DNET
MCID: DYS006
MIFTS: 36

Dysembryoplastic Neuroepithelial Tumor (DNET)

Categories: Cancer diseases, Neuronal diseases, Rare 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 pilocytic astrocytoma and rosette-forming glioneuronal 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. The drug Akynzeo has been mentioned in the context of this disorder. Affiliated tissues include temporal lobe, cerebellum and caudate nucleus, 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 129)
# Related Disease Score Top Affiliating Genes
1 pilocytic astrocytoma 30.8 GFAP IDH1
2 rosette-forming glioneuronal tumor 30.7 MAP2 S100B SYP
3 oligodendroglioma 30.6 GFAP IDH1 S100B SYP
4 glioma susceptibility 1 30.3 GFAP IDH1
5 atypical teratoid rhabdoid tumor 30.3 GFAP SYP
6 neuronal migration disorders 30.3 MAP2 NES
7 chiari malformation 30.2 GFAP MAP2 SYP
8 pleomorphic xanthoastrocytoma 30.2 GFAP IDH1 SYP
9 ganglioglioma 30.2 GFAP MAP2 NES S100B SYP
10 glioblastoma multiforme 30.0 GFAP IDH1 NES
11 temporal lobe epilepsy 30.0 GFAP GRIN1
12 hemimegalencephaly 30.0 GFAP GRIN1 MAP2
13 angiocentric glioma 30.0 GFAP IDH1 S100B SYP
14 epilepsy 10.8
15 astrocytoma 10.5
16 glioma 10.3
17 primary hepatic neuroendocrine carcinoma 10.2 S100B SYP
18 mixed type thymoma 10.2 S100B SYP
19 hypoganglionosis 10.2 S100B SYP
20 supratentorial primitive neuroectodermal tumor 10.2 S100B SYP
21 papillary tumor of the pineal region 10.2 MAP2 SYP
22 cerebellum cancer 10.2 MAP2 SYP
23 west nile virus 10.2
24 encephalocraniocutaneous lipomatosis 10.2
25 focal epilepsy 10.2
26 lipomatosis 10.2
27 rhabdoid cancer 10.2
28 glioblastoma 10.2
29 cerebral primitive neuroectodermal tumor 10.1 S100B SYP
30 desmoplastic infantile ganglioglioma 10.1 GFAP SYP
31 lung combined type small cell carcinoma 10.1 GFAP SYP
32 cellular ependymoma 10.1 GFAP SYP
33 ectopic cushing syndrome 10.1 S100B SYP
34 olfactory neuroblastoma 10.1 S100B SYP
35 clear cell ependymoma 10.1 GFAP SYP
36 cerebral neuroblastoma 10.1 GFAP SYP
37 subependymal glioma 10.1 GFAP SYP
38 intracranial cysts 10.1 GFAP S100B
39 cerebral ventricle cancer 10.1 GFAP SYP
40 gemistocytic astrocytoma 10.1 GFAP S100B
41 pineal gland cancer 10.1 GFAP SYP
42 melanotic neuroectodermal tumor 10.1 GFAP SYP
43 syringocystadenoma papilliferum 10.1 GFAP S100B
44 benign ependymoma 10.1 GFAP SYP
45 obstructive hydrocephalus 10.1 GFAP SYP
46 spindle cell rhabdomyosarcoma 10.1 GFAP S100B
47 benign glioma 10.1 GFAP SYP
48 myxopapillary ependymoma 10.1 GFAP S100B
49 chordoid glioma 10.1 GFAP S100B
50 pineoblastoma 10.1 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 GRIN1 NES
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 GRIN1 IDH1 NES
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

FDA approved drugs:

# Drug Name Active Ingredient(s) 18 Company Approval Date
1
Akynzeo 18 49 NETUPITANT AND PALONOSETRON (hydrochloride) Helsinn October 2014

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Molecular-Guided Therapy for Childhood Cancer Active, not 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, Cerebellum, Caudate Nucleus, Brain, Lung, Spinal Cord, Pineal

Publications for Dysembryoplastic Neuroepithelial Tumor

Articles related to Dysembryoplastic Neuroepithelial Tumor:

(show top 50) (show all 124)
# Title Authors Year
1
Dysembryoplastic neuroepithelial tumor-like pilocytic astrocytoma: A case report. ( 29768357 )
2018
2
Lethal disseminated dysembryoplastic neuroepithelial tumor following West Nile virus: Report of a very unusual combination. ( 29988761 )
2018
3
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. ( 28131206 )
2017
4
Cerebellar dysembryoplastic neuroepithelial tumor: report of a case and review of the literature. ( 28975449 )
2017
5
Long-term recurrence of dysembryoplastic neuroepithelial tumor: Clinical case report. ( 28781917 )
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
Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor. ( 27181792 )
2016
10
Atypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature. ( 26978682 )
2016
11
Volumetric growth analysis of an insular dysembryoplastic neuroepithelial tumor over a 10-year follow-up. ( 28194304 )
2016
12
Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis. ( 26921879 )
2016
13
A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures. ( 28018467 )
2016
14
Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient. ( 27127715 )
2016
15
Dysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases. ( 26796505 )
2016
16
Remitted epilepsy with dysembryoplastic neuroepithelial tumor involving the thalamus. ( 27163491 )
2016
17
Resection of a dysembryoplastic neuroepithelial tumor in the precentral gyrus. ( 26253415 )
2015
18
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. ( 24735014 )
2014
19
Manifestation and treatment of intraventricular dysembryoplastic neuroepithelial tumor. ( 24709201 )
2014
20
Dysembryoplastic neuroepithelial tumor transforming into malignancy: a case report. ( 25033864 )
2014
21
Oligodendroglioma (WHO grade I) in a young epilepsy patient: a specific entity lying within the spectrum of dysembryoplastic neuroepithelial tumor? ( 23432071 )
2013
22
Dysembryoplastic neuroepithelial tumor with rapid recurrence of pilocytic astrocytoma component. ( 23881357 )
2013
23
Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation. ( 23432479 )
2013
24
Dysembryoplastic Neuroepithelial Tumor: A Clinical, Neuroradiological, and Pathological Study of 15 Cases. ( 23752069 )
2013
25
Dysembryoplastic neuroepithelial tumor with atypical presentation: MRI and diffusion tensor characteristics. ( 24421925 )
2013
26
Glioblastoma Multiforme Arising From Dysembryoplastic Neuroepithelial Tumor in a Child in the Absence of Therapy. ( 24309599 )
2013
27
Electrocorticographic-histopathologic correlations implying epileptogenicity of dysembryoplastic neuroepithelial tumor. ( 24077279 )
2013
28
Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. ( 23163721 )
2013
29
Dysembryoplastic neuroepithelial tumor, a pure glial tumor? Immunohistochemical and morphometric studies. ( 23530928 )
2013
30
Recurrence and histological evolution of dysembryoplastic neuroepithelial tumor: A case report and review of the literature. ( 24137435 )
2013
31
Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma. ( 22990729 )
2012
32
A rare case of dysembryoplastic neuroepithelial tumor. ( 23230542 )
2012
33
Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: review of 2 cases with chromosome 1p/19q and IDH1 analysis. ( 22192702 )
2012
34
Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus. ( 22809555 )
2012
35
Complex form variant of dysembryoplastic neuroepithelial tumor of the cerebellum. ( 22988535 )
2012
36
Diffuse ependymal dysembryoplastic neuroepithelial tumor causing spinal drop metastases: a case report. ( 22778572 )
2012
37
Composite ganglioglioma/dysembryoplastic neuroepithelial tumor: a clinicopathologic study of 8 cases. ( 22221701 )
2012
38
Dysembryoplastic neuroepithelial tumor and calcifying pseudoneoplasms of the neuraxis: a collision of two seizure-associated lesions. ( 21726506 )
2011
39
Lhermitte-Duclos disease associated with dysembryoplastic neuroepithelial tumor differentiation with characteristic magnetic resonance appearance of "tiger striping". ( 21704939 )
2011
40
A patient with a dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery after initial seizure. ( 22777137 )
2011
41
Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report. ( 21899755 )
2011
42
Intraventricular dysembryoplastic neuroepithelial tumor in a pediatric patient: is it the most common extracortical location for DNT? ( 20959995 )
2011
43
Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy. ( 21456916 )
2011
44
A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter. ( 21121739 )
2010
45
Progressive biological behavior of a dysembryoplastic neuroepithelial tumor. ( 21196795 )
2010
46
Seizure control outcomes after resection of dysembryoplastic neuroepithelial tumor in 50 patients. ( 20043747 )
2010
47
Sudden death due to dysembryoplastic neuroepithelial tumor. ( 21056878 )
2010
48
Dysembryoplastic neuroepithelial tumor located in pericallosal and intraventricular area in a child. Case report. ( 19485727 )
2009
49
Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. ( 19267228 )
2009
50
Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum, seizure outcome, neuroradiology, and pathology. ( 19066912 )
2009

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 9.43 GRIN1 MAP2 SYP
2 cell body GO:0044297 9.32 GFAP MAP2
3 intermediate filament cytoskeleton GO:0045111 9.16 GFAP NES
4 terminal bouton GO:0043195 8.96 GRIN1 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 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

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