaimer: Health-care-clinic.org website is designed for educational purposes only. It is not intended to treat, diagnose, cure, or prevent any disease. Always take the advice of professional health care for specific medical advice, diagnoses, and treatment. We will not be liable for any complications, or other medical accidents arising from the use of any information on this web site. Please note that medical information is constantly changing. Therefore some information may be out of date.