MCID: GNG004
MIFTS: 46

Ganglioglioma malady

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Ganglioglioma

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Sources:
11Disease Ontology, 46NIH Rare Diseases, 13DISEASES, 52Orphanet, 48Novoseek, 37MeSH, 66UMLS, 43NCIt, 67UMLS via Orphanet, 60SNOMED-CT
See all MalaCards sources

Aliases & Descriptions for Ganglioglioma:

Name: Ganglioglioma 11 46 13 52 48 37 66
Childhood Ganglioglioma 11 66
Adult Ganglioglioma 11 66
 
Mixed Cell Tumors Containing Both Neural Ganglionic Cells and Neural Glial Cell Components 46
Cns Ganglioglioma 11

Classifications:



External Ids:

Disease Ontology11 DOID:5078
MeSH37 D018303
Orphanet52 ORPHA251949
UMLS via Orphanet67 C0206716

Summaries for Ganglioglioma

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NIH Rare Diseases:46 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 papillary glioneuronal tumor, 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 LKB1 signaling events. Affiliated tissues include brain, temporal lobe and spinal cord, and related mouse phenotypes are reproductive system and normal.

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

Wikipedia:69 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 215)
idRelated DiseaseScoreTop Affiliating Genes
1pleomorphic xanthoastrocytoma31.3BRAF, TP53
2papillary glioneuronal tumor30.6GFAP, SYP
3anaplastic oligodendroglioma30.4GFAP, NES
4pilocytic astrocytoma30.0CHGA, SYP
5giant cell glioblastoma29.4GFAP, NES, SYP, TP53
6anaplastic ganglioglioma12.3
7desmoplastic infantile ganglioglioma12.1
8nasal ganglioglioma12.0
9desmoplastic infantile astrocytoma/ganglioglioma12.0
10grubben de cock borghgraef syndrome10.7GFAP, SYP
11bladder flat intraepithelial lesion10.7GFAP, SYP
12cauda equina neoplasm10.7GFAP, SYP
13pulmonary venoocclusive disease10.7BRAF, GFAP
14glomus tumor10.7GFAP, SYP
15split hand split foot malformation autosomal recessive10.7BRAF, TP53
16choroid disease10.7GFAP, SYP
17vitamin metabolic disorder10.7GFAP, SYP
18epicardium cancer10.7BRAF, GFAP
19malignant granular cell esophageal tumor10.7GFAP, SYP
20skin meningioma10.7GFAP, TP53
21glomeruloid hemangioma10.7GFAP, TP53
22gallbladder adenoma10.6GFAP, SYP
23multiple carboxylase deficiency10.6BRAF, TP53
24pineal parenchymal tumor of intermediate differenciation10.6GFAP, SYP
25infiltrating lipoma10.6GFAP, TP53
26renal pelvis inverted papilloma10.6GFAP, TP53
27vulva fibroepithelial polyp10.6SYP, TP53
28external ear disease10.6GFAP, TP53
29childhood central nervous system primitive neuroectodermal neoplasm10.6GFAP, SMARCB1
30adult teratoma10.6SYP, TP53
31mediastinum teratoma10.6BRAF, TP53
32ganglioglioma10.6GFAP, TP53
33skin glomangioma10.6SYP, TP53
34gangliocytoma10.6
35parachordoma10.6SYP, TSC2
36colonic pseudo-obstruction10.6GFAP, SMARCB1
37empty sella syndrome10.6GFAP, NES
38large cell carcinoma10.6SMARCB1, SYP
39bone remodeling disease10.6SYP, TP53
40intrahepatic bile duct cystadenoma10.6GFAP, TP53
41immune system organ benign neoplasm10.6SYP, TP53
42benign mastocytoma10.5SYP, TP53
43cervical alveolar soft part sarcoma10.5GFAP, NES
44vibratory urticaria10.5SYP, TP53
45central centrifugal cicatricial alopecia10.5GFAP, SYP
46adult mesenchymal chondrosarcoma10.5SMARCB1, SYP
47cervical adenoid cystic carcinoma10.5GFAP, TP53
48l-cell glucagon-like peptide producing tumor10.5SYP, TP53
49early myoclonic encephalopathy10.4GFAP, TP53
50sialolithiasis10.4GFAP, TP53

Graphical network of the top 20 diseases related to Ganglioglioma:



Diseases related to ganglioglioma

Symptoms for Ganglioglioma

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UMLS symptoms related to Ganglioglioma:


seizures

Drugs & Therapeutics for Ganglioglioma

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

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
DabrafenibPhase 27544462760, 44516822
Synonyms:
BRAF inhibitor GSK2118436
Dabrafenib
 
GSK2118436
GSK2118436a
Tafinlar
dabrafenib
2topoisomerase I inhibitorsPhase 01313
3
IrinotecanPhase 0106297682-44-5, 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
CamptothecinPhase 05697689-03-4
Synonyms:
(+)-camptothecin
(+)-camptothecine
(s)-(+)-camptothecin
 
(s)-camptothecin
20(S)-Camptothecin
21,22-Secocamptothecin-21-oic acid lactone
Camptothecine
D-camptothecin
5
BevacizumabPhase 01938216974-75-3
Synonyms:
216974-75-3
Avastin
Avastin (TN)
Bevacizumab
Bevacizumab (genetical recombination)
 
Bevacizumab (genetical recombination) (JAN)
D06409
R-435
anti-VEGF monoclonal antibody
antiVEGF
bevacizumab

Interventional clinical trials:

idNameStatusNCT IDPhase
1Proton Radiotherapy for Pediatric Brain Tumors Requiring Partial Brain IrradiationRecruitingNCT01288235Phase 2
2Phase II Pediatric Study With Dabrafenib in HGG PatientsNot yet recruitingNCT02684058Phase 2
3Evaluation of FDOPA-PET/MRI in Pediatric Patients With CNS TumorsCompletedNCT01999270Phase 0

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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:

34
Brain, Temporal lobe, Spinal cord, Cerebellum, Pineal, Cortex, Thalamus

Animal Models for Ganglioglioma or affiliated genes

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MGI Mouse Phenotypes related to Ganglioglioma:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053898.3BRAF, CHGA, MAP2, SMARCB1, TP53, TSC2
2MP:00028738.2BRAF, DBH, GFAP, SYP, TP53, TSC2
3MP:00053797.6BRAF, CHGA, DBH, MAP2, TP53, TSC2
4MP:00036317.5BRAF, DBH, GFAP, MAP2, SMARCB1, SYP
5MP:00053857.4BRAF, CHGA, DBH, GFAP, SMARCB1, TP53
6MP:00053867.4BRAF, DBH, GFAP, MAP2, SMARCB1, SYP
7MP:00053786.8BRAF, CHGA, DBH, GFAP, MAP2, SMARCB1
8MP:00107686.4BRAF, CHGA, DBH, GFAP, MAP2, NSD1

Publications for Ganglioglioma

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Articles related to Ganglioglioma:

(show top 50)    (show all 431)
idTitleAuthorsYear
1
Successful Retreatment of a Child with a Refractory Brainstem Ganglioglioma with Vemurafenib. (26579623)
2016
2
Desmoplastic infantile ganglioglioma with focal cortical dysplasia: A rare double pathology in an infant with history of seizures. (26991995)
2016
3
Recurrent ganglioglioma in adults treated with BRAF inhibitors. (26680369)
2016
4
Non-infantile variant of desmoplastic ganglioglioma: Conventional and advanced MR imaging characteristics. (26246092)
2015
5
Pediatric brainstem gangliogliomas show BRAF(V600E) mutation in a high percentage of cases. (24238153)
2014
6
Ganglioglioma mimicking the cerebral abscess in advanced age: a case report. (24101283)
2013
7
Hemorrhagic ganglioglioma of the posterior fossa: case report. (24192758)
2013
8
Mutant BRAF V600E protein in ganglioglioma is predominantly expressed by neuronal tumor cells. (23435618)
2013
9
BRAF(V600E) mutation is a negative prognosticator in pediatric ganglioglioma. (23609006)
2013
10
Ganglioglioma with anaplastic transformation. (22990737)
2012
11
Primary malignant ganglioglioma of the dorsolumbar spine (D11-L1). (20817466)
2010
12
A report of a desmoplastic non-infantile ganglioglioma in a 6-year-old boy with review of the literature. (19280238)
2009
13
Desmoplastic infantile ganglioglioma with a malignant course. (18352812)
2008
14
Desmoplastic ganglioglioma with meningiomatous and myofibroblastic components presenting with epilepsy. (17961139)
2008
15
Anaplastic ganglioglioma in a middle-aged woman: a case report with a review of the literature. (17923741)
2007
16
Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. (17386372)
2007
17
Ganglioglioma in the third ventricle: report on two cases. (17492319)
2007
18
Spinal cord ganglioglioma presenting as acute paraparesis. (15946791)
2006
19
Anaplastic ganglioglioma of the cerebellopontine angle. Case report. (16308520)
2005
20
Cyanotic breath-holding spell: a life-threatening complication after radical resection of a cervicomedullary ganglioglioma. (15942280)
2005
21
Desmoplastic infantile ganglioglioma with high proliferation index: report of a case. (16519002)
2005
22
Conventional MR imaging with simultaneous measurements of cerebral blood volume and vascular permeability in ganglioglioma. (15172052)
2004
23
Residents' corner. Answer to case of the month #96. Right frontal lobe ganglioglioma. (15131935)
2004
24
Abnormal behavior in children with temporal lobe epilepsy and ganglioglioma. (15380137)
2004
25
Desmoplastic infantile astrocytoma and ganglioglioma: a search for genomic characteristics. (12111357)
2002
26
Desmoplastic infantile ganglioglioma: a potentially malignant tumor? (12409729)
2002
27
Congenital ganglioglioma. (12003306)
2002
28
Ganglioglioma of the lateral ventricle presenting with blepharospasm - case report and review of the literature. (11386809)
2001
29
Intraventricular trigonal ganglioglioma arising from the choroid plexus. (11685629)
2001
30
Ganglioglioma in a patient with Turcot syndrome. Case report. (10616098)
2000
31
Coexistence of a ganglioglioma and Rasmussen's encephalitis. (10461077)
1999
32
Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation? (10352415)
1999
33
Synaptophysin staining for ganglioglioma. (10219424)
1999
34
Intraventricular ganglioglioma: case report. (9689626)
1998
35
MR of spinal cord ganglioglioma. (9613504)
1998
36
Synaptophysin staining in normal brain: importance for diagnosis of ganglioglioma. (9591724)
1998
37
Anaplastic ganglioglioma: case report and review of the literature. (7984264)
1994
38
An immunohistochemical study of peripheral neuroblastoma, ganglioneuroblastoma, anaplastic ganglioglioma, schwannoma and neurofibroma in cattle. (7962722)
1994
39
Chronic neurological dysfunction attributable to a ganglioglioma. (1398525)
1992
40
Hemifacial spasm in an infant due to fourth ventricular ganglioglioma. Case report. (2045898)
1991
41
Intracranial ganglioglioma: MR imaging. (2119137)
1990
42
Brain ganglioglioma with large extension to subarachnoid space. (2401916)
1990
43
Cerebellar ganglioglioma. Case report. (2092096)
1990
44
Ganglioglioma containing osseous tissue and neurofibrillary tangles. (3132128)
1988
45
Ganglioglioma, a malignant tumor? Correlation with flow deoxyribonucleic acid cytometric analysis. (3226518)
1988
46
Ganglioglioma of the spinal cord in a calf. (3576915)
1987
47
Intractable facial pain associated with a ganglioglioma of the cervicomedullary junction: report of a case. (7099412)
1982
48
Cerebellar ganglioglioma. Case report. (479941)
1979
49
Ganglioglioma of the medulla oblongata. (451854)
1979
50
Ganglioglioma of the cerebellum: angiographic findings. (951566)
1976

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,brain,other,gangliogliomac.1799T>Ap.V600E0
2COSM28746IDH1central nervous system,brain,other,neoplasmc.395G>Ap.R132H0
3COSM28747IDH1central nervous system,brain,other,neoplasmc.394C>Tp.R132C0
4COSM28749IDH1central nervous system,brain,other,neoplasmc.394C>Gp.R132G0
5COSM28748IDH1central nervous system,brain,other,neoplasmc.394C>Ap.R132S0
6COSM28750IDH1central nervous system,brain,other,neoplasmc.395G>Tp.R132L0
7COSM99759IDH2central nervous system,temporal lobe,other,gangliogliomac.492G>Ap.W164*0

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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GO Terms for genes affiliated with Ganglioglioma

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

idNameGO IDScoreTop Affiliating Genes
1small GTPase bindingGO:00312679.5BRAF, TSC2

Sources for Ganglioglioma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet