Cases reported "Ependymoma"

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1/55. 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.
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ranking = 1
keywords = glioma
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2/55. Familial gliomas : a case report.

    Two non-twin brothers were found to have intracranial malignant neoplasms. The age of presentation was third and fourth decade but the onset was simultaneous, at the same time. diagnosis in each of them was made by computed tomography and confirmed by histopathology. Elder among them had cellular ependymoma and the younger had oligodendroglioma. Both the brothers received radiotherapy post operatively and were surviving asymptomatically without any neurological deficit, leading active life as police constable, 12 months after surgical treatment.
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ranking = 0.625
keywords = glioma
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3/55. 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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ranking = 0.625
keywords = glioma
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4/55. spinal cord gliomas: management and outcome with reference to adjuvant therapy.

    The authors review their experience with 19 consecutive cases with either astrocytic tumour (glioblastoma multiforme one, anaplastic astrocytoma one, astrocytoma 4, pilocytic astrocytoma 4) or ependymoma (10 tumours in 9 patients) of the spinal cord who were treated during the period from 1982 to 1996. The patients included 10 male and 9 female patients with a median age of 38 years. The main tumour locations included the cervicomedullary region 5 the cervical cord (8), the thoracic cord (5) and one each in the thoracolumbar region and conus medullaris. While a total removal of the tumour was achieved in 8 out of 10 ependymomas, the initial treatment for astrocytic tumours was a partial resection in 5, and biopsy in the remaining 5. As adjuvant treatment, 8 patients received radiation therapy and 2 received chemotherapy. Two patients with an astrocytic tumour received chemotherapy only, while the remaining 9 received neither radiation therapy nor chemotherapy initially. After these treatments, 6 out of the 8 patients with low grade astrocytoma have remained alive for 1.3-12.6 years, while 2 patients with high grade astrocytic tumours died within 15 months following surgery. Eight out of 9 patients with an ependymoma have remained alive for 3.0-12.3 years, while one committed suicide 2 years after surgery. As a result, 14 patients are still alive; half of them are accompanied by a mild neurological dysfunction, while the remaining one has a moderate deficit. The postoperative results and the rationale for surgery is discussed, and an approach for utilising adjuvant therapy for high grade tumours is also suggested.
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ranking = 0.5
keywords = glioma
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5/55. So-called malignant and extra-ventricular neurocytomas: reality or wrong diagnosis? A critical review about two overdiagnosed cases.

    Central neurocytoma (CN) is described as a rare intra-ventricular benign neuronal tumor of the brain. Two primary tumors first diagnosed as malignant and extra-ventricular neurocytomas are reported here. Histologically, the tumor of the first patient, a forty-one-year-old man, consisted of monotonous cells with round nuclei, but no fibrillar background. The second tumor, in a nineteen-year-old girl, showed areas of moderately pleomorphic round cells, with numerous rosettes and ganglion cell differentiation, in an abundant fibrillary network. Both presented calcifications. Mitoses were more frequent in recurrences and spinal locations than in the primaries. All tumors stained strongly for synaptophysin, and GFAP was partly positive in the first case only. patients received post-surgical radiotherapy and were still alive eight and six years, respectively, after initial surgery. The interpretation of atypical cases, such as ours is not easy: the diagnoses finally retained were oligodendroglioma in the first case and ganglioneuroblastoma in the second case. Furthermore, neurocytomas atypical either by their unusual topographical or histological presentation or by their poor prognosis, have been frequently entitled in this way on synaptophysin positivity. So, we were prompted to reassess the entity of CN, seventeen years after the first description, to re-appreciate the reality of anatomo-clinical variants and to discuss the value of synaptophysin positivity in these tumors. In conclusion, it seems preferable to individualize true classical CN, which has a favorable outcome, from so-called extra-ventricular, atypical and anaplastic, clinically malignant neurocytomas for which complementary treatment is required.
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ranking = 0.125
keywords = glioma
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6/55. Intramedullary clear cell ependymoma in the cervical spinal cord: case report.

    OBJECTIVE AND IMPORTANCE: Clear cell ependymoma of the spinal cord has not been reported in the literature, although ependymoma in the cerebral and cerebellar hemispheres has been described. We present the first case report of this rare histological type of ependymoma arising in the cervical spinal cord and emphasize the importance of recognizing this histological entity. CLINICAL PRESENTATION: A 42-year-old woman presented with numbness in both upper limbs and spastic gait. magnetic resonance imaging revealed an intramedullary tumor at the C6-T1 level with syringomyelia. INTERVENTION: The tumor was totally removed. Histological analysis revealed that the tumor was composed of round cells with perinuclear halos similar to those observed in oligodendroglioma. However, we diagnosed clear cell ependymoma because these tumor cells exhibited epithelial features and ependymal rosettes under light microscopic examination. CONCLUSION: Histological diagnosis was crucial to our determining whether to perform postoperative adjuvant therapy in this patient. Neurosurgeons should be aware of the possibility of this histological entity among intramedullary spinal cord tumors.
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ranking = 0.125
keywords = glioma
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7/55. brain surface ependymoma.

    A case of brain surface ependymoma is reported here. A 41-year-old woman presented with weakness of the right upper limb. magnetic resonance imaging revealed a left fronto-parietal cystic tumor with a calcified mural nodule. Total removal of the tumor was performed. This tumor was located within the surface portion of the fronto-parietal parenchyma and was sharply demarcated from the surrounding brain tissue, and there was no continuity with the ventricles. Histological examination of this tumor showed monotonous proliferation of tumor cells with moderate cellularity and occasional mitoses, forming true ependymal rosettes and perivascular pseudorosettes with fibrillary background. A clear cell component resembling oligodendroglioma was partly noted. The tumor cells showed positive immunoreaction for glial fibrillary acid protein and vimentin.
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ranking = 0.125
keywords = glioma
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8/55. An ependymoblastoma of the pons.

    A case of ependymoblastoma of the pons in a child is reported, the first so far described in this location. The case adds further evidence for the acceptance of ependymoblastomas as a specific variety of primitive human glioma.
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ranking = 0.125
keywords = glioma
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9/55. Hemispheric cerebral gliomas in children with NF1: arguments for a long-term follow-up.

    BACKGROUND: Neurofibromatosis type 1, or peripheral neurofibromatosis, is the most form of common phakomatosis. In some instances, it can be associated with tumors of the central nervous system. CASE REPORT: We describe four cases of hemispheric cerebral glioma in children with NF1. Two of these tumors were pilocytic astrocytomas and two, ependymomas. We reviewed the literature on hemispheric cerebral gliomas in NF1 patients in an attempt to find characteristic features of and some explanations for these lesions. CONCLUSION: We conclude that surgical resection of these tumors should be proposed whenever possible, with due consideration for their progressive nature and the uncertainties about malignancy. We advocate yearly clinical and neuroradiological follow-up over a long period in NF1 children, since they may develop additional CNS tumors during their lifetime.
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ranking = 0.75
keywords = glioma
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10/55. spinal cord tumor in a patient with multiple sclerosis: case report.

    The association between multiple (MS) sclerosis and cerebral gliomas has been sporadically reported in the literature, causing a long lasting discussion if these lesions occur coincidentally or if MS plaques may actually lead to the genesis of gliomas. We report a 36 year old man who developed a rapid onset of right side weakness and loss of vision, having established a diagnosis of MS which was confirmed by CSF analysis and MRI. Nine years later he developed progressive tetraparesis, leading initially to suspicion of illness relapse and a demyelinating plaque in the spinal cord. However, after MRI investigation, a spinal cord tumor was diagnosed. The patient underwent cervical spine laminotomy for microsurgical removal of the spinal cord tumor diagnosed as ependymoma. The neurological deficits improved significantly.
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ranking = 0.25
keywords = glioma
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