MCID: CNT019
MIFTS: 46

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 52 58 15 17 71
Neurocytoma 43 71
Neurocytoma Central 54
Neurolipocytoma 12

Characteristics:

Orphanet epidemiological data:

58
central neurocytoma
Inheritance: Not applicable; Age of onset: Adolescent,Adult,Childhood;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:14174
MeSH 43 D018306
NCIt 49 C3791
UMLS via Orphanet 72 C0206719
Orphanet 58 ORPHA73256
UMLS 71 C0206719 C1622510

Summaries for Central Neurocytoma

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 73256 Definition Central 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 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 Crest Differentiation. The drugs Trametinib and Dabrafenib have been mentioned in the context of this disorder. Affiliated tissues include brain, pituitary and spinal cord, and related phenotypes are hydrocephalus and intracranial cystic lesion

Wikipedia : 74 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 318)
# Related Disease Score Top Affiliating Genes
1 cerebellar liponeurocytoma 33.0 SYP MAP2 ENO2
2 hydrocephalus 31.2 SYP NES GFAP ENO2
3 extraventricular neurocytoma 31.1 SYP GFAP
4 cellular ependymoma 31.1 SYP OLIG2
5 benign ependymoma 31.0 SYP NES GFAP ENO2
6 cerebral neuroblastoma 30.8 SYP GFAP ENO2
7 mature teratoma 30.8 SYP PCNA
8 rosette-forming glioneuronal tumor 30.7 SYP MAP2
9 anaplastic ganglioglioma 30.6 NES GFAP
10 gangliocytoma 30.5 SYP GFAP CHGA
11 obstructive hydrocephalus 30.5 SYP OLIG2 GFAP ENO2
12 papilloma of choroid plexus 30.4 SYP GFAP ENO2
13 cystic teratoma 30.4 SYP GFAP ENO2
14 olfactory neuroblastoma 30.2 SYP ENO2 CHGA
15 hemangioblastoma 30.2 SYP GFAP ENO2
16 subependymoma 30.1 SYP NES GFAP ENO2 CHGA
17 paraganglioma 30.1 SYP GFAP ENO2 CHGA
18 oligodendroglioma 30.0 SYP PCNA OLIG2 GFAP ENO2 DAPK1
19 spinal cancer 30.0 SYP NES GFAP ENO2
20 neuroma 30.0 GFAP FGF2 ENO2
21 pineocytoma 29.9 SYP GFAP ENO2 CHGA
22 ganglioneuroma 29.9 SYP GFAP ENO2 CHGA
23 grade iii astrocytoma 29.9 PCNA OLIG2 NES GFAP
24 subependymal giant cell astrocytoma 29.8 SYP NES GFAP ENO2 CHGA
25 mixed glioma 29.8 OLIG2 NES GFAP
26 clear cell ependymoma 29.7 SYP OLIG2 GFAP ENO2
27 dysembryoplastic neuroepithelial tumor 29.6 SYP OLIG2 NES MAP2 GFAP
28 meningioma, familial 29.5 SYP PCNA OLIG2 GFAP ENO2
29 ganglioglioma 29.5 SYP NES MAP2 GFAP ENO2 CHGA
30 medulloblastoma 29.4 SYP NES MAP2 GFAP FGF2 ENO2
31 central nervous system benign neoplasm 29.3 SYP OLIG2 GFAP ENO2 CHGA
32 teratoma 29.3 SYP NES MAP2 GFAP ENO2 CHGA
33 juvenile pilocytic astrocytoma 29.0 SYP OLIG2 NES GFAP FGF2
34 central nervous system cancer 29.0 SYP OLIG2 NES GFAP FGF2
35 neuroblastoma 28.9 SYP SNCA NES MAP2 GFAP FGF2
36 papillary glioneuronal tumors 11.6
37 neuronal tumor 10.6
38 intracranial hypertension 10.5
39 desmoplastic infantile ganglioglioma 10.5 SYP GFAP
40 malignant melanocytic neoplasm of the peripheral nerve sheath 10.5 SYP ENO2
41 chordoid glioma 10.5 SYP GFAP
42 lung combined type small cell carcinoma 10.5 SYP GFAP
43 anal neuroendocrine tumor 10.5 SYP ENO2
44 intracranial cysts 10.5 GFAP ENO2
45 sinonasal undifferentiated carcinoma 10.5 SYP ENO2
46 primitive neuroectodermal tumor of the cervix uteri 10.5 GFAP ENO2
47 intracranial primitive neuroectodermal tumor 10.5 SYP ENO2
48 bile duct mucinous adenocarcinoma 10.5 SYP ENO2
49 nodular prostate 10.5 SYP ENO2
50 posterior pituitary gland neoplasm 10.5 SYP GFAP

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:

58 31 (show all 19)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 58 31 obligate (100%) Obligate (100%) HP:0000238
2 intracranial cystic lesion 58 31 hallmark (90%) Very frequent (99-80%) HP:0010576
3 abnormal cellular phenotype 58 31 hallmark (90%) Very frequent (99-80%) HP:0025354
4 abnormality of lateral ventricle 58 31 hallmark (90%) Very frequent (99-80%) HP:0030047
5 nausea and vomiting 58 31 frequent (33%) Frequent (79-30%) HP:0002017
6 depressivity 58 31 frequent (33%) Frequent (79-30%) HP:0000716
7 ataxia 58 31 frequent (33%) Frequent (79-30%) HP:0001251
8 cerebral calcification 58 31 frequent (33%) Frequent (79-30%) HP:0002514
9 increased intracranial pressure 58 31 frequent (33%) Frequent (79-30%) HP:0002516
10 abnormality of vision 58 31 frequent (33%) Frequent (79-30%) HP:0000504
11 headache 58 31 frequent (33%) Frequent (79-30%) HP:0002315
12 decreased corneal reflex 58 31 frequent (33%) Frequent (79-30%) HP:0008000
13 babinski sign 58 31 occasional (7.5%) Occasional (29-5%) HP:0003487
14 paresthesia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003401
15 coma 58 31 occasional (7.5%) Occasional (29-5%) HP:0001259
16 lethargy 58 31 occasional (7.5%) Occasional (29-5%) HP:0001254
17 tinnitus 58 31 occasional (7.5%) Occasional (29-5%) HP:0000360
18 postural instability 58 31 occasional (7.5%) Occasional (29-5%) HP:0002172
19 pain insensitivity 58 31 occasional (7.5%) Occasional (29-5%) HP:0007021

UMLS symptoms related to Central Neurocytoma:


vomiting, headache

MGI Mouse Phenotypes related to Central Neurocytoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.61 DAPK1 ENO2 FGF2 GFAP MAP2 NDRG1
2 nervous system MP:0003631 9.36 ARTN CHGA DAPK1 ENO2 FGF2 GFAP

Drugs & Therapeutics for Central Neurocytoma

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

(show all 6)
# 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
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
5
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
6 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:

40
Brain, Pituitary, Spinal Cord, Lung, Breast, Ovary, Pineal

Publications for Central Neurocytoma

Articles related to Central Neurocytoma:

(show top 50) (show all 657)
# Title Authors PMID Year
1
Synaptophysin negative central neurocytoma. 54 61
20429311 2010
2
Central neurocytoma: a clinical, radiological and pathological study of nine cases. 54 61
18022760 2008
3
Oligodendroglioma with neurocytic differentiation versus atypical extraventricular neurocytoma: a case report of unusual pathologic findings of a spinal cord tumor. 54 61
17039400 2007
4
OLIG2 is a useful immunohistochemical marker in differential diagnosis of clear cell primary CNS neoplasms. 54 61
17257132 2007
5
Pigmented central neurocytoma. 54 61
14991386 2004
6
Forskolin promotes astroglial differentiation of human central neurocytoma cells. 54 61
15031671 2004
7
Diagnostic value of microtubule-associated protein-2 (MAP-2) for neuroendocrine neoplasms. 54 61
12605092 2003
8
Genetic differences between neurocytoma and dysembryoplastic neuroepithelial tumor and oligodendroglial tumors. 54 61
12507133 2002
9
Skull base neurocytoma: case report and review of the literature of extraventricular neurocytomas. 54 61
17167646 2002
10
So-called malignant and extra-ventricular neurocytomas: reality or wrong diagnosis? A critical review about two overdiagnosed cases. 54 61
11083081 2000
11
alpha-Synuclein is expressed in a variety of brain tumors showing neuronal differentiation. 54 61
10672322 2000
12
Central neurocytoma with malignant course. Neuronal and glial differentiation and craniospinal dissemination. 54 61
10393370 1999
13
Tissue culture of human neurocytomas induces the expression of glial fibrilary acidic protein. 54 61
10451427 1998
14
Synaptophysin staining in normal brain: importance for diagnosis of ganglioglioma. 54 61
9591724 1998
15
Neuronal and glial characteristics of central neurocytoma: electron microscopical analysis of two cases. 54 61
8781655 1996
16
Immunohistochemistry of synapsin I and synaptophysin in human nervous system and neuroendocrine tumors. Applications in diagnostic neuro-oncology. 54 61
8287627 1993
17
Immunocytochemical detection of calcineurin and microtubule-associated protein 2 in central neurocytoma. 54 61
8410138 1993
18
Cytoskeletal immunohistochemistry of central neurocytomas. 54 61
1471723 1992
19
Periventricular neurocytoma: a pathological entity. 54 61
1615372 1992
20
Central neurocytoma: immunohistochemical and ultrastructural study. 54 61
2028745 1991
21
Patterns of differentiation in central neurocytoma. An immunohistochemical study of eleven biopsies. 54 61
2109481 1990
22
Molecular characterization of histopathological ependymoma variants. 61
31679042 2020
23
In Reply to the Letter to the Editor Regarding "Central Neurocytoma: Rare Presentation in Fourth Ventricle and Review of Literature". 61
31881568 2020
24
Central Neurocytoma Treatment Modalities: A Systematic Review Assessing the Outcomes of Combined Maximal Safe Resection (MSR) and Radiotherapy with Gross Total Resection. 61
32001392 2020
25
Letter to the Editor Regarding "Central Neurocytoma: Rare Presentation in Fourth Ventricle and Review of Literature". 61
31881567 2020
26
Far-anterior Interhemispheric Transcallosal Approach for a Central Neurocytoma in the Lateral Ventricle. 61
31656237 2019
27
Purely neuroendoscopic resection of intraventricular tumors with an endoscopic ultrasonic aspirator. 61
30019320 2019
28
Progeny in an Inhospitable Milieu-Solitary Intraventricular Metastasis From a Triple-Negative Breast Cancer Mimicking Central Neurocytoma: Case Report and Review of Diagnostic Pitfalls and Management Strategies. 61
31863888 2019
29
Application of diffusion-weighted imaging combined with apparent diffusion coefficient in differential diagnosis between central neurocytoma and ependymoma. 61
31853589 2019
30
The role of chemotherapy in the treatment of central neurocytoma. 61
31686534 2019
31
Central neurocytoma in the posterior fossa. 61
31527223 2019
32
Atypical Extraventricular Neurocytoma: Imaging Findings of Disregarded Diagnosis. 61
31426251 2019
33
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. 61
31361005 2019
34
Histogram Analysis Parameters ADC for Distinguishing Ventricular Neoplasms of Ependymoma, Choroid Plexus Papilloma, and Central Neurocytoma. 61
31390342 2019
35
Central Neurocytoma Presenting with Bleed and Obstructive Hydrocephalus: A Rare Presentation. 61
31497129 2019
36
Central neurocytoma: SNP array analyses, subtel FISH, and review of the literature. 61
31000381 2019
37
Somatostatin Receptor Ligand Therapy-A Potential Therapy for Neurocytoma. 61
30722009 2019
38
Bifocal germinoma of the septum pellucidum and the sellar-supra-sellar region: An uncommon presentation for a rare tumor. 61
30886913 2019
39
The epidemiology of central and extraventricular neurocytoma in the United States between 2006 and 2014. 61
30859483 2019
40
Successful Central Neurocytoma Management: A Rare Brain Tumor. 61
31036130 2019
41
Malignant progression of an extraventricular neurocytoma arising from the VIIIth cranial nerve: A case report and literature review. 61
30588667 2019
42
Trends in Primary Brain Tumors: A 5-Year Retrospective Histologically Confirmed Study in Tabriz, Iran, 2011-2016. 61
31143257 2019
43
Central Neurocytoma: Rare Presentation in Fourth Ventricle and Review of Literature. 61
30579012 2019
44
Tetraventricular Atypical Central Neurocytoma. 61
30439524 2019
45
Hypothalamic Vasopressin-Producing Tumors: Often Inappropriate Diuresis But Occasionally Cushing Disease. 61
30379651 2019
46
Two Extraordinary Sellar Neuronal Tumors: Literature Review and Comparison of Clinicopathologic Features. 61
30551183 2019
47
Flexible Fiber CO2 Laser in Microsurgical Treatment of Intraventricular Tumors: Usefulness and Limitations. 61
30342263 2019
48
Atypical central neurocytoma with novel EWSR1-ATF1 fusion and MUTYH mutation detected by next-generation sequencing. 61
30642852 2019
49
Neurocytoma mimicking macroadenoma. 61
30775062 2019
50
Complete resection of hypervascularised extraventricular neurocytoma after preoperative embolisation. 61
29680750 2019

Variations for Central Neurocytoma

Copy number variations for Central Neurocytoma from CNVD:

7 (show top 50) (show all 66)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 48339 11 1 2800000 Loss Neurocytoma
2 48340 11 1 2800000 Loss HRAS Central neurocytomas
3 49707 11 110400000 121200000 Gain Neurocytoma
4 49709 11 110400000 121200000 Gain MLL Central neurocytomas
5 51786 11 130800000 135006516 Gain Central neurocytomas
6 51787 11 130800000 135006516 Gain Neurocytoma
7 74691 13 110300000 115169878 Loss Central neurocytomas
8 74693 13 110300000 115169878 Loss Neurocytoma
9 81625 14 104000000 107349540 Gain Neurocytoma
10 81627 14 104000000 107349540 Gain IGH Central neurocytomas
11 89768 15 19000000 33600000 Gain Neurocytoma
12 96486 15 89100000 102531392 Loss Central neurocytomas
13 105389 16 79200000 81700000 Loss Neurocytoma
14 105390 16 79200000 81700000 Loss FRA16D Central neurocytomas
15 109411 17 25800000 38100000 Loss Neurocytoma
16 112094 17 37856253 37884915 Loss ERBB2 Central neurocytomas
17 112175 17 38100000 50200000 Loss Neurocytoma
18 112177 17 38100000 50200000 Loss BRCA1 Central neurocytomas
19 135912 2 122400000 129900000 Loss Neurocytoma
20 136164 2 127805598 127864903 Loss BIN1 Central neurocytomas
21 140117 2 19200000 24000000 Gain Neurocytoma
22 140118 2 19200000 24000000 Gain MYCN Central neurocytomas
23 150428 20 1 27500000 Loss Central neurocytomas
24 150458 20 1 5100000 Loss Neurocytoma
25 150539 20 10618331 10654694 Loss JAG1 Central neurocytomas
26 150626 20 12100000 21300000 Loss Neurocytoma
27 203394 5 9800000 15000000 Loss Central neurocytomas
28 203396 5 9800000 15000000 Loss Neurocytoma
29 203500 5 98200000 115200000 Loss Neurocytoma
30 203777 6 100600000 130300000 Loss Neurocytoma
31 204195 6 107656428 107856693 Loss Central neurocytomas
32 106841 17 1 3300000 Loss PAFAH Central neurocytomas
33 106839 17 1 3300000 Loss HIC1 Central neurocytomas
34 106838 17 1 3300000 Loss Neurocytoma
35 106531 16 88700000 90354753 Gain Neurocytoma
36 90906 15 25700000 28100000 Loss SNRPN Central neurocytomas
37 90905 15 25700000 28100000 Loss Neurocytoma
38 90822 15 23985488 24185809 Gain Central neurocytomas
39 77107 13 40100000 55300000 Loss RB1 Central neurocytomas
40 77106 13 40100000 55300000 Loss Neurocytoma
41 73747 12 96200000 109000000 Loss Neurocytoma
42 61836 12 101786902 101801572 Loss ARL1 Central neurocytomas
43 61830 12 101673905 101685205 Loss DRIM Central neurocytomas
44 54398 11 43500000 48800000 Gain KAI1 Central neurocytomas
45 54397 11 43500000 48800000 Gain Neurocytoma
46 47162 10 89623194 89728532 Gain PTEN Central neurocytomas
47 47057 10 89500000 92900000 Gain Neurocytoma
48 42691 10 38000000 42300000 Loss Neurocytoma
49 42690 10 38000000 42300000 Loss Central neurocytomas
50 32216 1 34600000 40100000 Loss MYCL1 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 11.97 SYP SNCA NDRG1 MAP2 GFAP ENO2
2 11.34 OLIG2 GFAP FGF2
3 11 SYP OLIG2 NES MAP2 GFAP FGF2

GO Terms for Central Neurocytoma

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

# Name GO ID Score Top Affiliating Genes
1 regulation of long-term neuronal synaptic plasticity GO:0048169 9.32 SYP SNCA
2 regulation of neuronal synaptic plasticity GO:0048168 9.26 SYP SNCA
3 negative regulation of microtubule polymerization GO:0031115 9.16 SNCA MAP2
4 stem cell proliferation GO:0072089 8.96 NES FGF2
5 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 syntaxin-1 binding GO:0017075 8.62 SYP DAPK1

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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