Cases reported "Ganglioglioma"

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11/25. A papillary glioneuronal tumor arising in an elderly woman: a case report.

    The papillary glioneuronal tumor (PGNT) was first reported by Komori et al. as a type of mixed neuronal-glial tumor. It is characterized by pseudopapillary structures, composed of hyaline vessels and outsheathing glial cells, and by the proliferation of neurocytic cells admixed with ganglioid and ganglion cells. Although it is most common in young adults, it can occur in the elderly. We report a case in a 75-year-old woman, the oldest reported person with PGNT described.
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12/25. Desmoplastic infantile ganglioglioma.

    BACKGROUND: Desmoplastic infantile gangliogliomas are rare intracranial tumors that mostly occur in the first 2 years of life. They are exclusively supratentorial, generally have a voluminous size, and are partially cystic. Histologically they are characterized by a divergent astrocytic and ganglionic differentiation and a prominent desmoplastic stroma; more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery is the treatment of choice. Data available from the literature suggest that no complimentary treatment is needed in cases of complete tumor resection. Chemotherapy is an option in infants with infiltration of eloquent CNS structures and progressive disease after surgery. DIGs have generally a good prognosis: recurrence-free intervals of up to 14 years have been reported and spontaneous disappearance of tumor residuals has also been described. case reports ND DISCUSSION:We report two cases of DIGs. The first child underwent a staged partial removal of a huge right fronto-temporo-parietal tumor when she was 2 months old. At that time histological diagnosis was anaplastic astrocytoma and on these grounds she underwent six chemotherapy cycles, with a partial reduction of the tumor residual. When she was 16 months old a new operation and complete removal of the tumor residual was performed; histological diagnosis was DIG. A review of the initial histological samples confirmed this diagnosis. Twenty-two months after surgery no tumor recurrence has been documented. This case is an example of a difficult differential diagnosis, which can lead to incorrect management choices. The second patient was operated on when he was 9 months old for a mostly cystic right temporo-parieto-occipital DIG. At surgery a deep nodule, strictly adherent to the Galen and internal cerebral veins, was not removed. MRI control 9 months after surgery showed the disappearance of the tumor residual. Eleven years after surgery no tumor recurrence has been documented. The history of this patient confirms that tumor residuals do not need complimentary treatment; indeed they do not usually grow and, as in our patient, they can spontaneously disappear.
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13/25. Angioganglioglioma: a transitional form between angioglioma and gangioglioma?

    The authors describe for the first time an unusual cerebral tumor with unique clinical history, composed of 3 components: pilocytic astrocytoma, vascular proliferations similar to those described as arteriovanous malformations, and a neoplastic ganglion component. These three components were intimately entangled and created the tumor mass. Thus the authors propose the term angioganglioglioma for this entity. The relation to the historically defined anglioglioma and tumors related to ganglioglioma and dysembryoplastic neuroepithelial tumor is discussed. The authors believe that this lesion, in regard to the clinical presentation (long course of the disease, clinical symptoms), is closely associated with ganglioglioma and, with other morphological features, also to angioglioma. Further, it may constitute a new distinct clinicopathological entity with neoplastic and hamartomatous features.
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14/25. Gangliogliomas: A report of five cases.

    Gangliogliomas are rare tumors of the central nervous system. Five gangliogliomas were diagnosed out of 1560 brain tumours surgically resected out in a period of 5 years accounting for 0. 32%. We have tried to discuss in detail the pathological features of these tumours and have mentioned the clinical and radiological features associated with them. All the slides, tissue blocks and pathology reports of the surgical specimens of gangliglioma were reviewed and the clinical and radiological data reviewed. The ages of the patients ranged from 7-65 years with 4 males and 1 female. The tumors were located in the lateral ventricle (a rare site), temporal, parietal and the frontal lobes with duration of seizures varying from 1-9 years. The tumors were diagnosed by the presence of a dual population of neoplastic ganglionic and glial components. The glial components consisted of pilocytic astrocytes (l case), fibrillary astrocytes (2 cases), oligodendrocytes (1 case) and anaplastic astrocytes and oligodendrocytes (1 case). There was one-grade I GG, three-Grade II GGs and one-grade III GG. astrocytes were the commonest glial component of GGs, either pilocytic or fibrillary. Oligodendrocytes as the glial component of GGs was seen in 2 cases one of which was anaplastic and this is a rare finding.
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15/25. Desmoplastic infantile ganglioglioma: a rare tumor with an unusual presentation.

    Desmoplastic infantile ganglioglioma (world health organization grade I) is a rare neoplasm. Despite their common large size and spectacular radiologic and histologic features, the prognosis after surgical resection is good. We present a new case of this tumor in a 14-month-old boy with a recent history of intracranial hypertension. magnetic resonance imaging revealed a large tumor involving the left collateral trigone with dilatation of the lateral ventricles. Surgery revealed two separate solid tumors: one in the left falco-tentorial region and the other in the left rolandic area. Microscopic examination showed a proliferation of neoplastic astrocytes in reticulin-rich desmoplastic stroma associated with scattered ganglion cells. One year after surgery follow-up magnetic resonance imaging did not show tumor progression.
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16/25. Malignant transformation of a spinal cord ganglioglioma--case report and review of the literature.

    Gangliogliomas are tumors of mixed glial and neuronal phenotype that usually have a benign clinical course. Rare cases display anaplastic features at the time of first presentation or progress to anaplastic gliomas over extended times. We report on a ganglioglioma of the spinal cord that recurred as a malignant glioma one and a half years after resection. The initial neoplasm was composed of a mixture of well-differentiated ganglionic and astrocytic cells. The recurrent tumor was an anaplastic small-cell glioma. The sole unusual aspect in the initial neoplasm was an abundance of small vessels with calcified walls, which mimicked a vascular malformation.
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17/25. Desmoplastic non-infantile ganglioglioma.

    Desmoplastic ganglioglioma is a rare, markedly desmoplastic variant of ganglioglioma that usually presents in the first year of life. It is a mixed glial and neuronal cerebral tumor. A few cases of desmoplastic ganglioglioma have been reported in non-infantile patients. We report a case of desmoplastic ganglioglioma in a 14-year-old boy. The patient presented with a large solid cystic mass with mild peritumoral edema in the right posterior parieto-occipital region with direct contact to the falx cerebri. Histopathological examination revealed a low-grade glial tumor with prominent desmoplasia including hypocellular collagenous areas. The tumor was well demarcated with respect to the surrounding brain. Ganglion cells with dysplastic features were present in the tumor and clustered in some areas. Focal lymphocytic infiltration was also observed. Mitotic activity was very low. immunohistochemistry revealed glial fibrillary acidic protein positivity in the astrocytic cells hidden in the desmoplastic tissue. synaptophysin and neuron specific enolase were positive in ganglion-like neuronal cells. The MIB-1 labeling index was less than 1%. This present case confirms that desmoplastic ganglioglioma can be present in young adult patients with its characteristic radiologic features.
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18/25. Aberrant TP53 protein accumulation in the neuronal component of ganglioglioma.

    Gangliogliomas are characterized by their different phenotypic composition of ganglion cells and glial cells. In contrast to the glial cells that are capable of mitotic activity, the ganglion cells are generally considered to lack a neoplastic nature. The authors report here the first unequivocal case of a ganglioglioma harboring aberrant TP53 product that was expressed predominantly in the neuronal component. GeneChip TP53 assay revealed a point mutation resulting in an exchange of amino acid. This case suggests that ganglion cells can participate in the neoplastic process of gangliogliomas.
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19/25. Desmoplastic infantile ganglioglioma: a questionably benign tumour.

    Desmoplastic infantile ganglioglioma is a rare intracranial tumour of childhood that involves the cerebral cortex and the leptomeninges. We report two patients with desmoplastic infantile gangliogliomas and multiple cerebrospinal metastases. To our knowledge, only two similar cases have been reported in the published literature. Pathologically, this rare intracranial tumour shows glial and ganglionic differentiation, accompanied by an extreme desmoplastic reaction. These are low-grade neoplasms that are questionably benign.
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20/25. Anaplastic ganglioglioma of the cerebellopontine angle. Case report.

    A 64-year-old woman presented with a rare anaplastic ganglioglioma in the right cerebellopontine angle manifesting as dizziness persisting for 2 weeks. Preoperative magnetic resonance (MR) imaging revealed a partially enhanced cystic lesion of the right cerebellopontine angle. The tumor was subtotally removed through a right lateral suboccipital craniectomy. The tumor was thought to originate from the brain stem with exophytic growth into the right cerebellopontine angle. Histological examination showed neoplastic ganglional and glial cells with anaplastic features such as mitosis, pleomorphism, and endothelial proliferation. The MIB-1 labeling index of the glial components was 40% to 60%. The diagnosis was anaplastic ganglioglioma (world health organization grade IV). She received postoperative radiotherapy but died of respiratory failure with tumor recurrence 11 months after the operation. Gangliogliomas usually have a good prognosis. Histological features of anaplasia and a high MIB-1 labeling index may be predictive of a poor clinical outcome.
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