Is the IDH Mutation a Good Target for Chondrosarcoma Treatment?
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Date
2020-03-01Author
Cojocaru, E
Wilding, C
Engelman, B
Huang, P
Jones, RL
Type
Journal Article
Metadata
Show full item recordAbstract
<jats:title>Abstract</jats:title><jats:p>Chondrosarcomas are rare cancers of bone that arise from the malignant transformation of cells of chondrocytic lineage. They are known to be resistant to systemic cytotoxic chemotherapy and radiotherapy. The mainstay of management of localised disease is <jats:italic>en bloc</jats:italic> surgical resection with curative intent. Metastatic chondrosarcoma has a dismal prognosis, and to date, there are no proven effective systemic therapies in the advanced setting. Genomic studies have demonstrated that 50 to 80% of chondrosarcomas harbour a mutation in either the <jats:italic>IDH1</jats:italic> or <jats:italic>IDH2</jats:italic> gene. IDH inhibitors are currently under investigation in clinical trials, after showing promising results in phase 1 studies in <jats:italic>IDH</jats:italic> mutated cancers. In chondrosarcoma, <jats:italic>IDH</jats:italic> mutations represent an attractive target, however, early results with IDH inhibitors in <jats:italic>IDH</jats:italic> mutated chondrosarcoma are modest and the final results of ongoing trials are eagerly awaited.</jats:p>
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Research team
Molecular and Systems Oncology
Language
eng
License start date
2020-03
Citation
Current Molecular Biology Reports, 2020, 6 (1), pp. 1 - 9
Publisher
Springer Science and Business Media LLC