McCune-Albright Syndrome with Fibrous Dysplasia

Archie R McGowan, MD
Daniel S Williamson, MD, PhD

March 5, 1996

Presentation

Imaging Findings

Single view of the left hand demonstrates multiple large expansile "bubbly" lytic lesions with sharp transition zones and without an associated periosteal reaction (arrows). The lesions are located in the phalanges, carpels, metacarpals, distal ulna and radial bones. In addition, areas of ground glass appearance of the medullary replaced bone are visible (3rd metacarpal). The cortex is very thin in many areas overlying the expansile lytic lesion, making it difficult to determine if a fracture has occurred.

Differential Diagnosis

Diagnosis

Discussion

The etiology of the syndrome is unknown, but it is thought to represent a developmental abnormality. The clinical course may not be recognized until adulthood in many individuals. The usual presenting complaint is leg pain, limp or fracture. The most common physical deformity is the so-called shepherd's hook deformity which causes a leg length discrepancy and a limp (3).

Histologically, the changes of fibrous dysplasia reveal a medullary cavity filled with poorly ossified fibrous tissue (2). Malignant transformation is a rare complication. The most common histologies of transformed tumors include osteosarcoma and fibrosarcoma. The clinical signs most specific for transformation are pain and swelling in the absence of a fracture. Radiographically, osteolysis and cortical destruction suggest transformation (3).

References

2. Resnick D. Bone and joint imaging. Philadelphia: Saunders, 1989;1227-1232.

3. Edeiken J, Dalinka M, Karasick D. Edeiken's roentgen diagnosis of diseases of bone. Baltimore: Williams & Wilkins, 1259-1292.

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