Cases reported "Fibroma, Ossifying"

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1/3. Osteofibrous dysplasia of the ulna.

    We experienced two patients with osteofibrous dysplasia of the ulna. Both of them showed typical radiographic findings of osteofibrous dysplasia, except for location. One patient underwent surgery at the age of 15 years to confirm the diagnosis histologically. Although the tumor recurred 5 months after the surgery, expansion of the lesion seemed to cease at the age of 25 years. In the other patient, the natural course was observed. The tumor showed a tendency toward spontaneous regression at the age of 11 years. Therefore, the nature of osteofibrous dysplasia in the ulna seems to be the same as that in the tibia and fibula. Surgery must be delayed for as long as possible in osteofibrous dysplasia of the ulna, as well as that of the tibia and fibula.
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keywords = tibia
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2/3. Osteofibrous dysplasia treated with distraction osteogenesis: a report of two cases.

    We treated two patients with osteofibrous dysplasia of the tibia using en bloc marginal excision of the lesion and bone transport, a distraction osteogenesis procedure. In one patient, we had performed curettage procedures twice, each time followed by local recurrence and deformity. In contrast, with marginal excision and bone transport using an Ilizarov apparatus, the disease healed. In the other patient, we performed marginal excision and bone transport as the first procedure, which resulted in a healed lesion. In patients with osteofibrous dysplasia requiring treatment by an open method, curettage frequently is followed by local recurrence and deformity. We now prefer marginal excision of the lesion to avoid long-term failures, as in our first case. Distraction osteogenesis has proven useful for restoring structure and function in the affected limb.
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keywords = tibia
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3/3. Clonal chromosomal abnormalities in osteofibrous dysplasia. Implications for histopathogenesis and its relationship with adamantinoma.

    BACKGROUND. The pathogenesis of osteofibrous dysplasia, a rare fibroosseous lesion occurring almost exclusively in the tibia of children younger than 10 years of age, is not known. One theory is that osteofibrous dysplasia results from excessive resorption of bone with fibrous repair of the defect. Alternatively, osteofibrous dysplasia has been considered a congenital lesion or a variant of fibrous dysplasia. It has been hypothesized that osteofibrous dysplasia is a secondary reactive process to adamantinoma. cytogenetic analysis is one form of investigation that has been instrumental in determining the origin of many disorders. methods. Short-term cultures of two separate osteofibrous dysplasia specimens (approximately 1 year apart) from the tibia of an 11-year-old boy and 2 separate specimens (approximately 2 years apart) from the tibia of a 16-year-old boy were cytogenetically examined using standard procedures. Additionally, fluorescence in situ hybridization (FISH) studies were performed on uncultured cells of both specimens of the first patient using an alpha-satellite probe for chromosome 12. RESULTS. Cytogenetic and FISH analysis revealed trisomy 12 in both specimens of the first patient. trisomy for chromosomes 7, 8, and 22 was seen in both specimens of the second patient. CONCLUSIONS. Osteofibrous dysplasia has not previously been subjected to cytogenetic analysis. trisomy 7 and 12, however, have been reported in a clonally aberrant adamantinoma, potentially providing further support for a relationship between these two lesions. Most importantly, these findings demonstrate a clonal and possibly neoplastic origin for osteofibrous dysplasia of long bone.
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ranking = 1.5
keywords = tibia
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