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J Am Acad Orthop Surg, Vol 12, No 5, September/October 2004, 305-313.
© 2004 the American Academy of Orthopaedic Surgeons
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Fibrous Dysplasia

Selene G. Parekh, MD, MBA, Rakesh Donthineni-Rao, MD, Eric Ricchetti, MD and Richard D. Lackman, MD

Dr. Parekh is Resident, Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA. Dr. Donthineni-Rao is Assistant Professor, Orthopaedic Oncology, Department of Orthopaedic Surgery, University of California, Davis, Sacramento, CA. Dr. Ricchetti is Resident, Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania. Dr. Lackman is Associate Professor and Chairman, Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania.

Reprint requests: Dr. Donthineni-Rao, UC Davis Medical Center, Suite 3800, 4860 Y Street, Sacramento, CA 95817.

Fibrous dysplasia is a developmental disorder of bone that can present in a monostotic or polyostotic form. Primarily affecting adolescents and young adults, it accounts for 7% of benign bone tumors. Many of the asymptomatic lesions are found incidentally; the remainder present with symptoms of swelling, deformity, or pain. Fibrous dysplasia has been associated with multiple endocrine and nonendocrine disorders and with McCune-Albright and Mazabraud’s syndromes. The etiology remains unclear, but molecular biology suggests a mutation in the Gs{alpha} subunit and activation of c-fos and other proto-oncogenes. Fibrous dysplasia has a characteristic radiographic appearance. Most cases do not require intervention, but those that do usually are managed surgically with curettage, bone grafting, and, in some cases, internal fixation. When some intervention is necessary but surgery is not practical, treatment is with bisphosphonates. The prognosis generally is good, although poor outcomes are more frequent in younger patients and in those with polyostotic forms of the disease. The risk of malignant transformation is low.






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Copyright © 2004 by the American Academy of Orthopaedic Surgeons.