Chordoma is a rare malignant (cancerous) bone tumor that forms in your spine or the base of your skull. It’s a type of sarcoma.
Chordomas can occur at any point along your spine:
About 35% develop in the base of your spine (sacrum).
About 35% develop where your spine meets your skull (skull base). These are called clival chordomas because the bone in the skull base that’s most often involved is the clivus.
About 30% develop in the vertebrae of your mobile spine — most commonly the second cervical vertebrae (in your neck) followed by your lumbar spine and then thoracic spine.
Chordomas typically grow slowly, but they can be difficult to treat due to how they invade nearby nervous system tissue.
They also tend to recur (come back) after treatment — usually in the same place. Chordomas spread to other parts of your body (metastasize) in 30% to 40% of cases.
If an advanced chordoma does metastasize, it most commonly spreads to your:
Lungs.
Nearby lymph nodes.
Other bones.
Liver.
Skin.
What are the symptoms of chordoma?
As a chordoma grows, it puts pressure on nearby areas of your spinal cord or brain. This pressure causes the symptoms of chordoma. Symptoms can also vary based on where the tumor is along your spine.
General chordoma symptoms include pain, weakness and/or numbness in your back, arms or legs.
Symptoms of a chordoma at the base of your skull may include:
Researchers don’t know exactly why chordomas form. But they think changes (mutations) in the TBXT gene are likely involved.
A few families have had multiple members who’ve developed a chordoma. Studies revealed that these people inherited a duplication of the TBXT gene. Researchers have also identified changes in the TBXT gene in people with chordoma who have no family history of the condition.
A chordoma develops from cells of the notochord. This is a structure that’s present in a developing embryo and is important for the future development of its spine. The notochord usually disappears by the time the fetus is eight weeks old. But in a small percentage of people, a few notochord cells can remain embedded in the bones of the spine or the base of the skull.
A change in the TBXT gene may trigger the growth of these cells, leading to a chordoma.
People with a genetic condition called tuberous sclerosis are at higher risk of developing chordoma. This condition causes a variety of medical issues, including epilepsy, developmental delay and tumors throughout your body. Tuberous sclerosis is caused by mutations in two genes: TSC1 and TSC2.
How is chordoma treated?
The go-to treatment option for chordoma is surgery. Total surgical removal of the tumor (en bloc resection) has the best chance of prolonging survival. However, this is often difficult due to the location of the tumors. Specifically, this isn’t possible for chordomas in the base of your skull.
A chordoma in your spine can invade your spinal cord and nearby important nerves and arteries, which could cause lasting issues or death if they’re damaged during surgery. A chordoma at the base of your skull is often difficult to completely remove because it’s close to essential structures such as your brainstem, cranial nerves and spinal cord. Neurosurgeons aim to remove as much of the chordoma as they safely can.
Chordomas are generally resistant to radiation therapy and chemotherapy as primary treatments. But your healthcare team might recommend radiation therapy after surgery to lower the chance that the tumor will grow back.
Researchers are currently studying experimental therapies for chordomas, such as targeted therapy and immunotherapy. There may be clinical trials available that you can participate in.
Your healthcare provider will ask about your symptoms and medical history. They’ll likely perform a physical exam and a neurological exam.
Your provider will likely refer you to a bone cancer specialist for a second opinion and confirmation of the diagnosis. You may need additional imaging tests to better determine the location of the chordoma and see if it’s spread to other areas of your body.
The only way healthcare providers can definitively diagnose chordoma is with a biopsy — usually a needle biopsy. This involves taking a small sample of the tumor so a specialist can examine it under a microscope.
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