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1/7. 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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2/7. Astroblastoma with unusual signet-ring-like cell components: a case report and literature review.

    We report a case of astroblastoma with unusual signet-ring-like cell components. A 33-year-old-woman presented with occasional partial seizures of the face. Radiological studies revealed an enhanced frontal mass lesion. At surgery, a gray, soft, well-circumscribed mass was seen and shelled out. Histologically, the tumor showed a perivascular arrangement and papillary-like patterns with compact cellularity. The tumor cells radiating from the hyalinized vessels showed broader, shorter, less tapered processes. A part of each tumor cell displayed prominent islands of signet-ring-like cells. glial fibrillary acidic protein reaction revealed strongly positive staining of tumor cells and signet-ring-like cells. Eight years after the operation the patient remains well with no tumor recurrence. It remains to be determined whether, in this astroblastoma, the unusual signet-ring-like cell components were related to benign biological characteristics or to the tumor's low-grade form with incidental signet-ring-like cell appearance.
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3/7. Papillary glioneuronal tumor--a new variant of benign mixed brain neoplasm.

    We report a case of a 14-year-old girl with papillary glioneuronal tumor (PGNT) in right parietal lobe. On MRI the tumor presented as a contrast enhancing mass with small central hypodense area and consisted of areas similar to central neurocytoma intermixed with vessels surrounded with glial cells. There were also small loose areas superficially reminding DNT. Neurocytic component presented strong synaptophysin immunostaining, while intermixed glial element presented GFAP-immunopositivity. Our case is similar to previously reported PGNT, but an important difference lies in not distinct cystic formation and a presence of loose, edematous tissue. PGNT may be regarded as a variant of ganglioglioma or as a complex variant of extraventricular neurocytoma and belongs to a wide group of benign, dysembryoplastic or even hamartomatous neuroepithelial tumors, which may differentiate into both, glial or neuronal direction: pleomorphic astrocytoma may posses neurocytic differentiation, desmoplastic infantile ganglioglioma may be regarded as complex superficial dural astrocytoma and DNT may present gliomatous areas (complex DNT). Also other rare tumors reminding DNT have been reported: DNT-like neoplasm of septum pellucidum and rosetted glioneuronal tumor. From this point of view it is important to remember that such a wide spectrum may be difficult to discriminate into very narrow clinico-pathological entities.
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4/7. Gliomatosis cerebri: quantitative proof of vessel recruitment by cooptation instead of angiogenesis.

    OBJECT: Gliomas are the most common primary brain tumors, many of which (especially astrocytic and oligodendroglial neoplasms) are characterized by diffuse infiltrative growth in the preexisting brain tissue. Gliomatosis cerebri is a rare glial tumor and represents an extreme example of such diffuse infiltrative growth. This growth pattern not only hampers curative treatment but also allows for vessel cooptation rather than tumor angiogenesis as a way of vessel recruitment by the tumor tissue. The goal of this study was to establish the extent to which tumor angiogenesis occurs in gliomatosis cerebri. methods: Computerized image analysis was performed to assess quantitatively two microvascular parameters (vessel density and diameter) in different areas of a brain harboring a gliomatosis cerebri. These regions were the cerebral white and gray matter in which there was a diffuse infiltrative tumor, cerebral white and gray matter in which there was a more compact growth pattern of tumor cells, and normal cerebral white and gray matter. In addition, the authors performed immunohistochemical stainings for blood-brain barrier (BBB) characteristics (Glut-1 and PgP) on samples obtained in these different areas. The results of the quantitative analysis strongly indicated that in gliomatosis cerebri tumor, angiogenesis was completely absent, a finding that is corroborated by the fact that the microvasculature in gliomatosis cerebri persists in exhibiting immunohistochemical characteristics of the BBB. CONCLUSIONS: The results of this study may help resolve the difficulties in radiological detection and delineation of the diffuse infiltrative part of glial brain tumors and put the expectations for antiangiogenic treatment of such tumors into perspective.
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5/7. Gliomatosis cerebri angiographically mimicking central nervous system angiitis: case report.

    OBJECTIVE: The diagnosis of isolated central nervous system angiitis often requires both cerebral angiography and biopsy for diagnosis. CLINICAL PRESENTATION: We present a case of gliomatosis cerebri with demonstrated angiographic changes suggestive of a central nervous system vasculitis. INTERVENTION: A 47-year-old woman presented with a 2-week history of worsening headache and progressive drowsiness. magnetic resonance imaging scans revealed confluent nonenhancing T2-weighted hyperintensity involving the frontal white matter bilaterally, hypothalamus, and both thalami. A four-vessel angiogram revealed diffuse arterial beading with stenoses of a right frontal middle cerebral artery branch and the distal posterior cerebral artery and anterior cerebral artery branches, bilaterally. The biopsy revealed a Grade 2 fibrillary astrocytoma consistent with gliomatosis cerebri. CONCLUSION: Gliomatosis cerebri can mimic central nervous system vasculitis angiographically. Perivascular infiltration by tumor cells may be responsible for these changes.
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6/7. Low-grade astroblastoma recurring with extensive invasion.

    A 17-year-old male presented with morning headache and double vision. neuroimaging revealed a lobulated enhanced mass lesion with a blurred margin and remarkable peritumoral edema, and high uptake of methionine. The gray, soft, well-circumscribed mass was grossly totally resected. Histological examination showed the tumor cells were well differentiated with the perivascular pseudorosette pattern with broad, non-tapering processes radiating towards a central vessel without anaplastic features such as necrosis and endothelial proliferation. The histological diagnosis was low-grade astroblastoma. Follow-up magnetic resonance imaging demonstrated local recurrence 5 months later. Second surgery was followed by adjuvant radiotherapy and combination chemotherapy. Histological examination disclosed wide invasion by tumor cells into the subpial and perivascular space of the surrounding brain tissue. Follow-up magnetic resonance imaging demonstrated further recurrence around the tumor cavity. Surgical removal followed by six courses of combination chemotherapy (ifosfamide, cisplatin, and etoposide) resulted in complete remission of the tumor. Although gross total resection of astroblastoma usually results in long-term survival, some of these yet unfamiliar tumors may develop a more malignant character.
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7/7. Dysembryoplastic neuroepithelial tumour of the cerebellum.

    A case of dysembryoplastic neuroepithelial tumour of the cerebellum occurring in a 28-year-old woman is presented. The lesion extended from the cortex of the inferior vermis upwards into the white matter. Histologically, it exhibited areas of microcystic cerebellar astrocytoma and glial regions with hamartomatous blood vessels as well as areas with oligodendrocyte-like cells (OLC) with a delicate, fibrillary stroma lying in a mucinous, often microcystic matrix. The OLC showed prominent rosette formation and immunohistochemical features suggesting neuronal, i.e. granule cell, differentiation.
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