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.
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:
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:
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:
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:
The following test may be done on the tissue that is removed:
The prognosis may be affected by certain factors before and after treatment.
The prognosis of untreated osteosarcoma and UPS may depend on the following:
After osteosarcoma or UPS is treated, prognosis also depends on the following:
Treatment options for osteosarcoma and UPS depend on the following:
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.
The following tests and procedures may be used to find out if the cancer has spread:
Cancer can spread through tissue, the lymph system, and the blood:
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.
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.
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.
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.
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:
For information about types and monitoring of late effects in childhood and adolescent cancer survivors, see Late Effects of Treatment for Childhood Cancer.
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, patients are given chemotherapy to kill any cancer cells that are left in the area where the tumor was removed or that have spread to other parts of the body. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).
Combination chemotherapy is the use of more than one anticancer drug.
Chemotherapy is usually given before and after surgery to remove the primary tumor.
For more information, see Drugs Approved for Bone Cancer.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
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 or used together with other treatments.
Samarium is a radioactive drug that targets areas where bone cells are growing, such as tumor cells in 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 after treatment in a different bone.
Treatment with samarium may be followed by stem cell transplant. Before treatment with samarium, stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After treatment with samarium is complete, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, or other molecules involved in the growth of benign or malignant tumor cells. There are different types of targeted therapy:
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:
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.
Information about ongoing clinical trials is available from the NCI website.
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. 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.
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).
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed localized osteosarcoma and UPS of bone may include the following:
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 information about the treatments listed below, see the Treatment Option Overview section.
Lung Metastasis
When osteosarcoma or UPS spreads, it usually spreads to the lung. Treatment of newly diagnosed osteosarcoma and UPS with lung metastasis may include the following:
Bone Metastasis or Bone with Lung Metastasis
Newly diagnosed osteosarcoma and UPS may spread to a distant bone and/or the lung. Treatment may include the following:
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 information about the treatments listed below, see the Treatment Option Overview section.
Treatment of recurrent osteosarcoma and UPS of bone may include the following:
Treatment depends on the area and type of recurrence, for example:
Clinical trials for treatment of recurrent osteosarcoma and UPS of bone may include the following:
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 information from the National Cancer Institute about osteosarcoma and UPS, see the following:
For more childhood cancer information and other general cancer resources, visit:
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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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