CPNET
MCID: PRM027
MIFTS: 50

Primitive Neuroectodermal Tumor (CPNET) malady

Summaries for Primitive Neuroectodermal Tumor

Sources:
33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
MalaCards: Primitive Neuroectodermal Tumor, also known as localized primitive neuroectodermal tumor, is related to supratentorial primitive neuroectodermal tumor and cerebral primitive neuroectodermal tumor. An important gene associated with Primitive Neuroectodermal Tumor is PMS2 (PMS2 postmeiotic segregation increased 2 (S. cerevisiae)), and among its related pathways are Transcriptional misregulation in cancer and Neuroscience. The compounds paraffin and retinoic acid have been mentioned in the context of this disorder. Related mouse phenotypes are normal and immune system.

Aliases & Classifications for Primitive Neuroectodermal Tumor

Sources:
8Disease Ontology, 61UMLS, 57SNOMED-CT, 35MeSH, 40NCIt
See all sources

Aliases & Descriptions:

primitive neuroectodermal tumor 8
localized primitive neuroectodermal tumor 8 61
extraosseous ewings sarcoma-primitive neuroepithelial tumor 61
primitive neuroectodermal tumour 8
neuroectodermal tumor, primitive 61
cns pnet 8
cpnet 8


External Ids:

Disease Ontology8 DOID:168
MeSH35 D018242
SNOMED-CT57 39781001, 189925001
NCIt40 C5398

Related Diseases for Primitive Neuroectodermal Tumor

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 240)
idRelated DiseaseScoreTop Affiliating Genes
1supratentorial primitive neuroectodermal tumor31.6SLITRK4, SLITRK2
2cerebral primitive neuroectodermal tumor31.3MYCN, MYC
3rhabdoid tumor31.1VIM, SYP, PTPRC
4neuroepithelioma31.0EWSR1, SYP, MYC
5teratoma30.9CD99, GFAP
6childhood medulloblastoma30.9MYC, MYCN
7extraskeletal ewing sarcoma30.8EWSR1, CD99
8astrocytoma30.7VIM, IFNB1, S100B, SYP, RASSF1, GFAP
9ependymoma30.7VIM, S100B, SYP, RASSF1, GFAP
10ganglioglioma30.5S100B, SYP, GFAP
11synovial sarcoma30.5VIM, S100B, MYCN, RASSF1
12olfactory neuroblastoma30.5S100B, SYP, CD99
13gastrointestinal stromal tumor30.5S100B, VIM
14acute leukemia30.5CSF3, EWSR1, PTPRC
15ganglioneuroma30.3S100B, SYP, MYCN, RASSF1, GFAP
16medulloepithelioma30.3SYP
17wilms tumor30.3PMS2, VIM, EWSR1, MYCN, RASSF1, PTPRC
18squamous cell carcinoma30.3VIM, SYP, GRP, MYC, MYCN, ETV7
19paraganglioma30.3S100B, SYP, GRP, SSTR2, GFAP
20pheochromocytoma30.3SYP, SSTR2, MYCN
21merkel cell carcinoma30.3SYP
22gliosarcoma30.3GFAP
23angiosarcoma30.3VIM
24bladder carcinoma30.3TP53, RASSF1
25clear cell sarcoma30.3S100B, EWSR1
26fibrous histiocytoma30.3VIM, S100B
27extraosseous ewings sarcoma-primitive neuroepithelial tumor10.8
28intracranial primitive neuroectodermal tumor10.8
29bone ewing's sarcoma10.7
30n syndrome10.7
31atypical teratoid rhabdoid tumor10.6
32soft tissue sarcoma10.6
33supratentorial primitive neuroectodermal tumors, childhood10.6
34cerebellopontine angle primitive neuroectodermal10.5
35unilateral retinoblastoma10.5
36localized peripheral primitive neuroectodermal tumor10.5
37vagina sarcoma10.5
38vulvar sarcoma10.5
39trilateral retinoblastoma10.4
40ovarian cystic teratoma10.4
41pediatric ependymoma10.4
42penis sarcoma10.4
43cauda equina syndrome10.4
44brain medulloblastoma10.4
45pineoblastoma, childhood10.4
46desmoplastic small round cell tumor10.4
47heart tumor10.4
48nonseminomatous germ cell tumor10.4
49germ cell tumors10.4
50ectomesenchymoma10.2

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



Diseases related to primitive neuroectodermal tumor

Clinical Features for Primitive Neuroectodermal Tumor

Drugs & Therapeutics for Primitive Neuroectodermal Tumor

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

Approved drugs:

Search CenterWatch for Primitive Neuroectodermal Tumor

Drug clinical trials:

Search ClinicalTrials for Primitive Neuroectodermal Tumor

Search NIH Clinical Center for Primitive Neuroectodermal Tumor

Search CenterWatch for Primitive Neuroectodermal Tumor

Genetic Tests for Primitive Neuroectodermal Tumor

Anatomical Context for Primitive Neuroectodermal Tumor

Animal Models for Primitive Neuroectodermal Tumor or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Primitive Neuroectodermal Tumor:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000287310.8TP53, IFNB1, S100B, EWSR1, SYP, MYC
2MP:000538710.7PMS2, VIM, IFNB1, CSF3, EWSR1, MYC
3MP:000200610.7PMS2, IFNB1, EWSR1, RASSF1, PTPRC
4MP:000538110.6PMS2, SSTR2, MYC, MYCN, GFAP, PTPRC
5MP:000363110.6PTPRC, GFAP, MYC, SSTR2, SYP
6MP:000538410.5PMS2, VIM, IFNB1, EWSR1, MYC, MYCN
7MP:000537610.3PMS2, IFNB1, MYCN, GFAP, PTPRC

Publications for Primitive Neuroectodermal Tumor

Sources:
51PubMed
See all sources

Articles related to Primitive Neuroectodermal Tumor:

(show top 50)    (show all 653)
idTitleAuthorsYear
1
Ewing sarcoma/primitive neuroectodermal tumor arising in the adrenal gland. (23714257)
2013
2
Adult soft tissue Ewing's sarcoma/primitive neuroectodermal tumor. (23815991)
2013
3
Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: Report of a case diagnosed by fine needle aspiration cytology and confirmed by immunocytochemistry and RT-PCR along with review of literature. (21548122)
2012
4
Connexin 43 is a potential prognostic biomarker for ewing sarcoma/primitive neuroectodermal tumor. (21647307)
2011
5
Primitive neuroectodermal tumor arising in the abdominopelvic region: CT features and pathology characteristics. (20959975)
2011
6
Rare Primitive Neuroectodermal Tumor (PNET) of Liver in a Young Woman. (22043328)
2011
7
Supratentorial primitive neuroectodermal tumor (PNET): an uncommon location. (22297899)
2011
8
An unusual primitive neuroectodermal tumor in the thoracic epidural space. (20036552)
2010
9
Newly described translocation (18;19)(q23;q13.2) in abdominal wall soft-tissue tumor resembling Ewing sarcoma/primitive neuroectodermal tumor. (20633761)
2010
10
Primary cutaneous ewing's sarcoma/primitive neuroectodermal tumor: report of the first case diagnosed on aspiration cytology. (20391978)
2010
11
Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases. (18452568)
2009
12
Cerebral primitive neuroectodermal tumor in an adult with a heterozygous MSH2 mutation. (19390556)
2009
13
Primitive neuroectodermal tumor of the kidney in a child. (19863203)
2009
14
Paraplegia after chest wall resection for primitive neuroectodermal tumor. (19349002)
2009
15
c-Myc and beta-catenin cooperate with loss of p53 to generate multiple members of the primitive neuroectodermal tumor family in mice. (18372915)
2008
16
Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. (17366107)
2007
17
Therapy-related myelodysplasia and acute myeloid leukemia after Ewing sarcoma and primitive neuroectodermal tumor of bone: A report from the Children's Oncology Group. (16985182)
2007
18
Peripheral primitive neuroectodermal tumor: a rare case of peripheral facial paralysis. (17505616)
2007
19
A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing's sarcoma of the kidney with renal vein tumor thrombus. (17310314)
2007
20
Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation. (16944972)
2006
21
Congenital cerebellar primitive neuroectodermal tumor in a newborn. (16586232)
2006
22
Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. (16904430)
2006
23
Metastatic primitive neuroectodermal tumor presenting as acute renal failure and hypercalcemia. (15786830)
2005
24
Mouth metastasis of peripheral primitive neuroectodermal tumor. (15641966)
2005
25
Molecular diagnosis of Ewing's sarcoma/primitive neuroectodermal tumor in formalin-fixed paraffin-embedded tissues by RT-PCR and fluorescence in situ hybridization. (15714060)
2005
26
Gene expression profiling of early- and late-relapse nonseminomatous germ cell tumor and primitive neuroectodermal tumor of the testis. (16217300)
2005
27
Primary primitive neuroectodermal tumor of the uterus: a report of two cases and review of the literature. (14766268)
2004
28
Primary Ewing's sarcoma-primitive neuroectodermal tumor of the vagina. (14766267)
2004
29
Ewing's sarcoma and peripheral primitive neuroectodermal tumor cells produce large quantities of bioactive tumor necrosis factor-alpha (TNF-alpha) after radiation exposure. (12873688)
2003
30
Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review. (12671870)
2003
31
Peripheral primitive neuroectodermal tumor of the maxillary gingivae with metastasis to cervical lymph nodes: report of a case. (12089700)
2002
32
Primitive neuroectodermal tumor of the transverse colonic mesentery defined by the presence of EWS-FLI1 chimeric mRNA in a Japanese woman. (12162413)
2002
33
Extraaxial primitive neuroectodermal tumor mimicking a vestibular schwannoma: diagnostic and therapeutic difficulties. Report of two cases. (11302660)
2001
34
Peripheral primitive neuroectodermal tumor: challenge for multimodal treatment. (11760735)
2001
35
Primitive neuroectodermal tumor of the pulmonary hilum in an adult. (11465208)
2001
36
Resistance to TRAIL-induced apoptosis in primitive neuroectodermal brain tumor cells correlates with a loss of caspase-8 expression. (11030149)
2000
37
Cerebral primitive neuroectodermal tumor in an adult male. A case report. (11127734)
2000
38
Primitive neuroectodermal tumor of the uterus. A case report. (10934964)
2000
39
Images in clinical urology. Magnetic resonance imaging of primitive neuroectodermal tumor of the kidney. (10688096)
2000
40
Endometrial endometrioid carcinomas associated with Ewing sarcoma/peripheral primitive neuroectodermal tumor. (10782408)
2000
41
Centrosome amplification as a possible mechanism for numerical chromosome aberrations in cerebral primitive neuroectodermal tumors with TP53 mutations. (10072601)
1998
42
Molecular analysis of childhood primitive neuroectodermal tumors defines markers associated with poor outcome. (9667267)
1998
43
Bone marrow metastasis in Ewing's sarcoma and peripheral primitive neuroectodermal tumor: An immunohistochemical study. (9507036)
1998
44
Primitive neuroectodermal tumor of the cervix uteri. A case report. (9065588)
1997
45
Mutation analysis and loss of heterozygosity of PEDF in central nervous system primitive neuroectodermal tumors. (9219833)
1997
46
The Ewing's sarcoma family of tumors: Ewing's sarcoma and peripheral primitive neuroectodermal tumor of bone and soft tissue. (9494764)
1997
47
Primitive neuroectodermal tumor (PNET). A case report. (8673418)
1995
48
Rhabdoid tumor of the kidney with primitive neuroectodermal tumor of the central nervous system: associated tumors with different histologic, cytogenetic, and molecular findings. (7530483)
1994
49
In vivo metabolism of childhood posterior fossa tumors and primitive neuroectodermal tumors before and after treatment. (8339230)
1993
50
Cutaneous angiosarcoma as a second malignant neoplasm after peripheral primitive neuroectodermal tumor. (1608360)
1992

Genetic Variations for Primitive Neuroectodermal Tumor

Expression for genes affiliated with Primitive Neuroectodermal Tumor

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Primitive Neuroectodermal Tumor

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

Pathways for genes affiliated with Primitive Neuroectodermal Tumor

Sources:
30KEGG, 4Cell Signaling Technology, 38NCBI BioSystems Database, 60Tocris Bioscience
See all sources

Pathways related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.5TP53, ETV7, MYCN, MYC, EWSR1
210.5S100B, SYP, MYCN, GFAP
310.5VIM, MYC, GFAP, TP53
410.5TP53, RASSF1, MYC, VIM
510.5MYC, MYCN

Compounds for genes affiliated with Primitive Neuroectodermal Tumor

Sources:
45Novoseek, 24HMDB, 50PharmGKB, 60Tocris Bioscience, 11DrugBank, 29IUPHAR, 2BitterDB
See all sources

Compounds related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 109)
idCompoundScoreTop Affiliating Genes
1paraffin4511.1TP53, PMS2, VIM, S100B, EWSR1, SYP
2retinoic acid45 2412.1EWSR1, CSF3, S100B, IFNB1, VIM, SYP
3vegf4511.1SYP, CSF3, S100B, IFNB1, VIM, GRP
4tyrosine4511.0IFNB1, S100B, CSF3, EWSR1, SYP, GRP
5cisplatin45 50 60 1114.0IFNB1, CSF3, SYP, GRP, MYC, MYCN
6genistein45 29 60 2 11 2416.0VIM, IFNB1, SYP, GRP, MYC, MYCN
7serine4511.0S100B, EWSR1, SYP, MYCN, RASSF1, GFAP
8acth4511.0IFNB1, S100B, SYP, GRP, SSTR2, GFAP
9dbc-amp4511.0VIM, IFNB1, S100B, SYP, MYCN, GFAP
10etoposide45 50 60 1114.0VIM, IFNB1, S100B, CSF3, GRP, MYCN
11thymidine45 2412.0IFNB1, S100B, CSF3, GRP, SSTR2, MYC
12cytarabine45 50 1113.0VIM, IFNB1, CSF3, MYC, GFAP, TP53
13vincristine45 50 1113.0VIM, IFNB1, CSF3, MYC, MYCN, TP53
14bromodeoxyuridine4511.0VIM, IFNB1, S100B, MYC, MYCN, GFAP
15dexamethasone45 50 29 1114.0IFNB1, S100B, GRP, MYC, GFAP, PTPRC
16progesterone45 60 29 11 2415.0VIM, IFNB1, S100B, SYP, SSTR2, GFAP
17s 1004511.0VIM, S100B, SYP, GFAP
18doxorubicin45 50 1112.9VIM, IFNB1, CSF3, GRP, MYC, MYCN
19paclitaxel45 50 1112.9IFNB1, S100B, CSF3, MYC, GFAP, TP53
20agar4510.9VIM, GRP, MYC, MYCN, RASSF1, GFAP
21hematoxylin4510.9VIM, S100B, SYP, GFAP, PTPRC
22cycloheximide4510.9IFNB1, MYCN
23estrogen4510.9GFAP
24reticulin4510.9VIM, S100B, SYP, GFAP
25adriamycin4510.9TP53, MYCN, MYC, CSF3, VIM
26dopamine45 29 11 2413.9GRP, SSTR2, MYCN, GFAP
27b 7234510.9PTPRC, SYP, S100B, VIM
28methionine4510.9PTPRC, GFAP, RASSF1, S100B, IFNB1, VIM
29choline45 11 2412.9VIM, S100B, SYP, GRP, GFAP
30formaldehyde45 2411.9PTPRC, GFAP, SYP, S100B, VIM
31tamoxifen45 50 29 1113.9IFNB1, GRP, SSTR2
32eosin4510.9VIM, S100B, GFAP
33actinomycin d4510.9VIM, IFNB1, GRP, MYC, MYCN, TP53
34glycogen45 2411.9SYP, MYC, GFAP, PTPRC
35forskolin45 50 1112.8VIM, S100B, SYP, GRP, SSTR2, GFAP
36acnu4510.8TP53, CSF3, IFNB1
375-aza-2deoxycytidine4510.8VIM, IFNB1, MYC, RASSF1, TP53
38oligonucleotide4510.8VIM, IFNB1, EWSR1, MYC, MYCN, RASSF1
39ganciclovir45 1111.8VIM, IFNB1, CSF3, GFAP
40glutamine4510.8VIM, S100B, EWSR1, SYP, GRP, GFAP
41neomycin4510.8VIM, IFNB1, GRP, GFAP
42ganglioside4510.7PTPRC, GFAP, SYP, VIM
43mucicarmine4510.7VIM, S100B, SYP
44chloramphenicol45 2 1112.7MYCN, MYC, S100B, IFNB1, VIM
45gaba4510.7VIM, S100B, SYP, GRP, GFAP
46retinoid4510.6TP53, MYCN, MYC, IFNB1, VIM
47hydroxyurea45 50 1112.5TP53, MYCN, MYC, CSF3
48creatinine4510.5IFNB1, SYP, GRP, GFAP
49leucine4510.5VIM, S100B, SYP, MYC, MYCN, GFAP
50gemcitabine45 50 1112.2IFNB1, CSF3, MYC, TP53

GO Terms for genes affiliated with Primitive Neuroectodermal Tumor

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1Bergmann glial cell differentiationGO:06002010.6GFAP, VIM
2axonogenesisGO:00740910.5SLITRK2, SLITRK4, S100B
3B cell proliferationGO:04210010.5PTPRC, IFNB1
4astrocyte developmentGO:01400210.4VIM, GFAP
5intermediate filament organizationGO:04510910.2GFAP, VIM

Molecular functions related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1identical protein bindingGO:04280210.6TP53, RASSF1, SYP, EWSR1, S100B, VIM

Products for genes affiliated with Primitive Neuroectodermal Tumor

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Primitive Neuroectodermal Tumor

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet