Objective: Mazabraud's syndrome is a rare form of bone fibrous dysplasia associated with
intramuscular myxomas. Fibrous dysplasia, is generally localized to pelvis and femur and it results in a
fragile bone with deformities, pain, pathological fractures and functional impairment. Intramuscular
myxomas, are rare benign mesenchymal neoplasms that exceptionally may evolve to malignant forms.
Methods: This case report describes a 66-year-old woman with Mazabraud’s Syndrome (MS), characterized
both by monostotic right femur fibrous dysplasia and by a solitary intramuscular myxoma at the
right quadriceps muscle, that underwent a long-term treatment (4 years) with intravenous zoledronic
Results: Zoledronic acid therapy rapidly lowered bone pain together with a reduction of intramuscular
myxoma volume, but did not affect the extension of fibrous dysplasia. No adverse effects have been
observed during treatment.
Conclusion: Highly active bisphosphonates are commonly used for the treatment of bone metabolic
disorders and they are generally well tolerated. Zoledronic acid may represent a promising alternative
to surgical intervention in MS, although its use in rare form of bone fibrous dysplasias is still controversial.