Osteofibrous Dysplasia Workup

Updated: Aug 17, 2021
  • Author: Darin Davidson, MD; Chief Editor: Omohodion (Odion) Binitie, MD  more...
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Workup

Imaging Studies

Radiography

In children, osteofibrous dysplasia initially engenders tremendous concern among clinicians and parents regarding the possibility of malignancy. However, the appearance usually is typical, and radiologic diagnosis generally proves sufficient.

The characteristic radiographic appearance of osteofibrous dysplasia (see the images below) has been well reported. Lesions are eccentric, intracortical, and osteolytic. Variable expansion of the external cortical surface is present, with sclerosis of the internal cortical surface. Frequently, a multilocular lesion gives rise to a bubbled appearance. Soft-tissue extension is absent, and periosteal reaction is rare, unless there is an associated pathologic fracture. The size of the lesion is variable. Usually, it affects the diaphysis, though metaphyseal encroachment has been reported.

Characteristic radiographic findings of osteofibro Characteristic radiographic findings of osteofibrous dysplasia. Note eccentric intracortical lesion with sclerosis of internal surface, bubbled appearance of lesion, and anterior tibial bowing.
Radiograph of osteofibrous dysplasia of tibia in 5 Radiograph of osteofibrous dysplasia of tibia in 5-year-old girl.

CT and MRI

To date,the  diagnostic characteristics of osteofibrous dysplasia on computed tomography (CT) or magnetic resonance imaging (MRI) have not been well established. [54] However, Jung et al reviewed MRI scans of 24 pathologically proven cases and concluded that osteofibrous dysplasia showed a range of features from limited to aggressive lesions. [55]

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Biopsy

Because the clinical course and the radiologic appearance of osteofibrous dysplasia are diagnostic in children, biopsy is seldom indicated and should be avoided if possible. In patients presenting at skeletal maturity, in whom the incidence of adamantinoma is higher, biopsy of the midportion of the lesion may be necessary for diagnosis. If a biopsy is performed, histologic examination is generally definitive.

If biopsy is necessary to confirm the diagnosis, consult with a radiologist and a pathologist to ensure an adequate specimen. Adhere to strict biopsy principles; a malignant process has not yet been excluded.

Biopsy the tibia away from the apex of the tibial curvature to minimize the development of a fatigue fracture, which is common after biopsy in osteofibrous dysplasia. Incise the skin longitudinally and minimize dissection to the greatest extent possible. Disrupt as few compartments as possible; dissect through, rather than adjacent to, muscle; fill bone defects; and strictly maintain hemostasis.

Biopsy material should include periosteum, cortical bone, and medullary material, both central and peripheral to the lesion. Tissue obtained must be representative of the lesion and adequate for histologic grading. Obtain a frozen section to ensure the specimen is sufficient. Avoid leaving sharp edges that may act as stress risers, leading to postbiopsy fracture.

After biopsy, protect the limb in a cast or splint for 3-6 weeks.

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Histologic Findings

Despite the characteristic radiographic appearance, Wang et al recommended that diagnosis should be based on biopsy and pathologic examination. [9] At the time of surgery, inspection reveals an intact periosteum. The cortex is thinned and may be perforated. The lesion itself is composed of soft, granular tissue that is whitish-yellow in color.

The histologic characteristics of osteofibrous dysplasia are well described. The overall appearance is that of zonal architecture (see the first image below). The lesion is fibrous at its center, with immature woven bone trabeculae. Vascular channels have been described within the lesion (see the second image below). At the periphery, a prominent border of active osteoblasts rims the bony trabeculae. The presence of such a border helps differentiate osteofibrous dysplasia from fibrous dysplasia, in which there is no border of active osteoblasts (see the third image below).

Typical histologic appearance of osteofibrous dysp Typical histologic appearance of osteofibrous dysplasia lesion (×100). Note zonal architecture with periphery of active osteoblasts surrounding bone trabeculae.
Histologic section (×100) demonstrating vascular c Histologic section (×100) demonstrating vascular channels within osteofibrous dysplasia lesion, which has been proposed as etiologic factor in development.
Histologic appearance of fibrous dysplasia, reveal Histologic appearance of fibrous dysplasia, revealing appearance similar to osteofibrous dysplasia but lacking periphery of active osteoblasts.

As examination proceeds from the center of the lesion to the periphery, the bone trabeculae become larger and more lamellar in appearance. Fibroblasts in the lesion have been noted to be well differentiated. Several studies have reported cytokeratin-positive elements on immunohistochemical staining. Occasional hemorrhagic zones, cysts, or foci of cartilaginous differentiation have been reported. Multinucleated giant cells have also been observed.

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