Is the IDH Mutation a Good Target for Chondrosarcoma Treatment?
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<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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Molecular and Systems Oncology
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Current Molecular Biology Reports, 2020, 6 (1), pp. 1 - 9
Springer Science and Business Media LLC