Difference between revisions of "Chordoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Chordoma_-_high_mag.jpg
| Width      =
| Caption    = Chordoma. [[HPS stain]].
| Micro      = ''physaliphorous cells'' (also ''bubble cells'') - very large clear bubble with a sharp border, bubble does not compress nucleus; islands of cells surrounded by fibrous tissue; myxoid background
| Subtypes  =
| LMDDx      = [[chondrosarcoma]], [[myxoid lesions]]
| Stains    =
| IHC        = S-100 +ve, AE1/AE3 +ve, Brachyury +ve, EMA +ve
| EM        =
| Molecular  =
| IF        =
| Gross      = myxoid
| Grossing  =
| Site      = sacrum or clivus
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Chordoma''' is an uncommon tumour in [[neuropathology]].
'''Chordoma''' is an uncommon tumour in [[neuropathology]].


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DDx:
DDx:
*[[Chondrosarcoma]].
*[[Chondrosarcoma]].
*[[Myxoid lesions]].


===Images===
===Images===
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==IHC==
==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>
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.
*S-100 +ve.
*AE1/AE3 +ve.
*AE1/AE3 +ve.
*Brachyury +ve -- '''key stain'''.
*Brachyury +ve -- '''key stain'''.

Revision as of 15:50, 1 October 2013

Chordoma
Diagnosis in short

Chordoma. HPS stain.
LM physaliphorous cells (also bubble cells) - very large clear bubble with a sharp border, bubble does not compress nucleus; islands of cells surrounded by fibrous tissue; myxoid background
LM DDx chondrosarcoma, myxoid lesions
IHC S-100 +ve, AE1/AE3 +ve, Brachyury +ve, EMA +ve
Gross myxoid
Site sacrum or clivus
Prevalence uncommon

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 - intermed. mag. (WC)

  • Chordoma - high mag. (WC)

  • Chordoma - very high mag. (WC)

www:

IHC

Features:[3][4]

  • S-100 +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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