CPNET
MCID: PRM027
MIFTS: 59

Primitive Neuroectodermal Tumor (CPNET) malady

Neuronal diseases, Cancer diseases categories

Summaries for Primitive Neuroectodermal Tumor

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MalaCards: Primitive Neuroectodermal Tumor, also known as localized primitive neuroectodermal tumor, is related to peripheral primitive neuroectodermal tumor and sarcoma. 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. Affiliated tissues include kidney, bone and brain, and related mouse phenotypes are normal and immune system.

Aliases & Classifications for Primitive Neuroectodermal Tumor

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60UMLS, 8Disease Ontology, 56SNOMED-CT, 34MeSH, 39NCIt
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Neuronal diseases


Aliases & Descriptions:

primitive neuroectodermal tumor 8
localized primitive neuroectodermal tumor 8 60
extraosseous ewings sarcoma-primitive neuroepithelial tumor 60
primitive neuroectodermal tumour 8
neuroectodermal tumor, primitive 60
cns pnet 8
cpnet 8


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Disease Ontology8 DOID:168
MeSH34 D018242
SNOMED-CT56 39781001, 189925001
NCIt39 C5398

Related Diseases for Primitive Neuroectodermal Tumor

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17GeneCards, 18GeneDecks
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Diseases related to Primitive Neuroectodermal Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 261)
idRelated DiseaseScoreTop Affiliating Genes
1peripheral primitive neuroectodermal tumor31.9S100B, EWSR1, GRP, VIM, MYCN, ETV7
2sarcoma31.8CD99, PTPRC, TP53, VIM, S100B, EWSR1
3supratentorial primitive neuroectodermal tumor31.6SLITRK4, SLITRK2
4medulloblastoma31.5SSTR2, TP53, GFAP, RASSF1, MYCN, MYC
5cerebral primitive neuroectodermal tumor31.2MYCN, MYC
6neuroblastoma31.0TP53, MYCN, MYC, SYP
7childhood medulloblastoma30.8MYCN, MYC
8retinoblastoma30.8TP53, GFAP, RASSF1, ETV7, MYCN, MYC
9pineoblastoma30.7SYP, GFAP
10rhabdomyosarcoma30.6TP53, EWSR1, VIM, S100B, PTPRC, CD99
11astrocytoma30.6RASSF1, GFAP, TP53, S100B, VIM, SYP
12neuroendocrine tumor30.6IFNB1, RASSF1, SSTR2, SYP, MYCN, GRP
13leukemia30.6EWSR1, GRP, MYC, MYCN, IFNB1, CD99
14embryonal rhabdomyosarcoma30.6EWSR1, MYCN, PTPRC
15endometrial carcinoma30.5TP53, MYCN, ETV7, MYC, RASSF1
16ganglioglioma30.5GFAP, S100B, SYP
17synovial sarcoma30.5VIM, S100B, RASSF1, MYCN
18olfactory neuroblastoma30.5CD99, S100B, SYP
19gastrointestinal stromal tumor30.5VIM, S100B
20acute leukemia30.5PTPRC, CSF3, EWSR1
21ganglioneuroma30.3S100B, RASSF1, GFAP, MYCN, SYP
22oligodendroglioma30.3SYP, GFAP, TP53
23medulloepithelioma30.3SYP
24meningioma30.3S100B, SYP, SSTR2, GFAP, VIM
25squamous cell carcinoma30.3VIM, ETV7, SYP, RASSF1, GRP, TP53
26malignant glioma30.3SYP, GRP, GFAP, TP53, IFNB1
27paraganglioma30.3GFAP, SYP, GRP, SSTR2, S100B
28osteosarcoma30.3MYCN, TP53, MYC, IFNB1
29pheochromocytoma30.3MYCN, SSTR2, SYP
30myeloid leukemia30.3CSF3, ETV7, PTPRC, MYC
31merkel cell carcinoma30.3SYP
32gliosarcoma30.3GFAP
33angiosarcoma30.3VIM
34herpes simplex30.3SSTR2, IFNB1
35bladder carcinoma30.3TP53, RASSF1
36neurofibroma30.3SYP, S100B, VIM
37clear cell sarcoma30.3S100B, EWSR1
38fibrous histiocytoma30.2S100B, VIM
39cerebritis10.8
40extraosseous ewings sarcoma-primitive neuroepithelial tumor10.8
41intracranial primitive neuroectodermal tumor10.8
42kidney sarcoma10.7
43atypical teratoid rhabdoid tumor10.6
44bone ewing's sarcoma10.6
45extracranial arteriovenous malformation10.5
46cervicitis10.5
47cerebellopontine angle primitive neuroectodermal10.5
48neuronitis10.5
49extraosseous ewing's sarcoma10.5
50prostatitis10.5

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



Diseases related to primitive neuroectodermal tumor

Clinical Features for Primitive Neuroectodermal Tumor

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Drugs & Therapeutics for Primitive Neuroectodermal Tumor

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Genetic Tests for Primitive Neuroectodermal Tumor

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Anatomical Context for Primitive Neuroectodermal Tumor

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32MalaCards
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MalaCards organs/tissues related to Primitive Neuroectodermal Tumor:

32
Kidney, Bone, Brain, Uterus, Breast, Ovary, Spinal cord, Adrenal gland, Cervix, Pancreas, Liver, Lung, Prostate, Testis, Thyroid, Thymus, Bone marrow, Heart, Pineal, Colon, B lymphoblasts, Retina, T cells, Myeloid, Testes, Cerebellum, Lymph node, Skin, Cortex

Animal Models for Primitive Neuroectodermal Tumor or affiliated genes

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MGI Mouse Phenotypes related to Primitive Neuroectodermal Tumor:

36
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

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50PubMed
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Articles related to Primitive Neuroectodermal Tumor:

(show top 50)    (show all 653)
idTitleAuthorsYear
1
Childhood pheochromocytoma in a survivor of central primitive neuroectodermal tumor. (23910809)
2013
2
Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus. (22532539)
2013
3
Peripheral primitive neuroectodermal tumor of the adrenal gland: A rare entity. (24235803)
2013
4
Primitive neuroectodermal tumor of the kidney: case report and review of literature. (23270507)
2012
5
A case of primitive neuroectodermal tumor of the kidney and review of the literature. (23455817)
2012
6
Primary primitive neuroectodermal tumor of the urinary bladder: a case report and literature review. (20852971)
2011
7
Primitive neuroectodermal tumor of the liver: a case report. (22242031)
2011
8
Displaced left kidney masquerading as splenic blush in 99mTc-DTPA GFR study in a patient of paravertebral primitive neuroectodermal tumor. (21546756)
2011
9
Genome-wide molecular characterization of central nervous system primitive neuroectodermal tumor and pineoblastoma. (21798848)
2011
10
Primary vulvar Ewing's sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: a case report. (19656640)
2010
11
Unusual presentation of peripheral primitive neuroectodermal tumor of the maxilla. (21119416)
2010
12
Impact of EWS-ETS fusion type on disease progression in Ewing's sarcoma/peripheral primitive neuroectodermal tumor: prospective results from the cooperative Euro-E.W.I.N.G. 99 trial. (20308673)
2010
13
Supratentorial primitive neuroectodermal tumor metastasis to the abdomen via a ventriculoperitoneal shunt. (20526591)
2010
14
Cathepsin B facilitates autophagy-mediated apoptosis in SPARC overexpressed primitive neuroectodermal tumor cells. (20339379)
2010
15
Uncommon pleural effusion: pleuropulmonary metastasis from primitive neuroectodermal tumor. (19618040)
2009
16
Glioblastoma with primitive neuroectodermal tumor-like features: case report. (19621291)
2009
17
A rare case of primitive neuroectodermal tumor in the soft tissues of the hand. (20387712)
2009
18
Primitive neuroectodermal tumor of the kidney: a single institute series of 16 patients. (18308106)
2008
19
An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival. (18095134)
2007
20
Identification of novel biomarkers in pediatric primitive neuroectodermal tumors and ependymomas by proteome-wide analysis. (17549010)
2007
21
F-18 FDG PET imaging in a primitive neuroectodermal tumor. (16855439)
2006
22
High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors. (16783165)
2006
23
Radiologic findings of peripheral primitive neuroectodermal tumor arising in the retroperitoneum. (16554591)
2006
24
Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. (17063089)
2006
25
Primitive neuroectodermal tumor of the kidney. Report of a case initially diagnosed by fine needle aspiration cytology. (15085765)
2004
26
Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. (15274413)
2004
27
Primitive neuroectodermal tumor of the kidney. A report of two cases diagnosed by fine needle aspiration cytology. (12789935)
2003
28
Primitive neuroectodermal tumor (PNET) of the urinary bladder. (14708642)
2003
29
Unusual aberration involving the short arm of chromosome 11 in an 8-month-old patient with a supratentorial primitive neuroectodermal tumor. (12606132)
2003
30
Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. (14520055)
2003
31
Organ-confined primitive neuroectodermal tumor arising from the kidney. (12677579)
2003
32
Metastatic peripheral primitive neuroectodermal tumor (PNET) masquerading as liver abscess: a case report of liver metastasis in orbital PNET. (12080235)
2002
33
Primitive neuroectodermal tumor: report of two cases and review of the literature. (12324744)
2002
34
Primary intraspinal primitive neuroectodermal tumor (PNET): a rare occurrence. (11960157)
2002
35
Molecular genetic analysis of a small bowel primitive neuroectodermal tumor. (11815873)
2002
36
Letter to the editor: tumor thrombus in a child with primitive neuroectodermal tumor. (11984814)
2002
37
Role of MAP kinase pathways in primitive neuroectodermal tumors. (11724348)
2001
38
Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: a case report. (11349224)
2001
39
Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall. (11481653)
2001
40
Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. (10623711)
2000
41
Is screening for primitive neuroectodermal tumors in patients with unilateral retinoblastoma necessary? (10675872)
2000
42
WT1 staining reliably differentiates desmoplastic small round cell tumor from Ewing sarcoma/primitive neuroectodermal tumor. An immunohistochemical and molecular diagnostic study. (10989634)
2000
43
Comparison of CSF cytology and spinal magnetic resonance imaging in the detection of leptomeningeal disease in pediatric medulloblastoma or primitive neuroectodermal tumor. (10506624)
1999
44
Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors. (10447606)
1999
45
Primitive neuroectodermal tumors of the cerebral hemispheres in two siblings with TP53 germline mutation. (9600210)
1998
46
Glial differentiation predicts poor clinical outcome in primitive neuroectodermal brain tumors. (8619526)
1996
47
Two primitive neuroectodermal tumor cell lines require an activated insulin-like growth factor I receptor for growth in vitro. (8938773)
1996
48
Reverse transcriptase PCR amplification of EWS/FLI-1 fusion transcripts as a diagnostic test for peripheral primitive neuroectodermal tumors of childhood. (7506981)
1993
49
Secretogranin II expression in Ewing's sarcomas and primitive neuroectodermal tumors. (1342962)
1992
50
Differentiation of a primitive neuroectodermal tumor into a benign ganglioglioma. (1460486)
1992

Genetic Variations for Primitive Neuroectodermal Tumor

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Expression for genes affiliated with Primitive Neuroectodermal Tumor

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Primitive Neuroectodermal Tumor

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Pathways for genes affiliated with Primitive Neuroectodermal Tumor

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29KEGG, 4Cell Signaling Technology, 37NCBI BioSystems Database, 59Tocris Bioscience
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Pathways related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

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

Compounds for genes affiliated with Primitive Neuroectodermal Tumor

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44Novoseek, 24HMDB, 49PharmGKB, 59Tocris Bioscience, 11DrugBank, 28IUPHAR, 2BitterDB
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Compounds related to Primitive Neuroectodermal Tumor according to GeneCards/GeneDecks:

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idCompoundScoreTop Affiliating Genes
1paraffin4411.1TP53, PMS2, VIM, PTPRC, EWSR1, SYP
2retinoic acid44 2412.1VIM, GFAP, RASSF1, ETV7, MYCN, MYC
3vegf4411.1MYCN, VIM, TP53, GFAP, CD99, MYC
4tyrosine4411.0SSTR2, SYP, MYC, MYCN, ETV7, GFAP
5cisplatin44 49 59 1114.0IFNB1, TP53, RASSF1, MYCN, MYC, GRP
6genistein44 28 59 2 11 2416.0MYC, GFAP, MYCN, SYP, IFNB1, PTPRC
7serine4411.0RASSF1, S100B, EWSR1, MYCN, GFAP, SYP
8acth4411.0PTPRC, GFAP, SSTR2, GRP, IFNB1, SYP
9dbc-amp4411.0IFNB1, S100B, VIM, MYCN, GFAP, SYP
10etoposide44 49 59 1114.0S100B, CSF3, GRP, MYCN, IFNB1, VIM
11thymidine44 2412.0GRP, CSF3, S100B, IFNB1, MYC, SSTR2
12cytarabine44 49 1113.0CSF3, VIM, MYC, TP53, IFNB1, GFAP
13vincristine44 49 1113.0TP53, MYC, VIM, IFNB1, CSF3, MYCN
14bromodeoxyuridine4411.0MYCN, VIM, GFAP, IFNB1, S100B, MYC
15dexamethasone44 49 28 1114.0GFAP, IFNB1, PTPRC, MYC, S100B, GRP
16progesterone44 59 28 11 2415.0SSTR2, S100B, SYP, GFAP, PTPRC, IFNB1
17s 1004411.0S100B, SYP, GFAP, VIM
18doxorubicin44 49 1112.9TP53, MYCN, MYC, GRP, VIM, IFNB1
19paclitaxel44 49 1112.9GFAP, MYC, CSF3, TP53, S100B, IFNB1
20agar4410.9MYC, MYCN, GFAP, VIM, GRP, RASSF1
21hematoxylin4410.9SYP, VIM, GFAP, PTPRC, S100B
22cycloheximide4410.9IFNB1, MYCN
23estrogen4410.9GFAP
24reticulin4410.9S100B, VIM, GFAP, SYP
25adriamycin4410.9TP53, MYC, CSF3, VIM, MYCN
26dopamine44 28 11 2413.9SSTR2, GFAP, MYCN, GRP
27b 7234410.9S100B, VIM, PTPRC, SYP
28methionine4410.9S100B, RASSF1, GFAP, PTPRC, VIM, IFNB1
29choline44 11 2412.9VIM, S100B, GRP, GFAP, SYP
30formaldehyde44 2411.9S100B, VIM, SYP, GFAP, PTPRC
31tamoxifen44 49 28 1113.9SSTR2, GRP, IFNB1
32eosin4410.9VIM, S100B, GFAP
33actinomycin d4410.9MYC, VIM, GRP, IFNB1, MYCN, TP53
34glycogen44 2411.9SYP, MYC, GFAP, PTPRC
35forskolin44 49 1112.8GFAP, SSTR2, GRP, SYP, S100B, VIM
36acnu4410.8TP53, CSF3, IFNB1
375-aza-2deoxycytidine4410.8RASSF1, MYC, IFNB1, VIM, TP53
38oligonucleotide4410.8EWSR1, VIM, TP53, IFNB1, MYC, MYCN
39ganciclovir44 1111.8VIM, CSF3, GFAP, IFNB1
40glutamine4410.8VIM, EWSR1, S100B, GRP, SYP, GFAP
41neomycin4410.8IFNB1, GFAP, GRP, VIM
42ganglioside4410.7PTPRC, GFAP, SYP, VIM
43mucicarmine4410.7VIM, S100B, SYP
44chloramphenicol44 2 1112.7MYC, S100B, IFNB1, VIM, MYCN
45gaba4410.7GRP, GFAP, VIM, S100B, SYP
46retinoid4410.6MYCN, MYC, TP53, IFNB1, VIM
47hydroxyurea44 49 1112.5CSF3, MYCN, TP53, MYC
48creatinine4410.5IFNB1, SYP, GFAP, GRP
49leucine4410.5S100B, GFAP, SYP, MYC, VIM, MYCN
50gemcitabine44 49 1112.2CSF3, MYC, TP53, IFNB1

GO Terms for genes affiliated with Primitive Neuroectodermal Tumor

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16Gene Ontology
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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

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