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1/45. Dysembryoplastic neuroepithelial tumor. A case report.

    Dysembryoplastic neuroepithelial tumor (DNT) is a rare, benign tumor encountered in the cortex. It is characterized by the presence of cells of different histogenesis. Due to its mixed nature (glial-neuronal), WHO histological classification of brain tumors included it into the group of neuronal and glial-neuronal mixed tumors. Case of tumor in a 19-year-old woman experiencing for three years seizure of temporal lobe epilepsy is presented. A cranial magnetic resonance imaging (MRI) showed "pseudocystic" tumor in temporal lobe. Histological and immunocytochemical examinations of the tumor fragment removed during surgery revealed large numbers of neuronalglial nodules occurring in the cerebral cortex. Columns of glial-neuronal structures crossing parallely to the cortex surface, surrounded by oligodendrocyte-like cells (OLC) were a characteristic feature of the tumor texture. In the tumor interstitium, "floating" maturated, dysplastic-free ganglionic cells were visible in numerous bright spaces. In addition, numerous lobuliform--structured areas consisted of oligodendrocyte-like cells. Oligodendrocyte-like cells were characterized by positive immunoreaction to the presence of S-100 protein and synaptophysin. Basing on clinical manifestation and histopathological findings dysembryoplastic neuroepithelial tumor was diagnosed.
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2/45. Astroblastoma: ultrastructural observations on a case of high-grade type.

    An astroblastoma of high-grade type arising in the brain of a 3-year-old child is reported. The first description of the ultrastructural, immunohistochemical, and cytogenetic findings in this rare tumor variant are presented.
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3/45. Oligodendrocytic gliomatosis cerebri.

    We describe a very unusual case of gliomatosis cerebri (GC) with oligodendrocytic differentiation. A 65-year-old man presented with a convulsive seizure. Magnetic resonance (MR) documented diffuse enlargement of cerebral hemispheres, brainstem, and right cerebellar hemisphere. After admission, the patient manifested a progressive deterioration of his neurological condition. A right temporal craniotomy and temporal lobectomy were performed to obtain brain decompression and diagnosis. Pathological findings were those of a GC consisting of neoplastic oligodendrocytes. Oligodendrocytic GC is a very rare pathological condition. diagnosis of GC is usually made at autopsy. Our case confirms that diagnosis by a combination of MR imaging and brain biopsy.
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4/45. Dysembryoplastic neuroepithelial tumor: a curable cause of seizures.

    Dysembryoplastic neuroepithelial tumor (DNT) was first characterized in 1988 by Daumas-Duport and Scheithauer [1], who described 39 cases in children with morphologically distinct brain tumors. Partial complex seizures (PCSs), well-defined neuroimages, and typical pathologic findings were characteristic. Surgical resection of the tumor was usually curative. We present a new case of a child with PCSs who was diagnosed and treated for DNT. The clinical presentation and the difficulties of the differential diagnosis are discussed.
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5/45. radiation therapy approach in gliomatosis cerebri--case reports and literature review.

    Gliomatosis cerebri is defined as a remarkably diffuse glioma, characterized by widespread infiltration of the central nervous system. Clinicopathologic characteristics and imaging findings have been published but valid classification remains controversial. Few reports exist regarding therapeutic options in gliomatosis cerebri. Here we review data on 17 patients treated with radiation therapy extracted from the literature, in which we focus our attention on available details of irradiation and clinical outcome and present the results of three additional patients treated at our two institutions. Radiologic-pathologic correlation in gliomatosis cerebri indicates that tumor delineation should be based on T2-weighted MRI. radiation therapy in gliomatosis cerebri is associated with a temporary improvement in or stabilization of clinical symptoms in the majority of cases. Duration of improvement was > or = 6 months in 50% of treated patients. survival from onset of symptoms was 23.8 months (range 8-42). Considerable variation in the natural course of the disease precludes conclusions regarding the impact of radiation therapy on survival.
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keywords = central nervous system, nervous system
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6/45. Leptomeningeal glioblastoma presenting with multiple cranial neuropathies and confusion.

    glioblastoma multiforme (GBM) is the commonest primary malignant neoplasm of the CNS. Usually, patients present with seizures and headache but in the elderly, confusion and generalised cognitive decline are more frequently the initial features. Multiple cranial nerve lesions as a manifestation of leptomeningeal meningitis is a rare presentation of GBM. The diagnosis is not often suggestive on either brain computed tomography (CT) or magnetic resonance imaging (MRI) and is usually confirmed by cerebrospinal fluid (CSF) cytology or histology. We describe the case of an 80-year-old man, who presented with multiple cranial nerve palsies and confusion secondary to leptomeningeal gliomatosis, in whom GBM was detected along the intra-ventricular lining of the left lateral ventricle at ventriculoscopy, in the absence of a distinct parenchymal lesion.
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7/45. Gliomatosis cerebri in a 10-year-old girl masquerading as diffuse encephalomyelitis and spinal cord tumour.

    Gliomatosis cerebri is the unifying term used when diffuse glial infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is difficult because of the vagueness of the physical, radiological and pathological findings. adult cases may simulate an acute diffuse encephalomyelitis and show postmortem evidence of a marked swelling of the spinal cord. Apparently benign intracranial hypertension with papilloedema has also been recorded. We report a 10-year-old girl who presented with a history and physical signs suggestive of benign intracranial hypertension. A diffuse encephalomyelopathy occurred, which was complicated by spinal cord swelling, followed by deterioration and death. Gliomatosis cerebri affecting the brain and spinal cord was found at postmortem examination.
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8/45. Primary multifocal leptomeningeal gliomatosis.

    A 23-year-old female university student was presented with recent onset of non-specific headache and dizziness. She had no neurological deficit on neurological examination and magnetic resonance imaging of the brain revealed diffuse enhancement in the basal cisterns and cerebral sulci. She was treated as tuberculous meningitis but she did not improve and developed respiratory arrest. autopsy showed primary multifocal leptomeningeal gliomatosis.
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9/45. Primary diffuse leptomeningeal gliomatosis.

    Primary diffuse leptomeningeal gliomatosis is a rare central nervous system neoplasm in which focal or diffuse evidence of gliomatous tissue is identified in the subarachnoid space with no evidence of a primary tumour. A case is presented and the differential diagnosis and management are discussed.
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keywords = central nervous system, nervous system
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10/45. Vascular abnormalities in surgical specimens obtained from the resected focus of intractable epilepsy.

    The histopathological features, particularly hypervascularity, were examined in specimens resected from 21 patients, 15 with intractable epilepsy accompanying cortical dysplasia or dysembryoplastic neuroepithelial tumor (DNT), and 6 with benign brain tumors, such as ganglioglioma and low-grade glioma. Hypervascularity was found in resected specimens from 15 of the 21 patients (71.4%) and in 10 of the 12 patients (83.3%) who had double pathology. Counting of numbers of vessels by CD31 immunohistochemistry revealed that hypervascularity was prominent, especially in cases of vascular malformation or cortical dysplasia. However, almost all cases were negative for vascular endothelial growth factor (VEGF) staining, except for some cases of benign brain tumors. Moreover, all cases showed low or no proliferative potential in MIB-1 immunohistochemistry. These results suggest that the etiology of hypervascularity in the dysplastic lesions is one of a variety of cerebral malformations, as is the case with abnormal maturation and differentiation in neuroglial elements.
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