Cases reported "Oligodendroglioma"

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1/7. hypersensitivity reactions to procarbazine with mechlorethamine, vincristine, and procarbazine chemotherapy in the treatment of glioma.

    The authors report the clinical features of hypersensitivity reactions believed to result from procarbazine in eight patients treated with mechlorethamine, vincristine, and procarbazine (MOP) for high-grade glioma. There was one instance of hypersensitivity in 7 patients treated for recurrent disease and seven instances in 16 patients treated with an adjuvant protocol using MOP directly after surgery. Maculopapular rash was seen in seven of eight, fever was seen in four of eight, and reversible abnormal liver function test results were seen in three of four patients. Pulmonary toxic effects were seen in five of eight patients and consisted of isolated interstitial pneumonitis in one, fever and infiltrate after rechallenge with procarbazine after previous rash in two, and cough accompanying rash in two. The toxic effects were mild to moderate in six patients but severe to life threatening in the two who were rechallenged after development of rash. The observed incidence of rash during adjuvant therapy was higher than that previously found by the authors for recurrent disease, and it appears to be higher than has been reported in Hodgkin's disease, lymphoma, and other solid tumors. The findings by the authors suggest that a high index of suspicion be kept for hypersensitivity reactions to procarbazine when treating primary brain tumors and that, contrary to the experience in other settings, procarbazine be stopped if rash develops.
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2/7. Papillary glioneuronal tumor.

    The descriptive term papillary glioneuronal tumor (PGNT) has been repeatedly applied to a morphologic subset of low-grade mixed glial-neuronal neoplasia of juvenile and young adult patients. We report on a 13-year-old boy with PGNT of the left temporal lobe, who presented with headaches and a single generalized seizure. On magnetic resonance imaging, tumor was seen as a large, moderately enhancing paraventricular mass with cyst-mural nodule configuration and slight midline shift. Perifocal edema was virtually absent. Gross total resection could be performed, followed by an uneventful recovery. Histologically, the tumor exhibited similar, if not identical, features as reported previously. These comprised a patterned biphasic mixture of sheets of synaptophysin-expressing small round cells and pseudorosettes of GFAP-positive rudimentary astrocytes along vascular cores. Focally, the latter imprinted a pseudopapillary aspect on this otherwise solid lesion. Both cellular components expressed non-polysialylated neural cell adhesion molecule (NCAM)-L species, and several overlapping areas of synaptophysin and GFAP immunoreactivity were present. The mean MIB-1 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation, or necrosis were consistently lacking. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular neurocytic neoplasia, whose paradigmatic presentation may allow for consideration as an entity.
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3/7. Papillary glioneuronal tumor with minigemistocytic components and increased proliferative activity.

    Papillary glioneuronal tumor (PGNT) is a rare and new type of glioneuronal neoplasm of the central nervous system. It is characterized by pseudopapillary structures composed of hyalinized vessels rimmed by cuboidal glial cells and the proliferation of neuronal cells. We report a peculiar PGNT arising in the parietal lobe of a 67-year-old man, which was characterized by proliferation of minigemistocytic cells as well as typical components. The minigemistocytic cells had eccentric nuclei and plump eosinophilic cytoplasm that was filled with glial filaments. The Ki-67 labeling index was as high as 10% in the minigemistocytic areas. Recently, the presence of oligodendroglial-like component was suggested in PGNT. Considering that oligodendroglioma sometimes accompanies minigemistocytic components, the minigemistocytic cells in PGNT were suggested to be a part of oligodendroglial differentiation. Although PGNT is defined as an indolent glioneuronal tumor, the presence of minigemistocytic components with the high Ki-67 labeling index may indicate more aggressive nature.
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4/7. Use of thallium-201 SPECT to quantitate malignancy grade of gliomas.

    A quantitative preoperative technique using thallium-201 single-photon emission computerized tomography is described which predicts whether specific gliomas are of high- or low-grade malignancy. An index, based on the ratio of thallium uptake in the tumor versus the homologous contralateral brain, was calculated and compared with tumor histology. The index in 14 patients with low-grade malignant gliomas was 1.27 /- 0.40 in contrast to an index of 2.40 /- 0.61 in 11 patients with high-grade malignant gliomas (p less than 0.0005). Whether gliomas were of low- or high-grade malignancy could be predicted with 89% accuracy using a threshold of 1.5. Low-grade gliomas with an index higher than 1.5 acted biologically more like high-grade tumors, and no tumor histologically classified as being of high-grade malignancy had an index lower than 1.7. This technique could help to reduce unrecognized sampling errors during needle biopsies of brain tumors, particularly of high-grade lesions classified in error as low-grade tumors due to inadequate biopsy material.
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5/7. Metastatic oligodendroglioma. Case report.

    We report a case of remote metastasis of oligodendroglioma. Similar cases are reported in the literature for malignant cerebral tumors. Our case seems rather different because of low grade histology. Potential malignancy was evidenced only by further investigations with labeling indexes.
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6/7. Congenital oligodendroglioma: a case report of a 34th-gestational week fetus with immunohistochemical study and review of the literature.

    A case of congenital oligodendroglioma occurring in a 34th-gestational week fetus is reported. The tumor was necrotic, hemorrhagic, and gelatinous. It covered the basal part of the brain, and almost the entire cerebellum was replaced by the tumor. The tumor cells had small, round, hyperchromatic nuclei and watery clear cytoplasm, and were arranged in a paved or alveolar pattern. Immunohistochemically, S100 protein, myelin-basic protein, neuron-specific enolase and Leu 7 were weakly positive for the cytoplasm, but glial fibrilliary acidic protein, synaptophysin, neurofilament, desmin, and vimentin were negative. Many tumor cell nuclei were positive for mutant p53 protein, and the labeling index was 85%. But there was no genetic alteration in exons 4 to 9 of p53 gene from the peripheral blood. The apoptosis index was 1.5%. Considering the p53 labeling index and the apoptosis index together, this congenital oligodendroglioma may be regarded as potentially malignant despite the benign morphological features.
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7/7. oligodendroglioma with signet-ring cell morphology: a case report with an immunohistochemical and ultrastructural study.

    A case of oligodendroglioma with signet-ring cell (SRC) morphology arising in the right thalamic region in a 12-year-old boy is described. Histopathologically, the tumor was a composite neoplasm consisting of typical oligodendroglioma and anaplastic components with aggregates of SRC. Immunohistochemically the SRC were negative for glial fibrillary acidic protein (GFAP) but surrounded by GFAP-positive anaplastic cells with high-grade nuclear features. Typical oligodendrogliomatous components were negative for GFAP. The Ki-67 labeling index evaluated with MIB-1 antibody was 1.3% in the SRC component, 9.2% in the GFAP-positive anaplastic cell component, and 0.8% in the typical oligodendrogliomatous component. Ultrastructurally, the cytoplasm of the SRC was filled with irregularly and widely dilated cisternae of rough endoplasmic reticulum containing granular material. intermediate filaments and a small number of other organelles were distributed in the perinuclear and peripheral areas. Both the SRC and anaplastic cells had slender cytoplasmic processes, although those of the SRC were short and few in number. These findings are distinct from those of SRC hitherto described in oligodendrogliomas to date, and suggest that there is a morphological heterogeneity in SRC rarely seen in oligodendrogliomas and that some examples of SRC are related to the anaplastic cells with astrocytic features in their origin.
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