| Literature DB >> 2835395 |
I Ben-Itzhak1, F A Denolf, G A Versfeld, B J Noll.
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.Entities:
Mesh:
Year: 1988 PMID: 2835395 DOI: 10.1097/01241398-198805000-00018
Source DB: PubMed Journal: J Pediatr Orthop ISSN: 0271-6798 Impact factor: 2.324