Have you ever heard of the rare bone cancer called chordoma? Let us explore what it means for your health

You may not have heard the word chordoma before today. That is completely normal because this is a very rare type of cancer. Even though it is rare, learning about it helps you stay informed and prepared. Chordoma is a rare tumor that can start in your spine or at the base of your skull. Let us take a close look at what a chordoma is, how it happens, what signs to look for, and what medical treatments are available today.

Discovering that you or a loved one has a rare medical condition can feel scary and overwhelming. Having clear, reliable information makes a big difference. Medical experts and patient health educators work hard to break down complex medical terms into simple, everyday language. Let us walk through everything you need to know about chordoma step by step.

What Is a Chordoma? Let Us Understand It Simply!

Put simply, a chordoma is a type of cancerous tumor that develops in your bones. These tumors most often form in your spine or at the base of your skull right between your neck and your brain. Doctors classify chordoma as a type of sarcoma. Sarcoma is a general medical term used for cancers that start in your bones and soft connective tissues.

Chordoma tumors usually grow very slowly. The main challenge with chordomas is that they grow very close to vital parts of your nervous system, including your brain and spinal cord. Because they invade nearby delicate tissues, treating them can be difficult and complex for doctors.

Another important factor is that even after successful treatment, chordomas have a high chance of coming back. This is known as medical recurrence. When a chordoma returns, it usually happens in the exact same spot where the original tumor was located. also, in about 30 percent to 40 percent of cases, chordoma cancer cells can spread to other parts of your body. Doctors call this process metastasis.

Where Can Chordoma Spread in Your Body?

If a chordoma metastasizes. This it travels to other parts of your body, it most commonly spreads to specific areas. You should know where these areas are located:

• Your lungs, which is one of the most frequent sites for distant spread.
• Lymph nodes that sit close to the original tumor location.
• Other bones throughout your skeletal system.
• Your liver, though this is less common.
• Your skin, in rare metastatic cases.

Where Can Chordomas Develop in Your Body?

A chordoma can develop anywhere along your spine. However, doctors see them most often in three main locations:

1. The base of your spine, known as the sacrum: This is the triangular bone located right between your hip bones in your lower back. About 35 percent of all chordomas develop in this exact area.

2. The skull base where your skull meets your spine: Specifically, this happens in the bones at the very bottom of your skull that support your brain. Doctors often call these clival chordomas because they often form on a specific bone called the clivus. This type also accounts for about 35 percent of cases.

3. The movable bones of your spine, called vertebrae: These are the bones in your neck, chest, and lower back. About 30 percent of chordomas form here. They appear most often in the second vertebra of your neck. They can also develop in your lower back, known as the lumbar spine, and in your chest area, known as the thoracic spine.

What Are the Main Types of Chordoma?

The World Health Organization, known as the WHO, divides chordomas into three main types. Doctors classify them based on how the cancer cells look under a microscope, which is called histology.

1. Classic or conventional chordoma: This is by far the most common type, accounting for 80 percent to 90 percent of all chordomas. The cells in this type have a special appearance that looks a bit like tiny bubbles. Chondroid chordoma is another variant of this type and makes up about 5 percent to 15 percent of cases. Chondroid chordomas usually develop at the base of your skull.

2. Dedifferentiated chordoma: This is a very rare type that accounts for less than 5 percent of cases. It contains a mixed group of abnormal cells. This type grows much faster, is more aggressive, and has a higher chance of spreading or metastasizing to other parts of your body compared to other types.

3. Poorly differentiated chordoma: This is an extremely rare type of chordoma. Medical literature has recorded fewer than 60 patients with this specific diagnosis worldwide. A key feature of this type is that a specific gene called SMARCB1 or INI1 is missing or inactive. Doctors usually find this type of chordoma in children and young adults.

Who Gets Chordoma the Most?

A chordoma can affect anyone at any age, but it is diagnosed most often in adults between 50 and 80 years old. Children rarely get chordomas, accounting for only about 5 percent of all patients.

Studies also show that men are about 1.5 times more likely to develop a chordoma than women.

How Common Is This Condition?

Chordoma is an extremely rare medical condition. It affects roughly one person out of every million people each year. For example, in the United States, doctors diagnose only about 300 new patients each year.

Out of all primary bone tumors that start in the skeletal system, chordomas make up only 1 percent to 4 percent of cases.

What Are the Symptoms of a Chordoma?

As a chordoma tumor slowly grows larger, it presses against nearby parts of your spine or brain. This physical pressure creates your symptoms. Your specific symptoms will depend entirely on where the tumor is growing in your body.

In general, common symptoms of a chordoma include persistent pain, along with weakness, numbness, or tingling sensations in your back, arms, or legs.

Symptoms of a Skull Base Chordoma.

If you have a chordoma growing at the base of your skull underneath your brain, you might experience specific warning signs:

• Seeing double, which medical professionals call diplopia.
• Blurry vision or other sudden changes in how well you see.
• Frequent headaches that do not go away with standard medicine.
• Numbness, tingling, or pain in your face.

Symptoms of a Sacral Chordoma in Your Tailbone Area.

If you have a chordoma in the lower end of your spine near your tailbone and sacrum, you may notice different signs:

• A noticeable lump or mass that you can feel through your skin.
• Trouble controlling your bladder or bowels, or difficulty urinating.
• Persistent pain in your lower back or tailbone area.

Sometimes these symptoms mimic other common health issues, which is why visiting a doctor for persistent or unusual health signs is so important.

Why Does Chordoma Happen? What Are the Causes?

Researchers do not yet know the exact cause of chordomas. However, medical scientists believe that changes or mutations in a specific gene called TBXT play a major role.

In rare family histories, multiple family members have developed chordomas. When doctors tested these families, they discovered that an extra copy or duplication of the TBXT gene was inherited. also, researchers have found genetic changes in the TBXT gene in patients who have no family history of the disease at all.

Chordomas develop from cells left behind by a temporary structure in your body called the notochord. During normal embryonic development, the notochord helps build your spine while you grow in the womb. This structure usually disappears by the eighth week of pregnancy. However, in a tiny number of people, small remnants of notochord cells remain trapped inside the bones of the spine or skull base.

If a genetic change occurs in the TBXT gene, these leftover notochord cells can start growing in an abnormal way, eventually forming a chordoma tumor. also, people with a genetic condition called tuberous sclerosis have a higher risk of developing tumors. Tuberous sclerosis can cause epilepsy, delayed development, and growths in various organs due to mutations in the TSC1 and TSC2 genes.

How Do Doctors Diagnose a Chordoma?

If you experience symptoms that suggest a chordoma, your doctor will start by asking about your medical history and current health signs. Next, they will perform a thorough physical examination and a neurological exam to check your nerve function.

If your doctor suspects a bone tumor, they will order imaging tests such as an X-ray, a computed tomography scan, known as a CT scan, or a magnetic resonance imaging scan, known as an MRI scan. An MRI scan is especially helpful for diagnosing chordomas because it shows soft tissues and nerves in great detail.

Your doctor will likely refer you to a bone cancer specialist to confirm your diagnosis and help you get a second opinion. Additional imaging tests help doctors pinpoint the exact tumor location and check whether the cancer has spread to other parts of your body.

The only way to confirm a chordoma diagnosis with absolute certainty is to perform a biopsy. During a biopsy, a doctor uses a thin needle to take a tiny tissue sample from the tumor. A specialist examines this sample under a microscope to confirm whether it is a chordoma.

What Treatments Are Available for Chordoma?

The primary and most effective treatment for chordoma is surgery to remove the tumor completely. Surgeons call this an en bloc resection. Removing the entire tumor in one piece gives you the best chance for long-term survival.

However, performing this surgery can be extremely difficult depending on where the tumor is located. Skull base chordomas are often impossible to remove entirely because they sit right next to critical brain structures.

Similarly, spinal chordomas grow very close to your spinal cord, major blood vessels, and essential nerves. Damaging these structures during surgery can lead to permanent disability or even death. Neurosurgeons work carefully to remove as much of the tumor as safely possible while protecting your nervous system.

Standard radiation therapy and chemotherapy do not work very well as primary treatments for chordoma. However, your medical team may recommend specialized radiation therapy after surgery to help lower the chances of the tumor coming back.

Medical researchers continue studying new treatment options, including targeted therapy and immunotherapy. Clinical trials exploring these advanced treatments may also be available to qualifying patients.

Can You Prevent Chordoma?

To be completely honest, there is currently no known way to prevent chordoma. These rare tumors usually occur due to random, unpredictable genetic events.

However, if you have a family history of chordomas or if you have tuberous sclerosis, you should visit your doctor for regular checkups. Routine medical monitoring helps doctors detect any early warning signs promptly. Catching a tumor early gives you the best opportunity for successful medical treatment.

What Is the Prognosis and Outlook for Chordoma?

Your prognosis, which is your expected health outlook, depends on several key factors:

• Tumor location and surgical removal: If surgeons can remove the entire tumor, your outlook is much better than if surgery is not possible.
• Distant spread: If the cancer has metastasized to other parts of your body, your long-term outlook is more challenging.
• Your age at diagnosis: Older adults over the age of 60 often face lower survival rates compared to younger patients.
• Tumor subtype: Aggressive types like poorly differentiated chordoma have a poorer prognosis compared to conventional chordoma.

Your medical team can give you personalized information about your specific outlook. Never hesitate to ask them questions about your diagnosis and care plan.

Can Chordoma Be Fatal?

Yes, chordoma can be fatal. Fatalities usually happen when a tumor returns after treatment and destroys vital tissues in your spinal cord, brain, or brainstem.

Data from a clinical study of 357 chordoma patients showed the following survival rates:

• Three-year survival rate: 80.5 percent .
• Five-year survival rate: 68.4 percent .
• Ten-year survival rate: 39.2 percent .

Keep in mind that these are general statistical averages. Your medical specialists can provide precise details tailored specifically to your personal health situation.

Is Chordoma Cancer or a Benign Growth?

Chordoma is always a malignant, cancerous tumor. No subtype of chordoma is considered noncancerous or benign.

When Should You See a Doctor?

Even after successful treatment, chordomas can return years later. Because of this risk, long-term follow-up care with your medical team is essential.

If you notice any new symptoms, or if your previous symptoms start getting worse, you should contact your doctor right away.