MCID: ANG050
MIFTS: 33

Angiocentric Glioma

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Angiocentric Glioma

MalaCards integrated aliases for Angiocentric Glioma:

Name: Angiocentric Glioma 59 73

Characteristics:

Orphanet epidemiological data:

59
angiocentric glioma
Prevalence: <1/1000000 (Worldwide); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA251671
UMLS via Orphanet 74 C2363903
ICD10 via Orphanet 34 C71.9
UMLS 73 C2363903

Summaries for Angiocentric Glioma

MalaCards based summary : Angiocentric Glioma is related to pilomyxoid astrocytoma and oligodendroglioma. An important gene associated with Angiocentric Glioma is QKI (QKI, KH Domain Containing RNA Binding), and among its related pathways/superpathways are Neural Stem Cell Differentiation Pathways and Lineage-specific Markers and Cell adhesion_Cadherin-mediated cell adhesion. The drugs Dabrafenib and Trametinib have been mentioned in the context of this disorder. Affiliated tissues include brain, temporal lobe and cortex, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Angiocentric Glioma

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

(show top 50) (show all 161)
# Related Disease Score Top Affiliating Genes
1 pilomyxoid astrocytoma 30.2 GFAP SYP
2 oligodendroglioma 29.5 GFAP IDH1 SYP
3 dysembryoplastic neuroepithelial tumor 28.7 GFAP IDH1 S100B SYP
4 ependymoma 28.2 GFAP S100B SYP VIM
5 anaplastic ependymoma 27.9 GFAP S100B SYP VIM
6 glioma 10.7
7 anaplastic ganglioglioma 10.5 ACTC1 GFAP
8 mixed glioma 10.5 GFAP IDH1
9 cutaneous leiomyosarcoma 10.5 ACTC1 GFAP
10 fibrillary astrocytoma 10.5 GFAP IDH1
11 gliomatosis cerebri 10.4 GFAP IDH1
12 malignant giant cell tumor of soft parts 10.4 ACTC1 S100B
13 malignant giant cell tumor 10.4 ACTC1 S100B
14 spinal cord astrocytoma 10.4 GFAP IDH1
15 myopericytoma 10.4 ACTC1 S100B
16 intracranial cysts 10.4 GFAP S100B
17 gemistocytic astrocytoma 10.4 GFAP S100B
18 necrotizing sialometaplasia 10.4 ACTC1 GFAP
19 spindle cell rhabdomyosarcoma 10.3 GFAP S100B
20 chordoid glioma 10.3 GFAP S100B
21 epilepsy 10.3
22 desmoplastic infantile ganglioglioma 10.3 GFAP SYP
23 lung combined type small cell carcinoma 10.3 GFAP SYP
24 cellular ependymoma 10.3 GFAP SYP
25 gangliocytoma 10.2 GFAP SYP
26 central nervous system primitive neuroectodermal neoplasm 10.2 GFAP SYP
27 glycogen-rich clear cell breast carcinoma 10.2 ACTC1 SYP
28 subependymal glioma 10.2 GFAP SYP
29 chiari malformation 10.2 GFAP SYP
30 cerebral neuroblastoma 10.2 GFAP SYP
31 benign ependymoma 10.2 GFAP SYP
32 cerebral ventricle cancer 10.2 GFAP SYP
33 cerebrum cancer 10.2 GFAP SYP
34 pineal gland cancer 10.2 GFAP SYP
35 traumatic brain injury 10.2 GFAP S100B
36 obstructive hydrocephalus 10.2 GFAP SYP
37 supratentorial cancer 10.2 GFAP SYP
38 subependymoma 10.2 GFAP SYP
39 mixed type thymoma 10.2 S100B SYP
40 tanycytic ependymoma 10.2 GFAP SYP
41 hypoganglionosis 10.2 S100B SYP
42 pineoblastoma 10.1 GFAP SYP
43 rosette-forming glioneuronal tumor 10.1 S100B SYP
44 horseshoe kidney 10.1 ACTC1 SYP
45 central neurocytoma 10.1 GFAP SYP
46 breast papillary carcinoma 10.1 IDH1 SYP
47 supratentorial primitive neuroectodermal tumor 10.1 S100B SYP
48 cystic teratoma 10.1 GFAP SYP
49 lymphangiectasis 10.1 ACTC1 VIM
50 cerebellar liponeurocytoma 10.1 GFAP SYP

Graphical network of the top 20 diseases related to Angiocentric Glioma:



Diseases related to Angiocentric Glioma

Symptoms & Phenotypes for Angiocentric Glioma

GenomeRNAi Phenotypes related to Angiocentric Glioma according to GeneCards Suite gene sharing:

26 (show all 19)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.66 MYB
2 Increased shRNA abundance (Z-score > 2) GR00366-A-107 9.66 ACTC1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.66 MYB
4 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.66 MYB
5 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.66 MYB ACTC1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.66 IDH1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-136 9.66 IDH1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.66 IDH1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.66 IDH1 MYB ACTC1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.66 MYB
11 Increased shRNA abundance (Z-score > 2) GR00366-A-164 9.66 MYB
12 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.66 MYB
13 Increased shRNA abundance (Z-score > 2) GR00366-A-172 9.66 IDH1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.66 MYB
15 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.66 ACTC1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.66 IDH1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.66 IDH1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.66 MYB
19 Increased shRNA abundance (Z-score > 2) GR00366-A-83 9.66 MYB

MGI Mouse Phenotypes related to Angiocentric Glioma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.43 ACTC1 GFAP MYB PTPRJ QKI VIM
2 normal MP:0002873 9.1 MYB PTPRJ S100B SYP ACTC1 GFAP

Drugs & Therapeutics for Angiocentric Glioma

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760 44516822
2
Trametinib Approved Phase 2 871700-17-3 11707110
3 Protein Kinase Inhibitors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG and LGG Recruiting NCT02684058 Phase 2 dabrafenib;trametinib;Carboplatin with vincristine

Search NIH Clinical Center for Angiocentric Glioma

Genetic Tests for Angiocentric Glioma

Anatomical Context for Angiocentric Glioma

MalaCards organs/tissues related to Angiocentric Glioma:

41
Brain, Temporal Lobe, Cortex

Publications for Angiocentric Glioma

Articles related to Angiocentric Glioma:

(show all 43)
# Title Authors Year
1
Brainstem angiocentric glioma: report of 2 cases. ( 28753090 )
2017
2
Angiocentric Glioma in an Elderly Patient: Case Report and Review of the Literature. ( 27756673 )
2017
3
Angiocentric glioma with MYB-QKI fusion located in the brainstem, rather than cerebral cortex. ( 28776091 )
2017
4
MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism. ( 26829751 )
2016
5
MYB-QKI Fusions Drive Angiocentric Glioma through Multiple Mechanisms. ( 26869254 )
2016
6
Angiocentric glioma of hippocampus-report of a rare intractable epilepsy-related tumor. ( 26954821 )
2016
7
Angiocentric glioma transformed into anaplastic ependymoma: Review of the evidence for malignant potential. ( 27742374 )
2016
8
Angiocentric glioma from a perspective of A-B-C classification of epilepsy associated tumors. ( 27179220 )
2016
9
&amp;quot;Unusual brain stone&amp;quot;: heavily calcified primary neoplasm with some features suggestive of angiocentric glioma. ( 26024003 )
2015
10
MR Spectroscopic Profile of an Angiocentric Glioma. ( 26504061 )
2015
11
Cystoid angiocentric glioma: A case report and literature review. ( 26629293 )
2015
12
Intractable epilepsy due to angiocentric glioma: A case report and minireview. ( 24348765 )
2014
13
Well-differentiated pediatric glial neoplasms with features of oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumors: a morphological diagnostic challenge. ( 24448703 )
2014
14
Angiocentric Glioma: The Infiltrative Glioma with Ependymal Differentiation. ( 24913299 )
2014
15
Angiocentric glioma: A report of nine new cases, including four with atypical histological features. ( 24861831 )
2014
16
Angiocentric glioma: a treatable cause of epilepsy: report of a rare case. ( 25591686 )
2014
17
Angiocentric glioma: a rare intractable epilepsy-related tumour in children. ( 25310736 )
2014
18
The treatment of angiocentric glioma: case report and literature review. ( 23596378 )
2013
19
Angiocentric glioma. ( 24116979 )
2013
20
Angiocentric glioma, a recently added WHO grade-I tumor. ( 27330646 )
2013
21
Angiocentric glioma: clinical, morphological, immunohistochemical and molecular features in three pediatric cases. ( 23073165 )
2013
22
Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. ( 22445362 )
2012
23
Adult-onset angiocentric glioma of epithelioid cell-predominant type of the mesial temporal lobe suggestive of a rare but distinct clinicopathological subset within a spectrum of angiocentric cortical ependymal tumors. ( 22151480 )
2012
24
Refractory temporal lobe epilepsy caused by angiocentric glioma complicated with focal cortical dysplasia: a surgical case series. ( 23065092 )
2012
25
Imaging characteristics of an unusual, high-grade angiocentric glioma: a case report and review of the literature. ( 23378871 )
2012
26
Angiocentric glioma in a 4-year-old boy: imaging characteristics and review of the literature. ( 22226445 )
2012
27
The high mitotic count detected by phospho-histone H3 immunostain does not alter the benign behavior of angiocentric glioma. ( 21892765 )
2012
28
Angiocentric glioma with high proliferative index: case report and review of the literature. ( 22075509 )
2011
29
A case of angiocentric glioma with unusual clinical and radiological features. ( 21887397 )
2011
30
Angiocentric glioma and surrounding cortical dysplasia manifesting as intractable frontal lobe epilepsy--case report. ( 21785249 )
2011
31
Angiocentric glioma: a clinicopathologic review of 5 tumors with identification of associated cortical dysplasia. ( 21809996 )
2011
32
Angiocentric glioma with rich blood supply. ( 20399664 )
2010
33
Angiocentric glioma: a case report and review of the literature. ( 19941376 )
2010
34
Paroxysmal ictal phonemes in a patient with angiocentric glioma. ( 20160231 )
2010
35
Angiocentric glioma-induced seizures in a 2-year-old child. ( 19304961 )
2009
36
Angiocentric glioma-like tumor of the midbrain. ( 20110754 )
2009
37
Angiocentric glioma: a case series. ( 19338465 )
2009
38
Cortical ependymoma or monomorphous angiocentric glioma? ( 18021197 )
2008
39
Brain surface spindle cell glioma in a patient with medically intractable partial epilepsy: a variant of monomorphous angiocentric glioma? ( 18179412 )
2008
40
Angiocentric glioma: presentation of two cases with dissimilar histology. ( 19130736 )
2008
41
Angiocentric glioma: report of clinico-pathologic and genetic findings in 8 cases. ( 18059228 )
2007
42
Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma. ( 17598825 )
2007
43
Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. ( 16215459 )
2005

Variations for Angiocentric Glioma

Expression for Angiocentric Glioma

Search GEO for disease gene expression data for Angiocentric Glioma.

Pathways for Angiocentric Glioma

Pathways related to Angiocentric Glioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.82 GFAP S100B SYP VIM
2 10.56 ACTC1 PTPRJ

GO Terms for Angiocentric Glioma

Biological processes related to Angiocentric Glioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 long-term synaptic potentiation GO:0060291 9.46 GFAP S100B
2 muscle filament sliding GO:0030049 9.43 ACTC1 VIM
3 positive regulation of collagen biosynthetic process GO:0032967 9.4 MYB VIM
4 regulation of neuronal synaptic plasticity GO:0048168 9.37 S100B SYP
5 astrocyte development GO:0014002 9.32 GFAP VIM
6 intermediate filament organization GO:0045109 9.26 GFAP VIM
7 positive regulation of glial cell proliferation GO:0060252 9.16 GFAP MYB
8 Bergmann glial cell differentiation GO:0060020 8.96 GFAP VIM
9 intermediate filament-based process GO:0045103 8.62 GFAP VIM

Molecular functions related to Angiocentric Glioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.1 GFAP IDH1 PTPRJ S100B SYP VIM

Sources for Angiocentric Glioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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