RGNT
MCID: RST024
MIFTS: 31

Rosette-Forming Glioneuronal Tumor (RGNT)

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 53 59 6
Rgnt 53 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 central neurocytoma. 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 FGFR1 mutant receptor activation. Affiliated tissues include spinal cord, pineal and cerebellum, 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 51)
# Related Disease Score Top Affiliating Genes
1 dysembryoplastic neuroepithelial tumor 30.4 MAP2 S100B SYP
2 central neurocytoma 30.2 MAP2 SYP
3 neurofibromatosis, type i 10.4
4 astrocytoma 10.4
5 noonan syndrome 1 10.2
6 epilepsy 10.2
7 papillary glioneuronal tumors 10.2
8 pilocytic astrocytoma 10.2
9 pseudo-turner syndrome 10.2
10 aggressive digital papillary adenocarcinoma 10.1 PIK3CA S100B
11 megalencephaly 10.1 PIK3CA S100B
12 lipomatosis 10.1 FGFR1 PIK3CA
13 hypogonadotropic hypogonadism 7 with or without anosmia 10.1 FGFR1 RNF216
14 brain stem glioma 10.1 FGFR1 PIK3CA
15 spinal chordoma 10.1 PIK3CA S100B
16 fibrolamellar carcinoma 10.0 FGFR1 SYP
17 osteochondroma 10.0 FGFR1 S100B
18 primary hepatic neuroendocrine carcinoma 10.0 S100B SYP
19 mixed type thymoma 10.0 S100B SYP
20 chordoid meningioma 10.0 S100B SYP
21 pilomyxoid astrocytoma 10.0 FGFR1 SYP
22 hypoganglionosis 10.0 S100B SYP
23 supratentorial primitive neuroectodermal tumor 10.0 S100B SYP
24 angiocentric glioma 10.0 S100B SYP
25 pineocytoma 10.0 S100B SYP
26 anaplastic ependymoma 10.0 S100B SYP
27 ependymoma 10.0 S100B SYP
28 papillary ependymoma 10.0 S100B SYP
29 papilloma of choroid plexus 10.0 S100B SYP
30 subependymal giant cell astrocytoma 10.0 S100B SYP
31 hemimegalencephaly 10.0 MAP2 PIK3CA
32 cerebral primitive neuroectodermal tumor 10.0 S100B SYP
33 ectopic cushing syndrome 10.0 S100B SYP
34 giant cell glioblastoma 10.0 FGFR1 SYP
35 olfactory neuroblastoma 10.0 S100B SYP
36 tracheoesophageal fistula 9.9 S100B SYP
37 papillary tumor of the pineal region 9.9 MAP2 SYP
38 large cell carcinoma 9.9 FGFR1 SYP
39 neurofibroma 9.9 S100B SYP
40 cerebellum cancer 9.9 MAP2 SYP
41 chiari malformation 9.9 MAP2 SYP
42 hemangioblastoma 9.9 S100B SYP
43 tuberous sclerosis 9.9 PIK3CA S100B SYP
44 cerebellar liponeurocytoma 9.9 MAP2 SYP
45 optic nerve neoplasm 9.9 MAP2 SYP
46 neuroblastoma 9.9 FGFR1 PIK3CA SYP
47 gliosarcoma 9.8 FGFR1 S100B
48 optic nerve glioma 9.7 MAP2 S100B SYP
49 ganglioglioma 9.7 MAP2 S100B SYP
50 merkel cell carcinoma 9.7 MAP2 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, Pineal, Cerebellum, Temporal Lobe, Thalamus, Lung, Brain

Publications for Rosette-Forming Glioneuronal Tumor

Articles related to Rosette-Forming Glioneuronal Tumor:

(show top 50) (show all 55)
# Title Authors Year
1
Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background. ( 29250844 )
2018
2
Rosette-forming glioneuronal tumor of the fourth ventricle. Two cases report and literature review. ( 29338911 )
2018
3
Rosette-Forming Glioneuronal Tumor of Spinal Cord. ( 30121405 )
2018
4
Mesial temporal lobe rosette-forming glioneuronal tumor : an unusual location for a rare tumor. ( 30178178 )
2018
5
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. ( 28131206 )
2017
6
Rosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review. ( 27915062 )
2017
7
Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle in Children: Case Report and Literature Review. ( 28826709 )
2017
8
Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. ( 29312599 )
2017
9
Rosette-forming Glioneuronal Tumor: A Rare Posterior Fossa Tumor in an Adolescent. ( 28904578 )
2017
10
Rosette-forming glioneuronal tumor of the fourth ventricle. ( 28879932 )
2017
11
Rosette-Forming Glioneuronal Tumor Arising from the Spinal Cord. ( 28847111 )
2017
12
Case Report: A Rosette-forming Glioneuronal Tumor in the Tectal Plate in a Patient with Neurofibromatosis Type I. ( 27917325 )
2016
13
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
14
Rosette-forming glioneuronal tumor of the thalamus. ( 27049064 )
2016
15
Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update. ( 26438552 )
2016
16
Rosette-forming Glioneuronal Tumor: Rare Case Presented with Spontaneous Disappearance of Contrast Enhancement. ( 28663967 )
2015
17
Rosette-forming glioneuronal tumor originating in the hypothalamus. ( 26156565 )
2015
18
Rosette-forming glioneuronal tumor with dissemination throughout the ventricular system: a case report. ( 25373141 )
2015
19
Molecular Profiling of a Rare Rosette-Forming Glioneuronal Tumor Arising in the Spinal Cord. ( 26371886 )
2015
20
Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle: a highly aggressive case. ( 24984922 )
2015
21
Rosette forming glioneuronal tumor of the fourth ventricle in squash cytology smear. ( 25745291 )
2014
22
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
23
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. ( 24735014 )
2014
24
Synchronous rosette-forming glioneuronal tumor and diffuse astrocytoma with molecular characterization: a case report. ( 24986181 )
2014
25
Rosette-forming glioneuronal tumor of the fourth ventricle with neurocytoma component. ( 23817994 )
2014
26
Mixed glioneuronal tumor: a dysembryoplastic neuroepithelial tumor with rosette-forming glioneuronal tumor component. ( 23163721 )
2013
27
Rosette-forming glioneuronal tumor of the cerebellum in statu nascendi: an incidentally detected diminutive example indicates derivation from the internal granule cell layer. ( 23547894 )
2013
28
Rosette-forming glioneuronal tumor of the fourth ventricle. ( 27330611 )
2013
29
Recurrent PIK3CA mutations in rosette-forming glioneuronal tumor. ( 21997360 )
2012
30
Rosette-forming glioneuronal tumor of the fourth ventricle. ( 21489633 )
2012
31
Rosette-forming glioneuronal tumor of the septum pellucidum with extension to the supratentorial ventricles: rare case with genetic analysis. ( 22017246 )
2012
32
Rosette-forming glioneuronal tumor -- evidence of stem cell origin with biphenotypic differentiation. ( 22971894 )
2012
33
Rosette-forming glioneuronal tumor in the pineal gland and the third ventricle: a case with radiological and clinical implications. ( 23256084 )
2012
34
Rosette forming glioneuronal tumor in association with Noonan syndrome: pathobiological implications. ( 22011734 )
2011
35
Rosette-forming glioneuronal tumor of the fourth ventricle in an elderly patient. ( 20865299 )
2011
36
Rosette-forming glioneuronal tumor: a pineal region case with IDH1 and IDH2 mutation analyses and literature review of 43 cases. ( 20872044 )
2011
37
Rosette-forming glioneuronal tumor of the fourth ventricle with advanced microvascular proliferation--a case report. ( 21092064 )
2011
38
Analysis of KIAA1549-BRAF fusion status in a case of rosette-forming glioneuronal tumor of the fourth ventricle (RGNT). ( 21518014 )
2011
39
Rosette-forming glioneuronal tumor of the fourth ventricle with bilateral olivary degeneration. ( 21786101 )
2011
40
Rosette forming glioneuronal tumor pineal gland and tectum: an intraoperative diagnosis on smear preparation. ( 19941371 )
2010
41
Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: Case report and review of literature. ( 20133047 )
2010
42
Rosette-forming glioneuronal tumor of the fourth ventricle--case report. ( 20339273 )
2010
43
Rosette-forming glioneuronal tumor of the fourth ventricle. ( 20358506 )
2010
44
A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosette-forming glioneuronal tumor originating from the spinal cord. ( 19269010 )
2009
45
Rosette-forming glioneuronal tumor: report of a chiasmal-optic nerve example in neurofibromatosis type 1: special pathology report. ( 19349806 )
2009
46
Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination. ( 19585134 )
2009
47
Rosette-forming glioneuronal tumor. ( 19616823 )
2009
48
Rosette-forming glioneuronal tumor of the fourth ventricle. A rare neoplasm of the central nervous system. ( 21048635 )
2009
49
Rosette-forming glioneuronal tumor: pathology case report. ( 18580784 )
2008
50
Clinical implications of the infratentorial rosette-forming glioneuronal tumor: case report. ( 18728557 )
2008

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

GO Terms for Rosette-Forming Glioneuronal Tumor

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

# Name GO ID Score Top Affiliating Genes
1 Schaffer collateral - CA1 synapse GO:0098685 8.62 RNF216 SYP

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

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9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
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62 PubMed
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69 SNOMED-CT via HPO
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74 UMLS via Orphanet
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