MCID: RST024
MIFTS: 27

Rosette-Forming Glioneuronal Tumor

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Rosette-Forming Glioneuronal Tumor

MalaCards integrated aliases for Rosette-Forming Glioneuronal Tumor:

Name: Rosette-Forming Glioneuronal Tumor 59 6
Rgnt 59

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA251975

Summaries for Rosette-Forming Glioneuronal Tumor

MalaCards based summary : Rosette-Forming Glioneuronal Tumor, also known as rgnt, is related to dysembryoplastic neuroepithelial tumor and pilocytic astrocytoma. An important gene associated with Rosette-Forming Glioneuronal Tumor is PIK3CA (Phosphatidylinositol-4,5-Bisphosphate 3-Kinase Catalytic Subunit Alpha), and among its related pathways/superpathways are Neuroscience and VEGF Pathway (Tocris). Affiliated tissues include spinal cord, hypothalamus and thalamus, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Rosette-Forming Glioneuronal Tumor

Diseases related to Rosette-Forming Glioneuronal Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 54)
# Related Disease Score Top Affiliating Genes
1 dysembryoplastic neuroepithelial tumor 29.3 MAP2 S100B SYP
2 pilocytic astrocytoma 28.4 FGFR1 PIK3CA S100B SYP
3 brain stem glioma 10.4 FGFR1 PIK3CA
4 lipomatosis 10.4 FGFR1 PIK3CA
5 hemimegalencephaly 10.3 MAP2 PIK3CA
6 aggressive digital papillary adenocarcinoma 10.2 PIK3CA S100B
7 astrocytoma 10.2
8 spinal chordoma 10.1 PIK3CA S100B
9 neurofibromatosis, type i 10.1
10 noonan syndrome 1 10.1
11 epilepsy 10.1
12 type i 10.1
13 papillary glioneuronal tumors 10.1
14 pseudo-turner syndrome 10.1
15 osteochondroma 10.0 FGFR1 S100B
16 megalencephaly 9.9 PIK3CA S100B
17 fibrolamellar carcinoma 9.9 FGFR1 SYP
18 papillary tumor of the pineal region 9.9 MAP2 SYP
19 cerebellum cancer 9.8 MAP2 SYP
20 chiari malformation 9.8 MAP2 SYP
21 pleomorphic xanthoastrocytoma 9.8 MAP2 SYP
22 giant cell glioblastoma 9.8 FGFR1 SYP
23 central neurocytoma 9.8 MAP2 SYP
24 cerebellar liponeurocytoma 9.8 MAP2 SYP
25 primary hepatic neuroendocrine carcinoma 9.7 S100B SYP
26 optic nerve neoplasm 9.7 MAP2 SYP
27 mixed type thymoma 9.7 S100B SYP
28 chordoid meningioma 9.7 S100B SYP
29 hypoganglionosis 9.7 S100B SYP
30 gliosarcoma 9.7 FGFR1 S100B
31 supratentorial primitive neuroectodermal tumor 9.7 S100B SYP
32 melanotic neuroectodermal tumor 9.7 S100B SYP
33 angiocentric glioma 9.7 S100B SYP
34 pineocytoma 9.7 S100B SYP
35 anaplastic ependymoma 9.7 S100B SYP
36 clear cell ependymoma 9.7 MAP2 SYP
37 papillary ependymoma 9.7 S100B SYP
38 subependymal giant cell astrocytoma 9.7 S100B SYP
39 papilloma of choroid plexus 9.7 S100B SYP
40 olfactory neuroblastoma 9.6 S100B SYP
41 cerebral primitive neuroectodermal tumor 9.6 S100B SYP
42 large cell carcinoma 9.6 FGFR1 SYP
43 ectopic cushing syndrome 9.6 S100B SYP
44 neurofibroma 9.5 S100B SYP
45 atypical teratoid rhabdoid tumor 9.5 S100B SYP
46 ependymoma 9.5 S100B SYP
47 hemangioblastoma 9.4 S100B SYP
48 merkel cell carcinoma 9.4 MAP2 SYP
49 tuberous sclerosis 9.3 PIK3CA S100B SYP
50 adrenocortical carcinoma, hereditary 9.2 PIK3CA SYP

Graphical network of the top 20 diseases related to Rosette-Forming Glioneuronal Tumor:



Diseases related to Rosette-Forming Glioneuronal Tumor

Symptoms & Phenotypes for Rosette-Forming Glioneuronal Tumor

GenomeRNAi Phenotypes related to Rosette-Forming Glioneuronal Tumor according to GeneCards Suite gene sharing:

26 (show all 17)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.6 FGFR1 PPP1R1A S100B
2 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.6 PPP1R1A
3 Increased shRNA abundance (Z-score > 2) GR00366-A-125 9.6 FGFR1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.6 FGFR1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-132 9.6 PPP1R1A
6 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.6 PPP1R1A
7 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.6 PPP1R1A
8 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.6 FGFR1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-183 9.6 FGFR1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-31 9.6 PPP1R1A
11 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.6 FGFR1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.6 FGFR1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-50 9.6 FGFR1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.6 S100B
15 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.6 FGFR1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-83 9.6 FGFR1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.6 S100B

MGI Mouse Phenotypes related to Rosette-Forming Glioneuronal Tumor:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.1 FGFR1 MAP2 PIK3CA PPP1R1A S100B SYP

Drugs & Therapeutics for Rosette-Forming Glioneuronal Tumor

Search Clinical Trials , NIH Clinical Center for Rosette-Forming Glioneuronal Tumor

Genetic Tests for Rosette-Forming Glioneuronal Tumor

Anatomical Context for Rosette-Forming Glioneuronal Tumor

MalaCards organs/tissues related to Rosette-Forming Glioneuronal Tumor:

41
Spinal Cord, Hypothalamus, Thalamus

Publications for Rosette-Forming Glioneuronal Tumor

Articles related to Rosette-Forming Glioneuronal Tumor:

(show all 27)
# Title Authors Year
1
Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background. ( 29250844 )
2018
2
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. ( 28131206 )
2017
3
Rosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review. ( 27915062 )
2017
4
Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle in Children: Case Report and Literature Review. ( 28826709 )
2017
5
Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. ( 29312599 )
2017
6
Rosette-forming Glioneuronal Tumor: A Rare Posterior Fossa Tumor in an Adolescent. ( 28904578 )
2017
7
Rosette-forming glioneuronal tumor of the fourth ventricle. ( 28879932 )
2017
8
Rosette-Forming Glioneuronal Tumor Arising from the Spinal Cord. ( 28847111 )
2017
9
Case Report: A Rosette-forming Glioneuronal Tumor in the Tectal Plate in a Patient with Neurofibromatosis Type I. ( 27917325 )
2016
10
Integrated tumor and germline whole-exome sequencing identifies mutations in MAPK and PI3K pathway genes in an adolescent with rosette-forming glioneuronal tumor of the fourth ventricle. ( 27626068 )
2016
11
Rosette-forming glioneuronal tumor of the thalamus. ( 27049064 )
2016
12
Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update. ( 26438552 )
2016
13
Rosette-forming Glioneuronal Tumor: Rare Case Presented with Spontaneous Disappearance of Contrast Enhancement. ( 28663967 )
2015
14
Rosette-forming glioneuronal tumor originating in the hypothalamus. ( 26156565 )
2015
15
Rosette-forming glioneuronal tumor with dissemination throughout the ventricular system: a case report. ( 25373141 )
2015
16
Molecular Profiling of a Rare Rosette-Forming Glioneuronal Tumor Arising in the Spinal Cord. ( 26371886 )
2015
17
Rosette forming glioneuronal tumor of the fourth ventricle in squash cytology smear. ( 25745291 )
2014
18
An individual patient data meta-analysis on characteristics and outcome of patients with papillary glioneuronal tumor, rosette glioneuronal tumor with neuropil-like islands and rosette forming glioneuronal tumor of the fourth ventricle. ( 24991807 )
2014
19
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. ( 24735014 )
2014
20
Synchronous rosette-forming glioneuronal tumor and diffuse astrocytoma with molecular characterization: a case report. ( 24986181 )
2014
21
FGFR1 mutations in Rosette-forming glioneuronal tumors of the fourth ventricle. ( 24806303 )
2014
22
Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. ( 23163721 )
2013
23
Recurrent PIK3CA mutations in rosette-forming glioneuronal tumor. ( 21997360 )
2012
24
Absence of KIAA1549-BRAF fusion in rosette-forming glioneuronal tumors of the fourth ventricle (RGNT). ( 22814862 )
2012
25
Rosette forming glioneuronal tumor in association with Noonan syndrome: pathobiological implications. ( 22011734 )
2011
26
Rosette-forming glioneuronal tumor of the fourth ventricle. ( 12915951 )
2003
27
A rosette-forming glioneuronal tumor of the fourth ventricle: infratentorial form of dysembryoplastic neuroepithelial tumor? ( 11979088 )
2002

Variations for Rosette-Forming Glioneuronal Tumor

ClinVar genetic disease variations for Rosette-Forming Glioneuronal Tumor:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 PIK3CA NM_006218.3(PIK3CA): c.3140A> G (p.His1047Arg) single nucleotide variant Pathogenic rs121913279 GRCh37 Chromosome 3, 178952085: 178952085
2 PIK3CA NM_006218.3(PIK3CA): c.3140A> G (p.His1047Arg) single nucleotide variant Pathogenic rs121913279 GRCh38 Chromosome 3, 179234297: 179234297
3 FGFR1 NM_023110.2(FGFR1): c.1638C> A (p.Asn546Lys) single nucleotide variant Pathogenic rs779707422 GRCh37 Chromosome 8, 38274849: 38274849
4 FGFR1 NM_023110.2(FGFR1): c.1638C> A (p.Asn546Lys) single nucleotide variant Pathogenic rs779707422 GRCh38 Chromosome 8, 38417331: 38417331
5 RNF216 NM_207116.2(RNF216): c.2297G> A (p.Arg766His) single nucleotide variant Uncertain significance rs761469213 GRCh38 Chromosome 7, 5623164: 5623164
6 RNF216 NM_207116.2(RNF216): c.2297G> A (p.Arg766His) single nucleotide variant Uncertain significance rs761469213 GRCh37 Chromosome 7, 5662795: 5662795
7 PPP1R1A NM_006741.3(PPP1R1A): c.372_373delGTinsCA (p.Glu124_Ser125delinsAspThr) indel Uncertain significance rs1064792893 GRCh38 Chromosome 12, 54582006: 54582007
8 PPP1R1A NM_006741.3(PPP1R1A): c.372_373delGTinsCA (p.Glu124_Ser125delinsAspThr) indel Uncertain significance rs1064792893 GRCh37 Chromosome 12, 54975790: 54975791

Expression for Rosette-Forming Glioneuronal Tumor

Search GEO for disease gene expression data for Rosette-Forming Glioneuronal Tumor.

Pathways for Rosette-Forming Glioneuronal Tumor

Pathways related to Rosette-Forming Glioneuronal Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 12.03 MAP2 S100B SYP
2
Show member pathways
11.32 FGFR1 PIK3CA
3 11.18 FGFR1 PIK3CA
4 10.95 FGFR1 PIK3CA
5 10.83 FGFR1 PIK3CA
6 10.82 FGFR1 MAP2 S100B SYP
7 10.47 FGFR1 PIK3CA

GO Terms for Rosette-Forming Glioneuronal Tumor

Biological processes related to Rosette-Forming Glioneuronal Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 phosphatidylinositol-3-phosphate biosynthetic process GO:0036092 9.26 FGFR1 PIK3CA
2 phosphatidylinositol-mediated signaling GO:0048015 9.16 FGFR1 PIK3CA
3 cellular response to organic substance GO:0071310 8.96 MAP2 SYP
4 regulation of neuronal synaptic plasticity GO:0048168 8.62 S100B SYP

Molecular functions related to Rosette-Forming Glioneuronal Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 8.96 FGFR1 PIK3CA
2 1-phosphatidylinositol-3-kinase activity GO:0016303 8.62 FGFR1 PIK3CA

Sources for Rosette-Forming Glioneuronal 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
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