Chordoma

November 6, 2025

This page was reviewed under our medical and editorial policy by Mike Y. Chen, M.D., Ph.D., associate professor, Division of Neurosurgery, Department of Surgery, City of Hope^® Cancer Center Duarte.

Chordoma belongs to a group of bone and soft tissue cancers called sarcomas. It is a rare cancer that forms in the spine and bones of the skull, affecting just 1 in 1,000,000 people per year. In the United States, about 300 people are diagnosed with chordoma annually — mostly individuals in their 50s and 60s, though it may occur at any age.

What Is Chordoma?

Chordoma is also called notochordal sarcoma, notochordoma, CHDM, chordocarcinoma or chordoepithelioma. These tumors may form anywhere along the spine, from the head to the tailbone, but most often occur in:

• The sacrum, the flat, triangular-shaped bone that connects the spine with the hip
• The clivus, the bone at the base of the skull near the spine
• The coccyx, the lower portion of the spine, commonly known as the tailbone

Most chordomas are slow-growing and unlikely to spread, but in 30% to 40% of patients, they eventually spread to other parts of the body, usually the lungs, liver, bones or lymph nodes.

Chordoma Causes and Risk Factors

Chordoma develops from residual cells that were necessary for spinal development before birth. When these cells, known as notochord cells, don’t diminish after birth, they may change into chordomas. The specific reason why they form into malignancies, however, isn’t clear.

Research suggests there are genetic factors associated with chordoma.

For instance, around 95% of people with chordoma have a small variation in the DNA sequence of their brachyury, a protein made by the TBXT gene, causing an increased risk for developing the disease.

A small number of families have several members with chordoma, indicating an inherited genetic predisposition for chordoma. These families have an extra copy of the brachyury gene.

The gene changes triggering tuberous sclerosis complex (TSC) in children may also increase risk of chordoma.

Chordoma Symptoms and Signs

Chordoma symptoms vary based on tumor size and location. There may be no symptoms at first, but if the tumors grow, they may press on the brain or spine, resulting in a range of symptoms.

Overall, the most common signs of chordoma are pain and neurological changes. Other symptoms include:

• Pain in the face, head, neck and lower back
• Vision problems, including double vision
• Paralysis of facial nerves
• Headaches
• Numbness and tingling
• Vomiting
• Trouble swallowing
• Bowel or bladder control problems
• Erectile dysfunction
• Leg pain

When chordoma spreads elsewhere in the body, other symptoms like extensive pain, fatigue and weakness may occur.

Tests for Diagnosing

Doctors typically diagnose chordoma through a comprehensive medical history and physical exam to look for signs (lumps, especially along the spine) of disease, imaging tests and a biopsy.

Common diagnostics include:

• A computed tomography (CT) scan, which uses a series of X-rays to see tumor location and size
• Magnetic resonance imaging (MRI), which uses a large magnet and radio waves to create clear images of specific body structures
• A biopsy, which involves removing a tumor sample for lab examination and testing to make a definitive chordoma diagnosis

Chordoma Types

Based on tumor appearance, doctors classify chordoma as conventional, chondroid or dedifferentiated, as detailed below.

Conventional: Also known as classic, conventional chordomas are the most common type, slow growing and make up 80% to 90% of all cases.

Chondroid chordomas: These account for between 5% to 15% of all cases, usually arise in the base of the skull and resemble a more common form of primary bone cancer called chondrosarcoma, which originates in the cartilage.

Dedifferentiated chordomas: These comprise fewer than 5% of all cases. They are more aggressive, more likely to spread and typically form in the sacrum, located in the base of the spine.

Chordoma Treatment

Because they form on the spine and near tissues like nerves and blood vessels, chordomas are often complicated to treat. This may necessitate surgery, followed by radiation therapy and chemotherapy or targeted therapy.

Treatment options vary based on the type of chordoma, tumor size and spread.

Surgery

When possible, chordomas are removed by surgery as first-line treatment. The goal of surgery is either complete removal (called “en bloc resection”), or, when that isn’t possible, removal of the bulk of the tumor (called “gross total resection.”) Gross total resection may leave behind microscopic cancer cells on critical structures, such as the spinal cord, and may therefore be linked to a higher recurrence rate.

Several surgeries may be required, in addition to radiation therapy, to destroy all the cancer cells while sparing healthy tissue. In addition, chordoma surgeries are often complex, requiring coordination among multiple experts.

Radiation Therapy

Radiation therapy, which uses high-intensity X-rays to kill cancer, is sometimes used after surgery, or alone if surgery isn’t feasible. Radiation therapy techniques for chordoma may include a type of focused radiation therapy called stereotactic radiation, which may deliver a very concentrated radiation dose to a small area of affected tissue with great precision.

Proton Beam Therapy

This type of radiation therapy uses proton radiation to precisely target tumors. It may be beneficial for chordoma patients because it reduces damage to tissue around the tumor.

Drug Therapies

Researchers are examining the potential of drug therapies, including chemotherapy, targeted therapy and immunotherapy, to treat chordomas that have spread and those untreatable through surgery or radiation therapy. For instance, scientists are exploring whether checkpoint inhibitors, which train the patient’s own immune system to detect and destroy cancer cells, may be effective in treating chordoma.

Who Treats Chordoma?

Choosing the right cancer care team is one of the most important decisions patients make. With chordoma, it’s important to partner with experts with experience treating bone cancers, including:

• Medical or pediatric oncologists, who treat cancer with chemotherapy and other drugs
• Surgical oncologists, who diagnose and treat cancer with surgery
• Neurosurgeons, who are experts in performing surgical procedures on the spine, brain and nervous system
• Orthopedic surgeons, who specialize in treating bone tumors
• Radiation oncologists, who use radiation therapy to destroy cancerous tissue
• Pathologists, who examine tissue samples for diagnostic purposes to help determine a treatment plan
• Radiologists, who utilize imaging tools and interventional techniques to diagnose and treat cancer
• Supportive care providers, who focus on cancer- and treatment-related side effects and symptom management

Chordoma Survival Rate

Because the disease is so rare, survival rates for chordoma are based on a small patient population and depend on tumor location, stage and how much disease was removed by surgery. It’s important to discuss individual prognosis with the care team.

The overall five-year relative survival rate for chordoma is 84%. This means that people with chordomas are 84% as likely to still be alive after five years compared to people without this cancer type. 

he five-year relative survival rates by stage for chordoma are:

• 94% for localized tumors, meaning the cancer hasn’t spread
• 85% for regional tumors, meaning the cancer has spread into nearby structures, bones or lymph nodes
• 54% for distant tumors, meaning the cancer has spread to other parts of the body

The patient’s care team may provide a more personalized prognosis that’s tailored to the patient’s cancer subtype, stage and other factors.