Cases reported "Neurocytoma"

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11/61. Papillary glioneuronal tumor.

    Tumors of mixed glioneuronal type are well recognized in the central nervous system. The most common examples of these lesions include gangliogliomas and dysembryoplastic neuroepithelial tumors. Recently, unusual examples of these lesions have been described, including the papillary glioneuronal tumor. This report describes a histologically similar-appearing lesion arising in the left parieto-occipital lobe of an 18-year-old man who presented with headaches and difficulties with vision. Imaging studies noted a large cystic neoplasm marked by a peripheral rim of enhancement. The patient underwent gross total resection of the tumor, which histologically was marked by a mixture of glial (glial fibrillary acidic protein-positive) and neural (synaptophysin-positive) components. Architecturally, the tumor was notable for a focal pseudopapillary pattern. Papillae were lined by predominantly glial cells, with intervening areas occupied by neurally differentiated cells. Mitotic activity, vascular proliferation, and necrosis were not noted. A MIB-1 labeling index of 1.1% was seen. p53 immunoreactivity was not observed. This report adds further evidence supporting the existence of this unusual mixed glioneuronal tumor of the central nervous system.
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12/61. Central neurocytoma: 2 case reports and review of the literature.

    Central neurocytoma is a rare benign tumor of the central nervous system occuring in young adults and typically located in the ventricles. The tumor is composed of small round cells with neuronal differentiation and has a favourable prognosis. We report two cases of giant central neurocytomas with a triventricular extension in two young women. The first case concerned a 26 years old righthanded woman in whom an intraventricular mass was discovered, after a car accident with head trauma. skull radiography showed an enlargment of the sella turcica. A CT scan performed in order to examine the pituitary gland revealed a voluminous and heterogenous intraventricular tumor with calcification. The second case concerned a 26 years old righthanded woman, presenting with a 4 glasgow coma scale Score preceded by an acute onset of headache with projectile vomiting. A CT scan performed in emergency revealed a voluminous intraventricular mass with significant hydrocephalus. We review the different pathological and topographical patterns of previously published neurocytomas and discuss surgical management, effectiveness of radiation therapy and biological behavior.
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13/61. Cerebellar liponeurocytoma: immunohistochemical and ultrastructural study of a case.

    The clinical, histopathological, and ultrastructural features of a cerebellar liponeurocytoma are reported. The tumor, a 3-cm mass localized in the right cerebellar hemisphere, was resected from a 61-year-old man clinically presenting with symptoms of intracranial hypertension. The lesion was composed of small, in some areas closely packed, medulloblastoma-like cells with prominent areas of lipidization phenomena. Moreover, mitoses, cellular atypia, and numerous vascular structures were focally observed. Glial and neuronal differentiation was immunohistochemically noted (glial fibrillary acidic protein, synaptophysin, neurofilaments, and neuron-specific enolase positivity). The p53 oncoprotein was detected in the majority of neoplastic cells and a moderate proliferation activity, evaluated by Mib-1 antibody, was focally appreciated. Ultrastructural study did not show evident neuritic processes, synapses, or dense core neuroendocrine granules. This cerebellar tumor previously called lipidized medulloblastoma and recently renamed cerebellar liponeurocytoma is considered an adult neoplasm with excellent prognosis histologically presenting prominent lipidized areas and, at the immunohistochemical and ultrastructural level, both glial and neuronal differentiation. The present study provides the first description of a less differentiated and histologically more aggressive form of this unusual tumor.
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14/61. Management of neurocytomas: case report and review of the literature.

    The purpose of this report is to review the available literature on the presentation, pathology, and treatment of central nervous system (CNS) neurocytomas. A case report of an extraventricular neurocytoma is presented along with a comprehensive literature search of patients with a diagnosis of CNS neurocytoma. CNS neurocytomas are rare neoplasms, with fewer than 240 cases reported in the literature. The majority of neurocytomas are found in the ventricular system of the brain. immunohistochemistry is frequently used to help distinguish this tumor from other CNS neoplasms. MIB-1 proliferation index is commonly used in an attempt to predict biologic behavior. Little is known about the management of patients with this tumor, because most reports are from the pathologic literature and contain sparse information regarding clinical management. Neurocytomas are rare CNS tumors with varied biologic behavior. MIB-1 index may help direct adjuvant therapy. An excellent prognosis can be expected if a gross total resection is achieved. Postoperative radiation therapy (RT) may be considered after subtotal resection. Otherwise, RT is an option for medically inoperable or recurrent disease.
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15/61. Central liponeurocytoma.

    The 2000 world health organization has included cerebellar liponeurocytoma in the category of glioneuronal tumors of the central nervous system. Once termed medullocytoma and considered an embryonal tumor, a variant of medulloblastoma, its indolent behavior and morphologic features prompted this nosologic change. Biphasic in appearance, the tumor consists of well-differentiated neurons with the cytology of neurocytes in addition to a population of lipidized cells resembling mature adipose tissue. Such tumors occur in older adults and have a relatively good prognosis. Linking the concept of liponeurocytoma to its occurrence in the cerebellum unnecessarily obscures the existence of similar neoplasms at other sites, such as among classic central neurocytomas of the lateral and third ventricles. Indeed, two such cases have briefly been reported. To these, we add a third example, the first to be ultrastructurally examined. Our case provides evidence that the lipid vacuoles progressively accumulate and coalesce within cells retaining neurocytic features. Thus, these distinctive lesions are the result of tumoral lipidization, rather than true adipose metaplasia.
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16/61. Proton magnetic resonance spectroscopy of neurocytoma outside the ventricular region--case report and review of the literature.

    Central neurocytoma is classically considered as an intraventricular benign tumour, largely based on data from small retrospective series and single case reports. We report on a 16-year-old girl who suffered from a large parietooccipital tumour that was diagnosed histologically as central neurocytoma. The features of CT, MRI and proton MR spectroscopy studies are discussed. This is the first report on spectroscopic findings in a case of extraventricular neurocytoma. As well as elevated choline (Cho), the tumour spectrum showed strongly decreased N-acetylaspartate (NAA). NAA is assumed to be produced in mature neurons, and we therefore expected to find high amounts of NAA in this well-differentiated tumour, which was histologically composed of mature neuronal tissue. This observation leads to the conclusion that even the highly differentiated cells of neurocytomas are too immature to produce NAA.
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17/61. Cerebellar liponeurocytoma. Case report with considerations on prognosis and management.

    BACKGROUND: Cerebellar liponeurocytoma has only recently been recognized as a distinct clinicopathological entity. Although the few cases described in literature support the relatively benign nature of this lesion, optimum treatment strategy and long term behaviour still have to be defined. CLINICAL PRESENTATION: This 61-year-old man came under our observation with unspecific signs of intracranial hypertension and a symptomathology revealing a probable posterior fossa lesion. Imaging studies showed a cerebellar mass lesion with prevalent adipose content. INTERVENTION: Gross total tumour removal was performed. The pathological examination revealed a lesion composed of medulloblastoma-like cells with prominent areas of lipidisation. A review of literature has been made in an attempt to investigate on recurrence patterns, importance of the extent of removal and the usefulness of radiotherapy. CONCLUSION: The few cases reported in literature so far seem to confirm the relatively benign nature of cerebellar liponeurocytoma. No radiotherapy was given in our case and the reasons for this decision are reported.
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18/61. Oligodendrogliomas with neurocytic differentiation. A report of 4 cases with diagnostic and histogenetic implications.

    oligodendroglioma represents a distinct type of diffuse glioma with a relatively favorable prognosis. Although an O2A-like glial progenitor cell of origin has been suggested, a neuronal-oligodendroglial progenitor cell is also of interest, particularly because variable degrees of neuronal marker expression have been reported in typical oligodendrogliomas. We present 2 female and 2 male patients (ages 34-54) with frontal lobe oligodendrogliomas containing a) morphologically distinct collections of small round cells with hyperchromatic nuclei, b) well-formed Homer Wright-like and perivascular rosettes, and c) demonstrable neuronal differentiation by immunohistochemistry and/or electron microscopy in the rosette-associated regions. Unlike extraventricular neurocytomas, these cases featured an infiltrative growth pattern and a classic oligodendroglioma immunophenotype in non-rosette bearing portions of each tumor. FISH analysis demonstrated chromosome 1p and 19q codeletions in 3 (75%) cases, both in regions with and without rosettes. Recurrences were common, although all patients are currently alive 4 months to 13 yr from initial diagnosis. Based on clinicopathologic and genetic features, we diagnosed these tumors as oligodendrogliomas with neurocytic differentiation. However, it is unclear whether they represent a) gliomas with divergent neuronal differentiation, b) a distinctive form of glioneuronal neoplasm, or c) a reflection of glioneuronal histogenesis in oligodendrogliomas in general. In any case, their occurrence suggests a histogenetic overlap between oligodendroglioma and extraventricular neurocytoma not previously recognized.
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19/61. Extraventricular neurocytoma: morphological and immunohistochemical considerations on differential diagnosis.

    neurocytoma is an unusual neuronal tumor especially affecting young people. It commonly arises in the ventricles and has a benign outcome. Herein, we report on a rare case of extraventricular neurocytomas (right parietal lobe) in a young girl admitted to hospital for a cranial trauma subsequent to a seizure. The tumor, radiologically well-circumscribed, cystic and enhancing, was surgically excised. The patient, who received no post surgical treatment, is alive and well after 18 months of follow-up. Pathological examination showed a well-differentiated lesion composed of uniform, round cells with perinuclear halos in a neuropil background and immunohistochemically positive for neuronal markers (synaptophysin, neuron-specific enolase, neurofilaments). The authors emphasize the role of the morphological and immunohistochemical evaluations to recognize this rare tumor.
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20/61. Cerebellar central liponeurocytoma.

    Cerebellar liponeurocytoma is a rare, benign neuroepithelial tumor that occurs exclusively in the cerebellum of adults. Its salient histological features include advanced neuronal/neurocytic differentiation, focal vacuolated cells resembling mature adipose cells, low mitotic activity, and lack of endothelial proliferation and/or necrosis. The morphological appearance of this neoplasm can be confused with that of oligodendroglioma, neurocytoma, ependymoma, medulloblastoma, hemangioblastoma, metastatic renal cell carcinoma, and other clear cell carcinomas. Its full biological potential and histological features, however, have not been fully exploited due to the rarity of this tumor. The authors describe a case with clinical, imaging, histological, immunohistochemical, and ultrastructural features.
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