Cases reported "Oligodendroglioma"

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1/6. 45-year-old male with symptomatic mass in the frontal lobe.

    A 45-year-old male patient developed focal seizures in his right arm. neuroimaging demonstrated a tumor of the left frontal lobe. Tumor classification was undecided after stereotactic biopsy. Neuropathological examination of the open biopsy specimen revealed overlapping morphological features of an oligodendroglioma and a central neurocytoma. Groups of tumor cell featured the typical "fried egg" appearance seen in oligodendroglioma; microcalcifications and a network of branching non-proliferating vessels were present. neurocytoma-like features included small nucleus-free areas of neuropil and perivascular pseudorosettes. Neuron specific enolase was strongly expressed cytoplasmically in the tumor cells and the "neuropil islands" were found to express synaptophysin. The final diagnosis of an oligodendroglioma with neurocytic differentiation was based on tumor location, clinicopathological findings and diagnostic genotyping. Combined loss of heterozygosity (LOH) on the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q), the "molecular signature" of oligodendrogliomas, was revealed. Besides supporting the diagnosis of an oligodendroglioma, the molecular data allow for additional therapeutic options. These tumors may point to the presence of yet another potential tumor precursor cell similar to the recently discovered "N-O"-cells in the cerebral cortex of rats, capable of differentiation into neurons and oligodendrocytes.
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2/6. 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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3/6. Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report.

    The authors report the case of a 2-year-old boy with a primary, diffuse leptomeningeal oligodendroglioma in which the deletion of chromosome arm lp was identified by performing a fluorescence in situ hybridization (FISH) analysis. This previously healthy child initially presented with malaise, anorexia, nausea, vomiting, and macrocephaly. Imaging studies confirmed the presence of hydrocephalus, and a ventriculoperitoneal shunt was placed. The postoperative course was complicated by emesis, continued weight loss, and numerous seizurelike episodes. A contrast-enhanced magnetic resonance imaging study performed approximately 10 weeks postoperatively showed diffuse leptomeningeal thickening and enhancement without evidence of an intraparenchymal mass lesion. A right frontal lobe brain biopsy revealed a hypercellular proliferation of small oligodendroglioma-like cells, which occupied the leptomeninges diffusely and spared the underlying cortical gray matter. The tumor cells displayed prominent perinuclear clearing and had evenly spaced, uniformly round nuclei. Occasional mitotic figures were observed. Background vessels were thin and delicate, and there was no evidence of necrosis. The tumor cells showed strong immunoreactivity for S100 protein; the results of immunohistochemical staining were negative for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, NeuN, and synaptophysin. The deletion of lp was demonstrated by FISH analysis; lq, 19p, and 19q were intact. This appears to be the first reported case of a primary diffuse leptomeningeal oligodendroglioma in which a lp deletion was identified.
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4/6. oligodendroglioma-like cells (clear cells) in ependymoma.

    A brain tumor of a 22-year-old man was composed mostly of round cells with perinuclear halos (clear cells), forming clusters intersected by small blood vessels. In some areas, the tumor cells showed perivascular arrangement and epithelial pattern. Phosphotungstic-acid hematoxylin stain and immunoperoxidase stain for glial fibrillary acidic protein (GFAP) technique failed to stain the clear cells. Electron microscopy of the clear cells revealed them to be classical ependymoma cells with well developed intercellular junctions, microvilli and cilia. As no reporters in the past showed the evidence to clarify the nature of the clear cells, this case is considered a good example to support the viewpoint that the clear cells (oligodendroglioma-like cells) commonly observed in ependymomas are in reality ependymoma cells. It is stressed that the diagnosis of "mixed glioma" or "oligoependymoma" should be made with sufficient caution despite the recent advances of GFAP technique.
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5/6. Extraventricular neoplasms with neurocytoma features. A clinicopathological study of 11 cases.

    The clinicopathological features of a series of neuronal and mixed neuronal and astrocytic neoplasms of the CNS are described. patients were aged 5 to 63 years. Six cases were composed predominantly of small round cells with clear cytoplasm resembling central neurocytoma but lacked the characteristic intraventricular location of that tumor. The remaining five cases had similar neurocytomatous features associated with a benign astrocytic component. Ganglion cells and hyalinization vessels were observed in both groups. The growth fraction evaluated with monoclonal antibody Ki67Mib1 was low, ranging from 1 to 1.5%. Immunohistochemical detection of synaptophysin played a crucial role in identifying the neuronal nature of these neoplasms and was instrumental in distinguishing them from oligodendrogliomas, with which they are readily confused. The neuronal nature of the oligodendroglial-like cells was confirmed ultrastructurally in one case. The present cases, together with others reported previously, suggest that neoplasms of the CNS with "neurocytic" components are more frequent than generally assumed and expand the morphologic spectrum of neuronal and mixed neuronal-glial tumors. Except for one patient who died postoperatively, all patients were alive at follow-up ranging from 6 to 80 months.
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6/6. sarcoma arising in oligodendroglioma of the brain.

    A case is reported of a tumor composed of both oligodendrogliomatous and sarcomatous elements. The interpretation is offered that the sarcoma arose secondarily by neoplastic transformation of the hyperplastic blood vessels formed in response to the presence of the oligodendroglioma. This tumor may be considered analogous to the astrocytoma-sarcoma, which is much more common, and to the metastatic carcinoma with secondary sarcoma, of which one case has been reported.
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