|Other names: Not recommended: Ossifying fibroma, Kempson-Campanacci lesion.|
|Osteofibrous dysplasia of tibia (lower leg)|
Signs and symptoms
Osteofibrous dysplasia most often presents as a localized firm painless swelling of the large long bone of the lower leg in young children. The leg may appear bent and it is possible that a break in the bone can occur.
The cause is unknown. Rarely, cases arise in a family.
Osteofibrous dysplasia may also be mistaken for fibrous dysplasia of bone, although osteofibrous dysplasia is more likely to show an immunohistochemical reaction to osteonectin, neurofibromin 1, and S-100 protein.
Generally no treatment is required. Observation is usually recommended and surgery performed if there is a significant bone deformity, severe symptoms, a potential break or a break in the affected bone, or if a definitive diagnosis is required.
Osteofibrous dysplasia may enlarge in children under 10 years and then become smaller or resolve after puberty. That this non-cancerous tumor might progress to adamantinoma is debated. Case studies suggesting a progression generally lack long term follow-up and have sampling issues.
It is rare, particularly over the age of 15 years. The frequency of occurrence is not known precisely. The tibia accounts for around 90% of cases. The long bones of the arm are unlikely to be affected. Boys and girls appear affected equally.
- WHO Classification of Tumours Editorial Board, ed. (2020). "Osteofibrous dysplasia". Soft Tissue and Bone Tumours: WHO Classification of Tumours. 3 (5th ed.). Lyon (France): International Agency for Research on Cancer. pp. 460–462. ISBN 978-92-832-4503-2.
- Davies, A. Mark; Sundaram, Murali; James, Steven J. Imaging of Bone Tumors and Tumor-Like Lesions: Techniques and Applications. Springer. p. 420. ISBN 978-3-540-77982-7.
- "Bone tumours. What are Bone Tumours?". patient.info. Archived from the original on 24 April 2021. Retrieved 24 April 2021.
- Campanacci, M. (August 1976). "Osteofibrous dysplasia of long bones a new clinical entity". Italian Journal of Orthopaedics and Traumatology. 2 (2): 221–237. ISSN 0390-5489. PMID 1024109.
- Steven P. Meyers (2008). MRI of bone and soft tissue tumors and tumorlike lesions. Thieme. ISBN 9783131354211.
- Robert Mervyn Letts, Osteofibrous Dysplasia