INTRAMUSCULAR MYXOMA AND FIBROUS DYSPLASIA OF BONE - MAZABRAUDS SYNDROME - A CASE-REPORT

We present a case of Mazabraud's syndrome, a rare benign disease, with multiple intramuscular myxomas of the thoracic wall associated with f ibrous dysplasia of bone. CT, MR imaging and ultrasonography (US) of t he thorax showed 2 well circumscribed homogeneous intramuscular tumors . A US-guided needle biopsy with a large-core needle (2.0 mm) and a fi ne needle (0.8 mm) showed that the tumors were intramuscular myxomas w ith no sign of malignancy. Tc-99m bone scintigraphy showed a markedly increased uptake in the right lower skull, and multiple smaller foci. CT of the skull revealed a right-sided unilateral bone thickening of t he orbit and the ethmoidal cells, and right-sided exophthalmia. This c ase history suggests that patients with multiple intramuscular myxomas should be preoperatively examined for osseous lesions. A postoperativ e follow-up should also be performed to detect other soft-tissue myxom as not as yet clinically detectable, or rare osseous complications.