Cases reported "Ganglioglioma"

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1/9. ganglioglioma with a tanycytic ependymoma as the glial component.

    We studied a cystic ganglioglioma (GG) located in the right frontal lobe of the brain. Interestingly, the fibrillary spindle glial cells were often arranged in a fascicular pattern, and the generally uniform, round-to-oval delicate nuclei appeared to resemble those of ependymoma; and the neoplastic neurons often contained neurofibrillary tangles (NFTs). The glial component was positive for glial fibrillary acidic protein and occasionally contained granular or microvesicular structures positive for epithelial membrane antigen. Ultrastructural investigation revealed that the glial cells were ependymal in nature; intracytoplasmic lumina and intercellular microrosettes lined with cilia and microvilli, as well as long zonulae adherentes, were evident. In addition, chromogranin a-positive granular staining, neurosecretory-granule-like structures, and parallel arrays of microtubules were sometimes associated with the blood vessels. We considered the present case to be an unusual example of GG with an ependymoma, more precisely a tanycytic ependymoma, as the glial component; to our knowledge, the existence of ependymoma as the main glial component of this particular tumor has not been described before. The occurrence of NFTs, which has been reported in several cases of GG, was an additional, unusual feature.
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2/9. Papillary glioneuronal tumour: clinicopathological and biochemical study of one case with 7-year follow up.

    Among the mixed glioneuronal tumours, a new variant called papillary glioneuronal tumour has recently been delineated. A case occurring in a 23-year-old man is reported. The tumour was cystic with a mural nodule enhanced by gadolinium injection. It was located within the left parieto-occipital lobe. Surgical excision showed a greyish friable tumour with cystic areas. Histopathological examination revealed a pseudopapillary component comprising a single layer of regular cells, arranged around hyalinised vessels. These cells were immunoreactive with anti-glial fibrillary acidic protein and HNK1 antibodies. A neurocytoma-like component coexisted with round blind cells and focal fibrillary rosettes. These cells were immunostained by anti-neuron-specific enolase and anti-synaptophysin antibodies. Neither mitoses nor ganglioid cells were seen. HNK1, the three isoforms of NCAM, and the L1 adhesion molecule were detected by Western blot analysis. Ultrastructural study showed three different types of cells. The first contained gliofilaments, the second showed long processes with true synapses, and the third was poorly differentiated. However, all had identical nuclei and contained dense bodies. These findings suggest a common origin for the tumour cells derived from a bipotential neuroglial progenitor. As for other mature mixed neuroglial tumours, the prognosis is good. Our patient is free of disease 7 years after complete surgical treatment.
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3/9. 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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4/9. September 2002: 24-year-old female with a 6-month history of seizures.

    The September 2002 COM. A 24-year-old female presented with a history of 3 generalized seizures, the first of which had occurred 6 months before admission. Her neurological examination was normal, but upon admission her MRI showed a small cystic lesion in the left parieto-occipital region. The lesion was hyper-intense on T-2 weighted images and did not show contrast enhancment. At surgery, the tumor was found to be deep to the cortex and was a cyst with amber fluid surrounded by gliotic brain. Microscopically, the tumor was well-demarcated from the surrounding tissues, which showed reactive changes, including Rosenthal fibers. The tumor was composed of GFAP-positive glial cells, which were arranged in a pseudopapillary fashion around blood vessels. In between, the tumor cells were positive for neuronal markers. The diagnosis was papillary glioneuronal tumor (PGNT), a relatively recently described lesion that may be a variant of ganglioglioma. The current literature on PGNTs is reviewed.
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5/9. Papillary glioneuronal tumor--a new variant of benign mixed brain neoplasm.

    We report a case of a 14-year-old girl with papillary glioneuronal tumor (PGNT) in right parietal lobe. On MRI the tumor presented as a contrast enhancing mass with small central hypodense area and consisted of areas similar to central neurocytoma intermixed with vessels surrounded with glial cells. There were also small loose areas superficially reminding DNT. Neurocytic component presented strong synaptophysin immunostaining, while intermixed glial element presented GFAP-immunopositivity. Our case is similar to previously reported PGNT, but an important difference lies in not distinct cystic formation and a presence of loose, edematous tissue. PGNT may be regarded as a variant of ganglioglioma or as a complex variant of extraventricular neurocytoma and belongs to a wide group of benign, dysembryoplastic or even hamartomatous neuroepithelial tumors, which may differentiate into both, glial or neuronal direction: pleomorphic astrocytoma may posses neurocytic differentiation, desmoplastic infantile ganglioglioma may be regarded as complex superficial dural astrocytoma and DNT may present gliomatous areas (complex DNT). Also other rare tumors reminding DNT have been reported: DNT-like neoplasm of septum pellucidum and rosetted glioneuronal tumor. From this point of view it is important to remember that such a wide spectrum may be difficult to discriminate into very narrow clinico-pathological entities.
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6/9. Long term follow-up in a patient with papillary glioneuronal tumor.

    We report a case of a young female patient with a rare and recently described form of brain tumor. This patient had a history of headache, hemiparesis and motor simple partial seizures. Her investigation revealed a brain tumor involving the left frontal and parietal lobes. The radiological images showed a cystic mass with multiple nodular masses and a rim of contrast enhancement extending from the right parietal cortex to the ipsilateral ventricle and corpus callosum. The patient underwent gross resection of the lesion and the histological analysis of the surgical specimen revealed a pseudopapillary structure formed by delicate vessels intermixed with a fibrillary pattern and bordered by intense astrocytic reaction with Rosenthal fibers. These features correspond to the recently described mixed neuronal-glial neoplasm, the papillary glioneuronal tumor. The patient has been followed for five years since the surgical treatment, without evidence of tumor recurrence, confirming the indolent behavior of this type of tumor.
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7/9. 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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8/9. Papillary glioneuronal tumor radiologically mimicking a cavernous hemangioma with hemorrhagic onset.

    Papillary glioneuronal tumor is a recently identified low-grade brain neoplasm classified as variant of ganglioglioma. Its salient morphological characteristics are the presence of pseudopapillary structures composed of blood vessels, often hyalinized, lined by uniform small astrocytes and a proliferation of neurocytic cells, eventually admixed with ganglioid and ganglion cells. We present a case of papillary glioneuronal tumor occurring in a 15-year-old female with an unusual hemorrhagic onset. The clinical, morphological and immunohistochemical features are discussed and the published literature is reviewed. This article proposes that papillary glioneuronal tumor should be included in the differential diagnosis of patients with tumoral related brain hemorrhage.
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9/9. Papillary glioneuronal tumor: a case report and review of the literature.

    Papillary glioneuronal tumor is a recently described central nervous system neoplasm that almost always occurs adjacent to the lateral ventricle. We present a case of this rare entity, representing the 21st case of this lesion, which exhibits a mixed astrocytic and neuronal differentiation. This case was an incidental finding in a young woman who presented secondary to a traumatic injury to the left eye. Histologic evaluation after surgical removal showed a cystic tumor consisting of 2 distinct components: a unique pseudopapillary architecture admixed with foci of solid areas. The pseudopapillae were composed of thick hyalinized vessels enclosed by a single layer of glial fibrillary acid protein-positive astrocytes and variously sized synaptophysin-positive and chromogranin-negative neuronal cells in the interpapillary regions. Abundant Rosenthal fibers, foci of calcification, areas of hemosiderin deposition, gliosis, areas of vascular proliferation associated with piloid gliosis, and chronic inflammatory infiltrate were identified. The combination of cytologic benignity, lack of necrosis, and low proliferative index as evidenced by immunohistochemistry using antibody to Ki-67 confirmed the low malignant potential of this tumor. knowledge and precise classification of this entity are important to avoid unnecessary use of chemo- and/or radiotherapy for treatment.
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