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Genetic and Rare Diseases Information Center (GARD)


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Your Question

Three years ago, I was diagnosed with "Clivus of Chordoma."  Since then, I have had four surgeries to remove as much of the tumor as possible, and I also went through a rigorous radiation therapy treatment program to shrink what was left of the tumor after the surgeries.  I would like any information you might have on this type of tumor. 

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is a chordoma?

A chordoma is a rare tumor that develops from cells of the notochord, a structure that is present in the developing embryo and is important for the development of the spine. The notochord usually disappears before birth, though a few cells may remain embedded in the bones of the spine or at the base of the skull.[1] [2] Chordomas can occur anywhere along the spine. Approximately half of all chordomas occur at the base of the spine; approximately one third occur at the base of the skull. Chordomas grow slowly, extending gradually into the surrounding bone and soft tissue. The actual symptoms depend on the location of the chordoma. A chordoma at the base of the skull may lead to double vision and headaches. A chordoma that occurs at the base of the spine may cause problems with bladder and bowel function. Chordomas typically occur in adults between the ages of 40 and 70. In many cases, the cause of the chordoma remains unknown. Recent studies have shown that changes in the T gene have been associated with chordoma in a small set of families. In these families an inherited duplication of the T gene is associated with an increased risk of developing chordoma. People with this inherited duplication inherit an increased risk for the condition, not the condition itself.[2]

Last updated: 9/9/2015

How might a chordoma be treated?

Unfortunately, because chordomas are quite rare, the best treatment for these tumors has yet to be determined.  The current treatment for chordoma of the clivus often begins with surgery (resection) to remove as much of the tumor as possible.  The extent of surgery, or the amount of tumor that may be removed, depends on the location of the tumor and how close it is to critical structures in the brain.[3]  Surgery is followed by radiation therapy to destroy any cancer cells that may remain after surgery.[3]  Several studies have suggested that proton beam radiation or combined proton/photon radiation may be more effect than conventional photon radiation therapy for treating chordomas of the skull base because proton radiation may allow for a greater dose of radiation to be delivered to the tumor without damaging the surrounding normal tissues.[4]  Approximately 60-70% of individuals treated with combined surgery and radiation therapy remained tumor-free for at least five years.[4]
Last updated: 10/24/2011

What is the prognosis for chordoma?

It is estimated that 51% of individuals diagnosed with chordoma of the skull base are alive 5 years after the initial diagnosis, and appromiately 35%  are alive at 10 years.[3]   For those individuals whose tumor has regrown after initial treatment (a recurrence), 43% are living 3 years after the recurrence, and 7% are alive at 5 years.[5]
Last updated: 10/24/2011

How can I learn about research involving chordoma?

For a list of resources where you can find out about chordoma research, visit the Research & Clinical Trials tab.

The U.S. National Institutes of Health, through the National Library of Medicine, developed to provide patients, family members, and members of the public with current information on clinical research studies.  Currently there are several studies enrolling people with chordoma and aim to improve treatment for this disease.  After you click on a study, review its "eligibility" criteria to determine its appropriateness.  Use the study's contact information to learn more.  Check the site often for updates.

You can also contact the Patient Recruitment and Public Liaison Office at the National Institutes of Health (NIH).  We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if there are any other trials for chordoma.

Patient Recruitment and Public Liaison Office
NIH Clinical Center
National Institutes of Health
Bethesda, Maryland 20892,2655
Toll-free: 800-411-1222
Fax: 301-480-9793
Last updated: 10/24/2011

See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.