PGNT
MCID: PPL060
MIFTS: 23

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 53
Papillary Glioneuronal Tumor 59 73
Pseudopapillary Neurocytoma with Glial Differentiation 59
Pseudopapillary Ganglioglioneurocytoma 59
Pgnt 59

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA251962
UMLS via Orphanet 74 C2985174
UMLS 73 C2985174

Summaries for Papillary Glioneuronal Tumors

NIH Rare Diseases : 53 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 encephalocraniocutaneous lipomatosis and hemangioma. An important gene associated with Papillary Glioneuronal Tumors is PRKCA (Protein Kinase C Alpha), and among its related pathways/superpathways is Phospholipase D signaling pathway. The drugs Vincristine and Carboplatin have been mentioned in the context of this disorder. Affiliated tissues include brain and pineal, and related phenotype is adipose tissue.

Related Diseases for Papillary Glioneuronal Tumors

Diseases related to Papillary Glioneuronal Tumors via text searches within MalaCards or GeneCards Suite gene sharing:

# Related Disease Score Top Affiliating Genes
1 encephalocraniocutaneous lipomatosis 10.0
2 hemangioma 10.0
3 siderosis 10.0
4 lipomatosis 10.0
5 cavernous hemangioma 10.0
6 ganglioglioma 10.0
7 rosette-forming glioneuronal tumor 10.0
8 superficial siderosis 10.0

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

Drugs for Papillary Glioneuronal Tumors (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
2
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
3
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760 44516822
4
Trametinib Approved Phase 2 871700-17-3 11707110
5 Antineoplastic Agents, Phytogenic Phase 2
6 Protein Kinase Inhibitors Phase 2
7 Antimitotic Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG and LGG Recruiting NCT02684058 Phase 2 dabrafenib;trametinib;Carboplatin with vincristine

Search 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:

41
Brain, Pineal

Publications for Papillary Glioneuronal Tumors

Articles related to Papillary Glioneuronal Tumors:

(show top 50) (show all 56)
# Title Authors Year
1
Cytopathologic features of papillary glioneuronal tumor. ( 29285892 )
2018
2
Papillary Glioneuronal Tumor with an Excessive Angiomatous Component in an Elderly Man. ( 29336376 )
2018
3
Papillary glioneuronal tumor. A case report. ( 29789203 )
2018
4
Enlargement of papillary glioneuronal tumor in an adult after a follow-up period of 10 years: a case report. ( 29942472 )
2018
5
Surgical resection and cellular proliferation index predict prognosis for patients with papillary glioneuronal tumor (PGNT): a systematic review and pooled analysis. ( 28823671 )
2017
6
Papillary glioneuronal tumors: A radiopathologic correlation. ( 29153366 )
2017
7
Clinicopathologic and neuroradiologic studies of papillary glioneuronal tumors. ( 26899971 )
2016
8
Papillary glioneuronal tumors: histological and molecular characteristics and diagnostic value of SLC44A1-PRKCA fusion. ( 26671581 )
2015
9
Papillary glioneuronal tumor: case report and review of literature. ( 26017104 )
2015
10
Recurrent papillary glioneuronal tumor presenting as a ganglioglioma with the BRAF V600E mutation. ( 26073619 )
2015
11
Clinical, radiological, and pathological features of 16 papillary glioneuronal tumors. ( 24553727 )
2014
12
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
13
Solid intraventricular papillary glioneuronal tumor: magnetic resonance imaging findings with histopathological correlation. ( 24314675 )
2014
14
FGFR1 N546K mutation in a case of papillary glioneuronal tumor (PGNT). ( 24777483 )
2014
15
Papillary glioneuronal tumor with an unusual bilateral intraventricular localization. ( 25250653 )
2014
16
Anaplastic papillary glioneuronal tumor with extraneural metastases. ( 24709681 )
2014
17
Neuroradiological features of papillary glioneuronal tumor: a study of 8 cases. ( 24879457 )
2014
18
Papillary glioneuronal tumor with a high proliferative component and minigemistocytes in a child. ( 24738632 )
2014
19
Malignant papillary glioneuronal tumor of the pineal gland: Case presentation and literature review of a distinct entity. ( 23826458 )
2013
20
Hemorrhagic papillary glioneuronal tumor mimicking cavernoma: two case reports. ( 22717600 )
2013
21
Identification of a novel, recurrent SLC44A1-PRKCA fusion in papillary glioneuronal tumor. ( 22725730 )
2013
22
Bilateral non-contiguous atypical papillary glioneuronal tumor: case report. ( 22385788 )
2012
23
Papillary glioneuronal tumor--a rare entity: report of four cases and brief review of literature. ( 22868530 )
2012
24
Papillary glioneuronal tumor: unexplored entity. ( 21842459 )
2012
25
Papillary glioneuronal tumors: a review of clinicopathologic and molecular genetic studies. ( 22020040 )
2011
26
Papillary glioneuronal tumor: radiological evidence of a newly established tumor entity. ( 20331495 )
2011
27
Papillary glioneuronal tumor present in a patient with encephalocraniocutaneous lipomatosis: case report. ( 20881536 )
2010
28
Papillary glioneuronal tumor of the fourth ventricle: case report and review of the literature. ( 20843438 )
2010
29
Papillary glioneuronal tumor of the frontal lobe. ( 20156688 )
2010
30
Papillary glioneuronal tumor--prognostic value of the extension of surgical resection. ( 19642508 )
2009
31
Aggressive variant of a papillary glioneuronal tumor. Report of 2 cases. ( 19119904 )
2009
32
Endoscopic diagnosis of a pineal papillary glioneuronal tumor with extensive ventricular involvement: case report with review of literature. ( 20139514 )
2009
33
Papillary glioneuronal tumor-evidence of stem cell origin with biphenotypic differentiation. ( 19404583 )
2009
34
Aggressive papillary glioneuronal tumor: case report and literature review. ( 18808063 )
2008
35
Papillary glioneuronal tumor--contribution to a new tumor entity and literature review. ( 18402385 )
2008
36
Genetic alterations in a papillary glioneuronal tumor. ( 18352813 )
2008
37
Papillary glioneuronal tumor: a case report and review of the literature. ( 18715860 )
2008
38
Papillary glioneuronal tumor: a new entity awaiting inclusion in WHO classification. ( 17288594 )
2007
39
Atypical papillary glioneuronal tumor. ( 17285229 )
2007
40
Papillary glioneuronal tumor. ( 18018482 )
2007
41
Papillary glioneuronal tumor: not always a benign tumor? ( 19157003 )
2007
42
Papillary glioneuronal tumor: a case report and review of the literature. ( 16784993 )
2006
43
Superficial siderosis due to papillary glioneuronal tumor. ( 17049863 )
2006
44
Papillary glioneuronal tumor with minigemistocytic components and increased proliferative activity. ( 16647962 )
2006
45
Papillary glioneuronal tumor: a clinicopathological and immunohistochemical study of two cases. ( 16771182 )
2006
46
Papillary glioneuronal tumor. ( 16413693 )
2006
47
Papillary glioneuronal tumor. Case report and literature review. ( 16703452 )
2006
48
Papillary glioneuronal tumor radiologically mimicking a cavernous hemangioma with hemorrhagic onset. ( 16771176 )
2006
49
A distinct pattern of Olig2-positive cellular distribution in papillary glioneuronal tumors: a manifestation of the oligodendroglial phenotype? ( 15906048 )
2005
50
Papillary glioneuronal tumor. ( 15972434 )
2005

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 PRKCA SLC44A1

GO Terms for Papillary Glioneuronal Tumors

Sources for Papillary Glioneuronal Tumors

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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