Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ganglioglioma, together with its counterparts-ganglioneuroma and gangliocytoma are relatively uncommon neoplasms of the brain composed of neoplastic neurons (ganglion and ganglioid cells) and glial cells. We report here a case of ganglioglioma studied by electron microscopy. The case was further characterized by peculiar chromosomal alterations, 46,XX[6]/43,XX[1], der(1)t(1;5)(q21;q12), der(8;13)(q10;q10),-9,i(10)(q10). Routine light microscopy revealed mixed neuro-glial tumor composed of pilocytic astrocytes with abundant Rosenthal fibers and relatively numerous ganglion cells. The latter were immunoreactive with antibodies (Abs) against synaptophysin and neurofilament protein (NFP). Anti-NFP Abs also immunostained numerous distorted axons embedded in the tumor mass. Some of these showed bullous swellings and thus were analogous to dystrophic neurites or spheroids. Ganglion cells were characterized by abundant intracytoplasmic dense-core vesicles, absence of intermediate filaments and numerous microtubules. Occasionally a close apposition of ganglion cells and Rosenthal fibers were seen. Dense-cored vesicles were pleomorphic and ranged in diameter from small synaptic vesicles to large lysosome-like neurosecretory granules. The former occasionally formed characteristic dumbbell shapes. Neoplastic astrocytes were identical to those of other glial tumors of astrocytic lineage; numerous Rosenthal fibers were frequently seen.
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PMID:The immunohistochemistry and ultrastructure of ganglioglioma with chromosomal alterations: a case report. 870 69

Ganglioglioma is a tumour containing both astrocytic and neuronal components. Most gangliogliomas are observed in the brain, but may also manifest as a nasal glioma. Approximately 250 cases of nasal gliomas have been described in the literature. Gliomas are classified as heterotopias of glia tissue. In the paper we describe the case of nasal ganglioglioma and the diagnostic difficulties. The differences between ganglioglioma, nasal glioma and other congenital midline nasal masses are discussed.
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PMID:Nasal ganglioglioma--difficulties in radiological imaging. 1809 65

Ganglioglioma is a tumor containing both astrocytic and neuronal components. It may occur any where in the central nervous system and spinal cord but is only encountered rarely. Nasal glial heterotopia (also known as ''nasal glioma''), is a rare developmental abnormality seen in a wide age group. Gangliogliomas may also manifest as a nasal glial heterotopia, and neurogenic tumors should be considered in the presence of a nasal mass. In this article, we present a case of ganglioglioma located in the right-nasal cavity. The mass was excised totally through an endoscopic approach. The ganglioglioma developed on a nasal glial heterotopia base. To our knowledge, a ganglioglioma arising from the nasal cavity has not been described previously in the literature.
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PMID:Ganglioglioma in the nasal cavity: a case report. 2081 7

Ganglioglioma is one of the rare mixed neuronal glial tumors of the central nerve system. It is responsible for 0.4 - 2% of the intracranial tumors observed in infants and young matures. Its most common localization is the supratentorial region. Typically, the first symptom is epilepsy. Due to the glial structure, that rare tumor can exhibit a malign transformation. Growing slowly through several months or years, it forms neurological dysfunction. The standard treatment of that supratentorial tumor is usually total resection. If an anaplastic quality is observed, the patient undergoes radiotherapy after the surgical intervention. In this article, we presented a 53-year-old patient who presented with headache and dysphasia. The patient was operated for the cystic mass in the left parietal lobe reported as an abscess. The pathology was reported as ganglioglioma and we discussed the case according to the literature.
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PMID:Ganglioglioma mimicking the cerebral abscess in advanced age: a case report. 2410 Dec 83

Ganglioglioma (GG) is an uncommon brain parenchymal neoplasm. Although most cases have indolent clinical behaviour, a subgroup of GGs does recur, especially in patients with unresectable disease. O6-methylguanine DNA methyltransferase (MGMT) is a DNA repair protein that removes mutagenic and cytotoxic adducts from O6-guanine in DNA. Lack of MGMT protein expression immunohistochemically is related to drug responses in patients with malignant glioma treated with alkylating agents. Furthermore, MGMT promoter methylation has also been investigated as an independent favourable prognostic factor for glioblastoma. The primary management is surgical resection for GGs and gross total resection is recommended. Despite infrequent use of chemotherapy for low-grade GGs, it was still introduced to a subset of patients, especially those who had unresectable disease. We assessed clinicopathological features of nine cases of low-grade GG to further elucidate the relationship between the status of the MGMT protein expression and the prognosis. This series included four men and five women with a mean age of 21.6 years at the first surgery. The mean postoperative follow-up period was 6 years. Only two patients had recurrent disease after 1.7 and 3.2 years of the first surgery. Immunohistochemically, 11.1% exhibited 3+ nuclear staining for MGMT protein, 11.1% exhibited 2+ staining, 33.3% exhibited 1+ staining, and 44.4% exhibited 0 staining. Tumours with more intensive MGMT protein expression (2+~3+ immunostaining) tended to recur more frequently (p < 0.05), corresponding to the worse prognostic predictive value of intensive MGMT staining.
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PMID:The prognostic impact of MGMT expression on low-grade gangliogliomas: a clinicopathological and immunohistochemical study. 2437 55

Ganglioglioma (GG) is a grade I tumor characterized by alterations in the MAPK pathway, including BRAF V600E mutation. Recently, diffuse midline glioma with an H3 K27M mutation was added to the WHO 2016 classification as a new grade IV entity. As co-occurrence of H3 K27M and BRAF V600E mutations has been reported in midline tumors and anaplastic GG, we searched for BRAF V600E and H3 K27M mutations in a series of 54 paediatric midline grade I GG (midline GG) to determine the frequency of double mutations and its relevance for prognosis. Twenty-seven patients (50%) possessed the BRAF V600E mutation. The frequency of the co-occurrence of H3F3A/BRAF mutations at diagnosis was 9.3%. No H3 K27M mutation was detected in the absence of the BRAF V600E mutation. Double-immunostaining revealed that BRAF V600E and H3 K27M mutant proteins were present in both the glial and neuronal components. Immunopositivity for the BRAF V600E mutant protein correlated with BRAF mutation status as detected by massARRAY or digital droplet PCR. The median follow-up of patients with double mutation was 4 years. One patient died of progressive disease 8 years after diagnosis, whereas the four other patients were all alive with stable disease at the last clinical follow-up (at 9 months, 1 year and 7 years) without adjuvant therapy. We demonstrate in this first series of midline GGs that the H3 K27M mutation can occur in association with the BRAF V600E mutation in grade I glioneuronal tumors. Despite the presence of H3 K27M mutations, these cases should not be graded and treated as grade IV tumors because they have a better spontaneous outcome than classic diffuse midline H3 K27M-mutant glioma. These data suggest that H3 K27M cannot be considered a specific hallmark of grade IV diffuse gliomas and highlight the importance of integrated histomolecular diagnosis in paediatric brain tumors.
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PMID:Co-occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma. 2798 73