Maffucci's syndrome: two case reports with a literature review

Abstract

Sixty-three cases of Maffucci's syndrome in the English literature plus two additional cases of our own are reviewed. This syndrome is nonhereditary and is characterized by multiple enchondromas and hemangiomas. It occurs in all races with no sex predominance. The enchondromas and hemangiomas can occur anywhere but are most common in the hands. Long bone involvement is common and leads to progressive skeletal deformity and pathologic fractures. The incidence of malignancies in patients with this syndrome is high. Chondrosarcomas are especially common and occur in 30% of the patients.