MCID: EXT052
MIFTS: 40

Extraskeletal Ewing Sarcoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Extraskeletal Ewing Sarcoma

MalaCards integrated aliases for Extraskeletal Ewing Sarcoma:

Name: Extraskeletal Ewing Sarcoma 60
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 74
Extraosseous Ewing Sarcoma 60
Extraskeletal Ewing Tumor 60
Extraosseous Ewing Tumor 60
Eoe 60

Classifications:



External Ids:

ICD10 via Orphanet 35 C49.9
UMLS via Orphanet 75 C0279980 C0852530
Orphanet 60 ORPHA370334
UMLS 74 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 neurilemmoma, 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 Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include breast, thyroid and lung, 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 234)
# Related Disease Score Top Affiliating Genes
1 extraosseous ewing's sarcoma 32.7 CD99 ENO2 EWSR1
2 neurilemmoma 30.6 DES ENO2 VIM
3 ewing's family of tumors 30.6 EWSR1 FLI1
4 spindle cell sarcoma 30.5 DES MUC1 VIM
5 ewing sarcoma 30.5 CD99 DES ENO2 ERG EWSR1 FLI1
6 sarcoma 30.4 ERG EWSR1 FLI1 FUS VIM
7 rhabdomyosarcoma 30.1 DES ENO2 EWSR1 PTPRC VIM
8 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.5
9 esophagitis, eosinophilic, 1 12.5
10 esophagitis 10.7
11 smooth muscle tumor 10.5 DES VIM
12 cutaneous fibrous histiocytoma 10.5 DES VIM
13 juvenile xanthogranuloma 10.5 DES VIM
14 cloacogenic carcinoma 10.5 ENO2 PTPRC
15 pseudosarcomatous fibromatosis 10.5 DES VIM
16 congenital fibrosarcoma 10.5 DES VIM
17 liver angiosarcoma 10.5 ENO2 VIM
18 embryonal sarcoma 10.5 DES VIM
19 pericardium cancer 10.5 CD99 ENO2
20 breast adenomyoepithelioma 10.5 CD99 ENO2
21 breast myoepithelial neoplasm 10.4 CD99 ENO2
22 epidural neoplasm 10.4 CD99 PTPRC
23 infantile digital fibromatosis 10.4 DES VIM
24 malignant ectomesenchymoma 10.4 DES ENO2
25 lymphangiomatosis 10.4 DES VIM
26 ectomesenchymoma 10.4 DES ENO2
27 angiolipoma 10.4 DES VIM
28 chondroid lipoma 10.4 ENO2 FUS
29 bednar tumor 10.4 ENO2 VIM
30 intracranial primitive neuroectodermal tumor 10.4 CD99 ENO2
31 spindle cell lipoma 10.4 DES VIM
32 focal hand dystonia 10.4 FUS VIM
33 multicentric reticulohistiocytosis 10.4 PTPRC VIM
34 retroperitoneal fibrosis 10.4 PTPRC VIM
35 small cell osteogenic sarcoma 10.4 ENO2 EWSR1
36 rete testis neoplasm 10.4 ENO2 NKX2-1
37 prostate small cell carcinoma 10.4 ENO2 NKX2-1
38 bladder sarcoma 10.4 CD99 EWSR1
39 hypereosinophilic syndrome 10.4
40 hidradenocarcinoma 10.3 NKX2-1 VIM
41 askin's tumor 10.3 ENO2 EWSR1
42 benign metastasizing leiomyoma 10.3 DES VIM
43 central nervous system benign neoplasm 10.3 ENO2 SYP
44 bone benign neoplasm 10.3 ENO2 SYP
45 connective tissue benign neoplasm 10.3 ENO2 SYP
46 central neurocytoma 10.3 ENO2 SYP
47 mixed cell type cancer 10.3 ENO2 SYP
48 conventional fibrosarcoma 10.3 ENO2 PTPRC
49 cranial nerve malignant neoplasm 10.3 ENO2 SYP
50 optic nerve neoplasm 10.3 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:

47
# 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 63)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
2
Ifosfamide Approved Phase 3,Phase 2,Not Applicable 3778-73-2 3690
3
Vincristine Approved, Investigational Phase 3,Phase 2 2068-78-2, 57-22-7 5978
4
Etoposide Approved Phase 3,Phase 2,Not Applicable 33419-42-0 36462
5
Topotecan Approved, Investigational Phase 3,Phase 2,Not Applicable 119413-54-6, 123948-87-8 60700
6
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
7
Mechlorethamine Approved, Investigational Phase 3,Not Applicable 51-75-2 4033
8
Histamine Approved, Investigational Phase 3 51-45-6 774
9
Cyproheptadine Approved Phase 3 129-03-3 2913
10
Doxil Approved June 1999 Phase 3,Phase 2 31703
11
Emodepside Investigational, Vet_approved Phase 3 155030-63-0
12 Antimitotic Agents Phase 3,Phase 2
13 Immunosuppressive Agents Phase 3,Phase 2,Not Applicable
14 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Not Applicable
15
Isophosphamide mustard Phase 3,Phase 2,Not Applicable 0
16 Alkylating Agents Phase 3,Phase 2,Not Applicable
17 Etoposide phosphate Phase 3,Phase 2,Not Applicable
18 Immunologic Factors Phase 3,Phase 2,Not Applicable
19 Antibiotics, Antitubercular Phase 3,Phase 2
20 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Not Applicable
21 Antirheumatic Agents Phase 3,Phase 2
22 Anti-Bacterial Agents Phase 3,Phase 2
23 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
24 topoisomerase I inhibitors Phase 3,Phase 2,Not Applicable
25 Dermatologic Agents Phase 3
26 Keratolytic Agents Phase 3
27 Podophyllotoxin Phase 3 518-28-5
28 Histamine Antagonists Phase 3
29 Neurotransmitter Agents Phase 3
30 Gastrointestinal Agents Phase 3
31 Serotonin Agents Phase 3
32 Serotonin Antagonists Phase 3
33 Anti-Allergic Agents Phase 3
34 Antipruritics Phase 3
35 Histamine H1 Antagonists Phase 3
36
Histamine Phosphate Phase 3 51-74-1 65513
37
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
38
Bevacizumab Approved, Investigational Phase 2 216974-75-3
39
Glycerol Approved, Investigational Phase 2 56-81-5 753
40
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
41
Epirubicin Approved Phase 2 56420-45-2 41867
42
Dactinomycin Approved, Investigational Phase 2 50-76-0 457193 2019
43
Lenograstim Approved, Investigational Phase 2 135968-09-1
44 Antibodies, Monoclonal Phase 2
45 Immunoglobulins Phase 2
46 Angiogenesis Modulating Agents Phase 2
47 Angiogenesis Inhibitors Phase 2
48 Antibodies Phase 2
49 Antineoplastic Agents, Immunological Phase 2
50 Pharmaceutical Solutions 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:

42
Breast, Thyroid, Lung, Kidney, Bone, Testis, Thymus

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show all 37)
# Title Authors Year
1
Gastrointestinal: Multi-modality imaging of extraskeletal Ewing sarcoma of the stomach. ( 30912185 )
2019
2
Extraskeletal Ewing Sarcoma: Report of an Extremely Rare Case in Temporal Region. ( 30842800 )
2018
3
Rare case of small size Extraskeletal Ewing Sarcoma arising from the median nerve with paresthesia. ( 27523258 )
2018
4
Treatment outcomes of Japanese patients with Ewing sarcoma: differences between skeletal and extraskeletal Ewing sarcoma. ( 27008849 )
2016
5
Primary extraskeletal Ewing sarcoma of the stomach: a rare disease in an uncommon location. ( 27179157 )
2016
6
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
7
Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma. ( 26425232 )
2015
8
Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. ( 25674292 )
2014
9
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients. ( 24734938 )
2014
10
An unusual tumor of the breast - extraskeletal ewing sarcoma. ( 24791212 )
2014
11
Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin. ( 23982393 )
2013
12
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
13
Extraskeletal Ewing sarcoma of the mesocolon in a child. ( 22974628 )
2012
14
Requesting clarifications concerning the case report on 'extraskeletal Ewing sarcoma of the sinonasal tract. ( 23361294 )
2012
15
Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. ( 21805144 )
2012
16
Primary extraskeletal Ewing sarcoma involving the carotid artery: a case report and review of the current literature. ( 22613280 )
2012
17
Metastatic renal extraskeletal Ewing sarcoma in complete remission for the last 8 years. ( 20535010 )
2012
18
CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. ( 20676795 )
2012
19
Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. ( 21692057 )
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
A case of extraskeletal Ewing sarcoma originating from the visceral pleura. ( 24391423 )
2011
22
A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. ( 21113140 )
2011
23
Image of the month--quiz case. Retroperitoneal extraskeletal Ewing sarcoma. ( 18490564 )
2008
24
Radiological findings of atypical extraskeletal Ewing sarcoma. ( 18569849 )
2008
25
Extraskeletal Ewing sarcoma of the abdominal wall. ( 18818133 )
2008
26
Spinal epidural extraskeletal Ewing sarcoma. ( 17316801 )
2007
27
Spinal epidural extraskeletal Ewing sarcoma in an adolescent boy: a case report. ( 16517542 )
2006
28
Extraskeletal Ewing sarcoma mimicking a thyroid nodule. ( 17042696 )
2006
29
Extraskeletal Ewing sarcoma of the diaphragm presenting with hemothorax. ( 15276562 )
2004
30
Primary orbital extraskeletal ewing sarcoma. ( 12860815 )
2003
31
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
32
Spinal epidural extraskeletal Ewing sarcoma: MR findings in two cases. ( 11290502 )
2001
33
Extraskeletal Ewing sarcoma in a 77-year-old woman. ( 11570916 )
2001
34
The cytology of extraskeletal Ewing sarcoma. ( 10385445 )
1999
35
Radiological features of extraskeletal Ewing sarcoma. ( 2379070 )
1990
36
Extraskeletal Ewing sarcoma with computed tomography correlation. ( 6867772 )
1983
37
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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