Difference between revisions of "Mesenchymal chondrosarcoma"

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==General==  
==General==  
   
   
*Arises 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.
*Rare variant of chondrosarcoma.
*2–10% of primary chondrosarcomas
*2–10% of primary chondrosarcomas
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*Female predilection
*Female predilection
*Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meminges)
*Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meminges)
*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>{{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==  

Revision as of 10:55, 20 November 2014

Mesenchymal chondrosarcoma is a rare type of chondrosarcoma found in the soft tissue.

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)
  • The 'mesenchymal' in the name refers to the ability to arise in soft tissues.[1]
  • 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 [2].

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

Images

www:

IHC

  • SOX9 (positive in small cells and chondrocytes).[3]
  • S100 (positive in chondrocytes not in small cells).
  • Osteocalcin (negative in small cells).

Molecular

t(8;8)(q21.1;q13.3) HEY1-NCOA2.[4]

See also

References

  1. Dowling EA (June 1964). "Mesenchymal chondrosarcoma". J Bone Joint Surg Am 46: 747–54. PMID 14161087. http://www.ejbjs.org/cgi/reprint/46/4/747.pdf.
  2. Fanburg-Smith, JC.; Auerbach, A.; Marwaha, JS.; Wang, Z.; Rushing, EJ. (May 2010). "Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases.". Hum Pathol 41 (5): 653-62. doi:10.1016/j.humpath.2009.11.006. PMID 20138330.
  3. Pang, ZG.; He, XZ.; Wu, LY.; Wei, W.; Liu, XY.; Liao, DY.; Li, FY.; Zhang, XL. (Jun 2011). "[Clinicopathologic and immunohistochemical study of 23 cases of mesenchymal chondrosarcoma].". Zhonghua Bing Li Xue Za Zhi 40 (6): 368-72. PMID 21914343.
  4. Panagopoulos, I.; Gorunova, L.; Bjerkehagen, B.; Boye, K.; Heim, S. (Jul 2014). "Chromosome aberrations and HEY1-NCOA2 fusion gene in a mesenchymal chondrosarcoma.". Oncol Rep 32 (1): 40-4. doi:10.3892/or.2014.3180. PMID 24839999.