Maffucci's syndrome combined with dedifferentiated chondrosarcoma.
We 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.
Department of Orthopedic Surgery, School of Medicine, Kanazawa University, Japan.
H
H
Tsuchiya
K
K
Tomita
H
H
Yasutake
Y
Y
Takagi
Y
Y
Ueda
M
M
Kadoya
D000368:Aged; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D002813:Chondrosarcoma / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D004687:Enchondromatosis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D006811:Humerus; D008279:Magnetic Resonance Imaging; D012540:Scapula; D014057:Tomography, X-Ray Computed