MCID: GNG004
MIFTS: 49

Ganglioglioma malady

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Ganglioglioma

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Sources:
11Disease Ontology, 13DISEASES, 39MeSH, 45NCIt, 48NIH Rare Diseases, 50Novoseek, 54Orphanet, 62SNOMED-CT, 68UMLS, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Ganglioglioma:

Name: Ganglioglioma 11 48 54 50 39 13 68
Childhood Ganglioglioma 11 68
Adult Ganglioglioma 11 68
 
Mixed Cell Tumors Containing Both Neural Ganglionic Cells and Neural Glial Cell Components 48
Cns Ganglioglioma 11

Classifications:



External Ids:

Disease Ontology11 DOID:5078
MeSH39 D018303
Orphanet54 ORPHA251949
UMLS via Orphanet69 C0206716

Summaries for Ganglioglioma

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NIH Rare Diseases:48 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 pilocytic astrocytoma, and has symptoms including seizures, seizures and seizures. An important gene associated with Ganglioglioma is SYP (Synaptophysin), and among its related pathways are Thyroid cancer and p53 Pathway (RnD). Affiliated tissues include temporal lobe, brain and spinal cord, and related mouse phenotypes are Increased proliferation and normal.

Disease Ontology:11 A cell type benign neoplasm that has material basis in glial-type cells.

Wikipedia:71 Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently... more...

Related Diseases for Ganglioglioma

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

(show top 50)    (show all 243)
idRelated DiseaseScoreTop Affiliating Genes
1pleomorphic xanthoastrocytoma30.6BRAF, TP53
2pilocytic astrocytoma30.5CHGA, SYP
3papillary glioneuronal tumor30.2GFAP, SYP
4anaplastic oligodendroglioma30.1GFAP, NES
5giant cell glioblastoma29.0GFAP, NES, SYP, TP53
6anaplastic ganglioglioma12.0
7desmoplastic infantile ganglioglioma12.0
8desmoplastic infantile astrocytoma/ganglioglioma11.9
9nasal ganglioglioma11.9
10gangliocytoma11.2
11grubben de cock borghgraef syndrome10.6GFAP, SYP
12bladder flat intraepithelial lesion10.6GFAP, SYP
13cauda equina neoplasm10.6GFAP, SYP
14glomus tumor10.6GFAP, SYP
15choroid disease10.6GFAP, SYP
16vitamin metabolic disorder10.6GFAP, SYP
17empty sella syndrome10.6GFAP, NES
18malignant granular cell esophageal tumor10.6GFAP, SYP
19ovary mixed epithelial carcinoma10.6CHGA, SYP
20esophagus small cell carcinoma10.6CHGA, SYP
21childhood malignant schwannoma10.5CHGA, SYP
22microglandular adenosis of breast10.5CHGA, SYP
23clear cell adenoma10.5CHGA, SYP
24cervical alveolar soft part sarcoma10.5GFAP, NES
25focal facial dermal dysplasia10.5CHGA, SYP
26punctate porokeratosis10.5CHGA, SYP
27osteoblastoma10.5CHGA, SYP
28urethra adenocarcinoma10.5CHGA, SYP
29esophagus adenocarcinoma10.5CHGA, SYP
30froelich syndrome10.5CHGA, SYP
31cutaneous mastocytosis10.5CHGA, SYP
32pancreatic serous cystic neoplasm10.5CHGA, SYP
33parachordoma10.5SYP, TSC2
34congenital structural myopathy10.5CHGA, GFAP
35pneumonia10.5CHGA, SYP
36verruciform xanthoma of skin10.5CHGA, SYP
37ovarian clear cell malignant adenofibroma10.5CHGA, SYP
38acanthoma10.5CHGA, SYP
39rectum signet ring adenocarcinoma10.4CHGA, SYP
40anaplastic oligoastrocytoma10.4S100B, SYP
41cranial nerve iii tumor10.4CHGA, SYP
42lyme disease - neurological complications10.4GFAP, S100B
43tricuspid valve stenosis10.4CHGA, SYP
44hypogonadism cardiomyopathy10.4S100B, SYP
45pineoblastoma10.4BRAF, GFAP, SYP
46chondroblastoma10.4GFAP, S100B
47buschke-ollendorff syndrome10.4TSC1, TSC2
48subacute leukemia10.4GFAP, S100B
49schwannoma of ureter10.4CHGA, SYP
50inflammatory mfh10.4S100B, SYP

Graphical network of the top 20 diseases related to Ganglioglioma:



Diseases related to ganglioglioma

Symptoms & Phenotypes for Ganglioglioma

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UMLS symptoms related to Ganglioglioma:


seizures

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

26
idDescriptionGenomeRNAi Source AccessionScoreTop Affiliating Genes
1GR00094-A10.0SMARCB1, TP53

MGI Mouse Phenotypes related to Ganglioglioma according to GeneCards Suite gene sharing:

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00028738.9BRAF, GFAP, S100B, SYP, TP53, TSC2
2MP:00053708.6BRAF, SMARCB1, TP53, TSC1, TSC2
3MP:00020068.6BRAF, SMARCB1, TP53, TSC1, TSC2
4MP:00053858.0BRAF, CHGA, GFAP, SMARCB1, TP53, TSC1
5MP:00036317.7BRAF, GFAP, MAP2, SMARCB1, SYP, TP53
6MP:00053787.6BRAF, CHGA, GFAP, MAP2, SMARCB1, TP53
7MP:00053867.3BRAF, GFAP, MAP2, S100B, SMARCB1, SYP
8MP:00053897.1BRAF, CHGA, MAP2, SMARCB1, TP53, TSC1

Drugs & Therapeutics for Ganglioglioma

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

(show all 10)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
DabrafenibapprovedPhase 27944462760, 44516822
Synonyms:
BRAF inhibitor GSK2118436
Dabrafenib
 
GSK2118436
GSK2118436a
Tafinlar
dabrafenib
2Protein Kinase InhibitorsPhase 23612
3
Irinotecanapproved, investigationalPhase 1108097682-44-5, 100286-90-660838
Synonyms:
(+)-Irinotecan
(4S)-4,11-DIETHYL-4-HYDROXY-3,14-DIOXO-3,4,12,14-TETRAHYDRO-1H-PYRANO[3',4':6,7]INDOLIZINO[1,2-B]QUINOLIN-9-YL 1,4'-BIPIPERIDINE-1'-CARBOXYLATE
(4S)-4,11-Diethyl-4-hydroxy-3,14-dioxo-4,12-dihydro-1H-pyrano[3,4-f]quinolino[2,3-a]indolizin-9-yl 4-piperidylpiperidinecarboxylate
1u65
97682-44-5
AC-7469
AC1L1U0Z
AC1Q6PGI
BRD-K08547377-003-02-4
BSPBio_002346
Bio-0054
Biotecan
Biotecan (TN)
C16641
C33H38N4O6
CHEBI:105985
CHEMBL481
CID60838
CP0
Campothecin-11
Campto
Camptosar
 
Camptosar, Campto, CPT-11, Irinotecan
Camptothecin-11
D08086
DB00762
FT-0083650
HSDB 7607
IRINOTECAN HYDROCHLORIDE Trihydrate
IRINOTECAN, CPT-11
Irinotecan (INN)
Irinotecan Hcl
Irinotecan Hydrochloride
Irinotecan Hydrochloride Trihydrate
Irinotecan [INN:BAN]
Irinotecan hydrochloride
Irinotecanum
Irinotecanum [INN-Latin]
LS-44589
NCI60_005051
NSC728073
S1198_Selleck
TL8006026
UNII-7673326042
irinotecan
4
Bevacizumabapproved, investigationalPhase 11968216974-75-3
Synonyms:
216974-75-3
Avastin
Avastin (TN)
Bevacizumab
Bevacizumab (genetical recombination)
 
Bevacizumab (genetical recombination) (JAN)
D06409
R-435
anti-VEGF monoclonal antibody
antiVEGF
bevacizumab
5
CamptothecinexperimentalPhase 16997689-03-4
Synonyms:
(+)-camptothecin
(+)-camptothecine
(s)-(+)-camptothecin
 
(s)-camptothecin
20(S)-Camptothecin
21,22-Secocamptothecin-21-oic acid lactone
Camptothecine
D-camptothecin
6topoisomerase I inhibitorsPhase 11328
7Topoisomerase InhibitorsPhase 14945
8Antineoplastic Agents, PhytogenicPhase 15420
9Angiogenesis InhibitorsPhase 14143
10Angiogenesis Modulating AgentsPhase 14072

Interventional clinical trials:

idNameStatusNCT IDPhase
1Antineoplaston Therapy in Treating Children With Brain TumorsCompletedNCT00003458Phase 2
2Proton Radiotherapy for Pediatric Brain Tumors Requiring Partial Brain IrradiationRecruitingNCT01288235Phase 2
3Phase II Pediatric Study With Dabrafenib in HGG PatientsNot yet recruitingNCT02684058Phase 2
4Evaluation of FDOPA-PET/MRI in Pediatric Patients With CNS TumorsCompletedNCT01999270Phase 1

Search NIH Clinical Center for Ganglioglioma


Cochrane evidence based reviews: ganglioglioma

Genetic Tests for Ganglioglioma

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Anatomical Context for Ganglioglioma

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MalaCards organs/tissues related to Ganglioglioma:

36
Temporal lobe, Brain, Spinal cord, Cerebellum, Pineal, Cortex, Thalamus

Publications for Ganglioglioma

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Articles related to Ganglioglioma:

(show top 50)    (show all 443)
idTitleAuthorsYear
1
Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature. (27668845)
2017
2
Longitudinal mutational analysis of a cerebellar pilocytic astrocytoma recurring as a ganglioglioma. (27718322)
2017
3
Successful Retreatment of a Child with a Refractory Brainstem Ganglioglioma with Vemurafenib. (26579623)
2016
4
Desmoplastic infantile ganglioglioma with focal cortical dysplasia: A rare double pathology in an infant with history of seizures. (26991995)
2016
5
Recurrent ganglioglioma in adults treated with BRAF inhibitors. (26680369)
2016
6
Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. (27659826)
2016
7
Clinical Neuropathology image 5-2016: nNeurofibrillary tangle-rich ganglioglioma. (27546017)
2016
8
Co-occurrence of Histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma. (27984673)
2016
9
Progression of Atypical Extraventricular Neurocytoma to Anaplastic Ganglioglioma. (27597523)
2016
10
Ganglioglioma of conus medullaris in a patient of neurofibromatosis type 1: A novel association? (27695570)
2016
11
Pediatric intracranial primary anaplastic ganglioglioma. (27921213)
2016
12
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
13
Application of diffusion-weighted imaging and dynamic susceptibility contrast perfusion-weighted imaging for ganglioglioma in adults: Comparison study with oligodendroglioma. (27502573)
2016
14
Combined pleomorphic xanthoastrocytoma-ganglioglioma with BRAF V600E mutation: case report. (27015517)
2016
15
Journal Club: Pediatric Low-Grade Ganglioglioma: Epidemiology, Treatments, and Outcome Analysis on 348 Children From the Surveillance, Epidemiology, and End Results Database. (27428631)
2016
16
Serial imaging and management of ganglioglioma with unusual presentation and meningeal spread. (27853879)
2016
17
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
18
Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma. (27539475)
2016
19
K27M mutation in H3F3A in ganglioglioma grade I with spontaneous malignant transformation extends the histopathological spectrum of the histone H3 oncogenic pathway. (27219822)
2016
20
Ganglioglioma arising from olfactory sheath: A rare site of an uncommon tumor. (26874814)
2016
21
Growth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma. (27068013)
2016
22
Concurrent Intraventricular and Sacral Spinal Drop Metastasis of Ganglioglioma in an Adult Patient: A Case Report and Review of Literature. (27158568)
2016
23
Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient. (27127715)
2016
24
Unusual ganglioglioma with extensive calcification and ossification. (27510688)
2016
25
Ganglioglioma of the posterior third ventricle region: An unusual pathology in an uncommon location. (26954825)
2016
26
Diffusion, Perfusion, and Histopathologic Characteristics of Desmoplastic Infantile Ganglioglioma. (27761184)
2016
27
Bilateral internal auditory canal gangliogliomas mimicking neurofibromatosis Type II. (27127704)
2016
28
Desmoplastic infantile astrocytoma/ganglioglioma with rare BRAF V600D mutation. (27860162)
2016
29
Newly diagnosed ganglioglioma in an adult patient with multiple sclerosis. (27653865)
2016
30
Genetic Characterization of a Multifocal Ganglioglioma Originating Within the Spinal Cord. (27671879)
2016
31
Ganglioglioma: Single-institutional experience of 24 cases with review of literature. (27695546)
2016
32
Infratentorial ganglioglioma mimicking pilocytic astrocytoma. (27936528)
2016
33
Non-infantile variant of desmoplastic ganglioglioma: Conventional and advanced MR imaging characteristics. (26246092)
2015
34
Recurrent papillary glioneuronal tumor presenting as a ganglioglioma with the BRAF V600E mutation. (26073619)
2015
35
Pigmented ganglioglioma in a patient with chronic epilepsy and cortical dysplasia. (26432496)
2015
36
Differential expression and clinical significance of three inflammation-related microRNAs in gangliogliomas. (25986346)
2015
37
Desmoplastic infantile ganglioglioma with associated giant aneurysma89case report. (25922053)
2015
38
Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course. (26431248)
2015
39
Coexistent ganglioglioma, focal cortical dysplasia, and hippocampal sclerosis (triple pathology) in chronic epilepsy. (26235882)
2015
40
An Unusual Case of Constitutional Mismatch Repair Deficiency Syndrome With Anaplastic Ganglioglioma, Colonic Adenocarcinoma, Osteosarcoma, Acute Myeloid Leukemia, and Signs of Neurofibromatosis Type 1: Case Report. (25850602)
2015
41
Anaplastic ganglioglioma: a report of three cases and review of the literature. (25862009)
2015
42
Ganglioglioma of the Spinal Cord. (26605127)
2015
43
Suggestive value of predilection site and imaging features of pediatric brainstem ganglioglioma including a case report. (25665772)
2015
44
Dysplastic Cerebellar Epilepsy: Complete Seizure Control Following Resection of a Ganglioglioma. (26208704)
2015
45
Bilateral Optic Pathway Ganglioglioma: The Fifth Case in the Literature. (26488468)
2015
46
Pediatric low-grade ganglioglioma: epidemiology, treatments, and outcome analysis on 348 children from the surveillance, epidemiology, and end results database. (25603107)
2015
47
BRAF V600E mutation in neocortical posterior temporal epileptogenic gangliogliomas. (25937573)
2015
48
Pediatric infratentorial ganglioglioma. (26351224)
2015
49
Pediatric brainstem gangliogliomas show BRAF(V600E) mutation in a high percentage of cases. (24238153)
2014
50
Pediatric posterior fossa ganglioglioma: unique MRI features and correlation with BRAF V600E mutation status. (24792487)
2014

Variations for Ganglioglioma

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Cosmic variations for Ganglioglioma:

8
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1COSM476BRAFcentral nervous system,temporal lobe,other,gangliogliomac.1799T>Ap.V600E8
2COSM99759IDH2central nervous system,temporal lobe,other,gangliogliomac.492G>Ap.W164*8

Expression for genes affiliated with Ganglioglioma

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Search GEO for disease gene expression data for Ganglioglioma.

Pathways for genes affiliated with Ganglioglioma

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Pathways related to Ganglioglioma according to GeneCards Suite gene sharing:

(show all 13)
idSuper pathwaysScoreTop Affiliating Genes
19.5BRAF, TP53
2
Show member pathways
9.2TP53, TSC1, TSC2
3
Show member pathways
9.2TP53, TSC1, TSC2
49.2TP53, TSC1, TSC2
5
Show member pathways
9.1BRAF, MAP2, TP53
69.1GFAP, MAP2, S100B, SYP
7
Show member pathways
8.8SMARCB1, TSC1, TSC2
88.8MAP2, TP53, TSC1, TSC2
98.7BRAF, TP53, TSC1, TSC2
108.7BRAF, TP53, TSC1, TSC2
11
Show member pathways
8.7BRAF, TP53, TSC1, TSC2
12
Show member pathways
8.7BRAF, TP53, TSC1, TSC2
138.5GFAP, MAP2, NES, S100B, SYP

GO Terms for genes affiliated with Ganglioglioma

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

idNameGO IDScoreTop Affiliating Genes
1TSC1-TSC2 complexGO:00335969.6TSC1, TSC2

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

idNameGO IDScoreTop Affiliating Genes
1negative regulation of insulin receptor signaling pathwayGO:004662710.1TSC1, TSC2
2negative regulation of TOR signalingGO:003200710.0TSC1, TSC2
3long-term synaptic potentiationGO:00602919.9BRAF, GFAP, S100B
4cell cycle arrestGO:00070509.8TP53, TSC1, TSC2
5regulation of neuronal synaptic plasticityGO:00481689.8S100B, SYP
6negative regulation of cell proliferationGO:00082858.9SMARCB1, TP53, TSC1, TSC2

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

idNameGO IDScoreTop Affiliating Genes
1small GTPase bindingGO:00312679.6BRAF, TSC2
2protein bindingGO:00055155.8BRAF, CHGA, GFAP, MAP2, NES, S100B

Sources for Ganglioglioma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet