17916 Chordoma

Overview

Chordoma is a very rare type of tumor that grows slowly in the bones of the skull base or the spine. It starts from leftovers of a structure called the notochord, which helps form the spine early in a baby’s development. Most chordomas are not cancer that spreads to other parts of the body, but they can be serious because they sit near important nerves and the brain.

No, chordoma is very rare. It affects about 1 person in every 1 million each year. This means it is not something most doctors see often.

Chordoma can happen at any age, but it is most often diagnosed in adults between 40 and 70 years old. It is slightly more common in males. Children can also get it, though it is rare.

Symptoms

Common symptoms

• Deep pain in the back, neck, or tailbone area
• Numbness, tingling, or weakness in the arms or legs
• Difficulty with bladder or bowel control
• Headaches, especially if the tumor is at the base of the skull
• Double vision or facial numbness if the tumor presses on nerves near the brain
• A noticeable lump, especially in the lower back or tailbone area

Symptoms in children

• Symptoms may be vague, like general back pain or tiredness
• Children may have trouble walking or complain of leg weakness
• Headaches or vision changes can occur if the tumor is in the skull base

Symptoms in older adults

• Symptoms are similar to those in adults, but might be mistaken for age-related joint or spine problems
• New or worsening back pain, especially with nerve symptoms like numbness or weakness, should be checked
• Changes in bladder or bowel habits can be a warning sign

Causes

Main causes

• Chordoma develops from leftover cells of the notochord, a structure that normally disappears before birth.
• In rare cases, these cells grow into a tumor later in life.
• The exact reason why this happens is not known.

Risk factors

• There are no proven lifestyle or environmental risk factors.
• A very small number of chordomas run in families, but most people who get it have no family history.

When to see a doctor

See a doctor urgently if:

• If you notice new weakness in your arms or legs
• If you lose bladder or bowel control
• If you have new double vision or facial numbness

Book a routine appointment if:

• If you have persistent pain in your back, neck, or tailbone that does not improve with rest
• If you feel a lump in your lower back or tailbone area
• If you have ongoing numbness or tingling in your hands or feet

Diagnosis

If your doctor suspects chordoma, they will examine you and ask about your symptoms. They may refer you to a specialist, such as a neurologist, orthopedic surgeon, or oncologist, for more tests.

Tests that may be done

• MRI (magnetic resonance imaging) to see the tumor in detail
• CT (computed tomography) scan to look at the bones
• A biopsy, where a small sample of the tumor is removed and checked under a microscope to confirm the diagnosis

What to expect at your appointment

It can take a few appointments to get a clear answer. Your healthcare team will explain the results to you in plain language and discuss treatment options if the diagnosis is confirmed.

Treatment

Treatment for chordoma is best planned by a team of specialists. The main goal is to remove the tumor safely while protecting nearby nerves and the brain. The approach depends on the tumor’s size, location, and your overall health.

Self-care at home

• Keep a journal of your symptoms to share with your healthcare team
• Ask for support with everyday tasks if you have pain or weakness
• Use gentle movement and rest to manage discomfort, as advised by your doctor
• Do not ignore new symptoms — tell your care team right away

Medical treatments

Surgery is often the first treatment if the tumor can be removed safely. Radiation therapy may be used after surgery or instead of surgery in some cases. Other treatments, such as drug therapies, may be offered through clinical trials or in certain situations. Your specialist team will discuss the best plan for your individual case. Medicines to control pain or improve symptoms may also be prescribed by your doctor.

When is surgery considered?

Surgery may be recommended when the tumor can be removed with acceptable risk to nearby nerves and structures. The goal is to remove as much of the tumor as possible while protecting function.

Living with this condition

Living with chordoma often means managing pain, staying active within your limits, and keeping follow-up appointments. Some people need physical therapy to keep their strength and balance. It’s also important to talk openly with your healthcare team about any new symptoms.

Lifestyle tips

• Stay active with low-impact activities like walking or swimming, as approved by your doctor
• Avoid heavy lifting or high-impact exercises unless your healthcare team says it is safe
• Ask about rehabilitation or physical therapy if you have weakness or balance issues
• Plan rest breaks during the day to avoid tiredness

Diet and exercise

Eat a balanced diet with plenty of fruits, vegetables, whole grains, and protein to support your body during treatment. Aim for regular, gentle exercise to keep your muscles and joints as healthy as possible. Always check with your doctor before starting a new exercise program.

Mental health and emotional wellbeing

A rare diagnosis can feel isolating and worrying. It is normal to feel anxious, sad, or overwhelmed. Talk to your healthcare team about seeing a counselor or joining a support group. Your mental well-being is just as important as your physical health.

Prevention

There is no known way to prevent chordoma. It is not caused by anything you did or did not do.

Complications

If left untreated

• The tumor may grow and press on the spinal cord or brain, causing nerve damage
• Untreated chordoma can lead to loss of movement, numbness, or paralysis
• Loss of bladder or bowel control can occur if the tumor presses on the lower spinal nerves

Long-term outlook

Many chordomas can be successfully treated with surgery and radiation. The outlook varies from person to person, depending on the tumor’s location and size. Some people may experience a return of the tumor, which is why regular follow-up is important. With today’s treatments, many people with chordoma live active and meaningful lives.

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