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 10 45 12 51
 
Ganglioneuroma 36 65

Classifications:



External Ids:

Disease Ontology10 DOID:2426
MeSH36 D005729
NCIt42 C3049, C6934
Orphanet51 251937
UMLS65 C0017075

Summaries for Gangliocytoma

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NIH Rare Diseases:45 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 adult medulloblastoma and ganglioneuroma, and has symptoms including seizures An important gene associated with Gangliocytoma is C9orf72 (Chromosome 9 Open Reading Frame 72), and among its related pathways is Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. Affiliated tissues include cerebellum, temporal lobe and spinal cord, and related mouse phenotypes are cellular and normal.

Wikipedia:68 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 99)
idRelated DiseaseScoreTop Affiliating Genes
1adult medulloblastoma29.3GFAP, SYP
2ganglioneuroma12.4
3cutaneous ganglioneuroma12.2
4cowden disease11.5
5cowden syndrome 110.9
6adenoma10.4
7pituitary adenoma10.3
8perry syndrome10.3C9orf72, TH
9acromegaly10.2
10cerebral neuroblastoma10.2SYP, TH
11cerebritis10.1
12ganglioglioma10.1
13prolactinoma10.0
14ischemic neuropathy10.0SYP, TH
15kidney cancer10.0SYP, TH
16anaplastic ganglioglioma10.0GFAP, PTEN
17medulloblastoma10.0
18male reproductive organ benign neoplasm10.0CRH, SYP
19organ system cancer10.0PTEN, SYP
20bronchitis9.9GFAP, PTEN
21urinary bladder villous adenoma9.9GFAP, SYP
22pediculus humanus capitis infestation9.9GFAP, SYP
23childhood pilocytic astrocytoma9.9GFAP, PTEN
24hyperglobulinemic purpura9.9CRH, TH
25complement component 3 deficiency9.9GFAP, SYP
26dementia9.9
27neuroendocrine tumor9.9
28astrocytoma9.9
29glioblastoma9.9
30adult malignant schwannoma9.9GFAP, SYP
31syphilis9.9GFAP, SYP
32cerebellar medulloblastoma9.9GFAP, SYP
33anovulation9.9GFAP, SYP
34childhood infratentorial neoplasm9.9GFAP, SYP
35benign ependymoma9.9GFAP, SYP
36mixed glioma9.9GFAP, SYP
37familial primary hypomagnesemia with hypercalciuria and nephrocalcinosis with severe ocular involvement9.9GFAP, SYP
38space motion sickness9.9GFAP, SYP
39adult choroid plexus cancer9.9GFAP, SYP
40pilomyxoid astrocytoma9.9GFAP, SYP
41pinheiro freire-maia miranda syndrome9.9GFAP, SYP
42bilateral hyperactive labyrinth9.9GFAP, SYP
43lung benign neoplasm9.9GFAP, SYP
44myoblastoma9.9GFAP, SYP
45pilocytic astrocytoma of cerebellum9.8GFAP, SYP
46melanotic neuroectodermal tumor9.8GFAP, SYP
47tanycytic ependymoma9.8GFAP, PTEN
48sympathetic neurilemmoma9.8GFAP, SYP
49papillary ependymoma9.8GFAP, SYP
50renal tubular acidosis, distal, autosomal recessive9.8GFAP, PTEN

Graphical network of the top 20 diseases related to Gangliocytoma:



Diseases related to gangliocytoma

Symptoms for Gangliocytoma

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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 7)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Irinotecanapproved, investigationalPhase 0104497682-44-5, 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
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 01875216974-75-3
Synonyms:
216974-75-3
Avastin
Avastin (TN)
Bevacizumab
Bevacizumab (genetical recombination)
 
Bevacizumab (genetical recombination) (JAN)
D06409
R-435
anti-VEGF monoclonal antibody
antiVEGF
bevacizumab
3Topoisomerase InhibitorsPhase 04081
4topoisomerase I inhibitorsPhase 01131
5Angiogenesis Modulating AgentsPhase 03611
6Angiogenesis InhibitorsPhase 03688
7Antineoplastic Agents, PhytogenicPhase 04294

Interventional clinical trials:

idNameStatusNCT IDPhase
1Evaluation of FDOPA-PET/MRI in Pediatric Patients With CNS TumorsRecruitingNCT01999270Phase 0
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:

33
Cerebellum, Temporal lobe, Spinal cord, Brain, Pituitary, Lung, Breast

Animal Models for Gangliocytoma or affiliated genes

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MGI Mouse Phenotypes related to Gangliocytoma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053847.7C9orf72, CRH, GFAP, PTEN, TH
2MP:00028737.7CRH, GFAP, PTEN, SYP, TH
3MP:00053867.0C9orf72, CRH, GFAP, PTEN, SYP, TH
4MP:00036316.8C9orf72, CRH, GFAP, PTEN, SYP, TH

Publications for Gangliocytoma

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Articles related to Gangliocytoma:

(show top 50)    (show all 114)
idTitleAuthorsYear
1
Growth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma. (27068013)
2016
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
Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma. (26314658)
2015
4
Infrasellar pituitary gangliocytoma causing Cushing's syndrome. (25183169)
2014
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
Combined gangliocytoma and non-functioning pituitary adenoma of the pituitary gland. (22824692)
2012
8
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) as a component of Cowden syndrome. (22499315)
2012
9
Intramedullary gangliocytoma with calcification and multiple intramedullary cysts. (22538840)
2012
10
Unusual presentation in adult medulloblastomas: imaging features mimicking cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease). (23135053)
2012
11
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
12
Assessing a dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) with 7T MR imaging. (20191074)
2010
13
Medulloblastoma with extensive nodularity undergoing post-therapeutic maturation to a gangliocytoma: a case report and literature review. (21389751)
2010
14
Cerebellar gangliocytoma presenting with hemifacial spasms: clinical report, literature review and possible mechanisms. (18509586)
2008
15
An unusual association of a sellar gangliocytoma with a prolactinoma. (17440820)
2008
16
Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report. (18095141)
2007
17
Pathologic quiz case: an unusual cause of acromegaly. Intrasellar gangliocytoma. (15737044)
2005
18
Acromegaly due to a growth hormone-releasing hormone-secreting intracranial gangliocytoma. (15887863)
2005
19
Radiology case of the month. Gait disturbance. Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). (15554090)
2004
20
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? (11886354)
2002
21
Malignant transformation of a gangliocytoma/ganglioglioma into a glioblastoma multiforme: a molecular genetic analysis. Case report. (11453385)
2001
22
Mixed pituitary adenoma-gangliocytoma in a female albino rat. (11307628)
2001
23
Pituitary gangliocytoma-adenoma presenting with acromegaly: response to treatment. (11295968)
2001
24
Supratentorial gangliocytoma mimicking extra-axial tumor: a report of two cases. (11752979)
2001
25
Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). (11516554)
2001
26
Gangliocytoma of the spinal cord: a case report. (11373931)
2001
27
Lhermitte-Duclos disease (cerebellar gangliocytoma). (8969359)
1996
28
Malignant occipital astrocytoma in a patient with Lhermitte-Duclos disease (cerebellar dysplastic gangliocytoma). (8672269)
1996
29
Gangliocytoma of the cervicothoracic spinal cord. (7611066)
1995
30
The striated cerebellum: an MR imaging sign in Lhermitte-Duclos disease (dysplastic gangliocytoma). (7862965)
1995
31
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) and its relation to the multiple hamartoma syndrome (Cowden disease). (7964980)
1994
32
Cerebral and cerebellar gangliocytomas: a morphological study of nine cases. (7810295)
1994
33
MRI of gangliocytoma of cerebellum and spinal cord. (8491919)
1993
34
Cushing's disease in a child caused by a corticotropin-releasing hormone-secreting intrasellar gangliocytoma associated with an adrenocorticotropic hormone-secreting pituitary adenoma. (8264895)
1993
35
Parvalbumin and calbindin D-28K immunoreactivity in central ganglioglioma and dysplastic gangliocytoma of the cerebellum. Report of two cases. (8416231)
1993
36
Intramedullary secretory gangliocytoma. (1767634)
1991
37
Mixed pituitary adenoma and gangliocytoma associated with acromegaly--case report. (1703643)
1990
38
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). Report of a case and review of the literature. (2239379)
1990
39
Gastrin in a hypothalamic gangliocytoma of an acromegalic patient. (2347089)
1990
40
Dysplastic gangliocytoma and intractable partial seizures in childhood. (2927689)
1989
41
Gangliocytoma of the cerebellum: ultrastructure and immunohistochemistry. (3362326)
1988
42
Magnetic resonance imaging in the diagnosis and treatment of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum). (3185886)
1988
43
Dysplastic gangliocytoma. (3419579)
1988
44
Cerebral gangliocytoma. An ultrastructural study. (2823521)
1987
45
Computerized tomographic (CT) and magnetic resonance imaging (MRI) appearance of a cerebral gangliocytoma. (3564519)
1987
46
Orthostatic hypotension from a cerebellar gangliocytoma (Lhermitte-Duclos disease). Case report. (3723183)
1986
47
Clinical and pathological study of dysplastic gangliocytoma. Case report. (7252559)
1981
48
Gangliocytoma of third ventricle: hyperphagia, somnolence, and dementia. (6955631)
1981
49
Cerebellar gangliocytoma dysplasticum in an infant. (724022)
1978
50
Diffuse amyelinic cerebellar gangliocytoma. Report of case verified at autopsy. (13696023)
1960

Variations for Gangliocytoma

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Cosmic variations for Gangliocytoma:

7
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Conf
1COSM28746IDH1central nervous system,brain,other,gangliocytoma6
2COSM476BRAFcentral nervous system,temporal lobe,other,ganglioglioma5
3COSM327928H3F3Acentral nervous system,spinal cord,other,ganglioglioma4

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
18.5GFAP, SYP, TH

GO Terms for genes affiliated with Gangliocytoma

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

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1response to zinc ionGO:00100439.8PTEN, TH
2response to etherGO:00454729.7CRH, TH
3response to corticosteroneGO:00514129.7CRH, TH
4response to ethanolGO:00454719.7CRH, PTEN
5response to immobilization stressGO:00359029.6CRH, TH
6response to organic cyclic compoundGO:00140709.5PTEN, TH
7synaptic transmission, dopaminergicGO:00019639.4CRH, TH
8memoryGO:00076139.4PTEN, TH
9long-term synaptic potentiationGO:00602919.2CRH, PTEN
10response to drugGO:00424939.0CRH, PTEN, TH

Sources for Gangliocytoma

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet