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Bone Tumor: Osteogenic Sarcoma

Osteogenic Sarcoma

Osteogenic Sarcoma (also called Osteosarcoma) is a primary bone cancer that is seen in adolescents and young adults. It is the most common type of bone cancer. In children, it occurs most commonly in the bones around the knee.

Ewing's sarcoma is another kind of bone cancer, but the cancer cells are different under a microscope than osteosarcoma cancer cells.

Like most cancers, osteosarcoma is best treated when it is found (diagnosed) early, that is, when it is a localized disease. If a patient has symptoms (such as pain and swelling of a bone or a bone region), a doctor may order x-rays and blood tests. If it is suspected that the problem is osteosarcoma, your doctor should recommend that you see a pediatric oncologist. If the pediatric oncologist suspects osteosarcoma, they will work with and refer you to an orthopedic oncologist. The orthopedic oncologist will cut out a piece of tissue from the affected area. This is called a biopsy. The tissue will be looked at under a microscope to see if there is any cancer cells. This test may be done in the hospital, and it is a critical step. The best thing you can do for your child, if a bone cancer is suspected, is work with a pediatric oncologist so that a surgeon experienced in bone cancer does the biopsy. The operation must be done by a surgeon selected by the pediatric oncologist so that it is done with future reconstructive surgery in mind.

The chance of recovery (prognosis) and choice of treatment depend on the size, location, type and stage of the cancer (how far the cancer has spread), how long the patient had symptoms, how much of the cancer is taken out by surgery and/or killed by chemotherapy, the patient's age, blood test results, and general health. However, the only feature that consistently predicts outcome is the degree of cell kill (necrosis) following the chemotherapy that was given before definitive surgery.

Stages of osteosarcoma

Once osteosarcoma has been found, more tests are done to find out if cancer cells have spread to other parts of the body. This is called staging. At present, there is no staging system for osteosarcoma. Instead, patients are grouped depending on whether cancer is found in only one part of the body (localized disease) or whether the cancer has spread from one part of the body to another (metastatic disease). Your oncologist needs to know where the cancer is located and how far the disease has spread to plan treatment. The following groups are used for osteosarcoma:

Localized

The cancer cells have not spread beyond the bone or nearby tissue in which the cancer began. In young patients most tumors occur around the knee.

Metastatic

The cancer cells have spread from the bone in which the cancer began to other parts of the body. This is the case in 10-20% of patients at diagnosis. The cancer most often spreads to the lungs. It may also spread to other bones.

Recurrence of disease

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the tissues where it first started or it may come back in another part of the body.

How osteosarcoma is treated

There are treatments for all patients with osteosarcoma. Three kinds of treatment are usually used:

  • surgery (taking out the cancer in an operation)
  • chemotherapy (using drugs to kill cancer cells)
  • radiation therapy (using high-dose x-rays to kill cancer cells)
Surgery

Surgery is a common and vital treatment for osteosarcoma. The surgeon will attempt to remove the cancer after the first cycles of chemotherapy. This may include removing some of the healthy tissue around the cancer. Sometimes all or part of an arm or leg may have to be removed (amputated) to make sure that all of the cancer is taken out. If cancer has spread to lymph nodes, the lymph nodes will be removed (lymph node dissection).

In patients with osteosarcoma that has not spread beyond the bone, surgery without amputation of the arm or leg (limb-sparing procedures) will most likely be recommended. Sometimes artificial devices or bones from other places in the body can be used to replace the bone that was removed. Pre-operative chemotherapy increases the number of patients who are able to undergo limb-sparing operations.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill or put into the body by a needle in a vein or muscle. Chemotherapy is called systemic treatment because the drug enters the blood stream, travels through the body, and can kill cancer cells throughout the body. Chemotherapy with more than one drug is called combination chemotherapy.

Chemotherapy may be injected directly into the area where the cancer is found (regional chemotherapy). Chemotherapy can be given before surgery to shrink the cancer so that it can be removed during surgery; this is called neoadjuvant chemotherapy. Chemotherapy is also given after surgery (called adjuvant chemotherapy) in an attempt to kill any remaining cancer cells left in the body.

Radiation therapy

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation for osteosarcoma usually comes form the machine outside the body (external radiation therapy).

Treatment by stage and clinical trial

Treatment for osteosarcoma depends on the stage of the disease, where the cancer is found, the patient's age and general health.

A patient may receive treatment that is considered standard based on its effectiveness in patients in previous clinical trials or may choose to enter into a clinical trial. Clinical trials are designed to compare potentially better therapy with therapy already accepted as standard of care in hope of finding better ways to treat cancer patients. Clinical trials for osteosarcoma are going on in many parts of the country. The good news is that Children's Oncology Group clinical trials are closely overseen by a data monitoring committee, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.

Localized osteosarcoma

Treatment will most likely be recommended as follows:

Chemotherapy will commence after the biopsy, followed by surgery to remove the cancer. After recovery from this major surgery, there will be further cycles of (adjuvant) chemotherapy. The exact regimen for adjuvant chemotherapy may be determined by degree of cellular death (necrosis) observed in the primary tumor. Relapse free survival ranges between 50-80%. Clinical trials are evaluating new methods of giving chemotherapy and new schedules of treatment. The use of radiation therapy is also under study.

Metastatic osteosarcoma

Treatment may be one of the following:

  • Chemotherapy followed by surgery to remove the cancer, followed by adjuvant- chemotherapy.
  • Surgery to remove the primary tumor and metastases followed by combination chemotherapy.
Recurrent osteosarcoma

Treatment depends on where the cancer recurred, what kind of treatment was given before, as well as other factors. A clinical trial is a reasonable treatment option.

If the cancer has come back only in the lungs, treatment may be surgery to remove the cancer in the lungs with or without chemotherapy. If the cancer has come back in other places besides the lungs, treatment may be combination chemotherapy. Clinical trials are evaluating new chemotherapy drugs.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Should you have more questions about the child or young adult in your life with cancer, please contact Cleveland Clinic Children's Cancer Answer line at 216.444.5517, option 1 or 800.223.2273, extension 4-5517, option 1. Your question will be forwarded to the Chair of the Pediatric Oncology Department.

We wish you and your child well.

For other websites regarding childhood cancer: