1. Mazabraud's syndrome: a new case and review of the literature
- Author
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Umberto Prencipe, Erba F, Carmine Zoccali, and Giuseppe Teori
- Subjects
Adult ,Male ,medicine.medical_specialty ,Review ,Thigh ,medicine ,Biomarkers, Tumor ,Humans ,Orthopedics and Sports Medicine ,Femur ,Right Thigh ,Pelvis ,Muscle Neoplasms ,S syndrome ,business.industry ,Fibrous dysplasia ,S100 Proteins ,Myxoma ,Anatomy ,Syndrome ,medicine.disease ,Magnetic Resonance Imaging ,medicine.anatomical_structure ,Positron-Emission Tomography ,Orthopedic surgery ,cardiovascular system ,Surgery ,Female ,business ,Fibrous Dysplasia, Monostotic - Abstract
The association between muscular myxomas and fibrous dysplasia is a rare condition known as Mazabraud’s syndrome, as reported by Henschen (Verh Dtsch Ges Pathol 21:93–97, 1926) and Mazabraud A and Girard (Rev Rhum Mal Osteoartic 24(9–10):652–659, 1957). We report a case of a 32-year-old woman with multiple myxomas in her right thigh and monomelic fibrous dysplasia. A review of the international literature referring to 67 cases to date was carried out. The syndrome is characterised by the following features: females are twice as likely to be affected as males; the lower limbs are the most frequently affected, fibrous dysplasia is more common in the femur and the pelvis and myxomas in the quadriceps muscle; myxoma is multiple in more than 70% of cases. Although there has never been any continuity between tumours and bone lesions, a significant correlation between dysplastic bone and myxoma has been revealed.
- Published
- 2007