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 47 53 13
 
Ganglioneuroma 38 67

Classifications:



External Ids:

Disease Ontology11 DOID:2426
MeSH38 D005729
NCIt44 C3049, C6934
Orphanet53 ORPHA251937
ICD10 via Orphanet30 D36.1

Summaries for Gangliocytoma

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Wikipedia:70 Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently... more...

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.

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 for Gangliocytoma

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Human phenotypes related to Gangliocytoma:

 53 (show all 26)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ganglioneuroma53 Very frequent (99-80%)
2 headache53 Frequent (79-30%)
3 spinal cord tumor53 Frequent (79-30%)
4 hemianopsia53 Frequent (79-30%)
5 neoplasm of the central nervous system53 Frequent (79-30%)
6 amenorrhea53 Occasional (29-5%)
7 impotence53 Occasional (29-5%)
8 growth hormone excess53 Occasional (29-5%)
9 hyperhidrosis53 Occasional (29-5%)
10 abnormality of brainstem morphology53 Occasional (29-5%)
11 distal muscle weakness53 Occasional (29-5%)
12 scoliosis53 Occasional (29-5%)
13 paresthesia53 Occasional (29-5%)
14 accelerated skeletal maturation53 Occasional (29-5%)
15 focal seizures53 Occasional (29-5%)
16 adrenocorticotropic hormone excess53 Occasional (29-5%)
17 pituitary null cell adenoma53 Occasional (29-5%)
18 abnormality of the pituitary gland53 Occasional (29-5%)
19 decreased female libido53 Occasional (29-5%)
20 abnormal prolactin level53 Occasional (29-5%)
21 dementia53 Very rare (<4-1%)
22 somnolence53 Very rare (<4-1%)
23 abnormality of the cerebellum53 Very rare (<4-1%)
24 polyphagia53 Very rare (<4-1%)
25 syringomyelia53 Very rare (<4-1%)
26 pituitary prolactin cell adenoma53 Very rare (<4-1%)

UMLS symptoms related to Gangliocytoma:


seizures

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:

35
Pituitary, Cerebellum, Temporal lobe, Spinal cord, Brain, Medulla oblongata, Pineal

Animal Models for Gangliocytoma or affiliated genes

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MGI Mouse Phenotypes related to Gangliocytoma:

40
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053868.7C9orf72, GFAP, PTEN, SYP, TH
2MP:00036317.9C9orf72, GFAP, PTEN, SYP, TH

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
Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature. (27057227)
2016
3
Gangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report. (27932167)
2016
4
Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma. (26314658)
2015
5
Cushing's disease due to mixed pituitary adenoma-gangliocytoma of the posterior pituitary gland presenting with Aspergillus sp. sinus infection. (23611590)
2013
6
Gangliocytoma of the medulla oblongata demonstrating high tracer uptake on positron emission tomography. (21732072)
2012
7
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) as a component of Cowden syndrome. (22499315)
2012
8
Intramedullary gangliocytoma with calcification and multiple intramedullary cysts. (22538840)
2012
9
Unusual presentation in adult medulloblastomas: imaging features mimicking cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease). (23135053)
2012
10
Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). (19332858)
2009
11
Subfrontal gangliocytoma masquerading as olfactory groove meningioma. (19234914)
2009
12
An unusual association of a sellar gangliocytoma with a prolactinoma. (17440820)
2008
13
Gangliocytoma of the spinal cord. (17460525)
2007
14
Diffuse cerebral gangliocytoma in an adult with late-onset refractory epilepsy. (17990996)
2007
15
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). (16934474)
2006
16
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma). (15670528)
2005
17
Radiology case of the month. Gait disturbance. Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). (15554090)
2004
18
Posterior fossa gangliocytoma with facial nerve invasion: case report. (12806511)
2003
19
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? (11886354)
2002
20
Mixed gangliocytoma--adenoma of the sella: case report. (12500383)
2002
21
Gangliocytoma of the spinal cord: a case report. (11373931)
2001
22
Mixed gangliocytoma/pituitary adenoma. (10768636)
2000
23
Anaplastic gangliocytoma with eosinophilic cytoplasmic granules in a cow. (10487247)
1999
24
Gangliocytoma masquerading as a prolactinoma. Case report. (10470826)
1999
25
Lhermitte-Duclos disease (cerebellar gangliocytoma). (8969359)
1996
26
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). (7656495)
1995
27
Intracranial gangliocytoma in a dog. (7771061)
1995
28
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) and its relation to the multiple hamartoma syndrome (Cowden disease). (7964980)
1994
29
Combined sellar gangliocytoma and pituitary adenoma in acromegaly or Cushing's disease. A report of 3 cases. (7921420)
1994
30
MRI of gangliocytoma of cerebellum and spinal cord. (8491919)
1993
31
Parvalbumin and calbindin D-28K immunoreactivity in central ganglioglioma and dysplastic gangliocytoma of the cerebellum. Report of two cases. (8416231)
1993
32
Holoprosencephaly, hamartomatous growth of the cerebrum, dysplastic gangliocytoma of the cerebellum, unique brain anomalies, and renal agenesis in a Brazilian infant born to a diabetic mother: a clinical and pathologic study. (8280891)
1993
33
Recurrent dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) presenting with subarachnoid haemorrhage. (1590970)
1992
34
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease)--a case report with review of the literature. (1473839)
1992
35
Intramedullary secretory gangliocytoma. (1767634)
1991
36
Gastrin in a hypothalamic gangliocytoma of an acromegalic patient. (2347089)
1990
37
Mixed gangliocytoma-adenoma: a distinct neuroendocrine tumor of the pituitary fossa. (2591950)
1989
38
Intrasellar gangliocytoma containing gastrin and growth hormone-releasing hormone associated with a growth hormone-secreting pituitary adenoma. (2512034)
1989
39
Gangliocytoma of the cerebellum: ultrastructure and immunohistochemistry. (3362326)
1988
40
Dysplastic gangliocytoma. (3419579)
1988
41
MR and CT characteristics of gangliocytoma: a rare cause of epilepsy in children. (3140636)
1988
42
Computerized tomographic (CT) and magnetic resonance imaging (MRI) appearance of a cerebral gangliocytoma. (3564519)
1987
43
Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). Case report. (3723183)
1986
44
Hypothalamic gangliocytoma. Selective appearance of neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies. (3022544)
1986
45
Gangliocytoma of the pineal body. A case report and review of the literature. (3872555)
1985
46
Dysplastic gangliocytoma of cerebellum in a newborn. Case report. (6707754)
1984
47
Clinical and pathological study of dysplastic gangliocytoma. Case report. (7252559)
1981
48
A Golgi and electron microscopic study of a dysplastic gangliocytoma of the cerebellum. (474077)
1979
49
Dysplastic gangliocytoma of the cerebellum--an ultrastructural study. (207069)
1978
50
Dysplastic gangliocytoma (Lhermitte-Duclos disease) of the cerebellum. Case report. (903815)
1977

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
1cytoplasmic side of plasma membraneGO:00098989.9PTEN, TH
2synaptic vesicleGO:00080219.6SYP, TH
3cell projectionGO:00429959.5GFAP, PTEN
4terminal boutonGO:00431959.4SYP, TH
5neuron projectionGO:00430059.0PTEN, SYP, TH

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

idNameGO IDScoreTop Affiliating Genes
1response to zinc ionGO:00100439.8PTEN, TH
2long-term synaptic potentiationGO:00602919.6GFAP, PTEN
3memoryGO:00076139.6PTEN, TH
4locomotory behaviorGO:00076269.5PTEN, TH
5social behaviorGO:00351769.2PTEN, TH

Sources for Gangliocytoma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
26GTR
27HGMD
28HMDB
29ICD10
30ICD10 via Orphanet
31ICD9CM
32IUPHAR
33KEGG
36MedGen
38MeSH
39MESH via Orphanet
40MGI
43NCI
44NCIt
45NDF-RT
48NINDS
49Novoseek
51OMIM
52OMIM via Orphanet
56PubMed
57QIAGEN
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
67UMLS
68UMLS via Orphanet