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Diseases

Genetic and Rare Diseases Information Center (GARD)

Chordoma


Other Names for this Disease
  • Notochordal sarcoma
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Treatment

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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.[1]  Surgery is followed by radiation therapy to destroy any cancer cells that may remain after surgery.[1]  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.[2]  Approximately 60-70% of individuals treated with combined surgery and radiation therapy remained tumor-free for at least five years.[2]
Last updated: 10/24/2011

References
  1. Lanzino G, Dumont AS, Lopes MB, Laws ER Jr. Skull base chordomas: overview of disease, management options, and outcome. Neurosurgical Focus. 2001; 10:E12. http://www.ncbi.nlm.nih.gov/pubmed/16734404. Accessed 10/17/2011.
  2. Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O. Comprehensive review of intracranial chordoma. Radiographics. 2003; 23:995-1009. http://www.ncbi.nlm.nih.gov/pubmed/12853676. Accessed 10/17/2011.


GARD Video Tutorial

  • Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.

    Finding Treatment Information

Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Chordoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • Notochordal sarcoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.