Advances in the Molecular Biology of Chondrosarcoma

Chondrosarcoma (CS) is a highly treatment-resistant tumor of cartilage-producing cells and the second most common primary bone malignancy following osteosarcoma. As CS is refractory to available chemotherapy and radiotherapy, aggressive surgical resection with wide margins remains the only effective treatment option. Cancer immunotherapies and targeted therapies have failed in clinical trials, and no prognostic biomarkers exist within the clinic. A deeper understanding of the molecular biology and dysregulated pathways in CS is necessary for more effective precision therapies. In response, several large-scale genetic and epigenetic studies have recently emerged, with several notable pathways holding therapeutic promise. In this review, we summarize the emerging works on CS biology, focusing on both genomic and epigenomic mechanisms and targets. These studies highlight several valuable clinical opportunities for earlier detection, prognostic accuracy, and therapeutic targeting that may potentially improve patient outcomes.