EOE
MCID: EXT052
MIFTS: 33

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 58
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 71
Extraosseous Ewing Sarcoma 58
Extraskeletal Ewing Tumor 58
Extraosseous Ewing Tumor 58
Eoe 58

Classifications:



External Ids:

ICD10 via Orphanet 33 C49.9
UMLS via Orphanet 72 C0279980 C0852530
Orphanet 58 ORPHA370334
UMLS 71 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 ewings sarcoma-primitive neuroepithelial tumor and spindle cell 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 Topotecan and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and ovary.

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 358)
# Related Disease Score Top Affiliating Genes
1 extraosseous ewings sarcoma-primitive neuroepithelial tumor 33.4 SYP PTPRC EWSR1 ENO2 CD99
2 spindle cell sarcoma 31.6 VIM MUC1 DES
3 rare tumor 31.0 EWSR1 DES
4 supratentorial primitive neuroectodermal tumor 30.3 SYP ENO2 CD99
5 extraosseous ewing sarcoma 30.2 SYP EWSR1 ENO2 CD99
6 ewing sarcoma of bone 30.1 VIM PTPRC EWSR1 DES CD99
7 liposarcoma 30.1 VIM FUS EWSR1 DES
8 neurilemmoma 30.1 VIM MUC1 ENO2 DES
9 ewing sarcoma 29.7 VIM SYP PTPRC FUS FLI1 EWSR1
10 rhabdomyosarcoma 28.9 VIM SYP PTPRC EWSR1 ENO2 DES
11 sarcoma 28.9 VIM PTPRC MUC1 FUS FLI1 EWSR1
12 esophagitis, eosinophilic, 1 12.6
13 esophagitis 10.8
14 pulmonary vein stenosis 10.5 DES CD99
15 infantile digital fibromatosis 10.5 VIM DES
16 lymphangiomatosis 10.5 VIM DES
17 retroperitoneal neuroblastoma 10.5 ENO2 CD99
18 hypereosinophilic syndrome 10.5
19 neurohypophysis granular cell tumor 10.5 VIM ENO2
20 benign metastasizing leiomyoma 10.5 VIM DES
21 bednar tumor 10.5 VIM ENO2
22 vulvar apocrine adenocarcinoma 10.5 PTPRC ENO2
23 extraskeletal mesenchymal chondrosarcoma 10.4 EWSR1 CD99
24 retroperitoneal fibrosis 10.4 VIM PTPRC
25 heart lymphoma 10.4 VIM PTPRC
26 angiolipoma 10.4 VIM DES
27 glomangioma 10.4 VIM DES
28 central nervous system mesenchymal non-meningothelial tumor 10.4 EWSR1 CD99
29 embryonal sarcoma 10.4 VIM DES
30 appendix lymphoma 10.4 PTPRC ENO2
31 kidney rhabdoid cancer 10.4 EWSR1 ENO2
32 myxofibrosarcoma 10.4 FUS DES
33 pilomatrix carcinoma 10.4 MUC1 ENO2
34 conventional fibrosarcoma 10.4 PTPRC ENO2
35 intracranial cysts 10.4 MUC1 ENO2
36 pediatric liposarcoma 10.4 VIM FUS
37 bladder leiomyoma 10.4 VIM EWSR1
38 myxoid liposarcoma of the ovary 10.3 FUS EWSR1
39 liposarcoma of the ovary 10.3 FUS EWSR1
40 chondromyxoid fibroma 10.3 VIM DES
41 syringocystadenoma papilliferum 10.3 MUC1 DES
42 meningeal melanocytoma 10.3 MUC1 ENO2
43 ectomesenchymoma 10.3 ENO2 DES CD99
44 primary cutaneous anaplastic large cell lymphoma 10.3 PTPRC MUC1
45 eccrine porocarcinoma 10.3 VIM MUC1
46 nodular hidradenoma 10.3 VIM MUC1
47 hidradenoma 10.3 VIM MUC1
48 endometrial mucinous adenocarcinoma 10.3 VIM MUC1
49 proximal spinal muscular atrophy 10.3 MUC1 DES
50 spindle cell lipoma 10.3 VIM DES CD99

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

Drugs & Therapeutics for Extraskeletal Ewing Sarcoma

Drugs for Extraskeletal Ewing Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 42)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Topotecan Approved, Investigational Phase 3 119413-54-6, 123948-87-8 60700
2
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
3
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
4
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
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 Immunologic Factors Phase 3
14 Alkylating Agents Phase 3
15 Anti-Bacterial Agents Phase 3
16 Antibiotics, Antitubercular Phase 3
17
Liposomal doxorubicin Phase 3 31703
18 Immunosuppressive Agents Phase 3
19 Antirheumatic Agents Phase 3
20 Antimitotic Agents Phase 3
21
Isophosphamide mustard Phase 3 0
22 Etoposide phosphate Phase 3
23 Podophyllotoxin Phase 3 518-28-5
24 Gastrointestinal Agents Phase 3
25 Neurotransmitter Agents Phase 3
26 Dermatologic Agents Phase 3
27 Antipruritics Phase 3
28 Histamine H1 Antagonists Phase 3
29 Serotonin Agents Phase 3
30 Histamine Antagonists Phase 3
31
Histamine Phosphate Phase 3 51-74-1 65513
32 Serotonin Antagonists Phase 3
33 Anti-Allergic Agents Phase 3
34
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
35
Glycerol Approved, Investigational Phase 2 56-81-5 753
36
Epirubicin Approved Phase 2 56420-45-2 41867
37
Dactinomycin Approved, Investigational Phase 2 50-76-0 2019 457193
38
Lenograstim Approved, Investigational Phase 2 135968-09-1
39
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
40 razoxane Phase 2
41 Protective Agents Phase 2
42 Anti-Infective Agents 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
5 MMT 98 Study For Metastatic Disease Rhabdomyosarcoma And Other Malignant Soft Tissue Sarcoma Of Childhood Completed NCT00025441 Phase 2 carboplatin;cyclophosphamide;doxorubicin hydrochloride;epirubicin hydrochloride;etoposide;ifosfamide;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:

40
Lung, Bone, Ovary, Kidney, Uterus, Pineal, Heart

Publications for Extraskeletal Ewing Sarcoma

Articles related to Extraskeletal Ewing Sarcoma:

(show top 50) (show all 74)
# Title Authors PMID Year
1
PAX8-positive, Cytokeratin-positive Intra-Abdominal Ewing Sarcoma in a Postmenopausal Female Masquerading as a Mullerian Carcinoma. 61
31855951 2019
2
Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature. 61
31688622 2019
3
Primary Extraskeletal Ewing Sarcoma of the Parotid Gland. 61
31547700 2019
4
FDG PET/CT in a Case of Primary Pulmonary Ewing Sarcoma. 61
31274619 2019
5
Gastrointestinal: Multi-modality imaging of extraskeletal Ewing sarcoma of the stomach. 61
30912185 2019
6
Large extraskeletal ewing sarcoma of the hand: A rare entity. 61
31417021 2019
7
FDG PET/CT in a Case of Primary Pulmonary Ewing Sarcoma. 61
31162265 2019
8
Extraskeletal Ewing Sarcoma of the Jejunum: A Case Report. 61
31314729 2019
9
Role of Radiation Therapy in Adult Extraskeletal Ewing's Sarcoma Patients Treated with Chemotherapy and Surgery. 61
31178655 2019
10
CIC-DUX4 sarcoma diagnosed by fine-needle aspiration cytology: A case report. 61
30353686 2018
11
Extraskeletal Ewing Sarcoma: Report of an Extremely Rare Case in Temporal Region. 61
30842800 2018
12
Extraskeletal versus Skeletal Ewing Sarcoma in the adult population: Controversies in care. 61
30217290 2018
13
Rare case of small size Extraskeletal Ewing Sarcoma arising from the median nerve with paresthesia. 61
27523258 2018
14
Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases. 61
30538569 2018
15
Ewing sarcoma in patients over 40 years of age: a prospective analysis of 31 patients treated at a single institution. 61
27443894 2016
16
Dumbbell Scoring System: A New Method for the Differential Diagnosis of Malignant and Benign Spinal Dumbbell Tumors. 61
27031767 2016
17
Primary extraskeletal Ewing sarcoma of the stomach: a rare disease in an uncommon location. 61
27179157 2016
18
Treatment outcomes of Japanese patients with Ewing sarcoma: differences between skeletal and extraskeletal Ewing sarcoma. 61
27008849 2016
19
Thoracic Radiculopathy due to Rare Causes. 61
27446792 2016
20
[Extraskeletal Ewing sarcoma in a 16-year-old female adolescent]. 61
29421208 2016
21
[Treatments of Soft Tissue Sarcomas by Orthopaedic Surgeons in Japan]. 61
26809525 2016
22
Wunderlich syndrome as the first manifestation of an extraskeletal Ewing sarcoma. 61
26425232 2015
23
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. 61
26175577 2015
24
Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma. 61
24814278 2015
25
[Epidural extraskeletal Ewing sarcoma. Case report and literature review]. 61
25497289 2015
26
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. 61
24696229 2014
27
Prevalence of malignant soft tissue tumors in extremities: an epidemiological study in syria. 61
25207328 2014
28
Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients. 61
24734938 2014
29
Primary spinal intradural extraskeletal Ewing sarcoma mimicking a giant nerve sheath tumor: case report and review of the literature. 61
25674292 2014
30
An unusual tumor of the breast - extraskeletal ewing sarcoma. 61
24791212 2014
31
Extraskeletal Ewing sarcoma in children and adolescents: impact of narrow but negative surgical margin. 61
23982393 2013
32
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. 61
24136179 2013
33
From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. 61
23674776 2013
34
Extraskeletal Ewing sarcomas in late adolescence and adults: a study of 37 patients. 61
23803063 2013
35
Requesting clarifications concerning the case report on 'extraskeletal Ewing sarcoma of the sinonasal tract. 61
23361294 2012
36
Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement. 61
22184129 2012
37
Extraskeletal Ewing sarcoma of the mesocolon in a child. 61
22974628 2012
38
High-grade sarcoma masquerading as growing teratoma syndrome after resection of ovarian immature teratoma: report of a case. 61
22498946 2012
39
Metastatic renal extraskeletal Ewing sarcoma in complete remission for the last 8 years. 61
20535010 2012
40
Primary extraskeletal Ewing sarcoma involving the carotid artery: a case report and review of the current literature. 61
22613280 2012
41
CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor. 61
20676795 2012
42
Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. 61
21805144 2012
43
Extradural nodular fasciitis arising in the spinal canal. 61
21629160 2012
44
A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. 61
22109315 2011
45
A case of extraskeletal Ewing sarcoma originating from the visceral pleura. 61
24391423 2011
46
Clinical features and outcomes in patients with extraskeletal Ewing sarcoma. 61
21692057 2011
47
A novel t(4;22)(q31;q12) produces an EWSR1-SMARCA5 fusion in extraskeletal Ewing sarcoma/primitive neuroectodermal tumor. 61
21113140 2011
48
Primary extraosseous ewing sarcoma of the kidney with level III inferior vena cava thrombus. 61
19815490 2009
49
Current concepts and future perspectives in retroperitoneal soft-tissue sarcoma management. 61
19671034 2009
50
Extraskeletal Ewing sarcoma of the abdominal wall. 61
18818133 2008

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 regulation of transcription by RNA polymerase II GO:0006357 9.35 SMARCA5 NKX2-1 FUS FLI1 ERG
2 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 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 8.92 NKX2-1 MUC1 FLI1 ERG

Sources for Extraskeletal Ewing Sarcoma

3 CDC
7 CNVD
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10 dbSNP
11 DGIdb
17 EFO
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35 IUPHAR
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48 NCI
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50 NDF-RT
53 NINDS
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56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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