Maffucci's syndrome with bilateral cartilaginous tumors of the cerebellopontine angle

Abstract

Maffucci's syndrome is characterized by the combination of multiple enchondromas (Ollier's disease) and hemangiomatosis. These hemangiomas develop in the subcutaneous tissue and form red-blue tumors dispersed over the whole body. Intracranial involvement is rare, making a preoperative radiological diagnosis and differentiation from other tumors rather difficult. The radiological characteristics and successful removal of the intracranial part of a chondrosarcoma of the cerebellopontine angle in a case of Maffucci's syndrome are reported in this paper.