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Arch Orthop Trauma Surg · Jan 1991
Case ReportsMaffucci's syndrome combined with dedifferentiated chondrosarcoma.
- H Tsuchiya, K Tomita, H Yasutake, Y Takagi, Y Ueda, and M Kadoya.
- Department of Orthopedic Surgery, School of Medicine, Kanazawa University, Japan.
- Arch Orthop Trauma Surg. 1991 Jan 1; 110 (5): 269-72.
AbstractWe report a rare case of Maffucci's syndrome combined with dedifferentiated chondrosarcoma in the right shoulder girdle developing from pre-existing enchondroma. In this case, magnetic resonance imaging was useful in diagnosing dedifferentiated chondrosarcoma before surgery. T2-weighted imaging was used to distinguish between the cartilaginous component and the dedifferentiated one. Histologically, there was enchondroma in the humerus and grade 2 chondrosarcoma in the scapula. Further, the dedifferentiated tumor had three mesenchymal elements: osteosarcoma, malignant fibrous histiocytoma, and fibrosarcoma. This histological heterogenicity may be due to mesodermal dysplasia of Maffucci's syndrome.
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