MCID: CNT019
MIFTS: 48

Central Neurocytoma

Categories: Cancer diseases, Eye diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Central Neurocytoma

MalaCards integrated aliases for Central Neurocytoma:

Name: Central Neurocytoma 12 54 60 15 17 74
Neurocytoma 45 74
Neurocytoma Central 56
Neurolipocytoma 12

Characteristics:

Orphanet epidemiological data:

60
central neurocytoma
Inheritance: Not applicable; Age of onset: Adolescent,Adult,Childhood;

Classifications:

Orphanet: 60  
Rare neurological diseases


Summaries for Central Neurocytoma

NIH Rare Diseases : 54 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 cerebellar liponeurocytoma and oligodendroglioma, and has symptoms including vomiting and headache. 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 Vincristine have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and cerebellum, and related phenotypes are hydrocephalus and abnormal cellular phenotype

Wikipedia : 77 Central neurocytoma (CNC) is an extremely rare, ordinarily benign intraventricular brain tumour that... 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 220)
# Related Disease Score Top Affiliating Genes
1 cerebellar liponeurocytoma 30.8 CHGA ENO2 GFAP MAP2 SYP
2 oligodendroglioma 30.5 ENO2 GFAP SYP
3 extraventricular neurocytoma 30.3 GFAP SYP
4 rosette-forming glioneuronal tumor 30.1 MAP2 SYP
5 anaplastic ganglioglioma 30.0 GFAP NES
6 cystic teratoma 29.9 ENO2 GFAP SYP
7 cerebral neuroblastoma 29.9 ENO2 GFAP SYP
8 spinal meningioma 29.9 CHGA ENO2
9 meningioma, familial 29.8 ENO2 GFAP SYP
10 dysembryoplastic neuroepithelial tumor 29.5 GFAP MAP2 NES SYP
11 olfactory neuroblastoma 29.5 CHGA ENO2 SYP
12 ependymoma 29.4 ENO2 GFAP NES SYP
13 teratoma 29.4 ENO2 GFAP NES SYP
14 hydrocephalus 29.3 ENO2 GFAP NES SYP
15 medulloblastoma 29.0 ENO2 GFAP MAP2 NES SYP
16 subependymoma 28.7 CHGA ENO2 GFAP NES SYP
17 ganglioglioma 28.4 CHGA ENO2 GFAP MAP2 NES SYP
18 papillary glioneuronal tumors 11.1
19 atypical teratoid rhabdoid tumor 10.3 GFAP SYP
20 giant cell glioblastoma 10.3 GFAP SYP
21 cerebellopontine angle tumor 10.3 GFAP SYP
22 pilomyxoid astrocytoma 10.3 GFAP SYP
23 alveolar soft part sarcoma 10.3 ENO2 SYP
24 chondrosarcoma, extraskeletal myxoid 10.3 ENO2 SYP
25 peripheral nervous system neoplasm 10.3 ENO2 SYP
26 mixed cell type cancer 10.3 ENO2 SYP
27 sweat gland cancer 10.3 ENO2 SYP
28 adrenal rest tumor 10.3 ENO2 SYP
29 adrenal carcinoma 10.3 ENO2 SYP
30 intraocular retinoblastoma 10.3 ENO2 SYP
31 pineal parenchymal tumor of intermediate differentiation 10.3 ENO2 SYP
32 basaloid squamous cell carcinoma 10.3 ENO2 SYP
33 cerebral primitive neuroectodermal tumor 10.3 ENO2 SYP
34 brain injury 10.3 ENO2 GFAP
35 traumatic brain injury 10.3 ENO2 GFAP
36 ileum cancer 10.3 ENO2 GFAP
37 desmoplastic infantile ganglioglioma 10.3 GFAP SYP
38 granular cell tumor 10.3 ENO2 GFAP
39 status epilepticus 10.3 ENO2 GFAP
40 ganglioneuroblastoma 10.3 ENO2 SYP
41 dysgerminoma of ovary 10.3 ENO2 GFAP
42 juvenile pilocytic astrocytoma 10.3 ENO2 GFAP
43 lung combined type small cell carcinoma 10.3 GFAP SYP
44 neuroma 10.3 ENO2 GFAP
45 carotid body cancer 10.3 ENO2 SYP
46 malignant sertoli cell tumor 10.3 ENO2 SYP
47 toxic encephalopathy 10.2 GFAP MAP2
48 sinonasal undifferentiated carcinoma 10.2 ENO2 SYP
49 pancreatic somatostatinoma 10.2 ENO2 SYP
50 cutaneous ganglioneuroma 10.2 ENO2 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:

60 33 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 60 33 obligate (100%) Obligate (100%) HP:0000238
2 abnormal cellular phenotype 60 33 hallmark (90%) Very frequent (99-80%) HP:0025354
3 intracranial cystic lesion 60 33 hallmark (90%) Very frequent (99-80%) HP:0010576
4 abnormality of lateral ventricle 60 33 hallmark (90%) Very frequent (99-80%) HP:0030047
5 depressivity 60 33 frequent (33%) Frequent (79-30%) HP:0000716
6 ataxia 60 33 frequent (33%) Frequent (79-30%) HP:0001251
7 nausea and vomiting 60 33 frequent (33%) Frequent (79-30%) HP:0002017
8 cerebral calcification 60 33 frequent (33%) Frequent (79-30%) HP:0002514
9 increased intracranial pressure 60 33 frequent (33%) Frequent (79-30%) HP:0002516
10 abnormality of vision 60 33 frequent (33%) Frequent (79-30%) HP:0000504
11 headache 60 33 frequent (33%) Frequent (79-30%) HP:0002315
12 decreased corneal reflex 60 33 frequent (33%) Frequent (79-30%) HP:0008000
13 babinski sign 60 33 occasional (7.5%) Occasional (29-5%) HP:0003487
14 paresthesia 60 33 occasional (7.5%) Occasional (29-5%) HP:0003401
15 coma 60 33 occasional (7.5%) Occasional (29-5%) HP:0001259
16 lethargy 60 33 occasional (7.5%) Occasional (29-5%) HP:0001254
17 tinnitus 60 33 occasional (7.5%) Occasional (29-5%) HP:0000360
18 postural instability 60 33 occasional (7.5%) Occasional (29-5%) HP:0002172
19 pain insensitivity 60 33 occasional (7.5%) Occasional (29-5%) HP:0007021

UMLS symptoms related to Central Neurocytoma:


vomiting, headache

MGI Mouse Phenotypes related to Central Neurocytoma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 9.1 CHGA ENO2 GFAP MAP2 NDRG1 SYP

Drugs & Therapeutics for Central Neurocytoma

Drugs for Central 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 Central Neurocytoma

Cochrane evidence based reviews: neurocytoma

Genetic Tests for Central Neurocytoma

Anatomical Context for Central Neurocytoma

MalaCards organs/tissues related to Central Neurocytoma:

42
Brain, Spinal Cord, Cerebellum, Ovary, Pituitary, Kidney, Retina

Publications for Central Neurocytoma

Articles related to Central Neurocytoma:

(show top 50) (show all 324)
# Title Authors Year
1
Atypical central neurocytoma with novel EWSR1-ATF1 fusion and MUTYH mutation detected by next-generation sequencing. ( 30642852 )
2019
2
Mesial temporal extraventricular neurocytoma (mtEVN): A case report and literature review. ( 30603610 )
2019
3
The epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014. ( 30859483 )
2019
4
Somatostatin Receptor Ligand Therapy-A Novel Therapy for Neurocytoma. ( 30722009 )
2019
5
Neurocytoma mimicking macroadenoma. ( 30775062 )
2019
6
Letter to the Editor Regarding "Clinical Features, Treatment, and Long-Term Outcomes of Central Neurocytoma: 20-Year Experience at a Single Center". ( 29499602 )
2018
7
Diagnostic value of six MRI features for central neurocytoma. ( 29713774 )
2018
8
Clinical Features, Treatment, and Long-term Outcomes of Central Neurocytoma: A 20-Year Experience at a Single Center. ( 28958923 )
2018
9
Central neurocytoma represents a tumor consisting of diverse neuronal phenotypes. ( 29747899 )
2018
10
Central neurocytoma: Clinical characteristics, patterns of care, and survival. ( 29685410 )
2018
11
The role of proton beam therapy in central neurocytoma: A single-institution experience. ( 29706302 )
2018
12
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
13
Intramedullary central neurocytoma of the thoracic spinal cord: A case report and literature review. ( 29541463 )
2018
14
Novel case of recurrent intraventricular atypical central neurocytoma with prominent gangliogliomatous differentiation in a 10-year-old boy with 10 years of follow up. ( 30039530 )
2018
15
Relative survival of patients with central neurocytoma. ( 30041900 )
2018
16
Central Neurocytoma - A Rare Brain Tumor. ( 30347963 )
2018
17
Paranoid Syndrome as the First Sign of Central Neurocytoma: A Case Report. ( 30427824 )
2018
18
Tetraventricular Atypical Central Neurocytoma - Case Report. ( 30439524 )
2018
19
Tumor control of third ventricular central neurocytoma after gamma knife radiosurgery in an elderly patient: A case report and literature review. ( 30558064 )
2018
20
Central Neurocytoma: A Rare Presentation in the Fourth Ventricle and Review of the Literature. ( 30579012 )
2018
21
Imaging characteristics of cerebral extraventricular neurocytoma with pathological correlation. ( 30062611 )
2018
22
Malignant progression of an extraventricular neurocytoma arising from the VIIIth cranial nerve: A case report and literature review. ( 30588667 )
2018
23
Extra-ventricular neurocytoma: An enigmatic tumor. ( 29547156 )
2018
24
Congenital Extra-Ventricular (Ganglio)Neurocytoma of the Brain Stem: A Case Report. ( 29868519 )
2018
25
Oridonin inhibits growth and induces apoptosis of human neurocytoma cells via the Wnt/β-catenin pathway. ( 30127932 )
2018
26
Systematic Analysis of Clinical Outcomes Following Stereotactic Radiosurgery for Central Neurocytoma. ( 28516073 )
2017
27
Clinical, radiological, pathological and prognostic aspects of intraventricular oligodendroglioma: comparison with central neurocytoma. ( 28900829 )
2017
28
Spontaneous intracerebral hemorrhage as presentation of atypical central neurocytoma: the role of angiogenesis through the characterization of tumor endothelial cells. ( 29215136 )
2017
29
Upfront Gamma Knife Surgery for Giant Central Neurocytoma. ( 27777164 )
2017
30
Teaching NeuroImages: Central neurocytoma. ( 28871071 )
2017
31
Ventricular Central Neurocytoma: Rate of Shunting and Outcome 2 Years After Total and Subtotal Excision. ( 28120072 )
2017
32
Clinical characteristics and prognostic factors of brain central neurocytoma. ( 27542237 )
2016
33
Central Neurocytoma: A Review of Clinical Management and Histopathologic Features. ( 27867912 )
2016
34
Magnetic Resonance Imaging of a Case of Central Neurocytoma. ( 28077908 )
2016
35
Rapid tumor growth with glial differentiation of central neurocytoma after stereotactic radiosurgery. ( 27242062 )
2016
36
Pediatric Central Neurocytoma: A Short Series With Literature Review. ( 27664193 )
2016
37
Atypical central neurocytoma with metastatic craniospinal dissemination: a case report. ( 27450443 )
2016
38
Pediatric central neurocytoma: Case report and review of literature. ( 28217163 )
2016
39
MR Spectroscopy to Distinguish between Supratentorial Intraventricular Subependymoma and Central Neurocytoma. ( 27941295 )
2016
40
Treatment results of gamma knife radiosurgery for central neurocytoma: report of a Japanese multi-institutional co-operative study. ( 26987638 )
2016
41
Intraoperative high-field MRI maximizes the extent of resection in intraventricular central neurocytoma surgery. ( 26964476 )
2016
42
A Case of Prenatal Neurocytoma Associated With ATR-16 Syndrome. ( 27235459 )
2016
43
Atypical thalamic neurocytoma: A rare neoplasm. ( 26755019 )
2016
44
Tetraventricular central neurocytoma: A rare presentation with imaging-pathologic correlation. ( 26752911 )
2015
45
Clinical outcome and quality of life after treatment of patients with central neurocytoma. ( 25432186 )
2015
46
(P043)A Central neurocytoma: impact of resection extent and adjuvant radiotherapy on survival outcomes. ( 25930937 )
2015
47
The management of central neurocytoma: radiosurgery. ( 25432182 )
2015
48
Central neurocytoma: establishment of the disease entity. ( 25432177 )
2015
49
Role of adjuvant radiation in the management of central neurocytoma: Experience from a tertiary cancer care center of India. ( 26960488 )
2015
50
Histology and molecular aspects of central neurocytoma. ( 25432180 )
2015

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 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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