You may not have heard of this name "chordoma" before. That's not surprising, because this is a relatively rare type of cancer. However, even though it is rare, it is very important to be aware of this. Because, this type of tumor can occur in our spine or at the base of the skull. So today, let's talk in detail and very simply about what this chordoma is, how it develops, what are the symptoms, and what are the treatments.
What is Chordoma? Let's find out simply!
Simply put, a chordoma is a cancerous tumor that forms in the bones . It most often develops in your spine or in the base of your skull, the bones above your neck and below your brain. Doctors classify it as a type of cancer called sarcoma. Sarcoma is a general term for cancers that form in the bones and soft tissues (connective tissues).
Chordomas usually grow slowly . However, the problem is that they can be a bit challenging to treat because they are located very close to our nervous system, that is, the brain and spinal cord, and invade the delicate tissues around them.
Another thing is that even after treatment, these tumors have a high chance of recurring . Most often, this happens in the same place where the original tumor was. Also, in 30% to 40% of cases, chordoma cancer cells can spread to other parts of the body. Doctors call this ``metastasizing''.
Where can Chordoma spread to other parts of the body?
If a chordoma metastasizes, that is, spreads to other parts of the body, the most common places it can spread are:
- Your lungs
- Lymph nodes near the tumor
- Other bones
- Liver
- Skin
Where can chordoma develop?
Chordoma can occur anywhere along your spine, but there are three most common locations:
1. The sacrum: This is the triangular bone between your buttocks. About 35% of chordomas develop here.
2. Skull base: Specifically, at the base of your skull, in the bones that support your brain. These are also called ``Clival chordomas'' because they most often form in the ``Clivus'' bone, which is located here. This type also accounts for about 35% of all chordomas.
3. In the moving vertebrae of the spine (`vertebrae`): That is, the vertebrae in your neck, chest, and back. Chordomas occur in about 30% of these. They are most common in the second cervical vertebra. They can also occur in the vertebrae in the lower back (lumbar spine) and chest (thoracic spine).
What are the main types of chordoma?
The World Health Organization (WHO) has divided chordoma into three main types. These classifications are based on how the cancer cells look under a microscope (`histology`).
1. Classic/conventional chordoma: This is the most common type (80% to 90% of all chordomas). The cells in this type are a special type of cell that has a slightly "bubble" appearance. Chondroid chordoma is another variant of this type. It accounts for between 5% and 15% of all chordomas. Chondroid chordoma most often develops at the base of the skull.
2. Dedifferentiated chordoma: This is a very rare type (less than 5%). It is characterized by an abnormal mixture of cells. This type is faster growing, more aggressive, and more likely to metastasize to other parts of the body than other types.
3. Poorly differentiated chordoma: This is also very rare . Less than 60 patients have been recorded in medical records so far. A special feature of this type is that a gene called `(SMARCB1)` or `(INI1)` is inactivated (deletion). This type of chordoma is most often seen in children and young people.
Who is most likely to develop chordoma?
Chordoma can occur at any age, in anyone. However, it is most common in adults between the ages of 50 and 80. Children are affected by only a small number of cases, about 5% of all cases.
It has also been found that men are about 1.5 times more likely to develop chordoma than women.
How common is this?
Chordoma is a very rare cancer . It affects about one in a million people each year. That means, for example, in the United States, only about 300 new cases are diagnosed each year.
Chordomas account for between 1% and 4% of all primary bone tumors.
What are the symptoms of a chordoma?
As a chordoma grows, it can put pressure on the surrounding spinal cord or brain. This pressure causes symptoms . Symptoms can also vary depending on where the tumor is located in the spine.
Common symptoms of chordoma include pain, weakness, and/or numbness in the back, arms, or legs .
Characteristics of a skull base chordoma:
If you have a chordoma at the base of your skull, which is under your brain, you may experience symptoms like:
- Double vision (Diplopia)
- Blurred vision
- Headache
- Facial numbness or pain
Characteristics of a chordoma in the tailbone/sacrum:
If you have a chordoma at the lowest part of your spine, near the sacrum, you may experience symptoms like:
- A lump or bump that protrudes through the skin
- Difficulty urinating or defecating (bladder or bowel dysfunction)
- Pain in the lower back or buttocks area
Keep in mind, sometimes these symptoms can be confused with other common medical conditions. That's why it's important to seek medical advice if you have any persistent, unusual symptoms.
Why does Chordoma develop? What are the causes?
Researchers still don't know exactly what causes chordoma, but they believe that mutations in a gene called TBXT are involved.
There have been reports of several families with chordoma. When they were tested, they were found to have inherited a duplication of the `(TBXT)` gene. Also, changes in the `(TBXT)` gene have been identified in people who have chordoma but no one in the family has the disease.
Chordoma develops from cells in a structure called the ``notochord.`` This ``notochord`` is a temporary structure that is very important for the development of the spinal cord of an embryo when it is developing. Usually, this ``notochord`` disappears by about eight weeks of gestation. However, in a very small number of people, some of these ``notochord`` cells may remain in the bones of the spine or the base of the skull.
So, the current view is that if there is a change in the previously mentioned `(TBXT)` gene, these remaining `(notochord)` cells may start to grow abnormally and form a chordoma.
Also, people with a genetic condition called ``Tuberous sclerosis`` are at increased risk of developing chordoma. This ``Tuberous sclerosis`` condition can cause epilepsy, developmental delay, and tumors in various parts of the body. This is caused by mutations in two genes called ``TSC1`` and ``TSC2``.
How to recognize a chordoma?
If you have symptoms of chordoma, a doctor will first ask about your symptoms and medical history. Then they will perform a physical exam and a neurological exam.
If the doctor suspects a tumor, he or she will order an imaging test such as an X-ray, a computed tomography (CT) scan, or an MRI scan. This MRI scan is often helpful in diagnosing a chordoma.
Then, your doctor will likely refer you to a bone cancer specialist to confirm the diagnosis and get a second opinion. They may also do further imaging tests to determine the exact location of the chordoma, see if it has spread to other parts of the body, and perform other tests.
However, the only way to definitively diagnose chordoma is to have a biopsy . This involves taking a small sample of the lump, usually using a needle biopsy, and examining it under a microscope. Only then can a specialist tell for sure whether it is a chordoma.
What are the treatments for Chordoma?
The main and best treatment for chordoma is to remove the entire tumor through surgery . Doctors call this ``En bloc resection.`` If the tumor is completely removed in this way, the patient has the best chance of living for a long time.
However, depending on where the tumors are located, this can be very difficult to do. In particular, it is often impossible to completely remove chordomas located at the base of the skull.
Because a chordoma in the spine can damage your spinal cord and the important nerves and blood vessels around it. If this is damaged during surgery, it can cause long-term disability or even death. Also, a chordoma at the base of the skull is difficult to completely remove because it is so close to the brainstem, cranial nerves, and spinal cord. Neurosurgeons try to remove as much of the tumor as possible, while being as safe as possible.
Chordomas usually do not respond well to radiation therapy or chemotherapy as primary treatment. However, after surgery, your medical team may recommend radiation therapy to prevent the tumor from coming back.
Researchers are currently investigating new treatments for chordoma, such as targeted therapy and immunotherapy. There may even be clinical trials you can participate in.
Can chordoma be prevented?
Honestly, there's nothing you can do to prevent chordomas from developing. Most of the time, they develop randomly.
However, if someone in your family has had Chordoma, or if you have a condition called ``Tuberous sclerosis,'' it is important that you see a doctor regularly for checkups. They can then monitor you for signs of Chordoma. The sooner this is identified, the more likely it is that treatment will be successful.
What are the chances of recovery from a chordoma? (Prognosis)
The prognosis, or outlook, for a chordoma depends on several factors:
- The location of the tumor and how much can be removed surgically: If the tumor can be completely removed, the chances of recovery are high. If only other treatments are used without surgery, the chances are low.
- Whether the cancer has spread to other parts of the body (metastasized): If it has spread to distant sites, survival rates may be reduced.
- Your age when diagnosed: In general, survival rates are slightly lower for people over 60 years of age.
- Whether the tumor is dedifferentiated or not: Types like ``Poorly differentiated chordoma`` have a worse prognosis than ``conventional chordoma``.
Your medical team can give you the most accurate information about what to expect based on your condition, so don't be afraid to ask them questions.
Could this be fatal?
Yes, chordoma can be fatal. Most often, death occurs after the tumor has recurred, destroying tissue in your spinal cord, brain, or brainstem.
A study of 357 chordoma patients revealed that the survival rates were as follows:
- After three years: 80.5%
- After five years: 68.4%
- Ten years later: 39.2%
But remember, these are just averages. Your medical team can provide more detailed information about survival rates based on your specific situation.
Is Chordoma Cancer or a Normal Tumor?
Chordoma is definitely malignant (cancerous). None of its subtypes are considered noncancerous.
When should I see a doctor?
Chordoma can recur even after treatment, even years later. Therefore, long-term follow-up with your medical team is very important.
If you develop any new symptoms, or if your existing symptoms get worse , be sure to talk to your doctor.
Finally, a few things to remember (Take-Home Message)
It's normal to feel scared and stressed when you learn that you have a rare bone cancer. Chordoma is a cancer that is difficult to treat because of its location.
However, trust that your medical team will create a detailed treatment plan that is tailored to you and will help you treat your chordoma and improve your quality of life .
- It's important to recognize symptoms early: seek medical advice if you have persistent pain, numbness, or changes in vision.
- Seek expert medical advice: Since chordoma is a rare disease, it is very important to seek treatment from an experienced medical team.
- Regular check-ups are necessary even after treatment: As there is a risk of recurrence of the disease, attend the check-ups as directed by the doctors.
I hope you find this information helpful. If you have any questions, don't hesitate to ask your doctor.
` Chordoma, cancer, bone cancer, spine, skull, sacrum, clivus
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