Chordoma Foundation

Diagnosing chordoma

Have you been recently diagnosed?

Our Newly Diagnosed page lists the next steps you can take to get the best possible care.

Chordoma is not always easy to diagnose and can be confused with other diseases. It is important to go to a hospital, treatment center, or network of treatment centers where doctors have experience diagnosing and treating chordoma as soon as chordoma is suspected. Our Doctor Directory can help you find doctors with chordoma experience.

Getting the correct diagnosis can have a major impact on the treatment you have. For this reason, it is very important for your diagnosis to be made by doctors who have experience diagnosing and treating chordoma patients. Getting a second opinion to confirm the diagnosis can be helpful before making treatment decisions. It is always OK to ask for a second or even a third opinion. If you have not yet had treatment, ask whether any additional tests are needed to rule out other possible tumor types before going forward with treatment.

Recommendations found in these sections come from the medical paper, “Building a global consensus approach to chordoma: a position paper from the medical and patient community”, published in The Lancet Oncology in February 2015. These consensus treatment guidelines were written by an international group of chordoma experts and patient advocates, including the Chordoma Foundation.

What are the signs and symptoms of chordoma?

The most common signs of chordoma are pain and neurological changes.

Skull base chordomas most often cause headache, neck pain, or double vision. If large enough, they may affect facial sensation or movement, voice, speech, and swallowing function.

Chordomas of the spine and sacrum can cause changes in bowel and/or bladder function, pain, aching, tingling, numbness, or weakness of the arms and legs. Often, sacral chordomas do not cause symptoms until the tumor is quite large, and sometimes a lump is the first sign of a sacral chordoma.

What types of diseases can be mistaken for chordoma?

Chordoma can be confused with other diseases, including:

  • Benign notochordal cell tumors (BNCT) – These benign spine tumors can be seen on an MRI or CT scan and can sometimes look like chordoma. However, BNCT stay confined within the bone and do not spread into other tissues like chordomas can. If you have a suspected BNCT, you should have an MRI or CT scan from time to time to look for changes. Images should be reviewed by a radiologist with expertise in bone tumors.
  • Chondrosarcoma – This type of bone cancer looks very similar to chordoma on CT and MRI. A specific type of MRI called diffusion MRI, or D-MRI, may help doctors tell the difference. Sometimes it is only possible to know a tumor is not chondrosarcoma after having a biopsy. Skull base chondrosarcomas usually respond better to radiation than skull base chordomas and have a better prognosis.
  • Giant cell tumor of the bone (GCTB) – These tumors look somewhat different on imaging tests than chordoma and tend to be located in the upper part of the sacrum.
  • Schwannoma – These tumors damage the bone differently than chordomas do, look different on imaging tests, and do not spread to nearby muscles or joints.
  • Other tumors of the spine and skull base – These include other bone cancers such as Ewing sarcoma and osteosarcoma, as well as a type of nervous system tumor called a myxopapillary ependymoma. Lymphoma, a cancer of the body’s immune system, and multiple myeloma, a blood cancer, can also cause tumors in these areas.
  • Metastasis (spread) of another cancer – Sometimes cancers in other places in the body can spread to the bones of the spine or skull base.

How is chordoma diagnosed?

Any biopsy or surgery to confirm the diagnosis of chordoma should be done at a center with expertise in chordoma treatment. If not done properly, these procedures can cause the chordoma to spread. If you cannot travel to see a doctor who has experience with chordoma, have your imaging tests sent to a doctor with chordoma experience for a second opinion before you get treatment or a biopsy.

Find an experienced doctor

Chordoma tumors are typically detected through imaging tests, which show organs and other structures inside the body, including tumors. The way the tumor looks on imaging tests can tell a radiologist whether it might be chordoma.

When a chordoma is suspected, you will need magnetic resonance imaging, also called MRI, to help doctors make a diagnosis and plan for treatment. An MRI is the best way to see a chordoma and how it is affecting the tissue around it, such as muscles, nerves, and blood vessels. No matter where the tumor is located, an MRI of the entire spine should be performed to see if the tumor may have spread to or developed in other areas of the spine. Chordoma is best seen on an MRI with a setting called T2 weighted imaging.

Another imaging test called computed tomography, also called CT or CAT scan, is recommended in addition to MRI if it is not certain whether the tumor is chordoma. CT scans of the chest, abdomen, and pelvis are recommended to make sure there is no spread of tumor. Imaging tests should be interpreted by a radiologist who has experience diagnosing bone tumors.

Imaging studies can show the possibility of a chordoma, but a definitive diagnosis can only be
made by a pathologist who examines a sample of tumor tissue under a microscope. For this reason, your medical team may consider taking a small sample of tissue from the tumor, called a biopsy, prior to surgery if the tumor can be reached safely (see important information below regarding biopsies).

Tissue samples should be evaluated by a pathologist who has experience diagnosing bone tumors. Your pathologist may test your tumor tissue for the presence of a protein called brachyury. Nearly all chordomas have high levels of brachyury, which makes it helpful for diagnosis.

Are biopsies recommended for suspected chordomas?

For sacral and mobile spine tumors, a trocar CT-guided biopsy is recommended and should be done from the back. Trocar CT-guided biopsy uses a CT scan to precisely direct the biopsy needle to the correct location. The biopsy needle is enclosed in a tube to keep tumor cells from spreading along the path of the needle – this is often called seeding. Talk with your doctors to learn if they plan to use this method if a biopsy is recommended.

Skull base tumors can be difficult to reach safely for a biopsy, so your surgeon may opt for a biopsy during surgery. This means that a pathologist will be prepared to examine a sample of tumor tissue removed at the start of surgery and give an immediate diagnosis, and the surgical team will proceed with surgery based on that information.

If you have a biopsy before surgery, it is recommended that your surgeon take out the tissue around the area of the biopsy during surgery in order to remove any chordoma cells that might have spread when the biopsy disturbed the tumor.


The following papers were used as references for the content on this page. They provide comprehensive information about the diagnosis, treatment, epidemiology, and prognostic factors of chordoma.

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