Childhood Chordoma (PDQ®)–Patient Version
What is childhood chordoma?
Childhood chordoma is a very rare type of bone cancer that begins in cells left over from early development that helped form disks of the spine. These cells usually go away before birth, but sometimes they stay in the body and may become cancer in rare cases. Chordoma can form in a bone at the base of the skull called the clivus, the spine, and the tailbone. In children and adolescents, the most common location is the clivus, making them hard to remove completely with surgery. Only about 20 out of the 300 cases of chordoma diagnosed in the United States each year occur in people younger than 20 years.
Causes and risk factors for childhood chordoma
Chordoma in children is caused by certain changes in the way cells function, especially how they grow and divide into new cells. Often, the exact cause of these cell changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Children who have an inherited condition called tuberous sclerosis may be at an increased risk of chordoma. Not every child with this risk factor will develop a chordoma. And it will develop in some children who don't have a known risk factor. Talk with your child's doctor if you think your child may be at risk.
Symptoms of childhood chordoma
Children may not have symptoms of a chordoma until the tumor has grown bigger. It's important to check with your child's doctor if your child has:
These symptoms may be caused by problems other than childhood chordoma. The only way to know is to see your child's doctor.
Tests to diagnose childhood chordoma
If your child has symptoms that suggest a chordoma, the doctor will need to find out if these are due to a chordoma 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 medical history and do a physical exam. Depending on these results, they may recommend other tests. If your child is diagnosed with a chordoma, the results of these tests will help you and your child's doctor plan treatment.
The tests used to diagnose childhood chordoma may include:
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the whole spine. This procedure is called nuclear magnetic resonance imaging.
CT scan (CAT scan)
A CT scan uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the brain and spine. The pictures are taken from different angles and are used to create 3-D views of tissue and organs. 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. Learn more about Computed Tomography (CT) Scans and Cancer.
Biopsy
Biopsy is a procedure in which a sample of tissue is removed from the tumor so that a pathologist can view it under a microscope to check for signs of cancer. The sample of tissue may also be checked for a high level of a protein called brachyury.
The following laboratory tests may be done on the tissue that was removed during the biopsy:
- Immunohistochemistry uses antibodies to check for certain antigens (markers) in a sample of a patient's tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and help tell one type of cancer from another type.
- Molecular test checks for certain genes, proteins, or other molecules in a sample of tissue, blood, or other body fluid. A molecular test may be done with other procedures, such as biopsies, to help diagnose some types of cancer. Molecular tests check for certain gene or chromosome changes that occur in some cancers.
The Molecular Characterization Initiative offers free molecular testing to children, adolescents, and young adults with certain types of newly diagnosed cancer. The program is offered through NCI's Childhood Cancer Data Initiative. To learn more, visit About the Molecular Characterization Initiative.
Getting a second opinion
You may want to get a second opinion to confirm your child's 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. They 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, see Finding Cancer Care. 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, see Questions to Ask Your Doctor about Cancer.
Who treats children with chordoma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment of a chordoma. The pediatric oncologist works with other health professionals who are experts in treating children with cancer and who specialize in other areas of medicine. Other specialists may include:
Treatment of childhood chordoma
There are different types of treatment for children and adolescents with chordoma. You and your child's care team will work together to decide treatment. Many factors will be considered, such as your child's overall health and whether the tumor is newly diagnosed or has come back.
Your child's treatment plan will include information about the cancer, 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, see our booklet, Children with Cancer: A Guide for Parents.
Surgery
Surgery may be used to remove as much of the tumor as possible. If the chordoma formed in or near the brain, or by important nerves or blood vessels, it cannot be completely removed by surgery without causing harm to your child.
Radiation therapy
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. Childhood chordoma may be treated with external beam radiation therapy. External beam 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. This type of radiation therapy may be used for tumors near the base of the skull.
Learn more about External Beam Radiation Therapy for Cancer and Radiation Therapy Side Effects.
If your cancer comes back after treatment, your child's doctor will talk with you about what to expect and possible next steps. There might be treatment options that may shrink the cancer or control its growth. If there are no treatments, your child can receive care to control symptoms from cancer so they can be as comfortable as possible.
Clinical trials
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.
Prognostic factors for childhood chordoma
If your child has been diagnosed with a chordoma, 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. The prognosis can be affected by your child's age, where the tumor has formed in the tissue along the spine, how the tumor responds to treatment, whether there were changes in bowel or bladder habits at diagnosis, whether the tumor has certain gene changes, and whether the tumor has been newly diagnosed or has recurred (come back).
No two people are alike, and responses 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.
Follow-up care
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose 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).
Coping with your child's cancer
When your child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child's treatment team and to people in your family and community for support. To learn more, see Support for Families When a Child Has Cancer and the booklet Children with Cancer: A Guide for Parents.
Related resources
For more childhood cancer information and other general cancer resources, see:
About This PDQ Summary
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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.
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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Chordoma. 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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