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Mazabraud's syndrome: intramuscular myxoma associated with fibrous dysplasia of bone.

A 53 year old male with a large swelling on the medial aspect of his right thigh was referred with a presumptive diagnosis of soft tissue sarcoma. However, biopsy revealed intramuscular myxoma and X-rays and CT scans suggested fibrous dysplasia of adjacent bone. Angiography had shown an expanded, hypervascular, intramedullary lesion in the femur, and a large avascular soft tissue mass lying medially in the distal thigh. Fibrous dysplasia of the femur was confirmed on bone biopsy. Subsequently one large and two smaller intramuscular myxomata were excised, with an uneventful postoperative course. This case illustrates Mazabraud's syndrome: the rare association between benign intramuscular myxoma and fibrous dysplasia of bone.

Femur↗

Soft-tissue myxomas and fibrous dysplasia of bone. A case report and review of the literature.

An association between fibrous dysplasia and myxomas (soft-tissue neoplasms of mesenchymal origin) has been described in the literature. The authors report another such case of fibrous dysplasia/myxoma coexistence: a patient with polyostotic fibrous dysplasia who developed right anterior thigh pain from a solitary intramuscular myxoma. After a thorough review of the literature, the authors found that a causal relationship between fibrous dysplasia and myxomata remains uncertain. To ensure an adequate and accurate diagnosis, however, patients with soft-tissue myxomas should be thoroughly evaluated for fibrous dysplasia. A proper investigation of soft-tissue masses also should be carried out to exclude malignancy.

Adult↗

Fibrous dysplasia of the temporal bone: the use of computerized tomography.

Fibrous dysplasia of the temporal bone is a rare condition characterized histologically by proliferation of fibrous tissue with scattered trabeculae of immature bone. Eighteen cases of monostotic fibrous dysplasia of the temporal bone have been reported in the literature. The clinical course of temporal bone fibrous dysplasia is unpredictable. Potential complications include cholesteatoma, recurrence, and malignant transformation. Surgery has been the recommended treatment, but the indications, approach, and extent have not been clearly established. The introduction of computerized tomography with high-resolution bone reconstruction is a significant advance in the therapeutic approach to temporal bone fibrous dysplasia. It accurately defines the extent of the disease within the temporal bone, and periodic scanning will reveal any progression. This information can be used to resolve many surgical dilemmas and to minimize secondary complications. This article includes a comprehensive review of the literature on temporal bone fibrous dysplasia and summarizes a case in which computerized tomography was used.

Adolescent↗