MCID: EXT052
MIFTS: 39

Extraskeletal Ewing Sarcoma

Categories: Rare diseases, Neuronal diseases, Cancer 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

Summaries for Extraskeletal Ewing Sarcoma

MalaCards based summary : Extraskeletal Ewing Sarcoma, also known as extraosseous ewings sarcoma-primitive neuroepithelial tumor, is related to ewing's family of tumors and 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 Etoposide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include liver, breast and thyroid, 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 227)
# Related Disease Score Top Affiliating Genes
1 ewing's family of tumors 30.6 EWSR1 FLI1
2 sarcoma 29.5 ERG EWSR1 FLI1 FUS
3 ewing sarcoma 27.4 CD99 DES ENO2 ERG EWSR1 FLI1
4 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.3
5 esophagitis, eosinophilic, 1 12.3
6 breast rhabdomyosarcoma 11.0 DES ENO2
7 malignant ectomesenchymoma 10.9 DES ENO2
8 ectomesenchymoma 10.9 DES ENO2
9 infantile digital fibromatosis 10.9 DES VIM
10 lymphangiomatosis 10.9 DES VIM
11 angiolipoma 10.9 DES VIM
12 bednar tumor 10.9 ENO2 VIM
13 spindle cell lipoma 10.9 DES VIM
14 intracranial primitive neuroectodermal tumor 10.9 CD99 ENO2
15 benign metastasizing leiomyoma 10.9 DES VIM
16 multicentric reticulohistiocytosis 10.9 PTPRC VIM
17 retroperitoneal fibrosis 10.9 PTPRC VIM
18 conventional fibrosarcoma 10.9 ENO2 PTPRC
19 small cell osteogenic sarcoma 10.8 ENO2 EWSR1
20 congenital fibrosarcoma 10.8 DES VIM
21 askin's tumor 10.8 ENO2 EWSR1
22 juvenile xanthogranuloma 10.8 DES VIM
23 pulmonary vein stenosis 10.8 CD99 DES
24 bladder sarcoma 10.8 CD99 EWSR1
25 vulvar sarcoma 10.8 EWSR1 PTPRC
26 cutaneous fibrous histiocytoma 10.7 DES VIM
27 cutaneous leiomyosarcoma 10.7 DES MUC1
28 pilomatrix carcinoma 10.7 ENO2 MUC1
29 spindle cell thymoma 10.7 DES MUC1
30 intracranial cysts 10.7 ENO2 MUC1
31 breast adenomyoepithelioma 10.7 CD99 ENO2
32 breast myoepithelial neoplasm 10.7 CD99 ENO2
33 epithelioid malignant peripheral nerve sheath tumor 10.7 ENO2 MUC1
34 papillary adenoma 10.7 NKX2-1 VIM
35 microcystic adenoma 10.7 ENO2 MUC1
36 rete testis adenocarcinoma 10.7 ENO2 NKX2-1
37 spindle cell rhabdomyosarcoma 10.7 DES MUC1
38 malignant peritoneal mesothelioma 10.7 MUC1 VIM
39 epidural neoplasm 10.7 CD99 PTPRC
40 pseudosarcomatous fibromatosis 10.7 DES VIM
41 primary cutaneous anaplastic large cell lymphoma 10.7 MUC1 PTPRC
42 epulis 10.6 DES ENO2 VIM
43 epithelioid leiomyosarcoma 10.6 DES ENO2 VIM
44 fibrous meningioma 10.6 MUC1 VIM
45 malignant mixed mullerian tumor 10.6 MUC1 VIM
46 nodular hidradenoma 10.6 MUC1 VIM
47 granular cell tumor 10.6 DES ENO2 VIM
48 congenital epulis 10.6 DES ENO2 VIM
49 eccrine porocarcinoma 10.6 MUC1 VIM
50 glomangioma 10.6 DES ENO2 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.56 SMARCA5 VIM DES ENO2 ERG FLI1
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 57)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etoposide Approved Phase 3,Phase 2,Not Applicable 33419-42-0 36462
2
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
3
Ifosfamide Approved Phase 3,Phase 2,Not Applicable 3778-73-2 3690
4
Topotecan Approved, Investigational Phase 3,Phase 2,Not Applicable 119413-54-6, 123948-87-8 60700
5
Vincristine Approved, Investigational Phase 3,Phase 2 2068-78-2, 57-22-7 5978
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
Cyproheptadine Approved Phase 3 129-03-3 2913
9
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
10
Doxil Approved June 1999 Phase 3,Phase 2 31703
11
Isophosphamide mustard Phase 3,Phase 2,Not Applicable 0
12 Alkylating Agents Phase 3,Phase 2,Not Applicable
13 topoisomerase I inhibitors Phase 3,Phase 2,Not Applicable
14 Topoisomerase Inhibitors Phase 3,Phase 2,Not Applicable
15 Anti-Bacterial Agents Phase 3,Phase 2
16 Antibiotics, Antitubercular Phase 3,Phase 2
17 Etoposide phosphate Phase 3,Phase 2,Not Applicable
18 Immunosuppressive Agents Phase 3,Phase 2,Not Applicable
19 Antirheumatic Agents Phase 3,Phase 2
20 Antimitotic Agents Phase 3,Phase 2
21 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Not Applicable
22 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Not Applicable
23
Histamine Phosphate Phase 3 51-74-1 65513
24 Serotonin Agents Phase 3
25 Serotonin Antagonists Phase 3
26 Gastrointestinal Agents Phase 3
27 Neurotransmitter Agents Phase 3
28 Histamine Antagonists Phase 3
29 Histamine H1 Antagonists Phase 3
30 Dermatologic Agents Phase 3
31 Anti-Allergic Agents Phase 3
32 Antipruritics Phase 3
33
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
34
Lenograstim Approved, Investigational Phase 2 135968-09-1
35
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
36
Glycerol Approved, Investigational Phase 2 56-81-5 753
37
Bevacizumab Approved, Investigational Phase 2 216974-75-3
38
Dactinomycin Approved, Investigational Phase 2 50-76-0 2019 457193
39
Epirubicin Approved Phase 2 56420-45-2 41867
40 razoxane Phase 2
41 Acetylmuramyl-Alanyl-Isoglutamine Phase 2
42 Adjuvants, Immunologic Phase 2
43 Nucleic Acid Synthesis Inhibitors Phase 2
44 Angiogenesis Inhibitors Phase 2
45 Angiogenesis Modulating Agents Phase 2
46 Pharmaceutical Solutions Phase 2
47 Antibodies Phase 2
48 Immunoglobulins Phase 2
49 Antibodies, Monoclonal Phase 2
50 Anti-Infective Agents Phase 2,Early Phase 1

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 vincristine sulfate;doxorubicin hydrochloride;cyclophosphamide;ifosfamide;etoposide;topotecan hydrochloride
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 Recruiting 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
Liver, Breast, Thyroid

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show all 28)
# 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
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:

# 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 transcription coregulator activity GO:0003712 9.13 EWSR1 FUS MUC1
2 identical protein binding GO:0042802 9.1 DES EWSR1 FUS PTPRC SYP VIM

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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