PNET
MCID: SPR008
MIFTS: 42

Supratentorial Primitive Neuroectodermal Tumor (PNET) malady

Neuronal, Cancer categories

Summaries for Supratentorial Primitive Neuroectodermal Tumor

Sources:
33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
MalaCards: Supratentorial Primitive Neuroectodermal Tumor, also known as neuroectodermal tumor, primitive, is related to astrocytoma and rhabdoid tumor. An important gene associated with Supratentorial Primitive Neuroectodermal Tumor is PMS2 (PMS2 postmeiotic segregation increased 2 (S. cerevisiae)), and among its related pathways are Tyrosine metabolism p.1 (dopamine) and catecholamine biosynthesis. The compounds 111in-pentetreotide and s 100 have been mentioned in the context of this disorder. Affiliated tissues include brain and t cells, and related mouse phenotypes are tumorigenesis and no phenotypic analysis.

Aliases & Classifications for Supratentorial Primitive Neuroectodermal Tumor

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 61UMLS, 57SNOMED-CT, 40NCIt
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Neuronal


Aliases & Descriptions:

supratentorial primitive neuroectodermal tumor 8 43 10 61
neuroectodermal tumor, primitive 61
supratentorial pnet 8
pnet 43


External Ids:

Disease Ontology8 DOID:4791
SNOMED-CT57 39781001
NCIt40 C6968

Related Diseases for Supratentorial Primitive Neuroectodermal Tumor

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Supratentorial Primitive Neuroectodermal Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 102)
idRelated DiseaseScoreTop Affiliating Genes
1astrocytoma30.5IDH1, S100B, PDGFRA, DMBT1, SYP, RASSF1
2rhabdoid tumor30.3SYP, PTPRC
3anaplastic astrocytoma30.3PDGFRA, DMBT1
4pilocytic astrocytoma30.3SYP, S100B, IDH1
5cerebral primitive neuroectodermal tumor30.1MYCN
6olfactory neuroblastoma29.9S100B, SYP, CD99
7neuroepithelioma29.9MYB, SYP
8supratentorial primitive neuroectodermal tumors, childhood10.7
9epidermolysis bullosa simplex10.4
10intracranial primitive neuroectodermal tumor10.4
11atypical teratoid rhabdoid tumor10.4
12childhood medulloblastoma10.4
13pineoblastoma, childhood10.4
14embryonal tumor with multilayered rosettes10.2
15penis sarcoma10.1
16soft tissue sarcoma10.1
17teratoma10.1
18merkel cell carcinoma10.0SYP
19adenoid cystic carcinoma10.0MYB
20synovial sarcoma10.0CD99
21dysembryoplastic neuroepithelial tumor10.0SYP
22myelofibrosis10.0PDGFRA
23gangliocytoma10.0SYP
24angiomyolipoma10.0PTPRC
25pituitary adenoma10.0SSTR2, SYP
26hepatitis b10.0MYB
27thymoma10.0PTPRC
28down syndrome10.0S100B
29vitiligo10.0DDC
30brain cancer10.0PMS2, MYCN
31deficiency anemia10.0MYB
32papillary ependymoma10.0SYP, S100B
33rosai-dorfman disease10.0S100B
34endometrial carcinoma10.0RASSF1, MYCN
35papilloma10.0MYCN, RASSF1
36histiocytosis10.0S100B
37seminoma10.0RASSF1, PDGFRA
38choroid plexus papilloma10.0SYP, S100B
39meningioma10.0S100B, SYP, SSTR2
40hepatitis10.0DDC, MYCN, CD99
41neuroblastoma10.0SYP, MYCN, TH
42pineocytoma10.0SYP, S100B
43subependymal giant cell astrocytoma10.0SYP
44neurofibromatosis10.0PMS2, S100B, PDGFRA
45ganglioglioma10.0S100B, SYP, TH
46addison's disease10.0TH, DDC
47adenoma10.0PMS2, SYP, SSTR2, RASSF1
48osteosarcoma10.0PDGFRA, MYCN, TH
49bipolar disorder10.0DDC, TH
50autoimmune polyendocrinopathy syndrome , type i, with or without reversible metaphyseal dysplasia10.0TH, DDC

Graphical network of the top 20 diseases related to Supratentorial Primitive Neuroectodermal Tumor:



Diseases related to supratentorial primitive neuroectodermal tumor

Clinical Features for Supratentorial Primitive Neuroectodermal Tumor

Drugs & Therapeutics for Supratentorial Primitive Neuroectodermal Tumor

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Supratentorial Primitive Neuroectodermal Tumor

Drug clinical trials:

Search ClinicalTrials for Supratentorial Primitive Neuroectodermal Tumor

Search NIH Clinical Center for Supratentorial Primitive Neuroectodermal Tumor

Search CenterWatch for Supratentorial Primitive Neuroectodermal Tumor

Genetic Tests for Supratentorial Primitive Neuroectodermal Tumor

Anatomical Context for Supratentorial Primitive Neuroectodermal Tumor

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Supratentorial Primitive Neuroectodermal Tumor:

33
Brain, T cells

Animal Models for Supratentorial Primitive Neuroectodermal Tumor or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Supratentorial Primitive Neuroectodermal Tumor

Sources:
51PubMed
See all sources

Articles related to Supratentorial Primitive Neuroectodermal Tumor:

(show all 48)
idTitleAuthorsYear
1
A supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in a 42-year-old man: a case report and review of the literature. (23537064)
2013
2
Proton radiation therapy for pediatric medulloblastoma and supratentorial primitive neuroectodermal tumors: outcomes for very young children treated with upfront chemotherapy. (23790826)
2013
3
Supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. (23095172)
2012
4
Multiple CDK/CYCLIND genes are amplified in medulloblastoma and supratentorial primitive neuroectodermal brain tumor. (22682621)
2012
5
Small supratentorial, extraaxial primitive neuroectodermal tumor causing large intracerebral hematoma. (21701110)
2011
6
Severe allergic reactions to thiol-based cytoprotective agents mesna and amifostine in a child with a supratentorial primitive neuroectodermal tumor. (21646919)
2011
7
Treatment of a supratentorial primitive neuroectodermal tumor using magnetic resonance-guided laser-induced thermal therapy. (22044371)
2011
8
Late complete remission of supratentorial primitive neuroectodermal tumor (CNS-PNET) after multiple relapses. (21113945)
2011
9
Supratentorial primitive neuroectodermal tumors of the central nervous system in adults: molecular and histopathologic analysis of 12 cases. (21378543)
2011
10
Supratentorial primitive neuroectodermal tumor (PNET): an uncommon location. (22297899)
2011
11
Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas. (20515335)
2010
12
Supratentorial primitive neuroectodermal tumor metastasis to the abdomen via a ventriculoperitoneal shunt. (20526591)
2010
13
Reduced-dose craniospinal radiotherapy followed by high-dose chemotherapy and autologous stem cell rescue for children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuroectodermal tumor. (21120191)
2010
14
Successful radiation therapy for supratentorial primitive neuroectodermal tumor and epidermolysis bullosa simplex. (19760773)
2010
15
Lipomatous supratentorial primitive neuroectodermal tumor with glioblastomatous differentiation. (19118780)
2009
16
Frequent IDH1 mutations in supratentorial primitive neuroectodermal tumors (sPNET) of adults but not children. (19411854)
2009
17
Secondary supratentorial primitive neuroectodermal tumor following treatment of childhood osteosarcoma. (19434734)
2009
18
Therapeutic Difficulty in a Case of Supratentorial Primitive Neuroectodermal Tumor Diagnosed during Pregnancy. (19242570)
2009
19
A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. (17713720)
2008
20
Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor]. (17374234)
2007
21
High-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk or relapsed medulloblastoma or supratentorial primitive neuroectodermal tumor. (17066462)
2007
22
A case of congenital supratentorial tumor: atypical teratoid/rhabdoid tumor or primitive neuroectodermal tumor? (18021375)
2007
23
Supratentorial primitive neuroectodermal tumor during pregnancy. Case report. (17505462)
2007
24
High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors. (16783165)
2006
25
Supratentorial primitive neuroectodermal tumor (sPNET). (16612986)
2006
26
Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report. (16528456)
2006
27
Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials. (16575007)
2006
28
Histone deacetylase inhibitors induce cell death in supratentorial primitive neuroectodermal tumor cells. (17016591)
2006
29
Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma. (16398467)
2005
30
Epigenetic inactivation of DLC-1 in supratentorial primitive neuroectodermal tumor. (15712180)
2005
31
High-dose chemotherapy with autologous stem cell rescue for children with high risk and recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors. (15719272)
2005
32
Supratentorial primitive neuroectodermal tumor in an aged patient--case report--. (16247240)
2005
33
Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma. (15661719)
2005
34
Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging. (15131035)
2004
35
Results of a phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor. (15310781)
2004
36
Patterns of failure in relation to radiotherapy fields in supratentorial primitive neuroectodermal tumor. (15001261)
2004
37
Supratentorial primitive neuroectodermal tumor: report of a surgical case]. (15352632)
2004
38
Valproic acid induces differentiation of a supratentorial primitive neuroectodermal tumor. (15739631)
2004
39
PTEN and DMBT1 homozygous deletion and expression in medulloblastomas and supratentorial primitive neuroectodermal tumors. (15547761)
2004
40
Unusual aberration involving the short arm of chromosome 11 in an 8-month-old patient with a supratentorial primitive neuroectodermal tumor. (12606132)
2003
41
Mediastinal T-cell lymphoma in a boy 7 years after treatment of supratentorial primitive neuroectodermal tumor. (12902924)
2003
42
A novel case of a CAT to AAT transversion in codon 179 of the p53 gene in a supratentorial primitive neuroectodermal tumor harbored by a young girl. Case report and review of the literature. (12837982)
2003
43
Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: a case report. (11349224)
2001
44
Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study. (11352962)
2001
45
Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. (10447606)
1999
46
Chromosome arm 17p deletion analysis reveals molecular genetic heterogeneity in supratentorial and infratentorial primitive neuroectodermal tumors of the central nervous system. (9242214)
1997
47
A continuous cell line (KK-2) from a supratentorial primitive neuroectodermal tumor. (1323907)
1992
48
Immunophenotype profile of childhood medulloblastomas and supratentorial primitive neuroectodermal tumors using 16 monoclonal antibodies. (2196109)
1990

Genetic Variations for Supratentorial Primitive Neuroectodermal Tumor

Expression for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

Search GEO for disease gene expression data for Supratentorial Primitive Neuroectodermal Tumor.

Pathways for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

Sources:
12EMD Millipore, 38NCBI BioSystems Database, 54Reactome, 50PharmGKB, 4Cell Signaling Technology
See all sources

Pathways related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Tyrosine metabolism p.1 (dopamine)
Hide members
10.1TH, DDC
2
Hide members
10.1TH, DDC
310.1TH, DDC
49.8DDC, SSTR2, MYB, TH
59.6TH, MYCN, SYP, DDC, S100B

Compounds for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

Sources:
45Novoseek, 29IUPHAR, 24HMDB, 11DrugBank, 2BitterDB, 60Tocris Bioscience, 50PharmGKB
See all sources

Compounds related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show all 43)
idCompoundScoreTop Affiliating Genes
1111in-pentetreotide4510.5SYP, SSTR2
2s 1004510.5S100B, SYP
3benserazide45 2911.5DDC, TH
43-hydroxybenzylhydrazine45 2911.5DDC, TH
5l-dops4510.5TH, DDC
6l-dopa29 2411.5TH, DDC
7dopamine45 29 11 2413.4TH, MYCN, SSTR2
8vanillylmandelic acid45 2411.4TH, MYCN
9beta-phenylethylamine4510.4DDC, TH
10carbidopa45 2911.4DDC, TH
11homovanillic acid45 2411.4DDC, MYCN, TH
12b 7234510.4PTPRC, SYP, S100B
13mdma4510.3TH, DDC
14dbc-amp4510.3MYCN, SYP, S100B
15hematoxylin4510.3PTPRC, SYP, S100B
16cytosine45 2411.3SSTR2, MYB, RASSF1
17L-Phenylalanine11 2411.2DDC, TH
18mhpg4510.2DDC, TH
19l-amino acid4510.2S100B, DDC, SYP, TH
20acth4510.2S100B, SYP, SSTR2, PTPRC
21agarose4510.2PTPRC, MYCN, DDC, S100B
225-hydroxytryptophan4510.2DDC, TH
23formaldehyde45 2411.2S100B, SYP, TH, PTPRC
24reticulin4510.2SYP, S100B
25phosphotyrosine4510.1PTPRC, SSTR2, SYP, PDGFRA
26gaba4510.1S100B, DDC, SYP, TH
27methionine4510.1S100B, MYB, RASSF1, PTPRC
28catecholamine4510.1S100B, DDC, SYP, MYCN, TH
29chloramphenicol45 2 1112.1S100B, DDC, MYCN, MYB, TH
30choline45 11 2412.0S100B, DDC, SYP, MYB, TH
31glutamine4510.0IDH1, S100B, SYP, MYB, TH
32histamine45 29 2412.0DDC, SYP, SSTR2, TH, PTPRC
33norepinephrine45 11 2412.0S100B, DDC, SYP, TH
34progesterone45 60 29 11 2414.0S100B, SYP, SSTR2, MYB, PTPRC
35sodium nitroprusside459.9TH, MYB, S100B
36mucicarmine459.9S100B, SYP
37retinoic acid45 2410.7S100B, PDGFRA, SYP, MYCN, MYB, RASSF1
38cysteine459.7S100B, DMBT1, SYP, MYB, TH, PTPRC
39vegf459.6S100B, PDGFRA, SYP, SSTR2, MYCN, MYB
40serine459.5S100B, DMBT1, SYP, MYCN, MYB, RASSF1
41paraffin459.4PTPRC, PMS2, S100B, PDGFRA, SYP, SSTR2
42tyrosine459.4S100B, PDGFRA, DDC, SYP, SSTR2, MYCN
43calcium45 50 11 2412.2PTPRC, MYB, SSTR2, SYP, DMBT1, PDGFRA

GO Terms for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

Sources:
16Gene Ontology
See all sources

Biological processes related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1synaptic vesicle amine transportGO:01584210.2TH, DDC
2phytoalexin metabolic processGO:05231410.2TH, DDC
3isoquinoline alkaloid metabolic processGO:03307610.2DDC, TH
4response to pyrethroidGO:04668410.1TH, DDC
5multicellular organismal agingGO:01025910.1TH, DDC
6catecholamine biosynthetic processGO:04242310.0TH, DDC
7dopamine biosynthetic processGO:0424169.8TH, DDC
8axonogenesisGO:0074099.4SLITRK2, S100B, SLITRK4, SLITRK3, SLITRK5, SLITRK6

Products for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Supratentorial Primitive Neuroectodermal Tumor

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet