MCID: EXT031
MIFTS: 44

Extraosseous Ewing's Sarcoma

Categories: Bone diseases, 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 15
Extraosseous Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 73
Sarcoma Extraosseous Ewings 55
Extraosseous Ewing Sarcoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:4232
NCIt 50 C27293

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 ewing sarcoma and extraskeletal 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 miRs in Muscle Cell Differentiation and Myogenesis. The drugs Vincristine and Topotecan have been mentioned in the context of this disorder. Affiliated tissues include bone, kidney and skin, 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 66)
# Related Disease Score Top Affiliating Genes
1 ewing sarcoma 32.8 CD99 ENO2 ETV4 EWSR1 MYCN MYOD1
2 extraskeletal ewing sarcoma 32.7 CD99 ENO2 EWSR1
3 rhabdomyosarcoma 29.4 ENO2 EWSR1 MYCN MYF5 MYF6 MYOD1
4 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.5
5 sarcoma 10.9
6 spindle cell sarcoma 10.5
7 bladder sarcoma 10.3 CD99 EWSR1
8 malignant triton tumor 10.3 MYOD1 S100B
9 vagina sarcoma 10.3
10 ewing's family of tumors 10.3
11 hemophagocytic lymphohistiocytosis, familial, 1 10.3 MYF6 MYOD1
12 intracranial cysts 10.3 ENO2 S100B
13 small cell osteogenic sarcoma 10.3 ENO2 EWSR1
14 bednar tumor 10.3 ENO2 S100B
15 spindle cell rhabdomyosarcoma 10.2 MYOD1 S100B
16 epithelioid malignant peripheral nerve sheath tumor 10.2 ENO2 S100B
17 ossifying fibromyxoid tumor 10.2 ENO2 S100B
18 cauda equina neoplasm 10.2 ENO2 EWSR1
19 extraosseous chondrosarcoma 10.2 ENO2 EWSR1
20 middle cerebral artery infarction 10.2 ENO2 S100B
21 head injury 10.2 ENO2 S100B
22 rhabdoid cancer 10.2 ENO2 EWSR1 MYOD1
23 connective tissue cancer 10.2 CD99 ENO2 EWSR1
24 olfactory neuroblastoma 10.2 ENO2 EWSR1 S100B
25 plexiform schwannoma 10.2 B3GAT1 ENO2
26 central nervous system mesenchymal non-meningothelial tumor 10.2 MYF6 MYOD1 TSFM
27 clear cell sarcoma 10.2 ENO2 EWSR1 S100B
28 pineocytoma 10.2 ENO2 S100B
29 alveolar soft part sarcoma 10.2 ENO2 MYF6 MYOD1
30 desmoplastic small round cell tumor 10.2 ENO2 EWSR1
31 gallbladder sarcoma 10.1 MYOD1 MYOG
32 undifferentiated embryonal sarcoma of the liver 10.1 MYOD1 MYOG
33 adrenal neuroblastoma 10.1 ENO2 MYCN
34 breast adenomyoepithelioma 10.1 CD99 ENO2
35 gastric small cell carcinoma 10.1 B3GAT1 ENO2
36 neuroblastoma 10.1
37 neurilemmoma 10.1
38 thyroid sarcoma 10.1
39 malignant mesenchymoma 10.1 EWSR1 MYOD1 MYOG
40 kidney rhabdoid cancer 10.1 B3GAT1 CD99 ENO2 EWSR1
41 autonomic nervous system benign neoplasm 10.1 ENO2 MYCN
42 adult fibrosarcoma 10.1 MYOD1 MYOG
43 embryonal sarcoma 10.1 MYOD1 MYOG S100B
44 leiomyosarcoma 10.1 ENO2 MYOG S100B
45 breast myoepithelial neoplasm 10.1 CD99 ENO2
46 askin's tumor 10.1 ENO2 EWSR1 MYCN
47 pericardium cancer 10.1 CD99 ENO2 MYCN
48 nodular ganglioneuroblastoma 10.1 ENO2 MYCN
49 nervous system cancer 10.1 ENO2 EWSR1 MYCN
50 spindle cell liposarcoma 10.0 MYOD1 MYOG

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 mortality/aging MP:0010768 10.18 B3GAT1 BSG ETV4 EZH2 FANCL KIDINS220
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 ETV4 EZH2 FANCL MYCN MYF5 MYF6

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 61)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vincristine Approved, Investigational Phase 3,Phase 2 57-22-7, 2068-78-2 5978
2
Topotecan Approved, Investigational Phase 3,Phase 2,Not Applicable 123948-87-8, 119413-54-6 60700
3
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
4
Ifosfamide Approved Phase 3,Phase 2,Not Applicable 3778-73-2 3690
5
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 6055-19-2, 50-18-0 2907
6
Etoposide Approved Phase 3,Phase 2,Not Applicable 33419-42-0 36462
7
Mechlorethamine Approved, Investigational Phase 3,Not Applicable 51-75-2 4033
8
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
9
Cyproheptadine Approved Phase 3 129-03-3 2913
10
Doxil Approved June 1999 Phase 3,Phase 2 31703
11
Isophosphamide mustard Phase 3,Phase 2,Not Applicable 0
12 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Not Applicable
13 Alkylating Agents Phase 3,Phase 2,Not Applicable
14 topoisomerase I inhibitors Phase 3,Phase 2,Not Applicable
15 Antimitotic Agents Phase 3,Phase 2
16 Antirheumatic Agents Phase 3,Phase 2
17 Immunologic Factors Phase 3,Phase 2,Not Applicable
18 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Not Applicable
19 Etoposide phosphate Phase 3,Phase 2,Not Applicable
20 Anti-Bacterial Agents Phase 3,Phase 2
21 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
22 Antibiotics, Antitubercular Phase 3,Phase 2
23 Immunosuppressive Agents Phase 3,Phase 2,Not Applicable
24 Dermatologic Agents Phase 3
25 Keratolytic Agents Phase 3
26 Podophyllotoxin Phase 3 518-28-5
27 Serotonin Agents Phase 3
28 Anti-Allergic Agents Phase 3
29 Serotonin Antagonists Phase 3
30 Histamine H1 Antagonists Phase 3
31
Histamine Phosphate Phase 3 51-74-1 65513
32 Antipruritics Phase 3
33 Neurotransmitter Agents Phase 3
34 Histamine Antagonists Phase 3
35 Gastrointestinal Agents Phase 3
36
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
37
Bevacizumab Approved, Investigational Phase 2 216974-75-3
38
Glycerol Approved, Investigational Phase 2 56-81-5 753
39
Lenograstim Approved, Investigational Phase 2 135968-09-1
40
Dactinomycin Approved, Investigational Phase 2 50-76-0 457193 2019
41
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
42
Epirubicin Approved Phase 2 56420-45-2 41867
43 Antibodies Phase 2
44 Immunoglobulins Phase 2
45 Antibodies, Monoclonal Phase 2
46 Angiogenesis Modulating Agents Phase 2
47 Angiogenesis Inhibitors Phase 2
48 Pharmaceutical Solutions Phase 2
49 Protective Agents Phase 2
50 Adjuvants, Immunologic Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma Active, not recruiting NCT01231906 Phase 3 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Ifosfamide;Topotecan Hydrochloride;Vincristine Sulfate
2 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
3 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
4 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
5 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
6 Chemotherapy in Treating Children With Recurrent or Refractory Sarcomas Completed NCT00003745 Phase 2 topotecan hydrochloride
7 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
8 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 Early Phase 1 fluorine F 18 fluorothymidine
9 Collecting and Storing Tissue From Young Patients With Cancer Completed NCT00898755
10 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 Not Applicable 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:

41
Bone, Kidney, Skin, Brain, Liver, Breast, Skeletal Muscle

Publications for Extraosseous Ewing's Sarcoma

Articles related to Extraosseous Ewing's Sarcoma:

(show all 44)
# Title Authors Year
1
Primary intracranial extraosseous Ewing's sarcoma. ( 30353305 )
2018
2
Extraosseous Ewing's Sarcoma of the Pancreas: An Uncommon but Treatable Disease. ( 29387511 )
2017
3
Primary intradural extraosseous Ewing's sarcoma in a young child. ( 26780782 )
2016
4
Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature. ( 27610254 )
2016
5
Pazopanib for recurrent extraosseous Ewing's sarcoma of the retroperitoneum. ( 25040171 )
2014
6
Primary intradural extraosseous Ewing's sarcoma of the lumbar spine presenting with acute bleeding. ( 23472623 )
2013
7
Extraosseous Ewing's sarcoma arising in the perineal region (Rectovaginal Septum): A case report with respect to the usefulness of cytological examination. ( 24106051 )
2013
8
An Unusual Location of Extraosseous Ewing's Sarcoma. ( 23898272 )
2013
9
Extraosseous Ewing's sarcoma of the pancreas. ( 21892669 )
2012
10
Radiology-pathology conference: primary perinephric and renal extraosseous Ewing's sarcoma. ( 22226450 )
2012
11
Metastatic extraosseous Ewing's sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the kidney: 8-year durable response after induction and maintenance chemotherapy. ( 22503609 )
2012
12
Spinal intradural extraosseous Ewing's sarcoma. ( 21464880 )
2011
13
Localized extraosseous Ewing's sarcoma of the chest wall resected by video-assisted thoracoscopic surgery. ( 21170637 )
2010
14
Extraosseous Ewing's sarcoma. ( 19999924 )
2009
15
Extraosseous Ewing's sarcoma / primitive neuroectodermal tumor of the sacral nerve plexus. ( 19881073 )
2009
16
Primary Intradural Extraosseous Ewing's Sarcoma. ( 19352481 )
2009
17
Extraosseous Ewing's sarcoma: 25 years later. ( 18779607 )
2008
18
Extraosseous Ewing's sarcoma. ( 17947735 )
2007
19
Primary spinal epidural extraosseous Ewing's sarcoma mimicking a spinal abscess. ( 17786790 )
2007
20
A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing's sarcoma of the kidney with renal vein tumor thrombus. ( 17310314 )
2007
21
Primitive neurectodermal tumors: a case of extraosseous Ewing's sarcoma of the small intestine and review of the literature. ( 17309187 )
2006
22
Laparoscopic removal of extraosseous Ewing's sarcoma of the kidney in a pediatric patient. ( 16494555 )
2006
23
Extraosseous Ewing's sarcoma of the kidney. ( 12187048 )
2002
24
The first report of extraosseous Ewing's sarcoma in the rectovaginal septum. ( 12400989 )
2002
25
Primary spinal epidural extraosseous Ewing's sarcoma: report of five cases and literature review. ( 11531770 )
2001
26
MR imaging of pancreatic metastasis from extraosseous Ewing's sarcoma. ( 10630390 )
2000
27
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
28
Primary vulvar and vaginal extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction. ( 10782405 )
2000
29
High-dose induction chemoradiotherapy followed by autologous bone marrow transplantation as consolidation therapy in rhabdomyosarcoma, extraosseous Ewing's sarcoma, and undifferentiated sarcoma. ( 9586881 )
1998
30
Paravertebral extraosseous Ewing's sarcoma. ( 10216655 )
1998
31
Primary endocervical extraosseous Ewing's sarcoma/PNET. ( 9475198 )
1998
32
Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. ( 9125062 )
1997
33
A novel chimera gene between EWS and E1A-F, encoding the adenovirus E1A enhancer-binding protein, in extraosseous Ewing's sarcoma. ( 8605035 )
1996
34
Extraosseous Ewing's sarcoma in the foot. ( 7550196 )
1995
35
Extraosseous Ewing's sarcoma. ( 7704678 )
1995
36
Primary meningeal extraosseous Ewing's sarcoma: case report. ( 7936137 )
1994
37
Patterns of differentiation in extraosseous Ewing's sarcoma cells. An in vitro study. ( 7507797 )
1994
38
Retroperitoneal extraosseous Ewing's sarcoma with renal involvement: US and MRI findings. ( 8348407 )
1993
39
Extraosseous Ewing's sarcoma: report of a case and review of literature. ( 1372194 )
1992
40
Primary spinal epidural extraosseous Ewing's sarcoma. ( 2065287 )
1991
41
Extraosseous Ewing's sarcoma. A study of 42 cases. ( 2776115 )
1989
42
Extraosseous Ewing's sarcoma. Computed tomography evaluation before and after chemotherapy. ( 2766077 )
1989
43
Pathologic features of extraosseous Ewing's sarcoma: a report from the Intergroup Rhabdomyosarcoma Study. ( 3284809 )
1988
44
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:

# Super pathways Score Top Affiliating Genes
1 10.69 EZH2 MYF5 MYOD1
2
Show member pathways
10.69 MYF5 MYF6 MYOD1 MYOG
3 10.39 EZH2 SUZ12

GO Terms for Extraosseous Ewing's Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.77 CENPV ETV4 EWSR1 EZH2 FANCL MYCN
2 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 18)
# Name GO ID Score Top Affiliating Genes
1 transcription by RNA polymerase II GO:0006366 9.92 ETV4 MYCN MYF5 MYF6 MYOD1
2 muscle organ development GO:0007517 9.73 MYF5 MYF6 MYOD1 MYOG
3 skeletal muscle tissue development GO:0007519 9.71 MYF5 MYF6 MYOD1 MYOG
4 skeletal muscle cell differentiation GO:0035914 9.67 MYF5 MYF6 MYOD1 MYOG
5 positive regulation of muscle cell differentiation GO:0051149 9.62 MYF5 MYF6 MYOD1 MYOG
6 skeletal muscle fiber development GO:0048741 9.58 MYOD1 MYOG
7 skeletal muscle tissue regeneration GO:0043403 9.57 MYF6 MYOG
8 positive regulation of myoblast differentiation GO:0045663 9.56 MYF5 MYF6 MYOD1 MYOG
9 histone methylation GO:0016571 9.55 EZH2 SUZ12
10 myoblast differentiation GO:0045445 9.54 MYOD1 MYOG
11 myotube differentiation GO:0014902 9.51 MYOD1 MYOG
12 histone H3-K27 methylation GO:0070734 9.49 EZH2 SUZ12
13 muscle tissue morphogenesis GO:0060415 9.48 MYF5 MYF6
14 positive regulation of myoblast fusion GO:1901741 9.46 MYF5 MYF6 MYOD1 MYOG
15 positive regulation of skeletal muscle fiber development GO:0048743 9.26 MYF5 MYF6 MYOD1 MYOG
16 muscle cell fate commitment GO:0042693 8.92 MYF5 MYF6 MYOD1 MYOG
17 regulation of transcription, DNA-templated GO:0006355 10.06 ETV4 EWSR1 EZH2 MYCN MYF5 MYF6
18 positive regulation of transcription by RNA polymerase II GO:0045944 10.03 ETV4 MYCN MYF5 MYF6 MYOD1 MYOG

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 sequence-specific DNA binding GO:0043565 9.85 ETV4 EZH2 MYF5 MYOD1 MYOG SUZ12
2 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.76 MYF5 MYF6 MYOD1 MYOG
3 protein dimerization activity GO:0046983 9.72 MYCN MYF5 MYF6 MYOD1 MYOG
4 promoter-specific chromatin binding GO:1990841 9.58 EZH2 MYOD1 SUZ12
5 transcription factor activity, RNA polymerase II distal enhancer sequence-specific binding GO:0003705 9.56 MYF5 MYF6 MYOD1 MYOG
6 histone methyltransferase activity GO:0042054 9.48 EZH2 SUZ12
7 chromatin DNA binding GO:0031490 9.46 EZH2 MYOD1 MYOG SUZ12
8 histone methyltransferase activity (H3-K27 specific) GO:0046976 9.43 EZH2 SUZ12
9 E-box binding GO:0070888 9.26 MYF5 MYF6 MYOD1 MYOG
10 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.23 ETV4 EZH2 MYCN MYF5 MYF6 MYOD1
11 DNA-binding transcription factor activity, RNA polymerase II-specific GO:0000981 10.04 ETV4 EZH2 MYCN MYF5 MYF6 MYOD1

Sources for Extraosseous Ewing's Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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