MCID: EXT031
MIFTS: 41

Extraosseous Ewing's Sarcoma

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Extraosseous Ewing's Sarcoma

MalaCards integrated aliases for Extraosseous Ewing's Sarcoma:

Name: Extraosseous Ewing's Sarcoma 12 14
Extraosseous Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 69
Sarcoma Extraosseous Ewings 52
Extraosseous Ewing Sarcoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:4232
NCIt 47 C27293
UMLS 69 C1333514

Summaries for Extraosseous Ewing's Sarcoma

MalaCards based summary : Extraosseous Ewing's Sarcoma, also known as extraosseous ewing's sarcoma/peripheral primitive neuroectodermal tumor, is related to extraosseous ewings sarcoma-primitive neuroepithelial tumor and ewing sarcoma, and has symptoms including fever An important gene associated with Extraosseous Ewing's Sarcoma is EWSR1 (EWS RNA Binding Protein 1), and among its related pathways/superpathways are Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers and Interactome of polycomb repressive complex 2 (PRC2). The drugs Doxorubicin and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include liver, kidney and bone marrow, and related phenotypes are mortality/aging and growth/size/body region

Related Diseases for Extraosseous Ewing's Sarcoma

Diseases related to Extraosseous Ewing's Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 65)
id Related Disease Score Top Affiliating Genes
1 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.1
2 ewing sarcoma 11.6
3 extraskeletal ewing sarcoma 11.2
4 vitreous syneresis 10.9 CD99 EWSR1
5 spleen cancer 10.8 MYOD1 S100B
6 liposarcoma of bone 10.8 ENO2 EWSR1
7 meningitis and encephalitis 10.8 ENO2 S100B
8 benallegue lacete syndrome 10.8 ENO2 S100B
9 mucinous ovarian cystadenoma 10.7 MYOD1 S100B
10 chondroid chordoma 10.7 ENO2 EWSR1
11 myoma 10.7 ENO2 S100B
12 enteric pattern testicular yolk sac tumor 10.7 ENO2 S100B
13 phosphoserine aminotransferase deficiency 10.7 MYF6 MYOD1
14 motor neuron disease 10.7 ENO2 S100B
15 pleomorphic xanthoastrocytoma 10.7 ENO2 EWSR1
16 female breast axillary tail cancer 10.7 CD99 ENO2
17 lobular neoplasia 10.6 CD99 ENO2
18 spondyloepimetaphyseal dysplasia, isidor type 10.6 CD99 ENO2 EWSR1
19 thymus large cell carcinoma 10.6 ENO2 EWSR1
20 gestational choriocarcinoma 10.6 CD99 ENO2 EWSR1
21 anal canal adenocarcinoma 10.6 ENO2 EWSR1 S100B
22 pineal parenchymal tumor of intermediate differentiation 10.6 MYF6 MYOD1 TSFM
23 kidney sarcoma 10.6 ENO2 EWSR1 S100B
24 cerebrum cancer 10.6 ENO2 EWSR1 MYOD1
25 hydromyelia 10.5 ENO2 S100B
26 46xy sex reversal 5 10.5 ENO2 MYF6 MYOD1
27 bile duct sarcoma 10.5 MYOD1 MYOG
28 cardiac tamponade 10.5 MYOD1 TSFM
29 seminal vesicle cystadenoma 10.5 ENO2 MYCN
30 immunodeficiency, centromere instability and facial anomalies syndrome 10.5 EZH2 MYOD1
31 testis rhabdomyosarcoma 10.5 MYOD1 MYOG
32 primary pulmonary lymphoma 10.4 MYOD1 MYOG
33 pharynx carcinoma in situ 10.4 ENO2 EWSR1 MYCN
34 hyperandrogenism 10.4 CD99 ENO2 MYCN
35 demyelinating disease 10.3 B3GAT1 ENO2
36 fallopian tube adenomatoid tumor 10.3 ENO2 MYCN
37 lung benign neoplasm 10.3 B3GAT1 CD99 ENO2 EWSR1
38 lung clear cell-sugar-tumor 10.3 EWSR1 MYOD1 MYOG
39 enamel hypoplasia cataract hydrocephaly 10.3 MYOD1 MYOG S100B
40 pituitary dwarfism with large sella turcica 10.3 ENO2 S100B
41 adenosarcoma 10.2 ENO2 MYOG S100B
42 supratentorial primitive neuroectodermal tumor 10.2 CD99 CENPV ENO2
43 nodular tenosynovitis 10.2 B3GAT1 MYOD1 MYOG
44 sarcoma 10.2
45 small cell sarcoma 10.2 CD99 ENO2 EWSR1 MYCN
46 bladder urachal squamous cell carcinoma 10.2 B3GAT1 MYOG S100B
47 lateral medullary syndrome 10.1 MYOD1 MYOG
48 cutaneous liposarcoma 10.1 MYOD1 MYOG
49 vulvar keratoacanthoma-like carcinoma 10.1 BSG ENO2 MYCN S100B
50 ethmoid sinus schneiderian papilloma 10.0 B3GAT1 ENO2

Graphical network of the top 20 diseases related to Extraosseous Ewing's Sarcoma:



Diseases related to Extraosseous Ewing's Sarcoma

Symptoms & Phenotypes for Extraosseous Ewing's Sarcoma

UMLS symptoms related to Extraosseous Ewing's Sarcoma:


fever

MGI Mouse Phenotypes related to Extraosseous Ewing's Sarcoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 mortality/aging MP:0010768 10.15 BSG ETV4 EZH2 FANCL KIDINS220 MYCN
2 growth/size/body region MP:0005378 10.14 BSG ENO2 EZH2 FANCL KIDINS220 MYCN
3 embryo MP:0005380 10.03 ETV4 EZH2 FANCL MYCN MYF5 MYF6
4 muscle MP:0005369 9.87 ETV4 EZH2 MYCN MYF5 MYF6 MYOD1
5 no phenotypic analysis MP:0003012 9.76 ETV4 EZH2 KIDINS220 MYCN MYF5 MYF6
6 neoplasm MP:0002006 9.73 ETV4 EZH2 MYF5 MYF6 MYOD1 SUZ12
7 respiratory system MP:0005388 9.5 BSG ENO2 MYCN MYF5 MYF6 MYOD1
8 skeleton MP:0005390 9.28 MYOG SUZ12 ETV4 EZH2 FANCL MYCN

Drugs & Therapeutics for Extraosseous Ewing's Sarcoma

Drugs for Extraosseous Ewing's Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 58)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
2
Vincristine Approved, Investigational Phase 3,Phase 2 2068-78-2, 57-22-7 5978
3
Etoposide Approved Phase 3,Phase 2 33419-42-0 36462
4
Topotecan Approved, Investigational Phase 3,Phase 2 119413-54-6, 123948-87-8 60700
5
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
6
Ifosfamide Approved Phase 3,Phase 2 3778-73-2 3690
7
Idarubicin Approved Phase 3 58957-92-9 42890
8
Mechlorethamine Approved Phase 3 51-75-2 4033
9
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
10
Cyproheptadine Approved Phase 3 129-03-3 2913
11
Doxil Approved June 1999 Phase 3,Phase 2 31703
12 Trofosfamide Investigational Phase 3 22089-22-1
13 Isophosphamide mustard Phase 3,Phase 2
14 Alkylating Agents Phase 3,Phase 2
15 topoisomerase I inhibitors Phase 3,Phase 2
16 Topoisomerase Inhibitors Phase 3,Phase 2
17 Anti-Bacterial Agents Phase 3,Phase 2
18 Etoposide phosphate Phase 3,Phase 2
19 Immunosuppressive Agents Phase 3,Phase 2
20 Antibiotics, Antitubercular Phase 3,Phase 2
21 Antirheumatic Agents Phase 3,Phase 2
22 Antimitotic Agents Phase 3,Phase 2
23 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
24
Histamine Phosphate Phase 3 51-74-1 65513
25
Serotonin Phase 3 50-67-9 5202
26 Serotonin Agents Phase 3
27 Serotonin Antagonists Phase 3
28 Gastrointestinal Agents Phase 3
29 Neurotransmitter Agents Phase 3
30 Histamine Antagonists Phase 3
31 Histamine H1 Antagonists Phase 3
32 Dermatologic Agents Phase 3
33 Anti-Allergic Agents Phase 3
34 Antipruritics Phase 3
35
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
36
Glycerol Approved, Investigational Phase 2 56-81-5 753
37
Lenograstim Approved Phase 2 135968-09-1
38
Bevacizumab Approved, Investigational Phase 2 216974-75-3
39
Dactinomycin Approved Phase 2 50-76-0 2019 457193
40
Epirubicin Approved Phase 2 56420-45-2 41867
41 razoxane Phase 2
42 Acetylmuramyl-Alanyl-Isoglutamine Phase 2
43 Adjuvants, Immunologic Phase 2
44 Nucleic Acid Synthesis Inhibitors Phase 2
45 Angiogenesis Inhibitors Phase 2
46 Angiogenesis Modulating Agents Phase 2
47 Pharmaceutical Solutions Phase 2
48 Anti-Infective Agents Phase 2
49 Immunoglobulins Phase 2
50 Antibodies, Monoclonal Phase 2

Interventional clinical trials:

(show all 11)

id Name Status NCT ID Phase Drugs
1 Trial for Localised High-risk Rhabdomyosarcoma and Rhabdomyosarcoma-like Soft Tissue Sarcoma Recruiting NCT00876031 Phase 3 trofosfamide, idarubicin, etoposide
2 Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma Active, not recruiting NCT01231906 Phase 3 vincristine sulfate;doxorubicin hydrochloride;cyclophosphamide;ifosfamide;etoposide;topotecan hydrochloride
3 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
4 Vincristine Sulfate, Topotecan Hydrochloride, and Cyclophosphamide With or Without Bevacizumab in Treating Young Patients With Refractory or First Recurrent Extracranial Ewing Sarcoma Completed NCT00516295 Phase 2 topotecan hydrochloride;vincristine sulfate;cyclophosphamide
5 Study of Zalypsis® (PM00104) in Patients With Unresectable Locally Advanced and/or Metastatic Ewing Family of Tumors (EFT) Progressing After at Least One Prior Line of Chemotherapy Completed NCT01222767 Phase 2 Zalypsis
6 Combination Chemotherapy and Biological Therapy in Treating Patients With High-Risk Ewing's Sarcoma Completed NCT00003667 Phase 2 cyclophosphamide;dexrazoxane hydrochloride;disaccharide tripeptide glycerol dipalmitoyl;doxorubicin hydrochloride;vincristine sulfate
7 Chemotherapy in Treating Children With Recurrent or Refractory Sarcomas Completed NCT00003745 Phase 2 topotecan hydrochloride
8 Combination Chemotherapy in Treating Children With Metastatic Rhabdomyosarcoma or Other Malignant Mesenchymal Tumors Completed NCT00025441 Phase 2 carboplatin;cyclophosphamide;doxorubicin hydrochloride;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
9 18F-FLT Positron Emission Tomography and Diffusion-Weighted Magnetic Resonance Imaging in Planning Surgery and Radiation Therapy and Measuring Response in Patients With Newly Diagnosed Ewing Sarcoma Completed NCT01825902
10 Collecting and Storing Tissue From Young Patients With Cancer Recruiting NCT00898755
11 Whole-Body Radiation Therapy, Systemic Chemotherapy, and High-Dose Chemotherapy Followed By Stem Cell Rescue in Treating Patients With Poor-Risk Ewing Sarcoma Withdrawn NCT01795430 etoposide;ifosfamide;topotecan hydrochloride;busulfan;melphalan

Search NIH Clinical Center for Extraosseous Ewing's Sarcoma

Genetic Tests for Extraosseous Ewing's Sarcoma

Anatomical Context for Extraosseous Ewing's Sarcoma

MalaCards organs/tissues related to Extraosseous Ewing's Sarcoma:

39
Liver, Kidney, Bone Marrow, Bone, Pancreas, Small Intestine

Publications for Extraosseous Ewing's Sarcoma

Articles related to Extraosseous Ewing's Sarcoma:

(show all 42)
id Title Authors Year
1
Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature. ( 27610254 )
2016
2
Primary intradural extraosseous Ewing's sarcoma in a young child. ( 26780782 )
2016
3
Pazopanib for recurrent extraosseous Ewing's sarcoma of the retroperitoneum. ( 25040171 )
2014
4
Extraosseous Ewing's sarcoma arising in the perineal region (Rectovaginal Septum): A case report with respect to the usefulness of cytological examination. ( 24106051 )
2013
5
Primary intradural extraosseous Ewing's sarcoma of the lumbar spine presenting with acute bleeding. ( 23472623 )
2013
6
An Unusual Location of Extraosseous Ewing's Sarcoma. ( 23898272 )
2013
7
Metastatic extraosseous Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the kidney: 8-year durable response after induction and maintenance chemotherapy. ( 22503609 )
2012
8
Radiology-pathology conference: primary perinephric and renal extraosseous Ewing's sarcoma. ( 22226450 )
2012
9
Extraosseous Ewing's sarcoma of the pancreas. ( 21892669 )
2012
10
Spinal intradural extraosseous Ewing's sarcoma. ( 21464880 )
2011
11
Localized extraosseous Ewing's sarcoma of the chest wall resected by video-assisted thoracoscopic surgery. ( 21170637 )
2010
12
Primary Intradural Extraosseous Ewing's Sarcoma. ( 19352481 )
2009
13
Extraosseous Ewing's sarcoma / primitive neuroectodermal tumor of the sacral nerve plexus. ( 19881073 )
2009
14
Extraosseous Ewing's sarcoma. ( 19999924 )
2009
15
Extraosseous Ewing's sarcoma: 25 years later. ( 18779607 )
2008
16
Extraosseous Ewing's sarcoma. ( 17947735 )
2007
17
A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing's sarcoma of the kidney with renal vein tumor thrombus. ( 17310314 )
2007
18
Primary spinal epidural extraosseous Ewing's sarcoma mimicking a spinal abscess. ( 17786790 )
2007
19
Primitive neurectodermal tumors: a case of extraosseous Ewing's sarcoma of the small intestine and review of the literature. ( 17309187 )
2006
20
Laparoscopic removal of extraosseous Ewing's sarcoma of the kidney in a pediatric patient. ( 16494555 )
2006
21
The first report of extraosseous Ewing's sarcoma in the rectovaginal septum. ( 12400989 )
2002
22
Extraosseous Ewing's sarcoma of the kidney. ( 12187048 )
2002
23
Primary spinal epidural extraosseous Ewing's sarcoma: report of five cases and literature review. ( 11531770 )
2001
24
MR imaging of pancreatic metastasis from extraosseous Ewing's sarcoma. ( 10630390 )
2000
25
Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction. ( 10782405 )
2000
26
A case with extraosseous Ewing's sarcoma: a late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome? ( 10914053 )
2000
27
Paravertebral extraosseous Ewing's sarcoma. ( 10216655 )
1998
28
High-dose induction chemoradiotherapy followed by autologous bone marrow transplantation as consolidation therapy in rhabdomyosarcoma, extraosseous Ewing's sarcoma, and undifferentiated sarcoma. ( 9586881 )
1998
29
Primary endocervical extraosseous Ewing's sarcoma/PNET. ( 9475198 )
1998
30
Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. ( 9125062 )
1997
31
A novel chimera gene between EWS and E1A-F, encoding the adenovirus E1A enhancer-binding protein, in extraosseous Ewing's sarcoma. ( 8605035 )
1996
32
Extraosseous Ewing's sarcoma. ( 7704678 )
1995
33
Extraosseous Ewing's sarcoma in the foot. ( 7550196 )
1995
34
Primary meningeal extraosseous Ewing's sarcoma: case report. ( 7936137 )
1994
35
Patterns of differentiation in extraosseous Ewing's sarcoma cells. An in vitro study. ( 7507797 )
1994
36
Retroperitoneal extraosseous Ewing's sarcoma with renal involvement: US and MRI findings. ( 8348407 )
1993
37
Extraosseous Ewing's sarcoma: report of a case and review of literature. ( 1372194 )
1992
38
Primary spinal epidural extraosseous Ewing's sarcoma. ( 2065287 )
1991
39
Extraosseous Ewing's sarcoma. A study of 42 cases. ( 2776115 )
1989
40
Extraosseous Ewing's sarcoma. Computed tomography evaluation before and after chemotherapy. ( 2766077 )
1989
41
Pathologic features of extraosseous Ewing's sarcoma: a report from the Intergroup Rhabdomyosarcoma Study. ( 3284809 )
1988
42
Extraosseous Ewing's sarcoma versus primitive rhabdomyosarcoma: diagnostic criteria and clinical correlation. ( 3759073 )
1986

Variations for Extraosseous Ewing's Sarcoma

Expression for Extraosseous Ewing's Sarcoma

Search GEO for disease gene expression data for Extraosseous Ewing's Sarcoma.

Pathways for Extraosseous Ewing's Sarcoma

Pathways related to Extraosseous Ewing's Sarcoma according to GeneCards Suite gene sharing:

id Super pathways Score Top Affiliating Genes
1 11.11 MYF5 MYOD1 MYOG
2 10.39 EZH2 SUZ12
3 10.36 EZH2 MYF5 MYOD1

GO Terms for Extraosseous Ewing's Sarcoma

Cellular components related to Extraosseous Ewing's Sarcoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 ESC/E(Z) complex GO:0035098 8.62 EZH2 SUZ12

Biological processes related to Extraosseous Ewing's Sarcoma according to GeneCards Suite gene sharing:

(show all 19)
id Name GO ID Score Top Affiliating Genes
1 regulation of transcription from RNA polymerase II promoter GO:0006357 9.91 EZH2 MYCN MYF5 MYF6 MYOD1
2 transcription from RNA polymerase II promoter GO:0006366 9.84 ETV4 MYF5 MYF6 MYOD1
3 muscle organ development GO:0007517 9.73 MYF5 MYF6 MYOD1 MYOG
4 skeletal muscle tissue development GO:0007519 9.71 MYF5 MYF6 MYOD1 MYOG
5 skeletal muscle cell differentiation GO:0035914 9.67 MYF5 MYF6 MYOD1 MYOG
6 positive regulation of muscle cell differentiation GO:0051149 9.62 MYF5 MYF6 MYOD1 MYOG
7 histone methylation GO:0016571 9.58 EZH2 SUZ12
8 myoblast differentiation GO:0045445 9.57 MYOD1 MYOG
9 cartilage condensation GO:0001502 9.56 MYCN MYF5
10 positive regulation of myoblast differentiation GO:0045663 9.56 MYF5 MYF6 MYOD1 MYOG
11 skeletal muscle tissue regeneration GO:0043403 9.55 MYF6 MYOG
12 myotube differentiation GO:0014902 9.49 MYOD1 MYOG
13 histone H3-K27 methylation GO:0070734 9.48 EZH2 SUZ12
14 muscle tissue morphogenesis GO:0060415 9.46 MYF5 MYF6
15 positive regulation of myoblast fusion GO:1901741 9.46 MYF5 MYF6 MYOD1 MYOG
16 positive regulation of skeletal muscle fiber development GO:0048743 9.26 MYF5 MYF6 MYOD1 MYOG
17 muscle cell fate commitment GO:0042693 8.92 MYF5 MYF6 MYOD1 MYOG
18 regulation of transcription, DNA-templated GO:0006355 10.18 ETV4 EWSR1 EZH2 MYCN MYF5 MYF6
19 positive regulation of transcription from RNA polymerase II promoter GO:0045944 10.06 ETV4 MYCN MYF5 MYF6 MYOD1 MYOG

Molecular functions related to Extraosseous Ewing's Sarcoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 sequence-specific DNA binding GO:0043565 9.85 ETV4 EZH2 MYF5 MYOD1 MYOG SUZ12
2 protein dimerization activity GO:0046983 9.72 MYCN MYF5 MYF6 MYOD1 MYOG
3 transcriptional activator activity, RNA polymerase II transcription regulatory region sequence-specific binding GO:0001228 9.71 MYF5 MYF6 MYOD1 MYOG
4 RNA polymerase II core promoter proximal region sequence-specific DNA binding GO:0000978 9.65 ETV4 MYF5 MYF6 MYOD1 MYOG
5 core promoter binding GO:0001047 9.63 EZH2 MYOD1 MYOG
6 histone methyltransferase activity GO:0042054 9.48 EZH2 SUZ12
7 transcription factor activity, RNA polymerase II distal enhancer sequence-specific binding GO:0003705 9.46 MYF5 MYF6 MYOD1 MYOG
8 histone methyltransferase activity (H3-K27 specific) GO:0046976 9.43 EZH2 SUZ12
9 chromatin DNA binding GO:0031490 9.26 EZH2 MYOD1 MYOG SUZ12
10 E-box binding GO:0070888 8.92 MYF5 MYF6 MYOD1 MYOG

Sources for Extraosseous Ewing's Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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