M. Courtpayen et al., INTRAMUSCULAR MYXOMA AND FIBROUS DYSPLASIA OF BONE - MAZABRAUDS SYNDROME - A CASE-REPORT, Acta radiologica, 38(3), 1997, pp. 368-371
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.