Overview
Chordoma is a rare type of bone cancer that starts in the bones of the spine or the skull. Chordomas are different from other bone tumors for reasons including where they grow, how they start and who they affect.
Chordomas can grow anywhere along the spine. But they often grow in two locations. The first is in a bone called the clivus. The clivus is located where the skull sits atop the spine, also called the skull base. The second is at the bottom of the spine, called the sacrum. Chordomas also can grow in the areas in between, sometimes called the mobile spine.
Chordomas start in leftover cells that help form the spine before birth. Usually, any leftover cells go away soon after a baby is born. But sometimes a few of the cells stay. Rarely, in about 1 in 1 million people, the leftover cells eventually become cancerous.
Chordomas can happen at any age. But they most often affect adults ages 40 to 60. Chordomas usually grow slowly. Surgery is usually the main treatment. However, chordomas can be hard to remove fully. This is because they grow very close to the spinal cord, blood vessels, nerves and brain. A chordoma often comes back after treatment.
Types
Different types of chordomas include:
- Conventional chordoma, also called classic chordoma and classical chordoma. This is the most common type. Under a microscope, it looks like bubbly cells in a slimy mix, similar to soap bubbles.
- Chondroid chordoma. Up to roughly 15% of chordomas are this type. Under a microscope, chondroid chordomas look similar to classic chordomas. But they also have tissue that looks similar to cartilage tissue found in parts of the body such as the nose.
- Dedifferentiated. Up to just under 10% of chordomas are this type. Under a microscope, this type of chordoma has two parts. One part looks like classic chordoma cells that also have a special protein called brachyury. The other part looks like a different type of cancer cell.
- Poorly differentiated. This rare type of chordoma mostly affects kids and young adults and is usually found in the skull base. It's identified due to a missing gene called SMARCB1 (INI1).
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Symptoms
Chordoma signs and symptoms may differ depending on where the tumor is located and how large or advanced it is. But no matter where a chordoma is located, symptoms may include:
- Pain near the location of the tumor.
- New nerve problems, such as numbness or weakness.
- New changes to how and when you pass stool or urine.
Skull base chordoma symptoms
The first signs and symptoms of a clival bone chordoma or a skull base chordoma may include:
- Headache.
- Facial numbness, weakness, drooping or paralysis.
- Vision trouble, including blurred or double vision or, sometimes, vision loss.
Other possible symptoms include:
- Hearing loss.
- Hoarseness.
- Trouble speaking as usual.
- Trouble swallowing.
Middle spine, also called mobile spine, chordoma symptoms
The first signs and symptoms of a chordoma in the part of the spine between the skull base and the sacrum may include:
- Arm or leg numbness or weakness.
- Back pain that may spread to the buttocks and legs.
- Breathing trouble.
- Headache.
- Neck stiffness or pain.
- Neck pain that may spread to the shoulders.
- Swallowing trouble.
- Walking trouble.
Bottom of the spine, also called sacral, chordoma symptoms
The first signs and symptoms of a sacral chordoma may include:
- Constipation.
- Lump at the site of the tumor.
- Numbness or tingling in the buttocks and inner thighs.
- Muscle weakness.
- Pain in the back, buttocks, pelvis or tailbone that may be worse when sitting.
- Sudden urge to urinate that's hard to control.
Because chordomas grow very slowly and have general symptoms caused by many things, it's common not to notice signs and symptoms for years.
When to see a doctor
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.
Causes
It's not clear what causes most chordomas. They usually happen randomly. Rarely, they can be hereditary, meaning chordomas can run in families. This cancer most often starts in the bones in the skull or spine.
Chordoma happens when cells in the bones develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.
The cancer cells form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. Cancer that spreads is called metastatic cancer.
Risk factors
Factors that may increase the risk of chordoma include:
- Age. Chordoma can happen at any age. Most often, this cancer happens in people ages 40 to 60.
- Family history. Most chordomas happen randomly. But people with a family history of chordoma may have a greater chance of getting it. When this happens, it's because of changes in certain genes, such as the TBXT gene. This gene plays a role in making a protein called brachyury. Chordomas have a lot of brachyury in them. This can help tell chordomas apart from other tumors.
- Sex assigned at birth. In adults, people assigned male at birth are somewhat more likely to be diagnosed with chordoma. About 60% of chordomas are found in people assigned male at birth. About 40% of chordomas are found in people assigned female at birth. In children, those assigned female at birth are slightly more likely to be diagnosed with chordoma than are those assigned male at birth.
Complications
Complications of chordomas typically are caused by the tumors growing and pressing into blood vessels, nerves and other tissues. Other complications happen if the cancer spreads to other parts of the body.
Chordoma complications of the nervous system may include:
- Cerebrospinal fluid leaks, though this is rare.
- Headaches.
- Hearing loss.
- Problems with balance, coordination and walking.
- Trouble swallowing or speaking.
- Trouble breathing.
- Vision problems.
- Water buildup on the brain, which can increase pressure inside the skull and cause headaches, nausea and vomiting.
- Weakness or numbness in the face, arms or legs.
Sometimes chordoma tumors can spread to other parts of the body. You may hear this called metastatic chordoma. Less than half of chordomas spread to other parts of the body. It's most common in chordomas that are found in areas of the spine below the neck and with the poorly differentiated type of chordoma. Sometimes metastatic chordoma may be called advanced chordoma or end-stage chordoma.
The most common place for a chordoma to spread is to the lungs. But it also can spread to the liver or other bones or soft tissues. Depending on where metastatic chordoma has spread, complications may include:
- Bone breaks.
- Extreme tiredness.
- Lumps.
- Pain.
- Swelling of the belly.
- Trouble breathing.
- Yellow skin or eyes, called jaundice.
- Weight loss.
Oct. 17, 2025