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Genetic and Rare Diseases Information Center (GARD)

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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]  A chordoma occurs when these notochord cells start to grow into a tumor and spread into surrounding bone.  Approximately one third of chordomas occur at the base of the skull in a bone called the clivus and the most common symptoms are double vision (diplopia) and headaches.[2]  The average age of diagnosis for chordomas of the skull base is 38 years. [3]  Treatment often begins with surgery to remove as much of the tumor as possible, and radiation therapy may follow surgery to destroy any remaining tumor cells.  Chordomas often grow back in the original location after treatment (known as a recurrence); they do not often spread to distant parts of the body (metastasize).[2]  Though the cause of chordomas is unknown, there is currently a study aimed at identifying a potential genetic predisposition to this disease; more information on this study can be found on the National Cancer Institute's web site.
Last updated: 10/24/2011


  1. National Cancer Institute. Chordoma research. Genetic Epidemiology Branch. 2011; Accessed 10/19/2011.
  2. Lanzino G, Dumont AS, Lopes MB, Laws ER Jr. Skull base chordomas: overview of disease, management options, and outcome. Neurosurgical Focus. 2001; 10:E12. Accessed 10/17/2011.
  3. Soo MY. Chordoma: review of clinicoradiological features and factors affecting survival. Australasian Radiology. 2001; 45:427-434. Accessed 10/17/2011.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Chordoma. This website is maintained by the National Library of Medicine.
  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.  Click on the link to view information on this topic. 

In Depth Information

  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Chordoma. Click on the link to view a sample search on this topic.