Monday, September 24, 2012

Maffucci Syndrome

Maffucci Syndrome
  • nonhereditary 
  • enchondromatosis combined with hemangiomas
  • enchondromas in Maffucci syndrome carry a relatively high risk of malignant transformation 
    • chondrosarcoma is the most common musculoskeletal malignancy 
  • high risk of developing non musculoskeletal malignancies
    • ovarian, pancreatic, gastrointestinal adenocarcinoma, glioma
Imaging Findings
  • expansile metaphyseal lesions 
  • sclerotic margins
  • chondroid mineralization with "ring and arc" pattern that is typical of cartilage containing lesions
  • increased signal intensity on T2W MR due to high water content of hyaline cartilage

Flach HZ, Ginai AZ, Oosterhuis JW. Maffucci syndrome: radiologic and pathologic findings. Radiographics 2001;21:1311-16.

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