MCID: CRB002
MIFTS: 38

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 55 15 73
Pnet of Cerebrum 12

Classifications:



External Ids:

Disease Ontology 12 DOID:7398
MeSH 44 D018242
NCIt 50 C4970
UMLS 73 C0751675

Summaries for Cerebral Primitive Neuroectodermal Tumor

MalaCards based summary : Cerebral Primitive Neuroectodermal Tumor, also known as pnet of cerebrum, is related to neuroblastoma and ewing's family of tumors. 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 Thiotepa and Temozolomide have been mentioned in the context of this disorder. Affiliated tissues include brain, lung and bone, 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 222)
# Related Disease Score Top Affiliating Genes
1 neuroblastoma 29.5 CD44 ENO2 MYC MYCN SYP TP53
2 ewing's family of tumors 10.6
3 medulloepithelioma 10.3 SYP VIM
4 nodular hidradenoma 10.3 MUC1 VIM
5 parachordoma 10.3 MUC1 VIM
6 adenofibroma 10.3 MUC1 VIM
7 endometrial mucinous adenocarcinoma 10.3 MUC1 VIM
8 clear cell meningioma 10.3 MUC1 VIM
9 secretory meningioma 10.3 MUC1 VIM
10 bile duct adenoma 10.3 MUC1 VIM
11 biphasic synovial sarcoma 10.3 MUC1 VIM
12 juvenile xanthogranuloma 10.3 S100B VIM
13 ovarian small cell carcinoma 10.3 MUC1 SYP
14 dysembryoplastic neuroepithelial tumor 10.3 S100B SYP
15 mixed type thymoma 10.3 S100B SYP
16 supratentorial primitive neuroectodermal tumor 10.3 S100B SYP
17 spindle cell rhabdomyosarcoma 10.3 MUC1 S100B
18 hypoganglionosis 10.3 S100B SYP
19 rosette-forming glioneuronal tumor 10.3 S100B SYP
20 aggressive digital papillary adenocarcinoma 10.3 MUC1 S100B
21 large cell medulloblastoma 10.3 MYC SYP
22 venous hemangioma 10.3 MYC SYP
23 sensory organ benign neoplasm 10.3 ENO2 SYP
24 epithelioid leiomyosarcoma 10.3 ENO2 VIM
25 extrahepatic bile duct adenocarcinoma 10.3 ENO2 SYP
26 olfactory nerve neoplasm 10.3 ENO2 SYP
27 orbital cancer 10.3 ENO2 SYP
28 extraosseous chondrosarcoma 10.3 ENO2 SYP
29 subependymal glioma 10.3 ENO2 SYP
30 benign ependymoma 10.3 ENO2 SYP
31 cauda equina neoplasm 10.3 ENO2 SYP
32 microcystic adenoma 10.3 ENO2 MUC1
33 pineal gland cancer 10.3 ENO2 SYP
34 pulmonary large cell neuroendocrine carcinoma 10.3 ENO2 SYP
35 endometrial small cell carcinoma 10.3 ENO2 SYP
36 small cell carcinoma of the bladder 10.3 ENO2 SYP
37 middle cerebral artery infarction 10.3 ENO2 S100B
38 localized hypertrophic neuropathy 10.2 MUC1 S100B
39 ependymoblastoma 10.2 TP53 VIM
40 sclerosing perineurioma 10.2 MUC1 S100B
41 head injury 10.2 ENO2 S100B
42 glycogen-rich clear cell breast carcinoma 10.2 SYP TP53
43 hydromyelia 10.2 S100B VIM
44 olfactory groove meningioma 10.2 SYP TP53
45 malignant giant cell tumor 10.2 S100B TP53
46 desmoplastic infantile ganglioglioma 10.2 MYCN SYP
47 epulis 10.2 ENO2 VIM
48 megaesophagus 10.2 MYC TP53
49 tuberous sclerosis 10.2 S100B SYP VIM
50 b cell prolymphocytic leukemia 10.2 MYC TP53

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:

26
# 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:

46
# 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
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
2
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
3
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
4
Etoposide Approved Phase 2 33419-42-0 36462
5 Topoisomerase Inhibitors Phase 2
6 Etoposide phosphate Phase 2
7 Alkylating Agents Phase 2
8 Immunosuppressive Agents Phase 2
9 Antineoplastic Agents, Alkylating Phase 2
10 Immunologic Factors 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 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:

41
Brain, Lung, Bone, Breast, Pituitary, Cervix, Pineal

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
Cerebral primitive neuroectodermal tumor in an adult, with spinal cord metastasis after 18-year dormancy. ( 2170592 )
1990
9
Congenital cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases. ( 2162735 )
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 22)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.09 CD44 IFNB1 MUC1 MYC SRC TP53
2
Show member pathways
12.41 MSH2 MYC SRC TP53
3 12.4 ENO2 MYCN S100B SYP
4
Show member pathways
12.33 MUC1 MYC SRC TP53
5 12.31 CD44 MYC TP53 VIM
6 12.1 CD44 MYC SRC TP53
7 11.9 MYC SRC TP53
8 11.89 MSH2 MYC SRC TP53
9 11.86 BSG CD44 MUC1
10 11.85 MYC TP53 VIM
11 11.82 MYC SRC TP53
12 11.78 MYC SRC TP53
13 11.61 MUC1 MYC TP53 VIM
14 11.56 MYC SRC TP53
15 11.51 MYC MYCN TP53
16 11.48 CD44 IFNB1 SRC TP53
17 11.34 CD44 MYC TP53
18 11.32 CD44 IFNB1 MYC TP53 VIM
19 11.24 MSH2 MYC TP53
20 11.15 CD44 S100B SYP VIM
21 11.09 CD44 LYVE1 MUC1
22 10.18 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 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
Content
Loading form....