Cases reported "Ganglioglioma"

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1/25. 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.
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2/25. 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.
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3/25. ganglioglioma in a patient with Turcot syndrome. Case report.

    A 33-year-old woman with Turcot syndrome harbored a brain tumor and colon cancer and had a familial history of this syndrome. On histological examination, the brain tumor was found to have large and diffusely scattered ganglion cells within a diffuse background of astrocytic cells in a fibrillary matrix. The tumor was diagnosed as a ganglioglioma. No germline mutation in the adenomatous polyposis coli gene was detected using a protein truncation assay. These findings indicate that this patient had brain tumor-polyposis syndrome Type 1 of Turcot syndrome. This is the first report of a ganglioglioma related to Turcot syndrome.
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4/25. 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.
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5/25. Ganglioneurocytoma mimicking a malignant tumor: case report with a literature review of the MRI appearance of neurocytomas and gangliogliomas.

    A 14-year-old girl presented with symptoms of increased intracranial pressure after her head was squeezed at a party. MRI demonstrated a mass that was hypointense to brain on T1WI and heterogeneous in signal on PD and T2WI; compression of the ventricle, midline shift, and mild ventriculomegaly also were present. Contrast enhancement was extensive and heterogeneous, mimicking a malignant tumor. Neuropathology revealed a ganglioneurocytoma.
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6/25. 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.
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7/25. ganglioglioma of the brainstem: report of three cases and review of the literature.

    BACKGROUND: Brainstem gangliogliomas are rare low-grade tumors that usually have a long clinical history. However, they may cause sudden death. There are only 31 cases of brainstem ganglioglioma reported in the literature, and only one has been studied with magnetic resonance (MR). We present three new cases of brainstem ganglion cell tumor studied with computed tomography (CT) (3 cases) and MR (2 cases) and discuss the clinical presentation, diagnostic imaging and treatment of these tumors. CASE DESCRIPTION: Age at presentation ranged from 19 to 59 years old. Two patients were female and 1 male. Duration of symptoms before diagnosis ranged from 1 year to nearly 14 years. Presenting complaints included syncope spells, cranial nerve deficits, headache, and gait instability. Imaging studies revealed well-circumscribed lesions involving the brainstem; the lesion was cystic in one case and calcified in one. They were iso- or hyperdense on CT scan, isodense on T1-weighted and hyperdense on T2-weighted MRI and frequently showed contrast enhancement.All tumors were operated through a posterior fossa craniectomy. Using microsurgical techniques only partial resection could be achieved, as there was no sharp delineation from the surrounding tissue in any case. Two of our patients had increased neurological deficits after surgery. radiotherapy was not given. Follow-up of tumoral remnants has not shown clear tumor growth after 1, 3.5, and 10 years. CONCLUSIONS: Imaging characteristics of brainstem gangliogliomas do not seem to differ from those in other locations and are not specific. Radical surgery is rarely if ever possible, nor is it advisable because of the risk of functional deterioration. However, because of their benign histology, partial resection seems to carry a similar prognosis as tumors in other locations that are amenable to complete resection.
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8/25. September 2001: 51-year-old man with seizures since childhood.

    The September 2001 COM. A 51 year old man with refractory seizures presented with a recent seizure while at work but no other neurologic deficit. Imaging studies demonstrated a solid and cystic lesion of the right temporal lobe. Microscopic and immunohistochemical studies demonstrated an atypical ganglioglioma due to the presence of neurofibrillary tangles within the neoplastic ganglion cell component. This is the fifth case reported in the literature. Neoplastic ganglion cells and neurofibrillary tangles are discussed both separately and as integral components of this rare tumor.
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9/25. Dysembrioplastic neuroepithelial tumor: cytological diagnosis by intraoperative smear preparation.

    The dysembrioplastic neuroepithelial tumor (DNT) is a benign and slow-growing temporal lobe cortical lesion that presents with clinically unresponsive partial and complex seizures, with onset before age 20 yr. DNT shows a multinodular architecture and the presence of mixed glioneural elements. The authors report on a case of DNT diagnosed by the intraoperative smear cytological technique. We describe the cytological characteristics of smear preparations of DNT, with emphasis on the differential diagnosis. The authors present a 5-yr-old male with partial seizures for the preceding 3 yr. The patient showed no other neurological deficits. Magnetic resonance identified a lesion in the cortical area of the right temporal lobe. The smear cytology showed small, round, oligodendroglial-like cells arranged in lobules, with microcystic spaces containing ganglion cells floating in a fine fibrillary background. histology confirmed the diagnosis of DNT. This report shows that the smear preparation can be trustworthy for the intraoperative diagnosis of DNTs, helping to determine the appropriate neurosurgical procedure and thus avoiding extensive and unnecessary resections.
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10/25. Desmoplastic infantile ganglioglioma--clinicopathological and immunohistochemical study of four cases.

    case reports: Four cases of desmoplastic infantile ganglioglioma (DIG) seen in india are described. These patients presented with large, supratentorial, superficially situated cystic tumours that showed glial and ganglionic differentiation; accompanied by a severe desmoplastic reaction. MIB-1 labelling was rare, despite foci of apparently primitive neuroepithelial cells. There was lacking p53 protein expression by tumour cells in all cases. The prognosis was good following either partial or complete tumour resection. DIGs are a distinct form of developmental neuroepithelial tumour, probably arising from neural progenitor cells in subcortical zone along with mature subpial astrocytes. CONCLUSIONS: In view of its favourable prognosis, this tumour has to be diagnosed accurately by immunohistochemical techniques using glial and neuronal markers. The absence of p53 protein expression suggests that DIG probably has different molecular genetic pathways from other supratentorial astrocytomas.
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