RGNT
MCID: RST024
MIFTS: 30

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 52 58 6
Rgnt 52 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Orphanet 58 ORPHA251975

Summaries for Rosette-Forming Glioneuronal Tumor

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 251975 Definition Rosette-forming glioneuronal tumor is a rare mixed neuronal-glial tumor characterized by the presence of uniform, rosette- (or pseudorosette-) forming neurocytes with an astrocytic component, together creating a biphasic pattern. It can present with signs of raised intracranial pressure (headache, vomiting, papilledema), hydrocephalus , seizures , ataxia and visual disturbances, or can be diagnosed incidentally in asymptomatic patients. The tumor usually arises in the midline, involving the fourth ventricle or the cerebellum. Visit the Orphanet disease page for more resources.

MalaCards based summary : Rosette-Forming Glioneuronal Tumor, also known as rgnt, is related to juvenile pilocytic astrocytoma and benign ependymoma. 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 Central carbon metabolism in cancer. Affiliated tissues include cerebellum, brain and spinal cord, 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 96)
# Related Disease Score Top Affiliating Genes
1 juvenile pilocytic astrocytoma 30.8 SYP S100B
2 benign ependymoma 30.2 SYP S100B
3 hydrocephalus 30.2 SYP S100B FGFR1
4 central neurocytoma 30.2 SYP MAP2
5 oligodendroglioma 29.8 SYP S100B FGFR1
6 dysembryoplastic neuroepithelial tumor 29.6 SYP S100B MAP2 FGFR1
7 hemangioblastoma 29.4 SYP S100B
8 astrocytoma 10.9
9 rare tumor 10.8
10 obstructive hydrocephalus 10.6
11 neurofibromatosis, type iv, of riccardi 10.5
12 glial tumor 10.5
13 cellular ependymoma 10.4
14 noonan syndrome 1 10.4
15 glioblastoma multiforme 10.4
16 fibrillary astrocytoma 10.4
17 papillary glioneuronal tumors 10.4
18 pseudo-turner syndrome 10.4
19 low-grade astrocytoma 10.4
20 neuronal tumor 10.4
21 mixed neuronal-glial tumor 10.4
22 neurofibromatosis, type i 10.2
23 alpha-thalassemia/mental retardation syndrome, x-linked 10.2
24 ataxia and polyneuropathy, adult-onset 10.2
25 alpha-thalassemia 10.2
26 aceruloplasminemia 10.2
27 meningioma, radiation-induced 10.2
28 meningioma, familial 10.2
29 thalassemia 10.2
30 spinal meningioma 10.2
31 epilepsy 10.2
32 central nervous system cancer 10.2
33 secretory meningioma 10.2
34 lymphoplasmacyte-rich meningioma 10.2
35 glioma 10.2
36 precocious puberty 10.2
37 headache 10.2
38 traumatic brain injury 10.2
39 lipomatosis 10.2 PIK3CA FGFR1
40 proteus syndrome 10.1 PIK3CA FGFR1
41 spinal cord oligodendroglioma 10.1 SYP FGFR1
42 nevus, epidermal 10.1 PIK3CA FGFR1
43 exophthalmos 10.1 SYP FGFR1
44 mixed cell type cancer 10.1 SYP PIK3CA
45 lung large cell carcinoma 10.1 SYP FGFR1
46 large cell carcinoma 10.1 SYP FGFR1
47 hemimegalencephaly 10.1 PIK3CA MAP2
48 brain stem glioma 10.0 PIK3CA FGFR1
49 fibrolamellar carcinoma 10.0 SYP FGFR1
50 papillary tumor of the pineal region 10.0 SYP MAP2

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 18)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.62 FGFR1 PPP1R1A S100B
2 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.62 PPP1R1A
3 Increased shRNA abundance (Z-score > 2) GR00366-A-125 9.62 FGFR1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.62 FGFR1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-132 9.62 PPP1R1A
6 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.62 PPP1R1A
7 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.62 PPP1R1A
8 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.62 FGFR1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-183 9.62 FGFR1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-26 9.62 FGFR1
11 Increased shRNA abundance (Z-score > 2) GR00366-A-31 9.62 PPP1R1A
12 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.62 FGFR1
13 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.62 FGFR1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-50 9.62 FGFR1
15 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.62 S100B
16 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.62 FGFR1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-83 9.62 FGFR1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.62 S100B

MGI Mouse Phenotypes related to Rosette-Forming Glioneuronal Tumor:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.17 FGFR1 MAP2 PIK3CA PPP1R1A RNF216 S100B

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:

40
Cerebellum, Brain, Spinal Cord, Pineal, Temporal Lobe, Thalamus, Hypothalamus

Publications for Rosette-Forming Glioneuronal Tumor

Articles related to Rosette-Forming Glioneuronal Tumor:

(show top 50) (show all 114)
# Title Authors PMID Year
1
A Multifocal Glioneuronal Tumor with RGNT-Like Morphology Occupying the Supratentorial Ventricular System and Infiltrating the Brain Parenchyma. 61
31606508 2020
2
Spinal rosette-forming glioneuronal tumor: A case report. 61
31804365 2019
3
Disseminated Rosette-Forming Glioneuronal Tumor with Spinal Drop Metastasis, a Uniquely Aggressive Presentation of Rare Tumor. 61
31442659 2019
4
A case of recurrent epilepsy-associated rosette-forming glioneuronal tumor with anaplastic transformation in the absence of therapy. 61
31435988 2019
5
Malignant Transformation of a Rosette-Forming Glioneuronal Tumor to Glioblastoma. 61
31203071 2019
6
Cytological features of rosette-forming glioneuronal tumor of the fourth ventricle. 61
31298792 2019
7
Rosette-forming Glioneuronal Tumor in the Optic Pathway of a Child. 61
31613844 2019
8
[Rosette-forming glioneuronal tumor in vermis: report of a case]. 61
31495094 2019
9
Rosette-forming glioneuronal tumors share a distinct DNA methylation profile and mutations in FGFR1, with recurrent co-mutation of PIK3CA and NF1. 61
31250151 2019
10
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. 61
31361005 2019
11
A case of a rosette-forming glioneuronal tumor arising from the pons with disappearance of contrast enhancement. 61
31193570 2019
12
Rosette-forming glioneuronal tumor: an update. 61
31313067 2019
13
Rosette-forming and papillary glioneuronal tumors - A clinicopathological and molecular analysis. 61
30900987 2019
14
An epilepsy-associated glioneuronal tumor with mixed morphology harboring FGFR1 mutation. 61
31218776 2019
15
Rosette-Forming Glioneuronal Tumor in Opticochiasmatic Region-Novel Entity in New Location. 61
30790744 2019
16
Malignant Transformation of a Rosette-Forming Glioneuronal Tumor with IDH1 Mutation: A Case Report and Literature Review. 61
31218281 2019
17
Rosette-Forming Glioneuronal Tumor of Spinal Cord. 61
30121405 2018
18
MRI of intracranial intraventricular lesions. 61
30138862 2018
19
Rosette-forming glioneural tumor in the tectal plate: Endoscopic diagnosis and conservative management. 61
29709373 2018
20
Magnetic resonance imaging findings of mixed neuronal-glial tumors with pathologic correlation: a review. 61
29987554 2018
21
Mesial temporal lobe rosette-forming glioneuronal tumor : an unusual location for a rare tumor. 61
30178178 2018
22
Rosette-forming glioneuronal tumor of the fourth ventricle. Two cases report and literature review. 61
29338911 2018
23
Intractable epilepsy due to a rosette-forming glioneuronal tumor with a dysembryoplastic neuroepithelial background. 61
29250844 2018
24
Imaging features of rosette-forming glioneuronal tumours. 61
29146003 2018
25
Comprehensive genetic characterization of rosette-forming glioneuronal tumors: independent component analysis by tissue microdissection. 61
27893178 2018
26
Rosette-forming glioneuronal tumour of the fourth ventricle. Not always a foreseeable development. 61
30783393 2018
27
Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. 61
29312599 2017
28
Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle in Children: Case Report and Literature Review. 61
28826709 2017
29
Diagnosis and treatment for pure aqueductal tumor. 61
28690017 2017
30
Rosette-forming glioneuronal tumor of the fourth ventricle. 61
28879932 2017
31
Rosette-Forming Glioneuronal Tumor Arising from the Spinal Cord. 61
28847111 2017
32
Rosette-forming Glioneuronal Tumor: A Rare Posterior Fossa Tumor in an Adolescent. 61
28904578 2017
33
Retrospective Analysis of Molecular and Immunohistochemical Characterization of 381 Primary Brain Tumors. 61
28395087 2017
34
PIK3CA mutation in a mixed dysembryoplastic neuroepithelial tumor and rosette forming glioneuronal tumor, a case report and literature review. 61
28131206 2017
35
Rosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review. 61
27915062 2017
36
Case Report: A Rosette-forming Glioneuronal Tumor in the Tectal Plate in a Patient with Neurofibromatosis Type I. 61
27917325 2016
37
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. 61
27626068 2016
38
Rosette-forming glioneuronal tumor of the thalamus. 61
27049064 2016
39
Thalamic rosette-forming a glioneuronal tumor in an elderly patient: Case report and literature review. 61
26705911 2016
40
Rosette-forming glioneuronal tumor outside the fourth ventricle: a case-based update. 61
26438552 2016
41
Imaging features of rosette-forming glioneuronal tumours (RGNTs): A Series of seven cases. 61
26017735 2016
42
Rare glial tumors. 61
26948368 2016
43
Rosette-forming glioneuronal tumour of the fourth ventricle: case report and review of the literature. 61
27179225 2016
44
SLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors. 61
26260115 2016
45
Rosette-forming glioneuronal tumor originating in the hypothalamus. 61
26156565 2015
46
Uncommon pediatric tumors of the posterior fossa: pathologic and molecular features. 61
26351226 2015
47
Rosette-forming Glioneuronal Tumor: Rare Case Presented with Spontaneous Disappearance of Contrast Enhancement. 61
28663967 2015
48
Rosette-forming glioneuronal tumour (RGNT) of the fourth ventricle: a highly aggressive case. 61
24984922 2015
49
Rosette-forming glioneuronal tumor with dissemination throughout the ventricular system: a case report. 61
25373141 2015
50
Glial tumors with neuronal differentiation. 61
25432191 2015

Variations for Rosette-Forming Glioneuronal Tumor

ClinVar genetic disease variations for Rosette-Forming Glioneuronal Tumor:

6 ‎‎ ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 FGFR1 NM_023110.2(FGFR1):c.1638C>A (p.Asn546Lys)SNV Pathogenic 224896 rs779707422 8:38274849-38274849 8:38417331-38417331
2 PIK3CA NM_006218.4(PIK3CA):c.3140A>G (p.His1047Arg)SNV Pathogenic 13652 rs121913279 3:178952085-178952085 3:179234297-179234297
3 RNF216 NM_207111.4(RNF216):c.2468G>A (p.Arg823His)SNV Uncertain significance 424631 rs761469213 7:5662795-5662795 7:5623164-5623164
4 PPP1R1A NM_006741.4(PPP1R1A):c.372_373delinsCA (p.Glu124_Ser125delinsAspThr)indel Uncertain significance 424630 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

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

# Super pathways Score Top Affiliating Genes
1 12.03 SYP S100B MAP2
2 11.21 PIK3CA FGFR1
3 10.95 PIK3CA FGFR1
4 10.83 PIK3CA FGFR1
5 10.82 SYP S100B MAP2 FGFR1
6 10.47 PIK3CA FGFR1

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-mediated signaling GO:0048015 9.16 PIK3CA FGFR1
2 cellular response to organic substance GO:0071310 8.96 SYP MAP2
3 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 tau protein binding GO:0048156 8.96 S100B MAP2
2 SH2 domain binding GO:0042169 8.62 SYP FGFR1

Sources for Rosette-Forming Glioneuronal Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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