Difference between revisions of "Chordoma"

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#redirect [[Neuropathology tumours#Chordoma]]
'''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]]

Revision as of 15:43, 1 October 2013

Chordoma is an uncommon tumour in neuropathology.

General

  • Location: usually sacrum or clivus.
  • It is a bone tumour.

Gross

  • Soft, gelatinous, lobulated.[1]

DDx:

  • Bony metastasis (mucinous carcinoma) - typically multifocal.

Image:

Microscopic

Features:[2]

  • 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:
    1. Abundant eosinophilic cytoplasm.
    2. 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:

Images

  • Chordoma - low mag. (WC)

  • Chordoma - high mag. (WC)

www:

IHC

Features:[3][4]

  • S100 +ve.
  • AE1/AE3 +ve.
  • Brachyury +ve -- key stain.
    • Protein important for axial development, affects notochord development.[5]
    • Brachyury literally means short tail.[6]
  • EMA +ve.

See also

References

  1. ↑ URL: http://www.histopathology-india.net/Chordoma.htm. Accessed on: 12 April 2012.
  2. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 184. ISBN 978-0470519035.
  3. ↑ URL: http://path.upmc.edu/cases/case312/micro.html. Accessed on: 14 January 2012.
  4. ↑ Coindre, JM.; Rivel, J.; Trojani, M.; De Mascarel, I.; De Mascarel, A. (Sep 1986). "Immunohistological study in chordomas.". J Pathol 150 (1): 61-3. doi:10.1002/path.1711500110. PMID 2431128.
  5. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601397
  6. ↑ URL: http://www.jstor.org/pss/86845. Accessed on: 18 May 2010.
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