MCID: ANG050
MIFTS: 34

Angiocentric Glioma

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Angiocentric Glioma

MalaCards integrated aliases for Angiocentric Glioma:

Name: Angiocentric Glioma 60 74

Characteristics:

Orphanet epidemiological data:

60
angiocentric glioma
Prevalence: <1/1000000 (Worldwide); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

Orphanet: 60  
Rare neurological diseases


External Ids:

ICD10 via Orphanet 35 C71.9
UMLS via Orphanet 75 C2363903
Orphanet 60 ORPHA251671
UMLS 74 C2363903

Summaries for Angiocentric Glioma

MalaCards based summary : Angiocentric Glioma is related to pilomyxoid astrocytoma and ependymoma. An important gene associated with Angiocentric Glioma is QKI (QKI, KH Domain Containing RNA Binding), and among its related pathways/superpathways are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Dabrafenib and Vincristine 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 168)
# Related Disease Score Top Affiliating Genes
1 pilomyxoid astrocytoma 30.1 GFAP SYP
2 ependymoma 29.7 GFAP S100B SYP VIM
3 oligodendroglioma 29.5 GFAP IDH1 S100B SYP
4 anaplastic ependymoma 29.4 GFAP S100B SYP VIM
5 glioma 10.8
6 epilepsy 10.5
7 mixed glioma 10.3 GFAP IDH1
8 glioma susceptibility 1 10.3 GFAP IDH1
9 fibrillary astrocytoma 10.3 GFAP IDH1
10 pilocytic astrocytoma 10.3 GFAP IDH1
11 anaplastic ganglioglioma 10.3 ACTC1 GFAP
12 cutaneous leiomyosarcoma 10.2 ACTC1 GFAP
13 spinal cord astrocytoma 10.2 GFAP IDH1
14 gliomatosis cerebri 10.2 GFAP IDH1
15 intracranial cysts 10.2 GFAP S100B
16 primitive neuroectodermal tumor of the cervix uteri 10.2 GFAP VIM
17 gemistocytic astrocytoma 10.2 GFAP S100B
18 rhabdoid meningioma 10.2 GFAP VIM
19 spindle cell rhabdomyosarcoma 10.2 GFAP S100B
20 clear cell meningioma 10.2 GFAP VIM
21 lymphangiectasis 10.2 ACTC1 VIM
22 ependymoblastoma 10.2 GFAP VIM
23 chordoid glioma 10.2 GFAP S100B
24 glomangiomyoma 10.2 ACTC1 VIM
25 necrotizing sialometaplasia 10.2 ACTC1 GFAP
26 angiolipoma 10.2 ACTC1 VIM
27 intravascular papillary endothelial hyperplasia 10.2 ACTC1 VIM
28 malignant giant cell tumor of soft parts 10.2 ACTC1 S100B
29 malignant giant cell tumor 10.2 ACTC1 S100B
30 myopericytoma 10.2 ACTC1 S100B
31 spindle cell lipoma 10.2 ACTC1 VIM
32 epithelioid leiomyosarcoma 10.2 ACTC1 VIM
33 benign metastasizing leiomyoma 10.2 ACTC1 VIM
34 adenofibroma 10.2 ACTC1 VIM
35 brain glioma 10.2 GFAP IDH1
36 spiradenoma 10.2 ACTC1 S100B
37 astrocytoma 10.2
38 desmoplastic infantile ganglioglioma 10.2 GFAP SYP
39 lung combined type small cell carcinoma 10.2 GFAP SYP
40 hydromyelia 10.1 S100B VIM
41 cellular ependymoma 10.1 GFAP SYP
42 bednar tumor 10.1 S100B VIM
43 congenital fibrosarcoma 10.1 ACTC1 VIM
44 reticulum cell sarcoma 10.1 ACTC1 VIM
45 central nervous system primitive neuroectodermal neoplasm 10.1 GFAP SYP
46 chiari malformation 10.1 GFAP SYP
47 cerebral neuroblastoma 10.1 GFAP SYP
48 fibrous meningioma 10.1 S100B VIM
49 eccrine porocarcinoma 10.1 S100B VIM
50 subependymal glioma 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:

27 (show all 20)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.68 MYB
2 Increased shRNA abundance (Z-score > 2) GR00366-A-107 9.68 ACTC1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.68 MYB
4 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.68 MYB
5 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.68 ACTC1 MYB
6 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.68 IDH1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-136 9.68 IDH1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.68 IDH1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.68 ACTC1 IDH1 MYB
10 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.68 MYB
11 Increased shRNA abundance (Z-score > 2) GR00366-A-164 9.68 MYB
12 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.68 MYB
13 Increased shRNA abundance (Z-score > 2) GR00366-A-172 9.68 IDH1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.68 MYB
15 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.68 ACTC1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.68 IDH1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.68 IDH1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-65 9.68 MYB
19 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.68 MYB
20 Increased shRNA abundance (Z-score > 2) GR00366-A-83 9.68 MYB

MGI Mouse Phenotypes related to Angiocentric Glioma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.63 ACTC1 GFAP MYB PTPRJ QKI VIM
2 homeostasis/metabolism MP:0005376 9.5 ACTC1 GFAP IDH1 PTPRJ QKI S100B
3 normal MP:0002873 9.1 ACTC1 GFAP MYB PTPRJ S100B SYP

Drugs & Therapeutics for Angiocentric Glioma

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

(show all 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760 44516822
2
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
3
Carboplatin Approved Phase 2 41575-94-4 38904 10339178 498142
4
Trametinib Approved Phase 2 871700-17-3 11707110
5 Protein Kinase Inhibitors Phase 2
6 Antimitotic Agents Phase 2
7 Antineoplastic Agents, Phytogenic 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:

42
Brain, Temporal Lobe, Cortex

Publications for Angiocentric Glioma

Articles related to Angiocentric Glioma:

(show all 44)
# Title Authors Year
1
Angiocentric Glioma: Report of a Rare Case Presenting with Psychosis. ( 30459891 )
2018
2
Brainstem angiocentric glioma: report of 2 cases. ( 28753090 )
2017
3
Angiocentric Glioma in an Elderly Patient: Case Report and Review of the Literature. ( 27756673 )
2017
4
Angiocentric glioma with MYB-QKI fusion located in the brainstem, rather than cerebral cortex. ( 28776091 )
2017
5
MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism. ( 26829751 )
2016
6
MYB-QKI Fusions Drive Angiocentric Glioma through Multiple Mechanisms. ( 26869254 )
2016
7
Angiocentric glioma of hippocampus-report of a rare intractable epilepsy-related tumor. ( 26954821 )
2016
8
Angiocentric glioma transformed into anaplastic ependymoma: Review of the evidence for malignant potential. ( 27742374 )
2016
9
Angiocentric glioma from a perspective of A-B-C classification of epilepsy associated tumors. ( 27179220 )
2016
10
&amp;quot;Unusual brain stone&amp;quot;: heavily calcified primary neoplasm with some features suggestive of angiocentric glioma. ( 26024003 )
2015
11
MR Spectroscopic Profile of an Angiocentric Glioma. ( 26504061 )
2015
12
Cystoid angiocentric glioma: A case report and literature review. ( 26629293 )
2015
13
Intractable epilepsy due to angiocentric glioma: A case report and minireview. ( 24348765 )
2014
14
Well-differentiated pediatric glial neoplasms with features of oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumors: a morphological diagnostic challenge. ( 24448703 )
2014
15
Angiocentric Glioma: The Infiltrative Glioma with Ependymal Differentiation. ( 24913299 )
2014
16
Angiocentric glioma: A report of nine new cases, including four with atypical histological features. ( 24861831 )
2014
17
Angiocentric glioma: a treatable cause of epilepsy: report of a rare case. ( 25591686 )
2014
18
Angiocentric glioma: a rare intractable epilepsy-related tumour in children. ( 25310736 )
2014
19
The treatment of angiocentric glioma: case report and literature review. ( 23596378 )
2013
20
Angiocentric glioma. ( 24116979 )
2013
21
Angiocentric glioma, a recently added WHO grade-I tumor. ( 27330646 )
2013
22
Angiocentric glioma: clinical, morphological, immunohistochemical and molecular features in three pediatric cases. ( 23073165 )
2013
23
Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. ( 22445362 )
2012
24
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
25
Refractory temporal lobe epilepsy caused by angiocentric glioma complicated with focal cortical dysplasia: a surgical case series. ( 23065092 )
2012
26
Imaging characteristics of an unusual, high-grade angiocentric glioma: a case report and review of the literature. ( 23378871 )
2012
27
Angiocentric glioma in a 4-year-old boy: imaging characteristics and review of the literature. ( 22226445 )
2012
28
The high mitotic count detected by phospho-histone H3 immunostain does not alter the benign behavior of angiocentric glioma. ( 21892765 )
2012
29
Angiocentric glioma with high proliferative index: case report and review of the literature. ( 22075509 )
2011
30
A case of angiocentric glioma with unusual clinical and radiological features. ( 21887397 )
2011
31
Angiocentric glioma and surrounding cortical dysplasia manifesting as intractable frontal lobe epilepsy--case report. ( 21785249 )
2011
32
Angiocentric glioma: a clinicopathologic review of 5 tumors with identification of associated cortical dysplasia. ( 21809996 )
2011
33
Angiocentric glioma with rich blood supply. ( 20399664 )
2010
34
Angiocentric glioma: a case report and review of the literature. ( 19941376 )
2010
35
Paroxysmal ictal phonemes in a patient with angiocentric glioma. ( 20160231 )
2010
36
Angiocentric glioma-induced seizures in a 2-year-old child. ( 19304961 )
2009
37
Angiocentric glioma-like tumor of the midbrain. ( 20110754 )
2009
38
Angiocentric glioma: a case series. ( 19338465 )
2009
39
Cortical ependymoma or monomorphous angiocentric glioma? ( 18021197 )
2008
40
Brain surface spindle cell glioma in a patient with medically intractable partial epilepsy: a variant of monomorphous angiocentric glioma? ( 18179412 )
2008
41
Angiocentric glioma: presentation of two cases with dissimilar histology. ( 19130736 )
2008
42
Angiocentric glioma: report of clinico-pathologic and genetic findings in 8 cases. ( 18059228 )
2007
43
Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma. ( 17598825 )
2007
44
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 11.87 GFAP S100B SYP
2 10.82 GFAP S100B SYP VIM
3 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 intermediate filament organization GO:0045109 9.32 GFAP VIM
6 astrocyte development GO:0014002 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 9.02 GFAP IDH1 S100B SYP VIM

Sources for Angiocentric Glioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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