The molecular pathogenesis of dedifferentiated chondrosarcoma

Dedifferentiated chondrosarcomas are cartilaginous tumors that consist of two distinguishable components, a lowgrade chondrosarcoma (chondrogenic) component and a highgrade dedifferentiated (anaplastic) component. The tumor cells in both components seem to originate from a single precursor, but there are a substantial number of genetic alterations in the anaplastic component. The underlying mechanism of dedifferentiation is unknown, but cell cycle regulators p16, p53 and retinoblastoma appear to have important roles in tumor development and dedifferentiation. In this article, molecular pathogenesis of dedifferentiated chondrosarcomas is reviewed.