TY - JOUR
T1 - Mazabraud's syndrome
T2 - A new case and review of the literature
AU - Zoccali, Carmine
AU - Teori, Giuseppe
AU - Prencipe, Umberto
AU - Erba, Fabio
PY - 2009/6
Y1 - 2009/6
N2 - 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.
AB - 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.
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U2 - 10.1007/s00264-007-0483-x
DO - 10.1007/s00264-007-0483-x
M3 - Article
C2 - 18214477
AN - SCOPUS:67449100488
VL - 33
SP - 605
EP - 610
JO - International Orthopaedics
JF - International Orthopaedics
SN - 0341-2695
IS - 3
ER -