Clinico-Genomic Profiling of Conventional and Dedifferentiated Chondrosarcomas Reveals TP53 Mutation to Be Associated with Worse Outcomes

Purpose: Chondrosarcomas are the most common primary bone more common in dedifferentiated chondrosarcomas compared tumor in adults. Isocitrate dehydrogenase 1 (IDH1) and IDH2 with grades 1–3 conventional chondrosarcoma. There was no mutations are prevalent. We aimed to assess the clinico-genomic difference in survival based on IDH mutation in univariate and properties of IDH mutant versus IDH wild-type (WT) chondromultivariate analyses. TP53 mutation was the next most prevsarcomas as well as alterations in other genes. alent (41.9%) and is associated with worse overall survival and Experimental Design: We included 93 patients with convenmetastasis-free survival in both univariate and multivariate tional and dedifferentiated chondrosarcoma for which there were analyses. TP53 mutation was also associated with higher risk available clinical next-generation sequencing data. Clinical and of recurrence following curative-intent surgery and worse surgenomic data were extracted and compared between IDH mutant vival among patients that presented with de novo metastatic and IDH WT chondrosarcomas and between TP53 mutant and disease. TP53 WT chondrosarcomas. Conclusions: IDH mutations are prevalent in chondrosarcoma Results: IDH1 and IDH2 mutations are prevalent in chonthough were not associated with survival outcomes in this cohort. drosarcoma (50.5%), more common in chondrosarcomas arising TP53 mutations were the next most common alteration and were in the extremities, associated with higher age at diagnosis, and associated with worse outcomes.