Osteosarcoma is a rare type of bone cancer that starts in the cells called osteoblasts, which form new bone. In osteosarcoma, the cancer cells make osteoid, the early form of bone tissue. Osteosarcoma usually forms in the long bones of the arms and legs, and over half of the people with osteosarcoma have it in the long bones near the knee. While rare, it can also form in other bones or, in very unusual cases, in soft tissue or organs in the chest or abdomen.
This cancer is most common in adolescents and young adults. In the United States, about 440 cases of osteosarcoma are diagnosed each year in people 19 years and younger.
Undifferentiated pleomorphic sarcoma (UPS) is another rare cancer that can start in bones. UPS of the bone used to be called malignant fibrous histiocytoma. Under a microscope, UPS of the bone can look similar to osteosarcoma. However, UPS does not produce osteoid.
Osteosarcoma and UPS are treated the same way.
Ewing sarcoma is another kind of bone cancer. Learn more at Ewing Sarcoma Treatment.
A risk factor is anything that increases the chance of getting a disease. 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 a known risk factor. Risk factors for osteosarcoma include:
Talk with your child's doctor if you think your child may be at risk.
The symptoms of osteosarcoma and UPS depend on which bone it forms in. It's important to check with your child's doctor if your child has:
These symptoms may be caused by osteosarcoma, UPS, or other problems. The only way to know is for your child to see a doctor.
If your child has symptoms that suggest bone cancer, the doctor will need to find out if they are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family health history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with osteosarcoma or UPS, the results of these tests will help you and your child's doctor plan treatment.
The tests used to diagnose osteosarcoma or UPS may include:
Imaging tests are done before a biopsy.
The type of biopsy depends on the size of the tumor and where it is in the body. There are two types of biopsy that may be used:
The following test may be done on the tissue that is removed:
The process used to find out whether the cancer has spread within the bone or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.
The following imaging tests and procedures may be used to find out if osteosarcoma or UPS has spread:
You may want to get a second opinion to confirm your child's cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes to the treatment plan, or provide more information about your child's cancer.
To learn more about choosing a doctor and getting a second opinion, visit Finding Health Care Services. You can contact NCI's Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your child's appointments, visit Questions to Ask Your Doctor About Cancer.
If your child has been diagnosed with osteosarcoma or UPS, you likely have questions about how serious the cancer is and your child's chances of survival. The likely outcome or course of a disease is called prognosis.
Many factors affect prognosis and treatment options.
Factors that may affect your child's prognosis before treatment include:
Factors that may affect your child's prognosis after treatment include:
No two people are alike, and response to treatment can vary greatly. Your child's cancer care team is in the best position to talk with you about your child's prognosis.
Treatment options for osteosarcoma and UPS depend on:
Cancer stage describes the extent of cancer in the body, such as the size of the tumor, whether it has spread, and how far it has spread from where it first formed. It is important to know the stage of the osteosarcoma to plan the best treatment. Instead of being grouped by stage, osteosarcoma and UPS are grouped according to whether the cancer is localized or metastatic.
The cancer may 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.
You and your child's care team will work together to decide treatment. Many factors will be considered, such as where the cancer is located and your child's age and overall health.
Your child's treatment plan will include information about the tumor, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child's care team before treatment begins about what to expect. For help every step of the way, visit our booklet, Children with Cancer: A Guide for Parents.
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of osteosarcoma. The pediatric oncologist works with other health care providers who are experts in treating osteosarcoma and UPS and who specialize in certain areas of medicine. Other specialists may include:
Surgery to remove the entire tumor will be done when possible. Chemotherapy may be given before surgery to make the tumor smaller. This is called neoadjuvant chemotherapy. Chemotherapy is given so less bone tissue needs to be removed and there are fewer problems after surgery.
The following types of surgery may be done:
Studies have shown that survival is the same whether the first surgery done is a limb-sparing surgery or an amputation.
After the doctor removes all the cancer that can be seen at the time of the surgery, chemotherapy is given to kill any cancer cells that are left. Treatment given after the surgery to lower the risk that the cancer will come back is called adjuvant therapy.
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment.
Chemotherapy for osteosarcoma and UPS is injected into a vein. When given this way, the drugs enter the bloodstream and can reach cancer cells throughout the body. Chemotherapy is usually given before and after surgery to remove the tumor.
Chemotherapy drugs used alone or in combination to treat osteosarcoma include:
Other chemotherapy drugs not listed here may also be used.
Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer.
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Osteosarcoma is treated with external beam radiation therapy. This type of therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
Osteosarcoma and UPS cells are not killed easily by external radiation therapy. It may be used when a small amount of cancer is left after surgery, used together with other treatments, or used as palliative therapy to relieve symptoms caused by the tumor in the bone.
Samarium is a radioactive drug that targets tumor cells in a bone. It helps relieve pain caused by cancer in the bone and it also kills blood cells in the bone marrow. It is used to treat osteosarcoma that has come back in a different bone after treatment.
Treatment with samarium may be followed by stem cell transplant. Before treatment with samarium, stem cells (immature blood cells) are removed from the child's blood or bone marrow and are frozen and stored. After treatment with samarium is complete, the stored stem cells are thawed and given back to the child through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child's age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child's treatment team about which side effects to look for and ways to manage them.
To learn more about side effects that begin during treatment for cancer, visit Side Effects.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
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. Learn more about Late Effects of Treatment for Childhood Cancer.
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).
Treatment of newly diagnosed localized osteosarcoma and UPS of bone may include:
Learn about these treatments in the Treatment Option Overview.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Lung Metastasis
When osteosarcoma or UPS spreads, it usually spreads to the lung. Treatment of newly diagnosed osteosarcoma and UPS that has spread to the lung may include chemotherapy followed by surgery to remove the primary cancer and more chemotherapy. Then, the doctor will remove the cancer in the lung and give additional chemotherapy.
Bone Metastasis or Bone with Lung Metastasis
Osteosarcoma and UPS may spread to a distant bone or to both the lung and a distant bone. Treatment of newly diagnosed osteosarcoma and UPS that has spread to a distant bone or to both the lung and a distant bone may include:
Learn more about these treatments in the Treatment Option Overview.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment depends on where the cancer recurred and whether it has recurred more than once.
Learn more about these treatments in the Treatment Option Overview.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
For more childhood cancer information and other general cancer resources, visit:
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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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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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