Chordoma Foundation

Diagnosing chordoma

Clinical guidelines for chordoma diagnosis and treatmentThe treatment recommendations you find here were developed by the Chordoma Global Consensus Group – a multidisciplinary, international group of over 60 doctors who specialize in caring for chordoma patients. The Chordoma Foundation and the European Society for Medical Oncology brought this group together to define the recommendations for treating and diagnosing chordoma based on all available medical and scientific evidence. The resulting consensus guidelines were published in the medical journal The Lancet Oncology in February 2015.

You can also find these recommendations in our Expert Recommendations for the Diagnosis and Treatment of Chordoma booklet.

Download or request a hard copy of the booklet »

Diagnosing chordoma is not always easy. It can be confused with other types of tumors. Getting the correct diagnosis from the start is critical to being able to find and choose the best treatment.

If you have had an MRI and been given a probable or differential diagnosis of chordoma, it is very important to find doctors who have experience diagnosing and treating chordoma patients to confirm the diagnosis. If you have had a biopsy and chordoma is confirmed, or if you have received a chordoma diagnosis following surgery, it is important for your case to be evaluated by a multidisciplinary team of chordoma experts who can talk with you about what next steps you should take.

Once you have been given a diagnosis, it can be helpful to get a second opinion to confirm the diagnosis before making treatment decisions. As with any rare cancer, second and even third opinions are an important and reasonable step to take.

Have you been recently diagnosed?

Our Facing a new diagnosis page lists the next steps you can take to get the best possible care.

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. You should avoid having a biopsy or surgery to confirm the diagnosis of chordoma outside of a referral center because, if not done properly, these procedures can cause the chordoma to spread.

Visit our Doctor Directory to find doctors with chordoma experience »

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.

Could my tumor be something else?

Chordoma can be confused with other benign and cancerous tumors, 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.
  • Ecchordosis physaliphora (EP) – A benign, congenital tumor that forms from notochordal remnants and can be found anywhere along the spine. Like BNCTs, they can sometimes look like chordoma but they do not usually spread into other tissues like chordomas can.
  • 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.

Patient Navigation ServiceRequest Help

If you or someone you love might have chordoma, Chordoma Foundation Patient Navigators are here to help you get the best care possible. Our Patient Navigators are available by email and phone, Monday through Friday from 8 AM to 5 PM EST.

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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.

Visit our Doctor Directory to find doctors with chordoma experience »

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.

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Types of imaging tests

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.

In some cases doctors may order a positron emission tomography, or PET, scan. These are usually whole-body scans that look for widespread tumor activity. PET scans provide information about your body’s metabolic function; for cancer specifically, PET scans pick up on tumors that are rapidly using glucose (sugar). Cancer cells metabolize much more glucose than regular cells, so tumors “light up” on the scan. Because chordoma cells typically grow slowly, however, they are not always picked up as well by PET scans, so they are not widely used in diagnosing chordoma.

Are biopsies recommended for suspected chordomas?

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).

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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.

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.


References and further information

  1. Stacchiotti S, Sommer J, Chordoma Global Consensus Group. Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncology. 2015 Feb;16(2):e71-83. doi: 10.1016/S1470-2045(14)71190-8.
  2. Stacchiotti S, Gronchi A, Fossati P, et al. Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 2017 Jun 1;28(6):1230-1242. doi: 10.1093/annonc/mdx054.
  3. Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncology. 2012 Feb;13(2):e69-76. doi: 10.1016/S1470-2045(11)70337-0.
  4. Diaz RJ, Cusimano MD. The biological basis for the modern treatment of chordoma. J Neurooncol. 2011 Sep;104(2):411-22. doi: 10.1007/s11060-011-0559-8. Epub 2011 Mar 8.
  5. Sciubba DM, Cheng JJ, Petteys RJ, Weber KL, Frassica DA, Gokaslan ZL. Chordoma of the sacrum and vertebral bodies. J Am Acad Orthop Surg. 2009 Nov;17(11):708-17.
  6. Koutourousiou M, Snyderman CH, Fernandez-Miranda J, Gardner PA. Skull base chordomas. Otolaryngol Clin North Am. 2011 Oct;44(5):1155-71. doi: 10.1016/j.otc.2011.06.002. Epub 2011 Jul 21.
  7. Our list of commonly used terms can help you understand the medical and scientific terms you may hear when discussing your diagnosis with your care team.


The information provided herein is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your or your child’s physician about any questions you have regarding your or your child’s medical care. Never disregard professional medical advice or delay in seeking it because of something you have read on this Website.

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