Home > G. Tumoral pathology > chondrosarcomas
chondrosarcomas
Thursday 4 March 2004, by
Digital cases
JRC:6283 : Chondrosarcoma Metastatic to the lungs.
Images
- https://twitter.com/a_sleeping_dog/status/788647137005309952
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dedifferentiated peripheral chondrosarcoma
Classification
conventional chondrosarcoma
myxoid chondrosarcoma
clear cell chondrosarcoma
mesenchymal chondrosarcoma
dedifferentiated peripheral chondrosarcoma
- dedifferentiated peripheral chondrosarcoma (DPCHS )
- dedifferentiated central chondrosarcoma (DCCHS )
According to the localization
skeletal chondrosarcoma
juxtacortical chondrosarcoma
extra-skeletal chondrosarcoma
peripheral chondrosarcoma
Cytogenetics
17p1 alterations (11793371)
CGH
Chondrosarcomas develop through a combination of genomic imbalances that often affect the RB1 signaling pathway. (23146407)
Homozygous deletions of cadherin genes in chondrosarcoma-an array comparative genomic hybridization study. (23146407)
- The inactivation of cadherin genes may be critical in the pathogenesis of the tumor. (23146407)
Genomic imbalances, in most tumors affecting large regions of the genome, are found in 90% of the cases. (19336518)
Several apparently distinctive aberrations affecting conventional central and peripheral tumors, respectively, have been identified.
- Some regions are specifically altered in conventional central and peripheral tumors, respectively. (19336518)
- Although rare, recurrent amplifications are found at 8q24.21-q24.22 and 11q22.1-q22.3. (19336518)
- Homozygous deletions of loci previously implicated in chondrosarcoma development affected the CDKN2A, EXT1, and EXT2 genes. (19336518)
- The chromosomal imbalances in two distinct groups of predominantly near-haploid and near-triploid tumors, respectively, support the notion that polyploidization of an initially hyperhaploid/hypodiploid cell population is a common mechanism of chondrosarcoma progression. (19336518)
- Increasing patient age as well as tumor grade are associated with adverse outcome, but no copy number imbalance affected metastasis development or tumor-associated death. (19336518)
Kinome profiling
Using kinome profiling, it has been found that the Src pathway to be active in chondrosarcoma. (19602594)
The inhibitor of the Src pathway, dasatinib, may provide a potential therapeutic benefit for chondrosarcoma patients who are not eligible for surgery. (19602594)
Treatment
Chondrosarcomas are notorious for their resistance to conventional chemotherapy and radiotherapy, indicating there are no curative treatment possibilities for patients with inoperable or metastatic disease.
See also
cartilaginous tumors / chondroid tumors
Reviews
Chow WA. Update on chondrosarcomas. Curr Opin Oncol. 2007 Jul;19(4):371-6. PMID: 17545802
Bovée JV, Cleton-Jansen AM, Taminiau AH, Hogendoorn PC. Emerging pathways in the development of chondrosarcoma of bone and implications for targeted treatment. Lancet Oncol. 2005 Aug;6(8):599-607. PMID: 16054571
Sandberg AA. Genetics of chondrosarcoma and related tumors. Curr Opin Oncol. 2004 Jul;16(4):342-54. PMID: 15187889
Aigner T. Towards a new understanding and classification of chondrogenic neoplasias of the skeleton—biochemistry and cell biology of chondrosarcoma and its variants. Virchows Arch. 2002 Sep;441(3):219-30. PMID: 12242518
Open References
Genomic profiling of chondrosarcoma: chromosomal patterns in central and peripheral tumors. Hallor KH, Staaf J, Bovée JV, Hogendoorn PC, Cleton-Jansen AM, Knuutila S, Savola S, Niini T, Brosjö O, Bauer HC, Vult von Steyern F, Jonsson K, Skorpil M, Mandahl N, Mertens F. Clin Cancer Res. 2009 Apr 15;15(8):2685-94. Epub 2009 Mar 31. PMID: 19336518 (Free)
References
Homozygous deletions of cadherin genes in chondrosarcoma-an array comparative genomic hybridization study. Niini T, Scheinin I, Lahti L, Savola S, Mertens F, Hollmén J, Böhling T, Kivioja A, Nord KH, Knuutila S. Cancer Genet. 2012 Nov;205(11):588-93. PMID: 23146407
Kinome profiling of chondrosarcoma reveals SRC-pathway activity and dasatinib as option for treatment. Schrage YM, Briaire-de Bruijn IH, de Miranda NF, van Oosterwijk J, Taminiau AH, van Wezel T, Hogendoorn PC, Bovée JV. Cancer Res. 2009 Aug 1;69(15):6216-22. PMID: 19602594 [Free]
Overexpression of A disintegrin and metalloproteinase 28 is correlated with high histologic grade in conventional chondrosarcoma. Matsuura S, Oda Y, Matono H, Izumi T, Yamamoto H, Tamiya S, Iwamoto Y, Tsuneyoshi M. Hum Pathol. 2009 Nov 6. PMID: 19896699
