Chondrosarcoma-from Molecular Pathology to Novel Therapies

SIMPLE SUMMARY: Chondrosarcoma (CHS) belongs to a broad group of sarcomas and is the second most frequent malignant bone tumor. Due to its resistance to chemo- and radiotherapy, treatment of this tumor is complicated and is mainly limited to surgery. In this review, we described the characteristics of CHS comprehensively from its molecular basics, through diagnosis, and finally to treatment methods with emphasizing the novel potential therapies and currently ongoing clinical trials. We discussed the potential of targeted therapies, including blockers of crucial in pathogenesis receptors, mutated genes inhibitors, and capabilities of immunotherapy. Ultimately, in this review we summarized the present possibilities in CHS treatment and its outcomes with novel trends which can become a point of interest in future researches on CHS. ABSTRACT: Chondrosarcoma (CHS) is the second most common primary malignant bone sarcoma. Overall survival and prognosis of this tumor are various and often extreme, depending on histological grade and tumor subtype. CHS treatment is difficult, and surgery remains still the gold standard due to the resistance of this tumor to other therapeutic options. Considering the role of differentiation of CHS subtypes and the need to develop new treatment strategies, in this review, we introduced a multidisciplinary characterization of CHS from its pathology to therapies. We described the morphology of each subtype with the role of immunohistochemical markers in diagnostics of CHS. We also summarized the most frequently mutated genes and genome regions with altered pathways involved in the pathology of this tumor. Subsequently, we discussed imaging methods and the role of currently used therapies, including surgery and the limitations of chemo and radiotherapy. Finally, in this review, we presented novel targeted therapies, including those at ongoing clinical trials, which can be a potential future target in designing new therapeutics for patients with CHS.