Chordomas are rare, slow-growing malignant bone tumors that arise from remnants of the notochord, the embryonic structure that helps form the spinal column. They most commonly develop in the clivus (base of the skull) or along the spine, especially in the sacrum (low back) and coccyx (tailbone). While chordomas grow slowly, they are locally aggressive, often infiltrating surrounding bones, nerves, and critical blood vessels.
UPMC takes a multidisciplinary approach to treatment, often combining advanced surgery with precision radiation. Our neurosurgeons and skull base specialists tailor treatment plans to maximize tumor control while preserving neurological function and quality of life.
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What Are Chordoma Tumors?
Chordomas are rare, slow-growing malignant bone tumors that arise from remnants of the notochord, the embryonic structure that helps form the spinal column. These tumors most commonly develop in the clivus (base of the skull) or along the spine, especially in the sacrum (low back) and coccyx (tailbone). They are a type of primary bone cancer, typically found in the skull or spine.
Although they grow slowly, they are locally aggressive and can infiltrate nearby bones, nerves, and critical blood vessels. Over time, chordomas may extend into surrounding organs and tissues, and about 30% eventually metastasize (spread) to other organs, most commonly the lungs. Even when they do not spread, chordomas can cause serious health problems and have a high tendency to recur after treatment.
What are the types of chordoma tumors?
Chordomas are most often found in:
- The clivus (skull base), causing neurological and cranial nerve symptoms.
- The sacrum or coccyx, leading to bowel, bladder, or mobility issues.
- Along the mobile spine, but less commonly.
Chordomas can occur at any point along the spine:
- About 20% occur in the vertebrae of the upper, middle, or lower back.
- About 30% grow in the base of the skull (clival chordomas).
- About 50% develop in the base of the spine near the tailbone (sacral chordomas).
Rare subtypes include:
- Classic or conventional chordomas — The most common type, making up about 80–90% of cases. These cells have a unique bubbly appearance and contain brachyury protein, a hallmark of chordoma.
- Dedifferentiated chordomas — Less than 5% of cases. This aggressive form is most common in the base of the spine and is more likely to spread.
- Poorly differentiated chordomas — Very rare, first recognized by the WHO in 2020, often associated with genetic alterations such as SMARCB1/INI1 loss, and more common in younger people.
How common are chordoma tumors?
Chordoma tumors are very rare, affecting one person per one million people each year, and making up less than 1% of tumors of the brain and spinal cord. In the U.S., about 300 people receive a chordoma diagnosis each year. Chordoma makes up 1% to 4% of all malignant bone tumors.
They are most commonly found in adults between the ages of 40 and 70, although pediatric cases have been reported. Men are slightly more frequently affected than women, and chordomas very rarely occur in children.
What causes chordoma tumors?
Chordomas develop from remnants of the notochord, which forms before the spinal column during fetal development. The spine replaces the notochord before birth, but if some notochord cells get left behind, they can turn into chordomas. Most chordomas are sporadic, occurring randomly without a clear pattern or consistent cause.
Genetic research has identified key associations:
- T gene (brachyury) duplication strongly predisposes to chordoma and is now used as a diagnostic biomarker.
- Tuberous sclerosis and other hereditary syndromes are also linked in rare cases.
Chordoma does not appear to be triggered by environmental or lifestyle factors, pre-existing health conditions, or medicines. It also doesn’t seem to run in families, although rare familial cases exist. Some people with chordoma have small changes in their genes, and researchers are studying these mutations to learn more.
Chordoma tumor risk factors
- Genetic changes that trigger abnormal cell growth.
- Having the genetic condition tuberous sclerosis.
Complications of chordoma tumors
Untreated or recurrent chordomas can lead to:
- Bladder and bowel dysfunction (sacral tumors).
- Chronic pain (spine or skull base).
- Double vision, difficulty swallowing, or hearing loss (skull base tumors).
- High recurrence rates even after aggressive therapy.
- Neurological deficits, such as weakness, numbness, or paralysis.
- Spread of tumors to other organs — most commonly the lungs — leading to disability and eventually death.
How can I prevent chordoma tumors?
There is nothing you can do to prevent a chordoma tumor, as it isn’t caused by lifestyle choices or environmental factors, but occurs spontaneously.
What Are the Signs and Symptoms of Chordoma Tumors?
Symptoms depend on tumor location and size.
Chordoma tumor symptoms may include:
- Bowel or bladder problems (sacral chordomas).
- Difficulty swallowing or speaking.
- Double vision or vision loss.
- Headache or facial pain.
- Hearing changes.
- Weakness, numbness, or back/leg pain.
A chordoma in the skull may produce:
- Double vision.
- Headaches.
- Trouble swallowing.
- Vision problems.
A chordoma in the spine may cause:
- Clumsiness.
- Numbness or tingling in the back, arms, or legs.
- Pain at the location of the tumor.
- Weakness or pain in the arms or legs.
A chordoma in the tailbone may cause:
- A lump large enough to be felt through the skin.
- Bowel and bladder problems.
- Numbness in the groin.
- Pain running down the legs.
- Tingling or weakness in the legs.
When should I see a doctor about my chordoma tumor symptoms?
If you have symptoms of a chordoma tumor, you should schedule an appointment with your doctor right away. If you have severe symptoms, dial 911 or go to the nearest hospital emergency department.
How Do You Diagnose a Chordoma Tumor?
To diagnose a chordoma tumor, your doctor will ask about your symptoms, perform a physical exam, and order imaging tests.
What to expect during your visit:
- Ask about your symptoms.
- Perform a physical exam.
- Order imaging tests.
- Review your health history and medications.
Tests to diagnose a chordoma tumor include:
- Biopsy — Confirms diagnosis with pathology and immunohistochemistry (brachyury staining is highly specific).
- CT scan — Reveals bone destruction patterns.
- Magnetic resonance imaging (MRI) — Best for showing tumor extent, soft tissue involvement, and relationship to nerves and vessels.
- Molecular testing — May be performed to identify mutations relevant for clinical trials or targeted therapies.
- X-rays — May reveal bone damage caused by the tumor, though chordomas don’t show up directly.
Additional tests include:
- Blood tests.
- Bone scans.
- CT scans of the lungs.
What is the prognosis for chordoma tumors?
Prognosis depends on the tumor’s location, your age, and whether the tumor can be surgically removed. Standard chordomas are slow-growing but highly recurrent. Dedifferentiated and poorly differentiated chordomas behave more aggressively and have worse survival rates. Five-year survival rates range between 50% and 70%, depending on tumor subtype and extent of treatment. Long-term monitoring is essential due to the risk of recurrence.
How Do You Treat Chordoma Tumors?
Chordomas require individualized, multimodal treatment. The neurosurgical team may recommend one or a combination of treatments. The high recurrence rate of these tumors requires frequent follow-up and monitoring.
Chordoma tumor surgery
Surgery is the main treatment for chordomas. The type of surgical procedure depends on the size and location of the tumor.
Endoscopic endonasal approach (EEA)
Minimally invasive techniques such as EEA allow access to skull base chordomas through the nose, avoiding facial incisions and shortening recovery. Complex spinal or sacral tumors may require en bloc resections, which remove the entirety of the tumors intact, along with reconstructive techniques.
Risks of surgery include:
- Incontinence - loss of bowel or bladder control.
- Loss of sensation in the groin area.
- Pelvic instability.
- Weakness of the legs.
Radiation therapy to treat chordoma tumors
Radiation therapy is commonly used after surgery to reduce the risk of chordoma recurrence.
Your doctor may deliver radiation therapy:
- After surgery to reduce recurrence or as primary therapy when tumors are inoperable.
- Using stereotactic radiosurgery, usually Gamma Knife®, for small residual or recurrent tumors.
- With proton beam radiation, which is preferred due to its ability to deliver high-dose radiation precisely while sparing surrounding tissues.
Side effects of radiation therapy include:
- Fatigue.
- Hair loss.
- Redness like sunburn.
Medical therapy for chordoma tumors
Traditional chemotherapy is largely ineffective for chordoma tumors. Research is ongoing with targeted therapies (such as inhibitors of EGFR, PDGFR, or brachyury-directed immunotherapies). Clinical trials are exploring immunotherapy and novel molecular therapies for advanced or recurrent chordomas.
Monitoring for chordoma tumors
Regular MRI follow-up is critical as chordomas often recur even after aggressive treatment.
How effective is treatment?
The outcome depends on many factors, including:
- If the entire tumor was able to be removed.
- The method of treatment.
- Where the tumor was located.
- Your age and overall health.
Your doctor will recommend coming back for MRI scans at regular intervals. You may need to keep having these scans for the rest of your life, to make sure the cancer hasn’t come back.
Why Choose UPMC for Chordoma Tumor Care?
When you choose UPMC for chordoma tumor care, you will receive:
- Access to world-class neurosurgery expertise — treating the full spectrum of neurological and cancer conditions using the latest diagnostic and treatment techniques.
- A full range of treatment options — including nonsurgical care, noninvasive, minimally invasive, and traditional open surgical procedures.
- Multidisciplinary care — Neurosurgeons partner with neurologists, medical oncologists, radiologists, rehabilitation specialists, and other medical experts to provide complete care that optimizes your recovery and quality of life.
