PGNT
MCID: PPL060
MIFTS: 21

Papillary Glioneuronal Tumors (PGNT)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Papillary Glioneuronal Tumors

MalaCards integrated aliases for Papillary Glioneuronal Tumors:

Name: Papillary Glioneuronal Tumors 20
Papillary Glioneuronal Tumor 58 70
Pseudopapillary Neurocytoma with Glial Differentiation 58
Pseudopapillary Ganglioglioneurocytoma 58
Pgnt 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

UMLS via Orphanet 71 C2985174
Orphanet 58 ORPHA251962
UMLS 70 C2985174

Summaries for Papillary Glioneuronal Tumors

GARD : 20 Papillary glioneuronal tumors (GNTs) are rare tumors. They may occur in any age (4-75 years), but are more common in young patients. Symptoms may include seizures, headaches, bleedings, and focal neurological deficits, impairing specific and small parts of the body. According to the 2016 WHO classification of CNS tumors the papillary GNTs are considered WHO grade I tumors. The grading of a tumor refers about how the tumor tissue look under a microscope, and indicates how quickly a tumor is likely to grow and spread. In grade 1 tumors, the tumor cells and the organization of the tumor tissue appear close to normal and they tend to grow and spread slowly. Papillary GNTs are usually located in the cerebral hemispheres (the two halves of the brain) next to the cerebral ventricles, the fluid-filled cavities of the brain. They are mixed tumors that contain both neural cells (neurons), which transmit the impulses in the brain, and glial cells (astrocytes) that help support the neurons. Treatment is done with surgery to remove the tumor and usually results in a good prognosis with long-term survival.

MalaCards based summary : Papillary Glioneuronal Tumors, also known as papillary glioneuronal tumor, is related to ganglioglioma and mixed neuronal-glial tumor. An important gene associated with Papillary Glioneuronal Tumors is OLIG2 (Oligodendrocyte Transcription Factor 2), and among its related pathways/superpathways is Phospholipase D signaling pathway. Affiliated tissues include brain and pineal, and related phenotype is adipose tissue.

Related Diseases for Papillary Glioneuronal Tumors

Graphical network of the top 20 diseases related to Papillary Glioneuronal Tumors:



Diseases related to Papillary Glioneuronal Tumors

Symptoms & Phenotypes for Papillary Glioneuronal Tumors

MGI Mouse Phenotypes related to Papillary Glioneuronal Tumors:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 adipose tissue MP:0005375 8.8 OLIG2 PRKCA SLC44A1

Drugs & Therapeutics for Papillary Glioneuronal Tumors

Search Clinical Trials , NIH Clinical Center for Papillary Glioneuronal Tumors

Genetic Tests for Papillary Glioneuronal Tumors

Anatomical Context for Papillary Glioneuronal Tumors

MalaCards organs/tissues related to Papillary Glioneuronal Tumors:

40
Brain, Pineal

Publications for Papillary Glioneuronal Tumors

Articles related to Papillary Glioneuronal Tumors:

(show top 50) (show all 88)
# Title Authors PMID Year
1
Rare glial tumors. 61 20
26948368 2016
2
Acute presentation of papillary glioneuronal tumor due to intra-tumoral hemorrhage in a toddler: an odd presentation of a rare pathology. 61
32781841 2020
3
A Case of Suprasellar Papillary Glioneuronal Tumor Mimicking Craniopharyngioma. 61
32695553 2020
4
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
5
Recurrent Papillary Glioneuronal Tumor. 61
31078809 2019
6
Rosette-forming and papillary glioneuronal tumors - A clinicopathological and molecular analysis. 61
30900987 2019
7
Papillary glioneuronal tumor. A case report. 61
29789203 2019
8
Papillary glioneuronal tumor (PGNT) exhibits a characteristic methylation profile and fusions involving PRKCA. 61
30759284 2019
9
Tectal region papillary neuroglial tumour: a case report. 61
30741021 2019
10
EWSR1-PATZ1 gene fusion may define a new glioneuronal tumor entity. 61
29679497 2019
11
Magnetic resonance imaging findings of mixed neuronal-glial tumors with pathologic correlation: a review. 61
29987554 2018
12
[BRAF V600E mutation and clinicopathologic characteristics in 250 cases of brain tumors associated with epilepsy]. 61
30220118 2018
13
Enlargement of papillary glioneuronal tumor in an adult after a follow-up period of 10 years: a case report. 61
29942472 2018
14
Cytopathologic features of papillary glioneuronal tumor. 61
29285892 2018
15
Papillary Glioneuronal Tumor with an Excessive Angiomatous Component in an Elderly Man. 61
29336376 2018
16
Papillary glioneuronal tumors: A radiopathologic correlation. 61
29153366 2017
17
Surgical Resection and Cellular Proliferation Index Predict Prognosis for Patients with Papillary Glioneuronal Tumor: Systematic Review and Pooled Analysis. 61
28823671 2017
18
Glioneuronal tumors of cerebral hemisphere in children: correlation of surgical resection with seizure outcomes and tumor recurrences. 61
27659827 2016
19
Treatment and Outcome in 65 Children with Optic Pathway Gliomas. 61
26898488 2016
20
Clinicopathologic and neuroradiologic studies of papillary glioneuronal tumors. 61
26899971 2016
21
SLC44A1-PRKCA fusion in papillary and rosette-forming glioneuronal tumors. 61
26260115 2016
22
Recurrent papillary glioneuronal tumor presenting as a ganglioglioma with the BRAF V600E mutation. 61
26073619 2015
23
Papillary glioneuronal tumors: histological and molecular characteristics and diagnostic value of SLC44A1-PRKCA fusion. 61
26671581 2015
24
Papillary glioneuronal tumor: case report and review of literature. 61
26017104 2015
25
Glial tumors with neuronal differentiation. 61
25432191 2015
26
Papillary glioneuronal tumor with an unusual bilateral intraventricular localization. 61
25250653 2015
27
Papillary glioneuronal tumor with a high proliferative component and minigemistocytes in a child. 61
24738632 2014
28
Neuroradiological features of papillary glioneuronal tumor: a study of 8 cases. 61
24879457 2014
29
Anaplastic papillary glioneuronal tumor with extraneural metastases. 61
24709681 2014
30
Clinical, radiological, and pathological features of 16 papillary glioneuronal tumors. 61
24553727 2014
31
Solid intraventricular papillary glioneuronal tumor: magnetic resonance imaging findings with histopathological correlation. 61
24314675 2014
32
FGFR1 N546K mutation in a case of papillary glioneuronal tumor (PGNT). 61
24777483 2014
33
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. 61
24991807 2014
34
Olig2-positive cells in glioneuronal tumors show both glial and neuronal characters: the implication of a common progenitor cell? 61
23025580 2013
35
Papillary glioneuronal tumour: a review of the literature with two illustrative cases. 61
23173837 2013
36
Identification of a novel, recurrent SLC44A1-PRKCA fusion in papillary glioneuronal tumor. 61
22725730 2013
37
Hemorrhagic papillary glioneuronal tumor mimicking cavernoma: two case reports. 61
22717600 2013
38
Malignant papillary glioneuronal tumor of the pineal gland: Case presentation and literature review of a distinct entity. 61
23826458 2013
39
Papillary glioneuronal tumor--a rare entity: report of four cases and brief review of literature. 61
22868530 2012
40
Papillary glioneuronal tumor: unexplored entity. 61
21842459 2012
41
Bilateral non-contiguous atypical papillary glioneuronal tumor: case report. 61
22385788 2012
42
Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute. 61
21410777 2012
43
Papillary glioneuronal tumors: a review of clinicopathologic and molecular genetic studies. 61
22020040 2011
44
Papillary glioneuronal tumor: radiological evidence of a newly established tumor entity. 61
20331495 2011
45
Papillary glioneuronal tumor present in a patient with encephalocraniocutaneous lipomatosis: case report. 61
20881536 2010
46
Papillary glioneuronal tumor of the fourth ventricle: case report and review of the literature. 61
20843438 2010
47
Papillary glioneuronal tumor of the frontal lobe. 61
20156688 2010
48
Endoscopic diagnosis of a pineal papillary glioneuronal tumor with extensive ventricular involvement: case report with review of literature. 61
20139514 2009
49
Papillary glioneuronal tumor-evidence of stem cell origin with biphenotypic differentiation. 61
19404583 2009
50
Papillary glioneuronal tumor--prognostic value of the extension of surgical resection. 61
19642508 2009

Variations for Papillary Glioneuronal Tumors

Expression for Papillary Glioneuronal Tumors

Search GEO for disease gene expression data for Papillary Glioneuronal Tumors.

Pathways for Papillary Glioneuronal Tumors

Pathways related to Papillary Glioneuronal Tumors according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.01 SLC44A1 PRKCA

GO Terms for Papillary Glioneuronal Tumors

Sources for Papillary Glioneuronal Tumors

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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