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1/9. Metastatic adenocarcinoma masquerading as basal pontine tuberculoma.

    Tuberculous infection of the central nervous system is common in hong kong. A 39-year-old woman presented with isolated right sixth nerve palsy which was non-progressive for 10 months. Neuro-imaging revealed a right pontine lesion. cerebrospinal fluid (CSF) examination showed lymphocytic meningitis with negative bacteriological and cytological studies. Empirical antituberculous drugs with initial corticosteroid resulted in improved CSF parameters. A diagnosis of cerebral tuberculoma complicated by meningitis was made. She subsequently deteriorated clinically and radiologically. Despite a number of clinical features which were atypical of leptomeningeal metastasis, adenosquamous carcinoma was found on biopsy. Her relatively indolent clinical course might be due to the initial corticosteroid treatment. This report illustrates the importance of early tissue diagnosis in uncertain cases of chronic lymphocytic meningitis.
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keywords = central nervous system, nervous system
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2/9. Pulmonary meningioma: false-positive positron emission tomography for malignant pulmonary nodules.

    An intraparenchymal lung tumor exhibiting the histopathologic and immunophenotypic characteristics of an intracranial meningioma is described. The tumor presented as a solitary asymptomatic pulmonary nodule with increased fluorodeoxyglucose uptake on positron emission tomography. After right upper lobectomy, benign psammomatous meningioma was diagnosed. There was no clinical or radiographic evidence of a central nervous system tumor, suggesting that this was a primary lung tumor. The authors describe a benign tumor that can present as a false-positive finding of positron emission tomography performed to evaluate a possible malignant pulmonary nodule.
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ranking = 1
keywords = central nervous system, nervous system
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3/9. Detection of occult CNS involvement of follicular small cleaved lymphoma by the polymerase chain reaction.

    A patient with follicular small cleaved lymphoma presented with an unusual clinical relapse in the central nervous system (CNS) without morphologic evidence of lymphoma cells in the cerebral spinal fluid (CSF). Molecular genetic analysis of the small number of cells in the CSF after in vitro dna amplification by the polymerase chain reaction demonstrated the presence of an abnormal translocation sequence between chromosomes 14 and 18. A similar translocation could be detected from the original fixed archival lymph node biopsy and from a small proportion of circulating mononuclear cells. These results indicated that occult lymphoma cells were present in the CSF and peripheral blood. Secondary CNS lymphoma involvement was identified at autopsy. This case demonstrates the enhanced sensitivity of lymphoma diagnosis from poorly cellular specimens after in vitro dna amplification.
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ranking = 1
keywords = central nervous system, nervous system
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4/9. Disseminated melanomatosis of the central nervous system and other organs: a case report.

    This case represents an example of clinically occult, disseminated neoplastic disease with almost asymptomatic but severe involvement of the central nervous system. The only helpful diagnostic investigation appeared to be cerebrospinal fluid cytology. Extensive post mortem examination was inconclusive as to whether the widespread CNS involvement with melanoma was primary or secondary.
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ranking = 5
keywords = central nervous system, nervous system
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5/9. Disseminated neuroblastoma with initial presentation as an intraoral mass: case report.

    neuroblastoma is an uncommon malignant neoplasm that derives from neural crest progenitor cells that normally give rise to the sympathetic nervous system. It represents 8 to 10% of all extracranial tumors in childhood. The purpose of this paper is to report a case in which a mandibular soft tissue mass was the initial presenting sign of disseminated neuroblastoma in a 2-year-old child, and to discuss the clinicopathologic features and biologic behavior of neuroblastoma.
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ranking = 0.29051694492635
keywords = nervous system
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6/9. Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass.

    Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.
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ranking = 1
keywords = central nervous system, nervous system
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7/9. Widespread neuroendocrine malignancy within the central nervous system: a diagnostic conundrum.

    A 75 year old female presented with a sellar tumour, and was subsequently found also to have a cauda equina tumour, a parietal dural tumour, a pontine tumour, an intradural spinal tumour, and several vertebral body tumours. Histological examination revealed a neuroendocrine tumour forming cell nests surrounded by reticulin. There was moderate nuclear pleomorphism, prominent mitoses, and focal necrosis. immunohistochemistry showed diffuse positive staining with cytokeratins, chromogranin and 5-hydroxytryptamine, and focal positive staining with S100. This case is an unusual and ultimately insoluble, diagnostic problem; however, the differential diagnoses include pituitary carcinoma, malignant paraganglioma, and atypical carcinoid.
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ranking = 4
keywords = central nervous system, nervous system
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8/9. Desmoplastic small round cell tumour of unknown primary origin with lymph node and lung metastases: histological, cytological, ultrastructural, cytogenetic and molecular findings.

    Desmoplastic small round cell tumour (DSRCT) is an extremely aggressive neoplasm belonging to the family of "small round blue cell tumours", which includes primitive neuroectodermal tumour (PNET), Wilms' tumour and Ewing's sarcoma. DSRCT is considered to be a neoplasm derived from a primitive cell. It is immunohistochemically reactive with epithelial, neuronal and mesenchymal cell markers, demonstrating divergent differentiation along three cell lines. Originally thought to arise from serosal surfaces, the tumour has recently been reported in the central nervous system and ovary. The tumour in this case is a neoplasm that meets the histological, immunohistochemical, cytological and cytogenetic criteria of DSRCT; it is not associated with serosal membranes, and it has supraclavicular and axillary lymph node deposits and multiple pulmonary and brain metastases. Tumour cells from our case show cytogenetic similarities with Ewing's sarcoma and PNET. Electron microscopic findings suggest similarities between DSRCT and Wilms' tumour. Cloning and sequencing of PCR products showed in-frame fusion of EWS exon 7 to WT1 exon 8.
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ranking = 1
keywords = central nervous system, nervous system
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9/9. Well-differentiated papillary adenocarcinoma arising in a supratentorial enterogenous cyst: case report.

    OBJECTIVE AND IMPORTANCE: We report a case of a well-differentiated papillary adenocarcinoma arising in an supratentorial enterogenous cyst. The clinicopathological features of this case and a brief review of the literature are presented. CLINICAL PRESENTATION: A 45-year-old woman presented with abrupt onset of sensory seizures and abnormal sensation on the left side of her face, left leg, and left arm. Radiological studies showed a cystic extraaxial tumor with mass effect in the right parietal area. The initial clinical impression was a metastatic lesion, and a comprehensive metastatic workup revealed no evidence of tumor elsewhere. INTERVENTION: A gross total resection of the solid cystic tumor was achieved by a frontoparietal craniotomy. Sixteen months after the initial surgery, the patient presented with signs of increased intracranial pressure and a large parietal cyst. The cyst was fenestrated at the time of the second craniotomy. CONCLUSION: A pathological study of the initial surgical material revealed it to be a well-differentiated papillary adenocarcinoma in association with an enterogenous cyst. The second surgical specimen consisted only of the benign cyst wall. The patient recovered uneventfully from the second surgery and was free of symptoms 6 months postoperatively. The importance of recognizing the rare possibility of malignant progression of a benign enterogenous cyst in the central nervous system is discussed.
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ranking = 1
keywords = central nervous system, nervous system
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