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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]]. | |||
*Rare variant of chondrosarcoma. | *2–10% of primary chondrosarcomas. | ||
*2–10% of primary chondrosarcomas | *Adolescents and young adults. | ||
*Adolescents and young adults | *Female predilection. | ||
*Female predilection | *Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meninges). | ||
*Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the | *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> | ||
*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> | |||
*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> | |||
==Gross== | ==Gross== | ||
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==Microscopic== | ==Microscopic== | ||
Features: | |||
*Malignant tumour with a characteristic biphasic pattern. | |||
**Cellular poorly differentiated [[small round blue cell tumours|small round blue cells]]. | |||
**Islands of well-differentiated hyaline [[cartilage]]. | |||
***Progressive maturation of cartilage towards the center. | |||
***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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*Osteocalcin (negative in small cells). | *Osteocalcin (negative in small cells). | ||
*CD99 - (positive in small cells) | *CD99 - (positive in small cells) | ||
==Molecular== | ==Molecular== | ||