Osteofibrous dysplasia is a benign fibro-osseous cortical lesion. It is most commonly seen in the mid-diaphysis of the tibia. It is considered as a separate condition and not synonymous with ossifying fibroma.
- Most are seen below the age of 5 years.
- Male children are more commonly affected than female children.
- Tibia is the most common bone. It can be seen in other long bones, such as fibula, radius, and ulna.
- It can be locally destructive and may result in pathologic fracture.
- 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.
- Most present with pain and swelling.
- May present secondary to pathologic fracture.
- It is benign condition, seen as trabecular bone woven within fibrous stroma.
- It is considered as part of spectrum of adamantinomas and 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 remains the initial and chief investigation.
- Location along the long axis: mid diaphysis
- Location along the transverse axis: cortical
- 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
- Has homogeneous hyperintensity on T2-weighted images.
- Can extend into the medullary cavity, but a large medullary component should raise suspicion for adamantinoma, which often have complete medullary canal involvement.
- No soft-tissue component.
- No cortical destruction.