Molecular drivers in chondrosarcoma

Chondrosarcomas are a heterogeneous group of malignant cartilage-forming tumors, which are resistant to conventional chemo-and radiotherapy. Consequently, patients presenting with inoperable disease cannot be offered any alternative treatment. Therefore it is important to identify the molecular pathways that are deregulated in chondrosarcoma. Different subgroups of chondrosarcomas exist, with conventional chondrosarcoma accounting for 85% of cases, which is also the most frequently observed subtype in the skull and spine. Chondrosarcomas develop following a multistep progression model in which they step-wise acquire different mutations and pathway alterations leading to a more malignant histological phenotype. Therapeutic interference with one of these alterations is the main strategy to develop an alternative treatment for chondrosarcoma patients.