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11/65. Papillary craniopharyngioma of the third ventricle--case report.

    A papillary craniopharyngioma localized in the third ventricle occurred in a 45-year-old male. The clinical presentation was unusual and the neuroradiological appearance resembled a choroid plexus papilloma of the third ventricle. The tumor originated from the right anterolateral wall of the third ventricle, forming a discrete mass with prominent papillae formation. The tumor was totally removed by a transcallosal approach without neurological or endocrinological sequelae.
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12/65. Clinicopathological and ultrastructural study in two cases of chordoid glioma.

    Chordoid glioma is a rare benign neoplasm of uncertain histogenesis occurring in the third ventricle/ /suprasellar region. Recently, data have emerged suggesting that chordoid glioma is a variant of ependymoma related to a specialised ependyma of the subcommisural organ or the lamina terminalis area. In this study, we report clinicopathological and ultrastructural findings in two chordoid glioma cases. In case 1, a tumour (1.5 cm in diameter) in a 62-year-old man invaded the anterior-basal part of the third ventricle in the lamina terminalis region. In case 2, a large tumour in a 51-year-old woman occupied the whole third ventricle. The tumour attached to the medio-basal hypothalamic region. Histologically, both cases revealed a distinct chordoma-like pattern and glial immunophenotype of tumour cells. Under the electron microscope the tumour cells exhibited microvilli, intercellular lumina, intermediate type junctions and focal basal lamina formations. These findings were similar to those previously reported in the chordoid glioma cases. Moreover, the intracytoplasmic cilia and subplasmalemmal pinocytic vesicles or caveoles were observed. The study supports the view of ependymal derivation of chordoid glioma. Its relation to lamina terminalis or infundibular/median eminence area presumably reflecting tumour origin from the modified ependyma of circumventricular organs of the third ventricle is discussed.
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13/65. Peritoneal dissemination from central neurocytoma: case report.

    OBJECTIVE: Central neurocytoma is a low grade tumor of neuroglial origin and a relatively new histological entity. Only a few cases have been reported and its biological behavior is still uncertain. Some cases have shown an aggressive behavior (local recurrence, malignant dedifferentiation or CSF dissemination) and challenged the initial view of its relative benignity. A case of central neurocytoma with peritoneal dissemination is presented. CASE: A six years-old boy with recurrent neurocytoma of III ventricle and left thalamus showed fast growth of tumor rest and ascites three and a half years after subtotal removal of the lesion. Tumor cells were identified in the ascitic fluid and implanted in the peritoneum. Chemotherapy was initiated immediately after diagnosis of peritoneal dissemination (etoposide, carboplatin, doxorubicin and cyclophosphamide). The patient developed metabolic imbalance and respiratory failure due to rapid formation of ascitic fluid and died 3 days after the diagnosis of peritoneal dissemination was established. CONCLUSION: Central neurocytoma is a low grade tumor with low values of the proliferative index in the majority of cases. In spite of that, some tumors may present a very aggressive behavior and extraneural dissemination. Evaluation of proliferative index may be a guideline parameter for planning adjuvant therapies after surgical treatment in selected cases. Extraneural dissemination may occur in some cases specially in patients with ventriculoperitoneal shunt.
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14/65. 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 = 1355.6636818626
keywords = rosette
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15/65. Postural intermittent headaches as the initial symptom of a cavernoma in the third ventricle.

    Cavernous malformations (CM) occur very rarely in the ventricular system. We report a patient with a rare location in the third ventricle who had intermittent headaches as the initial symptom.
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16/65. fourth ventricle neurocytoma with lipomatous and ependymal differentiation.

    We report the case of a 4-year-old girl with a fourth ventricle tumor diagnosed as a cerebellar liponeurocytoma which recurred, showing ependymal differentiation, 14 months after surgery. magnetic resonance imaging at initial presentation revealed a large mass in the fourth ventricle, and histology showed a neoplasm characterized by a combination of well-differentiated neurocytes and cells resembling adipocytes. The tumor recurrence was histologically identical to the original tumor in some regions, but with fewer adipose-like cells, while other areas presented an endocrine architecture with oligo-like or pleiomorphic cells, and rosette-like arrangements of tumoral cells were seen around the thin vessels, with features similar to cellular ependymoma. The cells in the liponeurocytoma areas expressed synaptophysin, chromogranin a, and epithelial membrane antigen. glial fibrillary acidic protein was expressed in some dispersed tumoral cells, in lipidized tumoral cells, and in reactive astrocytes. Cytokeratin was focally expressed in the ependymal region of the recurrence. The immunophenotype of our case, with glial, ependymal, and neuronal or neuroendocrine markers, suggest a neurocytoma with lipomatous and ependymal differentiation. This tumor resembled those derived from circumventricular organs. Its localization in the area postrema region led us to hypothesize that it may be derived from this circumventricular organ.
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ranking = 1355.6636818626
keywords = rosette
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17/65. Rosette-forming glioneuronal tumour of the fourth ventricle: report of a case with clinical and surgical implications.

    A 32-year-old woman presented with a 2-month history of episodic headache, cervical pain and neck rigidity. Neurological examination showed a moderate dysmetria. magnetic resonance imaging (MRI) revealed a mass occupying the fourth ventricle. The patient underwent median sub-occipital craniotomy with total excision of the lesion well demarcated except for a portion infiltrating the right side of the IV ventricle wall. In the post-operative course the patient developed VI and VII right cranial nerves palsy and worsening of dysmetria. MRI confirmed the complete removal of the tumour without signs of recurrence. The pathological diagnosis was rosette forming glio-neuronal tumour (RGNT). At present this is the 13th RGNT reported in literature. These lesions are considered low-grade tumours (WHO I). Nevertheless, the case here reported, like in 6 of the 12 cases in literature, developed disabling post-operative deficits. To establish the therapeutic choice long-term follow-up studies are needed.
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ranking = 1355.6636818626
keywords = rosette
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18/65. spatio-temporal analysis by voltage topography of ictal electroencephalogram on MR surface anatomy scan for the localization of epileptogenic areas.

    A 15-year-old girl developed intractable epilepsy following a right transcallosal resection of the intraventricular teratoma. Magnetic resonance (MR) imaging showed a T (2)-prolonged subcortical lesion in the right frontal lobe as well as a residual intraventricular tumor. The integration of the voltage topography of ictal onset activities of the scalp-recorded electroencephalogram (EEG) and a surface anatomy scan of MR images clearly revealed the epileptogenic area on the cortex above the subcortical lesion, with the propagation pattern towards the frontopolar area. Excision of the epileptogenic cortex and underlying gliosis resulted in a successful cessation of the epilepsy. This non-invasive EEG technique provided useful information that accurately localized the epileptogenic area on a large structural abnormality without invasive intracranial electrocorticographic monitoring.
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19/65. Mucinous metastases from occult breast carcinoma.

    The case of a 34-year-old female patient come to the Emergency Department for neurological symptoms of recent onset is presented. No-contrast CT documented the presence of a neoformation approximately 3 cm in size in the 4th ventricle. For an in-depth diagnostic study of the lesion contrast enhanced MRI was performed. The examination detected a second minute (< 1cm) intraparenchymal nodule highly suspicious of metastasis from primary extra-cerebral neoplasm. The differential diagnosis of infratentorial lesions is discussed. It is concluded that the diagnostic combination of standard MRI with contrast sequences in the three conventional planes plays a major role in typing the nature of focal brain lesions.
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20/65. Hemifacial infantile hemangioma with intracranial extension: a rare entity.

    Intracranial hemangiomas are uncommon, especially in the absence of diffuse hemangiomatosis or the syndrome consisting of posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, sternal clefting, and/or supra-umbilical raphe (PHACES). We saw an 8-month-old ex-premature girl with a large left-sided ocular and facial hemangioma that had been growing since early infancy. Examination revealed a 7 x 13 cm violaceous tumor involving the left periocular region and face. Ophthalmologic examination revealed deprivation amblyopia, anisometropia with myopia and astigmatism. magnetic resonance imaging demonstrated a vascular tumor mass involving the scalp, face, and calvarium with extension into the orbit, infratemporal fossa, nasopharynx, lateral medullary cistern, internal auditory canal, and fourth ventricle. Marked enhancement was seen with contrast, and no posterior fossa malformations were noted. She was treated with prednisolone, which was tapered over 12 months. Follow-up magnetic resonance imaging examination at 25 months showed a marked decrease in the size of all lesions, with residual hemangioma in the periorbital soft tissues and small foci in the orbit and intracranial sites. The brain and ventricular system were normal. Intracranial hemangioma may occur in the setting of a large facial hemangioma (especially segmental) in the absence of the PHACES syndrome or diffuse hemangiomatosis. Radiological imaging should be considered to assess for intracranial hemangioma as well as posterior fossa or arterial anomalies.
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