Difference between revisions of "Mesenchymal chondrosarcoma"

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Mesenchymal chondrosarcoma (view source)

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'''Mesenchymal chondrosarcoma''' is a rare type of [[chondrosarcoma]] found in the soft tissue.
'''Mesenchymal chondrosarcoma''' is a rare type of [[chondrosarcoma]] that is found in the [[soft tissue lesions|soft tissue]].


==General==  
==General==  
*Rare variant of [[chondrosarcoma]].
*Arise in soft tissue; this is where the name comes from.<ref name=pmid14161087>{{cite journal |author=Dowling EA |title=Mesenchymal chondrosarcoma |journal=J Bone Joint Surg Am |volume=46 |issue= |pages=747–54 |year=1964 |month=June |pmid=14161087 |doi= |url=http://www.ejbjs.org/cgi/reprint/46/4/747.pdf}}</ref>
*2–10% of primary chondrosarcomas.
*Adolescents and young adults.
*Rare variant of chondrosarcoma.
*Female predilection.
*2–10% of primary chondrosarcomas
*Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meninges).
*Adolescents and young adults
*The ''mesenchymal'' in the name refers to the ability to arise in soft tissues.<ref name=pmid14161087>{{cite journal |author=Dowling EA |title=Mesenchymal chondrosarcoma |journal=J Bone Joint Surg Am |volume=46 |issue= |pages=747–54 |year=1964 |month=June |pmid=14161087 |doi= |url=http://www.ejbjs.org/cgi/reprint/46/4/747.pdf}}</ref>
*Female predilection
*Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meminges)
*Conceptualized as originating from a pleuripotential mesenchymal cell with foci recapitulating enchondral ossification.
*Conceptualized as originating from a pleuripotential mesenchymal cell with foci recapitulating enchondral ossification.
*The small cells appear to be an undifferentiated cartilage stem cell which “differentiate” into benign cartilage <ref>{{Cite journal  | last1 = Fanburg-Smith | first1 = JC. | last2 = Auerbach | first2 = A. | last3 = Marwaha | first3 = JS. | last4 = Wang | first4 = Z. | last5 = Rushing | first5 = EJ. | title = Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 653-62 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.11.006 | PMID = 20138330 }}
*The small cells appear to be an undifferentiated cartilage stem cell which “differentiate” into benign cartilage.<ref name=pmid20138330>{{Cite journal  | last1 = Fanburg-Smith | first1 = JC. | last2 = Auerbach | first2 = A. | last3 = Marwaha | first3 = JS. | last4 = Wang | first4 = Z. | last5 = Rushing | first5 = EJ. | title = Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 653-62 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.11.006 | PMID = 20138330 }}</ref>
</ref>.
*This is where the name comes from.<ref name=pmid14161087>{{cite journal |author=Dowling EA |title=Mesenchymal chondrosarcoma |journal=J Bone Joint Surg Am |volume=46 |issue= |pages=747–54 |year=1964 |month=June |pmid=14161087 |doi= |url=http://www.ejbjs.org/cgi/reprint/46/4/747.pdf}}</ref>


==Gross==  
==Gross==  
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==Microscopic==
==Microscopic==
*"White clouds in a blue sky".
Features:
*Malignant tumor with a characteristic biphasic pattern
*Malignant tumour with a characteristic biphasic pattern.
**Cellular poorly differentiated small round blue cells
**Cellular poorly differentiated [[small round blue cell tumours|small round blue cells]].
**Islands of well-differentiated hyaline cartilage
**Islands of well-differentiated hyaline [[cartilage]].
***Progressive maturation of cartilage towards the center
***Progressive maturation of cartilage towards the center.
***Central calcification or bone formation
***Central calcification or [[bone]] formation.
**Can have a [[staghorn vessels|hemangiopericytomatous vascular pattern]].
 
Notes:
*May be described as ''white clouds in a dark blue sky''.
 
===DDX===
*[[Hemangiopericytoma]] - no cartilage.
*[[Lymphoma]] - Sox9 negative, CD45 positive.
*Metaplastic [[glioblastoma]] - usually older adults - GFAP positive.
*[[Chondrosarcoma]] (NOS) - usually older adults - hyaline cartilage is malignant.
*[[Small cell osteosarcoma]] - Sox10 negative, no cartilage.
*[[Ewing sarcoma]] - both are CD99 positive but ES is Sox9 negative, no cartilage.
*Monophasic [[synovial sarcoma]] - also can have the hemangiopericytomatous vasculature.
 
Note:
*Depends a bit on where the tumour is located and how much cartilage is readily visible.


===Images===
===Images===
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www:
www:
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)].
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)].
*Pathology Outlines - Hemangiopericytomatous example [http://pathologyoutlines.com/wick/chondrosarcoma%20mesenchymal%20type%20micro0007.jpg]
*Sarcoma Images - [http://www.sarcomaimages.com/index.php?v=Mesenchymal-Chondrosarcoma]
*CNS Atlas - [http://www.cnsatlas.com/medicalatlas/images/370/800_poIB6FYZQ73wh.jpg]
*CNS Atlas - [http://www.cnsatlas.com/medicalatlas/images/370/800_9oJtTQXXxaEB3.jpg]
*CNS Atlas - [http://www.cnsatlas.com/medicalatlas/images/370/800_8h2C7upeOj3EB.jpg]


==IHC==
==IHC==
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*S100 (positive in chondrocytes not in small cells).
*S100 (positive in chondrocytes not in small cells).
*Osteocalcin (negative in small cells).  
*Osteocalcin (negative in small cells).  
*CD99 - (positive in small cells)


==Molecular==
==Molecular==
t(8;8)(q21.1;q13.3) HEY1-NCOA2.<ref name=pmid24839999>{{Cite journal  | last1 = Panagopoulos | first1 = I. | last2 = Gorunova | first2 = L. | last3 = Bjerkehagen | first3 = B. | last4 = Boye | first4 = K. | last5 = Heim | first5 = S. | title = Chromosome aberrations and HEY1-NCOA2 fusion gene in a mesenchymal chondrosarcoma. | journal = Oncol Rep | volume = 32 | issue = 1 | pages = 40-4 | month = Jul | year = 2014 | doi = 10.3892/or.2014.3180 | PMID = 24839999 }}</ref>
*t(8;8)(q21.1;q13.3) HEY1-NCOA2.<ref name=pmid24839999>{{Cite journal  | last1 = Panagopoulos | first1 = I. | last2 = Gorunova | first2 = L. | last3 = Bjerkehagen | first3 = B. | last4 = Boye | first4 = K. | last5 = Heim | first5 = S. | title = Chromosome aberrations and HEY1-NCOA2 fusion gene in a mesenchymal chondrosarcoma. | journal = Oncol Rep | volume = 32 | issue = 1 | pages = 40-4 | month = Jul | year = 2014 | doi = 10.3892/or.2014.3180 | PMID = 24839999 }}</ref>
*Less commonly t(1;5)(q42;q32) IRF2BP2-CDX1<ref>{{cite journal |authors=Nyquist KB, Panagopoulos I, Thorsen J, Haugom L, Gorunova L, Bjerkehagen B, Fosså A, Guriby M, Nome T, Lothe RA, Skotheim RI, Heim S, Micci F |title=Whole-transcriptome sequencing identifies novel IRF2BP2-CDX1 fusion gene brought about by translocation t(1;5)(q42;q32) in mesenchymal chondrosarcoma |journal=PLoS ONE |volume=7 |issue=11 |pages=e49705 |date=2012 |pmid=23185413 |pmc=3504151 |doi=10.1371/journal.pone.0049705 |url=}}</ref>


==See also==
==See also==
*[[Cartilage]].
*[[Cartilage]].
*[[Chrondrosarcoma]].
*[[Chondrosarcoma]].


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
==External links==
*[http://www.webpathology.com/image.asp?case=332&n=41 Mesenchymal chondrosarcoma - images (webpathology.com)].
*[http://www.pathologyoutlines.com/topic/bonemesenchymalchondrosarcoma.html Mesenchymal chondrosarcoma (pathologyoutlines.com)].


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Chondro-osseous tumours]]
[[Category:Chondro-osseous tumours]]
Michael
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