MCID: EXT052
MIFTS: 37

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 55
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 69
Extraosseous Ewing Sarcoma 55
Extraskeletal Ewing Tumor 55
Extraosseous Ewing Tumor 55
Eoe 55

Classifications:



External Ids:

Orphanet 55 ORPHA370334
UMLS via Orphanet 70 C0852530 C0279980
ICD10 via Orphanet 33 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 sarcoma 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 liver 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 224)
# Related Disease Score Top Affiliating Genes
1 sarcoma 30.1 ERG EWSR1 FLI1 FUS
2 ewing sarcoma 29.3 CD99 DES ENO2 ERG EWSR1 FLI1
3 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.3
4 esophagitis, eosinophilic, 1 11.9
5 malignant ectomesenchymoma 10.6 DES ENO2
6 ectomesenchymoma 10.6 DES ENO2
7 skin glomus tumor 10.6 DES VIM
8 infantile digital fibromatosis 10.6 DES VIM
9 lymphangiomatosis 10.6 DES VIM
10 angiolipoma 10.6 DES VIM
11 cutaneous leiomyosarcoma 10.6 DES VIM
12 bednar tumor 10.6 ENO2 VIM
13 spindle cell lipoma 10.6 DES VIM
14 intracranial primitive neuroectodermal tumor 10.5 CD99 ENO2
15 multicentric reticulohistiocytosis 10.5 PTPRC VIM
16 benign metastasizing leiomyoma 10.5 DES VIM
17 retroperitoneal fibrosis 10.5 PTPRC VIM
18 small cell osteogenic sarcoma 10.5 ENO2 EWSR1
19 conventional fibrosarcoma 10.5 ENO2 PTPRC
20 congenital fibrosarcoma 10.5 DES VIM
21 askin's tumor 10.5 ENO2 EWSR1
22 juvenile xanthogranuloma 10.5 DES VIM
23 bladder sarcoma 10.5 CD99 EWSR1
24 pulmonary vein stenosis 10.5 CD99 DES
25 breast adenomyoepithelioma 10.5 CD99 ENO2
26 breast myoepithelial neoplasm 10.5 CD99 ENO2
27 vulvar sarcoma 10.5 EWSR1 PTPRC
28 cutaneous fibrous histiocytoma 10.5 DES VIM
29 pilomatrix carcinoma 10.4 ENO2 MUC1
30 spindle cell thymoma 10.4 DES MUC1
31 intracranial cysts 10.4 ENO2 MUC1
32 spindle cell rhabdomyosarcoma 10.4 DES MUC1
33 epithelioid malignant peripheral nerve sheath tumor 10.4 ENO2 MUC1
34 papillary adenoma 10.4 NKX2-1 VIM
35 microcystic adenoma 10.4 ENO2 MUC1
36 respiratory system benign neoplasm 10.4 ENO2 NKX2-1
37 rete testis adenocarcinoma 10.4 ENO2 NKX2-1
38 pseudosarcomatous fibromatosis 10.4 DES VIM
39 malignant peritoneal mesothelioma 10.4 MUC1 VIM
40 primary cutaneous anaplastic large cell lymphoma 10.4 MUC1 PTPRC
41 epidural neoplasm 10.4 CD99 PTPRC
42 epulis 10.4 DES ENO2 VIM
43 epithelioid leiomyosarcoma 10.4 DES ENO2 VIM
44 fibrous meningioma 10.4 MUC1 VIM
45 malignant mixed mullerian tumor 10.4 MUC1 VIM
46 nodular hidradenoma 10.4 MUC1 VIM
47 granular cell tumor 10.4 DES ENO2 VIM
48 congenital epulis 10.4 DES ENO2 VIM
49 clear cell meningioma 10.4 MUC1 VIM
50 eccrine porocarcinoma 10.4 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:

43
# 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 56)
# 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, Investigational Phase 3 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 0
12 Alkylating Agents Phase 3,Phase 2
13 topoisomerase I inhibitors Phase 3,Phase 2
14 Topoisomerase Inhibitors Phase 3,Phase 2
15 Anti-Bacterial Agents Phase 3,Phase 2
16 Etoposide phosphate Phase 3,Phase 2
17 Immunosuppressive Agents Phase 3,Phase 2
18 Antimitotic Agents Phase 3,Phase 2
19 Antibiotics, Antitubercular Phase 3,Phase 2
20 Antineoplastic Agents, Phytogenic Phase 3,Phase 2
21 Antirheumatic Agents Phase 3,Phase 2
22
Histamine Phosphate Phase 3 51-74-1 65513
23
Serotonin Phase 3 50-67-9 5202
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
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
34
Glycerol Approved, Investigational Phase 2 56-81-5 753
35
Lenograstim Approved, Investigational Phase 2 135968-09-1
36
Bevacizumab Approved, Investigational Phase 2 216974-75-3
37
Epirubicin Approved Phase 2 56420-45-2 41867
38
Dactinomycin Approved, Investigational Phase 2 50-76-0 457193 2019
39 razoxane Phase 2
40 Acetylmuramyl-Alanyl-Isoglutamine Phase 2
41 Adjuvants, Immunologic Phase 2
42 Nucleic Acid Synthesis Inhibitors Phase 2
43 Angiogenesis Inhibitors Phase 2
44 Angiogenesis Modulating Agents Phase 2
45 Antibodies, Monoclonal Phase 2
46 Pharmaceutical Solutions Phase 2
47 Anti-Infective Agents Phase 2,Early Phase 1
48 Immunoglobulins Phase 2
49 Protective Agents Phase 2
50 Antibodies 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 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 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:

38
Liver, Thyroid

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show all 27)
# Title Authors Year
1
Rare case of small size Extraskeletal Ewing Sarcoma arising from the median nerve with paresthesia. ( 27523258 )
2016
2
Primary extraskeletal Ewing sarcoma of the stomach: a rare disease in an uncommon location. ( 27179157 )
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
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
CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. ( 20676795 )
2012
12
Requesting clarifications concerning the case report on 'extraskeletal Ewing sarcoma of the sinonasal tract. ( 23361294 )
2012
13
Extraskeletal Ewing sarcoma of the mesocolon in a child. ( 22974628 )
2012
14
Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. ( 21805144 )
2012
15
Metastatic renal extraskeletal Ewing sarcoma in complete remission for the last 8 years. ( 20535010 )
2012
16
Primary extraskeletal Ewing sarcoma involving the carotid artery: a case report and review of the current literature. ( 22613280 )
2012
17
A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. ( 21113140 )
2011
18
Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. ( 21692057 )
2011
19
A case of extraskeletal Ewing sarcoma originating from the visceral pleura. ( 24391423 )
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
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
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:

# 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

Sources for Extraskeletal Ewing Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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