Osteosarcoma & Malignant Fibrous Histiocytoma of Bone Treatment

Osteosarcoma and undifferentiated pleomorphic sarcoma (UPS) of bone are diseases in which malignant (cancer) cells form in bone.

Osteosarcoma usually starts in osteoblasts, which are a type of bone cell that becomes new bone. Osteosarcoma is most common in adolescents. It commonly forms in the ends of the long bones of the body, which include bones of the arms and legs. In children and adolescents, it often forms in the long bones, near the knee. Rarely, osteosarcoma may be found in soft tissue or organs in the chest or abdomen.

Osteosarcoma is the most common type of bone cancer. UPS (formerly called malignant fibrous histiocytoma [MFH]) is a rare type of bone cancer that usually starts in soft tissue, but it may form in bone. In bone, UPS cells look similar to osteosarcoma under a microscope. UPS is treated like osteosarcoma.

Ewing sarcoma is another kind of bone cancer, but it is not covered in this summary. For more information, see Ewing Sarcoma Treatment.

Having past treatment with chemotherapy or radiation can increase the risk of osteosarcoma.

Anything that increases a person's risk of getting a disease is called a risk factor. Not every child with one or more of these risk factors will develop osteosarcoma, and it will develop in some children who don't have any known risk factors. Talk with your child's doctor if you think your child may be at risk. Risk factors for osteosarcoma include the following:

• Past treatment with chemotherapy or radiation therapy.
• Having a certain change in the RB1 gene.
• Having certain conditions, such as the following:
  • Bloom syndrome.
  • Diamond-Blackfan anemia.
  • Li-Fraumeni syndrome.
  • Paget disease.
  • Hereditary retinoblastoma.
  • Rothmund-Thomson syndrome.
  • Werner syndrome.

Signs and symptoms of osteosarcoma and UPS include swelling over a bone or a bony part of the body and joint pain.

These and other signs and symptoms may be caused by osteosarcoma or UPS or by other conditions. Check with a doctor if your child has any of the following:

• Swelling over a bone or bony part of the body.
• Pain in a bone or joint.
• A bone that breaks for no known reason.

Imaging tests are used to detect (find) osteosarcoma and UPS.

In addition to asking about your child's personal and family health history and doing a physical exam, your child's doctor may perform the following tests and procedures:

• X-ray: An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

A biopsy is done to diagnose osteosarcoma.

Imaging tests are done before a biopsy.

Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. It is important that the biopsy be done by a surgeon who is an expert in treating cancer of the bone. It is best if that surgeon is also the one who removes the tumor. The biopsy and the surgery to remove the tumor are planned together. The way the biopsy is done affects which type of surgery can be done later.

The type of biopsy that is done will be based on the size of the tumor and where it is in the body. There are two types of biopsy that may be used:

• Core biopsy: The removal of tissue using a wide needle.
• Incisional biopsy: The removal of part of a lump or a sample of tissue that doesn't look normal.

The following test may be done on the tissue that is removed:

• Electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.

Certain factors may affect prognosis (chance of recovery) and treatment options.

The prognosis may be affected by certain factors before and after treatment.

The prognosis of untreated osteosarcoma and UPS may depend on the following:

• Where the tumor is in the body and whether tumors formed in more than one bone.
• Whether the patient has two or more tumors in the same bone.
• The size of the tumor.
• Whether the cancer has spread to other parts of the body and where it has spread.
• The type of tumor (based on how the cancer cells look under a microscope).
• The patient's sex, age, and weight at the time of diagnosis.
• Whether the patient has had treatment for a different cancer.
• Whether the patient has certain genetic diseases.

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

• How much of the cancer was killed by chemotherapy.
• Whether the tumor has been completely removed by surgery.
• Whether the cancer has recurred (come back) within 2 years of diagnosis.

Treatment options for osteosarcoma and UPS depend on the following:

• Where the tumor is in the body and if it has spread.
• The size of the tumor.
• The grade of the cancer.
• Whether the bones are still growing.
• The patient's age and general health.
• The desire of the patient and family for the patient to be able to take part in activities such as sports or to look a certain way.
• Whether the cancer is newly diagnosed or has recurred after treatment.

After osteosarcoma or undifferentiated pleomorphic sarcoma (UPS) has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The process used to find out if cancer has spread to other parts of the body is called staging. Most patients with osteosarcoma and UPS are grouped according to whether cancer is localized or metastatic.

• Localized osteosarcoma or UPS has not spread out of the bone where the cancer started. There may be one or more areas of cancer in the bone that can be removed during surgery.
• Metastatic osteosarcoma or UPS has 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.

The following tests and procedures may be used to find out if the cancer has spread:

• X-ray: An x-ray of the organs, such as the chest, and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. X-rays will be taken of the chest and the area where the tumor formed.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. Pictures will be taken of the chest and the area where the tumor formed.
• PET scan: A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. This procedure is also called positron emission tomography (PET) scan.
• PET-CT scan: A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if osteosarcoma spreads to the lung, the cancer cells in the lung are actually osteosarcoma cells. The disease is metastatic osteosarcoma, not lung cancer.

Sometimes osteosarcoma and UPS of bone come back after treatment.

The cancer may recur (come back) in the bone or in other parts of the body. Osteosarcoma and UPS most often recur in the lung, bone, or both. When osteosarcoma recurs, it is usually within 18 months after treatment is completed.

There are different types of treatment for patients with osteosarcoma or undifferentiated pleomorphic sarcoma (UPS).

Different types of treatment are available for children with osteosarcoma or UPS of bone. 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.

Children with osteosarcoma or UPS should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating osteosarcoma and UPS and who specialize in certain areas of medicine. These may include the following specialists:

• Pediatrician.
• Orthopedic surgeon who has experience treating bone tumors.
• Radiation oncologist.
• Rehabilitation specialist.
• Pediatric nurse specialist.
• Social worker.
• Child-life specialist.
• Psychologist.
• Fertility specialist.

For information about types and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer.

Five types of standard treatment are used:

Treatment for osteosarcoma or UPS may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

• Physical problems, such as infertility.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer), such as breast cancer or acute myeloid leukemia.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. For more information, see Late Effects of Treatment for Childhood Cancer.

New types of treatment are being tested in clinical trials.

Information about ongoing clinical trials is available from the NCI website.

Patients may want to think about taking part 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.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back).

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Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of osteosarcoma and undifferentiated pleomorphic sarcoma (UPS) (formerly called malignant fibrous histiocytoma [MFH]) of bone. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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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." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

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PDQ® Pediatric Treatment Editorial Board. PDQ Osteosarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389380]

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