Abstract
Introduction: Mazabraud syndrome is a rare disorder characterised by fibrous dysplasia and intramuscular myxomas. We present six new cases.
Method: A reterospective review was performed on six patients from our institution. This is a consecutive series over 48 months.
Results: The patient group consisted of three females and three males. The six patients were referred to our bone tumour unit from other hospitals (four by orthopaedic teams and two by general surgeons). The referring hospitals had been unable to exclude a malignant process. Imaging was tailored to each individual case. Three of our six patients demonstrated polyostotic fibrous dysplasia. The commonest site of the osseous leision was the proximal femur (n=4). The majority of the patients had solitary myxomas (n=4) with only two being multiple myxomas. The average size of the myxomas was 3.7 cm. All soft tissue leisions were ipsilateral to the osseous leisions.
Five of the six patients were treated with surgical excision.
Discussion: In contrast to the existing literature the majority(n=4) of the six patients had solitary myxomas and the male to female distribution was equal. One of the cases was also unique to the current literature with the syndrome present in the right elbow.
The number of cases that we have seen in a short time may also be an indication that this syndrome is not as rare as scarcity of the published cases would imply.
Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35 - 43 Lincoln’s Inn Fields, London WC2A 3PN
