Fact-sheet: Osteofibrous dysplasia
Updated on 09/30/2019 at 4:28 PM
View all RADEOS cases associated with this fact-sheet
Definition
Also called Campanacci disease
This is a benign condition:
- Affecting children under 10 years of age (70% of children are under 5 years old)
- Main locations: Tibia +++ in isolation (78% of cases) / combined tibia and fibula involvement / fibula in isolation
Clinical features
- Asymptomatic
- Limb deformity (anterior bowing of the leg, increase in tibial volume) --> mimics Pagetic deformity in adults
- Pathologic fracture (25% of cases)
Laboratory findings
No biological abnormality has been described
Radiography
- Intracortical lesion resulting in thickening of the anterior cortex, expanding outward (bulging) and inward (narrowing of the medullary canal)
- Manifests as a zone of cortical hyperostosis containing one or more areas of osteolysis oriented along the long axis of the bone
- The areas of osteolysis may have a ground-glass appearance and contain ossified zones or be radiolucent
- The opposite cortex is normal
Management
- Surgery: high recurrence rate when performed before age 10. Indicated in cases of local aggressiveness and significant deformities: risk of pathologic fracture
- Simple surveillance is most often performed
- Favorable course with spontaneous resolution after puberty
Differential diagnosis
Adamantonoma +++:
- Rare malignant tumor (0.1 to 0.5% of primary malignant bone tumors), more common in young male adults
- Two histologic forms: classic Adamantonoma vs osteofibrous dysplasia-like Adamantonoma
- 90% of cases: tibial involvement
- Fibula involvement is possible (then often associated with tibial involvement)
- Local aggressiveness with risk of distant metastases
- Standard radiographs show cortical involvement with a well-circumscribed osteolytic lesion, uni- or multilocular, with sharp margins, surrounded by a zone of osteosclerosis
- CT and MRI are needed in addition to assess possible extension into the soft tissues and bone marrow
