Maffucci's Syndrome Complicated by an Intracranial Chondroma

ABSTRACT

Maffuci's syndrome is characterized by defects in cartilage bone formation (dyschondroplasia) with subcutaneous multiple vascular hemangiomas, and often with enchondromas. More than 120 cases of this syndrome, which was first described by Maffucci in 1881, has been reported. But intracranial tumors are rare complication of this syndrome. In this paper we describe the 6th case in the world report of Maffucci's syndrome complicated by an intracranial tumor, which was confirmed histologically as a chondroma, and review the literature with regard to this rare complication of Maffucci's syndrome.