Cases reported "Fibrous Dysplasia of Bone"

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1/40. Osteofibrous dysplasia of long bones a new clinical entity.

    A new clinico-pathologic entity is described. It is defined as osteofibrous dysplasia of long bones, and is based on twenty two personal observations to which are added seventeen cases from the literature. This dysplasic congenital lesion is clearly differentiated from fibrous dysplasia by clinical, radiographic and histological characteristics and by its clinical course. These features may be summarised as follows: 1) Slight predominance of the male sex. 2) Very early age of onset either at birth or in the first years of life. 3) Site almost exclusively tibial, sometimes also in the fibula. Localisation predominantly in the middle third of the tibial diaphysis, but sometimes in the distal or proximal third. In the fibula, it is always at the distal third. 4) The lesion is painless and generally causes bony enlargement. There is often slight anterior bowling and more rarely, slight varus of valgus bowing. Pathological fracture may occur; rarely there is a pseudarthrosis. 5) The radiographic appearances are very characteristic, with enlargement of the bone, intracortical osteolytic lesions with thinning or disappearance of the external cortex, sclerotic reaction on the medullary aspect, and narrowing of the medullary canal. 6) The histological features are also typical, consisting of fibrous tissue enclosing bone trabeculae lined by osteoblasts and a "zonal" architectural pattern. 7) Sometimes the lesion tends to heal spontaneously in the very early years of life; in other cases it is moderatley progressive. It relapses frequently after curettage, but such recurrences are generally non-progressive. In some cases slight anterior bowing persists permanently. 8) Surgery should be restricted to patients over the age of five in whom the lesion is extensive, with imminent or actual pathological fracture, and to the rare cases of pseudarthrosis. The results are good even in cases of relapse or pseudarthrosis. The correction of residual bowing, if indicated, can safely be carried out with one or more osteotomies at the age of ten to twelve years.
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keywords = tibia
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2/40. Two cases of secondary angiosarcoma arising from fibrous dysplasia.

    Angiosarcoma associated with fibrous dysplasia is very rare. We have recently experienced two cases of angiosarcoma that secondarily arose from fibrous dysplasia. The first patient, a 55 year-old man, had noticed a deformity in the right upper arm since he was five years old. At the age of 25 years, polyostotic fibrous dysplasia was diagnosed by X-ray examination. The patient complained of swelling and pain around the left shoulder. The diaphysis of the humerus was mostly non-observable due to severe bone destruction by tumor invasion and there was a large soft tissue tumor. biopsy examination revealed grade II or III hemangioendothelioma with typical histologic findings of fibrous dysplasia. After interscapulothoracic amputation for wide tumor resection, he died of DIC. autopsy revealed multiple liver metastatic lesions of angiosarcoma. The second patient was a 66-year-old woman. She presented with a pathological fracture in the right tibia, due to an osteolytic lesion with cortical bone swelling and thinning. The histologic diagnosis was fibrous dysplasia without any sarcomatous changes. However, after a 3-time recurrence, angiosarcoma arose from the same lesion. She is now still alive with disease-free period of 6.5 years after amputation.
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keywords = tibia
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3/40. Focal fibrocartilaginous dysplasia of long bones: report of eight additional cases and literature review.

    We report eight additional cases of focal fibrocartilaginous dysplasia (FFCD) in the proximal tibia (five), distal ulna (one), and distal femur (two). Spontaneous, complete resolution of the lesion was observed in two tibiae and one ulna. Three tibial lesions with genu varum deformity were managed with osteotomy. Two femoral FFCDs caused persistent or progressive deformity: one genu valgum with patellar dislocation, and one genu varum. These patients underwent concomitant deformity correction and lengthening by the Ilizarov method. The final results were satisfactory in all patients except one, who underwent valgus tibial osteotomy and developed mild postoperative genu valgum. The analysis of a total of 46 cases in the literature and our experience suggests that (a) FFCD has a wide histopathologic spectrum, ranging from purely dense, fibrous tendon-like tissue to benign fibrocartilaginous tissue; (b) at least 45% of tibial FFCD demonstrates progressive, spontaneous resolution; (c) in contrast, femoral and humeral FFCDs appear to have a slim possibility of spontaneous regression of the deformity; and (d) corrective osteotomy is indicated when the deformity is increasing or persistent, or when the existing deformity is severe enough to jeopardize adjacent joint mechanics and alignment.
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ranking = 2.5
keywords = tibia
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4/40. Extra copies of chromosomes 7, 8, 12, 19, and 21 are recurrent in adamantinoma.

    adamantinoma of long bones is a rare neoplasm predominantly involving the tibia. Cytogenetic studies of adamantinoma are few. Cytogenetic or molecular cytogenetic analysis of four adamantinomas, and a review of eleven cases in the literature reveals extra copies of chromosomes 7, 8, 12, 19, and 21 as recurrent in this neoplasm. adamantinoma may be confused with a variety of primary and metastatic epithelial and mesenchymal neoplasms. observation of these aneuploidies may be useful in establishing the diagnosis of adamantinoma.
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5/40. Osteofibrous dysplasia of the ulna.

    Osteofibrous dysplasia is a rare condition usually ffecting the tibiae and fibulae of males in the first two decades of life. Involvement at the ulna has also been reported in some cases. We describe an extensive, rapidly progressive lesion of the ulna in a three-year-old girl. The progression of the disease accelerated after a local marginal removal, and required extensive resection of the ulna with reconstruction using a fibular autograft. The particular features of this case are the change in the aggressive nature of the condition, which has not been previously reported. We believe that this is only the second recorded case of histologically proven involvement of the ulna.
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keywords = tibia
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6/40. tibia valga due to focal fibrocartilaginous dysplasia: case report.

    Focal fibrocartilaginous dysplasia is a rare benign entity, previously described as a factor responsible for causing tibia vara at the walking age. We report here the case of a child with tibia valga due to focal fibrocartilaginous dysplasia of the lateral part of the proximal tibia. To our knowledge, this is the first observation of a lesion located in the lateral proximal tibia. Clinical, radiographic and magnetic resonance imaging surveillance was performed. Spontaneous resolution of the lesion with correction of the angular deformity did occur.
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ranking = 2
keywords = tibia
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7/40. Concomitant focal fibrocartilaginous dysplasia of the tibia and eosinophilic granuloma of the jaw in a child.

    This 2-year-old child presented with concomitant eosinophilic granuloma of the lower jaw and focal fibrocartilaginous dysplasia of the right tibia. Her eosinophilic granuloma was diagnosed on the basis of the clinical picture, imaging studies and the characteristic histologic appearance. Focal fibrocartilaginous dysplasia was revealed incidentally during the eosinophilic granuloma staging process. After chemotherapy, all signs of eosinophilic granuloma subsided, but focal fibrocartilaginous dysplasia remained without signs of clinical or radiographic progression. The importance of differentiating these two conditions is stressed in order to avoid ineffective and inappropriate treatment of focal fibrocartilaginous dysplasia.
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ranking = 2.5
keywords = tibia
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8/40. Osteofibrous dysplasia: two affected male sibs and an unrelated girl with bilateral involvement.

    Osteofibrous dysplasia (OFD) is a tumor-like bone lesion that occurs most often in the tibia, presenting as a painless swelling or anterior bowing. Radiographs show a well-circumscribed intracortical lucency, or multiple lucencies separated by sclerotic borders, associated with a diaphyseal expansion. The histogenesis of OFD and its possible relationships to fibrous dysplasia and to adamantinoma have been the subject of significant discussion and investigation. We have been unable to find any reports of familial OFD, and have found only two references to bilateral involvement. In this article, we report both bilateral and familial involvement, and suggest that more thorough investigation of patients and their families may uncover similar cases, and perhaps support a genetic component to the etiology of this condition.
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ranking = 0.5
keywords = tibia
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9/40. Classic adamantinoma with osteofibrous dysplasia-like foci and secondary aneurysmal bone cyst.

    adamantinoma, a rare bone lesion of the tibia and fibula, has two distinct variants, classic adamantinoma and osteofibrous dysplasia-like adamantinoma. Composite lesions have not been described. Aneurysmal bone cyst is a benign cystic lesion which may also occur in the tibia and fibula. We report an unusual case of classic adamantinoma with osteofibrous dysplasia-like areas and foci of secondary aneurysmal bone cyst with prominent giant cells. A lesion was diagnosed in a 17-year-old girl with a 14-year history of a slowly enlarging left tibial mass and increasing deformity. Pathologically, the predominant pattern was classic adamantinoma, with minor foci of osteofibrous dysplasia-like adamantinoma and areas of secondary aneurysmal bone cyst with abundant multinucleated giant cells. We report the clinical, radiologic, and pathologic features of this case, and summarize lesions associated with secondary aneurysmal bone cyst. To our knowledge, the association of adamantinoma with secondary aneurysmal bone cyst has not been previously reported.
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ranking = 1.5
keywords = tibia
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10/40. Osteofibrous dysplasia of the tibia treated by bracing.

    Osteofibrous dysplasia of the tibia in children will commonly recur after surgical resection. The use of a leg brace until puberty offers a useful method of conservative management. Seven female patients, age three months to nine years at the time of brace treatment, have been followed for 3.5 to 18 years, an average of nine years, with evidence of satisfactory healing of the lesions in all. Three of the cases had recurred after surgery, two with fibular grafts and one required leg lengthening. The use of a brace to control bowing of the tibia while awaiting spontaneous regression is advised until epiphyseal closure.
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ranking = 3
keywords = tibia
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