EOE
MCID: EXT052
MIFTS: 39

Extraskeletal Ewing Sarcoma (EOE)

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Extraskeletal Ewing Sarcoma

MalaCards integrated aliases for Extraskeletal Ewing Sarcoma:

Name: Extraskeletal Ewing Sarcoma 59
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 73
Extraosseous Ewing Sarcoma 59
Extraskeletal Ewing Tumor 59
Extraosseous Ewing Tumor 59
Eoe 59

Classifications:



External Ids:

Orphanet 59 ORPHA370334
ICD10 via Orphanet 34 C49.9
UMLS via Orphanet 74 C0852530 C0279980
UMLS 73 C0279980

Summaries for Extraskeletal Ewing Sarcoma

MalaCards based summary : Extraskeletal Ewing Sarcoma, also known as extraosseous ewings sarcoma-primitive neuroepithelial tumor, is related to extraosseous ewing's sarcoma and ewing sarcoma, and has symptoms including fever An important gene associated with Extraskeletal Ewing Sarcoma is SMARCA5 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily A, Member 5), and among its related pathways/superpathways are Chromatin Regulation / Acetylation and Transcriptional misregulation in cancer. The drugs Vincristine and Topotecan have been mentioned in the context of this disorder. Affiliated tissues include breast, lung and kidney, and related phenotypes are cellular and 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 239)
# Related Disease Score Top Affiliating Genes
1 extraosseous ewing's sarcoma 32.6 CD99 ENO2 EWSR1
2 ewing sarcoma 31.3 CD99 DES ENO2 ERG EWSR1 FLI1
3 sarcoma 30.9 ERG EWSR1 FLI1 FUS
4 spindle cell sarcoma 30.8 DES MUC1 VIM
5 ewing's family of tumors 30.5 EWSR1 FLI1
6 neurilemmoma 30.4 DES ENO2 VIM
7 rhabdomyosarcoma 30.1 DES ENO2 EWSR1 PTPRC VIM
8 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.5
9 esophagitis, eosinophilic, 1 12.4
10 esophagitis 10.7
11 cystic nephroma 10.3 DES VIM
12 cutaneous fibrous histiocytoma 10.3 DES VIM
13 juvenile xanthogranuloma 10.3 DES VIM
14 smooth muscle tumor 10.3 DES VIM
15 pseudosarcomatous fibromatosis 10.3 DES VIM
16 liver angiosarcoma 10.3 ENO2 VIM
17 congenital fibrosarcoma 10.3 DES VIM
18 embryonal sarcoma 10.3 DES VIM
19 cloacogenic carcinoma 10.3 ENO2 PTPRC
20 pericardium cancer 10.3 CD99 ENO2
21 breast adenomyoepithelioma 10.3 CD99 ENO2
22 breast myoepithelial neoplasm 10.3 CD99 ENO2
23 epidural neoplasm 10.3 CD99 PTPRC
24 infantile digital fibromatosis 10.3 DES VIM
25 hypereosinophilic syndrome 10.3
26 lymphangiomatosis 10.3 DES VIM
27 angiolipoma 10.3 DES VIM
28 cutaneous leiomyosarcoma 10.3 DES VIM
29 bednar tumor 10.3 ENO2 VIM
30 malignant ectomesenchymoma 10.3 DES ENO2
31 ectomesenchymoma 10.3 DES ENO2
32 chondroid lipoma 10.3 ENO2 FUS
33 vagina sarcoma 10.3
34 focal hand dystonia 10.3 FUS VIM
35 spindle cell lipoma 10.3 DES VIM
36 intracranial primitive neuroectodermal tumor 10.3 CD99 ENO2
37 central nervous system benign neoplasm 10.2 ENO2 SYP
38 bone benign neoplasm 10.2 ENO2 SYP
39 connective tissue benign neoplasm 10.2 ENO2 SYP
40 benign glioma 10.2 ENO2 SYP
41 central neurocytoma 10.2 ENO2 SYP
42 mixed cell type cancer 10.2 ENO2 SYP
43 multicentric reticulohistiocytosis 10.2 PTPRC VIM
44 cranial nerve malignant neoplasm 10.2 ENO2 SYP
45 optic nerve neoplasm 10.2 ENO2 SYP
46 ganglioneuroblastoma 10.2 ENO2 SYP
47 angiomyoma 10.2 ENO2 SYP
48 intraocular retinoblastoma 10.2 ENO2 SYP
49 benign ependymoma 10.2 ENO2 SYP
50 nodular ganglioneuroblastoma 10.2 ENO2 SYP

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.56 DES ENO2 ERG FLI1 NKX2-1 PTPRC
2 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 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 Extraskeletal Ewing Sarcoma

Genetic Tests for Extraskeletal Ewing Sarcoma

Anatomical Context for Extraskeletal Ewing Sarcoma

MalaCards organs/tissues related to Extraskeletal Ewing Sarcoma:

41
Breast, Lung, Kidney, Bone, Testis, Thymus, Pineal

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show all 35)
# Title Authors Year
1
Treatment outcomes of Japanese patients with Ewing sarcoma: differences between skeletal and extraskeletal Ewing sarcoma. ( 27008849 )
2016
2
Rare case of small size Extraskeletal Ewing Sarcoma arising from the median nerve with paresthesia. ( 27523258 )
2016
3
Primary extraskeletal Ewing sarcoma of the stomach: a rare disease in an uncommon location. ( 27179157 )
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
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients. ( 24734938 )
2014
7
Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. ( 25674292 )
2014
8
An unusual tumor of the breast - extraskeletal ewing sarcoma. ( 24791212 )
2014
9
Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin. ( 23982393 )
2013
10
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
11
Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. ( 21805144 )
2012
12
Extraskeletal Ewing sarcoma of the mesocolon in a child. ( 22974628 )
2012
13
Primary extraskeletal Ewing sarcoma involving the carotid artery: a case report and review of the current literature. ( 22613280 )
2012
14
Requesting clarifications concerning the case report on 'extraskeletal Ewing sarcoma of the sinonasal tract. ( 23361294 )
2012
15
CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. ( 20676795 )
2012
16
Metastatic renal extraskeletal Ewing sarcoma in complete remission for the last 8 years. ( 20535010 )
2012
17
A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. ( 22109315 )
2011
18
A case of extraskeletal Ewing sarcoma originating from the visceral pleura. ( 24391423 )
2011
19
A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. ( 21113140 )
2011
20
Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. ( 21692057 )
2011
21
Radiological findings of atypical extraskeletal Ewing sarcoma. ( 18569849 )
2008
22
Extraskeletal Ewing sarcoma of the abdominal wall. ( 18818133 )
2008
23
Image of the month--quiz case. Retroperitoneal extraskeletal Ewing sarcoma. ( 18490564 )
2008
24
Spinal epidural extraskeletal Ewing sarcoma. ( 17316801 )
2007
25
Spinal epidural extraskeletal Ewing sarcoma in an adolescent boy: a case report. ( 16517542 )
2006
26
Extraskeletal Ewing sarcoma mimicking a thyroid nodule. ( 17042696 )
2006
27
Extraskeletal Ewing sarcoma of the diaphragm presenting with hemothorax. ( 15276562 )
2004
28
Primary orbital extraskeletal ewing sarcoma. ( 12860815 )
2003
29
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
30
Spinal epidural extraskeletal Ewing sarcoma: MR findings in two cases. ( 11290502 )
2001
31
Extraskeletal Ewing sarcoma in a 77-year-old woman. ( 11570916 )
2001
32
The cytology of extraskeletal Ewing sarcoma. ( 10385445 )
1999
33
Radiological features of extraskeletal Ewing sarcoma. ( 2379070 )
1990
34
Extraskeletal Ewing sarcoma with computed tomography correlation. ( 6867772 )
1983
35
Extraskeletal Ewing sarcoma. An ultrastructural study. ( 577677 )
1977

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:

# Super pathways Score Top Affiliating Genes
1 11.66 ERG EWSR1 FUS SMARCA5
2 11.19 ERG EWSR1 FLI1 FUS

GO Terms for Extraskeletal Ewing Sarcoma

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

# 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:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.4 CD99 DES ENO2 ERG EWSR1 FLI1
2 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.26 ERG FLI1 MUC1 NKX2-1

Sources for Extraskeletal Ewing 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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