DYSCHONDROPLASIA WITH GLIOMA OF THE BRAIN - 3RD HISTOLOGICALLY VERIFIED CASE

Dyschondroplasia is a mesodermal dysplasia with typical skeletal malformations of varying extent and severity. The combination with other malformations has led to numerous disease entities (Maffucci's syndrome, M. Kast, M. Ollier) that should be regarded as different forms of the same syndrome. The importance of the rare disease lies in the high risk of sarcomatous transformation of the skeletal lesions and furthermore in an increased risk of development of other tumours. Involvement of the central nervous system is possible through lesions of the bones of the skull or through primary intracranial tumours. The present report concerns a patient with dyschondroplasia complicated by the occurrence of a cerebral glioma and is the third reported case verified by biopsy. © 1979 J. F. Bergmann Verlag.