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Mazabraud s syndrome: a new case and review of the literature Zoccali C; Teori G; Prencipe U; Erba FInt Orthop 2009[Jun]; 33 (3): 605-10The 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.|Adult[MESH]|Biomarkers, Tumor/metabolism[MESH]|Female[MESH]|Fibrous Dysplasia, Monostotic/*complications/diagnosis/surgery[MESH]|Humans[MESH]|Magnetic Resonance Imaging[MESH]|Male[MESH]|Muscle Neoplasms/*complications/diagnosis/surgery[MESH]|Myxoma/*complications/diagnosis/surgery[MESH]|Positron-Emission Tomography[MESH]|S100 Proteins/metabolism[MESH]|Syndrome[MESH]|Thigh[MESH] |
