DNET
MCID: DYS006
MIFTS: 35

Dysembryoplastic Neuroepithelial Tumor (DNET) malady

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Dysembryoplastic Neuroepithelial Tumor

Aliases & Descriptions for Dysembryoplastic Neuroepithelial Tumor:

Name: Dysembryoplastic Neuroepithelial Tumor 12 50 56 14 69
Dysembryoplastic Neuroepithelial Neoplasm 12
Dnet 56

Characteristics:

Orphanet epidemiological data:

56
dysembryoplastic neuroepithelial tumor
Inheritance: Not applicable; Age of onset: All ages;

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:2679
NCIt 47 C9505
Orphanet 56 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 pilocytic astrocytoma and pleomorphic xanthoastrocytoma, 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 phenotype is 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 137)
id Related Disease Score Top Affiliating Genes
1 pilocytic astrocytoma 30.5 GFAP SYP
2 pleomorphic xanthoastrocytoma 30.4 GFAP SYP
3 rosette-forming glioneuronal tumor of fourth ventricule 11.1
4 epilepsy 10.5
5 oligodendroglioma 10.4
6 astrocytoma 10.3
7 ganglioglioma 10.3
8 jejunal neuroendocrine tumor 10.2 S100B SYP
9 corneal intraepithelial neoplasm 10.2 S100B SYP
10 hypoparathyroidism-intellectual disability-dysmorphism syndrome 10.2 S100B SYP
11 monkeypox 10.2 S100B SYP
12 movement disease 10.2 S100B SYP
13 autosomal recessive stickler syndrome 10.2 MAP2 SYP
14 early-onset parkinson disease 10.2 S100B SYP
15 bone deterioration disease 10.2 S100B SYP
16 diabetes hypogonadism deafness mental retardation 10.2 GFAP SYP
17 strabismus 10.2 S100B SYP
18 brain stem ependymoma 10.2 GFAP SYP
19 ampulla of vater mucinous adenocarcinoma 10.2 S100B SYP
20 intrahepatic bile duct adenoma 10.2 GFAP SYP
21 pilocytic astrocytoma of cerebellum 10.2 GFAP SYP
22 cervical benign neoplasm 10.2 GFAP SYP
23 myxopapillary ependymoma 10.2 GFAP SYP
24 epidermal appendage tumor 10.2 GFAP SYP
25 clear cell meningioma 10.2 GFAP SYP
26 myelinoclastic diffuse sclerosis 10.2 GFAP S100B
27 cerebral folate receptor alpha deficiency 10.2 GFAP SYP
28 bronchial mucus gland adenoma 10.2 GFAP S100B
29 spleen angiosarcoma 10.2 GFAP S100B
30 ovarian cystadenoma 10.2 GFAP S100B
31 blood group incompatibility 10.2 GFAP SYP
32 childhood brain germinoma 10.2 S100B SYP
33 trombiculiasis 10.2 GFAP S100B
34 biliary papillomatosis 10.2 GFAP S100B
35 mediastinal granular cell myoblastoma 10.2 GFAP SYP
36 central neurocytoma 10.2 GFAP SYP
37 mast-cell sarcoma 10.2 GFAP SYP
38 olfactory neuroblastoma 10.2 GFAP SYP
39 porokeratosis 10.2 GFAP S100B
40 sternum cancer 10.2 GFAP S100B
41 pineoblastoma 10.2 GFAP SYP
42 pneumothorax 10.2 GFAP SYP
43 vulvar squamous papilloma 10.2 GFAP S100B
44 arthus reaction 10.2 GFAP SYP
45 bone dysplasia azouz type 10.2 GFAP SYP
46 pancreatic cancer 10.2 GFAP SYP
47 hemorrhagic proctocolitis 10.2 GFAP MAP2
48 pacinian tumor 10.2 GFAP SYP
49 skin glomus tumor 10.2 GFAP S100B
50 pathologic nystagmus 10.1 S100B 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
id 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 GRIN1 IDH1 NES
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:


id Name Status NCT ID Phase
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:

39
Temporal Lobe, Brain, Cerebellum, Caudate Nucleus, Cortex, Thalamus, Parietal Lobe

Publications for Dysembryoplastic Neuroepithelial Tumor

Articles related to Dysembryoplastic Neuroepithelial Tumor:

(show top 50) (show all 126)
id Title Authors Year
1
Dysembryoplastic neuroepithelial tumor: A rare brain tumor not to be misdiagnosed. ( 27057233 )
2016
2
MicroRNA expression profiles in pediatric dysembryoplastic neuroepithelial tumors. ( 26698155 )
2016
3
Long-term drug-resistant temporal lobe epilepsy associated with a mixed ganglioglioma and dysembryoplastic neuroepithelial tumor in an elderly patient. ( 27127715 )
2016
4
Dysembryoplastic Neuroepithelial Tumor of the Septum Pellucidum and the Supratentorial Midline: Histopathologic, Neuroradiologic, and Molecular Features of 7 Cases. ( 26796505 )
2016
5
Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis. ( 26921879 )
2016
6
A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures. ( 28018467 )
2016
7
Remitted epilepsy with dysembryoplastic neuroepithelial tumor involving the thalamus. ( 27163491 )
2016
8
Atypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature. ( 26978682 )
2016
9
Radiologic response to radiation therapy concurrent with temozolomide for progressive simple dysembryoplastic neuroepithelial tumor. ( 27181792 )
2016
10
Resection of a dysembryoplastic neuroepithelial tumor in the precentral gyrus. ( 26253415 )
2015
11
BRAF V600E mutations are frequent in dysembryoplastic neuroepithelial tumors and subependymal giant cell astrocytomas. ( 25346165 )
2015
12
Well-differentiated pediatric glial neoplasms with features of oligodendroglioma, angiocentric glioma and dysembryoplastic neuroepithelial tumors: a morphological diagnostic challenge. ( 24448703 )
2014
13
Dysembryoplastic neuroepithelial tumor transforming into malignancy: a case report. ( 25033864 )
2014
14
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. ( 24735014 )
2014
15
Manifestation and treatment of intraventricular dysembryoplastic neuroepithelial tumor. ( 24709201 )
2014
16
Electrocorticographic-histopathologic correlations implying epileptogenicity of dysembryoplastic neuroepithelial tumor. ( 24077279 )
2013
17
Dysembryoplastic neuroepithelial tumor with rapid recurrence of pilocytic astrocytoma component. ( 23881357 )
2013
18
Oligodendroglioma (WHO grade I) in a young epilepsy patient: a specific entity lying within the spectrum of dysembryoplastic neuroepithelial tumor? ( 23432071 )
2013
19
Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E) mutation and expression. ( 23442159 )
2013
20
Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation. ( 23432479 )
2013
21
Dysembryoplastic Neuroepithelial Tumor: A Clinical, Neuroradiological, and Pathological Study of 15 Cases. ( 23752069 )
2013
22
Dysembryoplastic neuroepithelial tumor with atypical presentation: MRI and diffusion tensor characteristics. ( 24421925 )
2013
23
Recurrence and histological evolution of dysembryoplastic neuroepithelial tumor: A case report and review of the literature. ( 24137435 )
2013
24
Dysembryoplastic neuroepithelial tumor, a pure glial tumor? Immunohistochemical and morphometric studies. ( 23530928 )
2013
25
Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. ( 23163721 )
2013
26
Glioblastoma Multiforme Arising From Dysembryoplastic Neuroepithelial Tumor in a Child in the Absence of Therapy. ( 24309599 )
2013
27
Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma. ( 22990729 )
2012
28
Complex form variant of dysembryoplastic neuroepithelial tumor of the cerebellum. ( 22988535 )
2012
29
Dysembryoplastic neuroepithelial tumor-like neoplasm of the septum pellucidum: review of 2 cases with chromosome 1p/19q and IDH1 analysis. ( 22192702 )
2012
30
A rare case of dysembryoplastic neuroepithelial tumor. ( 23230542 )
2012
31
Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus. ( 22809555 )
2012
32
Composite ganglioglioma/dysembryoplastic neuroepithelial tumor: a clinicopathologic study of 8 cases. ( 22221701 )
2012
33
Diffuse ependymal dysembryoplastic neuroepithelial tumor causing spinal drop metastases: a case report. ( 22778572 )
2012
34
Intraventricular dysembryoplastic neuroepithelial tumor in a pediatric patient: is it the most common extracortical location for DNT? ( 20959995 )
2011
35
Lhermitte-Duclos disease associated with dysembryoplastic neuroepithelial tumor differentiation with characteristic magnetic resonance appearance of "tiger striping". ( 21704939 )
2011
36
Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report. ( 21899755 )
2011
37
Diffuse hemispheric dysembryoplastic neuroepithelial tumor: a new radiological variant associated with early-onset severe epilepsy. ( 21456916 )
2011
38
Dysembryoplastic neuroepithelial tumor and calcifying pseudoneoplasms of the neuraxis: a collision of two seizure-associated lesions. ( 21726506 )
2011
39
A patient with a dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery after initial seizure. ( 22777137 )
2011
40
Sudden death due to dysembryoplastic neuroepithelial tumor. ( 21056878 )
2010
41
Progressive biological behavior of a dysembryoplastic neuroepithelial tumor. ( 21196795 )
2010
42
Seizure control outcomes after resection of dysembryoplastic neuroepithelial tumor in 50 patients. ( 20043747 )
2010
43
A cortical dysembryoplastic neuroepithelial tumor initially occurring in the periventricular white matter. ( 21121739 )
2010
44
Editorial. Dysembryoplastic neuroepithelial tumor. ( 19485726 )
2009
45
F-18 FDG-PET imaging of dysembryoplastic neuroepithelial tumor-like astrocytoma. ( 19893407 )
2009
46
Dysembryoplastic neuroepithelial tumor located in pericallosal and intraventricular area in a child. Case report. ( 19485727 )
2009
47
Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum, seizure outcome, neuroradiology, and pathology. ( 19066912 )
2009
48
Dysembryoplastic neuroepithelial tumor: a clinicopathological study of 32 cases. ( 19165515 )
2009
49
Intraventricular dysembryoplastic neuroepithelial tumor-like neoplasm with disseminated spinal tumor. ( 19263357 )
2009
50
Clinicopathologic features of recurrent dysembryoplastic neuroepithelial tumor and rare malignant transformation: a report of 5 cases and review of the literature. ( 19267228 )
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:

id Super pathways Score Top Affiliating Genes
1 11.86 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:

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

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

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

id Name GO ID Score Top Affiliating Genes
1 receptor binding GO:0005102 8.8 GRIN1 IDH1 S100B

Sources for Dysembryoplastic Neuroepithelial Tumor

3 CDC
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11 DGIdb
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33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
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48 NDF-RT
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55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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