Cases reported "Fibrous Dysplasia of Bone"

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1/10. Giant central ossifying fibroma of the mandible: report of case.

    A case is presented of a patient with a giant ossifying fibroma of the mandible that had been diagnosed earlier as fibrous dysplasia. Surgical resection of the tumor and involved bone was chosen as the mode of treatment because of the extent of the lesion and continued infection. Surgeons should be aware that ossifying fibroma is a very distinct possibility in large growing lesions in age groups older than is deemed compatible with fibrous dysplasia.
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2/10. Cystic fibrous dysplasia mimicking giant cell tumor: MRI appearance.

    We report the case of a 43-year-old man who presented with an osteolytic and expansive lesion in the left distal femur mimicking a giant cell tumor. Magnetic resonance imaging (MRI) showed that most of the lesion was cystic, and histological examination revealed fibrous dysplasia with marked cystic degeneration. Radiographic findings of cystic fibrous dysplasia in the end of a long bone may be similar to those of a giant cell tumor, and a biopsy is essential for the final diagnosis.
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3/10. Establishment and characterization of a cell line from a malignant fibrous histiocytoma of bone developing in a patient with multiple fibrous dysplasia.

    PURPOSE AND methods: In order to provide material for genetic analysis of fibrous dysplasia (FD), a cell line designated GBS-1 was established from a secondary bone malignant fibrous histiocytoma (MFH) developing in a rib of a 44-year-old male polyostotic FD patient. RESULTS: The GBS-1 cells are characterized by a pleomorphic spindle cell morphology with abundant mucus production. On transplantation to nude mouse subcutis the cell line forms myxoid-spindle cell sarcomas with giant cells, the myxoid product being positive for periodic acid-Schiff (PAS) and alcian blue (Al-B) stains and completely digested by hyaluronidase, mimicking the original tumor. Chromosome and genetic analyses revealed multiple structural and numerical abnormalities of chromosomes with a large number of unidentifiable chromosomes and p53 mutation in exon 7 with LOH in the counterpart. CONCLUSIONS: Since cell lines for FD have hitherto not been available, the GBS-1 cells should prove useful for genetic analyses of FD and also MFH of bone origin.
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4/10. Aneurysmal bone cyst associated with fibrous dysplasia.

    We report the clinicopathologic features of a 22-year-old patient with aneurysmal bone cyst and fibrous dysplasia of the orbit. The patient was evaluated clinically with computed tomography of the orbit before surgery. An orbital biopsy specimen was examined histologically with conventional light microscopy. The lesion was treated with combined neurosurgical and orbital intervention. Clinical evaluation revealed axial and inferior displacement of the globe. Computed tomography revealed a cystic mass in the superotemporal left orbit with adjacent bone erosion. "Ground-glass" thickening of the adjacent frontal bone and sphenoid bone was observed. Microscopic examination showed fibrous stroma with giant cells and hemosiderin-laden macrophages with adjacent trabeculae of woven bone and osteoblast cells. A fronto-orbital craniotomy was performed, the cystic cavity was excised, and the hyperostotic bone was debulked. After treatment, the globe position and patient appearance have improved. There has been no sign of recurrence of the aneurysmal bone cyst. Aneurysmal bone cyst should be considered in patients with fibrous dysplasia that has a cystic component, or in patients with fibrous dysplasia who present with sudden expansion of their lesion.
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5/10. 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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6/10. cherubism in siblings: a case report.

    cherubism is a non-neoplastic bone disease characterized by clinically evident bilateral, painless enlargements of the jaws that are said to give the patient a cherubic appearance. cherubism may appear in solitary cases or in many members of the same family, often in multiple generations. On radiography, the lesions exhibit bilateral multilocular radiolucent areas. Histopathologic evaluation reveals proliferating fibrous connective tissue containing numerous multinucleated giant cells. Since the first description of this condition in 1933, almost 200 cases have been reported. We describe cherubism in 2 siblings and briefly review the literature on this subject.
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7/10. Multiple craniofacial surgical interventions during 25 years of follow-up in a case of giant fibrous dysplasia. Case report.

    Fibrous dysplasia may affect the jaws as a monostotic lesion which most often ceases to be active at the end of normal bone growth. In some cases there is no stabilization on completion of normal skeletal growth. This may lead to severe malformation and functional disturbance to the face. In these cases, the patient must be followed regularly and resection of the deformed tissue must be performed repeatedly as the recurrences appear. attention is also brought to the fact that fibrous dysplasia may be difficult to separate from chronic sclerosing osteomyelitis, a condition that should be treated differently.
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8/10. Central giant cell granuloma and periapical fibrous dysplasia occurring in the same jaw.

    A 19-year-old woman presented with two tumorlike conditions in the mandible. Seven vital mandibular teeth were associated with periapical lesions. One periapical lesion was enucleated and histopathologic examination confirmed the diagnosis of periapical fibrous dysplasia. The second lesion was a large multilocular radiolucency which extended from the mandibular left premolars to the ascending ramus. It was diagnosed as a central giant cell granuloma despite having many features in common with those of an ossifying fibroma. The lesion was removed and healing was uneventful.
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9/10. Osteoid-producing orbital ethmoid tumor.

    Fibro-osseus lesions of the paranasal sinuses, especially those that contain multinucleated giant cells, demonstrate a significant histopathologic overlap although they manifest themselves throughout a wide range of clinical behavior. They routinely require a critical clinical and pathologic interpretation. Often, the clinical course of the disease is the only guide to diagnosis and mode of treatment. This study presents the case of a 4-year-old boy with an osteoid-producing tumor of the ethmoid sinus. Despite an original diagnosis of fibrous dysplasia, the lesion followed a relentless course. Eventually, maxillectomy, orbital exenteration, and craniotomy were required to control the disease. The literature of fibro-osseous lesions of the paranasal sinuses is reviewed and an attempt is made to classify these lesions according to their clinical behavior.
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10/10. Maxillary giant cell reparative granuloma.

    "Giant cell reparative granuloma" was introduced into medical literature by Jaffe in 1953. Prior to that time most authors considered this lesion to be a variant of the benign giant cell tumor of the long bones, or a giant cell variant of osteitis fibrosa. Bernier and Cahn established the subdivision between the rare central giant cell reparative granuloma and the common peripheral epulis. In the past, considerable emphasis has been placed on the importance of differentiating the true giant cell tumor from the giant cell reparative granuloma of the jaw bones. Most authors now believe the true giant cell tumor does not appear in the jaw bones except in rare cases associated with Paget's disease of the skull. Developing from membranous, rather than cartilaginous, ossification might account for this. Both peripheral and central intraosseous lesions, parathyroid osteopathy and the pathologic tissue of cherubism show no appreciable histologic difference. These tumefactions are histologically a proliferative fibroblastic lesion with multinucleated giant cells. The histopathology of the giant cell tumor of the long bones is probably identical to the histopathology of the giant cell reparative granuloma of the jaw bones. The diagnosis of giant cell reparative granuloma must be made by physical examination, history, laboratory, X-ray parameters and clinical follow-up. Localized maxillary swelling is the most important clinical feature. The swelling is smooth and palpation can reveal a rubbery, elastic sensation where bone has been thinned. There are no specific radiographic signs. Conservative surgical management is indicated and adequate for giant cell reparative granulomas. radiation is not indicated because of long term risks. steroids have not been proven useful.
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