Abstract

Fibrous dysplasia is a common benign skeletal lesion that may involve one bone (monostotic) or multiple bones (polyostotic) and occurs throughout the skeleton with a predilection for the long bones, ribs, and craniofacial bones. The etiology of Fibrous dysplasia has been linked to the posygotic mutation in the GNAS 1 (guanine nucleotide-binding protein, alpha stimulating activity polypeptide 1) gene (20ql3.2–13.3) resulting in up-regulation of cAMP. It may be isolated to a single skeletal site or multiple sites and sometimes is associated with extraskeletal manifestations in the skin and/or endocrine organs (McCune-Albright syndrome).

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