Mesenchymal chondrosarcoma
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Mesenchymal chondrosarcoma is a rare type of chondrosarcoma found in the soft tissue.
General
- Arise in soft tissue; this is where the name comes from.[1]
- 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
Images
Islands of cartilage in a background of small blue cells.(SKB)
Islands of cartilage in a background of small blue cells.(SKB)
Islands of cartilage in a background of small blue cells.(SKB)
Islands of cartilage in a background of small blue cells.(SKB)
Islands of cartilage in a background of small blue cells.(SKB)
Islands of cartilage in a background of small blue cells.(SKB)
Small blue cells predominate.(SKB)
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
- ↑ 1.0 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.