1/42. Composite ganglioglioma and dysembryoplastic neuroepithelial tumor.Both ganglioglioma and dysembryoplastic neuroepithelial tumors are well-recognized glial-neuronal neoplasms associated with chronic epilepsy and cortical dysplasia (neuronal migration abnormalities). The exact relationship between these 2 glial-neuronal tumors continues to be debated. This article reports a case of a composite ganglioglioma and dysembryoplastic neuroepithelial tumor occurring in a 36-year-old woman in the left temporal lobe region. The resection histologically demonstrated distinct areas of ganglioglioma and dysembryoplastic neuroepithelial tumor. A focal area of cortical dysplasia is also identified. The MIB-1 labeling indexes in both components were low (<1% of tumor cell nuclei). The coexistence of these 2 lesions and cortical dysplasia suggest a possible etiologic relationship between these 2 tumors.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/42. Malignant supratentorial ganglioglioma (ganglion cell-giant cell glioblastoma): a case report and review of the literature.BACKGROUND: From both epidemiologic and pathologic viewpoints, gangliogliomas exhibiting components of giant cell glioblastomas are extraordinary neoplasms. We report herein the case of a 6-year-old girl who presented initially with a World health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell tumor-giant cell glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period. methods: Formalin-fixed, paraffin-embedded tissue blocks were sectioned at 5 microm for histochemical and immunohistochemical analyses. hematoxylin-eosin-stained sections and immunohistochemically stained sections from the primary and secondary resections were reviewed. Reactivity for glial fibrillary acidic protein, neurofilament protein, synaptophysin, and Ki67 nuclear antigen was evaluated. RESULTS: Histologically, 2 distinct cell populations were noted on both the primary and secondary resections. The primary resection revealed a neoplasm having a predominant glial component consistent with a glioblastoma. Interspersed were dysmorphic ganglion cells supporting a diagnosis of ganglioglioma. The second resection (following therapy) demonstrated a much more prominent dysmorphic ganglion cell component and a subdued glial component. CONCLUSION: Although immunohistochemical analysis clearly distinguished the 2 tumor cell populations, the identification of Nissl substance in neurons proved to be equally helpful. Although other cases of grade III gangliogliomas and rare cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the only report of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histologic findings in a giant cell glioblastoma and a ganglioglioma and documents the aggressive biologic behavior of this complex neoplasm.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
3/42. A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases.Four examples of a novel glioneuronal neoplasm are presented. All tumors affected adults (including two males and two females aged 25-40 years) as supratentorial, cerebral hemispheric masses with associated seizure activity and, in one case, symptoms of raised intracranial pressure and progressive hemiparesis. CT scans in two cases revealed hypodense masses without calcification. MRI scans at presentation demonstrated, in all cases, solid T1-hypointense and T2-hyperintense tumors with mass effect in one instance but no edema or contrast enhancement. Only one was relatively circumscribed on neuroradiologic study. All were infiltrative in their histologic growth pattern and predominantly glial in appearance, being composed mainly of fibrillary, gemistocytic, or protoplasmic astroglial elements of WHO grade II to III. Their distinguishing feature was their content of sharply delimited, neuropil-like islands of intense synaptophysin reactivity inhabited and rimmed in rosetted fashion by cells demonstrating strong nuclear immunolabeling for the neuronal antigens NeuN and Hu. These cells included small, oligodendrocyte-like ("neurocytic") elements as well as larger, more pleomorphic forms. Two cases contained, in addition, well-differentiated neurons of medium to ganglion-cell size. Proliferative activity was observed principally within the glial compartment; two cases contained mitotic figures and exhibited relatively elevated MIB-1 indices (6.8% and 8.2%). One of the latter progressed and proved fatal at 30 months following subtotal resection and radiotherapy. The three other patients are alive at intervals of 14 to 83 months, two tumor-free and one with extensive disease associated with the appearance of enhancement on MRI. This glioneuronal tumor variant may pursue an unfavorable clinical course.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/42. Orbital ganglioglioma arising from ectopic neural tissue.PURPOSE: To report a case of neonatal orbital ganglioglioma originating from ectopic neural tissue. METHOD: Case report. RESULTS: An African-American male presented at birth with proptosis and expansion of the left orbit. A discrete soft-tissue mass was located inferolateral to the globe, which proved to be a ganglioglioma. CONCLUSION: The tumor is presumed to have originated from ectopic neural tissue in the orbit. Although infrequent, this tumor should be included in the differential diagnosis of neonatal orbital neoplasms.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/42. Combined pleomorphic xanthoastrocytoma-ganglioglioma of the cerebellum.Combined pleomorphic xanthoastrocytoma-ganglioma is a rare neoplasm, occurring in patients younger than 30 years. The clinical course of these tumors is difficult to predict because of their rarity. We report a case of combined pleomorphic xanthoastrocytoma-ganglioma that, in addition to the patient's age, is unusual in several respects. The lesion was located in the cerebellar vermis of a 60-year-old man and was radiographically solid. Histologically, there was an admixture of markedly pleomorphic astrocytic cells and neoplastic ganglion cells, with permeation of the overlying leptomeninges and surrounding cerebellum. In addition, there was focal capillary endothelial proliferation. There was no necrosis, and mitotic activity was rare at 1 mitotic figure per 40 high-power fields. The patient underwent a near gross total resection and postoperative radiotherapy and remains well through 16 months of follow-up.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/42. Papillary glioneuronal tumor.Tumors of mixed glioneuronal type are well recognized in the central nervous system. The most common examples of these lesions include gangliogliomas and dysembryoplastic neuroepithelial tumors. Recently, unusual examples of these lesions have been described, including the papillary glioneuronal tumor. This report describes a histologically similar-appearing lesion arising in the left parieto-occipital lobe of an 18-year-old man who presented with headaches and difficulties with vision. Imaging studies noted a large cystic neoplasm marked by a peripheral rim of enhancement. The patient underwent gross total resection of the tumor, which histologically was marked by a mixture of glial (glial fibrillary acidic protein-positive) and neural (synaptophysin-positive) components. Architecturally, the tumor was notable for a focal pseudopapillary pattern. Papillae were lined by predominantly glial cells, with intervening areas occupied by neurally differentiated cells. Mitotic activity, vascular proliferation, and necrosis were not noted. A MIB-1 labeling index of 1.1% was seen. p53 immunoreactivity was not observed. This report adds further evidence supporting the existence of this unusual mixed glioneuronal tumor of the central nervous system.- - - - - - - - - - ranking = 5.9507646568551keywords = central nervous system, nervous system, neoplasm (Clic here for more details about this article) |
7/42. ganglioglioma of the lateral ventricle presenting with blepharospasm - case report and review of the literature.Gangliogliomas of the lateral ventricle are unusual tumours of the central nervous system. We report a 25-year-old female who presented with a 4-week history of generalized headaches associated with left-sided blepharospasm. Clinical examination revealed bilateral papilloedema but no focal neurological deficits. Computed tomography and magnetic resonance imaging demonstrated a large lesion within the left lateral ventricle. Gross total surgical resection of the lesion was performed through a para-sagittal, trans-callosal approach. Histopathological analysis revealed the diagnosis of ganglioglioma. The post-operative course was uneventful, with complete resolution of both the headaches and blepharospasm. The presentation with an ipsilateral blepharospasm has never previously been reported in the setting of a lateral ventricular tumour.- - - - - - - - - - ranking = 3.9429554762294keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/42. Mixed conventional and desmoplastic infantile ganglioglioma: an autopsied case with 6-year follow-up.We describe a case of desmoplastic infantile ganglioglioma (DIG) arising in the ventral diencephalon of a 3-1/2-month-old boy. On biopsy, the tumor featured a desmoplastic, S-100 protein and GFAP immunoreactive stromal element, as well as a variable spectrum of ganglion cells. Electron microscopy demonstrated astrocytes, and morphologically fibroblasts, as well as neurons containing 120-nm dense core granules. In addition, tubular structures composed of tightly apposed cells with features of astrocytes and of Schwann-like cells were also noted. Devoid of fibroblasts, the tubular structures were surrounded by a single basal lamina. At autopsy 6 years later, the multinodular, cystic mass had replaced the diencephalon, extended into both temporal lobes as well as the optic nerves, and showed marked leptomeningeal involvement. Microscopically, superficial portions of the tumor consisted of typical DIG, whereas deep, nondesmoplastic portions exhibited pattern variation ranging from pilocytic astrocytoma to ganglioglioma and gangliocytoma. There was also a minor element of small, 'primitive-appearing' neuroepithelial cells. Dysplastic ganglion cells variously reactive for neurofilament protein and synaptophysin were present throughout the tumor. Our study not only confirms DIG as a variant of ganglioglioma, one capable of slow growth, infiltration, and fatal progression but suggests that its differentiating potential includes elements of both the central and peripheral nervous systems. If so, their derivation may be from multipotential cells of the neural plate.- - - - - - - - - - ranking = 1.0078091806257keywords = nervous system (Clic here for more details about this article) |
9/42. ganglioglioma causing acute uncal herniation.Gangliogliomas are rare but mostly benign tumors of the central nervous system. Early surgical resection is recommended to prevent seizures and further growth. Therefore, an uncal herniation caused by a ganglioglioma is a very rare event. We report such a case, where a patient with an initially small lesion was lost to follow-up.- - - - - - - - - - ranking = 3.9429554762294keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/42. genome-wide survey for chromosomal imbalances in ganglioglioma using comparative genomic hybridization.ganglioglioma is a mixed neuronal and glial tumor first described by Perkin in 1926. Because of its rare occurrence in the central nervous system, the pathogenesis of this neoplasm is still largely unknown. Previous studies of ganglioglioma mainly focused on histologic features, immunohistochemical analysis, clinical treatment, and patient outcome. Very few cytogenetic and molecular genetic studies have been reported on this neoplasm. To better understand the mechanism underlying the development of ganglioglioma, we performed comparative genomic hybridization analysis to investigate chromosomal imbalances across the entire genome in five cases of gangliogliomas. Loss of genetic material on the short arm of chromosome 9 was a common genetic alteration found in three of five cases. Overrepresentation of partial or the whole chromosome 7 was another recurrent chromosomal imbalance, confirmed by fluorescence in situ hybridization. Immunohistochemical analysis was performed; all five cases revealed no reaction or low expression for epidermal growth factor receptor antibody. Our study highlights chromosomal regions for further fine mapping and investigation of candidate tumor suppressor genes involved in the pathogenesis of ganglioglioma.- - - - - - - - - - ranking = 5.9429554762294keywords = central nervous system, nervous system, neoplasm (Clic here for more details about this article) |
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