Fibrous Dysplasia




CLINICAL SUMMARY


Fibrous dysplasia results when fibrous tissue and metaplastic bone replace marrow and lamellar bone. It is typically an asymptomatic, incidental discovery; however, when a large segment of a weight-bearing bone is affected, pain and deformity can result. A small number of patients with polyostotic disease have associated endocrinopathies.


DIAGNOSTIC FEATURES





















History


  • Usually diagnosed between 2 and 30 years of age



  • Most commonly an asymptomatic, incidental finding



  • Sometimes activity-related pain with larger lesions in weight-bearing bones

Location


  • Diaphyseal or metadiaphyseal, central



  • Elongated lesion, aligned with longitudinal axis of long bones



  • Distribution: femur, 35% to 40%; tibia, 20%; skull and facial bones, 10% to 25%; ribs, 10%; uncommon sites—hands and feet, spine, clavicle

Margins


  • Typically geographic lysis, may have sclerotic rim (monostotic lesions)



  • Expansile remodeling in some cases



  • Broad endosteal scalloping and diffuse cortical thinning

Matrix


  • Fibrous or fibro-osseous



  • Woven bone in marrow creates a “ground glass” appearance




IMAGING
































  • Fibrous dysplasia in distal ulna demonstrating central, diaphyseal location and ground glass matrix ( left ).



  • Expansile remodeling and cortical thinning are seen in a humerus lesion ( right ).










  • In this tibial lesion, fibrous dysplasia extends from the metaphysis into diaphysis ( left ). Note also the long extent of cortical thinning.



  • The margin can have a prominent rind of sclerosis (type IA margin), as seen on this tomogram of a lesion in the intertrochanteric femur ( right ).










  • Fibrous dysplasia in a rib typically appears elongated ( arrows ), as seen in this expansile lesion with ground glass matrix ( left ).



  • Increased radionuclide uptake on bone scan reflects increased matrix turnover ( right ). The elongated shape of fibrous dysplasia is a pattern not typically seen in neoplasms or fractures ( arrows ).


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