The patient is a 59-year-old woman who had experienced a fracture of her left proximal femur at age 32. Radiographs revealed a fracture of the femoral neck through an abnormal expansile, ground-glass–like area.
Djamil Fertikh, MD
, Mercy Catholic Medical Center, Medical Imaging Department,
The patient is a 59-year-old woman who had experienced a
fracture of her left proximal femur at age 32. Radiographs revealed
a fracture of the femoral neck through an abnormal expansile,
ground-glasslike area. A whole-body technetium-99m methylene
disphosphonate (MDP) bone scan was performed, showing in addition
to the left femur, several areas of abnormal radiotracer uptake,
including the left mandible, both humeri, and the left tibia
(Figure 1). Magnetic resonance images (MRI) of the right hip were
obtained subsequently (Figure 2). Her medical history was
significant for precocious puberty, hypothyroidism, and the
presence of skin café-au-lait spots.
Fibrous dysplasia and intramuscular myxomas: Mazabraud's
Radiographs show ground-glass areas involving the left femur,
tibia, and fibula (Figure 3). A whole-body technetium-99m MDP bone
scan revealed abnormal areas of radiotracer uptake involving the
right proximal femur, the right mandible, both humeri, and the left
MRI of the patient's left hip showed multiple, well demarcated,
intramuscular soft-tissue masses of different sizes, which were
characterized by a low signal intensity (SI) on T1-weighted images
and a high SI on T2-weighted images (Figure 2).
Mazabraud's syndrome is a rare entity represented by the
association of fibrous dysplasia and intramuscular myxomas. Their
association was first reported in 1957 by A. Mazabraud, a French
Mazabraud's syndrome does not appear to be hereditary and occurs
mostly in women. The myxomas tend to develop and congregate around
the thighs, buttocks, and shoulder regions. Their neoplastic nature
has been questioned over time. Considering their tendency to group
close to the affected segments of the bone involved by fibrous
dysplasia, they are believed to be a reactive phenomenon due to
local mechanical stress factors. Supporting this hypothesis is the
fact that, histologically, they appear to be very close to synovial
mucoid cysts occurring in stressed articulations.
Others hypothesize that they may be extraosseous manifestations of
When compared with isolated myxomas, their tendency to reoccur
after removal is very low; however, note the postsurgical
recurrence in this patient. As in our patient, the polyostotic form
of fibrous dysplasia is predominant. Malignant transformation (eg,
osteosarcoma, fibrosarcoma) in Mazabraud's syndrome has been
reported, but the incidence remains very low.
For the myxomas, early surgery is usually advocated,
since involvement of neurovascular structures in advanced cases
renders excisions more laborious.
MRI has been used to evaluate the syndrome.
We found it a more useful imaging tool than the CT scan for
presurgical evaluation of the anatomy and the extent of the lesion.
Awareness of the syndrome and its presentation is essential in
distinguishing myxomas from other malignant mesenchyal tumors with
myxomal components such as liposarcomas, botyroid-type
rhabdomyosarcomas, malignant fibrous histocytomas, extraskeletal
chondrosarcomas, and neurofibromatosis, and consequently precluding
the need for additional expensive and often stressful