Three cases are reported in which an osteosarcoma developed in relation to an enchondroma in a long bone. Two of the cases were in the proximal femur whilst one occurred in the proximal humerus, both recognised sites for old calcified enchondromas or "cartilage rests". The ages of the patients at presentation were 55, 63 and 84 years and all were women. Two patients died with pulmonary metastas...

Dysspondyloenchondromatosis (DSC) is a rare skeletal dysplasia characterized by enchondroma-like lesions and anisospondyly. The former leads to discrepancies in limb length, and the latter, to progressive kyphoscoliosis. Two recent cases have highlighted the genetic heterogeneity of DSC, one demonstrating the presence and, the other, the absence of a COL2A1 mutation. This may have important cli...