Causes of Chordoma
Written by: Aaron Cohen-Gadol, MD
Last Updated: September 30, 2024
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Chordoma is a rare type of cancer found primarily at the ends of the spine, such as the skull base or sacrum. Understanding the origins of chordoma can provide valuable insight into why they manifest at these locations. In this article, we delve into the current understanding of the potential causes of chordoma formation and address common questions related to this condition.
How Do You Get Chordoma?
The exact cause of chordoma is poorly understood, but it is thought to result from the abnormal growth of notochord cells. These cells primarily help to develop the spinal cord and normally disappear prior to birth. In rare cases, notochord cells continue to grow in certain locations within the spine and give rise to chordomas.
Some studies have suggested that chordomas might be associated with mutations in certain genes, such as the T, SMARCB1, PTEN, and CDKN2A genes. These mutations may lead to the notochordal cells growing and dividing uncontrollably, forming a tumor.
Other potential factors that may increase the risk of developing a chordoma include excessive exposure to ionizing radiation, or exposure to chemicals like vinyl chloride, though this link has not been well established. Most cases of chordoma occur in people without any known risk factors, and the exact cause of chordoma is not yet known.
What Are My Chances of Getting a Chordoma?
Chordoma is a rare type of cancer, with an estimated annual incidence rate of about 1 to 3 cases per million people. It accounts for less than 1% of all bone tumors, and it is slightly more common in men than women. A chordoma will generally appear without any obvious cause or risk factors.
Although the risk factors for chordoma are not well understood, studies have suggested that people with certain genetic conditions, such as tuberous sclerosis, may have an increased risk of developing a chordoma.
If you are concerned about your risk of chordoma or are experiencing symptoms that are concerning, consult with a medical professional as soon as possible. They can help to determine if you are at risk and recommend appropriate screening and monitoring measures.
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At What Age Can a Chordoma Occur?
A chordoma can occur at any age, but it is more common in older adults. The majority of chordomas are diagnosed in people between the ages of 40 and 70 years. However, they have also been found in infants and older adults. The age range of people with a chordoma can vary depending on the location of the tumor; tumors located in the sacrum (the lower part of the spine) typically appear in older patients, and tumors located in the skull base tend to occur in younger ones.
Chordoma is a rare cancer, and the chance of developing a chordoma, as with most cancers, generally increases with age. However, if you are experiencing symptoms or have a family history of the condition, you should seek medical attention regardless of your age.
Does Chordoma Run in Families?
Chordoma doesn’t usually run in families. However, there have been a few reported cases of families with multiple members affected by chordoma, which suggests that there might be a genetic component to the development of this cancer.
Chordoma has also been reported in other genetic conditions, such as tuberous sclerosis. The link between chordoma, a nearly one-in-a-million cancer, and a rare genetic disorder is not well established due to the scarcity of reports.
If you have a family history of chordoma or a genetic condition associated with an increased risk of developing chordoma, discuss this history with your doctor. They might recommend genetic counseling and testing, as well as regular screenings for early detection of the cancer.
Key Takeaways
- Chordoma is a rare type of cancer that affects the skull and spinal cord, and its cause is not well understood.
- The incidence of chordoma is about 1 to 3 cases per million people each year, and it is more common in older adults (40−70 years old).
- There have been a few reported cases of families with multiple members affected by chordoma, which suggests that there could be a genetic component.
- Most cases of chordoma occur in people without any known risk factors, and the exact cause of chordoma is not yet known.
