MCID: PRP025
MIFTS: 50

Peripheral Primitive Neuroectodermal Tumor malady

Genetic diseases, Rare diseases, Bone diseases, Cancer diseases, Neuronal diseases categories

Aliases & Classifications for Peripheral Primitive Neuroectodermal Tumor

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Peripheral Primitive Neuroectodermal Tumor, Aliases & Descriptions:

Name: Peripheral Primitive Neuroectodermal Tumor 9 11 47
Ewings Sarcoma 9 43 60
Localized Peripheral Primitive Neuroectodermal Tumor 9 60
Ewings Sarcoma-Primitive Neuroectodermal Tumor 9 60
Localized Ewing's Sarcoma 9 60
Ewing's Tumor 9 41
Ewing Sarcoma 9 41
Localized Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 9
Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 9
Pnet of Thoracopulmonary Region 9
 
Ewing's Family Localized Tumor 9
Localized Ewing's Tumor 9
Localized Ewing Sarcoma 9
Sarcoma, Ewing's 41
Peripheral Pnet 47
Ewing's Sarcoma 41
Askin's Tumor 60
Ewing Tumor 41
Ppnet 47


Classifications:



Summaries for Peripheral Primitive Neuroectodermal Tumor

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NIH Rare Diseases:41 Ewing's sarcoma is a malignant (cancerous) bone tumor that affects children. it can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. the tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. it may also develop in the skull or the flat bones of the trunk. there are few symptoms. the most common is pain and occasionally swelling at the site of the tumor. fever may also be present. the tumor often spreads (metastasis) to the lungs and other bones. the cause of ewing's sarcoma is unknown. last updated: 6/4/2009

MalaCards based summary: Peripheral Primitive Neuroectodermal Tumor, also known as ewings sarcoma, is related to neuroectodermal tumor and sarcoma. An important gene associated with Peripheral Primitive Neuroectodermal Tumor is FLI1 (Fli-1 proto-oncogene, ETS transcription factor), and among its related pathways are Prostate cancer and Transcriptional misregulation in cancer. The drugs cyclophosphamide and etoposide and the compounds indium and metronidazole have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and kidney, and related mouse phenotypes are mortality/aging and normal.

Disease Ontology:9 A bone cancer that has material basis in neural crest cells derived from undeveloped, undifferentiated neuroectoderm.

Related Diseases for Peripheral Primitive Neuroectodermal Tumor

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

(show top 50)    (show all 97)
idRelated DiseaseScoreTop Affiliating Genes
1neuroectodermal tumor32.5EWSR1, FLI1
2sarcoma30.9DES, ATF1, EWSR1, FLI1, MUC1
3ewing's family of tumors30.9EWSR1, FLI1
4ewing sarcoma30.8DES, ATF1, EWSR1, FLI1, ERG
5rhabdomyosarcoma30.6FLI1, EWSR1, DES
6desmoplastic small round cell tumor30.6MUC1, EWSR1, DES
7angiosarcoma30.6MUC1, FLI1
8chondrosarcoma30.4MUC1, EWSR1, DES
9mesenchymal chondrosarcoma30.2DES, MUC1
10leukemia29.9ATF1, EWSR1, FLI1, PSG2, ERG
11breast cancer29.9ATF1, FLI1, MUC1, PSG2
12melanoma29.6DES, ATF1, EWSR1, FLI1, PSG2
13neuroblastoma10.5
14bone ewing's sarcoma10.5
15cervicitis10.5
16gastrointestinal stromal tumor10.5
17supratentorial primitive neuroectodermal tumor10.5
18extraskeletal ewing sarcoma10.5
19myxoid liposarcoma10.4ATF1
20extraosseous ewings sarcoma-primitive neuroepithelial tumor10.4
21pericardial effusion10.4PSG2
22glomus tumor10.4DES
23tetralogy of fallot10.3
24olfactory neuroblastoma10.3
25neuronitis10.3
26cauda equina syndrome10.3
27bone peripheral neuroepithelioma10.3
28soft tissue peripheral neuroepithelioma10.3
29paraganglioma10.3
30extraosseous ewing's sarcoma10.3
31facial paralysis10.3
32pancreatitis10.3
33thyroiditis10.3
34hemangioendothelioma10.3
35epithelioid hemangioendothelioma10.3
36hemangioma10.3FLI1
37connective tissue benign neoplasm10.3FLI1, EWSR1
38fibrous meningioma10.3MUC1
39syringoma10.3MUC1
40soft tissue sarcoma10.3
41clear cell sarcoma10.2EWSR1, ATF1
42embryonal rhabdomyosarcoma10.2DES, EWSR1
43insulin-like growth factor i10.2
44fibrous histiocytoma10.2DES, ATF1
45meningitis10.1
46medulloblastoma10.1
47prostate cancer10.1
48esophagitis, eosinophilic, 110.1
49nail-patella syndrome10.1
50retinoblastoma10.1

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



Diseases related to peripheral primitive neuroectodermal tumor

Symptoms for Peripheral Primitive Neuroectodermal Tumor

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Drugs & Therapeutics for Peripheral Primitive Neuroectodermal Tumor

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Drug clinical trials:

Search ClinicalTrials for Peripheral Primitive Neuroectodermal Tumor

Search NIH Clinical Center for Peripheral Primitive Neuroectodermal Tumor

Inferred drug relations via UMLS60/NDF-RT39:

Genetic Tests for Peripheral Primitive Neuroectodermal Tumor

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Anatomical Context for Peripheral Primitive Neuroectodermal Tumor

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MalaCards organs/tissues related to Peripheral Primitive Neuroectodermal Tumor:

31
Bone, Lung, Kidney, Ovary, Adrenal gland, Breast, Bone marrow, Lymph node, Thymus, Liver, Thyroid, Uterus, Testis, Testes, Cervix

Animal Models for Peripheral Primitive Neuroectodermal Tumor or affiliated genes

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MGI Mouse Phenotypes related to Peripheral Primitive Neuroectodermal Tumor:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107688.1ERG, FLI1, EWSR1, ATF1, DES
2MP:00028738.0ERG, MUC1, EWSR1, ATF1

Publications for Peripheral Primitive Neuroectodermal Tumor

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Articles related to Peripheral Primitive Neuroectodermal Tumor:

(show top 50)    (show all 134)
idTitleAuthorsYear
1
Peripheral primitive neuroectodermal tumor of chest wall in young adult. (25798010)
2015
2
Pancreatic peripheral primitive neuroectodermal tumor diagnosed by endoscopic ultrasound. (25603505)
2015
3
Mandibular peripheral primitive neuroectodermal tumor: a rare case report with review of literature. (25861194)
2015
4
Congenital Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature. (24480101)
2014
5
Intracranial ewings sarcoma/peripheral primitive neuroectodermal tumor. (25237951)
2014
6
Peripheral primitive neuroectodermal tumor: dynamic CT, MRI and clinicopathological characteristics--analysis of 36 cases and review of the literature. (25587032)
2014
7
Peripheral primitive neuroectodermal tumor of the dura in a 51-year-old woman following intensive treatment for breast cancer. (25045413)
2014
8
Primary peripheral primitive neuroectodermal tumor of the mandible. (24205482)
2013
9
Spinal Dumbbell-Shaped Peripheral Primitive Neuroectodermal Tumor in a Child. (24401730)
2013
10
Peripheral primitive neuroectodermal tumor in a two-year-old paint horse. (22621943)
2012
11
Peripheral primitive neuroectodermal tumor of the pleura in a 41-year-old female. (22821316)
2012
12
Paravaginal peripheral primitive neuroectodermal tumor: A rare tumor. (22557788)
2011
13
Giant peripheral primitive neuroectodermal tumor of the mons pubis: a case report. (20882290)
2011
14
Peripheral primitive neuroectodermal tumor of the thymus. (21068011)
2011
15
Unusual presentation of peripheral primitive neuroectodermal tumor of the maxilla. (21119416)
2010
16
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
17
Dumbbell-shaped peripheral primitive neuroectodermal tumor of the spine--case report and review of the literature. (19050994)
2009
18
Primary epidural peripheral primitive neuroectodermal tumor of the thoracic spine. (19940407)
2009
19
Imaging findings of abdominal peripheral primitive neuroectodermal tumor: report of four cases with pathological correlation. (19411024)
2009
20
Primary peripheral primitive neuroectodermal tumor of mandible. (18376294)
2008
21
Ewing's sarcoma and peripheral primitive neuroectodermal tumor in adults: different features of a rare neoplasm. (18418019)
2008
22
Peripheral primitive neuroectodermal tumor: a rare case of peripheral facial paralysis. (17505616)
2007
23
Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review. (16784984)
2006
24
Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation. (16944972)
2006
25
Peripheral primitive neuroectodermal tumor of bowel mesentery in adults. (16889176)
2006
26
Intracranial peripheral primitive neuroectodermal tumor manifesting as abducens nerve palsy. (15657787)
2005
27
Peripheral primitive neuroectodermal tumor of the mandible with cytogenetic and molecular biology aberrations. (16094594)
2005
28
Peripheral primitive neuroectodermal tumor of the cerebellopontine angle. (15823816)
2005
29
Analysis of chromosome abnormalities by comparative genomic hybridization in malignant peripheral primitive neuroectodermal tumor of the ovary. (14984937)
2004
30
Unique cellular features of peripheral primitive neuroectodermal tumor: ultrastructural evidence of its unique cytodifferentiation. (15449113)
2004
31
Peripheral primitive neuroectodermal tumor of the stomach in a 14-year-old boy: a case report. (15618851)
2004
32
A peripheral primitive neuroectodermal tumor with generalized bone metastases in a puppy. (15232149)
2004
33
Peripheral primitive neuroectodermal tumor after radiotherapy. (12897617)
2003
34
Peripheral primitive neuroectodermal tumor of the maxillary gingivae with metastasis to cervical lymph nodes: report of a case. (12089700)
2002
35
Metastatic peripheral primitive neuroectodermal tumor (PNET) masquerading as liver abscess: a case report of liver metastasis in orbital PNET. (12080235)
2002
36
Peripheral primitive neuroectodermal tumor presenting in the retroperitoneum: a case report with immunohistochemical study. (12240569)
2002
37
Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location. (11401207)
2001
38
Peripheral primitive neuroectodermal tumor: challenge for multimodal treatment. (11760735)
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
Peripheral primitive neuroectodermal tumor with neuronal and glial differentiation: report of a case arising in suprarenal region. (11460974)
2001
41
Peripheral primitive neuroectodermal tumor of the parotid gland region: report of a case with fine-needle aspiration findings. (10679996)
2000
42
Documentation of EWS gene rearrangements by fluorescence in-situ hybridization (FISH) in frozen sections of Ewing's sarcoma-peripheral primitive neuroectodermal tumor. (10078922)
1999
43
The Ewing tumor family of peripheral primitive neuroectodermal tumors expresses human gastrin-releasing peptide. (9622091)
1998
44
Peripheral primitive neuroectodermal tumor: report of a case arising in the kidney. (9211963)
1997
45
Very-high-dose short-term chemotherapy for poor-risk peripheral primitive neuroectodermal tumors, including Ewing's sarcoma, in children and young adults. (7595741)
1995
46
Gallium and thallium scintigraphy in pediatric peripheral primitive neuroectodermal tumor (Askin tumor) of the chest wall. (7738655)
1995
47
Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney. (8084311)
1994
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
MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration. (1848471)
1991
50
Overexpression of the pseudoautosomal gene MIC2 in Ewing's sarcoma and peripheral primitive neuroectodermal tumor. (1695726)
1990

Variations for Peripheral Primitive Neuroectodermal Tumor

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Expression for genes affiliated with Peripheral Primitive Neuroectodermal Tumor

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

Pathways for genes affiliated with Peripheral Primitive Neuroectodermal Tumor

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Pathways related to Peripheral Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Prostate Cancer36
Integrated Cancer pathway36
Steroid Biosynthesis36
9.5ERG, ATF1
28.7ERG, FLI1, EWSR1, ATF1

Compounds for genes affiliated with Peripheral Primitive Neuroectodermal Tumor

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Sources:
43Novoseek, 12DrugBank, 24HMDB, 28IUPHAR, 59Tocris Bioscience
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Compounds related to Peripheral Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

(show all 25)
idCompoundScoreTop Affiliating Genes
1indium439.5PSG2, MUC1
2metronidazole43 1210.4PSG2, MUC1
3chromium439.4PSG2, MUC1
4s 100439.4MUC1, DES
5reticulin439.3MUC1, DES
6mucicarmine439.3DES, MUC1
7oligonucleotide439.1ATF1, EWSR1, FLI1
8progestin439.1MUC1, DES
9p002439.0PSG2, DES
10biotin43 24 1210.9MUC1, DES
11b 723438.9PSG2, MUC1, DES
12hematoxylin438.9PSG2, MUC1, DES
13adriamycin438.8DES, MUC1, PSG2
14gold438.8PSG2, MUC1, DES
15formaldehyde43 249.8PSG2, MUC1, DES
1612-o-tetradecanoylphorbol 13-acetate438.7DES, ATF1, MUC1
17glycogen43 249.7PSG2, MUC1, DES
18thymidine43 249.7PSG2, MUC1, DES
19creatinine438.7DES, MUC1, PSG2
20progesterone43 28 59 24 1212.6MUC1, ATF1, DES
21vegf438.5PSG2, MUC1, DES
22paraffin438.4DES, EWSR1, MUC1, PSG2
23cyclic amp43 249.4MUC1, EWSR1, ATF1, DES
24glucose438.2DES, ATF1, MUC1, PSG2
25serine438.0DES, ATF1, EWSR1, FLI1, MUC1

GO Terms for genes affiliated with Peripheral Primitive Neuroectodermal Tumor

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Molecular functions related to Peripheral Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1sequence-specific DNA binding RNA polymerase II transcription factor activityGO:00009819.7ERG, FLI1
2sequence-specific DNA bindingGO:00435658.9ERG, FLI1, ATF1

Products for genes affiliated with Peripheral Primitive Neuroectodermal Tumor

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Sources for Peripheral Primitive Neuroectodermal Tumor

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet