Cases reported "Fibrous Dysplasia of Bone"

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11/40. Synovial sarcoma associated with osteofibrous dysplasia. A case report and review of the literature.

    We report on a 14-year-old boy who demonstrated an unusual association between osteofibrous dysplasia and synovial sarcoma. This case suggests that a patient who presents with osteofibrous dysplasia of the tibia can eventually develop a malignant musculoskeletal tumor in the same anatomical location (same limb). This experience suggests that a patient who presents with osteofibrous dysplasia should be followed up for the possibility of a coexisting synovial sarcoma in the same leg.
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12/40. 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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13/40. Juvenile intracortical adamantinoma of the tibia with predominant osteofibrous dysplasia-like features.

    In view of the still disputed relationship between adult adamantinoma and osteofibrous dysplasia in children, a unique case of adamantinoma, indicating a direct relationship between the two lesions, is presented with a review of the literature. The patient was a six-year-old boy who complained of pain and swelling in the left lower leg. Roentgenographs showed a loculate osteolysis surrounded by sclerosis within the cortex of the tibial shaft that would be typical of osteofibrous dysplasia. Although an osteofibrous-dysplastic component predominated histologically, some small islands of epithelial cells were scattered throughout the lesion. Immunohistochemically, the tumor cells of these epithelial islands gave a constant positive reaction for cytokeratin as well as vimentin, while the stromal cells in the osteofibrous dysplasia-like lesion were positive for vimentin only. This type of lesion is recorded in the Bone Tumor Registry of Westphalia at a rate of 8.3% for osteofibrous dysplasia, and of 25% for adamantinoma. A review of the literature, yielding reports with remarkable uniformity on 14 cases beyond the present one, suggests the existence of a separate clinicopathologic entity to be called juvenile intracortical adamantinoma with predominant osteofibrous dysplasia-like features, and which might be a regressing form of adamantinoma specific in childhood.
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keywords = tibia
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14/40. Focal fibrocartilaginous dysplasia: consideration of healing process.

    We report two patients with unilateral tibia vara due to focal fibrocartilaginous dysplasia of the medial aspect of the proximal tibia. It appears that tibia vara, caused by a primary self-limited lesion at the proximal medial metaphysis, is corrected by the response of the proximal tibial physis. This finding would indicate that an infantile growth plate of the proximal tibia has a rather large potential to correct an angular deformity at the adjacent metaphysis.
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keywords = tibia
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15/40. Case report 587: adamantinoma of the tibia mimicking osteofibrous dysplasia.

    This case emphasized that the differential diagnosis of osteofibrous dysplasia and adamantinoma of the tibia can be difficult on roentgengrams as well as on histological studies. A radiological and histological diagnosis of osteofibrous dysplasia of the tibia in a young adult should always raise the consideration of the possibility of an adamantinoma, and in the correlation of the radiological features with the biopsy findings it is very important to gain representative tissue material for establishing the correct diagnosis of adamantinoma.
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keywords = tibia
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16/40. Three additional cases of focal fibrocartilaginous dysplasia causing tibia vara.

    Three additional cases of unilateral tibia vara caused by focal fibrocartilaginous dysplasia are reported in children aged 5, 6, and 18 months. These three cases support the conclusion of our original case report that unilateral tibia vara is most likely a progressive deformity, but that documentation of the progression should be obtained before a valgus osteotomy is performed.
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ranking = 6
keywords = tibia
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17/40. MRI findings in osteofibrous dysplasia.

    A case of osteofibrodysplasia of the long bones in a 4-year-old girl is reported with ipsilateral tibial and fibular involvement. The radiological pattern of the lesions suggested fibrodysplasia but not their MRI signal characteristics. This diagnostic imaging mismatch and the bifocality of the lesion were the tell-tale indications for the confirmatory bone biopsy yielding osteofibrous dysplasia of Campanacci.
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keywords = tibia
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18/40. adamantinoma of the tibia masked by fibrous dysplasia. Report of three cases.

    Three cases of adamantinoma of the tibia associated with and mimicking fibrous dysplasia or osteofibrous dysplasia are reported in children aged three, nine, and 16 years. The roentgenographic features were typical of intracortical fibrous dysplasia (osteofibrous dysplasia). These entities are not two distinct diseases, but rather are different histologic expressions of essentially the same process, which varies depending on location (predominantly intracortical or medullary) and age. adamantinoma in children under ten years of age is not as rare as reported in the literature and was found in two of 14 cases in our files (14.3%). In some cases the fibrous dysplasialike component predominates over the scarce epithelioid islands of tumor cells and consequently is not recognized as adamantinoma. That may explain the frequent recurrences after incomplete excisions of supposed intracortical fibrous dysplasia lesions in young children. An extensive histopathologic study of the biopsy and/or surgical specimen by a specialized pathologist is therefore advisable.
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ranking = 5
keywords = tibia
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19/40. tibia vara caused by focal fibrocartilaginous dysplasia.

    A case of severe unilateral tibia vara in a young boy is reported. The clinical and radiological features were identical to the only three reported cases of focal fibrocartilaginous dysplasia. With conservative management the child showed a complete recovery. The importance of recognising this condition is emphasised, as unnecessary surgery may be avoided. The radiologist can play a major role in making the diagnosis.
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keywords = tibia
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20/40. Malignant degeneration in fibrous dysplasia (presentation of 6 cases and review of the literature).

    Malignant degeneration of fibrous dysplasia is rare. It occurs with similar frequency at all ages and in both sexes. It is more frequent in cases of polyostotic than in monostotic fibrous dysplasia. In cases of fibrous dysplasia that do show malignant degeneration it is common to find that a high level of alkaline phosphatase persists in the serum, even in adults. Previous radiotherapeutic treatment appears to me a predisposing factor. osteosarcoma is the most frequent neoplasm, followed at some distance by fibrosarcoma and chondrosarcoma. The tumour is most often localised in the femur; it is not unusual to find it in the tibia, maxilla and mandible. The treatment and prognosis are the same as those of the involved malignant neoplasm.
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