Biology and Management of High-Grade Chondrosarcoma: An Update on Targets and Treatment Options.

ABSTRACT: This review provides an overview of histopathology, clinical presentation, molecular pathways, and potential new systemic treatments of high-grade chondrosarcomas (CS), including grade 2-3 conventional, dedifferentiated, and mesenchymal CS. The diagnosis of CS combines radiological and histological data in conjunction with patient clinical presentations. Conventional CS is the most frequent subtype of CS (85%) and represents about 25% of primary bone tumors in adults; they can be categorized according to their bone location into central, peripheral, and periosteal chondrosarcomas. Central and peripheral CS differ at the molecular level with either IDH1/2 mutations or EXT1/2 mutations, respectively. CDKN2A/B deletions are also frequent in conventional CS, as well as

SUBMITTER: Tlemsani C 

PROVIDER: S-EPMC9862566 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature