1/13. Extraventricular neurocytoma with ganglionic differentiation associated with complex partial seizures.We report an unusual case of extraventricular ("cerebral") neurocytoma with ganglion cells located in the right temporal lobe in a 9-year-old girl with complex partial seizures and precocious puberty. CT showed a calcified mass with central cystic zones. MR imaging showed a markedly hyperintense predominately solid tumor on both T1- and T2-weighted images, without appreciable contrast enhancement. Cerebral neurocytomas are histologically benign and radical surgery is curative; they should be included in the differential diagnosis of temporal lobe tumors in children.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
2/13. A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases.Four examples of a novel glioneuronal neoplasm are presented. All tumors affected adults (including two males and two females aged 25-40 years) as supratentorial, cerebral hemispheric masses with associated seizure activity and, in one case, symptoms of raised intracranial pressure and progressive hemiparesis. CT scans in two cases revealed hypodense masses without calcification. MRI scans at presentation demonstrated, in all cases, solid T1-hypointense and T2-hyperintense tumors with mass effect in one instance but no edema or contrast enhancement. Only one was relatively circumscribed on neuroradiologic study. All were infiltrative in their histologic growth pattern and predominantly glial in appearance, being composed mainly of fibrillary, gemistocytic, or protoplasmic astroglial elements of WHO grade II to III. Their distinguishing feature was their content of sharply delimited, neuropil-like islands of intense synaptophysin reactivity inhabited and rimmed in rosetted fashion by cells demonstrating strong nuclear immunolabeling for the neuronal antigens NeuN and Hu. These cells included small, oligodendrocyte-like ("neurocytic") elements as well as larger, more pleomorphic forms. Two cases contained, in addition, well-differentiated neurons of medium to ganglion-cell size. Proliferative activity was observed principally within the glial compartment; two cases contained mitotic figures and exhibited relatively elevated MIB-1 indices (6.8% and 8.2%). One of the latter progressed and proved fatal at 30 months following subtotal resection and radiotherapy. The three other patients are alive at intervals of 14 to 83 months, two tumor-free and one with extensive disease associated with the appearance of enhancement on MRI. This glioneuronal tumor variant may pursue an unfavorable clinical course.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
3/13. January 2000: 12 year old boy with recent onset seizures.A 12-year old boy presented with new onset of seizures and a CT scan showed a left frontal lobe tumor which was removed completely. Neuropathological examination showed a pleomorphic ganglion cell tumor with necrosi, and endothelial proliferation. The diagnosis was extraventricular atypical neurocytic neoplasm ("cystic ganglioneurocytoma").- - - - - - - - - - ranking = 0.4keywords = ganglion (Clic here for more details about this article) |
4/13. Abnormal neuronal and glial argyrophilic fibrillary structures in the brain of an aged albino cynomolgus monkey (macaca fascicularis).An aged albino male cynomolgus monkey (macaca fascicularis) more than 35 years old died after showing neurological signs including gait disturbance, trembling, drowsing tendency and a decrease in activity. Neuropathological examination revealed glial fibrillary tangles (GFTs) mainly distributed in the putamen, caudate nucleus, thalamic nuclei, substantia nigra, red nucleus, globus pallidus, trapezoid body, pyramid, pons and medulla oblongata of the brain, and neurofibrillary tangles (NFTs) in the thalamic nuclei. These structures were positively stained by the modified Gallyas-Braak (GB) method and immunostained for tau. The tau-positive argyrophilic GFTs were morphologically classified into four types, as in human cases, i.e., tufts of abnormal fibers (TAFs), thornshaped astrocytes (TSAs), glial coiled bodies (GCBs) and argyrophilic threads (ATs) depending on their GB profiles, and GCBs were the major structures in this case. Some of these structures were also immunoreactive for alpha-synuclein. The glial cells possessing the structures were negative for glial fibrillary acidic protein, a marker for astrocytes, indicating that the argyrophilic GFTs were present in oligodendroglia. In addition, marked neuronal loss and ubiquitin-positive spheroid bodies were observed in the substantia nigra and globus pallidus. According to the characteristic distribution of the argyrophilic structures in neurons and glial cells as well as clinical signs, the monkey might have suffered from a neurodegenerative disease such as progressive supranuclear palsy (PSP). This is the first report of the occurrence of a neurodegenerative disease in a nonhuman animal.- - - - - - - - - - ranking = 0.1001015314259keywords = nucleus (Clic here for more details about this article) |
5/13. 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.- - - - - - - - - - ranking = 0.4keywords = ganglion (Clic here for more details about this article) |
6/13. Ganglioneurocytoma mimicking a malignant tumor: case report with a literature review of the MRI appearance of neurocytomas and gangliogliomas.A 14-year-old girl presented with symptoms of increased intracranial pressure after her head was squeezed at a party. MRI demonstrated a mass that was hypointense to brain on T1WI and heterogeneous in signal on PD and T2WI; compression of the ventricle, midline shift, and mild ventriculomegaly also were present. Contrast enhancement was extensive and heterogeneous, mimicking a malignant tumor. Neuropathology revealed a ganglioneurocytoma.- - - - - - - - - - ranking = 0.2keywords = ganglion (Clic here for more details about this article) |
7/13. MR imaging and histopathologic findings of a case of cerebral ganglioneurocytoma.We report a case of ganglioneurocytoma manifesting as a complex partial seizure in a young adult male. MR images depicted a well-marginated cystic mass with a heterogeneous solid portion abutting the dura in the parietal lobe. The solid portion showed minimal heterogeneous enhancement, and pressure erosion of the overlying calvarium had occurred. Following gross total resection, the clinical outcome was satisfactory, with no further seizures, and during the five-year follow-up period, the tumor did not recur.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
8/13. Cerebral neurocytoma without ganglion cell differentiation.Neurocytomas are rare neuronal tumours, and are defined as tumours comprising cells with neuronal differentiation; they commonly arise in the ventricles. We report a case of a cerebral neurocytoma in a young man who presented with hemiparesis. The tumour was a radiologically a well-circumscribed large cyst with solid mass; the histopathology showed a well-differentiated lesion comprising uniform, round cells with perinuclear halos in a neuropil background, and immunohistochemically positive for neuronal markers (synaptophysin, neuron-specific enolase and neurofilaments). We suggest that cerebral neurocytomas should be considered among the uncommon causes of a large intra-axial cystic mass with a solid component in young adults.- - - - - - - - - - ranking = 0.8keywords = ganglion (Clic here for more details about this article) |
9/13. Dysplastic ganglioneurocytoma with increased glucose metabolism: a heterotopia with unique histopathology.A 1 year and 7 month old boy was incidentally found to have an intracranial mass lesion at the frontal base. The mass was 45 x 54 x 47 mm in size, contained a calcification, a few small cysts, and extended downward to the sphenoid sinus and upper pharynx. The signal intensity of the lesion on magnetic resonance imaging was iso-high on T1-weighted images, and slightly high on T2-weighted images, and it did not enhance with gadolinium injection. Although there was no obvious mass effect, 18F-fluorode-oxyglucose positron-emission tomography demonstrated increased uptake, and a surgical resection was performed suspecting a neoplastic lesion. Histologically, the lesion consisted of small to large anomalous neurons and glial cells but lacked normal cortical architecture. Cellularity was high in some portion with MIB-1 labeling index of 2%, but there was no cellular atypia suggestive of neoplasm. Therefore, this lesion was considered to be a dysplasia that does not fit into the previously described entity. We suggest this lesion would be better described as dysplastic ganglioneurocytoma.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
10/13. neurocytoma of spinal cord.We report a case of spinal cord neurocytoma in a 67-year-old man who had experienced a progressive numbness of the left foot during the previous 4 years. magnetic resonance imaging showed a well-defined intramedullary tumor located at the T10-T11 level. The pathological examination revealed histological characteristics described in neurocytomas. The tumor cells showed a uniform small nucleus and clear or slightly eosinophilic cytoplasm with frequent perinuclear halos, resembling the picture of oligodendroglioma. Some tumor cells exhibited mature ganglion cell appearance. Electron microscopy showed cells with microtubules and dense-core vesicles in their cytoplasm and cytoplasmic process. Immunohistochemically, the majority of tumor cells expressed synaptophysin and neuron-specific enolase. We conclude that this tumor is an exceptional case of neurocytoma located in the spinal cord, and consider that the term neurocytoma can be applied to tumors with neuronal differentiation intermediate between neuroblastoma and ganglioneuroma, even if arising in CNS outside of the intracranial ventricular system.- - - - - - - - - - ranking = 0.45005076571295keywords = ganglion, nucleus (Clic here for more details about this article) |
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