What is Chordoma? Symptoms, Causes, & Treatment

Chordoma is a rare type of bone cancer that develops from leftover cells from when you were an embryo. These tumors grow slowly along your spine or at the base of your skull, where your backbone once formed during early development.

While chordomas are uncommon, affecting only about 1 in 1 million people each year, understanding this condition can help you recognize symptoms and seek appropriate care. These tumors tend to grow gradually over months or years, which means early detection and treatment can make a significant difference in outcomes.

What is chordoma?

Chordoma develops from remnants of the notochord, a flexible rod-like structure that helps form your spine during embryonic development. Normally, this structure disappears as your backbone develops, but sometimes small clusters of these cells remain.

These leftover cells can eventually grow into tumors, typically appearing in two main areas. About half of chordomas occur at the base of your skull, while the other half develop in your lower spine, particularly around your tailbone area.

The tumors grow very slowly, often taking years to become large enough to cause symptoms. This gradual growth pattern means chordomas can reach a considerable size before you notice any problems, which is why they're sometimes called "silent tumors."

What are the symptoms of chordoma?

Chordoma symptoms depend heavily on where the tumor is located and how large it has grown. Since these tumors develop slowly, symptoms typically appear gradually and may be subtle at first.

When chordomas occur at the base of your skull, you might experience:

• Persistent headaches that don't respond well to typical pain relievers
• Double vision or other vision changes
• Hearing problems or ringing in your ears
• Difficulty swallowing or speaking
• Numbness or tingling in your face
• Nasal congestion that doesn't improve
• Nosebleeds without an obvious cause

For chordomas in your spine, particularly in the lower back or tailbone area, symptoms may include:

• Persistent pain in your lower back or tailbone
• Pain that worsens when sitting or lying down
• Bowel or bladder control problems
• Numbness or weakness in your legs
• A noticeable lump or mass you can feel
• Difficulty walking or changes in your gait

In rare cases, chordomas can occur in the middle part of your spine, causing back pain, arm weakness, or problems with coordination. These locations are less common but can cause significant symptoms as the tumor grows.

What causes chordoma?

Chordoma develops when cells left over from embryonic development begin growing abnormally. During your earliest stages of development, a structure called the notochord helps form your spine and then typically disappears.

Sometimes, small groups of these primitive cells remain in your body after birth. In most people, these leftover cells never cause problems and remain dormant throughout life. However, in rare cases, these cells can start dividing and growing into tumors, though we don't fully understand what triggers this process.

Most chordomas occur randomly without any clear cause or trigger. Unlike some cancers, chordomas aren't typically linked to lifestyle factors like smoking, diet, or environmental exposures. They also don't appear to be caused by infections or injuries.

In very rare instances, chordomas can run in families due to genetic changes, but this accounts for less than 5% of all cases. The vast majority of people with chordoma have no family history of the condition.

What are the types of chordoma?

Doctors classify chordomas into three main types based on how they look under a microscope. Each type has slightly different characteristics and behaviors, though all chordomas are considered rare cancers.

Conventional chordoma is the most common type, making up about 85% of all chordomas. These tumors grow slowly and have a characteristic appearance with cells that look like soap bubbles under the microscope.

Chondroid chordoma accounts for about 10% of cases and contains both chordoma cells and cartilage-like tissue. This type tends to occur more often at the base of the skull and may have a slightly better outlook than conventional chordoma.

Dedifferentiated chordoma is the rarest and most aggressive type, representing less than 5% of all chordomas. These tumors grow faster than other types and are more likely to spread to other parts of your body, making them more challenging to treat.

When to see a doctor for chordoma?

You should see your doctor if you experience persistent symptoms that don't improve with typical treatments or rest. Since chordoma symptoms can be subtle and develop slowly, it's important not to dismiss ongoing problems.

Seek medical attention if you have headaches that are different from your usual headaches, especially if they're accompanied by vision changes, hearing problems, or facial numbness. These combinations of symptoms warrant prompt evaluation.

For spine-related symptoms, see your doctor if you have persistent back or tailbone pain that doesn't improve with rest, especially if it's accompanied by bowel or bladder problems, leg weakness, or numbness. These symptoms could indicate pressure on important nerves.

Don't wait if you notice any sudden changes in your symptoms or if they're getting worse quickly. While chordomas typically grow slowly, any tumor can occasionally cause rapid changes that need immediate attention.

What are the risk factors for chordoma?

Age is the most significant risk factor for chordoma, with most cases occurring in adults between 40 and 70 years old. However, these tumors can develop at any age, including in children and teenagers, though this is less common.

Men are slightly more likely to develop chordoma than women, particularly for tumors that occur in the spine. For skull-based chordomas, the risk is more evenly distributed between men and women.

Having a rare genetic condition called tuberous sclerosis complex slightly increases your risk of developing chordoma. However, this accounts for only a very small percentage of cases, and most people with tuberous sclerosis never develop chordoma.

In extremely rare cases, chordoma can run in families due to inherited genetic changes. If you have a close family member with chordoma, your risk may be slightly higher, but this is still very uncommon and most chordomas occur in people with no family history.

What are the possible complications of chordoma?

Chordoma complications primarily result from the tumor's location and size rather than its tendency to spread throughout your body. Since these tumors grow in critical areas near your brain and spinal cord, they can cause significant problems as they enlarge.

Common complications may include:

• Permanent nerve damage causing weakness, numbness, or paralysis
• Bowel or bladder dysfunction if the tumor affects spinal nerves
• Vision or hearing loss for skull-based tumors
• Difficulty swallowing or speaking
• Chronic pain that's difficult to manage
• Mobility problems or difficulty walking

In rare cases, chordoma can spread to other parts of your body, most commonly to your lungs, liver, or other bones. This happens in about 30% of cases, typically years after the initial diagnosis. When chordoma spreads, it becomes much more challenging to treat.

Treatment complications can also occur, particularly after surgery in these delicate areas. These might include infection, cerebrospinal fluid leaks, or additional nerve damage. However, modern surgical techniques have significantly reduced these risks.

How is chordoma diagnosed?

Diagnosing chordoma typically begins with your doctor taking a detailed history of your symptoms and performing a physical examination. Since chordoma symptoms can be similar to other conditions, your doctor will likely order imaging tests to get a better look at the area.

MRI scans are the most useful imaging test for chordoma because they provide detailed pictures of soft tissues and can show the tumor's exact location and size. CT scans may also be used to see how the tumor affects nearby bone structures.

The only way to definitively diagnose chordoma is through a biopsy, where a small sample of the tumor is removed and examined under a microscope. This procedure requires careful planning since chordomas occur in delicate areas near critical structures.

Your doctor may also order additional tests like PET scans to determine if the tumor has spread to other parts of your body. Blood tests aren't typically helpful for diagnosing chordoma since these tumors don't usually produce detectable markers in your blood.

What is the treatment for chordoma?

Surgery is the primary treatment for chordoma and offers the best chance for long-term control. The goal is to remove as much of the tumor as possible while preserving important nearby structures like nerves and blood vessels.

Complete surgical removal can be challenging because chordomas often grow very close to critical structures. Your surgical team will include specialists who have experience operating in these complex areas, such as neurosurgeons and orthopedic oncologists.

Radiation therapy is typically used after surgery to treat any remaining tumor cells that couldn't be safely removed. Advanced techniques like proton beam therapy or stereotactic radiosurgery can deliver high doses of radiation precisely to the tumor while minimizing damage to surrounding healthy tissue.

Chemotherapy isn't typically effective for most chordomas, but newer targeted therapies are showing promise. Some medications that block specific growth signals in cancer cells are being studied and may be recommended in certain situations.

For tumors that can't be surgically removed, radiation therapy alone may be used to slow growth and control symptoms. Your treatment team will work with you to develop the best approach based on your specific situation.

How to manage chordoma at home?

Managing chordoma at home focuses on maintaining your quality of life and supporting your overall health during treatment. Pain management is often a crucial part of home care, and your doctor can prescribe appropriate medications to keep you comfortable.

Staying active within your physical limitations can help maintain your strength and mobility. Physical therapy exercises, as recommended by your healthcare team, can help prevent muscle weakness and maintain function in affected areas.

Eating a balanced, nutritious diet supports your body's ability to heal and cope with treatment. Focus on getting adequate protein, vitamins, and minerals, and stay well-hydrated throughout your treatment.

Managing stress and emotional well-being is equally important. Consider joining support groups for people with rare cancers, practicing relaxation techniques, or working with a counselor who understands the challenges of living with a rare condition.

Keep track of any new or changing symptoms and communicate regularly with your healthcare team. Having a journal of your symptoms, pain levels, and how you're feeling can help your doctors adjust your treatment plan as needed.

How should you prepare for your doctor appointment?

Before your appointment, write down all your symptoms, including when they started, how they've changed over time, and what makes them better or worse. This information helps your doctor understand your condition better.

Bring a complete list of all medications you're taking, including prescription drugs, over-the-counter medications, and supplements. Also, gather any previous medical records, test results, or imaging studies related to your symptoms.

Prepare a list of questions you want to ask your doctor. Important questions might include what tests you need, what treatment options are available, and what to expect moving forward. Don't hesitate to ask for clarification if something isn't clear.

Consider bringing a trusted family member or friend to your appointment. They can help you remember important information and provide emotional support during what can be a stressful time.

What's the key takeaway about chordoma?

Chordoma is a rare but serious condition that requires specialized care from experienced medical teams. While the diagnosis can feel overwhelming, advances in surgical techniques and radiation therapy have significantly improved outcomes for many people.

Early detection and treatment are crucial for the best possible outcomes. If you're experiencing persistent symptoms, especially those affecting your head, neck, or spine, don't hesitate to seek medical evaluation.

Remember that having chordoma doesn't mean you're alone. Connect with healthcare providers who specialize in rare cancers, and consider reaching out to support groups where you can share experiences with others facing similar challenges.

With proper treatment and support, many people with chordoma can maintain good quality of life. Stay informed about your condition, advocate for yourself, and work closely with your healthcare team to develop the best treatment plan for your specific situation.