Cases reported "Neurofibromatosis 2"

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11/36. Malignant rhabdoid meningioma arising in the setting of preexisting ganglioglioma: a diagnosis supported by fluorescence in situ hybridization. Case report.

    A highly malignant brain neoplasm with rhabdoid morphological features emerged in the bed of a subtotally resected ganglioglioma in a 54-year-old retired nuclear submarine officer. A combined application of neuroimaging, immunohistochemical studies, electron microscopy, and fluorescence in situ hybridization (FISH) was used to establish the morphological identity of the tumor. The rhabdoid appearance of the tumor cells indicated either an especially malignant variant of rhabdoid meningioma or an atypical teratoid/rhabdoid tumor with an unusually late onset. Whereas immunohistochemical studies and electron microscopy could only be used to narrow down the differential diagnosis, FISH revealed loss of one copy of NF2 with preservation of the INI1 region on 22q, thus establishing the identity of the tumor.
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ranking = 1
keywords = meningioma
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12/36. meningioma showing VHL gene inactivation in a patient with von hippel-lindau disease.

    The genetic mechanism of the tumorigenesis of meningioma in conjunction with von Hippel-Lindau (VHL) disease is unclear. The authors present a case of VHL disease associated with a posterior fossa meningioma and with multiple cerebellar hemangioblastomas. A germline mutation of the VHL gene and loss of heterozygosity on the VHL gene locus in 3p were detected in the meningioma. Tumorigenesis of a meningioma associated with VHL disease could be caused by inactivation of both alleles of the VHL gene.
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ranking = 0.8
keywords = meningioma
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13/36. Bilateral optic nerve sheath meningiomas in a patient with neurofibromatosis type 2.

    A 34-year-old woman who presented with hearing loss and tinnitus was found to have reduced vision bilaterally. Computed tomography scan revealed bilateral acoustic neuromas and bilateral optic nerve sheath meningiomas. The presence of bilateral acoustic neuromas fulfils the criteria for the diagnosis of central neurofibromatosis (neurofibromatosis type 2). Although this is the first report of bilateral optic nerve sheath meningioma in neurofibromatosis type 2, meningiomas are commoner in this dominantly inherited disorder, than in its absence and both forms of central nervous system tumour may be caused by loss of tumour suppressor genes on chromosome 22.
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ranking = 1.6889502804173
keywords = meningioma, meningiomas
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14/36. June 2003: 33-year-old male with a frontal lobe mass.

    The June 2003 COM. A 33-year-old male with a history of seizures was found to have an intra- and extra-axial frontal lobe mass. The histology of the resected tumor showed a meningioma with underlying meningioangiomatosis. Meningioangiomatosis (MA) is a benign intracortical plaque-like proliferation of meningothelial cells, microvasculature and fibroblast-like cells probably of hamartomatous origin. Very rarely, MA is associated with an overlying meningioma as in this case. When MA is accompanied by a meningioma, it is generally not associated with clinical evidence of neurofibromatosis. It is important to distinguish MA from an invasive meningioma, because of its favorable prognosis after resection.
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ranking = 0.8
keywords = meningioma
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15/36. Retroperitoneal neurofibrosarcoma in a patient with neurofibromatosis. 2: A case report and review of the literature.

    Neurofibromatosis (NF) type I (NF1) is the most common familial cancer-predisposing syndrome in humans, while type 2 (NF2) accounts for an extremely small percentage of the total cases of NF. Tumors occurring in patients with NF1 are primarily peripheral neurofibromas, while NF2 patients present with central schwannomas. Malignant transformation has been described in NF1 patients; however, in NF2 the risk of malignant transformation is extremely rare. In this case report, the authors document a retroperitoneal neurogenic sarcoma occurring in a 20-year-old woman with NF2 (bilateral acoustic schwannomas, meningioma, and multiple intraspinal tumors).
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ranking = 0.2
keywords = meningioma
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16/36. spinal cord schwannomas mimicking drop metastases in a patient with intramedullary ependymoma and neurofibromatosis 2.

    The authors describe a 9-year-old boy with a large spinal cord ependymoma whose apparent drop metastases on imaging studies were later demonstrated to be schwannomas. This finding led to the discovery of small bilateral acoustic neuromas and an intracranial meningioma, establishing the diagnosis of neurofibromatosis 2. The presence of additional radiographic abnormalities in a patient with a spinal cord tumor should prompt careful consideration of the diagnosis of neurofibromatosis 2, as early identification of this disorder may significantly affect patient management.
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ranking = 0.2
keywords = meningioma
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17/36. Neurofibromatosis type 2 with multiple plexiform schwannomas.

    Multiple plexiform schwannomas (PS) or neurilemmomas are very rare nerve sheath tumors, showing multiple schwannomas of the skin and other soft tissues, brain, or spinal cord with histopathologic characteristic of interwoven Antonio-A type tissues(1) but without other signs of neurofibromatosis type 2 (NF2). Neurofibromatosis type 2 is much less common than neurofibromatosis type 1 (NF1), with a symptomatic prevalence of 1 in 210,000,(2) and, to the authors' knowledge, only six cases of NF2 with multiple plexiform schwannomas have been reported in the literature.(3-5) We report a rare case of multiple cutaneous plexiform schwannomas associated with characteristic features of NF2 including bilateral acoustic neurilemomas and intracranial meningioma.
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ranking = 0.2
keywords = meningioma
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18/36. Cervical dumbbell meningioma and thoracic dumbbell schwannoma in a patient with neurofibromatosis.

    The occurrence of both dumbbell meningioma and dumbbell schwannoma in one patient has not been reported in the literature. We present a 16-year-old female patient, who had progressive bilateral hearing impairment for 5 years and a progressively enlarged, non-tender neck mass for 1.5 years. Mild motor weakness over her right upper limb was noted 1 week before admission. No cafe-au-lait spot was noted. magnetic resonance imaging (MRI) revealed bilateral cerebellopontine angle tumors, a C1-2 dumbbell tumor, and a T5-6 dumbbell tumor. Neurofibromatosis type 2 was diagnosed. The cervical spine and thoracic spine tumors were removed via one-staged combined posterior (laminectomy) and antero-lateral (transforaminal or thoracoscopic) approaches during two operations performed 1 month apart. The pathology revealed meningioma and schwannoma, respectively. The patient had good recovery after these two operations and her motor function improved. Six months after the second surgery, radiosurgery was performed for the bilateral acoustic tumors, because of enlarged tumor size on follow-up MRI. To the best of our knowledge, this is the first case reported in the literature of a patient, having both dumbbell meningioma and dumbbell schwannoma. A literature review of the dumbbell tumors was done, and their treatment strategies were discussed.
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ranking = 1.4
keywords = meningioma
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19/36. Pneumosinus dilatans and orbital meningioma in neurofibromatosis type 2.

    We report on a 9-year-old boy with neurofibromatosis type 2 and a progressive proptosis of the left eye. Imaging studies revealed a pneumosinus dilatans of the left ethmoidal and frontal sinuses and an adjacent left orbital mass. Incision biopsy of the orbital mass showed a meningiothelial meningioma. Orbital and intracranial meningiomas are frequently associated with pneumosinus dilatans. This report illustrates the relationship of pneumosinus dilatans, intra-orbital meningioma and neurofibromatosis type 2.
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ranking = 1.4481583800696
keywords = meningioma, meningiomas
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20/36. Islets of meningioma in an acoustic schwannoma in a patient with neurofibromatosis-2: pathology and magnetic resonance imaging findings.

    Mixed tumors of the cerebellopontine angle, composed of meningioma and schwannoma components, are extremely rare; so far, only 12 cases have been reported in the literature. They are thought to be exclusively associated with neurofibromatosis-2. We present a mixed tumor of schwannoma and meningioma in a patient with neurofibromatosis-2 and discuss the pathology and magnetic resonance imaging (MRI) findings in relation to the literature. review of the literature shows that a typical MRI pattern has not been established for mixed tumors and it seems unlikely that a meningioma component can be differentiated within a schwannoma preoperatively.
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ranking = 1.4
keywords = meningioma
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