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11/96. Monostotic fibrous dysplasia of the spine: report of a case involving the lumbar transverse process and review of the literature.

    Monostotic fibrous dysplasia of the spine is rare. We report its clinical, radiologic and histologic features affecting a 47-year-old housewife. She presented with low-back pain of 1-year's duration, and radiographs showed a diffuse expansile lesion in the left transverse process of the fourth lumbar vertebra. The lesion was excised and histologically confirmed to be fibrous dysplasia. The patient remained well 8 years after operation. Including the present case, a total of 22 cases of monostotic fibrous dysplasia of the spine were found in the literature. Combining these reported cases, we found that the condition affects either sex with equal frequency and presents at any age, the mean being 32 years. There is no predilection for any part of the spinal column, though sacral or coccygeal involvement is distinctly rare. It most commonly involves the body and adjacent pedicle, although no part of the vertebra is spared. It is worth noting that a propensity for progressive enlargement, even to the extent of causing graft destruction, exists if the lesion is left untreated or incompletely treated. Complete removal of all involved bone, together with stabilization, should therefore be the treatment of choice for this condition.
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keywords = dysplasia
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12/96. Segmental odontomaxillary dysplasia: a case report and review of the literature.

    Segmental odontomaxillary dysplasia (SOD) is a rare, unilateral developmental disorder of the maxilla involving abnormal growth and maturation of the bone, lack of one or both premolars, altered primary molar structure, delayed tooth eruption, and fibrous hyperplasia of the gingiva. In this, the twenty-third reported case of SOD, the literature is reviewed, and the clinical, radiographic, and histopathologic data are described. Computed tomographic scans of this case showed that the involved segment of the maxilla extends mesiodistally from the permanent cuspid to the mesial portion of the first permanent molar, largely limited to the area of the missing premolars. However, the affected bone extends superiorly in the lateral wall of the maxilla to the zygoma and base of the orbit. This article is intended to serve as baseline data for a future article, describing the natural history and possible treatment of SOD, which remain undocumented.
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ranking = 0.71428571428571
keywords = dysplasia
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13/96. Fibrous dysplasia with barely increased uptake on bone scan: a case report.

    Fibrous dysplasia, in general, appears as an area of markedly increased uptake on bone scintigraphy. Therefore, the possibility of fibrous dysplasia is likely to be excluded when the lesion shows no or slightly increased uptake. The authors report a case of incidentally detected fibrous dysplasia that appeared as slightly increased uptake on bone scintigraphy and was found to harbor a bone infarction along with typical fibrous dysplasia by pathologic examination of a specimen sampled by curettage. Barely increased bone uptake in fibrous dysplasia may be associated with decreased vascularity and osteoblast activity of the lesion as a result of concurrent bone infarction. The authors suggest that not every case of fibrous dysplasia appears as an area of intensely increased uptake on a bone scan. Clinicians should be cautious in interpreting bone scans of radiographically indicated fibrous dysplasia.
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ranking = 1.5714285714286
keywords = dysplasia
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14/96. Fibrous dysplasia with cystic appearance in maxillary sinus.

    Fibrous dysplasia (FD) in the paranasal sinuses is uncommon, and its management may be difficult. We report the case of a 25-year-old female with FD exhibiting a cystic appearance in the maxillary sinus. The patient had been complaining of facial swelling for few years. Imagery study showed a cystic lesion and dense bone changes in the maxillary bone. Inferior meatal antrostomy with a nasal endoscope failed to confirm a histological diagnosis. After a 3-year follow-up, the degree of facial swelling was unchanged, and the patient underwent middle meatal antrostomy and was diagnosed with FD.
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ranking = 0.71428571428571
keywords = dysplasia
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15/96. Fibrous dysplasia of both alae of the sacrum.

    A case of monostotic fibrous dysplasia involving both alae of the sacrum is reported. Only 2 cases of monostotic sacral involvement were published previously. The lesion was detected in a 42-year-old man suffering from lumbosacral pain after minimal trauma. Radiographic studies revealed cystic images on both alae of the sacrum, with internal condensations and some liquid contents. A trephine biopsy did not produce enough diagnostic data, and a posterior surgical approach was elected. curettage and refilling with allograft were performed. The pathologic anatomy study diagnosed a fibrous dysplasia. Two years after surgery, the patient was asymptomatic.
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ranking = 0.85714285714286
keywords = dysplasia
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16/96. Treatment of fibrous dysplasia of the mandible with radical excision and immediate reconstruction: case report.

    Monostotic fibrous dysplasia of the mandible is an unusual manifestation of the disease that is usually benign, occurs in young individuals, and is managed by conservative curettage or debridement. We present a case of persistent fibrous dysplasia complicated by pain and intraoral bony exposure that was successfully managed by radical resection and reconstruction with a free fibular flap. Although mandibular fibrous dysplasia is preferentially managed conservatively, treatment of this disease has evolved to a point where total excision and immediate reconstruction may be the treatment of choice and offer the best outcome.
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keywords = dysplasia
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17/96. magnetic resonance imaging evaluation of monostotic fibrous dysplasia of the tibia.

    The authors present a case report documenting the evaluation of monostotic fibrous dysplasia by magnetic resonance imaging. This type of evaluation demonstrates specificity for this disease process when combined with other imaging studies, laboratory findings, and clinical presentation. This technique is extremely useful in the identification of a no-touch lesion, allowing avoidance of an unnecessary bone biopsy.
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ranking = 0.71428571428571
keywords = dysplasia
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18/96. Sequential computed tomography images demonstrating characteristic changes in fibrous dysplasia.

    Images of fibrous dysplasia (FD) on computed tomography (CT) or magnetic resonance imaging (MRI) vary depending on the relative proportions of the fibrous and osseous components. Based on analysis of the literature, Camilleri suggested that structural changes revealed by conventional radiography may be indicative of the progression of FD. Here we describe the case of a boy who presented at 12 years of age with monostotic FD located in the right maxilla. The lesion first appeared as a lamellar, "onion peel"-like structure on CT; then over the course of the next five years, the structure became what has been termed an "egg-shell lesion", before eventually assuming the form of a "ground-glass lesion".
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ranking = 0.71428571428571
keywords = dysplasia
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19/96. Immunoexpression of neurofibromin, S-100 protein, and leu-7 and mutation analysis of the NF1 gene at codon 1423 in osteofibrous dysplasia.

    The NF1 (neurofibromatosis type 1, or von Recklinghausen disease) gene, is a tumor-suppressor gene, and its product, neurofibromin, down-regulates ras protein by its guanosine triphosphatase-activating protein (GAP)-related domain. Osteofibrous dysplasia (OFD) is characterized by fibroblast-like spindle cells and osseous tissue and is generally seen in the tibia or fibula during childhood. The precise nature of OFD remains controversial. Cosegregations of OFD and NF1 have been reported, and it has been surmised that OFD is associated with the NF1 gene. We studied the expressions of NF1 gene product (neurofibromin) and so-called Schwann cell markers (S-100 protein, Leu-7) in 17 cases of OFD immunohistochemically. Ten cases of fibrous dysplasia (FD) were also used for the purpose of comparison. Five OFD and 7 FD cases were analyzed for NF1 gene mutation at codon 1423, which is a GAP-related domain, by single-strand conformation polymorphism. Fibroblast-like cells of OFD showed the expression of neurofibromin (5 of 17), S-100 protein (9 of 17), and Leu-7 (5 of 17), and those of FD did not show these expressions, with the exception of 1 case that showed Leu-7 expression. Regarding the OFD cases, significant correspondence was found between cases showing expression of neurofibromin and S-100 protein, between cases showing expression of neurofibromin and Leu-7, and between cases showing expression of S-100 protein and Leu-7 (P < .01). NF1 gene mutation at codon 1423 was not detected in either the OFD (0 of 5) or FD (0 of 7) cases. These results seem to suggest the possible involvement of neurofibromin in the development of OFD, which is associated with the expression of Schwann cell markers (S-100 protein and Leu-7). Furthermore, NF1 gene mutation at codon 1423 did not seem to be related to OFD.
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ranking = 0.85714285714286
keywords = dysplasia
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20/96. Transnasal endoscopic surgery for the treatment of fibrous dysplasia of maxillary sinus associated to aneurysmal bone cyst in a 5-year-old child.

    Fibrous dysplasia (FD) and aneurysmal bone cyst involving the facial skeleton are rare but their synchronous association in other parts of the body is well-known. The most common treatment for these lesions has been based on surgical resection using an external approach. Only recently has the endonasal endoscopic approach been utilized for the partial or complete removal of these lesions. In this report, we attempt to outline the effectiveness of the endonasal endoscopic approach for the treatment of a 5-year-old child affected by a fibrous dysplasia associated with an aneurysmal bone cyst of the right maxillary.
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ranking = 0.85714285714286
keywords = dysplasia
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