Chondrosarcoma of the skull in Maffucci's syndrome

Abstract

The syndrome described by Maffucci in 1881 is a rare, congenital, nonhereditary, mesodermal dysplasia characterized by enchondromas and cavernous or capillary hemangiomas. The bone and soft tissue lesions are not interdependent and occur in various combinations in different parts of the body or intermingled. The cartilaginous or vascular tumors may undergo malignant change, but only 2 cases of intracranial chondrosarcoma were reported. The present report concerns a patient in whom enchondroma of the sphenoid underwent sarcomatous change and is of particular interest as the investigation included computerized axial tomographic (C.T.) scanning.