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# | '''Chordoma''' is an uncommon tumour in [[neuropathology]]. | ||
==General== | |||
*Location: usually sacrum or clivus. | |||
*It is a [[bone tumour]]. | |||
==Gross== | |||
*Soft, gelatinous, lobulated.<ref>URL: [http://www.histopathology-india.net/Chordoma.htm http://www.histopathology-india.net/Chordoma.htm]. Accessed on: 12 April 2012.</ref> | |||
DDx: | |||
*Bony [[metastasis]] (mucinous carcinoma) - typically multifocal. | |||
Image: | |||
*[http://www.flickr.com/photos/bc_the_path/4428268879/in/photostream/ Chordoma (flickr.com)]. | |||
==Microscopic== | |||
Features:<ref name=Ref_DCHH184>{{Ref DCHH|184}}</ref> | |||
*Architecture: islands of cells surrounded by fibrous tissue. | |||
**Also described as "lobulated" architecture; may not be apparent. | |||
*[[Myxoid]] background - grey extracellular material, variable amount present. | |||
*Mixed cell population: | |||
*#Abundant eosinophilic cytoplasm. | |||
*#''Physaliphorous cells'' or ''bubble cells'' - '''key feature'''. | |||
*#*Have a very large clear bubble with a sharp border; bubble does not compress nucleus - nucleus may be in bubble. | |||
DDx: | |||
*[[Chondrosarcoma]]. | |||
===Images=== | |||
<gallery> | |||
Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC) | |||
Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case312/micro.html Chordoma - sacrum - several images (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case689.html Chordoma - additional case with several images (upmc.edu)]. | |||
==IHC== | |||
Features:<ref>URL: [http://path.upmc.edu/cases/case312/micro.html http://path.upmc.edu/cases/case312/micro.html]. Accessed on: 14 January 2012.</ref><ref name=pmid2431128>{{Cite journal | last1 = Coindre | first1 = JM. | last2 = Rivel | first2 = J. | last3 = Trojani | first3 = M. | last4 = De Mascarel | first4 = I. | last5 = De Mascarel | first5 = A. | title = Immunohistological study in chordomas. | journal = J Pathol | volume = 150 | issue = 1 | pages = 61-3 | month = Sep | year = 1986 | doi = 10.1002/path.1711500110 | PMID = 2431128 }}</ref> | |||
*S100 +ve. | |||
*AE1/AE3 +ve. | |||
*Brachyury +ve -- '''key stain'''. | |||
**Protein important for axial development, affects notochord development.<ref name=omim601397>{{OMIM|601397}}</ref> | |||
**''Brachyury'' literally means ''short tail''.<ref>URL: [http://www.jstor.org/pss/86845 http://www.jstor.org/pss/86845]. Accessed on: 18 May 2010.</ref> | |||
*EMA +ve. | |||
==See also== | |||
*[[CNS tumours]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology tumours]] |
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