MCID: CRB002
MIFTS: 40

Cerebral Primitive Neuroectodermal Tumor

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Cerebral Primitive Neuroectodermal Tumor

MalaCards integrated aliases for Cerebral Primitive Neuroectodermal Tumor:

Name: Cerebral Primitive Neuroectodermal Tumor 12 56 15 74
Pnet of Cerebrum 12

Classifications:



External Ids:

Disease Ontology 12 DOID:7398
MeSH 45 D018242
NCIt 51 C4970
UMLS 74 C0751675

Summaries for Cerebral Primitive Neuroectodermal Tumor

MalaCards based summary : Cerebral Primitive Neuroectodermal Tumor, also known as pnet of cerebrum, is related to ewing's family of tumors and dysembryoplastic neuroepithelial tumor. An important gene associated with Cerebral Primitive Neuroectodermal Tumor is MYCN (MYCN Proto-Oncogene, BHLH Transcription Factor), and among its related pathways/superpathways are Cytokine Signaling in Immune system and Immune response IL-23 signaling pathway. The drugs Etoposide and Temozolomide have been mentioned in the context of this disorder. Affiliated tissues include brain, lung and spinal cord, and related phenotypes are Increased viability with nutlin-3 and hematopoietic system

Related Diseases for Cerebral Primitive Neuroectodermal Tumor

Diseases related to Cerebral Primitive Neuroectodermal Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 219)
# Related Disease Score Top Affiliating Genes
1 ewing's family of tumors 10.6
2 dysembryoplastic neuroepithelial tumor 10.5 S100B SYP
3 mixed type thymoma 10.5 S100B SYP
4 supratentorial primitive neuroectodermal tumor 10.5 S100B SYP
5 hypoganglionosis 10.5 S100B SYP
6 rosette-forming glioneuronal tumor 10.5 S100B SYP
7 ovarian small cell carcinoma 10.5 MUC1 SYP
8 spindle cell rhabdomyosarcoma 10.5 MUC1 S100B
9 medulloepithelioma 10.5 SYP VIM
10 aggressive digital papillary adenocarcinoma 10.5 MUC1 S100B
11 juvenile xanthogranuloma 10.5 S100B VIM
12 nodular hidradenoma 10.5 MUC1 VIM
13 parachordoma 10.5 MUC1 VIM
14 adenofibroma 10.5 MUC1 VIM
15 endometrial mucinous adenocarcinoma 10.5 MUC1 VIM
16 clear cell meningioma 10.5 MUC1 VIM
17 secretory meningioma 10.5 MUC1 VIM
18 biphasic synovial sarcoma 10.5 MUC1 VIM
19 large cell medulloblastoma 10.5 MYC SYP
20 venous hemangioma 10.5 MYC SYP
21 sensory organ benign neoplasm 10.4 ENO2 SYP
22 extrahepatic bile duct adenocarcinoma 10.4 ENO2 SYP
23 olfactory nerve neoplasm 10.4 ENO2 SYP
24 orbital cancer 10.4 ENO2 SYP
25 extraosseous chondrosarcoma 10.4 ENO2 SYP
26 subependymal glioma 10.4 ENO2 SYP
27 benign ependymoma 10.4 ENO2 SYP
28 middle cerebral artery infarction 10.4 ENO2 S100B
29 cauda equina neoplasm 10.4 ENO2 SYP
30 pineal gland cancer 10.4 ENO2 SYP
31 pulmonary large cell neuroendocrine carcinoma 10.4 ENO2 SYP
32 gallbladder small cell carcinoma 10.4 ENO2 SYP
33 endometrial small cell carcinoma 10.4 ENO2 SYP
34 small cell carcinoma of the bladder 10.4 ENO2 SYP
35 microcystic adenoma 10.4 ENO2 MUC1
36 localized hypertrophic neuropathy 10.4 MUC1 S100B
37 sclerosing perineurioma 10.4 MUC1 S100B
38 head injury 10.4 ENO2 S100B
39 epithelioid leiomyosarcoma 10.4 ENO2 VIM
40 glycogen-rich clear cell breast carcinoma 10.4 SYP TP53
41 olfactory groove meningioma 10.4 SYP TP53
42 sarcomatoid renal cell carcinoma 10.4 MUC1 TP53
43 hydromyelia 10.4 S100B VIM
44 desmoplastic infantile ganglioglioma 10.4 MYCN SYP
45 ependymoblastoma 10.4 TP53 VIM
46 reticular perineurioma 10.3 MUC1 S100B
47 megaesophagus 10.3 MYC TP53
48 tuberous sclerosis 10.3 S100B SYP VIM
49 b cell prolymphocytic leukemia 10.3 MYC TP53
50 carotid body cancer 10.3 ENO2 SYP

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



Diseases related to Cerebral Primitive Neuroectodermal Tumor

Symptoms & Phenotypes for Cerebral Primitive Neuroectodermal Tumor

GenomeRNAi Phenotypes related to Cerebral Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

27
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased viability with nutlin-3 GR00123-A 8.62 MYCL TP53

MGI Mouse Phenotypes related to Cerebral Primitive Neuroectodermal Tumor:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.11 BSG CD44 IFNB1 LYVE1 MSH2 MYC
2 behavior/neurological MP:0005386 10.09 CD44 ENO2 MYC MYCN S100B SRC
3 cellular MP:0005384 10.06 CD44 ENO2 LYVE1 MSH2 MYC MYCN
4 immune system MP:0005387 10.02 BSG CD44 IFNB1 LYVE1 MSH2 MYC
5 nervous system MP:0003631 9.85 CD44 ENO2 IFNB1 MSH2 MYC MYCN
6 neoplasm MP:0002006 9.73 CD44 IFNB1 MSH2 MYC SRC TP53
7 normal MP:0002873 9.56 CD44 IFNB1 MYC MYCL MYCN S100B
8 respiratory system MP:0005388 9.17 BSG CD44 ENO2 MYCN SRC TP53

Drugs & Therapeutics for Cerebral Primitive Neuroectodermal Tumor

Drugs for Cerebral Primitive Neuroectodermal Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etoposide Approved Phase 2 33419-42-0 36462
2
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
3
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
4
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
5 Immunologic Factors Phase 2
6 Antineoplastic Agents, Alkylating Phase 2
7 Immunosuppressive Agents Phase 2
8 Topoisomerase Inhibitors Phase 2
9 Etoposide phosphate Phase 2
10 Alkylating Agents Phase 2
11 Antineoplastic Agents, Phytogenic Phase 2
12 Vaccines Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Treatment of High-Risk Cerebral Primitive Neuroectodermal Tumors in Children Aged Over 5 Years Completed NCT00936156 Phase 2 Chemotherapy (carboplatin, etoposide, thiotepa)
2 HSV G207 Alone or With a Single Radiation Dose in Children With Progressive or Recurrent Supratentorial Brain Tumors Recruiting NCT02457845 Phase 1
3 HSV G207 in Children With Recurrent or Refractory Cerebellar Brain Tumors Not yet recruiting NCT03911388 Phase 1
4 Multicenter Register for Children and Young Adults With Intracranial Localized Medulloblastoma, CNS-PNET or Ependymoma Completed NCT02238899

Search NIH Clinical Center for Cerebral Primitive Neuroectodermal Tumor

Genetic Tests for Cerebral Primitive Neuroectodermal Tumor

Anatomical Context for Cerebral Primitive Neuroectodermal Tumor

MalaCards organs/tissues related to Cerebral Primitive Neuroectodermal Tumor:

42
Brain, Lung, Spinal Cord, Breast, Cervix, Pineal, Bone

Publications for Cerebral Primitive Neuroectodermal Tumor

Articles related to Cerebral Primitive Neuroectodermal Tumor:

(show all 11)
# Title Authors Year
1
Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation. ( 19390556 )
2009
2
Neonatal macrocephaly: cerebral primitive neuroectodermal tumor or neuroblastoma as an infrequent cause--a case report and review of the literature. ( 17909991 )
2007
3
Prenatal imaging of congenital cerebral primitive neuroectodermal tumor. ( 12711864 )
2003
4
Cerebral primitive neuroectodermal tumor in an adult male. A case report. ( 11127734 )
2000
5
A cerebral primitive neuroectodermal tumor in a squirrel monkey (Saimiri sciureus). ( 10431699 )
1999
6
p53 gene mutation in cerebral primitive neuroectodermal tumor in Taiwan. ( 8640736 )
1996
7
"Undifferentiated" cerebral primitive neuroectodermal tumor in a young adult--case report. ( 7533271 )
1994
8
Congenital cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases. ( 2162735 )
1990
9
Cerebral primitive neuroectodermal tumor in an adult, with spinal cord metastasis after 18-year dormancy. ( 2170592 )
1990
10
Ultrastructural study of nuclear evagination in neoplastic cells of a cerebral primitive neuroectodermal tumor. ( 2545397 )
1989
11
A midline cerebral primitive neuroectodermal tumor: immunohistochemical evidence for a germinal matrix origin. ( 2839313 )
1988

Variations for Cerebral Primitive Neuroectodermal Tumor

Expression for Cerebral Primitive Neuroectodermal Tumor

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

Pathways for Cerebral Primitive Neuroectodermal Tumor

Pathways related to Cerebral Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

(show all 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.06 CD44 IFNB1 MUC1 MYC SRC TP53
2
Show member pathways
12.4 MSH2 MYC SRC TP53
3 12.39 ENO2 MYCN S100B SYP
4
Show member pathways
12.32 MUC1 MYC SRC TP53
5 12.3 CD44 MYC TP53 VIM
6 12.08 CD44 MYC SRC TP53
7 11.9 MYC SRC TP53
8 11.86 BSG CD44 MUC1
9 11.84 MYC TP53 VIM
10 11.84 MSH2 MYC SRC TP53
11 11.82 MYC SRC TP53
12 11.77 MYC SRC TP53
13 11.55 MYC SRC TP53
14 11.51 MUC1 MYC TP53 VIM
15 11.49 MYC MYCN TP53
16 11.32 CD44 MYC TP53
17 11.28 CD44 IFNB1 SRC TP53
18 11.21 MSH2 MYC TP53
19 11.02 CD44 LYVE1 MUC1
20 10.82 CD44 S100B SYP VIM
21 10.16 MYC MYCN

GO Terms for Cerebral Primitive Neuroectodermal Tumor

Biological processes related to Cerebral Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cell proliferation GO:0008283 9.83 MYC S100B SRC TP53
2 cell cycle arrest GO:0007050 9.72 MSH2 MYC TP53
3 positive regulation of gene expression GO:0010628 9.55 MYC MYCN SRC TP53 VIM
4 regulation of neuronal synaptic plasticity GO:0048168 9.54 S100B SYP
5 positive regulation of smooth muscle cell migration GO:0014911 9.51 MYC SRC
6 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.5 CD44 SRC TP53
7 hyaluronan catabolic process GO:0030214 9.49 CD44 LYVE1
8 negative regulation of intrinsic apoptotic signaling pathway in response to DNA damage by p53 class mediator GO:1902166 9.48 CD44 MUC1
9 determination of adult lifespan GO:0008340 9.46 MSH2 TP53
10 positive regulation of DNA biosynthetic process GO:2000573 9.43 MYC SRC
11 DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediator GO:0006978 9.4 MUC1 TP53
12 negative regulation of telomerase activity GO:0051974 9.37 SRC TP53
13 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.26 MYCN TP53
14 cellular response to hypoxia GO:0071456 9.26 MYC S100B SRC TP53
15 cytokine-mediated signaling pathway GO:0019221 9.02 IFNB1 MUC1 MYC TP53 VIM

Sources for Cerebral Primitive Neuroectodermal Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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