MCID: CNT019
MIFTS: 44

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 53 59 15 17 72
Neurocytoma 44 72
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
UMLS via Orphanet 73 C0206719
Orphanet 59 ORPHA73256
UMLS 72 C0206719 C1622510

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: 73256DefinitionCentral 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 obstructive hydrocephalus, 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 Trametinib and Dabrafenib have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and cerebellum, and related phenotypes are hydrocephalus and intracranial cystic lesion

Wikipedia : 75 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 273)
# Related Disease Score Top Affiliating Genes
1 cerebellar liponeurocytoma 31.6 SYP MAP2 GFAP ENO2 CHGA
2 obstructive hydrocephalus 31.5 SYP GFAP
3 extraventricular neurocytoma 31.3 SYP GFAP
4 benign ependymoma 31.3 SYP GFAP ENO2
5 oligodendroglioma 30.9 SYP PCNA GFAP ENO2
6 rosette-forming glioneuronal tumor 30.8 SYP MAP2
7 ganglioneuroblastoma 30.7 SYP ENO2
8 neuroma 30.7 GFAP ENO2
9 cerebral neuroblastoma 30.7 SYP GFAP ENO2
10 hydrocephalus 30.7 SYP NES GFAP ENO2
11 papilloma of choroid plexus 30.7 SYP GFAP
12 ependymoma 30.6 SYP NES GFAP ENO2
13 anaplastic ganglioglioma 30.5 NES GFAP
14 meningioma, radiation-induced 30.5 PCNA GFAP
15 fibrillary astrocytoma 30.4 PCNA GFAP
16 spinal meningioma 30.4 ENO2 CHGA
17 clear cell ependymoma 30.3 SYP GFAP ENO2
18 cystic teratoma 30.3 SYP GFAP ENO2
19 central nervous system benign neoplasm 30.3 SYP GFAP ENO2
20 hemangioblastoma 30.2 SYP GFAP ENO2
21 paraganglioma 30.0 SYP ENO2 CHGA
22 olfactory neuroblastoma 29.9 SYP ENO2 CHGA
23 meningioma, familial 29.9 SYP PCNA GFAP ENO2
24 cellular ependymoma 29.9 SYP GFAP CHGA
25 subependymal giant cell astrocytoma 29.9 SYP NES GFAP ENO2
26 dysembryoplastic neuroepithelial tumor 29.8 SYP NES MAP2 GFAP
27 central nervous system cancer 29.6 SYP NES GFAP ENO2
28 teratoma 29.6 SYP NES GFAP ENO2
29 pineocytoma 29.4 SYP GFAP ENO2 CHGA
30 ganglioneuroma 29.4 SYP GFAP ENO2 CHGA
31 medulloblastoma 29.4 SYP NES MAP2 GFAP ENO2
32 subependymoma 29.3 SYP NES GFAP ENO2 CHGA
33 ewing sarcoma 28.7 SYP NES GFAP ENO2 CHGA
34 ganglioglioma 28.4 SYP NES MAP2 GFAP ENO2 CHGA
35 papillary glioneuronal tumors 11.6
36 congenital hydrocephalus 10.7
37 desmoplastic infantile ganglioglioma 10.6 SYP GFAP
38 lung combined type small cell carcinoma 10.6 SYP GFAP
39 carotid body cancer 10.6 SYP ENO2
40 malignant sertoli cell tumor 10.6 SYP ENO2
41 sinonasal undifferentiated carcinoma 10.6 SYP ENO2
42 extrahepatic bile duct adenocarcinoma 10.6 SYP ENO2
43 cutaneous ganglioneuroma 10.6 SYP ENO2
44 pancreatic somatostatinoma 10.6 SYP ENO2
45 neuronal tumor 10.6
46 middle ear adenoma 10.6 SYP ENO2
47 angiocentric glioma 10.6 SYP GFAP
48 orbital cancer 10.6 SYP ENO2
49 sensory organ benign neoplasm 10.6 SYP ENO2
50 intracranial cysts 10.6 GFAP ENO2

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 19)
# 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 abnormal cellular phenotype 59 32 hallmark (90%) Very frequent (99-80%) HP:0025354
4 abnormality of lateral ventricle 59 32 hallmark (90%) Very frequent (99-80%) HP:0030047
5 depressivity 59 32 frequent (33%) Frequent (79-30%) HP:0000716
6 ataxia 59 32 frequent (33%) Frequent (79-30%) HP:0001251
7 nausea and vomiting 59 32 frequent (33%) Frequent (79-30%) HP:0002017
8 cerebral calcification 59 32 frequent (33%) Frequent (79-30%) HP:0002514
9 increased intracranial pressure 59 32 frequent (33%) Frequent (79-30%) HP:0002516
10 abnormality of vision 59 32 frequent (33%) Frequent (79-30%) HP:0000504
11 headache 59 32 frequent (33%) Frequent (79-30%) HP:0002315
12 decreased corneal reflex 59 32 frequent (33%) Frequent (79-30%) HP:0008000
13 babinski sign 59 32 occasional (7.5%) Occasional (29-5%) HP:0003487
14 paresthesia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003401
15 coma 59 32 occasional (7.5%) Occasional (29-5%) HP:0001259
16 lethargy 59 32 occasional (7.5%) Occasional (29-5%) HP:0001254
17 tinnitus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000360
18 postural instability 59 32 occasional (7.5%) Occasional (29-5%) HP:0002172
19 pain insensitivity 59 32 occasional (7.5%) Occasional (29-5%) HP:0007021

UMLS symptoms related to Central Neurocytoma:


vomiting, headache

MGI Mouse Phenotypes related to Central Neurocytoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 nervous system MP:0003631 9.17 CHGA DAPK1 ENO2 GFAP MAP2 NDRG1

Drugs & Therapeutics for Central Neurocytoma

Drugs for Central Neurocytoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Trametinib Approved Phase 4 871700-17-3 11707110
2
Dabrafenib Approved, Investigational Phase 4 1195765-45-7 44462760 44516822
3 Protein Kinase Inhibitors Phase 4
4
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
5
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
6 Antineoplastic Agents, Phytogenic Phase 2
7 Tubulin Modulators Phase 2
8 Antimitotic Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 An Open Label, Multi-center Roll-over Study to Assess Long-term Effect in Pediatric Patients Treated With Tafinlar (Dabrafenib) and/or Mekinist (Trametinib) Not yet recruiting NCT03975829 Phase 4 dabrafenib;trametinib
2 Phase II Study of Antineoplastons A10 and AS2-1 in Children With Brain Tumors Completed NCT00003458 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
3 Phase II Study Of Antineoplastons A10 And AS2-1 In Patients With Brain Tumors. Completed NCT00003457 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
4 Phase II Open-label Global Study to Evaluate the Effect of Dabrafenib in Combination With Trametinib in Children and Adolescent Patients With BRAF V600 Mutation Positive Low Grade Glioma (LGG) or Relapsed or Refractory High Grade Glioma (HGG) 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, Cerebellum, Temporal Lobe, Ovary, Pituitary, Liver

Publications for Central Neurocytoma

Articles related to Central Neurocytoma:

(show top 50) (show all 644)
# Title Authors PMID Year
1
Synaptophysin negative central neurocytoma. 9 38
20429311 2010
2
Central neurocytoma: a clinical, radiological and pathological study of nine cases. 9 38
18022760 2008
3
Oligodendroglioma with neurocytic differentiation versus atypical extraventricular neurocytoma: a case report of unusual pathologic findings of a spinal cord tumor. 9 38
17039400 2007
4
OLIG2 is a useful immunohistochemical marker in differential diagnosis of clear cell primary CNS neoplasms. 9 38
17257132 2007
5
Pigmented central neurocytoma. 9 38
14991386 2004
6
Forskolin promotes astroglial differentiation of human central neurocytoma cells. 9 38
15031671 2004
7
Diagnostic value of microtubule-associated protein-2 (MAP-2) for neuroendocrine neoplasms. 9 38
12605092 2003
8
Genetic differences between neurocytoma and dysembryoplastic neuroepithelial tumor and oligodendroglial tumors. 9 38
12507133 2002
9
Skull base neurocytoma: case report and review of the literature of extraventricular neurocytomas. 9 38
17167646 2002
10
So-called malignant and extra-ventricular neurocytomas: reality or wrong diagnosis? A critical review about two overdiagnosed cases. 9 38
11083081 2000
11
alpha-Synuclein is expressed in a variety of brain tumors showing neuronal differentiation. 9 38
10672322 2000
12
Central neurocytoma with malignant course. Neuronal and glial differentiation and craniospinal dissemination. 9 38
10393370 1999
13
Tissue culture of human neurocytomas induces the expression of glial fibrilary acidic protein. 9 38
10451427 1998
14
Synaptophysin staining in normal brain: importance for diagnosis of ganglioglioma. 9 38
9591724 1998
15
Neuronal and glial characteristics of central neurocytoma: electron microscopical analysis of two cases. 9 38
8781655 1996
16
Immunohistochemistry of synapsin I and synaptophysin in human nervous system and neuroendocrine tumors. Applications in diagnostic neuro-oncology. 9 38
8287627 1993
17
Immunocytochemical detection of calcineurin and microtubule-associated protein 2 in central neurocytoma. 9 38
8410138 1993
18
Cytoskeletal immunohistochemistry of central neurocytomas. 9 38
1471723 1992
19
Periventricular neurocytoma: a pathological entity. 9 38
1615372 1992
20
Central neurocytoma: immunohistochemical and ultrastructural study. 9 38
2028745 1991
21
Patterns of differentiation in central neurocytoma. An immunohistochemical study of eleven biopsies. 9 38
2109481 1990
22
Atypical Extraventricular Neurocytoma: Imaging Findings of Disregarded Diagnosis. 38
31426251 2019
23
Glioneuronal Tumor With Features of Ganglioglioma and Neurocytoma Arising in the Fourth Ventricle: A Report of 2 Unusual Cases and a Review of Infratentorial Gangliogliomas. 38
31361005 2019
24
Histogram Analysis Parameters ADC for Distinguishing Ventricular Neoplasms of Ependymoma, Choroid Plexus Papilloma, and Central Neurocytoma. 38
31390342 2019
25
Central neurocytoma: SNP array analyses, subtel FISH, and review of the literature. 38
31000381 2019
26
Somatostatin Receptor Ligand Therapy-A Potential Therapy for Neurocytoma. 38
30722009 2019
27
Bifocal germinoma of the septum pellucidum and the sellar-supra-sellar region: An uncommon presentation for a rare tumor. 38
30886913 2019
28
The epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014. 38
30859483 2019
29
Successful Central Neurocytoma Management: A Rare Brain Tumor. 38
31036130 2019
30
Malignant progression of an extraventricular neurocytoma arising from the VIIIth cranial nerve: A case report and literature review. 38
30588667 2019
31
Trends in Primary Brain Tumors: A 5-Year Retrospective Histologically Confirmed Study in Tabriz, Iran, 2011-2016. 38
31143257 2019
32
Central Neurocytoma: Rare Presentation in Fourth Ventricle and Review of Literature. 38
30579012 2019
33
Hypothalamic Vasopressin-Producing Tumors: Often Inappropriate Diuresis But Occasionally Cushing Disease. 38
30379651 2019
34
Two Extraordinary Sellar Neuronal Tumors: Literature Review and Comparison of Clinicopathologic Features. 38
30551183 2019
35
Tetraventricular Atypical Central Neurocytoma. 38
30439524 2019
36
Flexible Fiber CO2 Laser in Microsurgical Treatment of Intraventricular Tumors: Usefulness and Limitations. 38
30342263 2019
37
Radiosurgery for Central Neurocytoma. 38
31096256 2019
38
Complete resection of hypervascularised extraventricular neurocytoma after preoperative embolisation. 38
29680750 2019
39
Atypical central neurocytoma with novel EWSR1-ATF1 fusion and MUTYH mutation detected by next-generation sequencing. 38
30642852 2019
40
Neurocytoma mimicking macroadenoma. 38
30775062 2019
41
Mesial temporal extraventricular neurocytoma (mtEVN): A case report and literature review. 38
30603610 2019
42
Prognosis and treatment outcomes of central neurocytomas: clinical interrogation based on a single center experience. 38
30225773 2018
43
Tumor control of third ventricular central neurocytoma after gamma knife radiosurgery in an elderly patient: A case report and literature review. 38
30558064 2018
44
Imaging characteristics of cerebral extraventricular neurocytoma with pathological correlation. 38
30062611 2018
45
Central Nervous System-type Neuroepithelial Tumors and Tumor-like Proliferations Developing in the Gynecologic Tract and Pelvis: Clinicopathologic Analysis of 23 Cases. 38
30074494 2018
46
MRI of intracranial intraventricular lesions. 38
30138862 2018
47
Diagnostic value of six MRI features for central neurocytoma. 38
29713774 2018
48
Novel case of recurrent intraventricular atypical central neurocytoma with prominent gangliogliomatous differentiation in a 10-year-old boy with 10 years of follow up. 38
30039530 2018
49
A pituitary tumor turned-to-be a glioma: A surgical case from the Madras General Hospital 1928. 38
30829288 2018
50
Paranoid Syndrome as the First Sign of Central Neurocytoma: A Case Report. 38
30427824 2018

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 42690 10 38000000 42300000 Loss Central neurocytomas
4 42691 10 38000000 42300000 Loss Neurocytoma
5 47162 10 89623194 89728532 Gain PTEN Central neurocytomas
6 48339 11 1 2800000 Loss Neurocytoma
7 48340 11 1 2800000 Loss HRAS Central neurocytomas
8 49707 11 110400000 121200000 Gain Neurocytoma
9 49709 11 110400000 121200000 Gain MLL Central neurocytomas
10 54397 11 43500000 48800000 Gain Neurocytoma
11 54398 11 43500000 48800000 Gain KAI1 Central neurocytomas
12 73747 12 96200000 109000000 Loss Neurocytoma
13 74691 13 110300000 115169878 Loss Central neurocytomas
14 74693 13 110300000 115169878 Loss Neurocytoma
15 77106 13 40100000 55300000 Loss Neurocytoma
16 77107 13 40100000 55300000 Loss RB1 Central neurocytomas
17 106531 16 88700000 90354753 Gain Neurocytoma
18 106838 17 1 3300000 Loss Neurocytoma
19 106839 17 1 3300000 Loss HIC1 Central neurocytomas
20 106841 17 1 3300000 Loss PAFAH Central neurocytomas
21 109411 17 25800000 38100000 Loss Neurocytoma
22 112094 17 37856253 37884915 Loss ERBB2 Central neurocytomas
23 112175 17 38100000 50200000 Loss Neurocytoma
24 112177 17 38100000 50200000 Loss BRCA1 Central neurocytomas
25 115446 17 57600000 62600000 Loss Neurocytoma
26 115447 17 57600000 62600000 Loss RPS6KB1 Central neurocytomas
27 146290 2 47630262 47710360 Gain MSH2 Central neurocytomas
28 146298 2 47747914 47797470 Gain KCNK12 Central neurocytomas
29 150428 20 1 27500000 Loss Central neurocytomas
30 150458 20 1 5100000 Loss Neurocytoma
31 203500 5 98200000 115200000 Loss Neurocytoma
32 212677 6 40500000 57000000 Loss Neurocytoma
33 214137 6 56164088 56364441 Loss Central neurocytomas
34 217392 7 100770378 100782547 Loss SERPINE1 Central neurocytomas
35 192388 5 112043201 112181936 Loss APC Central neurocytomas
36 150626 20 12100000 21300000 Loss Neurocytoma
37 150539 20 10618331 10654694 Loss JAG1 Central neurocytomas
38 144961 2 32100000 41800000 Gain Neurocytoma
39 140118 2 19200000 24000000 Gain MYCN Central neurocytomas
40 140117 2 19200000 24000000 Gain Neurocytoma
41 136164 2 127805598 127864903 Loss BIN1 Central neurocytomas
42 135912 2 122400000 129900000 Loss Neurocytoma
43 133738 19 6900000 13900000 Loss ZNF439 Central neurocytomas
44 133735 19 6900000 13900000 Loss Neurocytoma
45 105390 16 79200000 81700000 Loss FRA16D Central neurocytomas
46 105389 16 79200000 81700000 Loss Neurocytoma
47 96486 15 89100000 102531392 Loss Central neurocytomas
48 90906 15 25700000 28100000 Loss SNRPN Central neurocytomas
49 90905 15 25700000 28100000 Loss Neurocytoma
50 90822 15 23985488 24185809 Gain 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 SYP NDRG1 MAP2 GFAP ENO2
2 10.82 SYP NES MAP2 GFAP

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 8.62 NES GFAP

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 SYP MAP2
2 mast cell activation GO:0045576 8.62 NDRG1 CHGA

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 8.62 MAP2 GFAP

Sources for Central Neurocytoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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