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1/4. A case of an unclassified tumor closely resembling dysembryoplastic neuroepithelial tumor with rapid growth.

    We describe a rare case of a tumor resembling dysembryoplastic neuroepithelial tumor. A 3-year-old girl had a generalized convulsion as the initial symptom, without other neurological deficits. Computed tomography showed a hypodense lesion with calcific hyperdensity in the left frontal lobe associated with deformity of the overlying calvarium. Four months later, she had a second seizure, and computed tomographic scan showed that the lesion had increased in size. Subtotal removal of the tumor was performed, and the postoperative course was uneventful without radiation therapy. Histological examination revealed a cortical lesion in which an oligodendrocyte-like area and an astrocyte-like area with cytological atypia were observed. Although the clinical course and the radiological findings closely resembled those of dysembryoplastic neuroepithelial tumor, specific glioneuronal elements were not found histologically. Daumas-Duport reported a complex form of dysembryoplastic neuroepithelial tumor that contained glial nodules in addition to a specific glioneuronal element. The histological findings of the glial nodules in this case were quite similar to those she described. We conclude that this could be an unclassified tumor closely resembling dysembryoplastic neuroepithelial tumor without a specific glioneuronal element.
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2/4. Gliomatosis cerebri and pituitary adenoma: case report and literature review.

    The diagnosis of Gliomatosis cerebri (GC) is known to be difficult and is still a matter of debate. We describe an in vivo case of GC associated with a pituitary tumor. A 47-year-old woman presented with short-term memory loss. A MRI revealed the presence of a pituitary enhancing tumor and a diffuse lesion involving the brain. A left pterional craniotomy with partial temporal lobectomy and removal of the pituitary lesion were performed in order to obtain diagnosis. The histological analyses showed a pituitary non-functioning tumor and a GC consisting of neoplastic oligodendrocytes and astrocytes. Both lesions showed nuclear immunoreactivity for progesterone receptors (PGr) and estrogen receptors (EGr). This result could suggest there is a common receptor substrate in these tumors. In this case hormones could constitute a common step in tumorigenesis of both lesions.
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3/4. Identification of phenotypic neural stem cells in a pediatric astroblastoma.

    OBJECT: The goal of this study was to illustrate the findings of a significant subpopulation of cells within a pediatric astroblastoma that have the specific cell surface phenotype found on known human neural stem cells. methods: Cells with a cell surface marker profile characteristic of human neural stem cells were isolated using fluorescence-activated cell sorting from a mostly nonmitotic astroblastoma removed from the brain of an 11-year-old girl. An unusually high proportion (24%) of the cells were CD133 positive and CD24, CD34, and CD45 negative (CD133( )CD24(-)CD34(-)CD45(-) cells), the phenotypic antigenic pattern associated with neural stem cells; very few CD133-positive cells were not also CD24, CD34, and CD45 negative. Some cells (12%) were CD34 positive, indicating the presence within the tumor of hematopoietic stem cells. Cells formed cytospheres that resembled neurospheres when seeded into stem cell media and coexpressed beta-tubulin and glial fibrillary acidic protein (GFAP) but did not express the oligodendrocyte marker O4. cell proliferation was demonstrated by incorporation of bromodeoxyuridine. The cells lost their capacity for self-renewal in vitro after four to six passages, although they continued to coexpress beta-tubulin and GFAP. The cells did not differentiate into neurons or astrocytes when placed in differentiation medium. CONCLUSIONS: Although this astroblastoma contained a high proportion of phenotypic neural stemlike cells, the cells had limited proliferative capacity and multipotency. Their role in astroblastoma formation and growth is unknown.
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4/4. Mixed dysembryoplastic neuroepithelial tumor and ganglioglioma.

    We report a case of a 15-year-old girl with new onset seizures, who had a mixed dysembryoplastic neuroepithelial tumor (DNT) and ganglioglioma of the right parieto-occipital lobe. The tumor appeared well demarcated and exhibited a low T1 and a high T2 signal on magnetic resonance imaging. Architecturally it was in large part intracortical and multinodular, but also featured a leptomeningeal component. The former corresponded to DNT, a proliferation of oligodendroglia-like cells (OLCs) arranged in nodules, as well as comprising a diffuse internodular element featuring "floating neurons" in a mucoid matrix. The leptomeningeal portion of the lesion was a ganglioglioma consisting of large neurons and astrocytes in association with marked desmoplasia. Spatially, the two components abutted one another but appeared distinct. immunohistochemistry showed the neurons of the ganglioglioma to be positive for class III beta-tubulin, synaptophysin, and chromogranin a, whereas the astrocytic cells stained only for glial fibrillary acidic protein. Most OLCs in the DNT were positive for S-100 protein. This apparently mixed lesion suggests that a close histogenetic relationship exists between DNT and ganglioglioma. We postulate that the pluripotential progenitor cells residing in the subpial granular layer may have given rise to the cortical DNT and to the leptomeningeal ganglioglioma. To our knowledge, this is the first detailed histological, immunochemical and ultrastructural report of a mixed DNT and ganglioglioma.
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