Mazabraud Syndrome

Mazabraud syndrome is the association of fibrous dysplasia with soft-tissue myxomas. The soft-tissue myxomas are much more common with polyostotic fibrous dysplasia but can also be seen in the monostotic form. The myxomas (which may be multiple) tend to occur close to the most severely affected bones.

Differential considerations include:

• Neurofibromatosis: There may be osseous changes in neurofibromatosis (bowing, pathologic fracture, and pseudarthrosis) in association with soft tissue masses. The osseous changes are easily differentiated from fibrous dysplasia.
• Metastases: The soft tissue mass will not generally have well-defined margins.
• Multiple myeloma: There may be extraosseous involvement in about 50% of patients. Subcutaneous nodules are common.
• Maffucci syndrome: Enchondromatosis with soft-tissue hemangiomas.
• Lymphoma: Focal muscle involvement is rare.

References

Kransdorf MJ, Murphey MD. Diagnosis please. Case 12: Mazabraud syndrome. Radiology. 1999 Jul;212(1):129-32.