EVN
MCID: EXT044
MIFTS: 27

Extraventricular Neurocytoma (EVN)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Extraventricular Neurocytoma

MalaCards integrated aliases for Extraventricular Neurocytoma:

Name: Extraventricular Neurocytoma 60 74
Evn 60

Classifications:

Orphanet: 60  
Rare neurological diseases


External Ids:

Orphanet 60 ORPHA251927
UMLS 74 C2985175

Summaries for Extraventricular Neurocytoma

MalaCards based summary : Extraventricular Neurocytoma, also known as evn, is related to central neurocytoma and ganglioglioma. An important gene associated with Extraventricular Neurocytoma is IDH1 (Isocitrate Dehydrogenase (NADP(+)) 1, Cytosolic), 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 spinal cord, temporal lobe and bone.

Related Diseases for Extraventricular Neurocytoma

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

(show top 50) (show all 73)
# Related Disease Score Top Affiliating Genes
1 central neurocytoma 30.6 GFAP SYP
2 ganglioglioma 30.2 GFAP SYP
3 oligodendroglioma 30.1 GFAP IDH1 SYP
4 neurofibromatosis, type i 10.2
5 neurofibromatosis, type iv, of riccardi 10.2
6 temporal lobe epilepsy 10.2
7 epilepsy 10.2
8 anaplastic ganglioglioma 10.2
9 pineal parenchymal tumor of intermediate differentiation 9.9 IDH1 SYP
10 desmoplastic infantile ganglioglioma 9.9 GFAP SYP
11 lung combined type small cell carcinoma 9.9 GFAP SYP
12 cellular ependymoma 9.9 GFAP SYP
13 ependymoma 9.9 GFAP SYP
14 chordoid meningioma 9.9 GFAP SYP
15 central nervous system primitive neuroectodermal neoplasm 9.9 GFAP SYP
16 clear cell ependymoma 9.9 GFAP SYP
17 chiari malformation 9.9 GFAP SYP
18 cerebral neuroblastoma 9.9 GFAP SYP
19 subependymal glioma 9.9 GFAP SYP
20 astroblastoma 9.9 GFAP SYP
21 cerebral ventricle cancer 9.9 GFAP SYP
22 pineal gland cancer 9.9 GFAP SYP
23 melanotic neuroectodermal tumor 9.9 GFAP SYP
24 benign ependymoma 9.9 GFAP SYP
25 obstructive hydrocephalus 9.9 GFAP SYP
26 hydrocephalus 9.9 GFAP SYP
27 medulloepithelioma 9.9 GFAP SYP
28 pineocytoma 9.9 GFAP SYP
29 subependymoma 9.9 GFAP SYP
30 anaplastic ependymoma 9.9 GFAP SYP
31 tanycytic ependymoma 9.9 GFAP SYP
32 papillary ependymoma 9.9 GFAP SYP
33 pineoblastoma 9.9 GFAP SYP
34 papilloma of choroid plexus 9.9 GFAP SYP
35 binswanger's disease 9.9 GFAP SYP
36 mixed glioma 9.9 GFAP IDH1
37 cystic teratoma 9.9 GFAP SYP
38 teratoma 9.9 GFAP SYP
39 subependymal giant cell astrocytoma 9.9 GFAP SYP
40 central nervous system benign neoplasm 9.9 GFAP SYP
41 cerebellar liponeurocytoma 9.9 GFAP SYP
42 glioma susceptibility 1 9.8 GFAP IDH1
43 pilomyxoid astrocytoma 9.8 GFAP SYP
44 fibrillary astrocytoma 9.8 GFAP IDH1
45 medullomyoblastoma 9.8 GFAP SYP
46 pilocytic astrocytoma 9.8 GFAP IDH1
47 spinal cord astrocytoma 9.8 GFAP IDH1
48 gliomatosis cerebri 9.8 GFAP IDH1
49 ganglioneuroma 9.8 GFAP SYP
50 optic nerve glioma 9.8 GFAP SYP

Graphical network of the top 20 diseases related to Extraventricular Neurocytoma:



Diseases related to Extraventricular Neurocytoma

Symptoms & Phenotypes for Extraventricular Neurocytoma

Drugs & Therapeutics for Extraventricular Neurocytoma

Drugs for Extraventricular Neurocytoma (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 Extraventricular Neurocytoma

Genetic Tests for Extraventricular Neurocytoma

Anatomical Context for Extraventricular Neurocytoma

MalaCards organs/tissues related to Extraventricular Neurocytoma:

42
Spinal Cord, Temporal Lobe, Bone

Publications for Extraventricular Neurocytoma

Articles related to Extraventricular Neurocytoma:

(show top 50) (show all 62)
# Title Authors Year
1
Mesial temporal extraventricular neurocytoma (mtEVN): A case report and literature review. ( 30603610 )
2019
2
The epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014. ( 30859483 )
2019
3
FGFR1:TACC1 fusion is a frequent event in molecularly defined extraventricular neurocytoma. ( 29978331 )
2018
4
Extraventricular neurocytoma in the left frontal lobe: A case report and literature review. ( 29378347 )
2018
5
Patterns of care and survival outcomes in patients with an extraventricular neurocytoma: An individual patient data analysis of 201 cases. ( 29547155 )
2018
6
Complete resection of hypervascularised extraventricular neurocytoma after preoperative embolisation. ( 29680750 )
2018
7
Imaging characteristics of cerebral extraventricular neurocytoma with pathological correlation. ( 30062611 )
2018
8
Malignant progression of an extraventricular neurocytoma arising from the VIIIth cranial nerve: A case report and literature review. ( 30588667 )
2018
9
Extraventricular neurocytoma: An uncommon tumor in a young boy. A review of literature. ( 28084281 )
2017
10
Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy. ( 27195261 )
2016
11
Densly calcified cystic extraventricular neurocytoma: Radiological-pathological correlation. ( 27841226 )
2016
12
A clinicopathologic study of extraventricular neurocytoma. ( 27864704 )
2016
13
Progression of Atypical Extraventricular Neurocytoma to Anaplastic Ganglioglioma. ( 27597523 )
2016
14
Central Versus Extraventricular Neurocytoma in Children: A Clinicopathologic Comparison and Review of the Literature. ( 27438020 )
2016
15
Extraventricular neurocytoma of the sellar region: case report and literature review. ( 27398267 )
2016
16
Extraventricular neurocytoma in the left temporal lobe: A case report and review of the literature. ( 27313678 )
2016
17
Extraventricular neurocytoma mimicking bone tumor in thoracic spinal column. ( 26235460 )
2015
18
Atypical extraventricular neurocytoma in a 3-year-old girl: case report with radiological-pathological correlation. ( 25894757 )
2015
19
Extraventricular neurocytoma treated with 177Lu DOTATATE PRRT induction and maintenance therapies. ( 25608145 )
2015
20
Mesial temporal extraventricular neurocytoma: a rare cause of refractory complex partial seizure. ( 24691302 )
2014
21
Extraventricular Neurocytoma and Ganglioneurocytoma: Advanced MR Imaging, Histopathological, and Chromosomal Findings. ( 24787229 )
2014
22
Behavioral disorders as unusual presentation of pediatric extraventricular neurocytoma: report on two cases and review of the literature. ( 25524219 )
2014
23
Extraventricular neurocytoma of the sellar region with spinal dissemination. ( 23250388 )
2014
24
Extraventricular neurocytoma in pediatric populations: A case report and review of the literature. ( 24179531 )
2013
25
Extensively calcified cystic extraventricular neurocytoma. ( 24441253 )
2013
26
Extraventricular neurocytoma, a comprehensive review. ( 24357019 )
2013
27
Rapid local recurrence of an extraventricular neurocytoma that had disappeared after gamma knife radiosurgery. ( 23324299 )
2013
28
Visual and auditory hallucinations revealing cerebellar extraventricular neurocytoma: uncommon presentation for uncommon tumor in uncommon location. ( 24199787 )
2013
29
Computed tomography and magnetic resonance features of extraventricular neurocytoma: a study of eight cases. ( 23332437 )
2013
30
MR imaging of cerebral extraventricular neurocytoma: a report of 9 cases. ( 23042917 )
2013
31
5-aminolevulinic Acid fluorescence discriminates the histological grade of extraventricular neurocytoma. ( 24904890 )
2013
32
Response to: Extraventricular neurocytoma of the sellar region. ( 23659217 )
2013
33
A rare case of a pediatric extraventricular neurocytoma: case report and review of the literature. ( 22167266 )
2012
34
Extraventricular neurocytoma. ( 23591250 )
2012
35
Atypical extraventricular neurocytoma: a rare and challenging case diagnosed on intraoperative cytology. ( 21410797 )
2012
36
MR imaging findings of extraventricular neurocytoma: a series of ten patients confirmed by immunohistochemistry of IDH1 gene mutation. ( 22945896 )
2012
37
Extraventricular neurocytoma of the sellar region. ( 22122710 )
2012
38
Pontine extraventricular neurocytoma in a child. ( 23838270 )
2012
39
Dural metastasis of atypical extraventricular neurocytoma with the codeletion of chromosomes 1p/19q. ( 22118007 )
2011
40
Atypical extraventricular neurocytoma. ( 22200023 )
2011
41
Temporo-mesial extraventricular neurocytoma and cortical dysplasia in focal temporal lobe epilepsy. ( 20851605 )
2011
42
Clinicopathological analysis of central and extraventricular neurocytoma: a report of 17 cases. ( 21181365 )
2010
43
Extraventricular neurocytoma of the vermis in a child. ( 20692164 )
2010
44
Extraventricular neurocytoma with atypical features and ganglionic differentiation. ( 20399667 )
2010
45
Comprehensive review of extraventricular neurocytoma with report of two cases, and comparison with central neurocytoma. ( 20423686 )
2010
46
Extraventricular neurocytoma--report of a case. ( 19484697 )
2009
47
Extraventricular neurocytoma in a child: case report and review of the literature. ( 19339288 )
2009
48
Nonenhancing hypovascular extraventricular neurocytoma. ( 19509069 )
2009
49
Extraventricular neurocytoma in a child mimicking oligodendroglioma: a diagnostic pitfall. ( 19886559 )
2009
50
Interphase cytogenetics for 1p19q and t(1;19)(q10;p10) may distinguish prognostically relevant subgroups in extraventricular neurocytoma. ( 18710393 )
2009

Variations for Extraventricular Neurocytoma

Expression for Extraventricular Neurocytoma

Search GEO for disease gene expression data for Extraventricular Neurocytoma.

Pathways for Extraventricular Neurocytoma

Pathways related to Extraventricular Neurocytoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.49 GFAP SYP
2 10.52 GFAP SYP

GO Terms for Extraventricular Neurocytoma

Molecular functions related to Extraventricular Neurocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 8.8 GFAP IDH1 SYP

Sources for Extraventricular Neurocytoma

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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