MCID: GNG004
MIFTS: 49

Ganglioglioma

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Ganglioglioma

MalaCards integrated aliases for Ganglioglioma:

Name: Ganglioglioma 12 50 56 52 42 14 69
Childhood Ganglioglioma 12 69
Adult Ganglioglioma 12 69
Mixed Cell Tumors Containing Both Neural Ganglionic Cells and Neural Glial Cell Components 50
Cns Ganglioglioma 12

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:5078
MeSH 42 D018303
Orphanet 56 ORPHA251949
UMLS via Orphanet 70 C0206716

Summaries for Ganglioglioma

NIH Rare Diseases : 50 a ganglioglioma is a rare type of brain tumor, accounting for approximately 1% of all brain tumors.  gangliogliomas occur when a single cell in the brain starts to divide into more cells, forming a tumor.  this can occur when the cell randomly acquires changes (mutations) in genes that regulate how a cell divides.  most gangliogliomas grow slowly and are considered benign.  however, up to 10% of gangliogliomas may grow more rapidly and become malignant, meaning the tumor affects the surrounding brain tissue.    the main treatment for ganglioglioma is removal of the entire tumor during surgery.  if the entire tumor is not removed, it has the potential to recur and may require additional surgery or treatments, such as radiation therapy or chemotherapy.  unfortunately, because gangliogliomas are quite rare, there is limited information to show that radiation therapy or chemotherapy are effective treatments for this condition. last updated: 7/11/2011

MalaCards based summary : Ganglioglioma, also known as childhood ganglioglioma, is related to pleomorphic xanthoastrocytoma and anaplastic oligodendroglioma, and has symptoms including seizures An important gene associated with Ganglioglioma is SYP (Synaptophysin), and among its related pathways/superpathways are mTOR signaling pathway (KEGG) and Neuroscience. The drugs Dabrafenib and Trametinib have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and spinal cord, and related phenotypes are Increased proliferation and Upregulation of Wnt/beta-catenin pathway after WNT3A stimulation

Disease Ontology : 12 A cell type benign neoplasm that has material basis in glial-type cells.

Wikipedia : 72 Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently... more...

Related Diseases for Ganglioglioma

Diseases related to Ganglioglioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 302)
id Related Disease Score Top Affiliating Genes
1 pleomorphic xanthoastrocytoma 30.7 CHGA ENO2 SYP
2 anaplastic oligodendroglioma 29.8 GFAP S100B SYP
3 papillary glioneuronal tumor 29.8 GFAP S100B SYP
4 giant cell glioblastoma 29.3 GFAP NES TP53
5 anaplastic ganglioglioma 12.3
6 desmoplastic infantile ganglioglioma 11.9
7 desmoplastic infantile astrocytoma/ganglioglioma 11.9
8 nasal ganglioglioma 11.9
9 gangliocytoma 11.2
10 devriendt syndrome 10.8 GFAP SYP
11 hypogonadotropic hypogonadism-frontoparietal alopecia syndrome 10.8 GFAP SYP
12 dystonia 10.8 GFAP SYP
13 laryngeal neuroendocrine tumor 10.8 BRAF GFAP
14 cerebellar astrocytoma 10.8 GFAP SYP
15 epidermal appendage tumor 10.8 GFAP SYP
16 sella turcica neoplasm 10.8 GFAP NES
17 anaplastic plasmacytoma 10.8 GFAP NES
18 paranasal sinus cancer 10.7 ENO2 SYP
19 situs inversus totalis with cystic dysplasia of kidneys and pancreas 10.7 ENO2 SYP
20 glomus tumor 10.7 ENO2 SYP
21 bronchus adenoma 10.7 ENO2 SYP
22 malignant ovarian mixed epithelial neoplasm 10.7 CHGA SYP
23 pemphigus foliaceus 10.7 ENO2 SYP
24 macular retinal edema 10.7 GFAP NES
25 gallbladder adenocarcinoma 10.7 ENO2 SYP
26 intellectual disability-polydactyly-uncombable hair syndrome 10.7 ENO2 GFAP
27 chondroid chordoma 10.7 ENO2 SYP
28 ludwig's angina 10.7 CHGA SYP
29 signet ring cell intrahepatic cholangiocarcinoma 10.7 CHGA SYP
30 central neurocytoma 10.7 GFAP SYP
31 skin papilloma 10.7 ENO2 SYP
32 esophagus adenocarcinoma 10.7 CHGA SYP
33 basophilic carcinoma 10.7 CHGA SYP
34 vulvar benign neoplasm 10.7 ENO2 GFAP
35 aicardi-goutieres syndrome 10.7 CHGA SYP
36 mature teratoma 10.7 ENO2 SYP
37 diffuse infiltrative lymphocytosis syndrome 10.7 CHGA SYP
38 thymus small cell carcinoma 10.7 ENO2 SYP
39 diffuse lipomatosis 10.6 CHGA SYP
40 proliferative type fibrocystic change of breast 10.6 ENO2 SYP
41 sebaceous adenoma 10.6 SYP TSC2
42 demyelinating disease 10.6 ENO2 GFAP
43 thyroid sarcoma 10.6 CHGA SYP
44 pyoderma 10.6 CHGA GFAP
45 supratentorial primitive neuroectodermal tumor 10.6 CHGA ENO2
46 sensory organ benign neoplasm 10.6 ENO2 SYP
47 encephalitozoonosis 10.6 ENO2 SYP
48 juvenile nasopharyngeal angiofibroma 10.6 BRAF S100B
49 central nervous system hematologic cancer 10.6 CHGA SYP
50 pneumothorax 10.6 GFAP SYP

Graphical network of the top 20 diseases related to Ganglioglioma:



Diseases related to Ganglioglioma

Symptoms & Phenotypes for Ganglioglioma

UMLS symptoms related to Ganglioglioma:


seizures

GenomeRNAi Phenotypes related to Ganglioglioma according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased proliferation GR00094-A 8.96 SMARCB1 TP53
2 Upregulation of Wnt/beta-catenin pathway after WNT3A stimulation GR00016-A 8.92 BRAF ENO2 TSC1 TSC2

MGI Mouse Phenotypes related to Ganglioglioma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.02 BRAF ENO2 GFAP MAP2 S100B SMARCB1
2 growth/size/body region MP:0005378 9.91 TSC1 TSC2 BRAF CHGA ENO2 GFAP
3 nervous system MP:0003631 9.61 BRAF ENO2 GFAP MAP2 SMARCB1 SYP
4 neoplasm MP:0002006 9.55 BRAF SMARCB1 TP53 TSC1 TSC2
5 reproductive system MP:0005389 9.17 BRAF CHGA MAP2 SMARCB1 TP53 TSC1

Drugs & Therapeutics for Ganglioglioma

Drugs for Ganglioglioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 16)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dabrafenib Approved Phase 2 44462760 44516822
2
Trametinib Approved Phase 2 871700-17-3 11707110
3 Protein Kinase Inhibitors Phase 2
4
Bevacizumab Approved, Investigational Phase 1 216974-75-3
5
Irinotecan Approved, Investigational Phase 1 97682-44-5, 100286-90-6 60838
6
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
7
Camptothecin Experimental Phase 1 7689-03-4
8 Angiogenesis Inhibitors Phase 1
9 Angiogenesis Modulating Agents Phase 1
10 Antineoplastic Agents, Phytogenic Phase 1
11 topoisomerase I inhibitors Phase 1
12 Topoisomerase Inhibitors Phase 1
13 Alkylating Agents Phase 1
14 Anesthetics Phase 1
15 Antirheumatic Agents Phase 1
16 Immunosuppressive Agents Phase 1

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Antineoplaston Therapy in Treating Children With Brain Tumors Completed NCT00003458 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
2 Proton Radiotherapy for Pediatric Brain Tumors Requiring Partial Brain Irradiation Active, not recruiting NCT01288235 Phase 2
3 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG Not yet recruiting NCT02684058 Phase 2 dabrafenib;trametinib
4 Evaluation of FDOPA-PET/MRI in Pediatric Patients With CNS Tumors Completed NCT01999270 Phase 1 Irinotecan;Bevacizumab
5 A Study of the Treatment of Recurrent Malignant Glioma With rQNestin34.5v.2 Recruiting NCT03152318 Phase 1 rQNestin34.5v.2;Cyclophosphamide
6 Astrocytoma / Desmoplastic Gamliogliomes (DIA / DIG) - Study of the French Cohort of the Last 20 Years : Clinical, Anatomopathological, Molecular and Radiological Charactersics Recruiting NCT03115164

Search NIH Clinical Center for Ganglioglioma

Cochrane evidence based reviews: ganglioglioma

Genetic Tests for Ganglioglioma

Anatomical Context for Ganglioglioma

MalaCards organs/tissues related to Ganglioglioma:

39
Brain, Temporal Lobe, Spinal Cord, Cerebellum, Pineal, Cortex, Thalamus

Publications for Ganglioglioma

Articles related to Ganglioglioma:

(show top 50) (show all 458)
id Title Authors Year
1
Malignant transformation and leptomeningeal spread of recurrent ganglioglioma: case report and review of literature. ( 28950177 )
2017
2
Ganglioglioma of brain stem and cervicomedullary junction: A 50years review of literature. ( 28687443 )
2017
3
Dentatorubral-pallidoluysian atrophy (DRPLA) with a small ganglioglioma component containing neurofibrillary tangles and polyglutamine aggregation. ( 28144989 )
2017
4
Ganglioglioma Arising From Desmoplastic Medulloblastoma: A Case Report and Review of Literature. ( 28232638 )
2017
5
Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature. ( 27668845 )
2017
6
Longitudinal mutational analysis of a cerebellar pilocytic astrocytoma recurring as a ganglioglioma. ( 27718322 )
2017
7
Atypical Presentation of a Pediatric Cerebellar Ganglioglioma. ( 28926844 )
2017
8
Ex vivo tissue imaging for radiology-pathology correlation: a pilot study with a small bore 7-T MRI in a rare pigmented ganglioglioma exhibiting complex MR signal characteristics associated with melanin and hemosiderin. ( 28924575 )
2017
9
Ganglioglioma progression to combined anaplastic ganglioglioma and anaplastic pleomorphic xanthoastrocytoma. Case report and literature review. ( 28939541 )
2017
10
A rare association of ganglioglioma and cavernous malformation: Report of two cases and literature review. ( 28607828 )
2017
11
Intraventricular ganglioglioma with extensive hemorrhage. ( 28406082 )
2017
12
Discrepancy between radiological and histological findings in ganglioglioma of the optic chasm: Case report. ( 28781923 )
2017
13
Hemorrhagic ganglioglioma of the third ventricle with atypical pathological findings. ( 28624861 )
2017
14
Parkin, PINK1 and DJ1 as possible modulators of mTOR pathway in ganglioglioma. ( 28803490 )
2017
15
miR-217-casein kinase-2 cross talk regulates ERK activation in ganglioglioma. ( 28840260 )
2017
16
Growth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma. ( 27068013 )
2016
17
Application of diffusion-weighted imaging and dynamic susceptibility contrast perfusion-weighted imaging for ganglioglioma in adults: Comparison study with oligodendroglioma. ( 27502573 )
2016
18
Ganglioglioma of conus medullaris in a patient of neurofibromatosis type 1: A novel association? ( 27695570 )
2016
19
Combined pleomorphic xanthoastrocytoma-ganglioglioma with BRAF V600E mutation: case report. ( 27015517 )
2016
20
Desmoplastic infantile astrocytoma/ganglioglioma with rare BRAF V600D mutation. ( 27860162 )
2016
21
Ganglioglioma: Single-institutional experience of 24 cases with review of literature. ( 27695546 )
2016
22
Infratentorial ganglioglioma mimicking pilocytic astrocytoma. ( 27936528 )
2016
23
Co-occurrence of Histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma. ( 27984673 )
2016
24
Pediatric intracranial primary anaplastic ganglioglioma. ( 27921213 )
2016
25
Ganglioglioma of the posterior third ventricle region: An unusual pathology in an uncommon location. ( 26954825 )
2016
26
Response to Journal Club: Pediatric Low-Grade Ganglioglioma: Epidemiology, Treatments, and Outcome Analysis of 348 Children From the Surveillance, Epidemiology, and End Results Database. ( 27428632 )
2016
27
K27M mutation in H3F3A in ganglioglioma grade I with spontaneous malignant transformation extends the histopathological spectrum of the histone H3 oncogenic pathway. ( 27219822 )
2016
28
Bilateral internal auditory canal gangliogliomas mimicking neurofibromatosis Type II. ( 27127704 )
2016
29
Successful Retreatment of a Child with a Refractory Brainstem Ganglioglioma with Vemurafenib. ( 26579623 )
2016
30
Ganglioglioma arising from olfactory sheath: A rare site of an uncommon tumor. ( 26874814 )
2016
31
Clinical Neuropathology image 5-2016: nNeurofibrillary tangle-rich ganglioglioma. ( 27546017 )
2016
32
Personalized Treatment for a Patient With a BRAF V600E Mutation Using Dabrafenib and a Tumor Treatment Fields Device in a High-Grade Glioma Arising From Ganglioglioma. ( 27799506 )
2016
33
Progression of Atypical Extraventricular Neurocytoma to Anaplastic Ganglioglioma. ( 27597523 )
2016
34
Genetic Characterization of a Multifocal Ganglioglioma Originating Within the Spinal Cord. ( 27671879 )
2016
35
Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. ( 27659826 )
2016
36
Concurrent Intraventricular and Sacral Spinal Drop Metastasis of Ganglioglioma in an Adult Patient: A Case Report and Review of Literature. ( 27158568 )
2016
37
Desmoplastic infantile ganglioglioma with focal cortical dysplasia: A rare double pathology in an infant with history of seizures. ( 26991995 )
2016
38
Unusual ganglioglioma with extensive calcification and ossification. ( 27510688 )
2016
39
Recurrent ganglioglioma in adults treated with BRAF inhibitors. ( 26680369 )
2016
40
Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient. ( 27127715 )
2016
41
Serial imaging and management of ganglioglioma with unusual presentation and meningeal spread. ( 27853879 )
2016
42
Newly diagnosed ganglioglioma in an adult patient with multiple sclerosis. ( 27653865 )
2016
43
Diffusion, Perfusion, and Histopathologic Characteristics of Desmoplastic Infantile Ganglioglioma. ( 27761184 )
2016
44
Abundance of LRP12 C-rs9694676 allelic promoter variant in epilepsy-associated gangliogliomas. ( 27142828 )
2016
45
Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma. ( 27539475 )
2016
46
Ganglioglioma in the Third Ventricle: A Case Report and Literature Review. ( 28664007 )
2016
47
Journal Club: Pediatric Low-Grade Ganglioglioma: Epidemiology, Treatments, and Outcome Analysis on 348 Children From the Surveillance, Epidemiology, and End Results Database. ( 27428631 )
2016
48
Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course. ( 26431248 )
2015
49
Pigmented ganglioglioma in a patient with chronic epilepsy and cortical dysplasia. ( 26432496 )
2015
50
Ganglioglioma of the Spinal Cord. ( 26605127 )
2015

Variations for Ganglioglioma

Cosmic variations for Ganglioglioma:

9
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM99759 IDH2 central nervous system,temporal lobe,other,ganglioglioma c.492G>A p.W164* 9
2 COSM476 BRAF central nervous system,brain,other,ganglioglioma c.1799T>A p.V600E 9
3 COSM5702591 NF2 central nervous system,brain,other,neoplasm c.575A>G p.Y192C 3
4 COSM28746 IDH1 central nervous system,brain,other,neoplasm c.395G>A p.R132H 3
5 COSM28747 IDH1 central nervous system,brain,other,neoplasm c.394C>T p.R132C 3
6 COSM28749 IDH1 central nervous system,brain,other,neoplasm c.394C>G p.R132G 3
7 COSM28748 IDH1 central nervous system,brain,other,neoplasm c.394C>A p.R132S 3
8 COSM28750 IDH1 central nervous system,brain,other,neoplasm c.395G>T p.R132L 3
9 COSM5702574 FOXO3 central nervous system,brain,other,neoplasm c.583A>T p.K195* 3
10 COSM5702592 central nervous system,brain,other,neoplasm c.575A>G p.Y192C 3

Expression for Ganglioglioma

Search GEO for disease gene expression data for Ganglioglioma.

Pathways for Ganglioglioma

GO Terms for Ganglioglioma

Cellular components related to Ganglioglioma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 cell body GO:0044297 8.65 BRAF
2 TSC1-TSC2 complex GO:0033596 8.62 TSC1 TSC2

Biological processes related to Ganglioglioma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 negative regulation of cell proliferation GO:0008285 9.56 SMARCB1 TP53 TSC1 TSC2
2 cell cycle arrest GO:0007050 9.33 TP53 TSC1 TSC2
3 regulation of neuronal synaptic plasticity GO:0048168 8.96 S100B SYP
4 long-term synaptic potentiation GO:0060291 8.62 BRAF S100B

Molecular functions related to Ganglioglioma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.02 BRAF GFAP S100B SYP TP53
2 small GTPase binding GO:0031267 8.96 BRAF TSC2

Sources for Ganglioglioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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