MCID: GNG005
MIFTS: 41

Gangliocytoma malady

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Gangliocytoma

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Aliases & Descriptions for Gangliocytoma:

Name: Gangliocytoma 11 48 54 13
 
Ganglioneuroma 39 68

Classifications:



External Ids:

Disease Ontology11 DOID:2426
MeSH39 D005729
NCIt45 C3049, C6934
Orphanet54 ORPHA251937
ICD10 via Orphanet31 D36.1

Summaries for Gangliocytoma

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NIH Rare Diseases:48 Gangliocytoma is a rare type of central nervous system (CNS) tumor made up of mature neurons. Gangliocytomas may occur in all age groups but most often occur in people between the ages of 10 and 30. The most common site is the temporal lobe of the brain, but they can arise anywhere in the CNS including the cerebellum, brainstem, floor of the third ventricle, and spinal cord. They are among the most frequent tumors associated with epilepsy. Signs and symptoms may depend on the tumor's location and may include seizures (most commonly); increased brain pressure; endocrine disorders; and focal symptoms. Gangliocytomas are generally slow-growing and usually do not become malignant. Treatment involves surgical removal of the tumor.Click here to view a separate page about dysplastic gangliocytoma of the cerebellum (also called Lhermitte-Duclose disease). Last updated: 5/11/2015

MalaCards based summary: Gangliocytoma, also known as ganglioneuroma, is related to ganglioneuroma and cutaneous ganglioneuroma, and has symptoms including seizures, Array and Array. An important gene associated with Gangliocytoma is C9orf72 (Chromosome 9 Open Reading Frame 72), and among its related pathways are Neural Stem Cell Differentiation Pathways and Lineage-specific Markers and Neuroscience. Affiliated tissues include pituitary, cerebellum and temporal lobe, and related mouse phenotypes are behavior/neurological and nervous system.

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

Related Diseases for Gangliocytoma

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

(show top 50)    (show all 111)
idRelated DiseaseScoreTop Affiliating Genes
1ganglioneuroma12.4
2cutaneous ganglioneuroma12.1
3cowden syndrome 111.6
4adenoma10.3
5pituitary adenoma10.2
6perry syndrome10.2C9orf72, TH
7hypogonadism cardiomyopathy10.2SYP, TH
8uterine corpus epithelioid leiomyosarcoma10.2GFAP, PTEN
9space motion sickness10.2SYP, TH
10early myoclonic encephalopathy10.1GFAP, PTEN
11grubben de cock borghgraef syndrome10.1GFAP, SYP
12papillary glioneuronal tumor10.1GFAP, SYP
13bladder flat intraepithelial lesion10.1GFAP, SYP
14lice infestation10.1GFAP, SYP
15compartment syndrome10.1GFAP, PTEN
16chiari malformation type 410.1GFAP, SYP
17cauda equina neoplasm10.1GFAP, SYP
18brain glioma10.1GFAP, SYP
19vulvar benign neoplasm10.1GFAP, SYP
20hypersensitivity reaction type ii disease10.1GFAP, SYP
21brainstem intraparenchymal clear cell meningioma10.1GFAP, SYP
22jewett-marshall bladder cancer10.1GFAP, SYP
23lumbar plexus neoplasm10.1GFAP, SYP
24yolk sac tumor of central nervous system10.1GFAP, SYP
25pilomyxoid astrocytoma10.1GFAP, SYP
26choroid disease10.1GFAP, SYP
27subpulmonary stenosis10.1GFAP, SYP
28vitamin metabolic disorder10.1GFAP, SYP
29pineoblastoma10.1GFAP, SYP
30intracystic papillary adenoma10.1GFAP, SYP
31malignant granular cell esophageal tumor10.1GFAP, SYP
32gallbladder adenoma10.1GFAP, SYP
33congenital hypogammaglobulinemia10.1GFAP, SYP
34ependymoblastoma10.1GFAP, SYP
35diencephalic astrocytomas10.1GFAP, SYP
36pineal parenchymal tumor of intermediate differenciation10.1GFAP, SYP
37mantle cell lymphoma10.1GFAP, PTEN
38pulmonary plasma cell granuloma10.1GFAP, SYP
39iodine hypothyroidism10.1GFAP, SYP
40central centrifugal cicatricial alopecia10.1GFAP, SYP
41neurilemmoma of the fifth cranial nerve10.1GFAP, SYP
42peritoneal benign neoplasm10.1GFAP, SYP
43mesenchymoma10.0GFAP, SYP
44renal tubular acidosis, distal, autosomal recessive10.0GFAP, PTEN
45central nervous system sarcoma10.0GFAP, SYP
46acromegaly10.0
47adult central nervous system primitive neuroectodermal neoplasm10.0GFAP, SYP
48mixed epithelial stromal tumour10.0GFAP, SYP
49gallbladder leiomyosarcoma10.0GFAP, SYP
50grade iii astrocytoma10.0GFAP, SYP

Graphical network of the top 20 diseases related to Gangliocytoma:



Diseases related to gangliocytoma

Symptoms & Phenotypes for Gangliocytoma

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Human phenotypes related to Gangliocytoma:

 54 (show all 26)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ganglioneuroma54 Very frequent (99-80%)
2 headache54 Frequent (79-30%)
3 spinal cord tumor54 Frequent (79-30%)
4 hemianopsia54 Frequent (79-30%)
5 neoplasm of the central nervous system54 Frequent (79-30%)
6 amenorrhea54 Occasional (29-5%)
7 impotence54 Occasional (29-5%)
8 growth hormone excess54 Occasional (29-5%)
9 hyperhidrosis54 Occasional (29-5%)
10 abnormality of brainstem morphology54 Occasional (29-5%)
11 distal muscle weakness54 Occasional (29-5%)
12 scoliosis54 Occasional (29-5%)
13 paresthesia54 Occasional (29-5%)
14 accelerated skeletal maturation54 Occasional (29-5%)
15 focal seizures54 Occasional (29-5%)
16 adrenocorticotropic hormone excess54 Occasional (29-5%)
17 pituitary null cell adenoma54 Occasional (29-5%)
18 abnormality of the pituitary gland54 Occasional (29-5%)
19 decreased female libido54 Occasional (29-5%)
20 abnormal prolactin level54 Occasional (29-5%)
21 dementia54 Very rare (<4-1%)
22 somnolence54 Very rare (<4-1%)
23 abnormality of the cerebellum54 Very rare (<4-1%)
24 polyphagia54 Very rare (<4-1%)
25 syringomyelia54 Very rare (<4-1%)
26 pituitary prolactin cell adenoma54 Very rare (<4-1%)

UMLS symptoms related to Gangliocytoma:


seizures

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

41
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053868.7C9orf72, GFAP, PTEN, SYP, TH
2MP:00036317.9C9orf72, GFAP, PTEN, SYP, TH

Drugs & Therapeutics for Gangliocytoma

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

(show all 8)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
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
2
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
3
CamptothecinexperimentalPhase 16997689-03-4
Synonyms:
(+)-camptothecin
(+)-camptothecine
(s)-(+)-camptothecin
 
(s)-camptothecin
20(S)-Camptothecin
21,22-Secocamptothecin-21-oic acid lactone
Camptothecine
D-camptothecin
4Topoisomerase InhibitorsPhase 14945
5topoisomerase I inhibitorsPhase 11328
6Angiogenesis Modulating AgentsPhase 14072
7Antineoplastic Agents, PhytogenicPhase 15420
8Angiogenesis InhibitorsPhase 14143

Interventional clinical trials:

idNameStatusNCT IDPhase
1Evaluation of FDOPA-PET/MRI in Pediatric Patients With CNS TumorsCompletedNCT01999270Phase 1
2Prospective Study Registry of Peripheral Neuroblastic Tumours Presenting With Spinal Canal Involvement (SCI)RecruitingNCT02559804

Search NIH Clinical Center for Gangliocytoma


Cochrane evidence based reviews: ganglioneuroma

Genetic Tests for Gangliocytoma

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Anatomical Context for Gangliocytoma

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MalaCards organs/tissues related to Gangliocytoma:

36
Pituitary, Cerebellum, Temporal lobe, Spinal cord, Brain, Medulla oblongata, Pineal

Publications for Gangliocytoma

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Articles related to Gangliocytoma:

(show top 50)    (show all 117)
idTitleAuthorsYear
1
Transdifferentiation of Neuroendocrine Cells: Gangliocytoma Associated With Two Pituitary Adenomas of Different Lineage in MEN1. (28079577)
2017
2
Mixed Gangliocytoma-Pituitary Adenoma: Insights on the Pathogenesis of a Rare Sellar Tumor. (28079576)
2017
3
Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature. (27057227)
2016
4
Gangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report. (27932167)
2016
5
A Rare Case of Concomitant Intramedullary Gangliocytoma at the Cervicomedullary Junction in Patient with Neuroendocrine Tumor of Lung. (26962423)
2016
6
Growth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma. (27068013)
2016
7
Mixed Pituitary Gangliocytoma and Prolactinoma Resistant to the Cabergoline Treatment. (27535625)
2016
8
Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma. (26314658)
2015
9
Autopsy case of undiagnosed gangliocytoma in the medulla oblongata complicated with cerebral palsy. (26210504)
2015
10
Pituitary adenoma with gangliocytoma: Report of two cases. (25013498)
2014
11
Infrasellar pituitary gangliocytoma causing Cushing's syndrome. (25183169)
2014
12
Endoscopic approach to a collision tumor of growth hormone-secreting adenoma and gangliocytoma in the pituitary gland. (25006908)
2014
13
Dysplastic cerebellar gangliocytoma lhermitte-duclos disease imaging and magnetic resonance spectroscopy. (25369219)
2014
14
A familial frontotemporal dementia associated with C9orf72 repeat expansion and dysplastic gangliocytoma. (24080172)
2014
15
Cushing's disease due to mixed pituitary adenoma-gangliocytoma of the posterior pituitary gland presenting with Aspergillus sp. sinus infection. (23611590)
2013
16
Acromegaly associated with mixed pituitary adenoma-gangliocytoma and Rathke's cleft cyst. (24101276)
2013
17
Gangliocytoma of the medulla oblongata demonstrating high tracer uptake on positron emission tomography. (21732072)
2012
18
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) as a component of Cowden syndrome. (22499315)
2012
19
Intramedullary gangliocytoma with calcification and multiple intramedullary cysts. (22538840)
2012
20
Unusual presentation in adult medulloblastomas: imaging features mimicking cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease). (23135053)
2012
21
Acromegaly associated with gangliocytoma. (19789938)
2012
22
Combined gangliocytoma and non-functioning pituitary adenoma of the pituitary gland. (22824692)
2012
23
Ossifying pituitary adenoma co-existing with astrocytoma and pituitary adenoma associated with gangliocytoma: two unusual conditions. (22274987)
2012
24
Direct correlation between the facial nerve nucleus and hemifacial seizures associated with a gangliocytoma of the floor of the fourth ventricle: a case report. (22050334)
2011
25
Assessing a dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) with 7T MR imaging. (20191074)
2010
26
Medulloblastoma with extensive nodularity undergoing post-therapeutic maturation to a gangliocytoma: a case report and literature review. (21389751)
2010
27
Cytomorphologic spectrum of mixed pituitary adenoma-gangliocytomas: a report of two cases. (21053582)
2010
28
Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). (19332858)
2009
29
Subfrontal gangliocytoma masquerading as olfactory groove meningioma. (19234914)
2009
30
An intrasellar mixed gangliocytoma-adenoma including ependymal component, and review of the literature. (21686419)
2009
31
From glioblastoma to gangliocytoma: an unforeseen but welcome shift in biological behavior. (19877783)
2009
32
Collision tumor of anaplastic oligodendroglioma and gangliocytoma: a case report. (19856221)
2009
33
An unusual association of a sellar gangliocytoma with a prolactinoma. (17440820)
2008
34
Cerebellar gangliocytoma presenting with hemifacial spasms: clinical report, literature review and possible mechanisms. (18509586)
2008
35
Combined gangliocytoma and prolactinoma of the pituitary gland. (18651251)
2008
36
Gangliocytoma associated with focal cortical dysplasia in a young-adult: a case report. (18814115)
2008
37
Gangliocytoma of the spinal cord. (17460525)
2007
38
Diffuse cerebral gangliocytoma in an adult with late-onset refractory epilepsy. (17990996)
2007
39
Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report. (18095141)
2007
40
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). (16934474)
2006
41
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma). (15670528)
2005
42
Pathologic quiz case: an unusual cause of acromegaly. Intrasellar gangliocytoma. (15737044)
2005
43
Acromegaly due to a growth hormone-releasing hormone-secreting intracranial gangliocytoma. (15887863)
2005
44
Intramedullary spinal cord gangliocytoma: case report and a review of the literature. (15739067)
2005
45
Radiology case of the month. Gait disturbance. Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). (15554090)
2004
46
Posterior fossa gangliocytoma with facial nerve invasion: case report. (12806511)
2003
47
Dysplastic gangliocytoma of the cerebellum: rare differential diagnosis in space occupying lesions of the posterior fossa. (12836078)
2003
48
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? (11886354)
2002
49
Mixed gangliocytoma--adenoma of the sella: case report. (12500383)
2002
50
Medulloblastoma or cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease)? (12504212)
2002

Variations for Gangliocytoma

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Cosmic variations for Gangliocytoma:

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.V600E3
2COSM99759IDH2central nervous system,temporal lobe,other,gangliogliomac.492G>Ap.W164*3

Expression for genes affiliated with Gangliocytoma

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Search GEO for disease gene expression data for Gangliocytoma.

Pathways for genes affiliated with Gangliocytoma

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Pathways related to Gangliocytoma according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
18.8GFAP, SYP, TH
28.0GFAP, RBFOX3, SYP, TH

GO Terms for genes affiliated with Gangliocytoma

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

idNameGO IDScoreTop Affiliating Genes
1cell projectionGO:004299510.2GFAP, PTEN
2cytoplasmic side of plasma membraneGO:000989810.2PTEN, TH
3synaptic vesicleGO:00080219.8SYP, TH
4neuron projectionGO:00430059.4PTEN, SYP, TH
5terminal boutonGO:00431959.3SYP, TH

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

idNameGO IDScoreTop Affiliating Genes
1locomotory behaviorGO:000762610.1PTEN, TH
2long-term synaptic potentiationGO:006029110.1GFAP, PTEN
3memoryGO:000761310.0PTEN, TH
4response to zinc ionGO:001004310.0PTEN, TH
5social behaviorGO:00351769.5PTEN, TH

Sources for Gangliocytoma

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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