EOE
MCID: EXT052
MIFTS: 34

Extraskeletal Ewing Sarcoma (EOE) malady

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Extraskeletal Ewing Sarcoma

Aliases & Descriptions for Extraskeletal Ewing Sarcoma:

Name: Extraskeletal Ewing Sarcoma 56
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 69
Extraosseous Ewing Sarcoma 56
Extraskeletal Ewing Tumor 56
Extraosseous Ewing Tumor 56
Eoe 56

Classifications:



External Ids:

Orphanet 56 ORPHA370334
ICD10 via Orphanet 34 C49.9

Summaries for Extraskeletal Ewing Sarcoma

MalaCards based summary : Extraskeletal Ewing Sarcoma, also known as extraosseous ewings sarcoma-primitive neuroepithelial tumor, is related to extraosseous ewings sarcoma-primitive neuroepithelial tumor and ewing sarcoma, and has symptoms including fever An important gene associated with Extraskeletal Ewing Sarcoma is EWSR1 (EWS RNA Binding Protein 1), and among its related pathways/superpathways are Chromatin Regulation / Acetylation and Transcriptional misregulation in cancer. The drugs Doxorubicin and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include breast and thyroid, and related phenotype is neoplasm.

Related Diseases for Extraskeletal Ewing Sarcoma

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

(show top 50) (show all 168)
id Related Disease Score Top Affiliating Genes
1 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.2
2 ewing sarcoma 11.6
3 esophagitis, eosinophilic, 1 11.5
4 sarcoma 10.6
5 malignant mixed mullerian tumor 10.3 DES ENO2
6 inflammatory linear verrucous epidermal nevus 10.3 DES VIM
7 lymphomatous thyroiditis 10.3 DES VIM
8 osteomyelitis 10.3 DES VIM
9 congenital myasthenic syndrome 10.3 DES VIM
10 breast rhabdomyosarcoma 10.3 DES ENO2
11 benign schwannoma 10.3 ENO2 VIM
12 hinman syndrome 10.3 PTPRC VIM
13 sm-ahnmd 10.3 CD99 ENO2
14 cortical blindness 10.3 PTPRC VIM
15 developmental coordination disorder 10.3 PTPRC VIM
16 urethral cancer 10.3 DES VIM
17 ribbing disease 10.3 PTPRC VIM
18 vitreous syneresis 10.3 CD99 EWSR1
19 heel spur 10.3 EWSR1 PTPRC
20 biemond syndrome 10.3 DES VIM
21 liver angiosarcoma 10.3 DES VIM
22 chromophil adenoma of the kidney 10.3 ENO2 NKX2-1
23 intestinal obstruction 10.3 DES VIM
24 cavernous sinus meningioma 10.3 DES VIM
25 malignant dermis tumor 10.2 DES ENO2 VIM
26 adult endodermal sinus tumor 10.2 DES ENO2 VIM
27 esophagitis 10.2
28 vagina leiomyosarcoma 10.2 DES MUC1
29 male urethral cancer 10.2 DES VIM
30 skin glomus tumor 10.2 DES ENO2 VIM
31 invasive malignant thymoma 10.2 DES MUC1
32 jewett-marshall bladder cancer 10.2 DES ENO2 VIM
33 myelinoclastic diffuse sclerosis 10.2 ENO2 MUC1
34 foix-alajouanine syndrome 10.2 DES FUS
35 hyperpituitarism 10.2 DES ENO2 VIM
36 inverted papilloma 10.2 NKX2-1 VIM
37 ovarian cystadenoma 10.2 DES MUC1
38 immunodeficiency due to a classical component pathway complement deficiency 10.2 MUC1 VIM
39 mediastinum sarcoma 10.2 DES ENO2 EWSR1
40 rhabdomyosarcoma 2, alveolar 10.2 DES ENO2 EWSR1
41 female breast axillary tail cancer 10.2 CD99 ENO2
42 postgerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma 10.2 MUC1 VIM
43 endometrial stromal sarcoma 10.2 MUC1 VIM
44 infiltrating renal pelvis transitional cell carcinoma 10.2 ENO2 SYP
45 thymus small cell carcinoma 10.2 ENO2 SYP
46 subglottis verrucous carcinoma 10.2 MUC1 VIM
47 glomus tumor 10.2 ENO2 SYP
48 kidney sarcoma 10.2 CD99 ENO2 EWSR1
49 bladder adenocarcinoma 10.2 MUC1 VIM
50 conjunctival cancer 10.2 MUC1 VIM

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



Diseases related to Extraskeletal Ewing Sarcoma

Symptoms & Phenotypes for Extraskeletal Ewing Sarcoma

UMLS symptoms related to Extraskeletal Ewing Sarcoma:


fever

MGI Mouse Phenotypes related to Extraskeletal Ewing Sarcoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 9.02 ERG FLI1 NKX2-1 PTPRC SMARCA5

Drugs & Therapeutics for Extraskeletal Ewing Sarcoma

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

(show top 50) (show all 63)
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
Mechlorethamine Approved Phase 3 51-75-2 4033
8
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
9
Cyproheptadine Approved Phase 3 129-03-3 2913
10
Doxil Approved June 1999 Phase 3,Phase 2 31703
11 Alkylating Agents Phase 3,Phase 2
12 topoisomerase I inhibitors Phase 3,Phase 2
13 Topoisomerase Inhibitors Phase 3,Phase 2
14 Anti-Bacterial Agents Phase 3,Phase 2
15 Etoposide phosphate Phase 3,Phase 2
16 Immunosuppressive Agents Phase 3,Phase 2
17 Isophosphamide mustard Phase 3,Phase 2
18 Antibiotics, Antitubercular Phase 3,Phase 2
19 Antimitotic Agents Phase 3,Phase 2
20 Antirheumatic Agents Phase 3,Phase 2
21 Antineoplastic Agents, Alkylating Phase 3,Phase 2
22 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
23
Histamine Phosphate Phase 3 51-74-1 65513
24
Serotonin Phase 3 50-67-9 5202
25 Serotonin Agents Phase 3
26 Serotonin Antagonists Phase 3
27 Gastrointestinal Agents Phase 3
28 Neurotransmitter Agents Phase 3
29 Histamine Antagonists Phase 3
30 Histamine H1 Antagonists Phase 3
31 Dermatologic Agents Phase 3
32 Anti-Allergic Agents Phase 3
33 Antipruritics Phase 3
34
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
35
Glycerol Approved, Experimental Phase 2 56-81-5 753
36
Lenograstim Approved Phase 2 135968-09-1
37
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
38
Bevacizumab Approved, Investigational Phase 2 216974-75-3
39
Dactinomycin Approved Phase 2 50-76-0 2019 457193
40
Romidepsin Approved, Investigational Phase 2 128517-07-7 5352062
41
Epirubicin Approved Phase 2 56420-45-2 41867
42 insulin Phase 2
43 razoxane Phase 2
44 Mitogens Phase 2
45 Acetylmuramyl-Alanyl-Isoglutamine Phase 2
46 Adjuvants, Immunologic Phase 2
47 Nucleic Acid Synthesis Inhibitors Phase 2
48 Angiogenesis Inhibitors Phase 2
49 Angiogenesis Modulating Agents Phase 2
50 Pharmaceutical Solutions Phase 2

Interventional clinical trials:

(show all 15)
id Name Status NCT ID Phase
1 Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma Active, not recruiting NCT01231906 Phase 3
2 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3
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
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
5 Combination Chemotherapy and Biological Therapy in Treating Patients With High-Risk Ewing's Sarcoma Completed NCT00003667 Phase 2
6 Chemotherapy in Treating Children With Recurrent or Refractory Sarcomas Completed NCT00003745 Phase 2
7 Combination Chemotherapy in Treating Children With Metastatic Rhabdomyosarcoma or Other Malignant Mesenchymal Tumors Completed NCT00025441 Phase 2
8 A Study of R1507 in Participants With Recurrent or Refractory Sarcoma Completed NCT00642941 Phase 2
9 Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma Completed NCT00112463 Phase 2
10 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2
11 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2
12 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Recruiting NCT02601950 Phase 2
13 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
14 Collecting and Storing Tissue From Young Patients With Cancer Recruiting NCT00898755
15 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

Search NIH Clinical Center for Extraskeletal Ewing Sarcoma

Genetic Tests for Extraskeletal Ewing Sarcoma

Anatomical Context for Extraskeletal Ewing Sarcoma

MalaCards organs/tissues related to Extraskeletal Ewing Sarcoma:

39
Breast, Thyroid

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show all 27)
id Title Authors Year
1
Primary extraskeletal Ewing sarcoma of the stomach: a rare disease in an uncommon location. ( 27179157 )
2016
2
Rare case of small size Extraskeletal Ewing Sarcoma arising from the median nerve with paresthesia. ( 27523258 )
2016
3
Treatment outcomes of Japanese patients with Ewing sarcoma: differences between skeletal and extraskeletal Ewing sarcoma. ( 27008849 )
2016
4
Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution. ( 26175577 )
2015
5
Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma. ( 26425232 )
2015
6
An unusual tumor of the breast - extraskeletal ewing sarcoma. ( 24791212 )
2014
7
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients. ( 24734938 )
2014
8
Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. ( 25674292 )
2014
9
Retraction note to: CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. Ir J Med Sci (2012) 181:123-5. ( 24136179 )
2013
10
Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin. ( 23982393 )
2013
11
Metastatic renal extraskeletal Ewing sarcoma in complete remission for the last 8 years. ( 20535010 )
2012
12
Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. ( 21805144 )
2012
13
CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. ( 20676795 )
2012
14
Extraskeletal Ewing sarcoma of the mesocolon in a child. ( 22974628 )
2012
15
Primary extraskeletal Ewing sarcoma involving the carotid artery: a case report and review of the current literature. ( 22613280 )
2012
16
Requesting clarifications concerning the case report on 'extraskeletal Ewing sarcoma of the sinonasal tract. ( 23361294 )
2012
17
A case of extraskeletal Ewing sarcoma originating from the visceral pleura. ( 24391423 )
2011
18
Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. ( 21692057 )
2011
19
A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. ( 21113140 )
2011
20
A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. ( 22109315 )
2011
21
Extraskeletal Ewing sarcoma of the abdominal wall. ( 18818133 )
2008
22
Image of the month--quiz case. Retroperitoneal extraskeletal Ewing sarcoma. ( 18490564 )
2008
23
Radiological findings of atypical extraskeletal Ewing sarcoma. ( 18569849 )
2008
24
Spinal epidural extraskeletal Ewing sarcoma. ( 17316801 )
2007
25
Extraskeletal Ewing sarcoma mimicking a thyroid nodule. ( 17042696 )
2006
26
Spinal epidural extraskeletal Ewing sarcoma in an adolescent boy: a case report. ( 16517542 )
2006
27
Primitive neuroectodermal tumor and extraskeletal Ewing sarcoma arising primarily around the spinal column: report of four cases and a review of the literature. ( 14520055 )
2003

Variations for Extraskeletal Ewing Sarcoma

Expression for Extraskeletal Ewing Sarcoma

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

Pathways for Extraskeletal Ewing Sarcoma

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

id Super pathways Score Top Affiliating Genes
1 11.63 ERG EWSR1 FUS SMARCA5
2 11.18 ERG EWSR1 FLI1 FUS

GO Terms for Extraskeletal Ewing Sarcoma

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

id Name GO ID Score Top Affiliating Genes
1 intermediate filament organization GO:0045109 8.62 DES VIM

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

id Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.73 CD99 DES ENO2 ERG EWSR1 FLI1
2 identical protein binding GO:0042802 9.02 DES EWSR1 FUS SYP VIM

Sources for Extraskeletal Ewing 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 Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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