MCID: DYS006
MIFTS: 37

Dysembryoplastic Neuroepithelial Tumor

Categories: Rare diseases, Cancer diseases, Neuronal diseases

Aliases & Classifications for Dysembryoplastic Neuroepithelial Tumor

MalaCards integrated aliases for Dysembryoplastic Neuroepithelial Tumor:

Name: Dysembryoplastic Neuroepithelial Tumor 12 49 55 14 69
Dysembryoplastic Neuroepithelial Neoplasm 12
Dnet 55

Characteristics:

Orphanet epidemiological data:

55
dysembryoplastic neuroepithelial tumor
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 55  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:2679
NCIt 46 C9505
Orphanet 55 ORPHA251946
UMLS via Orphanet 70 C1266177
UMLS 69 C1266177

Summaries for Dysembryoplastic Neuroepithelial Tumor

MalaCards based summary : Dysembryoplastic Neuroepithelial Tumor, also known as dysembryoplastic neuroepithelial neoplasm, is related to oligodendroglioma and atypical teratoid rhabdoid tumor, 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 126)
# Related Disease Score Top Affiliating Genes
1 oligodendroglioma 30.6 GFAP IDH1 SYP
2 atypical teratoid rhabdoid tumor 30.3 GFAP SYP
3 hemimegalencephaly 30.3 GFAP MAP2
4 pilocytic astrocytoma 30.2 GFAP IDH1 SYP
5 pleomorphic xanthoastrocytoma 30.1 GFAP IDH1 SYP
6 neuronal migration disorders 30.1 MAP2 NES
7 chiari malformation 30.0 GFAP MAP2 SYP
8 glioblastoma multiforme 29.5 GFAP IDH1 NES
9 angiocentric glioma 29.5 GFAP IDH1 S100B SYP
10 ganglioglioma 29.4 GFAP MAP2 NES S100B SYP
11 subependymal giant cell astrocytoma 29.3 GFAP NES S100B SYP
12 astrocytoma 29.3 GFAP IDH1 NES S100B SYP
13 rosette-forming glioneuronal tumor of fourth ventricule 11.3
14 epilepsy 10.6
15 primary hepatic neuroendocrine carcinoma 10.4 S100B SYP
16 mixed type thymoma 10.4 S100B SYP
17 hypoganglionosis 10.3 S100B SYP
18 supratentorial primitive neuroectodermal tumor 10.3 S100B SYP
19 papillary tumor of the pineal region 10.3 MAP2 SYP
20 desmoplastic infantile ganglioglioma 10.3 GFAP SYP
21 olfactory neuroblastoma 10.3 S100B SYP
22 lung combined type small cell carcinoma 10.3 GFAP SYP
23 cellular ependymoma 10.3 GFAP SYP
24 cerebral primitive neuroectodermal tumor 10.3 S100B SYP
25 gangliocytoma 10.3 GFAP SYP
26 rhabdoid meningioma 10.3 GFAP SYP
27 ectopic cushing syndrome 10.3 S100B SYP
28 pilomyxoid astrocytoma 10.3 GFAP SYP
29 subependymal glioma 10.3 GFAP SYP
30 cerebral neuroblastoma 10.3 GFAP SYP
31 intracranial cysts 10.3 GFAP S100B
32 benign ependymoma 10.3 GFAP SYP
33 gemistocytic astrocytoma 10.3 GFAP S100B
34 cerebral ventricle cancer 10.2 GFAP SYP
35 cerebrum cancer 10.2 GFAP SYP
36 pineal gland cancer 10.2 GFAP SYP
37 melanotic neuroectodermal tumor 10.2 GFAP SYP
38 syringocystadenoma papilliferum 10.2 GFAP S100B
39 obstructive hydrocephalus 10.2 GFAP SYP
40 supratentorial cancer 10.2 GFAP SYP
41 chordoid glioma 10.2 GFAP S100B
42 pineoblastoma 10.2 GFAP SYP
43 myxopapillary ependymoma 10.2 GFAP S100B
44 binswanger's disease 10.2 GFAP SYP
45 syringoma 10.2 GFAP S100B
46 cystic teratoma 10.2 GFAP SYP
47 desmoplastic small round cell tumor 10.2 S100B SYP
48 perineurioma 10.2 GFAP S100B
49 neurofibroma 10.2 S100B SYP
50 granular cell tumor 10.2 GFAP S100B

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:

25 (show all 17)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-11 9.62 NES
2 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.62 GRIN1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-123 9.62 NES GRIN1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.62 GRIN1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.62 IDH1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-136 9.62 IDH1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.62 IDH1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.62 NES GRIN1 IDH1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-16 9.62 NES
10 Increased shRNA abundance (Z-score > 2) GR00366-A-172 9.62 IDH1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-186 9.62 NES
12 Increased shRNA abundance (Z-score > 2) GR00366-A-196 9.62 NES
13 Increased shRNA abundance (Z-score > 2) GR00366-A-205 9.62 NES
14 Increased shRNA abundance (Z-score > 2) GR00366-A-22 9.62 GRIN1
15 Increased shRNA abundance (Z-score > 2) GR00366-A-39 9.62 IDH1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-45 9.62 GRIN1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.62 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

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:

38
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 134)
# Title Authors Year
1
Cerebellar dysembryoplastic neuroepithelial tumor: report of a case and review of the literature. ( 28975449 )
2017
2
Long-term recurrence of dysembryoplastic neuroepithelial tumor: Clinical case report. ( 28781917 )
2017
3
Utility of Stereoelectroencephalography in Children with Dysembryoplastic Neuroepithelial Tumor and Cortical Malformation. ( 28898173 )
2017
4
Occipital dysembryoplastic neuroepithelial tumor presenting as adult-onset temporal epilepsy. ( 29062689 )
2017
5
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. ( 28131206 )
2017
6
MicroRNA expression profiles in pediatric dysembryoplastic neuroepithelial tumors. ( 26698155 )
2016
7
Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis. ( 26921879 )
2016
8
Volumetric growth analysis of an insular dysembryoplastic neuroepithelial tumor over a 10-year follow-up. ( 28194304 )
2016
9
A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures. ( 28018467 )
2016
10
Remitted epilepsy with dysembryoplastic neuroepithelial tumor involving the thalamus. ( 27163491 )
2016
11
Atypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature. ( 26978682 )
2016
12
Dysembryoplastic neuroepithelial tumor: A rare brain tumor not to be misdiagnosed. ( 27057233 )
2016
13
Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient. ( 27127715 )
2016
14
Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor. ( 27181792 )
2016
15
Alterations in BRAF gene, and enhanced mTOR and MAPK signaling in dysembryoplastic neuroepithelial tumors (DNTs). ( 27599148 )
2016
16
Dysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases. ( 26796505 )
2016
17
Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors. ( 26920151 )
2016
18
BRAF V600E mutations are frequent in dysembryoplastic neuroepithelial tumors and subependymal giant cell astrocytomas. ( 25346165 )
2015
19
Resection of a dysembryoplastic neuroepithelial tumor in the precentral gyrus. ( 26253415 )
2015
20
Dysembryoplastic neuroepithelial tumor transforming into malignancy: a case report. ( 25033864 )
2014
21
Well-differentiated pediatric glial neoplasms with features of oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumors: a morphological diagnostic challenge. ( 24448703 )
2014
22
Manifestation and treatment of intraventricular dysembryoplastic neuroepithelial tumor. ( 24709201 )
2014
23
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. ( 24735014 )
2014
24
Recurrence and histological evolution of dysembryoplastic neuroepithelial tumor: A case report and review of the literature. ( 24137435 )
2013
25
Dysembryoplastic neuroepithelial tumor with atypical presentation: MRI and diffusion tensor characteristics. ( 24421925 )
2013
26
Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. ( 23163721 )
2013
27
Dysembryoplastic neuroepithelial tumor, a pure glial tumor? Immunohistochemical and morphometric studies. ( 23530928 )
2013
28
Dysembryoplastic neuroepithelial tumor with rapid recurrence of pilocytic astrocytoma component. ( 23881357 )
2013
29
Glioblastoma Multiforme Arising From Dysembryoplastic Neuroepithelial Tumor in a Child in the Absence of Therapy. ( 24309599 )
2013
30
Electrocorticographic-histopathologic correlations implying epileptogenicity of dysembryoplastic neuroepithelial tumor. ( 24077279 )
2013
31
Dysembryoplastic Neuroepithelial Tumor: A Clinical, Neuroradiological, and Pathological Study of 15 Cases. ( 23752069 )
2013
32
Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation. ( 23432479 )
2013
33
Oligodendroglioma (WHO grade I) in a young epilepsy patient: a specific entity lying within the spectrum of dysembryoplastic neuroepithelial tumor? ( 23432071 )
2013
34
Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E) mutation and expression. ( 23442159 )
2013
35
Diffuse ependymal dysembryoplastic neuroepithelial tumor causing spinal drop metastases: a case report. ( 22778572 )
2012
36
Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: review of 2 cases with chromosome 1p/19q and IDH1 analysis. ( 22192702 )
2012
37
Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma. ( 22990729 )
2012
38
Composite ganglioglioma/dysembryoplastic neuroepithelial tumor: a clinicopathologic study of 8 cases. ( 22221701 )
2012
39
Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus. ( 22809555 )
2012
40
A rare case of dysembryoplastic neuroepithelial tumor. ( 23230542 )
2012
41
Complex form variant of dysembryoplastic neuroepithelial tumor of the cerebellum. ( 22988535 )
2012
42
Intraventricular dysembryoplastic neuroepithelial tumor in a pediatric patient: is it the most common extracortical location for DNT? ( 20959995 )
2011
43
A patient with a dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery after initial seizure. ( 22777137 )
2011
44
Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy. ( 21456916 )
2011
45
Lhermitte-Duclos disease associated with dysembryoplastic neuroepithelial tumor differentiation with characteristic magnetic resonance appearance of "tiger striping". ( 21704939 )
2011
46
Dysembryoplastic neuroepithelial tumor and calcifying pseudoneoplasms of the neuraxis: a collision of two seizure-associated lesions. ( 21726506 )
2011
47
Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report. ( 21899755 )
2011
48
Sudden death due to dysembryoplastic neuroepithelial tumor. ( 21056878 )
2010
49
Progressive biological behavior of a dysembryoplastic neuroepithelial tumor. ( 21196795 )
2010
50
Seizure control outcomes after resection of dysembryoplastic neuroepithelial tumor in 50 patients. ( 20043747 )
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 cell body GO:0044297 9.32 GFAP MAP2
2 terminal bouton GO:0043195 9.26 GRIN1 SYP
3 intermediate filament cytoskeleton GO:0045111 9.16 GFAP NES
4 neuron projection GO:0043005 9.13 GRIN1 MAP2 SYP
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 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
16 ExPASy
18 FMA
27 GO
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31 HPO
32 ICD10
33 ICD10 via Orphanet
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35 IUPHAR
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41 MeSH
42 MESH via Orphanet
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50 NINDS
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54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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