Chordoma: Let's learn about this rare cancer that develops in the spinal cord.

Have you ever heard of the name 'Chordoma'? Probably not. Because it is a very rare, that is, a malignant condition that is rarely seen. This cancerous tumor develops in two very important places in our body. One is our spine, and the other is the base of our skull. It is reasonable to feel a little scared when the word cancer is mentioned in such a place. But don't worry. We will talk about everything in a simple, clear, and understandable way for you.

Simply put, what is Chordoma?

Chordoma is a cancer that develops in our bones. To be precise, it belongs to a group of cancers called sarcomas. It is a very slow-growing cancer. This means that it can remain asymptomatic for a long time.

This cancer can develop anywhere in the spine, but there are three most common locations:

• The lowest point of the spine (Sacrum): About 35% of all chordomas develop in this area.
• Skull base: Another 35% of cases develop here. The bone at this point is called the 'clivus'. This type is also called 'clival chordomas'.
• Other vertebrae of the spine: The remaining 30% occur in other parts of the spine. They are most common in the second cervical vertebra, followed by the lumbar and thoracic vertebrae.

The biggest challenge with this cancer is that, although it grows slowly, it invades important parts of the surrounding nervous system. This means that it can damage very sensitive areas such as the brain and spinal cord.

Another thing is that it has a high tendency to recur, even after treatment. It usually comes back in the same place it was before. Also, in about 30% - 40% of cases, this cancer can spread (metastasize) to other parts of the body. If it does, it most commonly spreads to:

• To the lungs
• To the surrounding lymph nodes
• To other bones
• To the liver
• To the skin

Are there types of Chordoma?

Yes, the World Health Organization (WHO) has identified three main types of this cancer. These classifications are based on how the cancer cells look under a microscope (histology). Let's see what they are.

Who is most likely to develop this disease? How common is it?

Although chondroma can develop at any age, it is most common in adults between the ages of 50 and 80. A small percentage, about 5%, develop it in children. Also, men are about 1.5 times more likely to develop the disease than women.

This is so rare that only one in a million (100,000) people in the population develops this disease each year . This means that even in a country like ours, very few new cases are reported each year.

What are the symptoms of this?

As the chordoma grows, it puts pressure on the surrounding brain or spinal cord. This pressure causes symptoms. Also, symptoms can vary depending on the location of the tumor.

Let's see in the table below how the symptoms vary depending on the location of the lump.

Why does this type of cancer develop? What is the cause?

Researchers are still unable to pinpoint the exact cause, but they believe that mutations in a gene called TBXT are involved.

Think about it, when a baby is first developing in the mother's womb, before the spine develops, a small skeleton called the notochord forms. This is what will later develop into the spinal cord. Usually, this notochord disappears completely by the time the baby is about 8 weeks old.

But very rarely, some of these cells can remain in the base of the skull or in the bones of the spine. It is believed that later, if there is a change in the TBXT gene that we talked about, these remaining cells can start to grow uncontrollably and become a chondroma cancer.

Additionally, people with the genetic condition tuberous sclerosis are at slightly higher risk of developing chordoma.

How to diagnose the disease? (Diagnosis)

If you have the symptoms listed above, you should first see your doctor. The doctor will ask you about your symptoms and your medical history. Then, they will perform a physical exam and a neurological exam.

If a cancerous lump is suspected, the doctor will refer you for imaging tests.

• X-ray
• Computed Tomography (CT) scan
• Magnetic Resonance Imaging (MRI) scan

After these tests, you will likely be referred to a doctor who specializes in bone cancer. Further tests may be needed to confirm the disease and see if it has spread to other parts of the body.

The only way to be 100% certain about the disease is to have a biopsy , which involves taking a very small sample of tissue from the lump using a small needle and examining it under a microscope.

How is it treated?

The main and best treatment for chordoma is surgery. The best results for the patient are achieved by removing as much of the cancerous lump as possible (en bloc resection).

However, this is not as easy as it seems because, as we discussed earlier, these tumors form near very sensitive, important organs and nerves.

• Tumors at the base of the skull: These are very difficult to completely remove because they are located near the brainstem, important nerves, and blood vessels.
• Spinal tumors: These can invade the spinal cord, nerves, and major blood vessels. If these are damaged during surgery, permanent disability or even death can occur.

Therefore, neurosurgeons try to remove the tumor as much as possible without harming the patient.

Chordoma cancer usually does not respond well to radiation therapy and chemotherapy, so these are rarely used as primary treatments. However, radiation therapy is given after surgery to destroy any remaining cancer cells and reduce the risk of the cancer coming back.

Researchers are currently investigating modern treatments such as targeted therapy and immunotherapy.

What is the prognosis of the disease?

"Prognosis" is a prediction of the likelihood of recovery and survival from a disease. In the case of chondroma, this depends on several factors:

• The location of the tumor and how much was removed during surgery: If the tumor was completely removed, the results are better.
• Whether the cancer has spread (metastasized): If it has spread to distant organs in the body, the survival rate decreases.
• Age at diagnosis: Results may be slightly lower in people over 60 years of age.
• Type of cancer: Conventional types have better outcomes than the more aggressive types like "Dedifferentiated" that we discussed.

According to one study, the survival rates of chordoma patients are as follows:

• After 3 years: 80.5%
• After 5 years: 68.4%
• After 10 years: 39.2%

Can this disease be prevented? When should I see a doctor?

There is nothing we can do to prevent the development of chondroma. Most cases occur randomly. If someone in your family has chondroma or tuberous sclerosis, it is important that you have regular medical checkups.

The most important thing is long-term follow-up after treatment. Chondroma is a cancer that can recur even years after treatment. Therefore, it is essential to go for regular check-ups as advised by your medical team.

If you develop any new symptoms or if your symptoms worsen, see your doctor immediately.

It's normal to feel scared and overwhelmed when you learn about such a rare cancer. But remember, your medical team will work with you to develop the best treatment plan for you and do their best to improve your quality of life.

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