CPNET
MCID: CNT044
MIFTS: 28

Central Nervous System Primitive Neuroectodermal Neoplasm (CPNET)

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Central Nervous System Primitive Neuroectodermal Neoplasm

MalaCards integrated aliases for Central Nervous System Primitive Neuroectodermal Neoplasm:

Name: Central Nervous System Primitive Neuroectodermal Neoplasm 12 15
Cpnet 71

Classifications:



External Ids:

Disease Ontology 12 DOID:0060103
UMLS 71 C3887678

Summaries for Central Nervous System Primitive Neuroectodermal Neoplasm

Disease Ontology : 12 A central nervous system cancer of children and young adults that develops from neural crest cells located in cerebrum, has material basis in abnormally proliferating cells derives from neuroectoderm.

MalaCards based summary : Central Nervous System Primitive Neuroectodermal Neoplasm, also known as cpnet, is related to childhood central nervous system primitive neuroectodermal neoplasm and adult central nervous system primitive neuroectodermal neoplasm. An important gene associated with Central Nervous System Primitive Neuroectodermal Neoplasm is DICER1 (Dicer 1, Ribonuclease III), and among its related pathways/superpathways are Neuroscience and Embryonic and Induced Pluripotent Stem Cells and Lineage-specific Markers. Affiliated tissues include cerebrum, brain and ovary, and related phenotypes are Increased proliferation and muscle

Related Diseases for Central Nervous System Primitive Neuroectodermal Neoplasm

Diseases in the Central Nervous System Primitive Neuroectodermal Neoplasm family:

Adult Central Nervous System Primitive Neuroectodermal Neoplasm

Diseases related to Central Nervous System Primitive Neuroectodermal Neoplasm via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 235)
# Related Disease Score Top Affiliating Genes
1 childhood central nervous system primitive neuroectodermal neoplasm 34.6 PCDHGA3 OR4C12
2 adult central nervous system primitive neuroectodermal neoplasm 12.8
3 myoblastoma 10.5 SYP ENO2
4 malignant melanocytic neoplasm of the peripheral nerve sheath 10.5 SYP ENO2
5 anal neuroendocrine tumor 10.5 SYP ENO2
6 breast neuroendocrine neoplasm 10.5 SYP ENO2
7 sinonasal undifferentiated carcinoma 10.5 SYP ENO2
8 papillary tumor of the pineal region 10.5 SYP ENO2
9 small cell carcinoma of the bladder 10.5 SYP ENO2
10 auditory system benign neoplasm 10.5 SYP ENO2
11 appendix carcinoid tumor 10.5 SYP ENO2
12 auditory system cancer 10.5 SYP ENO2
13 cauda equina neoplasm 10.5 SYP ENO2
14 duodenal benign neoplasm 10.5 SYP ENO2
15 nodular prostate 10.5 SYP ENO2
16 myocardium cancer 10.5 SYP CD99
17 ampulla of vater neoplasm 10.5 SYP ENO2
18 urinary bladder small cell neuroendocrine carcinoma 10.5 SYP ENO2
19 ureter small cell carcinoma 10.5 SYP ENO2
20 cerebellar liponeurocytoma 10.5 SYP ENO2
21 middle ear adenoma 10.5 SYP ENO2
22 extraventricular neurocytoma 10.5 SYP GFAP
23 large cell carcinoma with rhabdoid phenotype 10.5 SMARCB1 ENO2
24 jejunal neoplasm 10.5 SYP ENO2
25 carotid body cancer 10.5 SYP ENO2
26 gallbladder small cell carcinoma 10.5 SYP ENO2
27 gastric small cell carcinoma 10.5 SYP ENO2
28 sensory organ benign neoplasm 10.5 SYP ENO2
29 lung combined type small cell carcinoma 10.5 SYP GFAP
30 pineal parenchymal tumor of intermediate differentiation 10.5 SYP ENO2
31 ovarian large-cell neuroendocrine carcinoma 10.5 SYP ENO2
32 gastric hemangioma 10.5 SYP ENO2
33 lung oat cell carcinoma 10.5 SYP ENO2
34 cutaneous ganglioneuroma 10.5 SYP ENO2
35 cervix small cell carcinoma 10.5 SYP ENO2
36 carcinoid tumors, intestinal 10.5 SYP ENO2
37 intracranial cysts 10.5 GFAP ENO2
38 primitive neuroectodermal tumor of the cervix uteri 10.5 GFAP ENO2
39 anaplastic ganglioglioma 10.4 NES GFAP
40 orbital cancer 10.4 SYP ENO2
41 central nervous system immature teratoma 10.4 NES GFAP
42 kidney rhabdoid cancer 10.4 SMARCB1 ENO2
43 tanycytic ependymoma 10.4 SYP GFAP
44 carcinoid syndrome 10.4 SYP ENO2
45 angiocentric glioma 10.4 SYP GFAP
46 chiari malformation 10.4 SYP GFAP
47 endometrial small cell carcinoma 10.4 SYP ENO2
48 gliofibroma 10.4 GFAP CD99
49 colon neuroendocrine neoplasm 10.4 SYP ENO2
50 ewing sarcoma of bone 10.4 ENO2 CD99

Graphical network of the top 20 diseases related to Central Nervous System Primitive Neuroectodermal Neoplasm:



Diseases related to Central Nervous System Primitive Neuroectodermal Neoplasm

Symptoms & Phenotypes for Central Nervous System Primitive Neuroectodermal Neoplasm

GenomeRNAi Phenotypes related to Central Nervous System Primitive Neuroectodermal Neoplasm according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased proliferation GR00094-A 8.62 SMARCB1 TP53

MGI Mouse Phenotypes related to Central Nervous System Primitive Neuroectodermal Neoplasm:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 muscle MP:0005369 9.17 DICER1 GFAP LIN28A MYCN SHH SMARCB1

Drugs & Therapeutics for Central Nervous System Primitive Neuroectodermal Neoplasm

Search Clinical Trials , NIH Clinical Center for Central Nervous System Primitive Neuroectodermal Neoplasm

Genetic Tests for Central Nervous System Primitive Neuroectodermal Neoplasm

Anatomical Context for Central Nervous System Primitive Neuroectodermal Neoplasm

The Foundational Model of Anatomy Ontology organs/tissues related to Central Nervous System Primitive Neuroectodermal Neoplasm:

19
Cerebrum

MalaCards organs/tissues related to Central Nervous System Primitive Neuroectodermal Neoplasm:

40
Brain, Ovary, Uterus

Publications for Central Nervous System Primitive Neuroectodermal Neoplasm

Articles related to Central Nervous System Primitive Neuroectodermal Neoplasm:

(show all 27)
# Title Authors PMID Year
1
[Clinical effect of surgery combined with chemotherapy and radiotherapy in children with central primitive neuroectodermal tumor and prognostic analysis]. 61
32571457 2020
2
Intracranial Ewing Sarcoma - A case report. 61
32547821 2020
3
ZBTB16: A new biomarker for primitive neuroectodermal tumor element / Ewing sarcoma. 61
31326195 2019
4
Ewing-Like Sarcoma: A Case of a Primary Cutaneous Primitive Neuroectodermal Tumor With Atypical Immunohistochemistry and Lack of Fusion Oncogene Detection. 61
30067549 2018
5
[Survival and prognostic factors analysis of primitive neuroectodermal tumor]. 61
30139033 2018
6
Cystic pancreatic neuroendocrine tumor. Pancreatic endocrine or neuroendocrine tumor. Endoscopic ultrasonography. 61
29313698 2018
7
Clinical management of a unique case of PNET of the uterus during pregnancy, and review of the literature. 61
29480840 2018
8
Primary intracranial dural-based Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking a meningioma: A rare tumor with review of literature. 61
28761507 2017
9
Comparison of Primary Spinal Central and Peripheral Primitive Neuroectodermal Tumors in Clinical and Imaging Characteristics and Long-Term Outcome. 61
26748171 2016
10
Cervicomedullary intramedullary peripheral primitive neuroectodermal tumor with intratumoral bleed: Report of one case and review of literature. 61
27217659 2016
11
The imaging findings of infratentorial primitive neuroectodermal tumour: A case report. 61
26493270 2015
12
Novel treatment of a central type, primitive neuroectodermal tumor of the ovary with postoperative pediatric medulloblastoma chemotherapy regimen: A case report and review of the literature. 61
26425724 2015
13
Kinetics of porphyrin fluorescence accumulation in pediatric brain tumor cells incubated in 5-aminolevulinic acid. 61
24777761 2014
14
Central primary primitive neuroectodermal tumor (cPNET) arising from an ovarian mature cystic teratoma in pregnancy: A case report and review of medical literature. 61
24371678 2013
15
Extending the phenotypes associated with DICER1 mutations. 61
21882293 2011
16
Primary peripheral PNET/Ewing's sarcoma arising in the meninges, confirmed by the presence of the rare translocation t(21;22) (q22;q12). 61
21284749 2011
17
The evaluation of CD99 immunoreactivity and EWS/FLI1 translocation by fluorescence in situ hybridization in central PNETs and Ewing's sarcoma family of tumors. 61
21267687 2011
18
KIT expression and methylation in medulloblastoma and PNET cell lines and tumors. 61
20853134 2011
19
CD133+ cells from medulloblastoma and PNET cell lines are more resistant to cyclopamine inhibition of the sonic hedgehog signaling pathway than CD133- cells. 61
20480407 2010
20
Primary Ewing sarcoma of the brain: a case report and literature review. 61
17525681 2007
21
Temozolomide in resistant or relapsed pediatric solid tumors. 61
16047361 2006
22
Primitive neuroectodermal tumours (PNETs) located in the spinal canal; the relevance of classification as central or peripheral PNET : case report of a primary spinal PNET occurrence with a critical literature review. 61
16292490 2006
23
[A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base]. 61
15112448 2004
24
Features of proliferation and in vitro drug resistance in central primitive neuro-ectodermal tumours. 61
12060344 2002
25
Alternative EWS-FLI1 fusion gene and MIC2 expression in peripheral and central primitive neuroectodermal tumors. 61
11304041 2001
26
High expression of somatostatin receptor subtype 2 (sst2) in medulloblastoma: implications for diagnosis and therapy. 61
10231868 1999
27
Vasoactive intestinal peptide (VIP) and VIP receptors: gene expression and growth modulation in medulloblastoma and other central primitive neuroectodermal tumors of childhood. 61
10188714 1999

Variations for Central Nervous System Primitive Neuroectodermal Neoplasm

Expression for Central Nervous System Primitive Neuroectodermal Neoplasm

Search GEO for disease gene expression data for Central Nervous System Primitive Neuroectodermal Neoplasm.

Pathways for Central Nervous System Primitive Neuroectodermal Neoplasm

Pathways related to Central Nervous System Primitive Neuroectodermal Neoplasm according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.79 SYP MYCN GFAP ENO2
2 11.46 SHH NES LIN28A
3 10.82 SYP SHH NES GFAP

GO Terms for Central Nervous System Primitive Neuroectodermal Neoplasm

Biological processes related to Central Nervous System Primitive Neuroectodermal Neoplasm according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.56 TP53 SHH MYCN DICER1
2 intermediate filament organization GO:0045109 9.37 SHH GFAP
3 miRNA metabolic process GO:0010586 9.32 LIN28A DICER1
4 pre-miRNA processing GO:0031054 9.16 LIN28A DICER1
5 Bergmann glial cell differentiation GO:0060020 8.96 SHH GFAP
6 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 8.8 TP53 MYCN LIN28A

Sources for Central Nervous System Primitive Neuroectodermal Neoplasm

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10 dbSNP
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17 EFO
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61 PubMed
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68 SNOMED-CT via HPO
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72 UMLS via Orphanet
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