EOE
MCID: EXT052
MIFTS: 35

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 72
Extraosseous Ewing Sarcoma 59
Extraskeletal Ewing Tumor 59
Extraosseous Ewing Tumor 59
Eoe 59

Classifications:



External Ids:

ICD10 via Orphanet 34 C49.9
UMLS via Orphanet 73 C0279980 C0852530
Orphanet 59 ORPHA370334
UMLS 72 C0279980

Summaries for Extraskeletal Ewing Sarcoma

MalaCards based summary : Extraskeletal Ewing Sarcoma, also known as extraosseous ewings sarcoma-primitive neuroepithelial tumor, is related to spindle cell sarcoma and ewing's family of tumors, 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 Topotecan and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include bone, kidney and lymph node, 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 274)
# Related Disease Score Top Affiliating Genes
1 spindle cell sarcoma 31.8 VIM MUC1 DES
2 ewing's family of tumors 31.2 FLI1 EWSR1
3 vulvar sarcoma 31.1 PTPRC EWSR1
4 neurilemmoma 30.9 VIM ENO2 DES
5 liposarcoma 30.3 VIM FUS EWSR1 DES
6 rhabdomyosarcoma 30.1 VIM PTPRC EWSR1 ENO2 DES
7 sarcoma 29.9 VIM FUS FLI1 EWSR1 ERG
8 ewing sarcoma 29.0 VIM SYP PTPRC FUS FLI1 EWSR1
9 extraosseous ewings sarcoma-primitive neuroepithelial tumor 12.6
10 esophagitis, eosinophilic, 1 12.6
11 peroxisome disorders 11.3
12 esophagitis 10.7
13 malignant ectomesenchymoma 10.7 ENO2 DES
14 ectomesenchymoma 10.7 ENO2 DES
15 infantile digital fibromatosis 10.7 VIM DES
16 lymphangiomatosis 10.7 VIM DES
17 angiolipoma 10.7 VIM DES
18 bednar tumor 10.7 VIM ENO2
19 spindle cell lipoma 10.7 VIM DES
20 benign metastasizing leiomyoma 10.7 VIM DES
21 intracranial primitive neuroectodermal tumor 10.7 ENO2 CD99
22 conventional fibrosarcoma 10.7 PTPRC ENO2
23 retroperitoneal fibrosis 10.7 VIM PTPRC
24 multicentric reticulohistiocytosis 10.7 VIM PTPRC
25 small cell osteogenic sarcoma 10.7 EWSR1 ENO2
26 cystic nephroma 10.7 VIM DES
27 pulmonary vein stenosis 10.6 DES CD99
28 askin's tumor 10.6 EWSR1 ENO2
29 juvenile xanthogranuloma 10.6 VIM DES
30 bladder sarcoma 10.6 EWSR1 CD99
31 congenital fibrosarcoma 10.6 VIM DES
32 cutaneous fibrous histiocytoma 10.6 VIM DES
33 myxofibrosarcoma 10.6 FUS DES
34 pseudosarcomatous fibromatosis 10.6 VIM DES
35 cutaneous leiomyosarcoma 10.6 MUC1 DES
36 breast myoepithelial neoplasm 10.6 ENO2 CD99
37 spindle cell thymoma 10.6 MUC1 DES
38 pilomatrix carcinoma 10.6 MUC1 ENO2
39 breast adenomyoepithelioma 10.6 ENO2 CD99
40 intracranial cysts 10.6 MUC1 ENO2
41 epidural neoplasm 10.6 PTPRC CD99
42 epithelioid malignant peripheral nerve sheath tumor 10.5 MUC1 ENO2
43 microcystic adenoma 10.5 MUC1 ENO2
44 spindle cell rhabdomyosarcoma 10.5 MUC1 DES
45 primary cutaneous anaplastic large cell lymphoma 10.5 PTPRC MUC1
46 malignant peritoneal mesothelioma 10.5 VIM MUC1
47 embryonal sarcoma 10.5 VIM DES
48 secretory meningioma 10.5 VIM MUC1
49 chondroid lipoma 10.5 FUS ENO2
50 fibrous meningioma 10.5 VIM MUC1

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 all 44)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Topotecan Approved, Investigational Phase 3 123948-87-8, 119413-54-6 60700
2
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
3
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
4
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
5
Ifosfamide Approved Phase 3 3778-73-2 3690
6
Etoposide Approved Phase 3 33419-42-0 36462
7
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
8
Histamine Approved, Investigational Phase 3 51-45-6 774
9
Cyproheptadine Approved Phase 3 129-03-3 2913
10
Emodepside Investigational, Vet_approved Phase 3 155030-63-0
11 topoisomerase I inhibitors Phase 3
12 Topoisomerase Inhibitors Phase 3
13 Tubulin Modulators Phase 3
14 Alkylating Agents Phase 3
15
Liposomal doxorubicin Phase 3 31703
16 Antineoplastic Agents, Phytogenic Phase 3
17 Anti-Bacterial Agents Phase 3
18 Immunosuppressive Agents Phase 3
19 Antibiotics, Antitubercular Phase 3
20 Immunologic Factors Phase 3
21 Antimitotic Agents Phase 3
22 Antirheumatic Agents Phase 3
23 Antineoplastic Agents, Alkylating Phase 3
24 Keratolytic Agents Phase 3
25 Podophyllotoxin Phase 3 518-28-5
26
Isophosphamide mustard Phase 3 0
27 Etoposide phosphate Phase 3
28 Gastrointestinal Agents Phase 3
29 Neurotransmitter Agents Phase 3
30 Anti-Allergic Agents Phase 3
31 Serotonin Antagonists Phase 3
32 Histamine H1 Antagonists Phase 3
33 Histamine Antagonists Phase 3
34 Dermatologic Agents Phase 3
35 Serotonin Agents Phase 3
36
Histamine Phosphate Phase 3 51-74-1 65513
37 Antipruritics Phase 3
38
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
39
Glycerol Approved, Investigational Phase 2 56-81-5 753
40 razoxane Phase 2
41 Acetylmuramyl-Alanyl-Isoglutamine Phase 2
42 Cardiotonic Agents Phase 2
43 Protective Agents Phase 2
44 Adjuvants, Immunologic Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase III Randomized Trial of Adding Vincristine-Topotecan-Cyclophosphamide to Standard Chemotherapy in Initial Treatment of Non-Metastatic Ewing Sarcoma Active, not recruiting NCT01231906 Phase 3 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Ifosfamide;Topotecan Hydrochloride;Vincristine Sulfate
2 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
3 A Phase II Study of Continuous 21 Day Infusion of Topotecan (NSC # 609699) in Children With Relapsed Solid Tumors Completed NCT00003745 Phase 2 topotecan hydrochloride
4 Randomized Phase II Study of Vincristine, Doxorubicin, Cyclophosphamide and Dexrazoxane With and Without ImmTher for Newly Diagnosed High Risk Ewing's Sarcoma Completed NCT00003667 Phase 2 cyclophosphamide;dexrazoxane hydrochloride;disaccharide tripeptide glycerol dipalmitoyl;doxorubicin hydrochloride;vincristine sulfate

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
Bone, Kidney, Lymph Node, Breast, Thyroid

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show top 50) (show all 71)
# Title Authors PMID Year
1
FDG PET/CT in a Case of Primary Pulmonary Ewing Sarcoma. 38
31274619 2019
2
Large extraskeletal ewing sarcoma of the hand: A rare entity. 38
31417021 2019
3
FDG PET/CT in a Case of Primary Pulmonary Ewing Sarcoma. 38
31162265 2019
4
Gastrointestinal: Multi-modality imaging of extraskeletal Ewing sarcoma of the stomach. 38
30912185 2019
5
Role of Radiation Therapy in Adult Extraskeletal Ewing's Sarcoma Patients Treated with Chemotherapy and Surgery. 38
31178655 2019
6
Extraskeletal Ewing Sarcoma of the Jejunum: A Case Report. 38
31314729 2019
7
CIC-DUX4 sarcoma diagnosed by fine-needle aspiration cytology: A case report. 38
30353686 2018
8
Extraskeletal Ewing Sarcoma: Report of an Extremely Rare Case in Temporal Region. 38
30842800 2018
9
Extraskeletal versus Skeletal Ewing Sarcoma in the adult population: Controversies in care. 38
30217290 2018
10
Rare case of small size Extraskeletal Ewing Sarcoma arising from the median nerve with paresthesia. 38
27523258 2018
11
Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases. 38
30538569 2018
12
Ewing sarcoma in patients over 40 years of age: a prospective analysis of 31 patients treated at a single institution. 38
27443894 2016
13
Dumbbell Scoring System: A New Method for the Differential Diagnosis of Malignant and Benign Spinal Dumbbell Tumors. 38
27031767 2016
14
Primary extraskeletal Ewing sarcoma of the stomach: a rare disease in an uncommon location. 38
27179157 2016
15
Treatment outcomes of Japanese patients with Ewing sarcoma: differences between skeletal and extraskeletal Ewing sarcoma. 38
27008849 2016
16
Thoracic Radiculopathy due to Rare Causes. 38
27446792 2016
17
[Extraskeletal Ewing sarcoma in a 16-year-old female adolescent]. 38
29421208 2016
18
[Treatments of Soft Tissue Sarcomas by Orthopaedic Surgeons in Japan]. 38
26809525 2016
19
Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma. 38
26425232 2015
20
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. 38
26175577 2015
21
Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma. 38
24814278 2015
22
[Epidural extraskeletal Ewing sarcoma. Case report and literature review]. 38
25497289 2015
23
Phase I and pharmacokinetic study of trabectedin, a DNA minor groove binder, administered as a 24-h continuous infusion in Japanese patients with soft tissue sarcoma. 38
24696229 2014
24
Prevalence of malignant soft tissue tumors in extremities: an epidemiological study in syria. 38
25207328 2014
25
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients. 38
24734938 2014
26
Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. 38
25674292 2014
27
An unusual tumor of the breast - extraskeletal ewing sarcoma. 38
24791212 2014
28
Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin. 38
23982393 2013
29
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. 38
24136179 2013
30
From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. 38
23674776 2013
31
Extraskeletal Ewing sarcomas in late adolescence and adults: a study of 37 patients. 38
23803063 2013
32
Requesting clarifications concerning the case report on 'extraskeletal Ewing sarcoma of the sinonasal tract. 38
23361294 2012
33
Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement. 38
22184129 2012
34
Extraskeletal Ewing sarcoma of the mesocolon in a child. 38
22974628 2012
35
High-grade sarcoma masquerading as growing teratoma syndrome after resection of ovarian immature teratoma: report of a case. 38
22498946 2012
36
Metastatic renal extraskeletal Ewing sarcoma in complete remission for the last 8 years. 38
20535010 2012
37
Primary extraskeletal Ewing sarcoma involving the carotid artery: a case report and review of the current literature. 38
22613280 2012
38
Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. 38
21805144 2012
39
CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. 38
20676795 2012
40
Extradural nodular fasciitis arising in the spinal canal. 38
21629160 2012
41
A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. 38
22109315 2011
42
A case of extraskeletal Ewing sarcoma originating from the visceral pleura. 38
24391423 2011
43
Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. 38
21692057 2011
44
A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. 38
21113140 2011
45
Primary extraosseous ewing sarcoma of the kidney with level III inferior vena cava thrombus. 38
19815490 2009
46
Current concepts and future perspectives in retroperitoneal soft-tissue sarcoma management. 38
19671034 2009
47
Extraskeletal Ewing sarcoma of the abdominal wall. 38
18818133 2008
48
Radiological findings of atypical extraskeletal Ewing sarcoma. 38
18569849 2008
49
Image of the month--quiz case. Retroperitoneal extraskeletal Ewing sarcoma. 38
18490564 2008
50
Radiological findings of primary retroperitoneal Ewing sarcoma. 38
17729015 2007

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 SMARCA5 FUS EWSR1 ERG
2 11.19 FUS FLI1 EWSR1 ERG

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 VIM DES

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 VIM SMARCA5 PTPRC NKX2-1 MUC1 FUS
2 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.26 NKX2-1 MUC1 FLI1 ERG

Sources for Extraskeletal Ewing Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
Content
Loading form....