Cases reported "Astrocytoma"

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1/124. Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.

    OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.
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2/124. Intraoperative validation of functional magnetic resonance imaging and cortical reorganization patterns in patients with brain tumors involving the primary motor cortex.

    OBJECT: The purpose of the present study was to compare the results of functional magnetic resonance (fMR) imaging with those of intraoperative cortical stimulation in patients who harbored tumors close to or involving the primary motor area and to assess the usefulness of fMR imaging in the objective evaluation of motor function as part of the surgical strategy in the treatment of these patients. methods: A total of 11 consecutive patients, whose tumors were close to or involving the central region, underwent presurgical blood oxygen level-dependent fMR imaging while performing a motor paradigm that required them to clench and spread their hands contra- and ipsilateral to the tumor. Statistical cross-correlation functional maps covering the primary and secondary motor cortical areas were generated and overlaid onto high-resolution anatomical MR images. Intraoperative electrical cortical stimulation was performed to validate the presurgical fMR imaging findings. In nine (82%) of 11 patients, the anatomical fMR imaging localization of motor areas could be verified by intraoperative electrical cortical stimulation. In seven patients two or more activation sites were demonstrated on fMR imaging, which were considered a consequence of reorganization phenomena of the motor cortex: contralateral primary motor area (nine patients), contralateral premotor area (four patients), ipsilateral primary motor area (two patients), and ipsilateral premotor area (four patients). CONCLUSIONS: Functional MR imaging can be used to perform objective evaluation of motor function and surgical planning in patients who harbor lesions near or involving the primary motor cortex. Correlation between fMR imaging findings and the results of direct electrical brain stimulation is high, although not 100%. Based on their study, the authors believe that cortical reorganization patterns of motor areas might explain the differences in motor function and the diversity of postoperative motor function among patients with central tumors.
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3/124. Intra-operative optical method using intrinsic signals for localization of sensorimotor area in patients with brain tumor.

    The purpose of this study is intra-operatively to localize the sensorimotor area by intrinsic optical method detecting the changes in regional cerebral blood flow (rCBF) and cortical temperature following neuronal activity during median nerve stimulation. In 18 patients with brain tumors located around the sensorimotor cortex, cortical recording of somatosensory evoked potentials (SEPs) was performed and localized changes in rCBF during median nerve stimulation were measured by a laser-Doppler flowmeter on the locations of SEPs and around the activation area obtained by functional magnetic resonance imaging (fMRI). In two patients, cortical thermomapping was also performed during median nerve stimulation. In fMRI study, the significant activation area of sensorimotor could be obtained in 17 of 18 patients. In cortical recording of SEPs, the polarity reversal of N20 and P20 was observed in 14 of the 18 patients. In 9 of the 14 patients in whom SEPs could be recorded, the localized changes in rCBF, corresponding to the stimulation, were detected in the N20 area. In 2 of the 4 patients in whom N20 could not be recorded successfully, the localized changes in rCBF could be detected. The increase in rCBF during the stimulation was 18.3% /- 5.3% (mean /- SD, n = 11). Thermomapping could demonstrate the localized area, where the increase in rCBF was also detected, by observation of the changes in cortical temperature during the stimulation. The intra-operative intrinsic optical method detecting rCBF and cortical temperature in combination with recording of SEPs may be considered useful for brain functional localization related to neurosurgical disorders.
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4/124. Pupillary sparing in oculomotor palsy from temporal lobe astrocytoma. Report of two cases.

    Two cases are reported of pupillary sparing in oculomotor palsy from temporal lobe astrocytoma. In one of them it was the first manifestation of recurrence of tumour. Tumour infiltration with resultant nerve ischemia is suggested to be the responsible pathological process.
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5/124. Malignant pilocytic astrocytoma in the medulla oblongata: case report.

    A 27-year-old woman visited our hospital with chief complaints of abducens nerve palsy and cerebellar symptoms. On computerized tomographic scanning and magnetic resonance imaging, a tumor with strong enhancement was found on the dorsal side of the medulla oblongata. A tumor was excised by suboccipital craniotomy and C1 laminectomy. Histologically, many Rosenthal fibers together with pilocytic tumor cells were found in some regions, but a very high Ki-67 labeling rate accompanied by cells with nuclei of irregular size and giant cells was observed in other regions. The tumor was diagnosed as malignant pilocytic astrocytoma originating from pilocytic astrocytoma by transformation. The biological behavior of pilocytic astrocytoma is obscure in several respects. We report our experience of a case of malignant pilocytic astrocytoma that developed in the brain stem and progressed extremely rapidly.
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6/124. moyamoya disease associated with a brain stem glioma.

    An 8-year-old boy was found to have primary moyamoya disease associated with a brain stem glioma. For over 3 years the child had experienced transient ischemic attacks induced by hyperventilation. One month before referral to our hospital he had presented with progressive left facial nerve palsy. magnetic resonance imaging showed a cystic mass in the lower pons. angiography revealed severe bilateral stenosis of the internal carotid arteries and prominent moyamoya vessels in the basal ganglia. Partial resection of the tumor yielded a histological diagnosis of pilocytic astrocytoma. Local radiation therapy reduced the size of the tumor. Anastomosis of the superficial temporal arteries and middle cerebral arteries on both sides was then performed. After direct bypass surgery, the patient remained in a good condition for a 5-year follow-up period. Clinical investigation of the coincidence of primary moyamoya disease and brain stem glioma led the authors to conclude that these two diseases coexisted independently.
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7/124. Superior oblique paresis with contralateral relative afferent pupillary defect.

    BACKGROUND: The purpose of this study is to report a case of superior oblique paresis and contralateral relative afferent pupillary defect (RAPD) with normal vision in a patient with brainstem astrocytoma. methods: We correlated the patient's clinical findings with anatomical substrates on magnetic resonance imaging (MRI) findings. RESULTS: The patient had right-sided superior oblique paresis. There was a left-sided RAPD, although visual acuities and visual fields were normal in both eyes. T1-weighted, gadolinium-enhanced MRI demonstrated a hyperintense area in the right dorsal midbrain. CONCLUSION: It is suggested that the lesion damaged both the pretectal afferent pupillary pathway and fascicles of the trochlear nerve, causing a unique combination of neuro-ophthalmologic findings.
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8/124. Cranial nerve root entry zone primary cerebellopontine angle gliomas: a rare and poorly recognized subset of extraparenchymal tumors.

    With the exception of patients with neurofibromatosis type II, pediatric extraparenchymal cerebellopontine angle (CPA) tumors of any sort are extremely rare. Most gliomas encountered in the CPA in either children or adults involve the CPA as exophytic extensions of primary brain stem and/or cerebellar tumors. We encountered an unusual case of a giant CPA pilocytic astrocytoma arising from the proximal trigeminal nerve, completely separate from the brain stem. A nine-year-old girl with no evidence for any neurocutaneous syndrome, presented with headaches, mild obstructive hydrocephalus, trigeminal hypesthesia and a subtle peripheral facial paresis. Pre-operative neuroimaging suggested a petroclival meningioma. The tumor was completely resected via a right pre-sigmoid, retro-labyrinthine, subtemporal, transtentorial ('petrosal') approach, using intraoperative neurophysiological monitoring, with minimal morbidity. This appears to be the first reported case of a pediatric primary CPA glioma and the seventh reported case of primary CPA glioma, overall. It represents the second reported case of a primary CPA pilocytic astrocytoma. Given the findings in this case and the six other cases of primary CPA gliomas reported in the literature, as well as the results of histological studies of normal cranial nerves, we hypothesize that the point of origin of these rare and unusual tumors is the root entry zone of the involved cranial nerves. The differential diagnosis of primary CPA tumors should be expanded to include cranial nerve root entry zone primary CPA gliomas.
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9/124. Auditory brainstem response and temporal bone pathology findings in a brain-dead infant.

    The criteria for assessing adult brain death have been already established, but those for infant brain death have not been yet established in japan. We report auditory brainstem response (ABR) and postmortem pathology of the temporal bone and brain of a brain-dead 9-month-old female. During the comatose state, her ABR showed only waves I and II bilaterally. autopsy revealed the presence of a left cerebellar astrocytoma, herniation and anoxic encephalopathy. The pathological examination of the temporal bone revealed the destruction of the inner ear particularly on the left side. In the auditory pathway of brain-dead patients, degeneration occurs first in the cerebrum, followed by the cochlear nerve. Thus, ABR is one of the useful means to assess brain death even in infants.
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10/124. Secondary parathyroid hyperplasia in tuberous sclerosis: report of a case with large eosinophilic ganglion-like cells similar to those of subependymal giant cell astrocytoma, tubers, and atypical angiomyolipoma.

    We report a case of secondary parathyroid hyperplasia in a 49-year-old man with tuberous sclerosis. Two parathyroid glands had collections of large, eosinophilic ganglion-like endocrine cells that to our knowledge have not been previously described at this site. These cells are morphologically similar to those of subependymal giant cell astrocytoma, tubers, and atypical angiomyolipoma, all of which may arise in the setting of tuberous sclerosis. These large, eosinophilic ganglion-like cells found in different affected organs appear to be distinctive of tuberous sclerosis. We suggest these large eosinophilic cells arise from a common stem cell precursor that acquires variable phenotypes according to alterations in the cellular microenvironment.
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