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 ependymoma 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 brain, and related phenotype is behavior/neurological.

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 74)
# Related Disease Score Top Affiliating Genes
1 ependymoma 30.4 SYP S100B
2 central neurocytoma 30.3 SYP MAP2
3 dysembryoplastic neuroepithelial tumor 29.9 SYP S100B MAP2
4 hemangioblastoma 29.6 SYP S100B
5 astrocytoma 10.9
6 pilocytic astrocytoma 10.8
7 rare tumor 10.8
8 hydrocephalus 10.7
9 congenital hydrocephalus 10.7
10 neurofibromatosis, type iv, of riccardi 10.5
11 obstructive hydrocephalus 10.5
12 glial tumor 10.5
13 benign ependymoma 10.4
14 noonan syndrome 1 10.4
15 ataxia and polyneuropathy, adult-onset 10.4
16 glioblastoma multiforme 10.4
17 oligodendroglioma 10.4
18 glioblastoma 10.4
19 papillary glioneuronal tumors 10.4
20 pseudo-turner syndrome 10.4
21 low-grade astrocytoma 10.4
22 neuronal tumor 10.4
23 mixed neuronal-glial tumor 10.4
24 neurofibromatosis, type i 10.2
25 alpha-thalassemia/mental retardation syndrome, x-linked 10.2
26 alpha-thalassemia 10.2
27 aceruloplasminemia 10.2
28 thalassemia 10.2
29 epilepsy 10.2
30 central nervous system cancer 10.2
31 fibrillary astrocytoma 10.2
32 glioma 10.2
33 precocious puberty 10.2
34 headache 10.2
35 traumatic brain injury 10.2
36 lipomatosis 10.2 PIK3CA FGFR1
37 hypogonadotropic hypogonadism 7 with or without anosmia 10.2 RNF216 FGFR1
38 fibrolamellar carcinoma 10.1 SYP FGFR1
39 hemimegalencephaly 10.1 PIK3CA MAP2
40 pilomyxoid astrocytoma 10.1 SYP FGFR1
41 brain stem glioma 10.1 PIK3CA FGFR1
42 papillary tumor of the pineal region 10.1 SYP MAP2
43 chiari malformation 10.0 SYP MAP2
44 cerebellum cancer 10.0 SYP MAP2
45 giant cell glioblastoma 10.0 SYP FGFR1
46 aggressive digital papillary adenocarcinoma 10.0 S100B PIK3CA
47 cerebellar liponeurocytoma 10.0 SYP MAP2
48 megalencephaly 10.0 S100B PIK3CA
49 optic nerve neoplasm 9.9 SYP MAP2
50 primary hepatic neuroendocrine carcinoma 9.9 SYP S100B

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

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, Brain, Cerebellum, Hypothalamus, Temporal Lobe, Thalamus

Publications for Rosette-Forming Glioneuronal Tumor

Articles related to Rosette-Forming Glioneuronal Tumor:

(show top 50) (show all 109)
# Title Authors PMID Year
1
Glioneuronal Tumor With Features of Ganglioglioma and Neurocytoma Arising in the Fourth Ventricle: A Report of 2 Unusual Cases and a Review of Infratentorial Gangliogliomas. 38
31361005 2019
2
Rosette-forming glioneuronal tumors share a distinct DNA methylation profile and mutations in FGFR1, with recurrent co-mutation of PIK3CA and NF1. 38
31250151 2019
3
A case of a rosette-forming glioneuronal tumor arising from the pons with disappearance of contrast enhancement. 38
31193570 2019
4
A case of recurrent epilepsy-associated rosette-forming glioneuronal tumor with anaplastic transformation in the absence of therapy. 38
31435988 2019
5
Rosette-forming and papillary glioneuronal tumors - A clinicopathological and molecular analysis. 38
30900987 2019
6
Rosette-forming glioneuronal tumor: an update. 38
31313067 2019
7
Cytological features of rosette-forming glioneuronal tumor of the fourth ventricle. 38
31298792 2019
8
Malignant Transformation of a Rosette-Forming Glioneuronal Tumor to Glioblastoma. 38
31203071 2019
9
An epilepsy-associated glioneuronal tumor with mixed morphology harboring FGFR1 mutation. 38
31218776 2019
10
Malignant Transformation of a Rosette-Forming Glioneuronal Tumor with IDH1 Mutation: A Case Report and Literature Review. 38
31218281 2019
11
Rosette forming glioneuronal tumor in the optico-chiasmatic region-a novel entity in a new location. 38
30790744 2019
12
MRI of intracranial intraventricular lesions. 38
30138862 2018
13
Rosette-Forming Glioneuronal Tumor of Spinal Cord. 38
30121405 2018
14
Magnetic resonance imaging findings of mixed neuronal-glial tumors with pathologic correlation: a review. 38
29987554 2018
15
Rosette-forming glioneural tumor in the tectal plate: Endoscopic diagnosis and conservative management. 38
29709373 2018
16
Rosette-forming glioneuronal tumor of the fourth ventricle. Two cases report and literature review. 38
29338911 2018
17
Mesial temporal lobe rosette-forming glioneuronal tumor : an unusual location for a rare tumor. 38
30178178 2018
18
Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background. 38
29250844 2018
19
Imaging features of rosette-forming glioneuronal tumours. 38
29146003 2018
20
Comprehensive genetic characterization of rosette-forming glioneuronal tumors: independent component analysis by tissue microdissection. 38
27893178 2018
21
Rosette-forming glioneuronal tumour of the fourth ventricle. Not always a foreseeable development. 38
30783393 2018
22
Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. 38
29312599 2017
23
Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle in Children: Case Report and Literature Review. 38
28826709 2017
24
Diagnosis and treatment for pure aqueductal tumor. 38
28690017 2017
25
Rosette-forming glioneuronal tumor of the fourth ventricle. 38
28879932 2017
26
Rosette-Forming Glioneuronal Tumor Arising from the Spinal Cord. 38
28847111 2017
27
Rosette-forming Glioneuronal Tumor: A Rare Posterior Fossa Tumor in an Adolescent. 38
28904578 2017
28
Retrospective Analysis of Molecular and Immunohistochemical Characterization of 381 Primary Brain Tumors. 38
28395087 2017
29
Rosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review. 38
27915062 2017
30
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. 38
28131206 2017
31
Case Report: A Rosette-forming Glioneuronal Tumor in the Tectal Plate in a Patient with Neurofibromatosis Type I. 38
27917325 2016
32
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. 38
27626068 2016
33
Rosette-forming glioneuronal tumor of the thalamus. 38
27049064 2016
34
Thalamic rosette-forming a glioneuronal tumor in an elderly patient: Case report and literature review. 38
26705911 2016
35
Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update. 38
26438552 2016
36
Imaging features of rosette-forming glioneuronal tumours (RGNTs): A Series of seven cases. 38
26017735 2016
37
SLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors. 38
26260115 2016
38
Rare glial tumors. 38
26948368 2016
39
Rosette-forming glioneuronal tumour of the fourth ventricle: case report and review of the literature. 38
27179225 2016
40
Uncommon pediatric tumors of the posterior fossa: pathologic and molecular features. 38
26351226 2015
41
Rosette-forming glioneuronal tumor originating in the hypothalamus. 38
26156565 2015
42
Rosette-forming Glioneuronal Tumor: Rare Case Presented with Spontaneous Disappearance of Contrast Enhancement. 38
28663967 2015
43
Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle: a highly aggressive case. 38
24984922 2015
44
Rosette-forming glioneuronal tumor with dissemination throughout the ventricular system: a case report. 38
25373141 2015
45
Glial tumors with neuronal differentiation. 38
25432191 2015
46
Molecular Profiling of a Rare Rosette-Forming Glioneuronal Tumor Arising in the Spinal Cord. 38
26371886 2015
47
Rare pathologies in the posterior third ventricular region in children: case series and review. 38
25591930 2015
48
Synchronous rosette-forming glioneuronal tumor and diffuse astrocytoma with molecular characterization: a case report. 38
24986181 2014
49
A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. 38
24735014 2014
50
Rosette forming glioneuronal tumor of the fourth ventricle in squash cytology smear. 38
25745291 2014

Variations for Rosette-Forming Glioneuronal Tumor

ClinVar genetic disease variations for Rosette-Forming Glioneuronal Tumor:

6
# Gene Variation Type Significance SNP ID GRCh37 Pos GRCh38 Pos
1 PIK3CA NM_006218.4(PIK3CA): c.3140A> G (p.His1047Arg) single nucleotide variant Pathogenic rs121913279 3:178952085-178952085 3:179234297-179234297
2 FGFR1 NM_023110.2(FGFR1): c.1638C> A (p.Asn546Lys) single nucleotide variant Pathogenic rs779707422 8:38274849-38274849 8:38417331-38417331
3 RNF216 NM_207111.4(RNF216): c.2468G> A (p.Arg823His) single nucleotide variant Uncertain significance rs761469213 7:5662795-5662795 7:5623164-5623164
4 PPP1R1A NM_006741.4(PPP1R1A): c.372_373delinsCA (p.Glu124_Ser125delinsAspThr) indel Uncertain significance rs1064792893 12:54975790-54975791 12:54582006-54582007

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 SYP RNF216

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

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

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 8.65 PIK3CA
2 1-phosphatidylinositol-3-kinase activity GO:0016303 8.32 PIK3CA

Sources for Rosette-Forming Glioneuronal Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
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44 MeSH
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49 NCI
50 NCIt
51 NDF-RT
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55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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