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Diseases

Genetic and Rare Diseases Information Center (GARD)

Chordoma


Other Names for this Disease
  • Notochordal sarcoma
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Overview

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

References

  1. National Cancer Institute. Chordoma research. Genetic Epidemiology Branch. 2011; http://dceg.cancer.gov/geb/research/activeclinical/chordoma. Accessed 9/9/2015.
  2. Chordoma. Genetics Home Reference. May, 2015; http://ghr.nlm.nih.gov/condition/chordoma. Accessed 9/9/2015.
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Basic Information

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.
  • Online Mendelian Inheritance in Man (OMIM) is a 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. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • 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.
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.