Mesenchymal chondrosarcoma
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- updated 26 Nov 2022:
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Mesenchymal chondrosarcomas are rare highly malignant chondrosarcomas with a biphasic morphology of a primitive mesenchymal tumour and a well-differentiated cartilaginous component.
Epidemiology
Mesenchymal chondrosarcomas are rare chondrosarcoma subtype
Diagnosis
The diagnosis of mesenchymal chondrosarcomas is established by histology and molecular pathology 1.
Diagnostic criteria
Diagnostic criteria according to the WHO classification of soft tissue and bone tumours (5th edition)1:
undifferentiated tumour cells with a high nuclear-cytoplasm ratio
islands of cartilage
The following criteria are desirable 1:
presence of a staghorn vascular pattern
HEY-NCOA2 fusion
Pathology
They are found in both bone and soft tissues (see: extraskeletal chondrosarcoma).
HistologyMicroscopic appearance
Mesenchymal chondrosarcomas are characterised by bimorphicbiphasic histology:
-
densely packed small round blue cells similar to those seen in Ewing's sarcoma
cells are seen in a perivascular arrangement which is reminiscent of haemangiopericytomas
malignant cartilaginous tumour
-<p><strong>Mesenchymal chondrosarcomas</strong> are a rare <a href="/articles/chondrosarcoma">chondrosarcoma</a> subtype.</p><h4>Pathology</h4><p>They are found in both bone and soft tissues (see: <a href="/articles/extraskeletal-chondrosarcoma">extraskeletal chondrosarcoma</a>). </p><h5>Histology</h5><p>Mesenchymal chondrosarcomas are characterised by bimorphic histology:</p><ul>-<li>densely packed small round blue cells similar to those seen in <a href="/articles/ewing-s-sarcoma">Ewing's sarcoma</a><ul><li>cells are seen in a perivascular arrangement which is reminiscent of <a href="/articles/musculoskeletal-haemangiopericytoma">haemangiopericytomas</a>-</li></ul>- +<p><strong>Mesenchymal chondrosarcomas</strong> are rare highly malignant <a href="/articles/chondrosarcoma" title="Chondrosarcoma">chondrosarcomas </a>with a biphasic morphology of a primitive mesenchymal tumour and a well-differentiated cartilaginous component.</p><h4>Epidemiology</h4><p>Mesenchymal chondrosarcomas are rare tumours and make up for ~2-4% of all chondrosarcomas <sup>1</sup>. They occur in a wide age range and peak in the second and third decades of life <sup>1-3</sup>. They are slightly more common in men <sup>1,3</sup>.</p><h4>Diagnosis</h4><p>The diagnosis of mesenchymal chondrosarcomas is established by histology and molecular pathology <sup>1</sup>.</p><h5>Diagnostic criteria</h5><p>Diagnostic criteria according to the <a href="/articles/who-classification-of-tumors-of-bone" title="WHO classification of bone tumours">WHO classification of soft tissue and bone tumours (5th edition)</a> <sup>1</sup>:</p><ul>
- +<li><p>undifferentiated tumour cells with a high nuclear-cytoplasm ratio</p></li>
- +<li><p>islands of cartilage</p></li>
- +</ul><p>The following criteria are desirable <sup>1</sup>: </p><ul>
- +<li><p>presence of a staghorn vascular pattern</p></li>
- +<li><p>HEY-NCOA2 fusion</p></li>
- +</ul><h4>Pathology</h4><p>They are found in both bone and soft tissues (see: <a href="/articles/extraskeletal-chondrosarcoma">extraskeletal chondrosarcoma</a>). </p><h5>Microscopic appearance</h5><p>Mesenchymal chondrosarcomas are characterised by biphasic histology:</p><ul>
- +<li>
- +<p>densely packed small round blue cells similar to those seen in <a href="/articles/ewing-s-sarcoma">Ewing's sarcoma</a></p>
- +<ul><li><p>cells are seen in a perivascular arrangement which is reminiscent of <a href="/articles/musculoskeletal-haemangiopericytoma">haemangiopericytomas</a></p></li></ul>
-<li>malignant cartilaginous tumour</li>- +<li><p>malignant cartilaginous tumour</p></li>
References changed:
- 7. Murphey M, Walker E, Wilson A, Kransdorf M, Temple H, Gannon F. From the Archives of the AFIP: Imaging of Primary Chondrosarcoma: Radiologic-Pathologic Correlation. Radiographics. 2003;23(5):1245-78. <a href="https://doi.org/10.1148/rg.235035134">doi:10.1148/rg.235035134</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/12975513">Pubmed</a>
- 8. Varma D, Ayala A, Carrasco C, Guo S, Kumar R, Edeiken J. Chondrosarcoma: MR Imaging with Pathologic Correlation. Radiographics. 1992;12(4):687-704. <a href="https://doi.org/10.1148/radiographics.12.4.1636034">doi:10.1148/radiographics.12.4.1636034</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/1636034">Pubmed</a>
- 1. Fanburg-Smith JC, de Pinieux G, Ladanyi M. Mesenchymal chondrosaroma. In: WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). <a href="https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Soft-Tissue-And-Bone-Tumours-2020">https://publications.iarc.fr</a>
- 2. Gelderblom H, Hogendoorn P, Dijkstra S et al. The Clinical Approach Towards Chondrosarcoma. Oncologist. 2008;13(3):320-9. <a href="https://doi.org/10.1634/theoncologist.2007-0237">doi:10.1634/theoncologist.2007-0237</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18378543">Pubmed</a>
- 3. Strach M, Grimison P, Hong A et al. Mesenchymal Chondrosarcoma: An Australian Multi‐centre Cohort Study. Cancer Medicine. 2022;12(1):368-78. <a href="https://doi.org/10.1002/cam4.4849">doi:10.1002/cam4.4849</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/35603739">Pubmed</a>
- 1. Murphey MD, Walker EA, Wilson AJ et-al. From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 23 (5): 1245-78. <a href="http://dx.doi.org/10.1148/rg.235035134">doi:10.1148/rg.235035134</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/12975513">Pubmed citation</a><div class="ref_v2"></div>
- 2. Varma DG, Ayala AG, Carrasco CH et-al. Chondrosarcoma: MR imaging with pathologic correlation. Radiographics. 1992;12 (4): 687-704. <a href="http://radiographics.rsna.org/content/12/4/687.abstract">Radiographics (abstract)</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/1636034">Pubmed citation</a><div class="ref_v2"></div>
Systems changed:
- Oncology
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