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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The descriptive term papillary glioneuronal tumor (PGNT) has been repeatedly applied to a morphologic subset of low-grade mixed glial-neuronal neoplasia of juvenile and young adult patients. We report on a 13-year-old boy with PGNT of the left temporal lobe, who presented with headaches and a single generalized seizure. On magnetic resonance imaging, tumor was seen as a large, moderately enhancing paraventricular mass with cyst-mural nodule configuration and slight midline shift. Perifocal edema was virtually absent. Gross total resection could be performed, followed by an uneventful recovery. Histologically, the tumor exhibited similar, if not identical, features as reported previously. These comprised a patterned biphasic mixture of sheets of synaptophysin-expressing small round cells and pseudorosettes of GFAP-positive rudimentary astrocytes along vascular cores. Focally, the latter imprinted a pseudopapillary aspect on this otherwise solid lesion. Both cellular components expressed non-polysialylated neural cell adhesion molecule (NCAM)-L species, and several overlapping areas of synaptophysin and GFAP immunoreactivity were present. The mean MIB-1 labeling index remained below 1%. Signs of anaplasia, in particular mitotic figures, endothelial proliferation, or necrosis were consistently lacking. We perceive PGNT as a clinically and morphologically well-delineated subgroup of extraventricular neurocytic neoplasia, whose paradigmatic presentation may allow for consideration as an entity.
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PMID:Papillary glioneuronal tumor. 1641 93

The human polyomavirus JCV, the etiological agent of progressive multifocal leukoencephalopathy, has been associated with primitive neuroectodermal tumors and various glial-derived tumors, including glioblastoma multiforme (GBM). Here we describe the unique clinical case of a 54-year-old man who presented with headaches, hemiparesis and drowsiness. T1 and T2 magnetic resonance images revealed a large solid tumor with a cystic component located in the right temporal lobe, with extension into the parietal lobe. Histologically, the tumor was composed of two areas, a main area of large neoplastic cells with pleomorphic atypical nuclei and abundant cytoplasm, which by immunohistochemistry was reactive for glial fibrillary acidic protein, mixed with several foci of poorly differentiated tumoral cells with elongated nuclei and scant cytoplasm, negative for GFAP, but robustly immunoreactive for synaptophysin and phosphoneurofilaments. Results from PCR in laser capture microdissected cells from both areas of the tumor revealed the presence of DNA sequences corresponding to the early, late and control regions (CR) of the JCV genome and expression of JCV proteins T-antigen and Agnoprotein in both phenotypes. No evidence for capsid protein was observed, excluding productive viral infection. Sequencing demonstrated the presence of the JCV Mad-1 strain with distinct point mutations in the CR of isolates from both, GBM and small cell architectural areas. The presence of JCV DNA sequences and expression of viral proteins further reinforces the role of the widely spread human neurotropic virus in early transformation and in the development of brain tumors.
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PMID:Glioblastoma multiforme with small cell neuronal-like component: association with human neurotropic JC virus. 1655 92

Pineal parenchymal tumors (PPT), including pineoblastomas, are very uncommon, especially in adults. Because of the small number of reported cases, the histological and biological features of these tumors are still being defined, as is their optimal management. Also, the pathological variability of these tumors makes it difficult to draw general conclusions about their behavior. We report the case of a PPT of intermediate differentiation (PPTID) with an excellent outcome. A 3.2 cm tumor in the pineal and tectal region with obstructive hydrocephalus was found in a 28-year-old female complaining of headaches and hypoesthesia of the right side of the face. Stereotactic biopsy revealed a highly cellular tumor composed of small cells that contained little cytoplasm that were arranged in a diffuse pattern. Mitotic activity was low and there was no evidence of necrosis. Immunohistochemical examination demonstrated positive staining for neuron-specific enolase and synaptophysin. There was no expression of neurofilaments or GFAP. Ki-67 proliferation index was 12%. The diagnosis was a PPTID. Treatment consisted of combined neoadjuvant chemotherapy and craniospinal radiotherapy. Six years after treatment, the patient is alive and free of any clinical or radiological signs of relapse. This paper discusses the pathological and biological features of these tumors and the treatment options available.
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PMID:Pineal parenchymal tumors of intermediate differentiation in adults: case report and literature review. 1670 48

We report a rare skull base neurocytoma. A 44-year-old female with a history of focal seizure and progressive right-sided weakness sought treatment at an outside institution, where she underwent total resection of a "left medial sphenoid wing paraganglioma" in 1984. In 1995 after experiencing intense left-sided headaches for 3 weeks, the patient presented to our institution. Magnetic resonance imaging revealed a large local recurrence. She had deficits dating to her initial surgery, including moderate right-sided hemiparesis, complete left ophthalmoplegia, and left facial numbness.The patient underwent a craniotomy with extensive removal of the involved sphenoid bone and a subtotal resection of the tumor. Neurocytoma was diagnosed based on strong immunohistochemical staining for synaptophysin and no reactivity for glial fibrillary acidic protein. Postoperatively, her headaches resolved completely and her neurologic status remained at baseline. The residual tumor was treated with radiation therapy. After 5 years, she remains clinically and radiographically stable.Although typically located adjacent to the foramen of Monro, neurocytomas have now been reported in almost every subcompartment of the craniospinal axis. Finding neurocytomas in extraventricular locations may require revisiting the current theory that subependymal progenitor cells are the cells of origin for these tumors.
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PMID:Skull base neurocytoma: case report and review of the literature of extraventricular neurocytomas. 1716 46

We report on clinicopathological findings in two cases of rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) occurring in females aged 16 years (Case 1) and 30 years (Case 2). Symptoms included vertigo, nausea, cerebellar ataxia, as well as headaches, and had been present for 4-months and 1 week, respectively. Magnetic resonance imaging (MRI) indicated a cerebellar-based tumor of 1.8 cm (Case 1) and 5 cm (Case 2) diameter each, bulging into the fourth ventricle. Case 2 involved a cyst-mural-nodule configuration. In both instances, the solid component appeared isointense on T(1) sequences, hyperintense in the T(2) mode, and enhanced moderately. Gross total resection was achieved via suboccipital craniotomy. However, functional recovery was disappointing in Case 1. On microscopy, both tumors comprised an admixture of low-grade astrocytoma interspersed with circular aggregates of synaptophysin-expressing round cells harboring oligodendrocyte-like nuclei. The astrocytic moiety in Case 1 was nondescript, and overtly pilocytic in Case 2. The architecture of neuronal elements variously consisted of neurocytic rosettes, of pseudorosettes centered on a capillary core, as well as of concentric ribbons along irregular lumina. Gangliocytic maturation, especially "floating neurons", or a corresponding immunoreactivity for neurofilament protein was absent. Neither of these populations exhibited atypia, mitotic activity, or a significant labeling for MIB-1. Cerebellar parenchyma included in the surgical specimen did not reveal any preexisting malformative anomaly. Despite sharing some overlapping histologic traits with dysembryoplastic neuroepithelial tumor (DNT), the presentation of RGNT with respect to both patient age and location is consistent enough for this lesion to be singled out as an autonomous entity.
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PMID:Rosette-forming glioneuronal tumor of the fourth ventricle: report of two cases with a differential diagnostic overview. 1765 10

A 51-year-old woman presented with a 2-month history of double vision and numbness around her left ear. She subsequently became unsteady on her feet and developed further cranial nerve abnormalities, before complaining of headache, nausea and vomiting. Imaging revealed features suggestive of two intracranial lesions; one non-contrast-enhancing high-signal area in the cerebellum with associated calcification, and a second contrast-enhancing low-signal area in association with the fourth ventricle, and at surgery there were two apparent components to the tumor. The histopathological features were those of a low-grade, focally calcified tumor comprising atypical ganglion and glial cells with interspersed Rosenthal fibres. Mitotic figures were not seen, and there was no necrosis. An infiltrate of small reactive lymphocytes was interspersed among the neoplastic cells. Immunohistochemistry revealed expression of synaptophysin by many of the dysplastic ganglion cells, with some co-expressing neurofilament protein and occasionally glial fibrillary acidic protein (GFAP). Several of the dysplastic ganglion cells also expressed CD34. The glial cell population was highlighted by GFAP. Ki-67 (MIB-1) activity was not noted among the neoplastic populations--the few positive nuclei in these areas were those of interspersed reactive CD3-positive T lymphocytes. In addition, at the edge of one of the biopsies was a dense infiltrate of mitotically-active large atypical CD 20-positive B lymphocytes, among which the Ki-67 (MIB-1) labeling index reached 80%. The final diagnosis was diffuse large B cell lymphoma arising within a ganglioglioma of the cerebellum, and this is believed to be the first reported case.
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PMID:51-year-old woman with double vision. 1836 42

We report a 6-year-old boy who presented with a medulloblastoma demonstrating classic, myoblastic, neuronal, glial, and melanotic differentiation and manifesting as severe morning headache. Magnetic resonance imaging revealed a mass lesion with cystic components in the cerebellar vermis. He underwent suboccipital craniotomy and total resection of the tumor. The specimen consisted of three morphologically distinct components. The first component consisted of densely packed cells with round-to-oval highly hyperchromatic nuclei surrounded by scanty cytoplasm. Immunohistochemical staining revealed diffuse expression of neurofilament protein and focal expression of desmin and myoglobin. The second component consisted of long spindle-shaped cells with elongated nuclei and eosinophilic cytoplasm. Immunohistochemical staining revealed diffuse expression of neurofilament protein, desmin, and myoglobin. The third component consisted of cells with small, densely hyperchromatic nuclei and scanty cytoplasm in a fine fibrillary background. Mature ganglion cells and melanotic tumor cells were also observed. Immunohistochemical staining revealed diffuse expression of synaptophysin and neurofilament protein, and focal expression of glial fibrillary acidic protein, S-100 protein, desmin, and myoglobin. The diagnosis was medulloblastoma with myoblastic, neuronal, astrocytic, and melanotic differentiation. Medulloblastoma demonstrating multipotent differentiation is rare, but the features observed in this case support the idea that medulloblastoma originates from multipotent stem cells.
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PMID:Medulloblastoma demonstrating multipotent differentiation: case report. 1841 65

The article describes a case of a 15-year old boy after a head contusion with a five-month history of headaches and two seizure episodes. MR imaging revealed a partly solid and partly cystic cortical-subcortical tumour within the precentral gyrus with post-contrast enhancement. The patient underwent gross total resection of the lesion. Histologically the neoplasm was composed of pseudopapillary gliovascular structures surrounded by solid glioneuronal tumour areas. The expression of GFAP and nestin characterized the central parts of the tumour. Moreover the immunolabelling for synaptophysin, neurofilaments, Olig2 and NCAM was present in the peripheral part of the lesion. The neoplasm was consistent with a papillary glioneuronal tumour - one of the new entities in the last WHO CNS tumour classification.
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PMID:Papillary glioneuronal tumour of the precentral gyrus. 1858 11

Neuroenteric cysts of the CNS are uncommon benign lesions usually involving the spinal cord or rarely the cerebellopontine angle (CPA). We report a rare example of multiple neuroenteric cysts arising from the CPA and foramen magnum in a 20-year-old Caucasian woman who presented with headaches and dizziness. An MRI showed three separate lesions, not communicating with each other. The first lesion, within the left posterior lateral aspect of the CPA, demonstrated isointensity to gray matter on the fluid-attenuated inversion recovery (FLAIR) sequence. The second lesion, within the left foramen of Luschka at the level of the CPA, demonstrated hyperintensity on the T(2)-weighted sequences, intermediate to slightly hyperintense on T(1)-weighted sequence and hyperintensity on FLAIR. The third lesion, within the anterior/inferior left cerebellum at the level of the foramen magnum, followed CSF signal intensity throughout. None of the lesions demonstrated significant enhancement or bone lesions. Due to compression effect, surgery was performed. Pathologic examination revealed cystic structures lined by a single layer of non-ciliated well-differentiated mucin-producing columnar epithelium with eosinophilic to amphophilic cytoplasm and round to oval nuclei with focal pseudostratification. Immunohistochemical studies showed focal positivity for cytokeratin 7, CK 5/6, synaptophysin, and carcinoembryonic antigen (CEA), diffuse positive staining for epithelial membrane antigen (EMA) and BerEP4; and negative staining for cytokeratin 20, TTF-1, and GFAP. The MIB-1 proliferation index was < 1%. One-year follow-up has shown no recurrence. The differential diagnosis and a brief review of the literature are also presented.
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PMID:Multiple neuroenteric cysts at cerebello-pontine angle and foramen magnum: a case report and review of the literature. 1871 73

We report a papillary glioneuronal tumor occurring in the right frontal lobe of a 26-yr-old woman and we review the pertinent literature. Papillary glioneuronal tumor (PGNT) is a rare cerebral neoplasm, identified in approximately 37 cases to date. In 2007, the World Health Organization (WHO) classified the PGNT as a grade I neuronal-glial tumor because of its biphasic neurocytic and glial components and indolent clinical course. Patients commonly present with headaches or seizures, but may be asymptomatic with the mass discovered incidentally upon neuroimaging. Histology demonstrates a pseudopapillary architecture with a single or a pseudostratified layer of glial cells overlying hyalinized vasculature with interpapillary regions of neurocytic or ganglion cells. Peripheral eosinophilic granular bodies, Rosenthal fibers, hemosiderin, and areas of calcification are often noted. The PGNT displays moderate cellularity and is typically devoid of necrosis, microvascular proliferation, and mitoses. Its immunohistochemical profile includes glial fibrillary acidic protein (GFAP)-positive glial cells, synaptophysin-positive interpapillary neurocytes, and MIB-1 labeling in the range of 1-2%.
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PMID:Papillary glioneuronal tumor: a case report and review of the literature. 1871 60


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