PNET
MCID: SPR008
MIFTS: 46

Supratentorial Primitive Neuroectodermal Tumor (PNET) malady

Neuronal diseases, Cancer diseases categories

Summaries for Supratentorial Primitive Neuroectodermal Tumor

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MalaCards: Supratentorial Primitive Neuroectodermal Tumor, also known as peripheral neuroectodermal tumor, is related to medulloblastoma and sarcoma. 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

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8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 60UMLS, 56SNOMED-CT, 39NCIt
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Classifications:

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


Aliases & Descriptions:

supratentorial primitive neuroectodermal tumor 8 42 10 60
peripheral neuroectodermal tumor 42
neuroectodermal tumor, primitive 60
primative neuroectodermal tumor 42
supratentorial pnet 8
pnet 42


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Disease Ontology8 DOID:4791
SNOMED-CT56 39781001
NCIt39 C6968

Related Diseases for Supratentorial Primitive Neuroectodermal Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Supratentorial Primitive Neuroectodermal Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 121)
idRelated DiseaseScoreTop Affiliating Genes
1medulloblastoma31.3S100B, PDGFRA, DMBT1, SYP, SSTR2, MYCN
2sarcoma30.7S100B, PDGFRA, SSTR2, MYCN, CD99, TH
3osteosarcoma30.5PDGFRA, MYCN, TH
4astrocytoma30.5IDH1, S100B, PDGFRA, DMBT1, SYP, RASSF1
5peripheral primitive neuroectodermal tumor30.4S100B, PDGFRA, MYCN, CD99, TH, PTPRC
6merkel cell carcinoma30.1SYP
7cerebral primitive neuroectodermal tumor30.0MYCN
8neuroblastoma30.0SYP, MYCN, TH
9endometrial carcinoma29.8RASSF1, MYCN
10olfactory neuroblastoma29.8S100B, SYP, CD99
11neuroendocrine tumor29.8PDGFRA, SYP, SSTR2, MYCN, RASSF1
12retinoblastoma29.8SYP, MYCN, MYB, RASSF1, PTPRC
13primitive neuroectodermal tumor11.2
14supratentorial primitive neuroectodermal tumors, childhood10.5
15malignant peripheral neuroectodermal tumor of the cervix uteri10.4
16epidermolysis bullosa simplex10.3
17intracranial primitive neuroectodermal tumor10.3
18atypical teratoid rhabdoid tumor10.3
19childhood medulloblastoma10.3
20epidermolysis bullosa10.3
21mediastinitis10.3
22pineoblastoma10.3
23pineoblastoma, childhood10.3
24malignant peripheral neuroectodermal tumor of the corpus uteri10.3
25embryonal tumor with multilayered rosettes10.2
26pancreatitis10.1
27soft tissue peripheral neuroepithelioma10.1
28extraosseous ewing's sarcoma10.1
29localized ewing's sarcoma10.1
30prostatitis10.1
31vaginitis10.1
32vulvar sarcoma10.1
33cerebritis10.0
34neuronitis10.0
35penis sarcoma10.0
36malignant teratoma10.0
37adenoid cystic carcinoma10.0MYB
38synovial sarcoma10.0CD99
39dysembryoplastic neuroepithelial tumor10.0SYP
40myelofibrosis10.0PDGFRA
41gangliocytoma10.0SYP
42angiomyolipoma10.0PTPRC
43pituitary adenoma10.0SSTR2, SYP
44hepatitis b10.0MYB
45thymoma10.0PTPRC
46down syndrome10.0S100B
47vitiligo10.0DDC
48brain cancer10.0PMS2, MYCN
49deficiency anemia10.0MYB
50papillary ependymoma10.0SYP, S100B

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

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Drugs & Therapeutics for Supratentorial Primitive Neuroectodermal Tumor

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Genetic Tests for Supratentorial Primitive Neuroectodermal Tumor

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Anatomical Context for Supratentorial Primitive Neuroectodermal Tumor

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32MalaCards
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MalaCards organs/tissues related to Supratentorial Primitive Neuroectodermal Tumor:

32
Brain, T cells

Animal Models for Supratentorial Primitive Neuroectodermal Tumor or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Supratentorial Primitive Neuroectodermal Tumor:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.4PMS2, PDGFRA, DMBT1, RASSF1, TH, PTPRC
2MP:00030129.4PTPRC, PDGFRA, NEUROG2, SYP, MYCN, MYB
3MP:00028739.2S100B, PDGFRA, SYP, MYCN, MYB, RASSF1
4MP:00053859.1DDC, SSTR2, MYCN, MYB, RASSF1, TH
5MP:00036318.8PDGFRA, DDC, NEUROG2, SYP, SSTR2, MYCN
6MP:00053848.8PMS2, PDGFRA, DMBT1, DDC, NEUROG2, MYCN
7MP:00053868.8S100B, PDGFRA, DDC, NEUROG2, SSTR2, MYCN
8MP:00053768.5PMS2, IDH1, PDGFRA, DMBT1, DDC, SSTR2

Publications for Supratentorial Primitive Neuroectodermal Tumor

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50PubMed
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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

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Expression for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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

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Pathways for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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12EMD Millipore, 37NCBI BioSystems Database, 53Reactome, 49PharmGKB, 4Cell Signaling Technology
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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)
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10.1TH, DDC
2
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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

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44Novoseek, 28IUPHAR, 24HMDB, 11DrugBank, 2BitterDB, 59Tocris Bioscience, 49PharmGKB
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Compounds related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show all 43)
idCompoundScoreTop Affiliating Genes
1111in-pentetreotide4410.5SYP, SSTR2
2s 1004410.5S100B, SYP
3benserazide44 2811.5DDC, TH
43-hydroxybenzylhydrazine44 2811.5DDC, TH
5l-dops4410.5TH, DDC
6l-dopa28 2411.5TH, DDC
7dopamine44 28 11 2413.4TH, MYCN, SSTR2
8vanillylmandelic acid44 2411.4TH, MYCN
9beta-phenylethylamine4410.4DDC, TH
10carbidopa44 2811.4DDC, TH
11homovanillic acid44 2411.4DDC, MYCN, TH
12b 7234410.4PTPRC, SYP, S100B
13mdma4410.3TH, DDC
14dbc-amp4410.3MYCN, SYP, S100B
15hematoxylin4410.3PTPRC, SYP, S100B
16cytosine44 2411.3SSTR2, MYB, RASSF1
17L-Phenylalanine11 2411.2DDC, TH
18mhpg4410.2DDC, TH
19l-amino acid4410.2S100B, DDC, SYP, TH
20acth4410.2S100B, SYP, SSTR2, PTPRC
21agarose4410.2PTPRC, MYCN, DDC, S100B
225-hydroxytryptophan4410.2DDC, TH
23formaldehyde44 2411.2S100B, SYP, TH, PTPRC
24reticulin4410.2SYP, S100B
25phosphotyrosine4410.1PTPRC, SSTR2, SYP, PDGFRA
26gaba4410.1S100B, DDC, SYP, TH
27methionine4410.1S100B, MYB, RASSF1, PTPRC
28catecholamine4410.1S100B, DDC, SYP, MYCN, TH
29chloramphenicol44 2 1112.1S100B, DDC, MYCN, MYB, TH
30choline44 11 2412.0S100B, DDC, SYP, MYB, TH
31glutamine4410.0IDH1, S100B, SYP, MYB, TH
32histamine44 28 2412.0DDC, SYP, SSTR2, TH, PTPRC
33norepinephrine44 11 2412.0S100B, DDC, SYP, TH
34progesterone44 59 28 11 2414.0S100B, SYP, SSTR2, MYB, PTPRC
35sodium nitroprusside449.9TH, MYB, S100B
36mucicarmine449.9S100B, SYP
37retinoic acid44 2410.7S100B, PDGFRA, SYP, MYCN, MYB, RASSF1
38cysteine449.7S100B, DMBT1, SYP, MYB, TH, PTPRC
39vegf449.6S100B, PDGFRA, SYP, SSTR2, MYCN, MYB
40serine449.5S100B, DMBT1, SYP, MYCN, MYB, RASSF1
41paraffin449.4PTPRC, PMS2, S100B, PDGFRA, SYP, SSTR2
42tyrosine449.4S100B, PDGFRA, DDC, SYP, SSTR2, MYCN
43calcium44 49 11 2412.2PTPRC, MYB, SSTR2, SYP, DMBT1, PDGFRA

GO Terms for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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16Gene Ontology
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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

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Sources for Supratentorial Primitive Neuroectodermal Tumor

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3CDC
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14FMA
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24HMDB
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26ICD10 via Orphanet
27ICD9CM
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40NDF-RT
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