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- Also called Osteofibrous Dysplasia
Pathology
- May be variant of Fibrous Dysplasia
- Some believe true neoplasm with aggressive behaviour
Natural History
- Usually locally aggressive – may present with rapidly enlarging deformity
- Less aggressive as child gets older & no metastases
Presentation
- Young child
- Anterior tibial metaphysis & diaphysis
- Anterior bow or mass in tibia
- Pathological fracture common
Investigations
Xray
- Multiple small radiolucent lesions over extensive length
- Rim of reactive bone present & periosteum usually preserved
Pathology
Histology
- Similar to FD with irregular spicules of trabecular bone & unremarkable spindle cells
- But unlike FD the spicules lined with osteoblasts that produce layer of lamellar bone
- Thus distinction from FD is the woven bone with juxtaposed lamellar bone produced by plump osteoblasts
Differential Diagnosis
Treatment
- Treat with serial observation
- If progresses then biopsy & resection
- Curettage followed by aggressive recurrence