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Osteosarcoma

Pediatric and adult osteosarcoma patients at Johns Hopkins are treated by a team of experts that include medical and radiation oncologists, orthopedics and plastic surgeons.  Through the Johns Hopkins Sarcoma Center, patients also have access to the latest clinical trials and treatments integrated into a comprehensive care plan.

Osteosarcoma Experts

Johns Hopkins osteosarcoma experts use the latest technology, surgical techniques and clinical trials to treat sarcomas in patients of all ages.  Our multidisciplinary team meets weekly to review and coordinate care for all sarcoma patients.

About Osteosarcoma

Sarcomas are a rare type of malignant tumor that can be divided into two groups: bone sarcomas and soft tissue sarcomas. Each year, approximately 8,900 new cases of bone and soft tissue sarcomas are diagnosed. Of the approximate 8,900 new cases, 650-700 children and adolescents younger than 20 years of age are diagnosed with bone sarcomas and 850-900 with soft tissue sarcomas. Below is information on osteosarcomas in children and adults.

Osteosarcomas in Children

Osteosarcoma is the most common bone tumor in children. Classic osteosarcomas are high grade and begin to grow within the bone. It often starts in the ends of the bones where new bone tissue forms as a young person grows. Bones in the thigh, upper arm, and shin are the most common site, but osteosarcoma can occur anywhere in the body. It usually develops during the period of rapid growth during adolescence, when a teenager matures into an adult. The average age at diagnosis is 15 years. Boys and girls have a similar incidence of this tumor until late adolescence, at which time boys are more commonly affected. Osteosarcoma cells can be many different shapes and sizes. Only rarely does osteosarcoma run in families. A defective tumor suppressor gene (which allows tumors to grow) has been linked to an increased risk of this disease and is also associated with familial retinoblastoma, a childhood cancer of the eye. Children with familial retinoblastoma have a high risk for osteosarcoma in adolescence.

Osteosarcoma Treatment in Children

Chemotherapy

In general, chemotherapy (treatment with anticancer drugs) is given before surgery to shrink the main tumor. It is called neoadjuvant chemotherapy as it is often given before the tumor is removed by surgery. Neoadjuvant chemotherapy for osteosarcoma became standard of care at the beginning of the era when surgeons began repairing diseased limbs. New prosthesis to replace the limb required time to manufacture since they were custom-made to the patient’s size. Thus, chemotherapy was used to deliver treatment while the prosthesis was being made. Physicians found that three or four cycles of chemotherapy with multiple drugs given before surgery may help kill off more tumor cells so that it is easier to surgically remove the remaining tumor. Radiation therapy (treatment with targeted x-rays) is rarely used but may be recommended to control pain in late stage disease or if the cancer is centralized in the spine.

Surgery

The second stage of treatment is to remove the main tumor through surgery. After the tumor is surgically removed, pathologists study it to reveal a percentage necrosis rate, or the amount of dead tumor cells. The higher the percentage necrosis rate, the more dead tumors cells, and this figure represents, in essence, a “report card” to guide the medical/pediatric oncologist on how to tailor additional therapy. Studies have shown that the necrosis rate is highly predictive of overall survival. Some recent studies indicate that altering the type of chemotherapy drug given after surgery may not improve survival of patients with low necrosis rates. Medical/pediatric oncologists are testing different strategies to combat aggressive tumors including therapies that target individual cancer cells and varying dosing schedules of standard drugs.

Additional Chemotherapy

The third stage of treatment consists of between five to eight months of additional chemotherapy. The entire treatment process may take close to one year to complete.

Recurrence

Osteosarcomas have less than a five percent chance of recurring in the original location of the disease after surgical resection. It is most common for an osteosarcoma to metastasize (spread) to the lungs. Less commonly, osteosarcoma can spread to other bones.

Patients treated for osteosarcoma will have the area of the original tumor examined radiographically with x-rays and other imaging techniques at three to six month intervals for three to five years. At the same time, CT scans of the lungs will be performed at regular intervals since early discovery of lung lesions (tumor cells that have spread to the lungs) allows for easier removal by surgery. Bone scans are performed periodically to watch for spread to other bones. The area where the tumor was removed and the limb reconstructed will be followed with regular x-rays yearly for the remainder of the patient’s life, depending on the type of reconstruction used. The orthopedic surgeon will monitor the healing of the reconstruction or the durability of the metal prosthesis and provide suggested exercises to increase the function of the affected limb.

Drug therapy with doxorubicin (Adriamycin) can potentially affect the heart. Patients with osteosarcoma who have received this drug as part of their therapy will need to have echocardiograms and electrocardiograms performed at least every three years. Certain types of exercise are better for the survivor’s heart and should be discussed as part of follow-up care. In addition, hearing should be checked within the year of completing therapy and then three to five years later because some of the common drugs can cause minor or more severe hearing loss.

Osteosarcomas in Adults

Classic osteosarcomas are high grade and start within the bone. With time they can destroy the bone and break out into the surrounding soft tissues.

A telangiectatic osteosarcoma is a high grade osteosarcoma that starts in the bone and can break through into the surrounding tissues. It is called telangiectatic because it is filled with large blood-filled spaces and has a different appearance on x-rays and under the microscope.

Some high grade osteosarcomas are a result of receiving prior radiation for a different cancer. Patients with Paget’s Disease are also at higher risk of developing osteosarcoma in the bone that is affected by the Paget’s Disease.

Osteosarcoma Treatment in Adults

Chemotherapy

In general, treatment for classic and telangiectatic osteosarcomas in adults is similar to treatment for the disease in children. In the first stage of treatment, chemotherapy (treatment with anticancer drugs) is given before surgery to shrink the main tumor. It is called neoadjuvant chemotherapy as it is often given before the tumor is removed by surgery. Neoadjuvant chemotherapy for osteosarcoma became standard of care at the beginning of the era when surgeons began repairing diseased limbs. New prosthesis to replace the limb required time to manufacture since they were custom-made to the patient’s size. Thus, chemotherapy was used to deliver treatment while the prosthesis was being made. Physicians found that three or four cycles of chemotherapy with multiple drugs given before surgery may help kill off more tumor cells so that it is easier to surgically remove the remaining tumor. Radiation therapy (treatment with targeted x-rays) is rarely used but may be recommended to control pain in late stage disease or if the cancer is centralized in the spine.

Surgery

The second stage of treatment is to remove the main tumor through surgery. After the tumor is surgically removed, pathologists study it to reveal a percentage necrosis rate, or the amount of dead tumor cells. The higher the percentage necrosis rate, the more dead tumors cells, and this figure represents, in essence, a “report card” to guide the medical/pediatric oncologist on how to tailor additional therapy. Studies have shown that the necrosis rate is highly predictive of overall survival. Some recent studies indicate that altering the type of chemotherapy drug given after surgery may not improve survival of patients with low necrosis rates. Medical oncologists are testing different strategies to combat aggressive tumors including therapies that target individual cancer cells and varying dosing schedules of standard drugs.

Additional Chemotherapy

The third stage of treatment consists of between five to eight months of additional chemotherapy. The entire treatment process may take close to one year to complete.

Osteosarcomas have less than a five percent chance of recurring in the original location of the disease after surgical resection. It is most common for an osteosarcoma to metastasize (spread) to the lungs. Less commonly, osteosarcoma can spread to other bones.

Parosteal, Periosteal and Other Osteosarcomas

Surface osteosarcomas include parosteal and periosteal osteosarcomas. Parosteal osteosarcomas are low grade and grow on the surface of the bone. These tumors are easily removed by surgery but it is important to be sure the entire tumor is removed as these cancers have a high chance of recurrence if not removed properly. Chemotherapy is not necessary to treat these tumors. Periosteal osteosarcomas are very rare and also occur on the surface of the bone. They are intermediate in grade and are treated with surgery and possibly chemotherapy.

Other bone tumors occurring in adults include: malignant fibrous histiocytoma or MFH (which is similar to osteosarcoma and treated similarly), fibrosarcoma, adamantoma (which occurs in the tibia), and chordoma (occurring in the sacrum or base of the skull).

All bone tumors in adults are treated with surgery. Most also are treated with chemotherapy pre-operatively and often post-operatively depending on the grade and degree of spread of the tumor. Radiation has proven ineffective in most adult bone tumors.

The most common primary malignant bone tumor in adults is multiple myeloma, which is not a sarcoma. This tumor can involve the bone marrow and multiple different bony sites and is treated with chemotherapy and radiation. Surgery is reserved for stabilizing a bone to prevent fracture.

 

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