PNET
MCID: SPR008
MIFTS: 40

Supratentorial Primitive Neuroectodermal Tumor (PNET) malady

Rare diseases, Cancer diseases, Neuronal diseases categories

Summaries for Supratentorial Primitive Neuroectodermal Tumor

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34MalaCards
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MalaCards: Supratentorial Primitive Neuroectodermal Tumor, also known as peripheral neuroectodermal tumor, is related to medulloblastoma and merkel cell carcinoma. An important gene associated with Supratentorial Primitive Neuroectodermal Tumor is SLITRK6 (SLIT and NTRK-like family, member 6), and among its related pathways are SIDS Susceptibility Pathways and Neuroscience. The compounds 111in-pentetreotide and amine have been mentioned in the context of this disorder. Affiliated tissues include t cells, and related mouse phenotypes are nervous system and behavior/neurological.

Aliases & Classifications for Supratentorial Primitive Neuroectodermal Tumor

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9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 63UMLS, 41NCIt, 59SNOMED-CT
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Classifications:

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


Aliases & Descriptions:

supratentorial primitive neuroectodermal tumor 9 44 11 63
peripheral neuroectodermal tumor 44
neuroectodermal tumor, primitive 63
primative neuroectodermal tumor 44
supratentorial pnet 9
pnet 44


External Ids:

Disease Ontology9 DOID:4791
SNOMED-CT59 39781001
NCIt41 C6968

Related Diseases for Supratentorial Primitive Neuroectodermal Tumor

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18GeneCards, 19GeneDecks
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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 87)
idRelated DiseaseScoreTop Affiliating Genes
1medulloblastoma31.3SSTR2, SYP, S100B
2merkel cell carcinoma30.4SYP
3pilocytic astrocytoma30.3SYP, S100B
4neuroblastoma30.3SYP
5neuroendocrine tumor30.0SSTR2
6olfactory neuroblastoma29.9S100B, SYP
7primitive neuroectodermal tumor11.2
8sarcoma10.5
9supratentorial primitive neuroectodermal tumors, childhood10.5
10malignant peripheral neuroectodermal tumor of the cervix uteri10.4
11atypical teratoid rhabdoid tumor10.4
12epidermolysis bullosa simplex10.4
13childhood medulloblastoma10.4
14astrocytoma10.4
15epidermolysis bullosa10.4
16mediastinitis10.4
17pineoblastoma10.4
18pineoblastoma, childhood10.4
19peripheral primitive neuroectodermal tumor10.4
20malignant peripheral neuroectodermal tumor of the corpus uteri10.3
21rhabdoid tumor10.2
22pancreatitis10.2
23soft tissue peripheral neuroepithelioma10.2
24extraosseous ewing's sarcoma10.2
25prostatitis10.2
26vaginitis10.2
27subependymal giant cell astrocytoma10.1SYP
28dysembryoplastic neuroepithelial tumor10.1SYP
29glomus tumor10.1SYP
30cerebral primitive neuroectodermal tumor10.1
31neuronitis10.1
32cerebritis10.1
33teratoma10.1
34amyotrophic lateral sclerosis10.1SYP
35teratocarcinoma10.0SYP, ASCL1
36thyroid medullary carcinoma10.0ASCL1, SSTR2
37gastrinoma10.0SYP, SSTR2
38papillary ependymoma10.0S100B, SYP
39choroid plexus papilloma10.0SYP, S100B
40papillary carcinoma10.0SYP, SSTR2
41pineocytoma10.0S100B, SYP
42granular cell tumor10.0SYP, S100B
43ganglioneuroblastoma10.0SYP, S100B
44hemangioblastoma10.0SYP, S100B
45pancreatic cancer10.0SYP, SSTR2
46ganglioglioma10.0S100B, SYP
47ganglioneuroma10.0SYP, S100B
48neurofibroma10.0S100B, SYP
49insulinoma10.0SSTR2, DDC
50ependymoma10.0S100B, SYP

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



Diseases related to supratentorial primitive neuroectodermal tumor

Symptoms for Supratentorial Primitive Neuroectodermal Tumor

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Drugs & Therapeutics for Supratentorial Primitive Neuroectodermal Tumor

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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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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34MalaCards
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MalaCards organs/tissues related to Supratentorial Primitive Neuroectodermal Tumor:

34
T cells

Animal Models for Supratentorial Primitive Neuroectodermal Tumor or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Supratentorial Primitive Neuroectodermal Tumor:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00036317.9ASCL1, SSTR2, SYP, SLITRK6, DDC
2MP:00053867.3SLITRK6, S100B, SSTR2, ASCL1, DDC

Publications for Supratentorial Primitive Neuroectodermal Tumor

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53PubMed
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Articles related to Supratentorial Primitive Neuroectodermal Tumor:

(show all 43)
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
Severe allergic reactions to thiol-based cytoprotective agents mesna and amifostine in a child with a supratentorial primitive neuroectodermal tumor. (21646919)
2011
5
Treatment of a supratentorial primitive neuroectodermal tumor using magnetic resonance-guided laser-induced thermal therapy. (22044371)
2011
6
Late complete remission of supratentorial primitive neuroectodermal tumor (CNS-PNET) after multiple relapses. (21113945)
2011
7
Supratentorial primitive neuroectodermal tumors of the central nervous system in adults: molecular and histopathologic analysis of 12 cases. (21378543)
2011
8
Supratentorial primitive neuroectodermal tumor (PNET): an uncommon location. (22297899)
2011
9
Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas. (20515335)
2010
10
Supratentorial primitive neuroectodermal tumor metastasis to the abdomen via a ventriculoperitoneal shunt. (20526591)
2010
11
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
12
Successful radiation therapy for supratentorial primitive neuroectodermal tumor and epidermolysis bullosa simplex. (19760773)
2010
13
Lipomatous supratentorial primitive neuroectodermal tumor with glioblastomatous differentiation. (19118780)
2009
14
Frequent IDH1 mutations in supratentorial primitive neuroectodermal tumors (sPNET) of adults but not children. (19411854)
2009
15
Therapeutic Difficulty in a Case of Supratentorial Primitive Neuroectodermal Tumor Diagnosed during Pregnancy. (19242570)
2009
16
A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. (17713720)
2008
17
Different hypermethylation status of RASSF1A in medulloblastoma and supratentorial primitive neuroectodermal tumor]. (17374234)
2007
18
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
19
Supratentorial primitive neuroectodermal tumor during pregnancy. Case report. (17505462)
2007
20
High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors. (16783165)
2006
21
Supratentorial primitive neuroectodermal tumor (sPNET). (16612986)
2006
22
Long-term relapse-free survival with supratentorial primitive neuroectodermal tumor in an adult: a case report. (16528456)
2006
23
Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials. (16575007)
2006
24
Histone deacetylase inhibitors induce cell death in supratentorial primitive neuroectodermal tumor cells. (17016591)
2006
25
Molecular genetics of supratentorial primitive neuroectodermal tumors and pineoblastoma. (16398467)
2005
26
Epigenetic inactivation of DLC-1 in supratentorial primitive neuroectodermal tumor. (15712180)
2005
27
High-dose chemotherapy with autologous stem cell rescue for children with high risk and recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors. (15719272)
2005
28
Supratentorial primitive neuroectodermal tumor in an aged patient--case report--. (16247240)
2005
29
Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma. (15661719)
2005
30
Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging. (15131035)
2004
31
Results of a phase II upfront window of pharmacokinetically guided topotecan in high-risk medulloblastoma and supratentorial primitive neuroectodermal tumor. (15310781)
2004
32
Patterns of failure in relation to radiotherapy fields in supratentorial primitive neuroectodermal tumor. (15001261)
2004
33
Supratentorial primitive neuroectodermal tumor: report of a surgical case]. (15352632)
2004
34
Valproic acid induces differentiation of a supratentorial primitive neuroectodermal tumor. (15739631)
2004
35
PTEN and DMBT1 homozygous deletion and expression in medulloblastomas and supratentorial primitive neuroectodermal tumors. (15547761)
2004
36
Unusual aberration involving the short arm of chromosome 11 in an 8-month-old patient with a supratentorial primitive neuroectodermal tumor. (12606132)
2003
37
Mediastinal T-cell lymphoma in a boy 7 years after treatment of supratentorial primitive neuroectodermal tumor. (12902924)
2003
38
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
39
Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: a case report. (11349224)
2001
40
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
41
Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. (10447606)
1999
42
A continuous cell line (KK-2) from a supratentorial primitive neuroectodermal tumor. (1323907)
1992
43
Immunophenotype profile of childhood medulloblastomas and supratentorial primitive neuroectodermal tumors using 16 monoclonal antibodies. (2196109)
1990

Variations for Supratentorial Primitive Neuroectodermal Tumor

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Expression for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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2BioGPS, 16Gene Expression Omnibus DataSets
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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

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51PathCards, 39NCBI BioSystems Database, 5Cell 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
18.9SSTR2, ASCL1, DDC
28.6S100B, SYP, DDC

Compounds for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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46Novoseek, 25HMDB, 30IUPHAR, 12DrugBank, 52PharmGKB, 62Tocris Bioscience
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Compounds related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show all 32)
idCompoundScoreTop Affiliating Genes
1111in-pentetreotide469.7SYP, SSTR2
2amine469.7SYP, DDC
3spec-t469.6DDC, SSTR2
45-hydroxytryptamine469.6DDC, SYP
5s 100469.5S100B, SYP
6reticulin469.5S100B, SYP
7mucicarmine469.5S100B, SYP
8b 723469.5SYP, S100B
9hematoxylin469.4S100B, SYP
10dbc-amp469.4S100B, SYP
11agarose469.2S100B, DDC
12formaldehyde46 2510.2S100B, SYP
13histamine46 30 2511.1SYP, SSTR2, DDC
14l-amino acid469.0S100B, SYP, DDC
15catecholamine469.0DDC, SYP, S100B
16choline46 25 1211.0DDC, SYP, S100B
17gaba469.0DDC, SYP, S100B
18histidine469.0S100B, SYP, DDC
19norepinephrine46 25 1210.9DDC, SYP, S100B
20gtp46 309.9DDC, SYP, S100B
21acth468.9S100B, SYP, SSTR2
22ionomycin468.8S100B, SYP
23paraffin468.8S100B, SYP, SSTR2
24forskolin46 52 1210.8S100B, SYP, SSTR2
25glutamate468.8S100B, SYP, DDC
26progesterone46 30 62 25 1212.8SSTR2, SYP, S100B
27vegf468.7SSTR2, SYP, S100B
28retinoic acid46 259.6S100B, SYP, ASCL1
29estrogen468.5SSTR2, SYP, S100B
30glucose468.5S100B, SSTR2, DDC
31dopamine46 30 25 1210.9S100B, SYP, SSTR2, ASCL1, DDC
32tyrosine467.9DDC, ASCL1, SSTR2, SYP, S100B

GO Terms for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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17Gene Ontology
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Cellular components related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1neuronal cell bodyGO:0430258.7S100B, ASCL1, DDC

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

idNameGO IDScoreTop Affiliating Genes
1axonogenesisGO:0074099.2SLITRK6, S100B
2regulation of neuronal synaptic plasticityGO:0481688.8S100B, SYP

Products for genes affiliated with Supratentorial Primitive Neuroectodermal Tumor

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Sources for Supratentorial Primitive Neuroectodermal Tumor

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet