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MAFFUCCI SYNDROME

“ Numerous enchondromas with haemangiomas and lymphangiomas . It also tends to be unilateral. Complications are similar to those of Ollier d...

Numerous enchondromas with haemangiomas and lymphangiomas. It also tends to be unilateral. Complications are similar to those of Ollier disease. There is an increased risk of malignant transformation(20%) (mostly chondrosarcoma) compared to Ollier disease.
Plain film 
•  Originate in the metaphysis.
•  Well-defined lucent lesion with ground-glass matrix (occasionally chondroid matrix).
•  Thinning, expansion and occasionally disappearance of the cortex.
•  Usually smaller than 3 cm.
•  Pathological fracture.
•  Outside the hands and feet, chondrosarcoma is five-time more common than enchondroma.
•  Frank cortical destruction, a lesion >5 cm or a soft tissue mass suggests malignancy.
Enchondroma. Frontal and lateral radiographs of the left middle finger demonstrating a lytic expansile lesion within the proximal phalanx with a narrow zone of transition and a chondroid matrix.