1. Is the IDH Mutation a Good Target for Chondrosarcoma Treatment?
- Author
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Bodil Engelman, Christopher P Wilding, Paul H. Huang, Elena Cojocaru, and Robin L. Jones
- Subjects
musculoskeletal diseases ,0301 basic medicine ,Metastatic Chondrosarcoma ,Mutation ,IDH1 ,business.industry ,medicine.medical_treatment ,General Engineering ,medicine.disease ,medicine.disease_cause ,Molecular medicine ,Malignant transformation ,Clinical trial ,Radiation therapy ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,030220 oncology & carcinogenesis ,medicine ,Cancer research ,Chondrosarcoma ,business - Abstract
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 en bloc 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 IDH1 or IDH2 gene. IDH inhibitors are currently under investigation in clinical trials, after showing promising results in phase 1 studies in IDH mutated cancers. In chondrosarcoma, IDH mutations represent an attractive target, however, early results with IDH inhibitors in IDH mutated chondrosarcoma are modest and the final results of ongoing trials are eagerly awaited.
- Published
- 2020