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The Maffucci syndrome
I Ben-Itzhak1, F A Denolf, G A Versfeld
1Department of Orthopaedic Surgery, University of the Witwatersrand, Johannesburg, South Africa.
Journal of Pediatric Orthopedics
|May 1, 1988
Abstract:
Maffucci syndrome consists of subcutaneous hemangiomata and multiple enchondromata. The incidence of malignancy associated with the disease is 23%, of which 15.2% is a malignant transformation of enchondromata to chondrosarcoma. Periodic follow-up of the patient is advised. When malignant transformation is suspected, a bone scan may be useful for diagnosis, especially when done sequentially. Ninety-four percent of the patients can live a reasonably normal life, although two-thirds of them require some surgical intervention.