Osteosarcoma and Bone Fibrous Histiocytoma

Osteosarcoma is a disease in which cancer (malignant) cells are found in the bone. It is the most common type of bone cancer. Osteosarcoma most often occurs in adolescents and young adults. In children and adolescents, tumors appear most often in the bones around the knee. The symptoms and chance for recovery in children and adolescents appear to be the same.

Malignant fibrous histiocytoma (MFH) of bone is a rare tumor of the bone. It may occur following radiation treatments. MFH is generally treated the same as osteosarcoma and appears to have a similar response to treatment.

Ewing sarcoma is another kind of bone cancer, but the cancer cells look different under a microscope than osteosarcoma cancer cells. (See page onEwing Family of Tumors Treatment for more information.

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 may recommend seeing a specialist called an orthopedic oncologist. The orthopedic oncologist may 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 are any cancer cells. This test may be done in the hospital.

Prognosis

The prognosis (chance of recovery) is affected by certain factors before and after treatment.

The prognosis of untreated osteosarcoma depends on the following:

• The location of the tumor.
• The size of the tumor.
• The stage of the cancer (whether it spread from where it started to other places in the body).
• The age of the patient.
• The results of blood tests and other tests.
• The type of tumor (based on how the cancer cells look under a microscope).

After osteosarcoma is treated, prognosis also depends on the following:

• How much of the cancer was killed by chemotherapy; and/or
• How much of the tumor was taken out by surgery.

Treatment

Treatment options depend on the following:

• The location of the tumor.
• The stage of the cancer.
• Whether the cancer has recurred (come back) after treatment.
• The patient's age and general health.
• Having certain genetic diseases such as Rothmund-Thomson syndrome.

Stage Explanation

Stages of osteosarcoma

Once osteosarcoma has been found, more tests may be 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, most 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 doctor 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 osteosarcoma

The cancer cells have not spread beyond the bone or nearby tissue in which the cancer began.

Metastatic osteosarcoma

The cancer cells have spread from the bone in which the cancer began to other parts of the body. The cancer most often spreads to the lungs. It may also spread to other bones. About one in five patients with osteosarcoma has cancer that has metastasized by the time it is diagnosed.

In multifocal osteosarcoma, tumors appear in 2 or more bones, but have not spread to the lungs.

Recurrent

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. Osteosarcoma most often recurs in the lung. When osteosarcoma recurs, it is usually within 2 to 3 years after treatment is completed. Later recurrence is possible, but rare.

Treatment Option Overview

How osteosarcoma is treated

If it is suspected that the problem is osteosarcoma, before the first biopsy, your doctor may recommend a specialist called an orthopedic oncologist.

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

• Surgery (taking out the cancer in an operation).
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• Chemotherapy (using drugs to kill cancer cells).
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• Radiation therapy (using high- dose x-rays to kill cancer cells).
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In addition to these standard therapies, a treatment called biologic therapy is being tested for localized and metastatic osteosarcoma. Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

All patients with localized osteosarcoma should have surgery to remove the tumor, if possible. The doctor may remove only the cancer and some of the healthy tissue around the cancer (limb-sparing surgery). When the tumor is in a weight-bearing bone, the bone should be protected during activity to avoid fractures that could prevent limb-sparing surgery. 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, researchers have found no difference in overall survival whether patients have limb-sparing surgery or whether they have surgery with amputation. When the cancer can be taken out without amputation, artificial devices or bones from other places in the body can be used to replace the bone that was removed. The process of rebuilding (reconstructing) a part of the body changed by previous surgery is called reconstructive surgery. Options for reconstructive surgery in patients with osteosarcoma depend on many factors, including where the tumor is, how large it is, the age of the patient, and how much the patient will continue to grow.

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.

Sometimes chemotherapy is injected directly into the area where the cancer is found (regional chemotherapy). In osteosarcoma, surgery is often used to remove the local tumor and chemotherapy is then given to kill any cancer cells that remain in the body. Chemotherapy given after surgery has removed the cancer is called adjuvant chemotherapy. Chemotherapy can also be given before surgery to shrink the cancer so that it can be removed during surgery; this is called neoadjuvant chemotherapy.

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

A patient may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or may choose to go into a clinical trial. Not all patients are cured with standard therapy, and some standard treatments may have unwanted side effects. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials for osteosarcoma are ongoing in many parts of the country. If you want more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include physical problems; changes in mood, feelings, thinking, learning or memory; and having second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. See the PDQ summary on [/cancertopics/pdq/treatment/lateeffects/Patient Late Effects of Treatment for Childhood Cancer] for more information.

Localized Osteosarcoma and Malignant Fibrous Histiocytoma of Bone

Treatment of osteosarcoma may be the following:

• Neoadjuvant chemotherapy followed by surgery followed by adjuvant chemotherapy.
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• Clinical trials of new methods of giving chemotherapy and new schedules of treatment.
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• A clinical trial of high-dose radiation therapy with or without chemotherapy.
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• A clinical trial of chemotherapy with or without biologic therapy.
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Treatment of malignant fibrous histiocytoma of bone may be the following:

• Neoadjuvant chemotherapy followed by wide local excision of the tumor.

Treatment of osteosarcoma that develops following radiation therapy includes chemotherapy and surgery.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with localized osteosarcoma and localized childhood malignant fibrous histiocytoma of bone.

Metastatic Disease at Diagnosis

Metastatic disease is cancer that has spread from the place in which it started to other parts of the body. When the cancer has spread to the lungs, the prognosis is better if the cancer is in only one lung and in fewer places in the lung. For cancer that has spread to the bone, prognosis is better if the tumors are all in the same bone.

Osteosarcoma

Treatment of osteosarcoma with metastasis may be one of the following:

• Chemotherapy followed by surgery to remove the cancer followed by adjuvant chemotherapy.
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• Surgery to remove the cancer followed by adjuvant chemotherapy.
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• A clinical trial of chemotherapy with or without biologic therapy.
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Surgery often includes removal of cancer that has spread to the lungs.

Malignant fibrous histiocytoma of bone

Treatment of malignant fibrous histiocytoma of bone with metastasis is the same as for osteosarcoma with metastasis.

Multifocal osteosarcoma

Treatment of multifocal osteosarcoma includes chemotherapy and surgery.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic osteosarcoma and metastatic childhood malignant fibrous histiocytoma of bone.

Recurrent Osteosarcoma

Treatment depends on where the cancer recurred, what kind of treatment was given before, as well as other factors. A clinical trial may be 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 and peripheral stem cell support as palliative therapy, to relieve pain and improve quality of life.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent osteosarcoma and recurrent childhood malignant fibrous histiocytoma of bone.

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