MCID: DYS006
MIFTS: 37

Dysembryoplastic Neuroepithelial Tumor

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Dysembryoplastic Neuroepithelial Tumor

MalaCards integrated aliases for Dysembryoplastic Neuroepithelial Tumor:

Name: Dysembryoplastic Neuroepithelial Tumor 12 53 59 15 73
Dysembryoplastic Neuroepithelial Neoplasm 12
Dnet 59

Characteristics:

Orphanet epidemiological data:

59
dysembryoplastic neuroepithelial tumor
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:2679
NCIt 50 C9505
Orphanet 59 ORPHA251946
UMLS via Orphanet 74 C1266177
UMLS 73 C1266177

Summaries for Dysembryoplastic Neuroepithelial Tumor

MalaCards based summary : Dysembryoplastic Neuroepithelial Tumor, also known as dysembryoplastic neuroepithelial neoplasm, is related to rosette-forming glioneuronal tumor and oligodendroglioma, and has symptoms including seizures An important gene associated with Dysembryoplastic Neuroepithelial Tumor is SYP (Synaptophysin), and among its related pathways/superpathways are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. Affiliated tissues include temporal lobe, brain and cerebellum, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Dysembryoplastic Neuroepithelial Tumor

Diseases related to Dysembryoplastic Neuroepithelial Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 127)
# Related Disease Score Top Affiliating Genes
1 rosette-forming glioneuronal tumor 30.6 MAP2 S100B SYP
2 oligodendroglioma 30.5 GFAP IDH1 SYP
3 hemimegalencephaly 30.4 GFAP MAP2
4 atypical teratoid rhabdoid tumor 30.0 GFAP S100B SYP
5 chiari malformation 29.9 GFAP MAP2 SYP
6 pilocytic astrocytoma 29.5 GFAP IDH1 S100B SYP
7 pleomorphic xanthoastrocytoma 29.2 GFAP IDH1 MAP2 SYP
8 glioblastoma multiforme 29.2 GFAP IDH1 NES
9 angiocentric glioma 29.1 GFAP IDH1 S100B SYP
10 subependymal giant cell astrocytoma 28.9 GFAP NES S100B SYP
11 ganglioglioma 28.7 GFAP MAP2 NES S100B SYP
12 epilepsy 10.6
13 primary hepatic neuroendocrine carcinoma 10.6 S100B SYP
14 mixed type thymoma 10.6 S100B SYP
15 hypoganglionosis 10.6 S100B SYP
16 papillary tumor of the pineal region 10.5 MAP2 SYP
17 supratentorial primitive neuroectodermal tumor 10.5 S100B SYP
18 cerebellum cancer 10.5 MAP2 SYP
19 desmoplastic infantile ganglioglioma 10.5 GFAP SYP
20 lung combined type small cell carcinoma 10.4 GFAP SYP
21 cellular ependymoma 10.4 GFAP SYP
22 gangliocytoma 10.4 GFAP SYP
23 rhabdoid meningioma 10.4 GFAP SYP
24 olfactory neuroblastoma 10.4 S100B SYP
25 pilomyxoid astrocytoma 10.4 GFAP SYP
26 astrocytoma 10.4
27 cerebral primitive neuroectodermal tumor 10.4 S100B SYP
28 subependymal glioma 10.4 GFAP SYP
29 ectopic cushing syndrome 10.4 S100B SYP
30 intracranial cysts 10.4 GFAP S100B
31 cerebral neuroblastoma 10.4 GFAP SYP
32 gemistocytic astrocytoma 10.4 GFAP S100B
33 benign ependymoma 10.4 GFAP SYP
34 cerebral ventricle cancer 10.4 GFAP SYP
35 cerebrum cancer 10.4 GFAP SYP
36 pineal gland cancer 10.4 GFAP SYP
37 syringocystadenoma papilliferum 10.4 GFAP S100B
38 spindle cell rhabdomyosarcoma 10.4 GFAP S100B
39 obstructive hydrocephalus 10.4 GFAP SYP
40 supratentorial cancer 10.4 GFAP SYP
41 myxopapillary ependymoma 10.4 GFAP S100B
42 chordoid glioma 10.3 GFAP S100B
43 pineoblastoma 10.3 GFAP SYP
44 syringoma 10.3 GFAP S100B
45 binswanger's disease 10.3 GFAP SYP
46 neurofibroma 10.3 S100B SYP
47 cystic teratoma 10.3 GFAP SYP
48 perineurioma 10.3 GFAP S100B
49 granular cell tumor 10.3 GFAP S100B
50 medullomyoblastoma 10.3 GFAP SYP

Graphical network of the top 20 diseases related to Dysembryoplastic Neuroepithelial Tumor:



Diseases related to Dysembryoplastic Neuroepithelial Tumor

Symptoms & Phenotypes for Dysembryoplastic Neuroepithelial Tumor

UMLS symptoms related to Dysembryoplastic Neuroepithelial Tumor:


seizures

GenomeRNAi Phenotypes related to Dysembryoplastic Neuroepithelial Tumor according to GeneCards Suite gene sharing:

26 (show all 16)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-11 9.6 NES
2 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.6 GRIN1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-123 9.6 NES GRIN1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.6 GRIN1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.6 IDH1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-136 9.6 IDH1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.6 IDH1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.6 NES GRIN1 IDH1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-16 9.6 NES
10 Increased shRNA abundance (Z-score > 2) GR00366-A-172 9.6 IDH1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-186 9.6 NES
12 Increased shRNA abundance (Z-score > 2) GR00366-A-196 9.6 NES
13 Increased shRNA abundance (Z-score > 2) GR00366-A-205 9.6 NES
14 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.6 IDH1
15 Increased shRNA abundance (Z-score > 2) GR00366-A-45 9.6 GRIN1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.6 IDH1

Drugs & Therapeutics for Dysembryoplastic Neuroepithelial Tumor

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Molecular-Guided Therapy for Childhood Cancer Recruiting NCT02162732 Not Applicable

Search NIH Clinical Center for Dysembryoplastic Neuroepithelial Tumor

Genetic Tests for Dysembryoplastic Neuroepithelial Tumor

Anatomical Context for Dysembryoplastic Neuroepithelial Tumor

MalaCards organs/tissues related to Dysembryoplastic Neuroepithelial Tumor:

41
Temporal Lobe, Brain, Cerebellum, Caudate Nucleus, Thalamus, Parietal Lobe, Cortex

Publications for Dysembryoplastic Neuroepithelial Tumor

Articles related to Dysembryoplastic Neuroepithelial Tumor:

(show top 50) (show all 136)
# Title Authors Year
1
Dysembryoplastic neuroepithelial tumor-like pilocytic astrocytoma: A case report. ( 29768357 )
2018
2
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. ( 28131206 )
2017
3
Cerebellar dysembryoplastic neuroepithelial tumor: report of a case and review of the literature. ( 28975449 )
2017
4
Long-term recurrence of dysembryoplastic neuroepithelial tumor: Clinical case report. ( 28781917 )
2017
5
Droplet digital PCR is a powerful technique to demonstrate frequent FGFR1 duplication in dysembryoplastic neuroepithelial tumors. ( 27791984 )
2017
6
Utility of Stereoelectroencephalography in Children with Dysembryoplastic Neuroepithelial Tumor and Cortical Malformation. ( 28898173 )
2017
7
Occipital dysembryoplastic neuroepithelial tumor presenting as adult-onset temporal epilepsy. ( 29062689 )
2017
8
Dysembryoplastic neuroepithelial tumor: A rare brain tumor not to be misdiagnosed. ( 27057233 )
2016
9
Alterations in BRAF gene, and enhanced mTOR and MAPK signaling in dysembryoplastic neuroepithelial tumors (DNTs). ( 27599148 )
2016
10
Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor. ( 27181792 )
2016
11
Atypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature. ( 26978682 )
2016
12
Volumetric growth analysis of an insular dysembryoplastic neuroepithelial tumor over a 10-year follow-up. ( 28194304 )
2016
13
Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors. ( 26920151 )
2016
14
Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis. ( 26921879 )
2016
15
MicroRNA expression profiles in pediatric dysembryoplastic neuroepithelial tumors. ( 26698155 )
2016
16
A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures. ( 28018467 )
2016
17
Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient. ( 27127715 )
2016
18
Dysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases. ( 26796505 )
2016
19
Remitted epilepsy with dysembryoplastic neuroepithelial tumor involving the thalamus. ( 27163491 )
2016
20
Resection of a dysembryoplastic neuroepithelial tumor in the precentral gyrus. ( 26253415 )
2015
21
BRAF V600E mutations are frequent in dysembryoplastic neuroepithelial tumors and subependymal giant cell astrocytomas. ( 25346165 )
2015
22
Well-differentiated pediatric glial neoplasms with features of oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumors: a morphological diagnostic challenge. ( 24448703 )
2014
23
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. ( 24735014 )
2014
24
Manifestation and treatment of intraventricular dysembryoplastic neuroepithelial tumor. ( 24709201 )
2014
25
Dysembryoplastic neuroepithelial tumor transforming into malignancy: a case report. ( 25033864 )
2014
26
Oligodendroglioma (WHO grade I) in a young epilepsy patient: a specific entity lying within the spectrum of dysembryoplastic neuroepithelial tumor? ( 23432071 )
2013
27
Dysembryoplastic neuroepithelial tumor with rapid recurrence of pilocytic astrocytoma component. ( 23881357 )
2013
28
Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation. ( 23432479 )
2013
29
Dysembryoplastic Neuroepithelial Tumor: A Clinical, Neuroradiological, and Pathological Study of 15 Cases. ( 23752069 )
2013
30
Dysembryoplastic neuroepithelial tumor with atypical presentation: MRI and diffusion tensor characteristics. ( 24421925 )
2013
31
Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E) mutation and expression. ( 23442159 )
2013
32
Glioblastoma Multiforme Arising From Dysembryoplastic Neuroepithelial Tumor in a Child in the Absence of Therapy. ( 24309599 )
2013
33
Electrocorticographic-histopathologic correlations implying epileptogenicity of dysembryoplastic neuroepithelial tumor. ( 24077279 )
2013
34
Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. ( 23163721 )
2013
35
Dysembryoplastic neuroepithelial tumor, a pure glial tumor? Immunohistochemical and morphometric studies. ( 23530928 )
2013
36
Recurrence and histological evolution of dysembryoplastic neuroepithelial tumor: A case report and review of the literature. ( 24137435 )
2013
37
Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma. ( 22990729 )
2012
38
A rare case of dysembryoplastic neuroepithelial tumor. ( 23230542 )
2012
39
Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: review of 2 cases with chromosome 1p/19q and IDH1 analysis. ( 22192702 )
2012
40
Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus. ( 22809555 )
2012
41
Complex form variant of dysembryoplastic neuroepithelial tumor of the cerebellum. ( 22988535 )
2012
42
Diffuse ependymal dysembryoplastic neuroepithelial tumor causing spinal drop metastases: a case report. ( 22778572 )
2012
43
Composite ganglioglioma/dysembryoplastic neuroepithelial tumor: a clinicopathologic study of 8 cases. ( 22221701 )
2012
44
Dysembryoplastic neuroepithelial tumor and calcifying pseudoneoplasms of the neuraxis: a collision of two seizure-associated lesions. ( 21726506 )
2011
45
Lhermitte-Duclos disease associated with dysembryoplastic neuroepithelial tumor differentiation with characteristic magnetic resonance appearance of "tiger striping". ( 21704939 )
2011
46
A patient with a dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery after initial seizure. ( 22777137 )
2011
47
Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report. ( 21899755 )
2011
48
Intraventricular dysembryoplastic neuroepithelial tumor in a pediatric patient: is it the most common extracortical location for DNT? ( 20959995 )
2011
49
Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy. ( 21456916 )
2011
50
A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter. ( 21121739 )
2010

Variations for Dysembryoplastic Neuroepithelial Tumor

Expression for Dysembryoplastic Neuroepithelial Tumor

Search GEO for disease gene expression data for Dysembryoplastic Neuroepithelial Tumor.

Pathways for Dysembryoplastic Neuroepithelial Tumor

Pathways related to Dysembryoplastic Neuroepithelial Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.89 GFAP GRIN1 MAP2 S100B SYP
2 10.92 GFAP MAP2 NES S100B SYP

GO Terms for Dysembryoplastic Neuroepithelial Tumor

Cellular components related to Dysembryoplastic Neuroepithelial Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neuron projection GO:0043005 9.33 GRIN1 MAP2 SYP
2 cell body GO:0044297 9.32 GFAP MAP2
3 terminal bouton GO:0043195 9.26 GRIN1 SYP
4 intermediate filament cytoskeleton GO:0045111 8.96 GFAP NES
5 excitatory synapse GO:0060076 8.62 GRIN1 SYP

Biological processes related to Dysembryoplastic Neuroepithelial Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 memory GO:0007613 9.4 GRIN1 S100B
2 learning or memory GO:0007611 9.37 GRIN1 S100B
3 long-term synaptic potentiation GO:0060291 9.32 GFAP S100B
4 cellular response to organic substance GO:0071310 9.26 MAP2 SYP
5 regulation of axonogenesis GO:0050770 9.16 GRIN1 MAP2
6 regulation of long-term neuronal synaptic plasticity GO:0048169 8.96 GRIN1 SYP
7 regulation of neuronal synaptic plasticity GO:0048168 8.8 GRIN1 S100B SYP

Molecular functions related to Dysembryoplastic Neuroepithelial Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 9.33 GRIN1 IDH1 S100B
2 identical protein binding GO:0042802 9.26 GFAP IDH1 S100B SYP
3 structural molecule activity GO:0005198 8.8 GFAP MAP2 NES

Sources for Dysembryoplastic Neuroepithelial Tumor

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
Content
Loading form....