Etiology and treatment of fibrous dysplasia
Fibrous dysplasia is a benign bone lesion composed of a proliferation of spindle cells producing dysplastic bone. It occurs in a monostotic form, a polyostic form, or as part of McCune-Albright syndrome (polyostotic form with endocrinopathies). In all three cases, it is caused by a somatic activating mutation in the trimeric stimulatory guanine nucleotide binding protein, Gs. Treatment depends on the severity of involvement and location of the lesion and consists of observation, internal fixation to stabilize the bone, or osteotomies to correct mechanical deformity. Bisphosphonate therapy increases radiographic bone density and may be helpful in cases that are otherwise difficult to manage. Future treatment may be developed based on the molecular pathology of the Gs mutation.
Current Opinion in Orthopaedics
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