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 its mobile parts at the cranial or caudal end. The prevalence is about 1 in a million in the United States.[2]
At least one susceptibility gene has been identified[3] but currently, no therapy is in sight, leaving affected patients with an average life expectancy of five to ten years after diagnosis.
(CC) Photo: Larizza et al., 2005
Different immunohistochemical markers of chondroma. A: E-E, × 5; chordoma composed of nests and cords of tumour cells; B: E-E, × 20; physaliphorous cells containing multiple clear cytoplasmic vacuoles; C: S-100, × 20; immunohistochemistry showing staining for S-100 protein; D: CK, × 20; immunohistochemistry showing staining for cytokeratin; E: EMA, × 20; immunohistochemistry showing staining for EMA; F: Vimentin, × 20; immunohistochemistry showing staining for Vimentin.
Different immunohistochemical markers of chondroma. A: E-E, × 5; chordoma composed of nests and cords of tumour cells; B: E-E, × 20; physaliphorous cells containing multiple clear cytoplasmic vacuoles; C: S-100, × 20; immunohistochemistry showing staining for S-100 protein; D: CK, × 20; immunohistochemistry showing staining for cytokeratin; E: EMA, × 20; immunohistochemistry showing staining for EMA; F: Vimentin, × 20; immunohistochemistry showing staining for Vimentin.
References
- ↑ Anonymous (2024), Chordoma (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM (2001). "Chordoma: incidence and survival patterns in the United States, 1973-1995". Cancer Causes Control 12 (1): 1-11. DOI:10.1023/A:1008947301735. PMID 11227920. Research Blogging. [e]
- Reports on a long-term study involving 400 patients with microscopically confirmed chordoma, revealing an overall prevalence of about 1 per million in the United States and a median survival time of about 6 years after diagnosis. It also shows that cranial, spinal and sacral locations each represent about one third of the total number of cases, and that males are affected about twice as often as females.
- ↑ 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.