The Maffucci Syndrome
- 1 May 1988
- journal article
- case report
- Published by Wolters Kluwer Health in Journal of Pediatric Orthopaedics
- Vol. 8 (3) , 345-348
- https://doi.org/10.1097/01241398-198805000-00018
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.Keywords
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