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Home > Living Well > Health Library > Childhood Chordoma Treatment (PDQ®): Treatment - Patient Information [NCI]
This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
Childhood chordoma is a disease in which malignant (cancer) cells form in the tissue found inside the spine.
Chordoma is a type of slow-growing tumor that forms anywhere along the spine, from the base of the skull (a bone called the clivus) to the tailbone. In children and adolescents, chordomas form most often in the tissue near the spine at the base of the skull or the tailbone, making them hard to remove completely with surgery.
Anatomy of the spine. The spine is made up of bones, muscles, tendons, nerves, and other tissues that reach from the base of the skull near the spinal cord (clivus) to the coccyx (tailbone). The vertebrae (back bones) of the spine include the cervical spine (C1-C7), thoracic spine (T1-T12), lumbar spine (L1-L5), sacral spine (S1-S5), and the tailbone. Each vertebra is separated by a disc. The vertebrae surround and protect the spinal cord. The spinal cord is divided into segments, each containing a pair of spinal nerves that send messages between the brain and the rest of the body. Many spinal nerves extend beyond the conus medullaris (the end of the spinal cord) to form a bundle of nerves called the cauda equina.
Signs and symptoms of chordoma depend on where the tumor forms in the tissue in the spine.
These and other signs and symptoms may be caused by chordoma or by other conditions.
Check with your child's doctor if your child has any of the following:
Tests that examine the spine are used to help diagnose chordoma.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery).
The prognosis depends on the following:
After chordoma has been diagnosed, tests are done to find out if cancer cells have spread within the spine or to other parts of the body.
The process used to find out whether the cancer has spread within the spine or to other parts of the body is called staging. The information gathered from the staging process is used to plan treatment. The results of some of the tests and procedures used to diagnose chordoma are also used to stage the disease.
The following tests and procedures may also be used to find out if cancer has spread:
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
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.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if chordoma spreads to the lung, the cancer cells in the lung are actually chordoma cells. The disease is metastatic chordoma, not lung cancer.
Sometimes childhood chordoma comes back after treatment.
The cancer may come back in the area where it first formed or in other parts of the body, such as the bone or lung.
There are different types of treatment for children with chordoma.
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 chordoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
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 professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:
Two types of standard treatment are used:
Whether a chordoma can be completely removed depends on where the chordoma formed. If it formed in or near the brain, or by important nerves or blood vessels, it cannot be completely removed by surgery without causing harm to the child.
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. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. Proton beam radiation therapy is a type of high-energy, external radiation therapy that aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. It may be used to treat childhood chordoma.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
Tazemetostat is being studied for the treatment of childhood chordoma that has recurred (come back).
Treatment for childhood chordoma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
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.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order 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). These tests are sometimes called follow-up tests or check-ups.
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed chordoma in children 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.
Treatment of recurrent chordoma in children may include the following:
For more information from the National Cancer Institute about childhood chordoma, see the following:
For more childhood cancer information and other general cancer resources, see the following:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood chordoma. 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.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
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.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary]."
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Chordoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/bone/patient/child-chordoma-treatment-pdq. Accessed <MM/DD/YYYY>.
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Last Revised: 2020-11-19
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