Fibrous dysplasia.
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 G(s)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.
Department of Orthopaedic Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
Selene G
SG
Parekh
Rakesh
R
Donthineni-Rao
Eric
E
Ricchetti
Richard D
RD
Lackman
D016025:Bone Transplantation; D003475:Curettage; D004164:Diphosphonates / Q000627:therapeutic use*; D005358:Fibrous Dysplasia, Monostotic / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy; D005359:Fibrous Dysplasia, Polyostotic / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy; D016762:Genes, fos; D006801:Humans; D009154:Mutation; D011859:Radiography; D016896:Treatment Outcome
D004164:Diphosphonates