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Chordoma is a rare type of malignant (cancerous) tumor that can develop anywhere along the spine. It is a serious cancer that occurs mostly in middle-aged and older people.

Treatment is usually a combination of surgery and radiation, and can be difficult because of the location of the tumor.

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What Is Chordoma? 

Doctors define chordoma as a slow-growing, serious cancer of the tissue inside the spine. These tumors can occur anywhere along your backbone. They most often form near the sacrum (base of the spine), the coccyx (tailbone), or the base of the skull. 

Chordomas can be life-threatening. About 30% metastasize (spread) to other organs in the body, most often the lungs. Even if the tumors don’t spread, they can cause serious health issues. 

Tailbone pain is one of the most common symptoms of chordoma. In addition to tailbone pain, symptoms of cancer include headaches, vision problems, and numbness in arms and legs.

Because chordomas grow slowly, many people don’t notice them for years. The tumor may grow large before they have any symptoms.

Chordomas tend to come back, even after treatment. 

How common is chordoma?

Chordoma cancer is rare. It's diagnosed in one in a million people worldwide each year, according to the National Cancer Institute. About 300 people in the U.S. receive a chordoma diagnosis each year. 

Chordoma makes up 1% to 4% of all malignant bone tumors. 

Most people who have chordoma are diagnosed between ages 50 and 70, although the cancer can occur at any age. More men get chordomas than women. They very rarely occur in children.

What are the types of chordoma?

There are three types of chordoma:

  • Classic or conventional chordoma. The most common type of chordoma, they make up about 80% to 90% of all cases, according to the Chordoma Foundation. The cells have a unique bubbly appearance and grow slowly. They contain brachyury protein, which is a hallmark of chordoma and sets it apart from other bone tumors.
  • Dedifferentiated chordoma. Accounting for less than 5% of all chordoma cases, this aggressive form of tumor is most common in the base of the spine (sacrum). It's more likely to spread to other parts of the body than other forms of chordoma.
  • Poorly differentiated chordoma. A very rare type, it was first recognized by the World Health Organization (WHO) in 2020. It's an aggressive form of the disease more common in younger people.

Chordomas can occur at any point along your spine.

  • About 20% of chordomas occur in the vertebrae of the upper, middle, or lower back.
  • About 30% of chordomas grow in the base of the skull, where the spine joins the skull. They're called clival chordomas. 
  • About 50% of chordomas develop in the base of the spine near the tailbone. They're called sacral chordomas.

What causes chordoma? 

Chordoma develops from the notochord, a cartilage-like structure formed in the first few weeks of fetal development. The spine replaces the notochord before birth. But if some notochord cells get left behind, they can turn into chordomas.

There's no known cause for why chordomas form in some people and not others. Chordoma doesn't appear to be triggered by environmental or lifestyle factors. It’s not caused by a pre-existing health condition or by using any medicines.

Chordoma also doesn’t seem to run in families, although there are rare cases of more than one person in a family having chordoma. 

Some people with chordoma have small changes in their genes. Researchers are using this information to try to discover how chordomas form.

What are chordoma risk factors and complications?

Because there's no known cause of chordomas, risk factors are also unknown. Complications include the cancer spreading to other parts of the body.

Chordoma risk factors

There are no known risk factors for chordoma.

Complications of chordoma

As a tumor grows, it can press on nerves in the brain and spine, causing pain, numbness, and vision changes. Some chordomas can spread to other organs — most commonly the lungs — leading to disability and eventually death.

How can I prevent chordoma?

Since chordoma isn’t caused by lifestyle choices or environmental factors but occurs spontaneously, there’s nothing you can do to prevent it.

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What Are the Signs and Symptoms of Chordoma?

The symptoms of chordoma vary depending on where the tumor is located on the spine. Tailbone pain cancer symptoms are different from bone cancer skull pain.

A chordoma in the skull may produce:

  • Double vision.
  • Headaches.
  • Trouble swallowing.
  • Vision problems.

A chordoma in the spine may cause:

  • Clumsiness.
  • Numbness or tingling in the back, arms, or legs.
  • Pain at the location of the tumor. The tumor can press on nearby nerves and cause symptoms similar to a slipped disk.
  • Weakness or pain in the arms or legs.

A chordoma in the tailbone may cause:

  • A lump large enough to be felt through the skin.
  • Bowel and bladder problems.
  • Numbness in the groin.
  • Pain running down the legs.
  • Tingling or weakness in the legs.

When should I see a doctor about my chordoma symptoms?

Because chordoma is usually a slow-growing cancer, you may not notice any signs at first. But it's important to call your doctor if you have any of the symptoms above. The sooner treatment for chordoma begins, the more likely you’ll have a good outcome.

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How Do You Diagnose Chordoma?

Your primary care provider will likely refer you to an orthopaedic specialist if they suspect a chordoma. A specialist has experience with various bone tumors.

What to expect during your visit

Your doctor will take a complete medical history and ask you about any health problems that run in your family. They'll ask about your symptoms, and how long you’ve been having them.

Your doctor will also do a physical exam. 

Tests to diagnose chordoma

Some of the symptoms of chordoma —such as back pain and tingling — can be mistaken for other health problems. Tests will help your doctor confirm a diagnosis.

Imaging tests

To diagnose chordoma, your doctor may run the following imaging tests:

  • CT scan, to see if the tumor has spread.
  • MRI, to determine the size and location of the tumor.
  • X-rays. Chordomas don’t show up on x-rays, but the test may reveal bone damage caused by the tumor.


If the imaging tests indicate a chordoma, your doctor will do a biopsy to confirm the diagnosis. They'll take a small sample of tissue from the tumor. A pathologist will look at it under a microscope to study the cells and identify what kind of tumor it is. 

Additional tests for chordoma

If your doctor verifies the tumor is a chordoma, they'll do more tests to determine if the cancer has spread, and how far. These tests may include:

  • Blood tests.
  • Bone scans.
  • CT scans of the lungs. 

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How Do You Treat Chordoma? 

Chordomas can be very difficult to treat. The tumors are often enmeshed in the delicate tissues, nerves, and blood vessels of the spinal column.

Surgery for chordoma

Doctors usually treat chordoma with surgery to remove as much of the tumor as possible. It can be difficult to get rid of all the tumor and not harm the essential nerves and blood vessels of the spine. The surgeon must remove the tumor and a portion of healthy tissue around it to make sure the cancer doesn’t spread.

The risks of surgery include a loss of function in some areas of the body, such as:

  • Incontinence (loss of bowel or bladder control).
  • Loss of sensation in the groin area.
  • Pelvic instability.
  • Weakness of the legs.

Radiation to treat chordoma

Radiation alone rarely cures chordoma, but doctors will often use it along with surgery. Because it's often difficult to remove all the cancer with surgery, high-dose radiation can help kill any remaining cancer cells.

Your doctor may recommend proton beam therapy, a type of radiotherapy where beams of protons are used instead of beams of x-rays. Proton beam therapy is more targeted than traditional radiation, so it does less damage to the healthy tissues around the tumor. It allows doctors to treat sensitive areas near the spinal cord.

Side effects of proton beam therapy include:

  • Fatigue.
  • Hair loss.
  • Redness like sunburn. 

Medication for chordoma

Chemotherapy usually isn't effective against chordomas. The exception is dedifferentiated chordomas, which grow faster than other forms of chordoma and are more sensitive to chemotherapy.

How effective is treatment?

The outcome of your treatment will depend on many factors. They include:

  • If the entire tumor was able to be removed.
  • The method of treatment.
  • Where the tumor was located.
  • Your age and overall health. 

Your doctor will recommend coming back for MRI scans at regular intervals. You may need to keep having these scans for the rest of your life, to make sure the cancer hasn’t come back.

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Learn More About Chordoma

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