MCID: CRB002
MIFTS: 39

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 localized hypertrophic neuropathy. 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 Neuroscience. The drugs Etoposide and Carboplatin have been mentioned in the context of this disorder. Affiliated tissues include brain 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 206)
# Related Disease Score Top Affiliating Genes
1 neuroblastoma 28.0 CD44 ENO2 MYC MYCN SYP TP53
2 localized hypertrophic neuropathy 11.0 MUC1 S100B
3 sclerosing perineurioma 11.0 MUC1 S100B
4 reticular perineurioma 10.9 MUC1 S100B
5 hydromyelia 10.9 S100B VIM
6 non-functioning pituitary adenoma 10.9 MUC1 S100B
7 malignant peritoneal mesothelioma 10.9 MUC1 VIM
8 parachordoma 10.9 MUC1 VIM
9 desmoplastic infantile ganglioglioma 10.8 MYCN SYP
10 lower lip cancer 10.8 MSH2 MYCL
11 malignant mixed mullerian tumor 10.8 MUC1 VIM
12 nodular hidradenoma 10.8 MUC1 VIM
13 rhabdoid meningioma 10.8 SYP VIM
14 mixed type thymoma 10.8 S100B SYP
15 spindle cell rhabdomyosarcoma 10.8 MUC1 S100B
16 hypoganglionosis 10.8 S100B SYP
17 endometrial mucinous adenocarcinoma 10.8 MUC1 VIM
18 pilomatrix carcinoma 10.8 ENO2 MUC1
19 dysembryoplastic neuroepithelial tumor 10.7 S100B SYP
20 aggressive digital papillary adenocarcinoma 10.7 MUC1 S100B
21 biphasic synovial sarcoma 10.7 MUC1 VIM
22 ureter small cell carcinoma 10.7 ENO2 SYP
23 fibrous meningioma 10.7 MUC1 S100B VIM
24 malignant sertoli cell tumor 10.7 ENO2 SYP
25 primitive neuroectodermal tumor of the cervix uteri 10.7 ENO2 VIM
26 urinary bladder small cell neuroendocrine carcinoma 10.7 ENO2 SYP
27 syringocystadenoma papilliferum 10.7 MUC1 S100B VIM
28 eccrine porocarcinoma 10.7 MUC1 S100B VIM
29 ovarian small cell carcinoma 10.7 MUC1 SYP
30 myxopapillary ependymoma 10.7 MUC1 S100B VIM
31 monophasic synovial sarcoma 10.7 MUC1 S100B VIM
32 adenofibroma 10.7 MUC1 VIM
33 syringoma 10.7 MUC1 S100B VIM
34 pleomorphic liposarcoma 10.7 MUC1 S100B VIM
35 auditory system cancer 10.7 ENO2 SYP
36 perineurioma 10.7 MUC1 S100B VIM
37 spindle cell sarcoma 10.7 MUC1 S100B VIM
38 sensory organ benign neoplasm 10.7 ENO2 SYP
39 rosette-forming glioneuronal tumor 10.7 S100B SYP
40 actinic cheilitis 10.7 MSH2 TP53
41 dendritic cell tumor 10.7 MUC1 S100B VIM
42 gallbladder small cell carcinoma 10.7 ENO2 SYP
43 microcystic adenoma 10.7 ENO2 MUC1
44 hemangiopericytoma, malignant 10.7 MUC1 S100B VIM
45 polymorphous low-grade adenocarcinoma 10.7 MUC1 S100B VIM
46 primary hepatic neuroendocrine carcinoma 10.7 S100B SYP VIM
47 pancreatic somatostatinoma 10.7 ENO2 SYP
48 malignant fibroxanthoma 10.7 MUC1 S100B VIM
49 perivascular epithelioid cell tumor 10.6 MUC1 SYP VIM
50 adrenal neuroblastoma 10.6 ENO2 MYCN

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 MYCL MYCN IFNB1 SRC LYVE1 MSH2
2 behavior/neurological MP:0005386 10.09 CD44 ENO2 MYCN S100B SRC SYP
3 cellular MP:0005384 10.06 CD44 ENO2 MYCN LYVE1 SRC MSH2
4 immune system MP:0005387 10.02 MYCL IFNB1 SRC LYVE1 MSH2 MYC
5 nervous system MP:0003631 9.85 MYCN ENO2 IFNB1 SRC SYP MSH2
6 neoplasm MP:0002006 9.73 CD44 IFNB1 SRC MSH2 MYC TP53
7 normal MP:0002873 9.56 MYCL MYCN S100B IFNB1 SYP MYC
8 respiratory system MP:0005388 9.17 CD44 ENO2 MYCN SRC BSG VIM

Drugs & Therapeutics for Cerebral Primitive Neuroectodermal Tumor

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

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

Publications for Cerebral Primitive Neuroectodermal Tumor

Articles related to Cerebral Primitive Neuroectodermal Tumor:

(show all 14)
# 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
Centrosome amplification as a possible mechanism for numerical chromosome aberrations in cerebral primitive neuroectodermal tumors with TP53 mutations. ( 10072601 )
1998
7
p53 gene mutation in cerebral primitive neuroectodermal tumor in Taiwan. ( 8640736 )
1996
8
Human interferon-beta therapy for cerebral primitive neuroectodermal tumors--two case reports. ( 7753313 )
1995
9
"Undifferentiated" cerebral primitive neuroectodermal tumor in a young adult--case report. ( 7533271 )
1994
10
Expression of N-myc, c-myc and c-erbB-1 proto-oncogenes in cerebral primitive neuroectodermal tumors (PNET). ( 7889335 )
1994
11
Cerebral primitive neuroectodermal tumor in an adult, with spinal cord metastasis after 18-year dormancy. ( 2170592 )
1990
12
Congenital cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases. ( 2162735 )
1990
13
Ultrastructural study of nuclear evagination in neoplastic cells of a cerebral primitive neuroectodermal tumor. ( 2545397 )
1989
14
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 23)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.11 CD44 IFNB1 MUC1 MYC SRC TP53
2 12.42 ENO2 MYCN S100B SYP
3
Show member pathways
12.41 MSH2 MYC SRC TP53
4
Show member pathways
12.38 IFNB1 MYC SRC TP53
5
Show member pathways
12.33 MUC1 MYC SRC TP53
6 12.31 CD44 MYC TP53 VIM
7 12.1 CD44 MYC TP53 VIM
8 12.08 CD44 MYC SRC TP53
9 11.91 MYC SRC TP53
10 11.87 BSG CD44 MUC1
11 11.85 MYC TP53 VIM
12 11.84 MSH2 MYC SRC TP53
13 11.83 MYC SRC TP53
14 11.79 MYC SRC TP53
15 11.56 MYC SRC TP53
16 11.51 MYC MYCN TP53
17 11.51 MUC1 MYC TP53 VIM
18 11.29 CD44 MYC TP53
19 11.28 CD44 IFNB1 SRC TP53
20 11.21 MSH2 MYC TP53
21 11.02 CD44 LYVE1 MUC1
22 10.82 CD44 S100B SYP VIM
23 10.13 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 14)
# Name GO ID Score Top Affiliating Genes
1 cell proliferation GO:0008283 9.84 MYC S100B SRC TP53
2 positive regulation of gene expression GO:0010628 9.55 MYC MYCN SRC TP53 VIM
3 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.54 CD44 SRC TP53
4 regulation of neuronal synaptic plasticity GO:0048168 9.52 S100B SYP
5 positive regulation of smooth muscle cell migration GO:0014911 9.51 MYC SRC
6 DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediator GO:0006978 9.49 MUC1 TP53
7 hyaluronan catabolic process GO:0030214 9.48 CD44 LYVE1
8 determination of adult lifespan GO:0008340 9.46 MSH2 TP53
9 positive regulation of DNA biosynthetic process GO:2000573 9.43 MYC SRC
10 negative regulation of intrinsic apoptotic signaling pathway in response to DNA damage by p53 class mediator GO:1902166 9.4 CD44 MUC1
11 cytokine-mediated signaling pathway GO:0019221 9.35 IFNB1 MUC1 MYC TP53 VIM
12 negative regulation of telomerase activity GO:0051974 9.32 SRC TP53
13 regulation of inner ear auditory receptor cell differentiation GO:0045607 9.26 MYCL MYCN
14 cellular response to hypoxia GO:0071456 8.92 MYC S100B SRC TP53

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....