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Mesenchymal chondrosarcoma (view source)
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'''Mesenchymal chondrosarcoma''' is a type [[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> | |||
*Rare variant of chondrosarcoma. | |||
Microscopic: | |||
Features: | |||
*"White clouds in a blue sky". | |||
Image: | |||
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)]. | |||
==General== | |||
*Rare variant of chondrosarcoma. | |||
*2–10% of primary chondrosarcomas | |||
*Adolescents and young adults | |||
*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. | |||
*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 }} | |||
</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== | |||
Pink and fleshy with foci of calcification. | |||
==Microscopic== | |||
*"White clouds in a blue sky". | |||
*Malignant tumor with a characteristic biphasic pattern | |||
**Cellular poorly differentiated small round blue cells | |||
**Islands of well-differentiated hyaline cartilage | |||
***Progressive maturation of cartilage towards the center | |||
***Central calcification or bone formation | |||
==IHC== | |||
*SOX9 (positive in small cells and chondrocytes) <ref>{{Cite journal | last1 = Pang | first1 = ZG. | last2 = He | first2 = XZ. | last3 = Wu | first3 = LY. | last4 = Wei | first4 = W. | last5 = Liu | first5 = XY. | last6 = Liao | first6 = DY. | last7 = Li | first7 = FY. | last8 = Zhang | first8 = XL. | title = [Clinicopathologic and immunohistochemical study of 23 cases of mesenchymal chondrosarcoma]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 40 | issue = 6 | pages = 368-72 | month = Jun | year = 2011 | doi = | PMID = 21914343 }}</ref> | |||
*S100 (positive in chondrocytes not in small cells) | |||
*Osteocalcin (negative in small cells) | |||
==Molecular== | |||
t(8;8)(q21.1;q13.3) HEY1-NCOA2 <ref>{{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> | |||
==Photos== | |||
<gallery> | |||
*Image:Bone Chondrosarcoma Mesenchymal MP5 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB) | |||
*Image:Bone Chondrosarcoma Mesenchymal MP PA.JPG|Islands of cartilage in a background of small blue cells.(SKB) | |||
*Image:Bone Chondrosarcoma Mesenchymal LP2 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB) | |||
*Image:Bone Chondrosarcoma Mesenchymal HP5 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB) | |||
*Image:Bone Chondrosarcoma Mesenchymal MP2 PA.JPG|Islands of cartilage in a background of small blue cells.(SKB) | |||
*Image:Bone Chondrosarcoma Mesenchymal MP7.JPG|Islands of cartilage in a background of small blue cells.(SKB) | |||
*Image:Bone Chondrosarcoma Mesenchymal HP3 PA.JPG|Small blue cells predominate.(SKB) | |||
</gallery> | |||
==See also== | |||
Image: | |||
*[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Chondro-osseous tumours]] | |||