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Osteosarcoma/Malignant Fibrous Histiocytoma of Bone Treatment (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 05/23/2008
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Table of Contents

Description
What is osteosarcoma?
Stage Explanation
Stages of osteosarcoma
Localized osteosarcoma
Metastatic osteosarcoma
Recurrent
Treatment Option Overview
How osteosarcoma is treated
Treatment in a clinical trial
Localized Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
Metastatic Disease at Diagnosis
Recurrent Osteosarcoma
To Learn More About Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
Get More Information From NCI
Changes to This Summary (05/23/2008)
About PDQ

Description



What is osteosarcoma?

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 the PDQ summary on Ewing 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.

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 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.

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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.

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Treatment Option Overview



How osteosarcoma is treated

Different types of treatment are available for children with osteosarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

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

Three kinds of standard treatment are used:

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).

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 Late Effects of Treatment for Childhood Cancer for more information.

Treatment in a clinical trial

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. In the following lists of treatments for the different stages, a link to search results for current clinical trials is included for each section. These have been retrieved from NCI's clinical trials database. For some types or stages of cancer, there may not be any trials listed. Check with your child's doctor for clinical trials that are not listed here but that may be right for your child.

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Localized Osteosarcoma and Malignant Fibrous Histiocytoma of Bone

Treatment of osteosarcoma may be the following:

Treatment of malignant fibrous histiocytoma of bone may be the following:

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

Check for U.S. 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.

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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:

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 U.S. 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.

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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 U.S. 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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To Learn More About Osteosarcoma and Malignant Fibrous Histiocytoma of Bone

For more information from the National Cancer Institute about osteosarcoma and malignant fibrous histiocytoma of bone, see Bone Cancer: Questions and Answers.

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

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Get More Information From NCI

Call 1-800-4-CANCER

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Changes to This Summary (05/23/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

  • Information about patients taking part in clinical trials was added to the Treatment Option Overview section.
  • Links to ongoing clinical trials listed in NCI’s PDQ Cancer Clinical Trials Registry were added to the treatment sections.
  • A new section called “ To Learn More ” was added. It includes links to more information about this type of cancer and about cancer in general.
  • The “Get More Information from NCI” section (originally called “To Learn More”) was revised

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About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

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