Chordoma
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A chordoma (also known as choroid meningioma, chordocarcinoma, chordoepithelioma, or notochordoma) is a malignant tumor arising from the embryonic remains of the notochord.[1]
Such malformations can occur anywhere in the bony tissue surrounding the spinal cord, though typically at its cranial or caudal end. The incidence rate is about 1 in a million in the United States.
At least one susceptibility gene has been identified [2] but currently, no therapy is in sight, leaving affected patients with an average life expectancy of five to ten years after diagnosis.
References
- ↑ Anonymous (2024), Chordoma (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ Yang XR, Ng D, Alcorta DA, Liebsch NJ, Sheridan E, Li S et al. (2009). "T (brachyury) gene duplication confers major susceptibility to familial chordoma". Nat Genet 41 (11): 1176-8. DOI:10.1038/ng.454. PMID 19801981. Research Blogging. [e]
- Based on comparative genomic hybridization data from four affected families, variations in the 6q27 region of the human chromosome 6 were found to be correlated with chordoma, thus suggesting the T gene as a potential susceptibility gene for the disease.