Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017636 (glioblastoma)
 18,345 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the case of a 8-years-old boy, admitted with a history of headache, nausea and vomiting. Cerebral angiography showed a non-vascular mass on frontal lobe. The patient underwent craniotomy and the lesion was removed. Neuropathological study revealed that the tumor was a ganglioglioma. The patient received pos-operative radiotherapy. On follow-up, 16 years after, a computed tomographic scan showed a recurrence of the tumor, and a second surgery revealed a glioblastoma multiform. Gangliogliomas are rare tumors of the central nervous system containing neoplastic ganglion cells and low grade neoplastic glial cells. The malignant degeneration occurs only in the glial component, so the prognosis of these tumors is related to the grade of that component.
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PMID:[Malignant course of a ganglioglioma: case report]. 975 34

Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.
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PMID:Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation? 1035 15

Cysts, which are not uncommonly associated with brain tumors, may be responsible for neurological dysfunction. Stereotactic aspiration of such lesions can lead to clinical improvement, but cyst recurrence is common and multiple aspirations may be necessary. Thirteen cases of brain tumors with cystic components were treated by radiosurgery with follow-up of 5-29 months (median 14 months). The tumor diagnoses were three cystic craniopharyngiomas, two brain stem cystic astrocytomas, two cystic cerebellar astrocytomas, one cerebellar hemangioblastoma, one ganglioglioma, one fourth ventricle tumor, one cerebellopontine angle pilocytic astrocytoma, one metastasis from lung cancer and one glioblastoma. The dose at the tumor margin ranged between 10 and 20.5 Gy (mean 15.5 Gy) and the maximum dose ranged between 18 and 45 Gy (mean 32.3 Gy). In 11 of these cases the cystic component recurred in spite of a decrease in the size of the solid tumor component. An Ommaya reservoir was inserted in six cases, stereotactic aspiration was performed in two cases, microsurgery was undertaken in two cases after 2-8 months (mean 4.8 months) and one patient refused further treatment. Multiple aspirations through the Ommaya reservoir were performed in the outpatients on the two patients who required them. It may be appropriate to be cautious in advising radiosurgery for intracranial tumors with a significant cystic component. Microsurgery if possible may be preferable in this situation.
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PMID:Brain tumors with cysts treated with Gamma Knife radiosurgery: is microsurgery indicated? 1068 89

Seven granular cell tumours (GCT) were studied by comparative genomic hybridisation. These consisted of two cerebral and one pituitary GCT as well as four other central nervous system tumours predominantly composed of granular cells (one glioblastoma, meningioma, ganglioglioma and neurinoma each). DNA copy number changes were found in four of seven tumours. Overall, losses (mean: 1.0 per tumour) were more frequent than gains (mean: 0.6 per tumour) and no high-level gains were found. The only DNA copy number change found in two tumours (both cerebral GCT) was loss of 13q21, while the other nine aberrations were only encountered once each: the granular cells of one cerebral GCT additionally showed +1p32-pter, +9q33-qter, +20q, -4, and -18q, of the glioblastoma +7 and -10, and of the meningioma -1p and -22q, while the pituitary GCT, the ganglioglioma and the neurinoma showed no imbalances. Our data suggest that a variety of genetic changes are associated with granular cell formation and support the notion that GCT are not a distinct tumour entity characterised by specific chromosomal imbalances but rather a degenerative phenomenon of cells of various origin showing DNA copy number changes akin to the underlying tumour.
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PMID:Genetic characterisation of granular cell tumours. 1190 49

In order to investigate the clinical characteristics, neuroimaging findings, pathological features, treatment, and outcomes, and to provide valuable guidance for the diagnosis and management of intracranial gangliogliomas, 34 cases of intracranial gangliogliomas were retrospectively analyzed. This study included 23 males and 11 females. Age at operation ranged from 17 days to 50 years. All patients had preoperative magnetic resonance imaging (MRI). Computed tomography (CT), magnetic resonance spectroscopy (MRS), 18F-FDG-PET (positron emission tomography), and 99Tc-HMPAO-SPECT (single photon emission computed tomography) were also performed in some cases. All pathological specimens and all available neuroimages were re-evaluated. The follow-up period varied from 12 to 89 months (mean 36 months). Seizure was the most common presenting symptom (85%). Tumor calcification was detected by CT scan in six of 11 cases. Seventeen gangliogliomas (50%) showed cystic components and 18 tumors (53%) were enhanced on MRI. All temporal lobe gangliogliomas were located intracortically and most of them had poor demarcation on MRI. In comparison with the contralateral normal area, the gangliogliomas showed a reduced Cho/Cr and NAA/Cr ratio, and an increased Cho/NAA ratio. On 18F-FDG-PET, gangliogliomas were usually hypometabolic. HMPAO-SPECT indicated tumor hypoperfusion or isoperfusion. A gross total resection was achieved in 25 patients. Tumor progression was observed in three patients who underwent an incomplete tumor resection. Two of them underwent a malignant transformation to a glioblastoma. Twenty-seven patients could carry on their normal life activity with the Karnofsky Performance Scale (KPS) of more than 80. Even though ganglioglioma is a slowly growing benign tumor, which could be demonstrated by magnetic resonance spectroscopy (MRS), PET, and SPECT, there is a chance of malignant transformation, especially in cases of incomplete tumor resection. Gangliogliomas should be resected gross totally, if feasible, to achieve the best long-term outcomes.
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PMID:Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery. 1224 Nov 12

Advances in the immunohistochemical detection of neuron-specific and neuronal-associated antigens have resulted in the discovery of neuronal elements in certain primary human brain tumors. The results have been not only to expand what neuropathologists commonly recognize as gangliogliomas, including the tumors now known as glioneurocytic tumor with neuropil rosettes and papillary ganglioneuroma, but also to expand the spectrum of tumor types to now include tumors such as central neurocytoma, dysembryoplastic neuroepithelial tumor, and desmoplastic infantile ganglioglioma. These discoveries have helped us to better understand the biology of these tumors and to refine our classification of them. Distinctions among these tumors include sites of predilection, such as the temporal lobe with the dysembryoplastic neuroepithelial tumors, and a spectrum of clinical aggressiveness that spans indolent "quasi-hamartomatous" lesions, such as the dysembryoplastic neuroepithelial tumor, to high-grade, highly aggressive tumors, such as the supratentorial primitive neuroectodermal tumor (World Health Organization Grade IV). Many of these tumors also commonly exhibit a glial component, as determined by both their histologic appearance and their immunoreactivity for glial fibrillary acidic protein. This review covers these recently described lesions, including the desmoplastic infantile ganglioglioma, the dysembryoplastic neuroepithelial tumor, the papillary glioneuronal tumor, the glioneuronal tumor with neuropil rosettes, and the mixed glioblastoma-cerebral neuroblastoma (supratentorial primitive neuroectodermal tumor), as well as the known tumors, ganglioglioma, medulloepithelioma, and medulloblastoma. For pathologists confronted by this growing array of tumors and subtypes, it is appropriate to focus on them and understand the differential diagnosis to be considered when confronted by them.
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PMID:Glioneuronal tumors of the central nervous system. 1262 33

Gangliogliomas generally behave as benign indolent tumors. However, gangliogliomas undergoing malignant transformation have also been reported. The molecular basis for the malignant transformation of gangliogliomas remains unclear. We describe a case of ganglioglioma, which had transformed to glioblastoma after the gross total resection of the original tumor, in a 4-year-old girl. The present case is unusual in four aspects: (i) it arose from a low-grade ganglioglioma in the absence of previous radiation or chemotherapy, which is the fourth reported case; (ii) the original tumor showed a high proliferative index on flow cytometry but a low Ki-67 labeling index, implying that the application of flow cytometry might play a certain role in predicting biological and clinical behavior of low grade gangliogliomas; (iii) p53 mutation and deletion appeared in the secondary glioblastoma, which was not shown in the original well-differentiated ganglioglioma; and (iv) the transformed glioblastoma showed p16 inactivation detected by methylation and deletion, which are relatively uncommon genetic events in secondary glioblastomas. This is the first report of a genetic alteration in glioblastoma arising from a well differentiated ganglioglioma prior to radiation or chemotherapy. Based on the above findings, irrespective of radiotherapy or chemotherapy, rare recurrence of malignant evolution, especially tumors of high S-phase fraction on flow cytometry, warrants long-term follow-up, even in a well-differentiated ganglioglioma.
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PMID:Glioblastomatous transformation of ganglioglioma: case report with reference to molecular genetic and flow cytometric analysis. 1462 54

Polypyrimidine tract-binding protein (PTB) is a nuclear factor that binds to the polypyrimidine tract of pre-mRNA introns, where it is associated with negative regulation of RNA splicing and with exon silencing. We have previously demonstrated that PTB expression is increased during glial cell transformation and that this increase correlates brain and in glial and neuronal tumors. Paraffin sections were stained by using a primary monoclonal antibody against PTB. Tissues that were analyzed included normal with changes in the RNA splicing of the fibroblast growth factor receptor 1. In this paper we examine the specific cellular distribution of PTB expression in normal brain (n = 2) and tumors of various types (low-grade astrocytoma, n = 2; anaplastic astrocytoma, n = 2; glioblastoma, n = 4; medulloblastoma, n = 4; central neurocytoma, n = 2; dysplastic gangliocytoma, n = 1; ganglioglioma, n = 1; paraganglioma, n = 1). In glial cell populations the majority of astrocytes and oligodendrocytes were negative, but occasional positively staining cells were observed. Strongly positive PTB staining was observed in ependymocytes, choroid plexus epithelium, microglia, arachnoid membrane, and adenohypophysis, and weak staining was found in the neurohypophysis. In all cases vascular endothelium and smooth muscle stained strongly. In tumor samples, intense positive nuclear staining was observed in transformed cells of low-grade astrocytoma, anaplastic astrocytoma, glioblastoma multiforme, medulloblastoma, paraganglioma, and the glial population of both ganglioglioma and dysplastic gangliocytoma (the neuronal cells of both were negative). In medulloblastoma, neoplastic neuronal cells were positive, as were other cell lineages. In normal brain, all neuron populations and pineocytes were negative for PTB. We conclude that although glial cells show derepression of PTB expression, a similar mechanism is absent in both nonneoplastic neurons and in most neuronally derived tumor cells. Strong upregulation of PTB expression in tumor cells of glial or primitive neuroectodermal origin suggests involvement of this protein in cellular transformation. Whether PTB affects splicing of RNAs critical to cellular transformation or proliferation is an important question for future research.
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PMID:Expression of the splicing regulator polypyrimidine tract-binding protein in normal and neoplastic brain. 1476 34

A study of the (10)B-enriched p-boronophenylalanine-fructose complex ((10)BPA-F) infusion procedure in potential BNCT patients, including four melanoma of extremities and two high-grade gliomas (glioblastoma and ganglioglioma) was performed. T/B and S/B ratios for (10)B concentrations in tumor (T), blood (B) and skin (S) were determined. The T/B ratio for the glioblastoma was in the 1.8-3.4 range. The ganglioglioma did not show any significant boron uptake. For the nodular metastasic melanoma T/B values were between 1.5 and 2.6 (average 2.1+/-0.4), corresponding to the lower limit of the mean values reported for different melanoma categories. This result might suggest a lower boron uptake for nodular metastasic melanomas. S/B was 1.5+/-0.4. An open two-compartment pharmacokinetic model was applied to predict the boron concentration during the course and at the end of a BNCT irradiation.
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PMID:Biodistribution studies of boronophenylalanine-fructose in melanoma and brain tumor patients in Argentina. 1530 98

Doublecortin (DCX) is required for neuroblastic migration during the development of the cerebral cortex. DCX is a microtubule-associated protein that plays a role in cellular motility. These facts led us to hypothesize that DCX is increased in invasive brain tumors. DCX expression was assessed in 69 paraffin-embedded brain tumors of neuroepithelial origin. In addition, mouse brain sections of the subventricular zone and dentate gyrus were used as positive controls for immunostaining, and specificity of antibody staining was demonstrated by peptide neutralization. DCX was highly expressed in both high-grade invasive tumors (glioblastoma, n=11; anaplastic astrocytoma/oligoastrocytoma, n=7; and medulloblastoma/PNET, n=6) and low-grade invasive tumors (oligodendroglioma, n=3; and astrocytoma/oligoastrocytoma, n=5). However, DCX was less intensely expressed in the circumscribed group of tumors (pilocytic astrocytoma, n=6; ependymoma/subependymoma, n=7; dysembryoplastic neuroepithelial tumor, n=4; ganglioglioma, n=2; meningioma, n=9; and schwannoma, n=9). By the Cochran-Mantel-Haenszel statistical test, the circumscribed group was significantly different from both the high-grade invasive group (P<0.0001) and the low-grade invasive group (P<0.0001). We conclude that DCX is preferentially expressed in invasive brain tumors. In addition, DCX immunostaining was stronger at the margin of the tumor than at the center. For a subset of these tumors, we also detected DCX mRNA and protein by Northern and Western blotting. DCX mRNA and protein was detected in glioma cell lines by Northern blotting, immunofluorescence microscopy and Western blotting. Collectively, the immunohistochemistry, Western blots and Northern blots conclusively demonstrate expression of DCX by human brain tumors.
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PMID:Doublecortin is preferentially expressed in invasive human brain tumors. 1619 16


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