Osteofibrous Dysplasia




Campanacci Disease, Cortical Osteofibrous Dysplasia, Ossifying Fibroma


CLINICAL SUMMARY


Osteofibrous dysplasia is a distinctive intracortical lesion almost always seen in the anterior tibial cortex. Histologically, it resembles fibrous dysplasia. Small lesions can be observed, whereas curettage may be needed for larger ones, although it is associated with a high recurrence rate. Extensive bone involvement may lead to deformity and repeated fractures.


DIAGNOSTIC FEATURES





















History


  • Most commonly younger than 10 years of age; rare after 16 years



  • Persistent mild pain



  • Extensive disease can present with multiple fractures or tibial bowing

Location


  • Intracortical, diaphyseal, elongated



  • Larger lesions can appear to involve the medullary canal



  • Distribution: almost exclusively found in the anterior tibia and/or fibula



  • May be multifocal in the tibia and fibula

Margins


  • Lytic regions demonstrate geographic margins



  • Some expansile remodeling can be seen, but no soft tissue mass

Matrix


  • Fibrous or fibro-osseous



  • Remnant cortex may demonstrate marked sclerosis




IMAGING
















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