Age / Sex : 38 / F

Chief complaint:

Kyung-Sik Ahn, Korea University Anam Hospital

Mazabraud syndrome

Discussion

Findings:

• Intramuscular myxomas with skeletal fibrous dysplasia

Differential Diagnosis:

• Myxolipoma, myxoid neurofibroma, myxochondroma


• Richly myxoid malignant tumors (myxoid liposarcoma, low grade fibromyxoid sarcoma, extraskeletal myxoid chondrosarcoma, and botryoid type rhabdomyosarcoma, etc.)

Discussion:

Intramuscular myxoma

• Benign, soft tissue neoplasm with prominent myxoid stroma


• Age: 40-70 years


• Gender: ~ 57- 66% in female patients


• Predilection for large muscles (thigh 51%, buttocks 7% shoulder girdle/upper arm 9%)


• Well-circumscribed, ovoid mass


• Fluid-sensitive MR sequences often show flame-shaped extension of signal, especially along long muscle axis


• Multiple tumors more likely to be seen in Mazabraud syndrome

Fibrous dysplasia

• Benign fibroosseous lesion


• Diaphyseal; often extends to metaphysis and occasionally to epiphysis


• Usually central within bone, causing varying degrees of expansion


• Polyostotic in 15-20%


• Polyostotic form associated with McCune-Albright syndrome, Mazabraud syndrome

References:

Manaster BJ, Roberts CC, Petersilge CA. Diagnostic imaging. Musculoskeletal: Non-traumatic disease. Amirsys. 2010.