MCID: EXT065
MIFTS: 40

Extraosseous Ewing Sarcoma

Categories: Bone diseases, Cancer diseases, Neuronal diseases

Aliases & Classifications for Extraosseous Ewing Sarcoma

MalaCards integrated aliases for Extraosseous Ewing Sarcoma:

Name: Extraosseous Ewing Sarcoma 12
Extraosseous Ewing's Sarcoma 12 15
Extraosseous Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Sarcoma Extraosseous Ewings 55
Extraskeletal Ewing Sarcom 12

Classifications:



External Ids:

Disease Ontology 12 DOID:4232
NCIt 50 C27293
UMLS 72 C1333514

Summaries for Extraosseous Ewing Sarcoma

Disease Ontology : 12 A Ewing sarcoma that is morphologically indistinguishable from skeletal Ewing sarcoma but is located in extraosseous locations.

MalaCards based summary : Extraosseous Ewing Sarcoma, also known as extraosseous ewing's sarcoma, is related to extraskeletal ewing sarcoma and ewing sarcoma. An important gene associated with Extraosseous Ewing Sarcoma is EWSR1 (EWS RNA Binding Protein 1), and among its related pathways/superpathways are Immune response Function of MEF2 in T lymphocytes and Interactome of polycomb repressive complex 2 (PRC2). The drugs Histamine and Cyproheptadine have been mentioned in the context of this disorder. Affiliated tissues include bone, kidney and lung, and related phenotypes are no effect and behavior/neurological

Related Diseases for Extraosseous Ewing Sarcoma

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

(show top 50) (show all 96)
# Related Disease Score Top Affiliating Genes
1 extraskeletal ewing sarcoma 33.9 EWSR1 ENO2 CD99
2 ewing sarcoma 32.2 S100B MYOG MYOD1 MYCN EWSR1 ETV4
3 askin's tumor 30.6 MYCN EWSR1 ENO2
4 embryonal sarcoma 30.6 S100B MYOG MYOD1
5 rhabdomyosarcoma 28.6 MYOG MYOD1 MYF6 MYF5 MYCN EWSR1
6 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.6
7 sarcoma 10.9
8 spindle cell sarcoma 10.9
9 bladder sarcoma 10.7 EWSR1 CD99
10 malignant triton tumor 10.7 S100B MYOD1
11 intracranial cysts 10.7 S100B ENO2
12 small cell osteogenic sarcoma 10.6 EWSR1 ENO2
13 bednar tumor 10.6 S100B ENO2
14 hemophagocytic lymphohistiocytosis, familial, 1 10.6 MYOD1 MYF6
15 ewing's family of tumors 10.6
16 spindle cell rhabdomyosarcoma 10.6 S100B MYOD1
17 epithelioid malignant peripheral nerve sheath tumor 10.6 S100B ENO2
18 cauda equina neoplasm 10.6 EWSR1 ENO2
19 ossifying fibromyxoid tumor 10.6 S100B ENO2
20 extraosseous chondrosarcoma 10.5 EWSR1 ENO2
21 head injury 10.5 S100B ENO2
22 rhabdoid cancer 10.5 MYOD1 EWSR1 ENO2
23 myxoid chondrosarcoma 10.5 S100B EWSR1
24 connective tissue cancer 10.5 EWSR1 ENO2 CD99
25 middle cerebral artery infarction 10.5 S100B ENO2
26 olfactory neuroblastoma 10.5 S100B EWSR1 ENO2
27 central nervous system mesenchymal non-meningothelial tumor 10.5 TSFM MYOD1 MYF6
28 clear cell sarcoma 10.5 S100B EWSR1 ENO2
29 alveolar soft part sarcoma 10.4 MYOD1 MYF6 ENO2
30 plexiform schwannoma 10.4 ENO2 B3GAT1
31 breast rhabdomyosarcoma 10.4 MYOG MYOD1
32 adrenal neuroblastoma 10.4 MYCN ENO2
33 pineocytoma 10.4 S100B ENO2
34 undifferentiated embryonal sarcoma of the liver 10.3 MYOG MYOD1
35 gallbladder sarcoma 10.3 MYOG MYOD1
36 pericardium cancer 10.3 MYCN ENO2 CD99
37 kidney rhabdoid cancer 10.2 EWSR1 ENO2 CD99 B3GAT1
38 leiomyosarcoma 10.2 S100B MYOG ENO2
39 malignant mesenchymoma 10.2 MYOG MYOD1 EWSR1
40 nervous system cancer 10.2 MYCN EWSR1 ENO2
41 desmoplastic small round cell tumor 10.2 EWSR1 ENO2
42 nodular ganglioneuroblastoma 10.2 MYCN ENO2
43 autonomic nervous system benign neoplasm 10.2 MYCN ENO2
44 li-fraumeni syndrome 10.1
45 meningioma, radiation-induced 10.1
46 meningioma, familial 10.1
47 li-fraumeni syndrome 2 10.1
48 beta-thalassemia 10.1
49 connective tissue benign neoplasm 10.1
50 thalassemia 10.1

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



Diseases related to Extraosseous Ewing Sarcoma

Symptoms & Phenotypes for Extraosseous Ewing Sarcoma

GenomeRNAi Phenotypes related to Extraosseous Ewing Sarcoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 no effect GR00402-S-1 9.92 B3GAT1 BSG C1orf56 CD99 CENPV CTNS

MGI Mouse Phenotypes related to Extraosseous Ewing Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.22 B3GAT1 C1orf56 CTNS ENO2 ETV4 EZH2
2 mortality/aging MP:0010768 10.21 B3GAT1 BSG ETV4 EZH2 FANCL MYCN
3 embryo MP:0005380 10.11 ETV4 EZH2 FANCL LCK MYCN MYF5
4 muscle MP:0005369 10.01 CTNS ETV4 EZH2 MYCN MYF5 MYF6
5 no phenotypic analysis MP:0003012 9.87 ETV4 EZH2 MYCN MYF5 MYF6 MYOD1
6 normal MP:0002873 9.86 C1orf56 EZH2 MYCN MYF5 MYF6 MYOD1
7 neoplasm MP:0002006 9.85 ETV4 EZH2 MYF5 MYF6 MYOD1 SUZ12
8 reproductive system MP:0005389 9.76 B3GAT1 BSG C1orf56 ETV4 FANCL LCK
9 respiratory system MP:0005388 9.5 BSG ENO2 MYCN MYF5 MYF6 MYOD1
10 skeleton MP:0005390 9.32 CTNS ETV4 EZH2 FANCL MYCN MYF5

Drugs & Therapeutics for Extraosseous Ewing Sarcoma

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

(show all 36)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Histamine Approved, Investigational Phase 3 51-45-6 774
2
Cyproheptadine Approved Phase 3 129-03-3 2913
3 Gastrointestinal Agents Phase 3
4 Neurotransmitter Agents Phase 3
5 Anti-Allergic Agents Phase 3
6 Serotonin Antagonists Phase 3
7 Histamine H1 Antagonists Phase 3
8 Histamine Antagonists Phase 3
9 Dermatologic Agents Phase 3
10 Serotonin Agents Phase 3
11
Histamine Phosphate Phase 3 51-74-1 65513
12 Antipruritics Phase 3
13
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
14
Topotecan Approved, Investigational Phase 2 123948-87-8, 119413-54-6 60700
15
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
16
Glycerol Approved, Investigational Phase 2 56-81-5 753
17
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
18
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
19 topoisomerase I inhibitors Phase 2
20 Topoisomerase Inhibitors Phase 2
21 Tubulin Modulators Phase 2
22 razoxane Phase 2
23 Alkylating Agents Phase 2
24
Liposomal doxorubicin Phase 2 31703
25 Antineoplastic Agents, Phytogenic Phase 2
26 Acetylmuramyl-Alanyl-Isoglutamine Phase 2
27 Cardiotonic Agents Phase 2
28 Anti-Bacterial Agents Phase 2
29 Immunosuppressive Agents Phase 2
30 Antibiotics, Antitubercular Phase 2
31 Protective Agents Phase 2
32 Adjuvants, Immunologic Phase 2
33 Immunologic Factors Phase 2
34 Antimitotic Agents Phase 2
35 Antirheumatic Agents Phase 2
36 Antineoplastic Agents, Alkylating Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
2 A Phase II Study of Continuous 21 Day Infusion of Topotecan (NSC # 609699) in Children With Relapsed Solid Tumors Completed NCT00003745 Phase 2 topotecan hydrochloride
3 Randomized Phase II Study of Vincristine, Doxorubicin, Cyclophosphamide and Dexrazoxane With and Without ImmTher for Newly Diagnosed High Risk Ewing's Sarcoma Completed NCT00003667 Phase 2 cyclophosphamide;dexrazoxane hydrochloride;disaccharide tripeptide glycerol dipalmitoyl;doxorubicin hydrochloride;vincristine sulfate

Search NIH Clinical Center for Extraosseous Ewing Sarcoma

Genetic Tests for Extraosseous Ewing Sarcoma

Anatomical Context for Extraosseous Ewing Sarcoma

MalaCards organs/tissues related to Extraosseous Ewing Sarcoma:

41
Bone, Kidney, Lung, Bone Marrow, Pancreas, Spinal Cord, Thyroid

Publications for Extraosseous Ewing Sarcoma

Articles related to Extraosseous Ewing Sarcoma:

(show top 50) (show all 171)
# Title Authors PMID Year
1
The first report of extraosseous Ewing's sarcoma in the rectovaginal septum. 9 38
12400989 2002
2
A novel chimera gene between EWS and E1A-F, encoding the adenovirus E1A enhancer-binding protein, in extraosseous Ewing's sarcoma. 9 38
8605035 1996
3
Myogenic regulatory protein (MyoD1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma. 9 38
2260621 1990
4
An Extraosseous Ewing Sarcoma of the Index Finger Masquerading as a Benign Tumor. 38
31327500 2019
5
Ewing Sarcoma Presenting as a Congenital Scalp Mass. 38
29989837 2019
6
Primary intracranial extraosseous Ewing's sarcoma. 38
30353305 2019
7
Primary intradural Ewing's sarcoma of the spine: a systematic review of the literature. 38
30579048 2019
8
[Primary extra-osseous Ewing sarcoma of the dorsal spine with spinal cord compression at diagnosis: Case report]. 38
30652454 2019
9
Extraosseous Ewing's Sarcoma of the Parapharyngeal Space - A Rare Entity - with Review of Literature. 38
30783599 2019
10
[Extraosseous Ewing sarcoma]. 38
31284263 2019
11
Rhabdomyosarcoma and Extraosseous Ewing Sarcoma. 38
30544742 2018
12
Augmented reality in computer-assisted interventions based on patient-specific 3D printed reference. 38
30464847 2018
13
Extraosseous Ewing sarcoma arising in a chronically lymphedematous limb. 38
30148241 2018
14
The Successful Treatment of Metastatic Extraosseous Ewing Sarcoma with Pazopanib. 38
29780156 2018
15
Extra-Osseous Ewing Sarcoma of Sciatic Nerve Masquerading as Benign Nerve Sheath Tumor and Presented as Lumbar Radiculopathy: Case Report and Review of Literature. 38
29673820 2018
16
Peripheral primitive neuroendocrine tumor of the chest wall-A case report with pathological correlation. 38
29904480 2018
17
Extraosseous Ewing sarcoma arising at the papilla of Vater. 38
29557408 2018
18
Lower Extremity Weakness in an Adolescent Female - A Rare Presentation of Ewing Sarcoma. 38
30559628 2018
19
Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature. 38
29804110 2018
20
Extraosseous Ewing's Sarcoma of the Pancreas: An Uncommon but Treatable Disease. 38
29387511 2017
21
Extraosseous Ewing sarcoma: Expanding the differential diagnosis of supraclavicular fossa tumors. 38
28122109 2017
22
Extraosseous Ewing sarcoma in the lumbar epidural space: an uncommon malignant tumor mimicking a spinal schwannoma. 38
26968238 2016
23
The Importance of Surgery as Part of Multimodal Therapy in Rapid Progressive Primary Extraosseous Ewing Sarcoma of the Cervical Intra- and Epidural Space. 38
28176976 2016
24
Primary intradural extraosseous Ewing's sarcoma in a young child. 38
26780782 2016
25
Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management. 38
27186040 2016
26
High EZH2 expression is correlated to metastatic disease in pediatric soft tissue sarcomas. 38
27471434 2016
27
Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature. 38
27610254 2016
28
Primary Ewing Sarcoma Presenting as a Vulvar Mass in an Adolescent: Case Report and Review of Literature. 38
26211932 2015
29
Extraosseous Ewing sarcoma in the mesentery: the first report of cases in children. 38
26280743 2015
30
Intradural extramedullary extraosseous Ewing sarcoma/PNET of foramen magnum. 38
25092552 2015
31
[Primary presentation of extraosseous Ewing's sarcoma]. 38
25002348 2015
32
Primary extraosseous Ewing sarcoma of the lumbar spine presenting with left leg weakness. 38
25724431 2015
33
Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma. 38
26266019 2015
34
Extraskeletal Ewing's Sarcoma Arising from the Sciatic Nerve: A Diagnostic Challenge. 38
26236533 2015
35
Pazopanib for recurrent extraosseous Ewing's sarcoma of the retroperitoneum. 38
25040171 2014
36
Evaluation of outcome and prognostic factors in extraosseous Ewing sarcoma. 38
25132242 2014
37
Extraosseous Ewing's sarcoma arising in the perineal region (Rectovaginal Septum): a case report with respect to the usefulness of cytological examination. 38
24106051 2014
38
Appearance of extraosseous pelvic Ewing sarcoma on triphasic bone scan. 38
24566405 2014
39
Primary Ewing's Sarcoma of the Lung. 38
24570867 2014
40
Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis. 38
24678438 2014
41
Primary intradural extraosseous Ewing's sarcoma of the lumbar spine presenting with acute bleeding. 38
23472623 2013
42
A rare case of extraosseous Ewing sarcoma primarily arising in the ovary. 38
24286435 2013
43
Role of color-Doppler US in the evaluation of scrotal edema. 38
24432179 2013
44
An Unusual Location of Extraosseous Ewing's Sarcoma. 38
23898272 2013
45
Primary pancreatic Ewing's sarcoma with portal vein tumor thrombosis. 38
23192427 2013
46
Primary extraosseous Ewing sarcoma of the orbit. 38
23247035 2013
47
Extraosseous Ewing sarcoma and peripheral primitive neuroectodermal tumor of the thyroid gland: Case report and review. 38
23599116 2013
48
Extraosseous Ewing sarcoma of the vagina: a rare entity. 38
23563009 2013
49
Retraction: Primary extraosseous Ewing sarcoma of the lung in children. 38
23858324 2013
50
Primary pediatric endobronchial Ewing sarcoma family of tumors. 38
23569566 2013

Variations for Extraosseous Ewing Sarcoma

Expression for Extraosseous Ewing Sarcoma

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

Pathways for Extraosseous Ewing Sarcoma

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.41 MYOG MYOD1 MYF6 LCK
2 10.39 SUZ12 EZH2
3 10.36 MYOD1 MYF5 EZH2

GO Terms for Extraosseous Ewing Sarcoma

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

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

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 muscle organ development GO:0007517 9.73 MYOG MYOD1 MYF6 MYF5
2 skeletal muscle tissue development GO:0007519 9.71 MYOG MYOD1 MYF6 MYF5
3 skeletal muscle cell differentiation GO:0035914 9.67 MYOG MYOD1 MYF6 MYF5
4 positive regulation of muscle cell differentiation GO:0051149 9.62 MYOG MYOD1 MYF6 MYF5
5 skeletal muscle tissue regeneration GO:0043403 9.56 MYOG MYF6
6 positive regulation of myoblast differentiation GO:0045663 9.56 MYOG MYOD1 MYF6 MYF5
7 histone methylation GO:0016571 9.55 SUZ12 EZH2
8 myoblast differentiation GO:0045445 9.54 MYOG MYOD1
9 myotube differentiation GO:0014902 9.51 MYOG MYOD1
10 muscle tissue morphogenesis GO:0060415 9.48 MYF6 MYF5
11 histone H3-K27 methylation GO:0070734 9.46 SUZ12 EZH2
12 positive regulation of myoblast fusion GO:1901741 9.46 MYOG MYOD1 MYF6 MYF5
13 positive regulation of skeletal muscle fiber development GO:0048743 9.26 MYOG MYOD1 MYF6 MYF5
14 muscle cell fate commitment GO:0042693 8.92 MYOG MYOD1 MYF6 MYF5
15 regulation of transcription, DNA-templated GO:0006355 10.1 MYOG MYOD1 MYF6 MYF5 MYCN EZH2
16 positive regulation of transcription by RNA polymerase II GO:0045944 10.04 MYOG MYOD1 MYF6 MYF5 MYCN ETV4
17 cell differentiation GO:0030154 10 MYOG MYOD1 MYF6 MYF5 LCK ETV4

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

# Name GO ID Score Top Affiliating Genes
1 sequence-specific DNA binding GO:0043565 9.88 SUZ12 MYOG MYOD1 MYF5 EZH2 ETV4
2 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.73 MYOG MYOD1 MYF6 MYF5
3 protein dimerization activity GO:0046983 9.65 MYOG MYOD1 MYF6 MYF5 MYCN
4 promoter-specific chromatin binding GO:1990841 9.5 SUZ12 MYOD1 EZH2
5 histone methyltransferase activity GO:0042054 9.46 SUZ12 EZH2
6 chromatin DNA binding GO:0031490 9.46 SUZ12 MYOG MYOD1 EZH2
7 histone methyltransferase activity (H3-K27 specific) GO:0046976 9.37 SUZ12 EZH2
8 E-box binding GO:0070888 9.26 MYOG MYOD1 MYF6 MYF5
9 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.23 SUZ12 MYOG MYOD1 MYF6 MYF5 MYCN
10 DNA-binding transcription factor activity, RNA polymerase II-specific GO:0000981 10.02 MYOG MYOD1 MYF6 MYF5 MYCN EZH2

Sources for Extraosseous Ewing Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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