Maffucciʼs Syndrome

Maffucci's syndrome is a congenital, non-hereditary mesodermal dysplasia manifested by multiple enchondromas and hemangiomas. It is associated with diverse secondary musculoskeletal deformities. Sarcomatous transformation has been recorded in sixteen of 105 cases in the literature. A case report, illustrating the variety of deformities occurring in the syndrome, is presented. It is the first documentation of the occurrence of multiple primary sarcomas, and the second incidence of a mesenchymal ovarian tumor developing in a patient with Maffucci's syndrome. Surgery appears to be the treatment of choice for functional and neoplastic complications of the disease.