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Key Features


  • Dysplastic fibrous tissue replacement of the medullary canal is accompanied by the formation of metaplastic bone in areas with fibrous dysplasia

  • Three forms of the disease are recognized

    • Monostotic

    • Polyostotic

    • Polyostotic with endocrine disturbances (precocious puberty in females, hyperthyroidism, and hyperadrenalism [Albright syndrome])


Clinical Findings


  • Characteristics of lesions

    • May be asymptomatic

    • Begins centrally within the medullary canal, usually of a long bone, and expands slowly

  • Pain, if present, is probably due to pathologic fractures

  • In females, endocrine disturbances may be present in the polyostotic variety and are associated with café au lait spots




  • If metaplastic bone predominates, the contents of the lesion have the density of bone

  • The disease is often asymmetrical, and limb length disturbances may occur as a result of stimulation of epiphysial cartilage growth

  • Marked deformity of the bone may result, and a shepherd's crook deformity of the upper femur is a classic feature of the disease




  • No treatment is needed if the lesion is small and asymptomatic

  • Curettage and bone grafting are indicated if the lesion is large and produces or threatens pathologic fracture

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