Sarcomatous transformation in diaphyseal aclasis

Multiple hereditary exostosis (or diaphyseal aclasis) is a condition characterized by the development of multiple osteochondromas. The tendency for malignant transformation into chondrosarcoma is well known. Malignancy typically arises from the cartilaginous cap of the osteochondroma. Radiographs supplemented by computed tomography have an important role in the diagnosis of this condition. Magnetic resonance imaging shows the features of sarcomatous change and aids in differentiating malignancy from pseudotumours.