Difference between revisions of "Mesenchymal chondrosarcoma"

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#redirect [[Chondrosarcoma#Mesenchymal_chondrosarcoma]]
'''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]]

Revision as of 09:06, 20 November 2014

Mesenchymal chondrosarcoma is a type chondrosarcoma.

  • Arise in soft tissue; this is where the name comes from.[1]
  • Rare variant of chondrosarcoma.

Microscopic: Features:

  • "White clouds in a blue sky".

Image:

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 [2].
  • This is where the name comes from.[1]

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) [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]

Photos

  • *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)

See also

Image:

References

  1. ↑ 1.0 1.1 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.
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