Cases reported "Ganglioglioma"

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1/165. 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. ( info)

2/165. spinal cord ganglioglioma in a child with neurofibromatosis type 2. Case report and literature review.

    Gangliogliomas of the spinal cord are rare disease entities that occur in early childhood. Their occurrence in association with neurofibromatosis Type 2 (NF2) has not been described. The authors describe the unique case of a 2-year-old child with stigmata of NF2 who harbored a spinal cord ganglioglioma that presented as a rapidly growing, exophytic intramedullary mass lesion at the cervicomedullary junction. Treatment consisted of complete surgical resection. Histopathological analysis of the lesion demonstrated a mixed population of neoplastic cells, of both neuronal and glial lineage, that supported the diagnosis of ganglioglioma. ( info)

3/165. 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. ( info)

4/165. 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. ( info)

5/165. Extracranial metastasis of anaplastic ganglioglioma through a ventriculoperitoneal shunt: a case report.

    Extracranial metastasis of an intracranial anaplastic ganglioglioma through a ventriculoperitoneal shunt is reported. A 53-year-old woman was treated by surgery, radiation and chemotherapy and died 2 years later. At autopsy, multiple metastatic lesions were found in the spinal cord, and the abdominal and pleural cavities. Histologically, all the metastatic lesions were composed of atypical cells which resembled primitive glial elements found in intracranial anaplastic ganglioglioma, suggesting that anaplastic glial elements have a metastatic potential. Extracranial metastasis of ganglioglioma is a rare occurrence; however, the spread of glial elements through the shunt further suggests that caution is required in therapy and indicates a need for protective filters in the shunt system. ( info)

6/165. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?

    Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation. ( info)

7/165. thallium-201 single-photon emission computed tomographic and proton magnetic resonance spectroscopic characteristics of intracranial ganglioglioma: three technical case reports.

    OBJECTIVE: The correlation between thallium-201 (201TI) uptake, semiquantitative choline-containing compound values measured by proton magnetic resonance spectroscopy (1H-MRS), and Ki-67 labeling indexes (LIs) was investigated in three gangliogliomas. methods: The early and delayed 201TI indexes were calculated as the ratio of tumor to normal brain tissue uptake by 201TI single-photon emission computed tomography. Single-voxel 1H-MRS was performed to measure the levels of metabolites in the tumors. Ki-67 LI was measured in the surgical specimens. RESULTS: All three gangliogliomas showed very high 201TI uptake on both early and delayed images. 1H-MRS demonstrated malignancy based on the high choline peak relative to the creatine and N-acetylaspartate peaks. Ki-67 LI was less than 1% in two gangliogliomas and 3.5% in an anaplastic ganglioglioma. CONCLUSION: Both 201TI single-photon emission computed tomography and 1H-MRS indicated malignancy, whereas Ki-67 LI indicated low growth activity. 201TI single-photon emission computed tomography and 1H-MRS of ganglioglioma might be affected by metabolic characteristics other than growth activity. ( info)

8/165. ganglioglioma of the trigeminal nerve: MRI.

    ganglioglioma of the cranial nerves is extremely rare; only a few cases involving the optic nerves have been reported. We present a case of ganglioglioma of the trigeminal nerve, which was isointense with the brain stem on all MRI sequences and showed no contrast enhancement. ( info)

9/165. Coexistence of a ganglioglioma and Rasmussen's encephalitis.

    A small number of recent reports have documented coexisting cerebral pathologic entities in patients with Rasmussen's encephalitis. We report the case of a 4-year-old boy who presented with refractory seizures and was found to have both a ganglioglioma and Rasmussen's encephalitis of the same hemisphere. The patient ultimately underwent a functional hemispherectomy with excellent seizure control. We also review the clinical and pathologic hallmarks of both gangliogliomas and Rasmussen's encephalitis. We hope that the addition of this patient to the small but growing literature on dual pathology in Rasmussen's encephalitis may help shed some light on the etiology of this mysterious disease. ( info)

10/165. language area localization with three-dimensional functional magnetic resonance imaging matches intrasulcal electrostimulation in Broca's area.

    In this study, intraoperative electrocortical stimulation mapping (ioESM), the current gold standard for the localization of critical language areas, is compared with functional magnetic resonance imaging (fMRI) in a 14-year-old girl with medically intractable epilepsy caused by a tumor in the region of Broca's area. Prior to the operation, four different fMRI tasks that target inferior frontal language areas were applied. Prior to the resection, ioESM as well as fMRI detected no language areas at the exposed cortical area. After removal of the tumor, a unique opportunity presented itself, where ioESM could be performed in the depth of a now exposed and intact gyrus. One specific locus that was indicated to be a critical language area by multiple-task fMRI was targeted. IoESM selectively confirmed the location of this language area to within an estimated 3 mm. We propose that the combined use of different fMRI tasks increases the sensitivity and specificity for the detection of essential language areas. ( info)
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