MCID: GNG005
MIFTS: 45

Gangliocytoma

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Gangliocytoma

Summaries for Gangliocytoma

NIH Rare Diseases : 50 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 anaplastic oligodendroglioma and ganglioneuroma, and has symptoms including scoliosis, headache and focal seizures. An important gene associated with Gangliocytoma is PTEN (Phosphatase And Tensin Homolog), and among its related pathways/superpathways are Circadian rythm related genes and Neuroscience. The drugs Bevacizumab and Irinotecan have been mentioned in the context of this disorder. Affiliated tissues include cerebellum, temporal lobe and pituitary, and related phenotypes are behavior/neurological and nervous system

Wikipedia : 72 Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently... more...

Related Diseases for Gangliocytoma

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

(show top 50) (show all 115)
id Related Disease Score Top Affiliating Genes
1 anaplastic oligodendroglioma 28.8 GFAP PTEN SYP
2 ganglioneuroma 12.3
3 cutaneous ganglioneuroma 12.0
4 cowden disease 11.3
5 cowden syndrome 1 11.0
6 hypogonadotropic hypogonadism without anosmia, x-linked 10.4 SYP TH
7 sm-ahnmd 10.4 SYP TH
8 perry syndrome 10.4 C9orf72 TH
9 invasive malignant thymoma 10.4 PTEN SYP
10 adenoma 10.3
11 transitional cell cancer of the renal pelvis and ureter 10.3 PTEN SYP
12 pituitary adenoma 10.2
13 early-onset parkinson disease 10.2 CRH SYP
14 olfactory nerve neoplasm 10.2 RBFOX3 SYP
15 osteoclast-like giant cell neoplasm of the pancreas 10.2 RBFOX3 SYP
16 pleomorphic xanthoastrocytoma 10.2 RBFOX3 SYP
17 postmenopausal atrophic vaginitis 10.2 SYP TH
18 brachyolmia 10.1 PTEN SYP
19 cystadenoma 10.1 SYP TH
20 myoblastoma 10.1 RBFOX3 SYP
21 vestibular gland benign neoplasm 10.1 CRH SYP
22 acromegaly 10.1
23 ovarian mucinous cystadenofibroma 10.1 GFAP PTEN
24 devriendt syndrome 10.0 GFAP SYP
25 hypogonadotropic hypogonadism-frontoparietal alopecia syndrome 10.0 GFAP SYP
26 dystonia 10.0 GFAP SYP
27 spinal cord ependymoma 10.0 GFAP SYP
28 chilaiditi syndrome 10.0 GFAP SYP
29 adrenal cortical adenocarcinoma 10.0 CRH SYP
30 cerebellar astrocytoma 10.0 GFAP SYP
31 lymphopenia 10.0 GFAP PTEN
32 reticular pattern testicular yolk sac tumor 10.0 GFAP SYP
33 hypogonadotropic hypogonadism-retinitis pigmentosa syndrome 10.0 GFAP SYP
34 brain glioma 10.0 GFAP SYP
35 cauda equina intradural extramedullary astrocytoma 10.0 GFAP SYP
36 adenofibroma 10.0 GFAP SYP
37 rh isoimmunization 10.0 GFAP SYP
38 cerebritis 10.0
39 ganglioglioma 10.0
40 pleural empyema 10.0 GFAP SYP
41 cauda equina neoplasm 10.0 GFAP SYP
42 axonal neuropathy 10.0 GFAP SYP
43 malignant granular cell myoblastoma 9.9 GFAP SYP
44 behcet's syndrome arthropathy 9.9 GFAP SYP
45 space motion sickness 9.9 GFAP SYP
46 sudden sensorineural hearing loss 9.9 GFAP SYP
47 papillary glioneuronal tumor 9.9 GFAP SYP
48 central neurocytoma 9.9 GFAP SYP
49 pituitary dwarfism with large sella turcica 9.9 GFAP SYP
50 pneumothorax 9.9 GFAP SYP

Graphical network of the top 20 diseases related to Gangliocytoma:



Diseases related to Gangliocytoma

Symptoms & Phenotypes for Gangliocytoma

Human phenotypes related to Gangliocytoma:

56 32 (show all 28)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 scoliosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0002650
2 headache 56 32 frequent (33%) Frequent (79-30%) HP:0002315
3 focal seizures 56 32 occasional (7.5%) Occasional (29-5%) HP:0007359
4 amenorrhea 56 32 occasional (7.5%) Occasional (29-5%) HP:0000141
5 syringomyelia 56 32 very rare (1%) Very rare (<4-1%) HP:0003396
6 dementia 56 32 very rare (1%) Very rare (<4-1%) HP:0000726
7 distal muscle weakness 56 32 occasional (7.5%) Occasional (29-5%) HP:0002460
8 hyperhidrosis 56 32 occasional (7.5%) Occasional (29-5%) HP:0000975
9 impotence 56 32 occasional (7.5%) Occasional (29-5%) HP:0000802
10 paresthesia 56 32 occasional (7.5%) Occasional (29-5%) HP:0003401
11 ganglioneuroma 56 32 hallmark (90%) Very frequent (99-80%) HP:0003005
12 polyphagia 56 32 very rare (1%) Very rare (<4-1%) HP:0002591
13 growth hormone excess 56 32 occasional (7.5%) Occasional (29-5%) HP:0000845
14 pituitary prolactin cell adenoma 56 32 very rare (1%) Very rare (<4-1%) HP:0006767
15 pituitary null cell adenoma 56 32 occasional (7.5%) Occasional (29-5%) HP:0011761
16 abnormality of the cerebellum 56 32 very rare (1%) Very rare (<4-1%) HP:0001317
17 accelerated skeletal maturation 56 32 occasional (7.5%) Occasional (29-5%) HP:0005616
18 spinal cord tumor 56 32 frequent (33%) Frequent (79-30%) HP:0010302
19 abnormality of brainstem morphology 56 32 occasional (7.5%) Occasional (29-5%) HP:0002363
20 adrenocorticotropic hormone excess 56 32 occasional (7.5%) Occasional (29-5%) HP:0011749
21 decreased female libido 56 32 occasional (7.5%) Occasional (29-5%) HP:0030018
22 abnormal prolactin level 56 32 occasional (7.5%) Occasional (29-5%) HP:0040086
23 somnolence 56 Very rare (<4-1%)
24 hemianopsia 56 Frequent (79-30%)
25 neoplasm of the central nervous system 56 Frequent (79-30%)
26 abnormality of the pituitary gland 56 Occasional (29-5%)
27 hemianopia 32 frequent (33%) HP:0012377
28 excessive daytime somnolence 32 very rare (1%) HP:0001262

UMLS symptoms related to Gangliocytoma:


seizures

MGI Mouse Phenotypes related to Gangliocytoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.63 TH C9orf72 CRH GFAP PTEN SYP
2 nervous system MP:0003631 9.43 TH C9orf72 CRH GFAP PTEN SYP
3 normal MP:0002873 9.02 SYP TH CRH GFAP PTEN

Drugs & Therapeutics for Gangliocytoma

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

(show all 8)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bevacizumab Approved, Investigational Phase 1 216974-75-3
2
Irinotecan Approved, Investigational Phase 1 97682-44-5, 100286-90-6 60838
3
Camptothecin Experimental Phase 1 7689-03-4
4 Angiogenesis Inhibitors Phase 1
5 Angiogenesis Modulating Agents Phase 1
6 Antineoplastic Agents, Phytogenic Phase 1
7 topoisomerase I inhibitors Phase 1
8 Topoisomerase Inhibitors Phase 1

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Evaluation of FDOPA-PET/MRI in Pediatric Patients With CNS Tumors Completed NCT01999270 Phase 1 Irinotecan;Bevacizumab
2 Prospective Study Registry of Peripheral Neuroblastic Tumours Presenting With Spinal Canal Involvement (SCI) Recruiting NCT02559804
3 International Congenital Central Hypoventilation Syndrome (CCHS) Registry Recruiting NCT03088020

Search NIH Clinical Center for Gangliocytoma

Cochrane evidence based reviews: ganglioneuroma

Genetic Tests for Gangliocytoma

Anatomical Context for Gangliocytoma

MalaCards organs/tissues related to Gangliocytoma:

39
Cerebellum, Temporal Lobe, Pituitary, Spinal Cord, Brain, Medulla Oblongata, Pineal

Publications for Gangliocytoma

Articles related to Gangliocytoma:

(show top 50) (show all 123)
id Title Authors Year
1
An intrasellar pituitary adenoma-gangliocytoma presenting as acromegaly. ( 28469929 )
2017
2
Transdifferentiation of Neuroendocrine Cells: Gangliocytoma Associated With Two Pituitary Adenomas of Different Lineage in MEN1. ( 28079577 )
2017
3
Endoscopic Endonasal Resection of a Mixed Lesion of Gangliocytoma and Nonfunctioning Pituitary Adenoma. ( 28532920 )
2017
4
Lhermitte-Duclos Disease and Cerebellar Gangliocytoma-An Incidental Finding in a Patient with Gradual Vision Loss. ( 28512508 )
2017
5
Gangliocytoma: outcome of a rare silent pituitary tumour. ( 28232376 )
2017
6
Lhermitte-Duclos Disease (Dysplastic Gangliocytoma of the Cerebellum) and Cowden Syndrome: Clinical Experience From a Single Institution with Long-Term Follow-Up. ( 28479525 )
2017
7
Mixed Gangliocytoma-Pituitary Adenoma: Insights on the Pathogenesis of a Rare Sellar Tumor. ( 28079576 )
2017
8
Transsphenoidal surgery for mixed pituitary gangliocytoma-adenomas. ( 28887280 )
2017
9
Growth hormone secreting pituitary adenoma with admixed gangliocytoma and ganglioglioma. ( 27068013 )
2016
10
Gangliocytoma Presenting With Tacrolimus Neurotoxicity in a Renal Transplant Recipient: Case Report. ( 27932167 )
2016
11
Mixed Pituitary Gangliocytoma and Prolactinoma Resistant to the Cabergoline Treatment. ( 27535625 )
2016
12
Management of unusual dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) in a developing country: Case report and review of the literature. ( 27057227 )
2016
13
A Rare Case of Concomitant Intramedullary Gangliocytoma at the Cervicomedullary Junction in Patient with Neuroendocrine Tumor of Lung. ( 26962423 )
2016
14
Autopsy case of undiagnosed gangliocytoma in the medulla oblongata complicated with cerebral palsy. ( 26210504 )
2015
15
Sellar gangliocytoma with adrenocorticotropic and prolactin adenoma. ( 26314658 )
2015
16
A familial frontotemporal dementia associated with C9orf72 repeat expansion and dysplastic gangliocytoma. ( 24080172 )
2014
17
Pituitary adenoma with gangliocytoma: Report of two cases. ( 25013498 )
2014
18
Dysplastic cerebellar gangliocytoma lhermitte-duclos disease imaging and magnetic resonance spectroscopy. ( 25369219 )
2014
19
Endoscopic approach to a collision tumor of growth hormone-secreting adenoma and gangliocytoma in the pituitary gland. ( 25006908 )
2014
20
Infrasellar pituitary gangliocytoma causing Cushing's syndrome. ( 25183169 )
2014
21
Acromegaly associated with mixed pituitary adenoma-gangliocytoma and Rathke's cleft cyst. ( 24101276 )
2013
22
Cushing's disease due to mixed pituitary adenoma-gangliocytoma of the posterior pituitary gland presenting with Aspergillus sp. sinus infection. ( 23611590 )
2013
23
Gangliocytoma of the medulla oblongata demonstrating high tracer uptake on positron emission tomography. ( 21732072 )
2012
24
Ossifying pituitary adenoma co-existing with astrocytoma and pituitary adenoma associated with gangliocytoma: two unusual conditions. ( 22274987 )
2012
25
Combined gangliocytoma and non-functioning pituitary adenoma of the pituitary gland. ( 22824692 )
2012
26
Acromegaly associated with gangliocytoma. ( 19789938 )
2012
27
Unusual presentation in adult medulloblastomas: imaging features mimicking cerebellar dysplastic gangliocytoma (Lhermitte-Duclos disease). ( 23135053 )
2012
28
Intramedullary gangliocytoma with calcification and multiple intramedullary cysts. ( 22538840 )
2012
29
Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum) as a component of Cowden syndrome. ( 22499315 )
2012
30
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
31
Assessing a dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease) with 7T MR imaging. ( 20191074 )
2010
32
Cytomorphologic spectrum of mixed pituitary adenoma-gangliocytomas: a report of two cases. ( 21053582 )
2010
33
Medulloblastoma with extensive nodularity undergoing post-therapeutic maturation to a gangliocytoma: a case report and literature review. ( 21389751 )
2010
34
Case 144: Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease). ( 19332858 )
2009
35
Subfrontal gangliocytoma masquerading as olfactory groove meningioma. ( 19234914 )
2009
36
From glioblastoma to gangliocytoma: an unforeseen but welcome shift in biological behavior. ( 19877783 )
2009
37
An intrasellar mixed gangliocytoma-adenoma including ependymal component, and review of the literature. ( 21686419 )
2009
38
Collision tumor of anaplastic oligodendroglioma and gangliocytoma: a case report. ( 19856221 )
2009
39
Gangliocytoma associated with focal cortical dysplasia in a young-adult: a case report. ( 18814115 )
2008
40
An unusual association of a sellar gangliocytoma with a prolactinoma. ( 17440820 )
2008
41
Cerebellar gangliocytoma presenting with hemifacial spasms: clinical report, literature review and possible mechanisms. ( 18509586 )
2008
42
Combined gangliocytoma and prolactinoma of the pituitary gland. ( 18651251 )
2008
43
Ectopic recurrence of dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease): a case report. ( 18095141 )
2007
44
Diffuse cerebral gangliocytoma in an adult with late-onset refractory epilepsy. ( 17990996 )
2007
45
Gangliocytoma of the spinal cord. ( 17460525 )
2007
46
Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease). ( 16934474 )
2006
47
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma). ( 15670528 )
2005
48
Pathologic quiz case: an unusual cause of acromegaly. Intrasellar gangliocytoma. ( 15737044 )
2005
49
Acromegaly due to a growth hormone-releasing hormone-secreting intracranial gangliocytoma. ( 15887863 )
2005
50
Intramedullary spinal cord gangliocytoma: case report and a review of the literature. ( 15739067 )
2005

Variations for Gangliocytoma

Cosmic variations for Gangliocytoma:

9
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM99759 IDH2 central nervous system,temporal lobe,other,ganglioglioma c.492G>A p.W164* 5
2 COSM28746 IDH1 central nervous system,brain,other,ganglioglioma c.395G>A p.R132H 4
3 COSM327928 H3F3A central nervous system,spinal cord,other,ganglioglioma c.83A>T p.K28M 4
4 COSM476 BRAF central nervous system,cerebellum,other,ganglioglioma c.1799T>A p.V600E 4

Expression for Gangliocytoma

Search GEO for disease gene expression data for Gangliocytoma.

Pathways for Gangliocytoma

Pathways related to Gangliocytoma according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
2 11.43 GFAP RBFOX3 SYP TH
3 11.03 GFAP SYP TH

GO Terms for Gangliocytoma

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

id Name GO ID Score Top Affiliating Genes
1 neuronal cell body GO:0043025 9.43 CRH RBFOX3 TH
2 neuron projection GO:0043005 9.33 PTEN SYP TH
3 terminal bouton GO:0043195 9.32 SYP TH
4 cytoplasmic side of plasma membrane GO:0009898 8.96 PTEN TH
5 perikaryon GO:0043204 8.8 CRH RBFOX3 TH

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

(show all 12)
id Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.63 CRH PTEN TH
2 memory GO:0007613 9.51 PTEN TH
3 learning or memory GO:0007611 9.49 CRH PTEN
4 social behavior GO:0035176 9.48 PTEN TH
5 response to activity GO:0014823 9.43 PTEN TH
6 response to zinc ion GO:0010043 9.4 PTEN TH
7 response to immobilization stress GO:0035902 9.37 CRH TH
8 response to ethanol GO:0045471 9.33 CRH PTEN TH
9 synaptic transmission, dopaminergic GO:0001963 9.32 CRH TH
10 response to corticosterone GO:0051412 9.26 CRH TH
11 response to ether GO:0045472 8.96 CRH TH
12 long-term synaptic potentiation GO:0060291 8.62 CRH PTEN

Sources for Gangliocytoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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