MAFFUCCIS-SYNDROME AND INTRACRANIAL CHONDROSARCOMA

Maffucci's syndrome is a rare, congenital mesenchymal dysplasia charac terized by multiple enchondromata and hemangimata, both of which may u ndergo malignant degeneration. Intracranial involvement is uncommon. A literature review yielded only six cases of Maffucci's syndrome with intracranial chondrosarcoma and two cases of Oilier's disease (enchond romata alone) with intracranial chondrosarcoma. We report the seventh case of Maffucci's syndrome in a patient with a very large and extensi ve skull base chondrosarcoma requiring staged operations for removal.