MCID: CNT019
MIFTS: 44

Central Neurocytoma

Categories: Cancer diseases, Eye diseases, Mental diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Central Neurocytoma

MalaCards integrated aliases for Central Neurocytoma:

Name: Central Neurocytoma 11 19 58 14 16 71 75
Neurocytoma 43 71
Neurocytoma Central 53
Neurolipocytoma 11

Characteristics:


Age Of Onset:

Adolescent,Adult,Childhood 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 11 DOID:14174
MeSH 43 D018306
NCIt 49 C3791
SNOMED-CT 68 302832007
UMLS via Orphanet 72 C0206719
Orphanet 58 ORPHA73256
UMLS 71 C0206719 C1622510

Summaries for Central Neurocytoma

GARD: 19 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.

MalaCards based summary: Central Neurocytoma, also known as neurocytoma, is related to cerebellar liponeurocytoma and cellular ependymoma, 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, spinal cord and medulla oblongata, and related phenotypes are hydrocephalus and intracranial cystic lesion

Orphanet: 58 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.

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 371)
# Related Disease Score Top Affiliating Genes
1 cerebellar liponeurocytoma 31.7 SYP RBFOX3 MAP2 ENO2
2 cellular ependymoma 31.1 SYP NES GFAP
3 hydrocephalus 30.7 SYP SNCA NES GFAP ENO2
4 mature teratoma 30.4 SYP ENO2
5 oligodendroglioma 30.4 SYP OLIG2 IDH1 GFAP ENO2 CHGA
6 obstructive hydrocephalus 30.4 SYP OLIG2 KIAA1549 GFAP ENO2
7 cauda equina syndrome 30.3 SYP GFAP
8 cerebral neuroblastoma 30.3 SYP GFAP ENO2 CHGA
9 papilloma of choroid plexus 30.1 SYP GFAP ENO2
10 olfactory neuroblastoma 30.1 SYP ENO2 CHGA
11 cystic teratoma 30.1 SYP ENO2 CHGA
12 brain cancer 30.0 SYP OLIG2 NES IDH1 GFAP FGF2
13 benign teratoma 30.0 SYP ENO2 CHGA
14 islet cell tumor 30.0 SYP ENO2 CHGA
15 ewing sarcoma 30.0 SYP NES ENO2 CHGA
16 neuroma 29.9 GFAP FGF2 ENO2
17 hemangioblastoma 29.9 SYP GFAP ENO2
18 clear cell ependymoma 29.9 SYP RBFOX3 OLIG2 GFAP
19 subependymal giant cell astrocytoma 29.8 SYP KIAA1549 GFAP ENO2
20 ganglioneuroma 29.7 SYP GFAP ENO2 CHGA
21 anaplastic astrocytoma 29.6 OLIG2 NES KIAA1549 IDH1 GFAP
22 toxic encephalopathy 29.6 SYP SNCA RBFOX3 MAP2 GFAP
23 neuroblastoma 29.6 SYP SNCA NES MAP2 GFAP FGF2
24 high grade ependymoma 29.5 OLIG2 KIAA1549 IDH1 GFAP
25 paraganglioma 29.5 SYP GFAP FGF2 ENO2 CHGA
26 ganglioglioma 29.5 SYP NES MAP2 GFAP ENO2 CHGA
27 teratoma 29.5 SYP NES MAP2 GFAP ENO2 CHGA
28 neurofibromatosis, type i 29.3 KIAA1549 IDH1 ENO2
29 diffuse astrocytoma 29.3 OLIG2 KIAA1549 IDH1 GFAP
30 stroke, ischemic 29.3 RBFOX3 NES MAP2 GFAP ENO2
31 anaplastic oligodendroglioma 29.3 OLIG2 NES KIAA1549 IDH1 GFAP
32 meningioma, familial 29.3 SYP OLIG2 KIAA1549 IDH1 GFAP ENO2
33 dysembryoplastic neuroepithelial tumor 29.1 SYP RBFOX3 OLIG2 KIAA1549 IDH1 GFAP
34 medulloblastoma 29.0 SYP OLIG2 NES MAP2 IDH1 GFAP
35 spinal cord disease 28.9 SYP OLIG2 NES KIAA1549 IDH1 GFAP
36 pilocytic astrocytoma 28.9 SYP OLIG2 NES KIAA1549 IDH1 GFAP
37 central nervous system benign neoplasm 28.8 SYP OLIG2 NES KIAA1549 IDH1 GFAP
38 gangliocytoma 28.7 SYP RBFOX3 KIAA1549 IDH1 GFAP ENO2
39 extraventricular neurocytoma 11.2
40 papillary glioneuronal tumors 11.0
41 glioma susceptibility 1 10.7
42 neuronal tumor 10.5
43 intracranial hypertension 10.4
44 rare tumor 10.4
45 central nervous system cancer 10.4
46 papillary ependymoma 10.4 SYP GFAP
47 third ventricle chordoid glioma 10.3 OLIG2 GFAP
48 central nervous system immature teratoma 10.3 NES GFAP
49 bladder small cell carcinoma 10.3 SYP ENO2
50 pancreas lymphoma 10.3 SYP CHGA

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 30 (show all 20)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hydrocephalus 58 30 Obligate (100%) Obligate (100%)
HP:0000238
2 intracranial cystic lesion 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0010576
3 abnormal cellular phenotype 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0025354
4 abnormal lateral ventricle morphology 30 Hallmark (90%) HP:0030047
5 nausea and vomiting 58 30 Frequent (33%) Frequent (79-30%)
HP:0002017
6 depression 58 30 Frequent (33%) Frequent (79-30%)
HP:0000716
7 ataxia 58 30 Frequent (33%) Frequent (79-30%)
HP:0001251
8 cerebral calcification 58 30 Frequent (33%) Frequent (79-30%)
HP:0002514
9 increased intracranial pressure 58 30 Frequent (33%) Frequent (79-30%)
HP:0002516
10 abnormality of vision 58 30 Frequent (33%) Frequent (79-30%)
HP:0000504
11 headache 58 30 Frequent (33%) Frequent (79-30%)
HP:0002315
12 decreased corneal reflex 58 30 Frequent (33%) Frequent (79-30%)
HP:0008000
13 paresthesia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003401
14 lethargy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001254
15 babinski sign 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0003487
16 coma 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001259
17 tinnitus 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000360
18 pain insensitivity 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0007021
19 postural instability 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002172
20 abnormality of lateral ventricle 58 Very frequent (99-80%)

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 nervous system MP:0003631 9.7 CHGA ENO2 FGF2 GFAP IDH1 MAP2
2 behavior/neurological MP:0005386 9.32 ENO2 FGF2 GFAP KIAA1549 MAP2 NES

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
Trametinib Approved Phase 4 871700-17-3 11707110
2
Dabrafenib Approved, Investigational Phase 4 1195765-45-7 44462760
3 Protein Kinase Inhibitors Phase 4
4
Carboplatin Approved Phase 2 41575-94-4 10339178 38904
5
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
6 Antimitotic Agents Phase 2
7 Tubulin Modulators 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) Recruiting NCT03975829 Phase 4 dabrafenib;trametinib
2 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) Active, not 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

Organs/tissues related to Central Neurocytoma:

MalaCards : Brain, Spinal Cord, Medulla Oblongata, Occipital Lobe, Temporal Lobe, Cerebellum, Ovary

Publications for Central Neurocytoma

Articles related to Central Neurocytoma:

(show top 50) (show all 484)
# Title Authors PMID Year
1
Synaptophysin negative central neurocytoma. 53 62
20429311 2010
2
Central neurocytoma: a clinical, radiological and pathological study of nine cases. 53 62
18022760 2008
3
OLIG2 is a useful immunohistochemical marker in differential diagnosis of clear cell primary CNS neoplasms. 53 62
17257132 2007
4
Pigmented central neurocytoma. 53 62
14991386 2004
5
Forskolin promotes astroglial differentiation of human central neurocytoma cells. 53 62
15031671 2004
6
So-called malignant and extra-ventricular neurocytomas: reality or wrong diagnosis? A critical review about two overdiagnosed cases. 53 62
11083081 2000
7
alpha-Synuclein is expressed in a variety of brain tumors showing neuronal differentiation. 53 62
10672322 2000
8
Central neurocytoma with malignant course. Neuronal and glial differentiation and craniospinal dissemination. 53 62
10393370 1999
9
Neuronal and glial characteristics of central neurocytoma: electron microscopical analysis of two cases. 53 62
8781655 1996
10
Immunocytochemical detection of calcineurin and microtubule-associated protein 2 in central neurocytoma. 53 62
8410138 1993
11
Cytoskeletal immunohistochemistry of central neurocytomas. 53 62
1471723 1992
12
Central neurocytoma: immunohistochemical and ultrastructural study. 53 62
2028745 1991
13
Patterns of differentiation in central neurocytoma. An immunohistochemical study of eleven biopsies. 53 62
2109481 1990
14
Clinical application of 3D Slicer combined with Sina/MosoCam multimodal system in preoperative planning of brain lesions surgery. 62
36357434 2022
15
Comparison of surgery with or without adjuvant radiotherapy in treating central neurocytoma: a single-center retrospective real-world study. 62
36307664 2022
16
The role of single fraction Gamma Knife radiosurgery for intraventricular central neurocytomas and the utility of F-18 fluroethyltyrosine: two case reports. 62
36437467 2022
17
Diffuse GFAP Immunopositivity in the Oligodendrocyte-like Component of Pilocytic Astrocytoma Distinguishes It from Mimickers. 62
35885538 2022
18
Uncommon Glioneuronal Tumors: A Radiologic and Pathologic Synopsis. 62
35512827 2022
19
A 22-Year Course of a Case with Central Neurocytoma. 62
35864706 2022
20
TTF-1: A Well-Favored Addition to the Immunohistochemistry Armamentarium as a Diagnostic Marker of SEGA. 62
34871801 2022
21
Malignant transformation of central neurocytoma with dissemination 17 years after initial treatment: illustrative case. 62
36130558 2022
22
Clinical Outcomes and Prognostic Analysis of 101 Patients of Central Neurocytoma: A 10-Year Treatment Experience at a Single Institution. 62
35692765 2022
23
Central Neurocytoma with Hemorrhagic Presentation Case Report and Review of the Literature. 62
35310930 2022
24
The Diagnostic Value of Pan-Trk Expression to Detect Neurotrophic Tyrosine Receptor Kinase (NTRK) Gene Fusion in CNS Tumours: A Study Using Next-Generation Sequencing Platform. 62
35295612 2022
25
Extraventricular Neurocytoma: Clinical Investigation of Heterogenous Prognosis. 62
35118844 2022
26
A Clinicopathological and Molecular Analysis of Sellar/Suprasellar Neurocytoma Mimicking Pituitary Adenoma. 62
35663322 2022
27
Gamma Knife radiosurgery for the treatment of central neurocytoma: a single-institution experience of 25 patients. 62
33733378 2021
28
The importance of staged surgery for giant atypical central neurocytoma. 62
32857303 2021
29
MRI features of an atypical case of extraventricular neurocytoma: A case report. 62
34941079 2021
30
Lateral ventricular liponeurocytoma: Review of literature and case illustration. 62
33766564 2021
31
Differential Diagnosis of Central Neurocytoma: Two Cases. 62
33352608 2021
32
Organic bipolar disorder secondary to central neurocytoma resection: a case report. 62
34533207 2021
33
Impact of Adjuvant Radiotherapy in Patients with Central Neurocytoma: A Multicentric International Analysis. 62
34503124 2021
34
A Rare Case of Isolated Intraventricular Primary Central Nervous System Lymphoma in an 85-Year-Old Man. 62
34660383 2021
35
Transcortical Approach to Deep-Seated Intraventricular and Intra-axial Tumors Using a Tubular Retractor System: A Technical Note and Review of the Literature. 62
33321519 2021
36
Tumor Spheroids of an Aggressive Form of Central Neurocytoma Have Transit-Amplifying Progenitor Characteristics with Enhanced EGFR and Tumor Stem Cell Signaling. 62
33972466 2021
37
Erratum to "Retraction notice to neuroimaging diagnosis of intraventricular Central neurocytoma" [Neurosci. Lett. 735, 14 September (2020) 135143]. 62
33551320 2021
38
Atypical Central Neurocytoma: An Investigation of Prognostic Factors. 62
33091649 2021
39
Clinico-radiological characteristics, histo-pathological features and long-term survival outcomes in central neurocytoma: A single-institutional audit. 62
33358093 2021
40
[Central neurocytomas: long-term treatment outcomes]. 62
33864664 2021
41
A rare diagnosis of an extraventricular neurocytoma. 62
33767892 2021
42
Corpus Callosum Swelling after Resection of Intraventricular Central Neurocytoma. 62
35079514 2021
43
Clinical Outcome After Microsurgical Resection of Central Neurocytoma: A Single-Centre Analysis of 15 Years. 62
35002932 2021
44
Refined Temporal-to-Frontal Horn Shunt for Treatment of Trapped Temporal Horn After Surgery of Peri- or Intraventricular Tumor: A Case Series Study. 62
34900736 2021
45
Immunohistochemical expression of Olig2, CD99, and EMA to differentiate oligodendroglial-like neoplasms. 62
34628794 2021
46
The last decade's experience of management of central neurocytomas: Treatment strategies and new options. 62
34345477 2021
47
Central neurocytoma originating in third ventricle with expansion into the cerebral aqueduct and fourth ventricle: Case report and review of literature. 62
32502563 2020
48
Antibiotic-induced black hairy tongue: two case reports and a review of the literature. 62
33044871 2020
49
Neuroimaging diagnosis of intraventricular Central neurocytoma. 62
32544597 2020
50
Cerebellar Neurocytoma with Excellent Response to Radiotherapy. 62
32593767 2020

Variations for Central Neurocytoma

Copy number variations for Central Neurocytoma from CNVD:

6 (show top 50) (show all 66)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 13818 1 1 7200000 Loss Neurocytoma
2 13819 1 1 7200000 Loss CDK11B Central neurocytomas
3 32215 1 34600000 40100000 Loss Neurocytoma
4 32216 1 34600000 40100000 Loss MYCL Central neurocytomas
5 42690 10 38000000 42300000 Loss Central neurocytomas
6 42691 10 38000000 42300000 Loss Neurocytoma
7 47057 10 89500000 92900000 Gain Neurocytoma
8 47162 10 89623194 89728532 Gain PTEN Central neurocytomas
9 48339 11 1 2800000 Loss Neurocytoma
10 48340 11 1 2800000 Loss HRAS Central neurocytomas
11 49707 11 110400000 121200000 Gain Neurocytoma
12 49709 11 110400000 121200000 Gain KMT2A Central neurocytomas
13 51786 11 130800000 135006516 Gain Central neurocytomas
14 51787 11 130800000 135006516 Gain Neurocytoma
15 54397 11 43500000 48800000 Gain Neurocytoma
16 54398 11 43500000 48800000 Gain CD82 Central neurocytomas
17 61830 12 101673905 101685205 Loss UTP20 Central neurocytomas
18 61836 12 101786902 101801572 Loss ARL1 Central neurocytomas
19 73747 12 96200000 109000000 Loss Neurocytoma
20 74691 13 110300000 115169878 Loss Central neurocytomas
21 74693 13 110300000 115169878 Loss Neurocytoma
22 77106 13 40100000 55300000 Loss Neurocytoma
23 77107 13 40100000 55300000 Loss RB1 Central neurocytomas
24 81625 14 104000000 107349540 Gain Neurocytoma
25 81627 14 104000000 107349540 Gain IGH Central neurocytomas
26 89768 15 19000000 33600000 Gain Neurocytoma
27 90822 15 23985488 24185809 Gain Central neurocytomas
28 90905 15 25700000 28100000 Loss Neurocytoma
29 90906 15 25700000 28100000 Loss SNRPN Central neurocytomas
30 96486 15 89100000 102531392 Loss Central neurocytomas
31 105389 16 79200000 81700000 Loss Neurocytoma
32 105390 16 79200000 81700000 Loss FRA16D Central neurocytomas
33 106531 16 88700000 90354753 Gain Neurocytoma
34 106838 17 1 3300000 Loss Neurocytoma
35 30472 1 243700000 249250621 Gain OR14C36 Central neurocytomas
36 30471 1 243700000 249250621 Gain Neurocytoma
37 106839 17 1 3300000 Loss HIC1 Central neurocytomas
38 106841 17 1 3300000 Loss PAFAH1B1 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 12.03 SYP SNCA SCGN RBFOX3 MAP2 GFAP
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 negative regulation of microtubule polymerization GO:0031115 9.26 MAP2 SNCA
2 negative regulation of neuron death GO:1901215 9.1 SNCA ENO2 CHGA

Sources for Central Neurocytoma

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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