Difference between revisions of "Chordoma"

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Chordoma_-_high_mag.jpg
| Image      = Chordoma_-_high_mag.jpg  
| Width      =
| Width      =
| Caption    = Chordoma. [[HPS stain]].
| 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
| 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  =
| Subtypes  =
| LMDDx      = [[chondrosarcoma]], [[myxoid lesions]], [[parachordoma]]
| LMDDx      = [[chondrosarcoma]], [[myxoid lesions]], [[parachordoma]], chordoid lesions (e.g. chordoid glioma, [[chordoid meningioma]]), [[metastasis]] (e.g. [[clear cell renal cell carcinoma]])
| Stains    =
| Stains    =
| IHC        = S-100 +ve, AE1/AE3 +ve, Brachyury +ve, EMA +ve
| IHC        = S-100 +ve, [[AE1/AE3]] +ve, Brachyury +ve, [[EMA]] +ve
| EM        =
| EM        =
| Molecular  =
| Molecular  =
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==General==
==General==
*Location: usually sacrum or clivus.
*Location: usually sacrum or clivus.
*It is a [[bone tumour]].
*It is a malignant [[bone tumour]] (1-4% of all primary bone tumors).
*Usually after age 30.
 
===Classification===
* Chordoma, NOS (ICD-O: 9370/3).
* Chondroid chordoma (ICD-O: 9371/3).
* Dedifferentiated chordoma (ICD-O: 9372/3).


==Gross==
==Gross==
Line 54: Line 60:


DDx:
DDx:
*[[Chondrosarcoma]].
*[[Chondrosarcoma]] - negative for EMA and cytokeratins. Beware of 'chondroid' chordoma.
*[[Myxoid lesions]].
*[[Myxoid lesions]].
**Myxopapillary ependymoma.
**[[Myxopapillary ependymoma]].
**Myxoid liposarcoma
**[[Myxoid liposarcoma]]  - negative for EMA and cytokeratins.
*'Choroid lesions'
*Chordoid lesions.
**Choroid meningioma.
**[[Chordoid meningioma]].
**Choroid glioma
**Chordoid glioma<ref>{{Cite journal  | last1 = Zarghouni | first1 = M. | last2 = Vandergriff | first2 = C. | last3 = Layton | first3 = KF. | last4 = McGowan | first4 = JB. | last5 = Coimbra | first5 = C. | last6 = Bhakti | first6 = A. | last7 = Opatowsky | first7 = MJ. | title = Chordoid glioma of the third ventricle. | journal = Proc (Bayl Univ Med Cent) | volume = 25 | issue = 3 | pages = 285-6 | month = Jul | year = 2012 | doi =  | PMID = 22754136 }}</ref> - location, location, location.
**Large notochordal rest.
**Large notochordal rest - only evidence of destructive growth can identify a chordoma.
*Metastasis
*[[Metastasis]].
**Metastatic signet ring cell adenocarcinoma.
**Metastatic [[signet ring cell carcinoma]] - negative for S100 and brachyury, [[clinical history]] (important).
**Metastatic clear cell renal cell carcinoma.
**Metastatic [[clear cell renal cell carcinoma]] - negative for S100 and brachyury, clinical history (important).
*[[Parachordoma]] - extremely rare.
*[[Parachordoma]] - extremely rare.


===Images===
===Images===
====Case 1====
<gallery>
<gallery>
Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC)
Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC)
Line 73: Line 80:
Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC)
Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC)
Image:Chordoma - very high mag.jpg| Chordoma - very high mag. (WC)
Image:Chordoma - very high mag.jpg| Chordoma - very high mag. (WC)
</gallery>
====Case 2====
<gallery>
Image: Chordoma -- low mag.jpg | Chordoma - low mag. (WC)
Image: Chordoma -- intermed mag.jpg | Chordoma - intermed. mag. (WC)
Image: Chordoma - alt -- intermed mag.jpg | Chordoma - intermed. mag. (WC)
Image: Chordoma -- high mag.jpg | Chordoma - high mag. (WC)
</gallery>
====Case 3====
<gallery>
Image:Bone Chordoma 2 HP2.JPG|Physaliphorous cells. (SKB)
Image:Bone Chordoma 2 HP2.JPG|Physaliphorous cells. (SKB)
Image:Bone Chordoma 2 LP PA.JPG|Low power view.  Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB)
Image:Bone Chordoma 2 LP PA.JPG|Low power view.  Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB)
Line 80: Line 97:
Image:Bone Chordoma HP PA.JPG|Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB)
Image:Bone Chordoma HP PA.JPG|Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB)
</gallery>
</gallery>
www:
====www====
*[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)].
*[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/case312/micro.html Chordoma - sacrum - several images (upmc.edu)].
Line 87: Line 104:
==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>
*S-100 +ve.
*S-100 +ve - '''important'''.
*AE1/AE3 +ve.
*[[AE1/AE3]] +ve - '''important'''.
*Brachyury +ve -- '''key stain'''.
*Brachyury +ve - '''key stain'''.
**Protein important for axial development, affects notochord development.<ref name=omim601397>{{OMIM|601397}}</ref>
**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>
**''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.
*[[EMA]] +ve.
*[[PAX8]] -ve (1 +ve (weak) of 12.<ref name=pmid21102418>{{Cite journal  | last1 = Sangoi | first1 = AR. | last2 = Karamchandani | first2 = J. | last3 = Lane | first3 = B. | last4 = Higgins | first4 = JP. | last5 = Rouse | first5 = RV. | last6 = Brooks | first6 = JD. | last7 = McKenney | first7 = JK. | title = Specificity of brachyury in the distinction of chordoma from clear cell renal cell carcinoma and germ cell tumors: a study of 305 cases. | journal = Mod Pathol | volume = 24 | issue = 3 | pages = 425-9 | month = Mar | year = 2011 | doi = 10.1038/modpathol.2010.196 | PMID = 21102418 }}</ref>).
*Other keratins:<ref name=pmid9195570>{{Cite journal  | last1 = Naka | first1 = T. | last2 = Iwamoto | first2 = Y. | last3 = Shinohara | first3 = N. | last4 = Chuman | first4 = H. | last5 = Fukui | first5 = M. | last6 = Tsuneyoshi | first6 = M. | title = Cytokeratin subtyping in chordomas and the fetal notochord: an immunohistochemical analysis of aberrant expression. | journal = Mod Pathol | volume = 10 | issue = 6 | pages = 545-51 | month = Jun | year = 1997 | doi =  | PMID = 9195570 }}</ref>
**CK8 +ve (16/16).
**CK19 +ve (16/16).
**CK18 +ve/-ve (9 +ve/16).
**[[CK7]] -ve (1 +ve/16).
**[[CK20]] -ve (0 +ve/16).
 
Classic chordoma panel:<ref name=pmid24296480>{{Cite journal  | last1 = Shen | first1 = J. | last2 = Shi | first2 = Q. | last3 = Lu | first3 = J. | last4 = Wang | first4 = DL. | last5 = Zou | first5 = TM. | last6 = Yang | first6 = HL. | last7 = Zhu | first7 = GQ. | title = Histological study of chordoma origin from fetal notochordal cell rests. | journal = Spine (Phila Pa 1976) | volume = 38 | issue = 25 | pages = 2165-70 | month = Dec | year = 2013 | doi = 10.1097/BRS.0000000000000010 | PMID = 24296480 }}</ref>
*EMA, AE1/AE3, CAM5.2, vimentin, S-100.
 
Key points: 
*Brachyury is not a commonly stocked antibody.
*Chordoma will be S100 '''''and''''' epithelial marker positive. 
*Many other items in the DDx will be either S100 '''''or''''' epithelial marker positive.


==See also==
==See also==
*[[CNS tumours]].
*[[CNS tumours]].
*[[Parachordoma]].


==References==
==References==

Latest revision as of 19:36, 31 July 2016

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, parachordoma, chordoid lesions (e.g. chordoid glioma, chordoid meningioma), metastasis (e.g. clear cell renal cell carcinoma)
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 malignant bone tumour (1-4% of all primary bone tumors).
  • Usually after age 30.

Classification

  • Chordoma, NOS (ICD-O: 9370/3).
  • Chondroid chordoma (ICD-O: 9371/3).
  • Dedifferentiated chordoma (ICD-O: 9372/3).

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

Case 1

  • Chordoma - low mag. (WC)

  • Chordoma - intermed. mag. (WC)

  • Chordoma - high mag. (WC)

  • Chordoma - very high mag. (WC)

Case 2

  • Chordoma - low mag. (WC)

  • Chordoma - intermed. mag. (WC)

  • Chordoma - intermed. mag. (WC)

  • Chordoma - high mag. (WC)

Case 3

  • Physaliphorous cells. (SKB)

  • Low power view. Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB)

  • Physaliphorous cells. (SKB)

  • Physaliphorous cells. (SKB)

  • Physaliphorous cells.(SKB)

  • Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB)

www

IHC

Features:[4][5]

  • S-100 +ve - important.
  • AE1/AE3 +ve - important.
  • Brachyury +ve - key stain.
    • Protein important for axial development, affects notochord development.[6]
    • Brachyury literally means short tail.[7]
  • EMA +ve.
  • PAX8 -ve (1 +ve (weak) of 12.[8]).
  • Other keratins:[9]
    • CK8 +ve (16/16).
    • CK19 +ve (16/16).
    • CK18 +ve/-ve (9 +ve/16).
    • CK7 -ve (1 +ve/16).
    • CK20 -ve (0 +ve/16).

Classic chordoma panel:[10]

  • EMA, AE1/AE3, CAM5.2, vimentin, S-100.

Key points:

  • Brachyury is not a commonly stocked antibody.
  • Chordoma will be S100 and epithelial marker positive.
  • Many other items in the DDx will be either S100 or epithelial marker positive.

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. ↑ Zarghouni, M.; Vandergriff, C.; Layton, KF.; McGowan, JB.; Coimbra, C.; Bhakti, A.; Opatowsky, MJ. (Jul 2012). "Chordoid glioma of the third ventricle.". Proc (Bayl Univ Med Cent) 25 (3): 285-6. PMID 22754136.
  4. ↑ URL: http://path.upmc.edu/cases/case312/micro.html. Accessed on: 14 January 2012.
  5. ↑ 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.
  6. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601397
  7. ↑ URL: http://www.jstor.org/pss/86845. Accessed on: 18 May 2010.
  8. ↑ Sangoi, AR.; Karamchandani, J.; Lane, B.; Higgins, JP.; Rouse, RV.; Brooks, JD.; McKenney, JK. (Mar 2011). "Specificity of brachyury in the distinction of chordoma from clear cell renal cell carcinoma and germ cell tumors: a study of 305 cases.". Mod Pathol 24 (3): 425-9. doi:10.1038/modpathol.2010.196. PMID 21102418.
  9. ↑ Naka, T.; Iwamoto, Y.; Shinohara, N.; Chuman, H.; Fukui, M.; Tsuneyoshi, M. (Jun 1997). "Cytokeratin subtyping in chordomas and the fetal notochord: an immunohistochemical analysis of aberrant expression.". Mod Pathol 10 (6): 545-51. PMID 9195570.
  10. ↑ Shen, J.; Shi, Q.; Lu, J.; Wang, DL.; Zou, TM.; Yang, HL.; Zhu, GQ. (Dec 2013). "Histological study of chordoma origin from fetal notochordal cell rests.". Spine (Phila Pa 1976) 38 (25): 2165-70. doi:10.1097/BRS.0000000000000010. PMID 24296480.
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