Chordoma: Symptoms, Causes, Diagnosis, and Treatment (2026 Guide)

Medically reviewed by Dr. Baraa Alnahhal, MD. Last updated: September 2026.

TL;DR

Chordoma is a rare bone cancer that starts in the bones of the spine or skull, most often at the skull base (clivus) or the sacrum. It develops from leftover cells that helped form the spine before birth, affecting roughly 1 in 1 million people, usually adults ages 40 to 60. Symptoms vary by tumor location and can go unnoticed for years because the tumors grow slowly. Surgery is the main treatment and offers the best chance of a cure when the tumor can be removed fully, usually followed by specialized radiation; chordoma generally does not shrink with standard chemotherapy.

Quick Answer

What is chordoma?

• A rare bone cancer starting in leftover spine-forming cells, found in the skull base, spine, or sacrum; about 60% occur in adults assigned male at birth and most often affect ages 40 to 60.

• Symptoms depend on tumor location: headaches and vision changes at the skull base, back pain and numbness in the spine, and pelvic pain with bowel and bladder changes in the sacrum.

• It is confirmed by imaging (MRI and CT) plus a biopsy looking for bubbly "physaliphorous" cells and the brachyury protein.

• Treatment is led by surgery to remove the tumor in one piece when possible, followed by advanced radiation such as proton therapy; targeted therapy is used when it spreads or returns.

What Is Chordoma?

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 because of where they grow, how they start, and who they affect.

They can grow anywhere along the spine, but most often grow in two locations. The first is a bone called the clivus, located where the skull sits atop the spine (the skull base). The second is at the bottom of the spine, called the sacrum. They can also grow in the areas in between, sometimes called the mobile spine.

Chordomas start in leftover cells that help form the spine before birth. Usually these leftover cells go away soon after birth. But sometimes a few remain. Rarely, in about 1 in 1 million people, the leftover cells eventually become cancerous.

Chordomas can happen at any age, but most often affect adults ages 40 to 60. They usually grow slowly, which is why they often go unnoticed for years. Surgery is the main treatment, but chordomas can be hard to remove fully because they grow close to the spinal cord, blood vessels, nerves, and brain. They often come back after treatment.

What Are the Types of Chordoma?

Chordomas come in four recognized types, distinguished by how their cells look under a microscope.

Type	Share of chordomas	Key features
Conventional (classic)	Most common	Bubbly cells in a slimy mix, similar to soap bubbles
Chondroid	Up to roughly 15%	Classic chordoma appearance plus cartilage-like tissue
Dedifferentiated	Up to just under 10%	Two parts: classic brachyury-positive cells and a different cancer cell type
Poorly differentiated	Rare	Mostly affects kids and young adults, usually at the skull base; identified by a missing SMARCB1 (INI1) gene

What Are the Symptoms of Chordoma?

Signs and symptoms differ depending on where the tumor is located and how large or advanced it is. Regardless of location, symptoms may include pain near the tumor, new nerve problems such as numbness or weakness, and new changes in bowel or urine function.

Skull Base Chordoma

The first signs of a chordoma in the clivus or skull base may include headache, facial numbness, weakness, drooping, or paralysis, and vision trouble, including blurred or double vision and sometimes vision loss. Other possible symptoms are hearing loss, hoarseness, trouble speaking, and trouble swallowing.

Mid-Spine (Mobile Spine) Chordoma

Early signs in 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 that may spread to the shoulders, swallowing trouble, and walking trouble.

Sacral Chordoma

Early signs at the bottom of the spine may include constipation, a lump at the tumor site, 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, and a sudden urge to urinate that is hard to control.

Because chordomas grow very slowly and produce general symptoms shared by many common conditions, it is common not to notice signs and symptoms for years.

Location	First warning signs	Other possible symptoms
Skull base (clivus)	Headache, facial numbness or drooping, blurred or double vision	Hearing loss, hoarseness, trouble speaking or swallowing
Mid-spine (mobile spine)	Arm or leg numbness/weakness, back pain, neck pain	Breathing trouble, headache, trouble walking or swallowing
Sacrum (tailbone)	Pelvic/tailbone pain worse when sitting, numbness in buttocks and inner thighs	Constipation, a palpable lump, hard-to-control urge to urinate

What Causes Chordoma?

It is not clear what causes most chordomas; they usually happen randomly. Rarely, they can be hereditary and run in families. The cancer most often starts in the bones of the skull or spine.

Chordoma begins when cells in the bones develop changes in their DNA. DNA holds the instructions that tell a cell what to do. In healthy cells, DNA instructs growth and multiplication at a set rate and tells cells to die at a set time. In cancer cells, DNA changes give different instructions: grow and multiply quickly, and stay alive when healthy cells would die. This produces too many cells.

The cancer cells form a mass called a tumor. The tumor can invade and destroy healthy tissue, and in time cancer cells can break away and spread to other parts of the body. Cancer that spreads is called metastatic cancer.

Who Is at Higher Risk of Chordoma?

Factors that may increase the risk of chordoma include age, family history, and sex assigned at birth.

Risk factor	Details
Age	Can occur at any age; most often diagnosed between ages 40 and 60
Family history	Rarely hereditary; linked to changes in genes such as TBXT, which produces the brachyury protein that chordomas carry in large amounts
Sex assigned at birth	In adults, about 60% are found in people assigned male at birth and about 40% in people assigned female at birth; in children, those assigned female are slightly more likely to be diagnosed

What Health Problems Can Chordoma Cause?

Complications typically result from tumors growing and pressing into blood vessels, nerves, and other tissues. Others occur if the cancer spreads.

Nervous system complications may include rare cerebrospinal fluid leaks, headaches, hearing loss, problems with balance and walking, trouble swallowing or speaking, trouble breathing, vision problems, water buildup on the brain (which raises skull pressure and causes headaches, nausea, and vomiting), and weakness or numbness in the face, arms, or legs.

Sometimes chordoma spreads to other parts of the body, called metastatic chordoma (also called advanced or end-stage chordoma). Less than half of chordomas spread. Spread is most common with tumors below the neck and with the poorly differentiated type. The most common destination is the lungs, though it can also spread to the liver, other bones, or soft tissues. Complications of metastatic chordoma include bone breaks, extreme tiredness, lumps, pain, belly swelling, trouble breathing, jaundice (yellow skin or eyes), and weight loss.

When Should You See a Doctor?

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. This matters especially for persistent, unexplained symptoms such as chronic back or pelvic pain that worsens when sitting, new numbness or weakness, changes in bowel or bladder habits, or recurring headaches with vision changes, since these can build gradually over years in slow-growing chordoma.

How Is Chordoma Diagnosed?

To diagnose chordoma, a healthcare professional may perform a physical exam, ask about your health history, order imaging tests, and take a tissue sample for lab testing.

Imaging tests. CT scans and MRI scans make pictures of the body to show the size and location of a cancer. CT scans are best for seeing bone changes, such as holes or weak spots, and chordomas usually appear as light or dark spots with damaged bone around them. A contrast dye can highlight areas being scanned. MRI scans are better for showing tumors and the blood vessels and tissues around them; chordomas usually appear as bright spots with darker areas inside.

Biopsy. A biopsy removes a tissue sample for lab testing and is essential to confirm chordoma, because on imaging scans chordoma can look like other conditions that need different treatments. The sample may be collected by inserting a thin needle through the skin (not always possible for skull base chordomas) or by removing a piece during surgery through an incision. Planning the biopsy carefully matters, since it must allow for future surgery. Seeking care at a medical center that sees many people with chordoma is recommended for this reason.

To confirm chordoma, the pathology team looks for cells that look bubbly inside, called physaliphorous cells, the brachyury protein common in chordoma cells, and other proteins such as S100, vimentin, and pancytokeratins.

Genetic testing is not routine but may be suggested for families with a history of chordoma or for children and young adults with the disease.

How Is Chordoma Treated?

Treatment depends on the size and location of the cancer and whether it has invaded nerves or other tissue. Surgery is often the first step: if a tumor can be removed fully, it offers the best chance at a cure. Radiation therapy usually follows surgery to kill remaining cells, though it is sometimes used before surgery to shrink a tumor or alone when surgery is not possible. Targeted therapy medicines may be used for cancer that has spread or returned.

In some cases, chordoma can be cured. But it is difficult to treat. Tumors are hard to remove fully because they grow near the brain and spine, and they resist conventional radiation and chemotherapy. Chordomas often return, and when they do, treatment aims to control or manage the cancer over time rather than cure it.

Surgery

Surgeons aim to remove as much of the tumor as possible in one piece. When done in the mobile spine or sacrum, this is called en bloc resection; removing the tumor fully in one piece lowers the chance of the cancer spreading and returning. Removing the whole chordoma is difficult because it grows around the brain, spinal cord, nerves, and blood vessels, and parts of the skull base are challenging to reach safely.

Surgery types differ by location. Skull base chordoma surgery sometimes proceeds through the nose using endoscopic skull base surgery, where a long, thin endoscope and special tools remove the cancer; open surgery through an incision at the back of the head or neck, or other approaches, may also be used. Mobile spine and sacral surgery removes as much of the tumor as possible along with surrounding bone and tissue; if the tumor is in the sacrum, the whole sacrum may be removed in a procedure called a sacrectomy, performed through an incision in the belly or the back. Rarely, a second surgery stabilizes the area.

Surgical complications include general surgery risks such as infection, blood clots, and bleeding, plus complications from nerve damage: double vision, bowel or bladder changes, loss of sexual function, numbness, trouble walking or sitting, and cerebrospinal fluid leaks.

Radiation Therapy

Radiation therapy treats cancer with powerful energy beams from X-rays, photons, protons, or other sources, delivered by a machine that directs radiation to precise points while the patient lies on a table.

Chordomas have resisted standard radiation, which had to be given at doses low enough to protect nearby spinal cord and brain tissue. Newer treatments deliver a higher dose directly to the tumor while protecting surrounding tissue. Options include proton therapy, intensity-modulated radiation therapy (IMRT), stereotactic radiosurgery, stereotactic body radiotherapy, and carbon ion therapy. The type used varies with tumor location and the surgical center's available options.

Side effects may include tiredness, skin swelling or irritation, headache, hormone changes such as hypopituitarism, brain changes or damage, hearing and vision changes, trouble swallowing, and a slight risk of a secondary cancer developing later.

Targeted Therapy

Chordoma usually does not shrink with standard chemotherapy. Targeted therapy uses medicines that attack specific chemicals in cancer cells, causing them to die by blocking those chemicals. It is sometimes used in clinical trials or off-label for metastatic or recurrent chordoma; off-label means the drug is approved by regulators for a different condition.

Medicines sometimes used first include imatinib, dasatinib, and sunitinib. Other options include imatinib combined with a chemotherapy drug or with sirolimus, plus erlotinib, lapatinib, and sorafenib. Side effects may include tiredness, upset stomach, rash, diarrhea, swelling, and thyroid and kidney changes.

Treatments Being Studied

Research continues on new chordoma treatments. Checkpoint inhibitors are drugs that help the immune system find and attack cancer cells. Brachyury-targeted therapies include vaccines and new drugs aimed at the brachyury protein that chordomas almost always produce.

How Can Patients Cope and Find Support?

With time, most people find what helps them cope with the uncertainty of a cancer diagnosis. Helpful steps include learning enough about the disease to make informed care decisions, asking the healthcare team about test results, treatment options, and prognosis, and using assistive devices for mobility concerns and available pain treatments.

Staying close to friends, family, and community provides practical support, such as help at home during hospital stays. Finding someone to talk with, whether a friend, counselor, medical social worker, clergy member, or cancer support group, also helps. Dedicated organizations offer directories of chordoma specialists and online patient communities.

Conclusion

Chordoma is rare, but its rarity is exactly why early attention to symptoms matters. Because it grows slowly from leftover embryonic cells, it can quietly press on nerves, the brainstem, and the pelvic organs for years before causing unmistakable symptoms. That slow pace makes it easy to dismiss as ordinary back pain, sciatica, or migraine, yet the combination of persistent pain, new numbness or weakness, and bowel or bladder changes deserves a proper evaluation.

The good news is that chordoma is treatable, and sometimes curable. Complete surgical removal, often with advanced radiation such as proton therapy, offers the best outcomes, and treatment options continue to expand through clinical trials. Because the disease often returns and benefits from specialized care, seeking a medical center experienced with chordoma is one of the most important decisions a patient can make.

Noticed persistent symptoms that match this guide? Talk with your healthcare professional about whether imaging such as an MRI is appropriate, and ask whether your case could benefit from a center that regularly treats chordoma. If you or a loved one has been diagnosed, ask about surgery plans, radiation options, and whether a clinical trial might fit.

Frequently Asked Questions

How rare is chordoma?

Very rare. It affects about 1 in 1 million people. Chordomas can occur at any age but most often are diagnosed in adults ages 40 to 60.

Where do chordomas grow?

They can grow anywhere along the spine but most often form in two spots: the clivus at the skull base and the sacrum at the bottom of the spine. Tumors in between occur in the mobile spine.

Is chordoma hereditary?

Most chordomas happen randomly, but rarely they run in families. Hereditary cases are linked to changes in genes such as TBXT, which produces the brachyury protein found in large amounts in chordoma cells.

Why is chordoma hard to treat?

The tumors grow very close to the brain, spinal cord, nerves, and blood vessels, making complete removal difficult. They also resist conventional radiation and standard chemotherapy, and they often come back after treatment.

What is the first-line treatment?

Surgery is usually the first step, aiming to remove the tumor in one piece (en bloc resection) when possible, followed by advanced radiation therapy such as proton therapy or IMRT to kill remaining cells.

Does chemotherapy work for chordoma?

Standard chemotherapy usually does not shrink chordoma. Targeted therapy medicines such as imatinib, dasatinib, and sunitinib may be used when the cancer has spread or returned.

Can chordoma spread to other parts of the body?

Yes, though less than half of chordomas spread. The lungs are the most common site, followed by the liver, other bones, and soft tissues. Spread is most common with tumors below the neck and with the poorly differentiated type.

References

This article is based on medically reviewed clinical information, including Chordoma — Symptoms & causes and Chordoma — Diagnosis & treatment, supplemented by the National Cancer Institute's overview of chordoma and the National Comprehensive Cancer Network's bone cancer guidelines.

This article is for educational purposes only and is not a substitute for professional medical advice. Chordoma requires evaluation and treatment by an experienced oncology team; always consult a qualified healthcare professional for diagnosis and care decisions.