MCID: CNT019
MIFTS: 45

Central Neurocytoma

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Central Neurocytoma

MalaCards integrated aliases for Central Neurocytoma:

Name: Central Neurocytoma 12 53 59 15 73
Neurocytoma 44 73
Neurocytoma Central 55
Neurolipocytoma 12

Characteristics:

Orphanet epidemiological data:

59
central neurocytoma
Inheritance: Not applicable; Age of onset: Adolescent,Adult,Childhood;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:14174
MeSH 44 D018306
NCIt 50 C3791
SNOMED-CT 68 128858006 302832007
Orphanet 59 ORPHA73256
UMLS via Orphanet 74 C0206719

Summaries for Central Neurocytoma

NIH Rare Diseases : 53 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 73256Disease definitionCentral neurocytoma is a very rare brain tumor of young adults (over 100 cases reported worldwide). It is typically found in the lateral ventricles and occasionally in the third ventricle. Symptoms are those of increased intracranial pressure: headache, nausea and vomiting, drowsiness, vision problems and mental changes. Total removal of the tumor is the therapy of choice. Post-operative prognosis is generally good.Visit the Orphanet disease page for more resources.

MalaCards based summary : Central Neurocytoma, also known as neurocytoma, is related to extraventricular neurocytoma and oligodendroglioma, and has symptoms including headache and vomiting. An important gene associated with Central Neurocytoma is SYP (Synaptophysin), and among its related pathways/superpathways are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Dabrafenib and Trametinib have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and thalamus, and related phenotypes are hydrocephalus and intracranial cystic lesion

Wikipedia : 76 Central neurocytoma, abbreviated CNC, is an extremely rare, ordinarily benign intraventricular brain... more...

Related Diseases for Central Neurocytoma

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

(show top 50) (show all 206)
# Related Disease Score Top Affiliating Genes
1 extraventricular neurocytoma 30.6 GFAP SYP
2 oligodendroglioma 30.4 ENO2 GFAP SYP
3 cystic teratoma 29.8 ENO2 GFAP SYP
4 cerebellar liponeurocytoma 29.5 CHGA ENO2 GFAP MAP2 SYP
5 subependymal giant cell astrocytoma 28.8 ENO2 GFAP NES SYP
6 ependymoma 28.8 ENO2 GFAP NES SYP
7 teratoma 28.8 ENO2 GFAP NES SYP
8 medulloblastoma 27.8 ENO2 GFAP MAP2 NES SYP
9 subependymoma 27.6 CHGA ENO2 GFAP NES SYP
10 desmoplastic infantile ganglioglioma 10.6 GFAP SYP
11 lung combined type small cell carcinoma 10.6 GFAP SYP
12 gangliocytoma 10.6 GFAP SYP
13 rhabdoid meningioma 10.6 GFAP SYP
14 pilomyxoid astrocytoma 10.6 GFAP SYP
15 malignant sertoli cell tumor 10.6 ENO2 SYP
16 angiocentric glioma 10.6 GFAP SYP
17 obstructive hydrocephalus 10.6 GFAP SYP
18 sinonasal undifferentiated carcinoma 10.6 ENO2 SYP
19 sensory organ benign neoplasm 10.6 ENO2 SYP
20 pancreatic somatostatinoma 10.6 ENO2 SYP
21 cutaneous ganglioneuroma 10.6 ENO2 SYP
22 extrahepatic bile duct adenocarcinoma 10.6 ENO2 SYP
23 anaplastic ependymoma 10.6 GFAP SYP
24 orbital cancer 10.6 ENO2 SYP
25 middle ear adenoma 10.6 ENO2 SYP
26 ovarian carcinosarcoma 10.6 ENO2 SYP
27 childhood oligodendroglioma 10.5 GFAP SYP
28 extraosseous chondrosarcoma 10.5 ENO2 SYP
29 intracranial cysts 10.5 ENO2 GFAP
30 malignant teratoma 10.5 ENO2 SYP
31 primitive neuroectodermal tumor of the cervix uteri 10.5 ENO2 GFAP
32 endometrial small cell carcinoma 10.5 ENO2 SYP
33 melanotic medulloblastoma 10.5 ENO2 GFAP
34 pineoblastoma 10.5 GFAP SYP
35 mucinous ovarian cystadenoma 10.5 ENO2 SYP
36 papilloma of choroid plexus 10.5 GFAP SYP
37 angiomyoma 10.5 ENO2 SYP
38 desmoplastic small round cell tumor 10.5 ENO2 SYP
39 cerebellar medulloblastoma 10.5 ENO2 GFAP
40 bone benign neoplasm 10.5 ENO2 SYP
41 meningeal melanomatosis 10.5 ENO2 SYP
42 plexiform schwannoma 10.5 ENO2 GFAP
43 cervical clear cell adenocarcinoma 10.5 ENO2 SYP
44 extraskeletal ewing sarcoma 10.5 ENO2 SYP
45 connective tissue benign neoplasm 10.5 ENO2 SYP
46 glomus tumor 10.5 ENO2 SYP
47 rosette-forming glioneuronal tumor 10.5 MAP2 SYP
48 microcystic meningioma 10.5 ENO2 GFAP
49 intraocular retinoblastoma 10.5 ENO2 SYP
50 cerebellopontine angle tumor 10.5 GFAP SYP

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



Diseases related to Central Neurocytoma

Symptoms & Phenotypes for Central Neurocytoma

Human phenotypes related to Central Neurocytoma:

59 32 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 59 32 obligate (100%) Obligate (100%) HP:0000238
2 intracranial cystic lesion 59 32 hallmark (90%) Very frequent (99-80%) HP:0010576
3 ataxia 59 32 frequent (33%) Frequent (79-30%) HP:0001251
4 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
5 headache 59 32 frequent (33%) Frequent (79-30%) HP:0002315
6 cerebral calcification 59 32 frequent (33%) Frequent (79-30%) HP:0002514
7 increased intracranial pressure 59 32 frequent (33%) Frequent (79-30%) HP:0002516
8 tinnitus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000360
9 lethargy 59 32 occasional (7.5%) Occasional (29-5%) HP:0001254
10 coma 59 32 occasional (7.5%) Occasional (29-5%) HP:0001259
11 paresthesia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003401
12 babinski sign 59 32 occasional (7.5%) Occasional (29-5%) HP:0003487
13 pain insensitivity 59 32 occasional (7.5%) Occasional (29-5%) HP:0007021

UMLS symptoms related to Central Neurocytoma:


headache, vomiting

Drugs & Therapeutics for Central Neurocytoma

Drugs for Central Neurocytoma (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 Central Neurocytoma

Cochrane evidence based reviews: neurocytoma

Genetic Tests for Central Neurocytoma

Anatomical Context for Central Neurocytoma

MalaCards organs/tissues related to Central Neurocytoma:

41
Brain, Spinal Cord, Thalamus, Endothelial, Ovary, Cerebellum

Publications for Central Neurocytoma

Articles related to Central Neurocytoma:

(show top 50) (show all 234)
# Title Authors Year
1
Letter to the Editor Regarding "Clinical Features, Treatment, and Long-Term Outcomes of Central Neurocytoma: 20-Year Experience at a Single Center". ( 29499602 )
2018
2
Diagnostic value of six MRI features for central neurocytoma. ( 29713774 )
2018
3
Clinical Features, Treatment, and Long-term Outcomes of Central Neurocytoma: A 20-Year Experience at a Single Center. ( 28958923 )
2018
4
Central neurocytoma represents a tumor consisting of diverse neuronal phenotypes. ( 29747899 )
2018
5
Central neurocytoma: Clinical characteristics, patterns of care, and survival. ( 29685410 )
2018
6
The role of proton beam therapy in central neurocytoma: A single-institution experience. ( 29706302 )
2018
7
In Reply to Letter to the Editor Regarding "Clinical Features, Treatment, and Long-Term Outcomes of Central Neurocytoma: 20-Year Experience at a Single Center". ( 29499603 )
2018
8
Intramedullary central neurocytoma of the thoracic spinal cord: A case report and literature review. ( 29541463 )
2018
9
Systematic Analysis of Clinical Outcomes Following Stereotactic Radiosurgery for Central Neurocytoma. ( 28516073 )
2017
10
Clinical, radiological, pathological and prognostic aspects of intraventricular oligodendroglioma: comparison with central neurocytoma. ( 28900829 )
2017
11
Spontaneous intracerebral hemorrhage as presentation of atypical central neurocytoma: the role of angiogenesis through the characterization of tumor endothelial cells. ( 29215136 )
2017
12
Upfront Gamma Knife Surgery for Giant Central Neurocytoma. ( 27777164 )
2017
13
Teaching NeuroImages: Central neurocytoma. ( 28871071 )
2017
14
Ventricular Central Neurocytoma: Rate of Shunting and Outcome 2 Years After Total and Subtotal Excision. ( 28120072 )
2017
15
Clinical characteristics and prognostic factors of brain central neurocytoma. ( 27542237 )
2016
16
Central Neurocytoma: A Review of Clinical Management and Histopathologic Features. ( 27867912 )
2016
17
Magnetic Resonance Imaging of a Case of Central Neurocytoma. ( 28077908 )
2016
18
Rapid tumor growth with glial differentiation of central neurocytoma after stereotactic radiosurgery. ( 27242062 )
2016
19
Pediatric Central Neurocytoma: A Short Series With Literature Review. ( 27664193 )
2016
20
Atypical central neurocytoma with metastatic craniospinal dissemination: a case report. ( 27450443 )
2016
21
Pediatric central neurocytoma: Case report and review of literature. ( 28217163 )
2016
22
MR Spectroscopy to Distinguish between Supratentorial Intraventricular Subependymoma and Central Neurocytoma. ( 27941295 )
2016
23
Treatment results of gamma knife radiosurgery for central neurocytoma: report of a Japanese multi-institutional co-operative study. ( 26987638 )
2016
24
Intraoperative high-field MRI maximizes the extent of resection in intraventricular central neurocytoma surgery. ( 26964476 )
2016
25
Tetraventricular central neurocytoma: A rare presentation with imaging-pathologic correlation. ( 26752911 )
2015
26
Clinical outcome and quality of life after treatment of patients with central neurocytoma. ( 25432186 )
2015
27
(P043)A Central neurocytoma: impact of resection extent and adjuvant radiotherapy on survival outcomes. ( 25930937 )
2015
28
The management of central neurocytoma: radiosurgery. ( 25432182 )
2015
29
Central neurocytoma: establishment of the disease entity. ( 25432177 )
2015
30
Role of adjuvant radiation in the management of central neurocytoma: Experience from a tertiary cancer care center of India. ( 26960488 )
2015
31
Histology and molecular aspects of central neurocytoma. ( 25432180 )
2015
32
The characteristics of neuronal stem cells of central neurocytoma. ( 25432181 )
2015
33
Clinical manifestations of central neurocytoma. ( 25432178 )
2015
34
Clinical outcome and prognostic factors for central neurocytoma: twenty year institutional experience. ( 26493740 )
2015
35
The management of residual or recurrent central neurocytoma. ( 25432185 )
2015
36
The management of incidental central neurocytoma. ( 25432184 )
2015
37
The management of central neurocytoma: radiotherapy. ( 25432183 )
2015
38
Estimation of proliferative potentiality of central neurocytoma: correlational analysis of minimum ADC and maximum SUV with MIB-1 labeling index. ( 24477268 )
2014
39
Intraventricular hemorrhage--unusual presentation of central neurocytoma. ( 25591695 )
2014
40
Atypical central neurocytoma with sarcomatous differentiation. ( 25171707 )
2014
41
Long-term outcomes of adjuvant radiotherapy after surgical resection of central neurocytoma. ( 25373333 )
2014
42
Central neurocytoma with ependymoma-like glial component. ( 25348672 )
2014
43
Differential hypermethylation of death-associated protein kinase promoter in central neurocytoma and oligodendroglioma. ( 24877104 )
2014
44
Postoperative complications of central neurocytoma. ( 24036838 )
2013
45
Primary central neurocytoma of the mesencephalic tectum in a pediatric patient. ( 23958899 )
2013
46
Interhemispheric transcallosal approach for resection of intraventricular central neurocytoma. ( 23282154 )
2013
47
Clinical Course of Central Neurocytoma with Malignant Transformation-An Indication for Craniospinal Irradiation. ( 24122623 )
2013
48
Molecular characterization of central neurocytomas: potential markers for tumor typing and progression. ( 22816789 )
2013
49
Update on the diagnosis, pathogenesis, and treatment strategies for central neurocytoma. ( 23810386 )
2013
50
Radiosurgery for central neurocytoma: long-term outcome and failure pattern. ( 24065568 )
2013

Variations for Central Neurocytoma

Copy number variations for Central Neurocytoma from CNVD:

7 (show top 50) (show all 66)
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 13818 1 1 7200000 Loss Neurocytoma
2 13819 1 1 7200000 Loss CDC2L1 Central neurocytomas
3 30471 1 243700000 249250621 Gain Neurocytoma
4 30472 1 243700000 249250621 Gain OR5BF1 Central neurocytomas
5 32215 1 34600000 40100000 Loss Neurocytoma
6 32216 1 34600000 40100000 Loss MYCL1 Central neurocytomas
7 42690 10 38000000 42300000 Loss Central neurocytomas
8 42691 10 38000000 42300000 Loss Neurocytoma
9 47057 10 89500000 92900000 Gain Neurocytoma
10 47162 10 89623194 89728532 Gain PTEN Central neurocytomas
11 48339 11 1 2800000 Loss Neurocytoma
12 48340 11 1 2800000 Loss HRAS Central neurocytomas
13 49707 11 110400000 121200000 Gain Neurocytoma
14 49709 11 110400000 121200000 Gain MLL Central neurocytomas
15 51786 11 130800000 135006516 Gain Central neurocytomas
16 51787 11 130800000 135006516 Gain Neurocytoma
17 54397 11 43500000 48800000 Gain Neurocytoma
18 54398 11 43500000 48800000 Gain KAI1 Central neurocytomas
19 61830 12 101673905 101685205 Loss DRIM Central neurocytomas
20 61836 12 101786902 101801572 Loss ARL1 Central neurocytomas
21 73747 12 96200000 109000000 Loss Neurocytoma
22 74691 13 110300000 115169878 Loss Central neurocytomas
23 74693 13 110300000 115169878 Loss Neurocytoma
24 77106 13 40100000 55300000 Loss Neurocytoma
25 77107 13 40100000 55300000 Loss RB1 Central neurocytomas
26 81625 14 104000000 107349540 Gain Neurocytoma
27 81627 14 104000000 107349540 Gain IGH Central neurocytomas
28 89768 15 19000000 33600000 Gain Neurocytoma
29 90822 15 23985488 24185809 Gain Central neurocytomas
30 90905 15 25700000 28100000 Loss Neurocytoma
31 90906 15 25700000 28100000 Loss SNRPN Central neurocytomas
32 96486 15 89100000 102531392 Loss Central neurocytomas
33 105389 16 79200000 81700000 Loss Neurocytoma
34 105390 16 79200000 81700000 Loss FRA16D Central neurocytomas
35 106531 16 88700000 90354753 Gain Neurocytoma
36 106838 17 1 3300000 Loss Neurocytoma
37 106839 17 1 3300000 Loss HIC1 Central neurocytomas
38 106841 17 1 3300000 Loss PAFAH Central neurocytomas
39 109411 17 25800000 38100000 Loss Neurocytoma
40 112094 17 37856253 37884915 Loss ERBB2 Central neurocytomas
41 112175 17 38100000 50200000 Loss Neurocytoma
42 112177 17 38100000 50200000 Loss BRCA1 Central neurocytomas
43 115446 17 57600000 62600000 Loss Neurocytoma
44 115447 17 57600000 62600000 Loss RPS6KB1 Central neurocytomas
45 133735 19 6900000 13900000 Loss Neurocytoma
46 133738 19 6900000 13900000 Loss ZNF439 Central neurocytomas
47 135912 2 122400000 129900000 Loss Neurocytoma
48 136164 2 127805598 127864903 Loss BIN1 Central neurocytomas
49 140117 2 19200000 24000000 Gain Neurocytoma
50 140118 2 19200000 24000000 Gain MYCN Central neurocytomas

Expression for Central Neurocytoma

Search GEO for disease gene expression data for Central Neurocytoma.

Pathways for Central Neurocytoma

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

# Super pathways Score Top Affiliating Genes
1 11.89 ENO2 GFAP MAP2 NDRG1 SYP
2 10.82 GFAP MAP2 NES SYP

GO Terms for Central Neurocytoma

Cellular components related to Central Neurocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament cytoskeleton GO:0045111 8.96 GFAP NES
2 myelin sheath GO:0043209 8.8 ENO2 GFAP NDRG1

Biological processes related to Central Neurocytoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cellular response to organic substance GO:0071310 8.96 MAP2 SYP
2 mast cell activation GO:0045576 8.62 CHGA NDRG1

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

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 8.8 GFAP MAP2 NES

Sources for Central Neurocytoma

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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