Chordoma: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Chordoma?
An orthopedic oncologist (a surgeon who treats bone tumors), a neurosurgeon (a brain and spine surgeon), and a radiation oncologist (a doctor who treats cancer with radiation) often care for people with a chordoma. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is chordoma?
Chordoma is a rare type of cancerous tumor. It can occur anywhere along the spine, from the base of the skull to the tailbone. Chordomas grow slowly into the bone and soft tissue around them. They often come back after treatment.
A chordoma starts in leftover cells from the notochord. The notochord is a cartilage-like structure that helps form the early spine before birth. Small areas of it can remain, and it is believed that chordomas can form in these areas. Chordomas are potentially life-threatening tumors. In some cases, they can spread to other parts of the body, most often to the lungs.
Symptoms
Chordomas grow slowly, so people often have symptoms for a long time before they get medical care. Symptoms depend on where the tumor is. Common signs include:
- A tumor in the skull can cause headaches, trouble swallowing, or vision problems.
- A tumor in the spine can cause pain in the neck, back, or tailbone area.
- A tumor pressing on nearby nerves can cause pain, weakness, numbness, clumsiness, or tingling in the arms or legs.
- A tumor in the tailbone area can cause a lump you can feel, numbness in the groin, or bowel and bladder problems.
Causes and who gets it
Chordoma is rare. It affects about 1 in a million people each year. Most chordomas occur in people between 40 and 70 years of age. They can also occur in younger people, including children. Chordomas are more common in males than in females.
Changes in a gene called TBXT have been linked to chordoma. In a few families, an inherited extra copy of this gene raises the risk. In people with no family history, the gene changes occur only in the tumor cells and are not inherited. Chordomas are not known to be caused by injury, the environment, or diet. Children who have an inherited condition called tuberous sclerosis may be at higher risk.
How doctors diagnose it
A doctor does a physical exam and uses imaging tests to diagnose a chordoma. Imaging tests may include X-rays, CT scans, and MRI scans. Chordomas do not show up well on X-rays, but an X-ray may show bone damage from the tumor. An MRI is considered the best test for finding the total size of the tumor.
A biopsy is needed to confirm the diagnosis. In a biopsy, a doctor takes a tissue sample of the tumor, and it is looked at under a microscope. Doctors then do more tests to see whether the cancer has spread. This is called staging.
Treatment
Treatment for a chordoma often combines surgery and radiation therapy. Radiation therapy uses high-energy rays to kill cancer cells or keep them from growing. Whenever possible, surgery is the preferred treatment. The surgeon must remove the tumor and some normal tissue around it. Surgery can be hard to do because important structures are near the tumor. It often causes some loss of nerve function. For example, removing a tumor in the sacrum, the bone at the base of the spine, may cause loss of bowel and bladder control, weakness in the legs, loss of feeling in the groin, or unstable pelvic bones. Your doctor will talk with you about the risks in your own case.
If a tumor cannot be removed with surgery, radiation therapy may help stop its growth. Newer types of radiation, such as proton beam therapy, may be more effective at stopping the tumor from growing. Proton beam therapy aims tiny particles called protons at the cancer cells to kill them. Traditional chemotherapy, which is cancer-fighting medicine, is not usually very effective.
When to see a doctor
See a doctor if you have headaches, trouble swallowing, vision problems, or pain in the neck, back, or tailbone area. Also see a doctor for pain, weakness, numbness, clumsiness, or tingling in the arms or legs, numbness in the groin area, or bowel and bladder problems. Call 911 or go to the emergency room if you have sudden weakness, a sudden change in vision, trouble breathing, or fainting.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Either type of doctor can listen to your symptoms, help arrange scans or other tests, and refer you to specialists. They can also help keep track of your follow-up care over time.
Common questions
Is chordoma cancer?
Yes, a chordoma is a cancerous tumor. It grows slowly, but it is potentially life-threatening.
Can a chordoma spread?
Yes, in some cases a chordoma can spread to other parts of the body. When it spreads, it most often goes to the lungs.
Does chordoma run in families?
In a few families, yes. In people with no family history, the gene changes occur only in the tumor cells and are not inherited.
Why is chordoma hard to treat?
A chordoma is hard to treat because it sits near vital structures such as the spinal cord and brain.
Sources
- MedlinePlus Genetics - Chordoma
- National Cancer Institute - Childhood Chordoma (PDQ)
- American Academy of Orthopaedic Surgeons - OrthoInfo - Chordoma
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
