Revision 6 for 'Osteofibrous dysplasia'

All Revisions - View changeset

Osteofibrous Dysplasia

Osteofibrous dysplasia is a benign fibro-osseous cortical lesion, almost exclusively occurs in tibia and fibula. It is most commonly seen in the mid-diaphysis of the tibia. It is considered as a separate condition and some consider synonymous with ossifying fibroma. The commonly used synonym is 'ossifying fibroma of long bones'.

Epidemiology and Natural History

  • Most are seen below the age of 5 years.
  • Self-limited intracortical fibroosseous lesion, almost exclusively affecting tibia and fibula of children
  • Male children are more commonly affected than female children.
  • It can be locally destructive and may result in pathologic fracture.
  • Usually has excellent prognosis and regresses as patients mature. They usually stabilize or spontaneously regress once the child is older than 10 years old. 
  • High rates of local recurrence is noted after surgery with some aggressive recurrence, possibly due to components of adamantinoma or possible transformation to adamantinoma.



Clinical Presentation

  • Most present with pain and swelling. 
  • May present secondary to pathologic fracture.


  • It is benign considered as a non-neoplastic benign condition
  • Histology shows trabecular bone woven within fibrous stroma. It shows osteoblastic rimming and mature lamellar bone.
  • It is considered as part of spectrum of adamantinomas and osteofibrous dysplasia-like adamantinoma.
  • It is closely related to fibrous dysplasia (fibrous dyplasia is predominantly medullary). Osteofibrous dysplasia was considered as a variant of fibrous dysplasia, but is now established as a distinct lesion, as zonal phenomenon and osteoblastic rimming of bony trabeculae are absent in fibrous dysplasia.
  • Osteofibrous dysplasia and ossifying fibroma of the jaw have similar histological characteristics, but osteofibrous dysplasia shows cytokeratin-positive cells and the ossifying fibroma of the jaw shows psammomatous calcification, which are exclusive features.
  • Although adamantinoma can contain osteofibrous dysplasia-like componentsa, nests or strands of epithelioid cell is the differentiating feature between adamantioma and osteofibrous dysplasia.
  • Adamantinoma with scarce epithelioid component and mostly composed of osteofibrous dysplasia-like tissue are considered as osteofibrous dysplasia-like adamantinoma.

Imaging features: General

  • Usually smaller than adamantinomas (mean 6-7 cm compared to 10-17 cm for adamantinoma).
  • Osteofibrous dysplasia is usually seen in pediatric age group (adamantinoma are usually seen in adults).

Plain radiograph

Plain radiograph remains the initial and chief investigation.

  • Location along the long axis: mid diaphysis, especially anteriorly
  • Location along the transverse axis: cortical with medullary encroachment
  • Appreance: lobular loculations to bubbly
  • Desnity: lucent or ground-glass
  • Zone of transition: narrow
  • Margins: well-defined and sclerotic
  • Other features which may be present: Pseudotrabeculations, anterior bowing
  • Negative features: no periosteal reaction, no nidus


MRI is the next step in investigating suspected fubrosseusys dysplasia

  • Intermediate signal on T1.
  • Intermediate-to high signal on T2.
  • Diffue and intense enhancement.
  • No soft-tissue component.
  • No cortical destruction.


  • Surgery is reserved for large size or aggressive behavior.

Differential Diagnosis


Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.