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| TEACHING IMAGES |
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| Year : 2014 | Volume
: 2
| Issue : 2 | Page : 263 |
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Maffucci syndrome revisited
Sandhya K Hemraj, Devdas K Acharya, G Ravichandra
Department of Radiology and Imaging, Yenepoya Medical College, Nithyananda Nagara, Deralakatte, Mangalore, Karnataka, India
| Date of Web Publication | 11-Nov-2014 |
Correspondence Address:
Sandhya K Hemraj
Department of Radiology and Imaging, Yenepoya Medical College, Mangalore 575 018, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2321-4848.144369
How to cite this article:
Hemraj SK, Acharya DK, Ravichandra G. Maffucci syndrome revisited
. Arch Med Health Sci 2014;2:263 |
| Case Report | |  |
A 49 year old lady presented with pain, restricted movements of right shoulder, and soft tissue swellings in right wrist. Radiographs revealed multiple osseous enchondromas and soft tissue phleboliths in the hand suggesting hemangiomas. Skeletal survey revealed similar lesions in other bones. A diagnosis of Maffucci's syndrome was made.
This is a rare, congenital disorder manifested by enchondromatosis and multiple soft tissue hemangiomas. [1] The pathognomonic radiological findings include multiple radiolucent well demarcated osseous lesions causing expansile remodeling; some showing matrix mineralization suggesting chondroid origin [Figure 1]. [2] There are multiple soft tissue hemangiomas [Figure 2]. Bone scintigraphy is useful to detect chondrosarcomatous changes. [3]  | Figure 1: Radiograph of right shoulder showing a large, osteolytic, expansile, multiseptated lesion involving the proximal metadiaphysis of right Humerus; with focal areas of cortical breech suggesting extraosseous extension and increased surrounding soft tissue density. There is another lesion in the scapula showing arc and rings calcification suggesting chondroid origin
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 | Figure 2: Radiograph of right forearm AP and Lateral views show a multiloculated, mildly expansile, osteolytic lesion involving shaft of right ulna causing cortical thinning and bony remodeling. There are multiple, rounded calcific densities in the soft tissues of right wrist in lateral view, suggesting phleboliths associated with hemangiomas
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| References | | |
| 1. | Subhash Desai, Kubeyinje, Belagavi, Desai S. Maffucci's Syndrome. Ann. Saudi Med 1997;17: 451-3. 
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| 2. | Zwenneke Flach H, Ginai A Z, Wolter Oosterhuis J. Maffucci Syndrome: Radiologic and pathologic findings. Radiographics 2001;21:1311-6.
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| 3. | Murphey MD, Walker, Wilson AJ, Kransdorf MJ, Gannon FH. Imaging of primary chondrosarcoma: Radiologic-pathologic correlation. Radiographics, 2003; 23:1245-78.
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[Figure 1], [Figure 2]
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