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
acid.
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.
Monosotic fibrous dysplasia and solitary intramuscular myxoma of the head and neck: A unique presentation of Mazabraud's syndrome and a literature review
