Ben-Itzhak I, Denolf F A, Versfeld G A, Noll B J
Department of Orthopaedic Surgery, University of the Witwatersrand, Johannesburg, South Africa.
J Pediatr Orthop. 1988 May-Jun;8(3):345-8. doi: 10.1097/01241398-198805000-00018.
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.
马富西综合征由皮下血管瘤和多发性内生软骨瘤组成。与该疾病相关的恶性肿瘤发生率为23%，其中15.2%是内生软骨瘤恶变为软骨肉瘤。建议对患者进行定期随访。当怀疑发生恶性转化时，骨扫描可能有助于诊断，尤其是连续进行时。94%的患者可以过上基本正常的生活，尽管其中三分之二的患者需要一些手术干预。
