Difference between revisions of "Mesenchymal chondrosarcoma"
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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== | ||
| Line 17: | Line 15: | ||
==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=== | ||
| Line 49: | Line 61: | ||
*Osteocalcin (negative in small cells). | *Osteocalcin (negative in small cells). | ||
*CD99 - (positive in small cells) | *CD99 - (positive in small cells) | ||
==Molecular== | ==Molecular== | ||
t(8;8)(q21.1;q13.3) HEY1-NCOA2.<ref name=pmid24839999>{{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> | *t(8;8)(q21.1;q13.3) HEY1-NCOA2.<ref name=pmid24839999>{{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> | ||
*Less commonly t(1;5)(q42;q32) IRF2BP2-CDX1<ref>{{cite journal |authors=Nyquist KB, Panagopoulos I, Thorsen J, Haugom L, Gorunova L, Bjerkehagen B, Fosså A, Guriby M, Nome T, Lothe RA, Skotheim RI, Heim S, Micci F |title=Whole-transcriptome sequencing identifies novel IRF2BP2-CDX1 fusion gene brought about by translocation t(1;5)(q42;q32) in mesenchymal chondrosarcoma |journal=PLoS ONE |volume=7 |issue=11 |pages=e49705 |date=2012 |pmid=23185413 |pmc=3504151 |doi=10.1371/journal.pone.0049705 |url=}}</ref> | |||
==See also== | ==See also== | ||
*[[Cartilage]]. | *[[Cartilage]]. | ||
*[[Chondrosarcoma]]. | *[[Chondrosarcoma]]. | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
==External links== | |||
*[http://www.webpathology.com/image.asp?case=332&n=41 Mesenchymal chondrosarcoma - images (webpathology.com)]. | |||
*[http://www.pathologyoutlines.com/topic/bonemesenchymalchondrosarcoma.html Mesenchymal chondrosarcoma (pathologyoutlines.com)]. | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Chondro-osseous tumours]] | [[Category:Chondro-osseous tumours]] | ||
Latest revision as of 20:30, 24 May 2020
Mesenchymal chondrosarcoma is a rare type of chondrosarcoma that is 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 meninges).
- 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
Features:
- Malignant tumour 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.
- Can have a 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
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:
- Mesenchymal chondrosarcoma (ouhsc.edu).
- Pathology Outlines - Hemangiopericytomatous example [1]
- Sarcoma Images - [2]
- CNS Atlas - [3]
- CNS Atlas - [4]
- CNS Atlas - [5]
![[1]](http://pathologyoutlines.com/wick/chondrosarcoma%20mesenchymal%20type%20micro0007.jpg){kind=link}
![[3]](http://www.cnsatlas.com/medicalatlas/images/370/800_poIB6FYZQ73wh.jpg){kind=link}
![[4]](http://www.cnsatlas.com/medicalatlas/images/370/800_9oJtTQXXxaEB3.jpg){kind=link}
![[5]](http://www.cnsatlas.com/medicalatlas/images/370/800_8h2C7upeOj3EB.jpg){kind=link}
IHC
- SOX9 (positive in small cells and chondrocytes).[3]
- S100 (positive in chondrocytes not in small cells).
- Osteocalcin (negative in small cells).
- CD99 - (positive in small cells)
Molecular
See also
References
- ↑ 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.
- ↑ Nyquist KB, Panagopoulos I, Thorsen J, Haugom L, Gorunova L, Bjerkehagen B, Fosså A, Guriby M, Nome T, Lothe RA, Skotheim RI, Heim S, Micci F (2012). "Whole-transcriptome sequencing identifies novel IRF2BP2-CDX1 fusion gene brought about by translocation t(1;5)(q42;q32) in mesenchymal chondrosarcoma". PLoS ONE 7 (11): e49705. doi:10.1371/journal.pone.0049705. PMC 3504151. PMID 23185413. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3504151/.