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Fibrous Dysplasia : Mazabraud Syndrome

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Mazabraud syndrome consists of fibrous dysplasia (usually polyostotic form) with multiple intramuscular myxomas affecting the same anatomical region.

Case History: 40 y/o male with hip pain. No history of trauma. The proximal right femur shows a diffusely abnormal irregular trabecular pattern with areas of increased and decreased osseous density and ground-glass opacity. This lesion causes moderate cortical thinning with endosteal irregularity. There is no associated periosteal reaction or definitive soft-tissue mass. The proximal epiphysis of the right femur is spared. Similar lesions are present within the right ilium in the supra-acetabular region and along the iliac crest. The right femoral neck demonstrates a focally expansile lytic lesion with severe cortical thinning consistent with aneurysmal bone cyst. The cortex along the inferior margin of the right femur is not definitively intact, and may represent an area of cortical disruption indicating a pathologic fracture. MRI scan showed 3 distinct intramuscular myxomas in the vastus medialis muscle near the affected areas of femur (See next three images).

Case courtesy of Dr Matt Skalski, Radiopaedia.org. From the case rID: 37966

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