Mazabraud's syndrome: a new case and review of the literature.
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.
Zoccali
Carmine
C
Oncological Orthopaedics Department, Muscular-Skeletal Tissue Bank, IFO-IRE, Roma, Italy. carminezoccali@libero.it
Teori
Giuseppe
G
Prencipe
Umberto
U
Erba
Fabio
F
D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D005260:Female; D005358:Fibrous Dysplasia, Monostotic / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019042:Muscle Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D009232:Myxoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D049268:Positron-Emission Tomography; D009418:S100 Proteins / Q000378:metabolism; D013577:Syndrome; D013848:Thigh
D014408:Biomarkers, Tumor; D009418:S100 Proteins