| Literature DB >> 22735375 |
Abstract
Mafucci's syndrome is a rare genetic but non-hereditary disorder, characterized by multiple enchondromas (enchondromatosis), hemangiomas and, rarely, lymphangiomas. The risk for malignant transformation of enchondromas is very high, and chondrosarcomas can develop which mainly metastatize to the lungs. A case report of a 61-years-old male, whose enchondromatosis developed at his age of 10 years, is described. The initial diagnosis had been Ollier's disease at that time, and it was modified to Mafucci's syndrome only in 1995 when hemangiomatosis developed on the right hand. He had a unilateral disorder affecting his right upper and lower extremities. In 2010, a chondrosarcoma developed on his right leg and amputation was performed. In 2012, a thoracic CT scan revealed pulmonary metastases on both sides. This case report underlines the importance of the multidisciplinary approach and cooperation between various specialties in diagnosing and early detecting this type of cancer. Orv. Hetil., 2012, 153, 1035-1038.Entities:
Mesh:
Year: 2012 PMID: 22735375 DOI: 10.1556/OH.2012.29386
Source DB: PubMed Journal: Orv Hetil ISSN: 0030-6002 Impact factor: 0.540