Osteofibrous dysplasia

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Osteofibrous dysplasia
Other names: Not recommended: Ossifying fibroma, Kempson-Campanacci lesion.[1]
Osteofibrous-dysplasia-of-the-tibia.jpeg
Osteofibrous dysplasia of tibia (lower leg)

Osteofibrous dysplasia is a noncancerous bone tumor.[1] It most often affects the large long bone of the lower leg in young children.[2][3]

It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula,[4] and coined the term.

This condition should be differentiated from fibrous dysplasia of bone[1] and nonossifying fibroma.

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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.[2] The leg may appear bent and it is possible that a break in the bone can occur.[2][5]

Cause

The cause is unknown. Rarely, cases arise in a family.[1]

Diagnosis

Diagnosis is by x-ray.[2]

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.[5]

Treatment

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.[1]

Outcomes

Osteofibrous dysplasia may enlarge in children under 10 years and then become smaller or resolve after puberty.[1] 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.[1]

Epidemiology

It is rare, particularly over the age of 15 years.[2] The frequency of occurrence is not known precisely.[2] The tibia accounts for around 90% of cases.[2][6] The long bones of the arm are unlikely to be affected.[2] Boys and girls appear affected equally.[2]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 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.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 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.
  3. "Bone tumours. What are Bone Tumours?". patient.info. Archived from the original on 24 April 2021. Retrieved 24 April 2021.
  4. 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.
  5. 5.0 5.1 Steven P. Meyers (2008). MRI of bone and soft tissue tumors and tumorlike lesions. Thieme. ISBN 9783131354211.
  6. Robert Mervyn Letts, Osteofibrous Dysplasia

External links

External resources