Osteofibrous dysplasia

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Osteofibrous dysplasia
Other names: Ossifying fibroma

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

It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula,[3] and coined the term. This condition should be differentiated from nonossifying fibroma and fibrous dysplasia of bone.

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.[1] The leg may appear bent and it is possible that a break in the bone can occur.[1][4]

Several authors have related this non-neoplastic lesion to adamantinoma - a tumor involving subcutaneous long bones - stating the common cause to be fibrovascular defect.[5] However, the latter is distinguished from an osteofibrous dysplasia by the presence of soft tissue extension, intramedullary extension, periosteal reaction and presence of hyperchromic epithelial cells under the microscope.

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.[4]

Diagnosis

Diagnosis is by x-ray.[1]

Treatment

Osteofibrous dysplasia is treated with marginal resection with or without bone grafting, depending on the size of the lesion and the extent of bony involvement. However, due to the high rate of recurrence in skeletally immature individuals, this procedure is usually postponed until skeletal maturity.[citation needed]

Epidemiology

It is rare, particularly over the age of 15 years.[1] The frequency of occurrence is not known precisely.[1] The tibia accounts for around 90% of cases.[1][6] The long bones of the arm are unlikely to be affected.[1] Boys and girls appear affected equally.[1]

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 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.
  2. "Bone tumours. What are Bone Tumours?". patient.info. Archived from the original on 24 April 2021. Retrieved 24 April 2021.
  3. 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.
  4. 4.0 4.1 Steven P. Meyers (2008). MRI of bone and soft tissue tumors and tumorlike lesions. Thieme. ISBN 9783131354211.
  5. Hatori M, Watanabe M, Hosaka M, Sasano H, Narita M, Kokubun S (2006). "A classic adamantinoma arising from osteofibrous dysplasia-like adamantinoma in the lower leg: a case report and review of the literature". Tohoku J Exp Med. 209 (1): 53–59. doi:10.1620/tjem.209.53. PMID 16636523.
  6. Robert Mervyn Letts, Osteofibrous Dysplasia

External links

External resources