DNET
MCID: DYS006
MIFTS: 35

Dysembryoplastic Neuroepithelial Tumor (DNET) malady

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Dysembryoplastic Neuroepithelial Tumor

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Aliases & Descriptions for Dysembryoplastic Neuroepithelial Tumor:

Name: Dysembryoplastic Neuroepithelial Tumor 11 48 54 13 68
Dysembryoplastic Neuroepithelial Neoplasm 11
 
Dnet 54

Characteristics:

Orphanet epidemiological data:

54
dysembryoplastic neuroepithelial tumor:
Inheritance: Not applicable; Age of onset: All ages

Classifications:



External Ids:

Disease Ontology11 DOID:2679
NCIt45 C9505
Orphanet54 ORPHA251946
UMLS via Orphanet69 C1266177

Summaries for Dysembryoplastic Neuroepithelial Tumor

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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 are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. Affiliated tissues include temporal lobe, brain and cerebellum, and related mouse phenotype Increased shRNA abundance (Z-score > 2).

Related Diseases for Dysembryoplastic Neuroepithelial Tumor

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

(show top 50)    (show all 137)
idRelated DiseaseScoreTop Affiliating Genes
1pilocytic astrocytoma30.5GFAP, SYP
2pleomorphic xanthoastrocytoma30.4GFAP, SYP
3rosette-forming glioneuronal tumor of fourth ventricule11.1
4epilepsy10.5
5oligodendroglioma10.4
6astrocytoma10.3
7ganglioglioma10.3
8jejunal neuroendocrine tumor10.2S100B, SYP
9corneal intraepithelial neoplasm10.2S100B, SYP
10hypoparathyroidism-intellectual disability-dysmorphism syndrome10.2S100B, SYP
11monkeypox10.2S100B, SYP
12movement disease10.2S100B, SYP
13autosomal recessive stickler syndrome10.2MAP2, SYP
14early-onset parkinson disease10.2S100B, SYP
15bone deterioration disease10.2S100B, SYP
16diabetes hypogonadism deafness mental retardation10.2GFAP, SYP
17strabismus10.2S100B, SYP
18brain stem ependymoma10.2GFAP, SYP
19ampulla of vater mucinous adenocarcinoma10.2S100B, SYP
20intrahepatic bile duct adenoma10.2GFAP, SYP
21pilocytic astrocytoma of cerebellum10.2GFAP, SYP
22cervical benign neoplasm10.2GFAP, SYP
23myxopapillary ependymoma10.2GFAP, SYP
24epidermal appendage tumor10.2GFAP, SYP
25clear cell meningioma10.2GFAP, SYP
26myelinoclastic diffuse sclerosis10.2GFAP, S100B
27cerebral folate receptor alpha deficiency10.2GFAP, SYP
28bronchial mucus gland adenoma10.2GFAP, S100B
29spleen angiosarcoma10.2GFAP, S100B
30ovarian cystadenoma10.2GFAP, S100B
31blood group incompatibility10.2GFAP, SYP
32childhood brain germinoma10.2S100B, SYP
33trombiculiasis10.2GFAP, S100B
34biliary papillomatosis10.2GFAP, S100B
35mediastinal granular cell myoblastoma10.2GFAP, SYP
36central neurocytoma10.2GFAP, SYP
37mast-cell sarcoma10.2GFAP, SYP
38olfactory neuroblastoma10.2GFAP, SYP
39porokeratosis10.2GFAP, S100B
40sternum cancer10.2GFAP, S100B
41pineoblastoma10.2GFAP, SYP
42pneumothorax10.2GFAP, SYP
43vulvar squamous papilloma10.2GFAP, S100B
44arthus reaction10.2GFAP, SYP
45bone dysplasia azouz type10.2GFAP, SYP
46pancreatic cancer10.2GFAP, SYP
47hemorrhagic proctocolitis10.2GFAP, MAP2
48pacinian tumor10.2GFAP, SYP
49skin glomus tumor10.2GFAP, S100B
50pathologic nystagmus10.1S100B, 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

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UMLS symptoms related to Dysembryoplastic Neuroepithelial Tumor:


seizures

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

26
idDescriptionGenomeRNAi Source AccessionScoreTop Affiliating Genes
1GR00366-A-1928.7GRIN1, IDH1, NES

Drugs & Therapeutics for Dysembryoplastic Neuroepithelial Tumor

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Interventional clinical trials:

idNameStatusNCT IDPhase
1Molecular-Guided Therapy for Childhood CancerRecruitingNCT02162732

Search NIH Clinical Center for Dysembryoplastic Neuroepithelial Tumor

Genetic Tests for Dysembryoplastic Neuroepithelial Tumor

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Anatomical Context for Dysembryoplastic Neuroepithelial Tumor

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MalaCards organs/tissues related to Dysembryoplastic Neuroepithelial Tumor:

36
Temporal lobe, Brain, Cerebellum, Caudate nucleus, Thalamus, Parietal lobe, Cortex

Publications for Dysembryoplastic Neuroepithelial Tumor

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Articles related to Dysembryoplastic Neuroepithelial Tumor:

(show top 50)    (show all 126)
idTitleAuthorsYear
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

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Expression for genes affiliated with Dysembryoplastic Neuroepithelial Tumor

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

Pathways for genes affiliated with Dysembryoplastic Neuroepithelial Tumor

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

idSuper pathwaysScoreTop Affiliating Genes
18.0GFAP, GRIN1, MAP2, S100B, SYP
27.7GFAP, MAP2, NES, S100B, SYP

GO Terms for genes affiliated with Dysembryoplastic Neuroepithelial Tumor

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

idNameGO IDScoreTop Affiliating Genes
1excitatory synapseGO:006007610.2GRIN1, SYP
2intermediate filament cytoskeletonGO:00451119.8GFAP, NES
3terminal boutonGO:00431959.6GRIN1, SYP
4neuron projectionGO:00430059.4GRIN1, MAP2, SYP

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

idNameGO IDScoreTop Affiliating Genes
1cellular response to organic substanceGO:007131010.1MAP2, SYP
2learning or memoryGO:000761110.0GRIN1, S100B
3regulation of long-term neuronal synaptic plasticityGO:00481699.9GRIN1, SYP
4memoryGO:00076139.9GRIN1, S100B
5regulation of axonogenesisGO:00507709.8GRIN1, MAP2
6regulation of neuronal synaptic plasticityGO:00481689.1GRIN1, S100B, SYP

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

idNameGO IDScoreTop Affiliating Genes
1receptor bindingGO:00051029.0GRIN1, IDH1, S100B

Sources for Dysembryoplastic Neuroepithelial Tumor

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet