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.

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:

• Headache.
• Double vision.
• Blocked or stuffy nose.
• Trouble speaking.
• Trouble swallowing.
• Neck or back pain.
• Pain down the back of the legs.
• Numbness, tingling, or weakness of the arms and legs.
• A change in bowel or bladder habits.

Tests that examine the spine are used to help diagnose chordoma.

The following tests and procedures may be used:

• Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• MRI (magnetic resonance imaging): A procedure that 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 also called nuclear magnetic resonance imaging (NMRI).
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the brain and spine, 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.

• Biopsy: The removal of cells or tissues using a needle so they can be viewed under a microscope by a pathologist to check for signs of cancer. The sample of tissue may also be checked for a high level of a protein called brachyury.

KEY POINTS

• 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.
• There are three ways that cancer spreads in the body.
• Cancer may spread from where it began to other parts of the body.
• Sometimes childhood chordoma comes back after treatment.

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:

• 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.

• 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.

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 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.

Treatment Option Overview

KEY POINTS

• There are different types of treatment for children with chordoma.
• Children with chordoma should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
• Two types of standard treatment are used:
  • Surgery
  • Radiation therapy
• New types of treatment are being tested in clinical trials.
  • Targeted therapy
• Treatment for childhood chordoma may cause side effects.
• Patients may want to think about taking part in a clinical trial.
• Patients can enter clinical trials before, during, or after starting their cancer treatment.
• Follow-up tests may be needed.

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:

• Pediatrician.
• Pediatric neurosurgeon.
• Radiation oncologist.
• Pathologist.
• Pediatric nurse specialist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.

Two types of standard treatment are used:

Surgery

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

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.

Targeted therapy

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.

Treatment of Childhood Chordoma

Treatment of newly diagnosed chordoma in children may include the following:

• Surgery to remove as much of the tumor as possible, followed by radiation therapy. Proton beam radiation therapy may be used for tumors near the base of the skull.

Treatment of Recurrent Childhood Chordoma

Treatment of recurrent chordoma in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change. Patients with changes in the SMARCB1 gene may be treated with tazemetostat in this clinical trial.

Certain factors affect prognosis (chance of recovery).

The prognosis depends on the following:

• The child's age.
• Where the tumor forms 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 just been diagnosed or has recurred (come back).