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1/12. ependymoma with extensive lipidization mimicking adipose tissue: a report of five cases.

    Lipomatous ependymoma is a recently described entity and only 3 cases of this variant have been reported in the literature. We report 5 cases of this rare variant of ependymoma. patients age ranged from 4 years to 45 years and, interestingly, all of them were males. Two tumors were supratentorial in location, 2 in the fourth ventricle and 1 was intramedullary. Microscopically all of them showed the classical histology of ependymoma along with lipomatous differentiation. The lipomatous component was composed of cells with a large clear vacuole pushing the nucleus to the periphery and giving a signet ring cell appearance. This component demonstrated positivity for GFAP and S-100 protein thereby confirming its glial lineage. Three of the 5 tumors were high grade (WHO-grade III), had a high MIB-1 labelling index (MIB-1 LI) and showed recurrence on follow-up. However, 2 were low grade (WHO grade II) and patients are free of disease till the last follow up.
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ranking = 1
keywords = nucleus
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2/12. 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 = 4.5706603520501
keywords = ganglion
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3/12. 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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ranking = 2.285330176025
keywords = ganglion
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4/12. Infantile hemifacial spasm.

    A 6-week-old infant had recurrent contractions of the facial musculature on the left side, which continued throughout early childhood. Surgical exploration at 5 1/2 years of age revealed a ganglioneuroma of the fourth ventricle. hemifacial spasm (HFS) in infancy and childhood suggests the possibility of serious intracranial pathologic findings.
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ranking = 2.285330176025
keywords = ganglion
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5/12. Anterior lateral ventricular subependymal giant cell astrocytomas. Microsurgical aspects of two cases.

    The surgical management of two cases of lateral ventricular subependymal giant cell astrocytoma, arising in the head of the caudate nucleus, and causing hydrocephalus due to obstruction of the foramen of Monro is described. One lesion, in a patient with tuberous sclerosis, was resected using a transcallosal approach and the other, in a patient with no stigmata of tuberous sclerosis, using a frontal, trans-cortical transcystoventricular approach. The microsurgical aspects of excision and pathological anatomy of both tumours were very similar. Following tumour excision and pellucidotomy both patients had partial resolution of their hydrocephalus with complete resolution of their preoperative symptoms. The merits of transcallosal and transcortical approaches to these lesions are discussed.
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keywords = nucleus
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6/12. Clinicopathological experience with intraventricular neurocytomas.

    Intraventricular neurocytoma is a rare clinicopathological entity that has been recently described. We are reporting our experience with four diagnosed cases and the previously reported cases from the available literature are reviewed. These neoplasms occur mainly in young adults, and their histological diagnosis is difficult on light microscope, because they are almost indistinguishable from oligodendrogliomas. Nevertheless, the presence of tumoral cells arranged around nucleus-free fibrillary zones, resembling the large rosettes of pineocytomas and the immunohistochemical demonstration of synaptophysin are useful data for the pathological diagnosis. This diagnosis is easy on electron microscope, because it demonstrates the neuronal nature of tumoral cells. Regarding prognosis, we have found increasing evidence that these tumors are associated with a favourable course after surgery, and at present there is no clear evidence of the usefulness of radiotherapy.
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ranking = 1
keywords = nucleus
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7/12. Ganglioneurocytoma of the third ventricle.

    We present a very rare case of an intracranial ganglioneurocytoma. This 57-year-old female patient noticed some concentration difficulties for about 5 months. visual acuity was 80% on both sides. CT and MRI of her head demonstrated a 3 x 2.5 x 2.8 cm3 lesion within the third ventricle with inhomogenous enhancement of contrast medium. After a right pterional approach the tumor could be removed completely. Postoperatively there was a paresis of the oculomotor nerve on the right side and psychological changes. Histological examination revealed neuronal differentiation with neurocytes and small ganglionic cells and the tumor was graded as a ganglioneurocytoma (WHO grade II). Follow-up examination 6 months after the operation showed improvement of her third nerve paresis and of her neuropsychological deficits. MRI showed no recurrence.
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ranking = 6.8559905280751
keywords = ganglion
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8/12. 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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ranking = 2.285330176025
keywords = ganglion
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9/12. Intraventricular neuroblastoma. A light and electron microscopic study and review of the literature.

    The neuroblastoma can generally be diagnosed from the histologic findings, but electron microscopic evidence is often required. A neuroblastoma occurring in the lateral ventricle of a 17-year-old female is described in this report. The tumor cells, which were strongly stained with hematoxylin, contained a small round or oval nucleus with scant cytoplasm and an obscure cell border as observed by light microscopy. No calcification, mitoses and typical ganglion cells were detected. An electron microscopic examination disclosed dense-cored vesicles, cell processes, clear vesicles, dumbbell-shaped dense bodies, microtubules and zonulae adherentes in the tumor cells. These findings suggest differentiation of the tumor cells to neuronal cells.
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ranking = 3.285330176025
keywords = ganglion, nucleus
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10/12. Germ cell tumors (germinoma and yolk sac tumor) in unusual sites in the brain.

    This report presents three cases of primary intracranial germ cell tumor encountered in unusual sites, or essentially non-midline structures. The three provided the opportunity to examine surgically obtained tissues with the electron microscope. The histological diagnosis was initially made by light microscopic observations. The first case was a 10-year-old boy in whom the tumor occupied the right thalamic and basal ganglionic region and was diagnosed as a yolk sac tumor. The second an 11-year-old boy who presented with a mass lesion on the left thalamic and basal ganglionic region, diagnosed as germinoma. The third was a 39-year-old man who presented with multiple tumors in the ventricular system and posterior fossa, also diagnosed as germinoma. Among the three cases, neither diabetes insipidus nor ophthalmologic disorder was manifested. Extensive examination and autopsy findings indicated that these intracranial lesions had not metastasized from primary extracranial tumors, including those of the genital organs.
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ranking = 4.5706603520501
keywords = ganglion
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