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

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
Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in the Adrenal Gland of an Adolescent: A Case Report and Review of the Literature. (24276043)
2013
2
Intracranial metastasis from primary spinal primitive neuroectodermal tumor. (23741262)
2013
3
Rare pulmonary primitive neuroectodermal tumor metastasizing to the right atrium: a case report. (22392199)
2012
4
Low-grade bone lesions in survivors of childhood medulloblastoma/primitive neuroectodermal tumor. (22054800)
2012
5
Primitive neuroectodermal tumor of the kidney with renal failure. (22977975)
2012
6
Peripheral primitive neuroectodermal tumor in a two-year-old paint horse. (22621943)
2012
7
Review: In vivo models for defining molecular subtypes of the primitive neuroectodermal tumor genome: current challenges and solutions. (22773561)
2012
8
Primitive neuroectodermal tumor/Ewing's sarcoma of the urinary bladder: a case report and its molecular diagnosis. (21063743)
2011
9
Peripheral primitive neuroectodermal tumor of the thymus. (21068011)
2011
10
Peripheral primitive neuroectodermal tumor of the chest wall in childhood: clinico-pathological significance, management and literature review. (21539764)
2011
11
Primary primitive neuroectodermal tumor of the breast: a rare entity. (21045465)
2010
12
Detection of SYT and EWS gene rearrangements by dual-color break-apart CISH in liquid-based cytology samples of synovial sarcoma and Ewing sarcoma/primitive neuroectodermal tumor. (20660338)
2010
13
De novo appearance of primitive neuroectodermal tumor in a patient with systemic lupus erythematosus and moyamoya disease. (20410155)
2010
14
A phase II study of preradiotherapy chemotherapy followed by hyperfractionated radiotherapy for newly diagnosed high-risk medulloblastoma/primitive neuroectodermal tumor: a report from the Children's Oncology Group (CCG 9931). (19356859)
2009
15
Frequent amplification of a chr19q13.41 microRNA polycistron in aggressive primitive neuroectodermal brain tumors. (19962671)
2009
16
A phase II study of the farnesyl transferase inhibitor, tipifarnib, in children with recurrent or progressive high-grade glioma, medulloblastoma/primitive neuroectodermal tumor, or brainstem glioma: a Children's Oncology Group study. (17932894)
2007
17
Metastatic primitive neuroectodermal tumor of the ovary in pregnancy. (17291265)
2007
18
Primitive neuroectodermal tumor of the uterus. (17537492)
2007
19
Primitive neuroectodermal tumor of the lung with pericardial extension: a case report. (18005874)
2007
20
Peripheral primitive neuroectodermal tumor of bowel mesentery in adults. (16889176)
2006
21
Primitive neuroectodermal tumor of the brain in a girl with Turner syndrome diagnosed after 4 years of growth hormone therapy. (16450159)
2006
22
Role of radiotherapy in supratentorial primitive neuroectodermal tumor in young children: results of the German HIT-SKK87 and HIT-SKK92 trials. (16575007)
2006
23
Primary primitive neuroectodermal tumor of the kidney: a case report. (16868682)
2006
24
A primitive neuroectodermal tumor on the face: case report. (16033149)
2005
25
Molecular detection of EWS-Ets fusion transcripts and their clinicopathologic significance in Ewing's sarcoma/peripheral primitive neuroectodermal tumor. (16157025)
2005
26
Analysis of chromosome abnormalities by comparative genomic hybridization in malignant peripheral primitive neuroectodermal tumor of the ovary. (14984937)
2004
27
Prenatal imaging of congenital cerebral primitive neuroectodermal tumor. (12711864)
2003
28
Primitive neuroectodermal tumor arising in long-standing cerebellar atrophy. (12566839)
2003
29
Primitive neuroectodermal tumor of the mandible: report of a rare case. (12696243)
2003
30
Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: a clinicopathologic study of five cases and review of the literature. (12784241)
2003
31
Primitive neuroectodermal tumor of the cervix: a clinicopathologic and immunohistochemical study of two cases. (12376920)
2002
32
Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. (11859203)
2002
33
Adult primitive neuroectodermal tumor: proton MR spectroscopic findings with possible application for differential diagnosis. (12409595)
2002
34
Primitive neuroectodermal tumor of the spermatic cord. (11912418)
2002
35
Malignant peripheral primitive neuroectodermal tumor of the kidney. (12547163)
2002
36
Metastasis of primitive neuroectodermal tumor to the breast. (12116099)
2002
37
Atypical pleomorphic extraosseous ewing tumor/peripheral primitive neuroectodermal tumor with unusual phenotypic/genotypic profile. (11854596)
2002
38
A case of canine primitive neuroectodermal tumor (PNET). (11217053)
2001
39
Primary Ewing's sarcoma/peripheral primitive neuroectodermal tumor at the vertex of the skull with elevated serum carcinoembryonic antigen: case report. (11508817)
2001
40
Cervical primitive neuroectodermal tumor. (11585426)
2001
41
Primitive neuroectodermal tumor of the orbit in a 5-year-old girl with microphthalmia. (11045350)
2000
42
Primitive neuroectodermal tumor arising from the kidney. (11117344)
2000
43
Testicular immature teratoma with primitive neuroectodermal tumor in early childhood. (11061928)
2000
44
Immunohistochemical detection of FLI-1 protein expression: a study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing's sarcoma/primitive neuroectodermal tumor. (11117787)
2000
45
Lovastatin induces apoptosis in a primitive neuroectodermal tumor cell line in association with RB down-regulation and loss of the G1 checkpoint. (11146561)
2000
46
Molecular insight into medulloblastoma and central nervous system primitive neuroectodermal tumor biology from hereditary syndromes: a review. (11014429)
2000
47
Neuroendocrine differentiation in Ewing's sarcomas and primitive neuroectodermal tumors revealed by reverse transcriptase-polymerase chain reaction of chromogranin mRNA. (9646033)
1998
48
Primitive neuroectodermal tumor of the kidney confirmed by fluorescence in situ hybridization. (9130994)
1997
49
A case of primitive neuroectodermal tumor in the chest wall ("Askin's tumor")]. (8176303)
1994
50
Primitive neuroectodermal tumor of the retroperitoneal cavity. (8042253)
1994

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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26ICD10 via Orphanet
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