MCID: EXT065
MIFTS: 34

Extraosseous Ewing Sarcoma

Categories: Bone diseases, Cancer diseases, Neuronal diseases

Aliases & Classifications for Extraosseous Ewing Sarcoma

MalaCards integrated aliases for Extraosseous Ewing Sarcoma:

Name: Extraosseous Ewing Sarcoma 12
Extraosseous Ewing's Sarcoma 12 15
Extraosseous Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Sarcoma Extraosseous Ewings 54
Extraskeletal Ewing Sarcom 12

Classifications:



External Ids:

Disease Ontology 12 DOID:4232
NCIt 49 C27293
UMLS 71 C1333514

Summaries for Extraosseous Ewing Sarcoma

Disease Ontology : 12 A Ewing sarcoma that is morphologically indistinguishable from skeletal Ewing sarcoma but is located in extraosseous locations.

MalaCards based summary : Extraosseous Ewing Sarcoma, also known as extraosseous ewing's sarcoma, is related to extraosseous ewings sarcoma-primitive neuroepithelial tumor and extraskeletal ewing sarcoma. An important gene associated with Extraosseous Ewing Sarcoma is CD99 (CD99 Molecule (Xg Blood Group)), and among its related pathways/superpathways is Transcriptional misregulation in cancer. The drugs Histamine and Cyproheptadine have been mentioned in the context of this disorder. Affiliated tissues include bone, kidney and lung.

Related Diseases for Extraosseous Ewing Sarcoma

Diseases related to Extraosseous Ewing Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 261)
# Related Disease Score Top Affiliating Genes
1 extraosseous ewings sarcoma-primitive neuroepithelial tumor 33.6 SYP EWSR1 ENO2 CD99
2 extraskeletal ewing sarcoma 32.7 SYP EWSR1 ENO2 CD99
3 spindle cell sarcoma 31.3 S100B MYOG
4 ewing sarcoma 30.9 SYP S100B MYOG EWSR1 ETV4 ETV1
5 embryonal sarcoma 30.1 S100B MYOG MYOD1
6 supratentorial primitive neuroectodermal tumor 29.8 SYP S100B ENO2 CD99
7 sarcoma 29.4 S100B MYOG MYOD1 EWSR1 ETV1 ENO2
8 medulloblastoma 28.9 SYP S100B MYOG MYOD1 ENO2
9 askin's tumor 28.5 SYP EWSR1 ETV4 ETV1 ENO2 CD99
10 rhabdomyosarcoma 28.3 SYP S100B MYOG MYOD1 EWSR1 ENO2
11 extraskeletal mesenchymal chondrosarcoma 10.5 EWSR1 CD99
12 ewing sarcoma of bone 10.5 EWSR1 CD99
13 pulmonary vein leiomyosarcoma 10.4 MYOD1 CD99
14 bladder leiomyoma 10.4 MYOD1 EWSR1
15 conventional central osteosarcoma 10.4 EWSR1 CD99
16 ovarian cystic teratoma 10.3 SYP CD99
17 primary hepatic neuroendocrine carcinoma 10.3 SYP S100B
18 cellular ependymoma 10.3 SYP CD99
19 bladder benign neoplasm 10.3 MYOD1 EWSR1
20 hypoganglionosis 10.3 SYP S100B
21 cerebral primitive neuroectodermal tumor 10.3 SYP S100B
22 retroperitoneal neuroblastoma 10.3 ENO2 CD99
23 rosette-forming glioneuronal tumor 10.3 SYP S100B
24 middle ear carcinoma 10.3 SYP EWSR1
25 angiocentric glioma 10.3 SYP S100B
26 pleomorphic liposarcoma 10.3 S100B EWSR1
27 kidney rhabdoid cancer 10.3 EWSR1 ENO2
28 ectopic cushing syndrome 10.3 SYP S100B
29 anaplastic ependymoma 10.3 SYP S100B
30 intracranial cysts 10.3 S100B ENO2
31 chordoid meningioma 10.3 SYP S100B
32 bone osteosarcoma 10.3 EWSR1 CD99
33 bednar tumor 10.3 S100B ENO2
34 testicular sex cord-stromal neoplasm 10.2 SYP CD99
35 head injury 10.2 S100B ENO2
36 meningeal melanocytoma 10.2 S100B ENO2
37 ovarian small cell carcinoma 10.2 SYP S100B
38 clear cell meningioma 10.2 SYP S100B
39 post-cardiac arrest syndrome 10.2 S100B ENO2
40 akinetic mutism 10.2 S100B ENO2
41 ossifying fibromyxoid tumor 10.2 S100B ENO2
42 traumatic brain injury 10.2 S100B ENO2
43 chordoid glioma 10.2 SYP S100B
44 brain injury 10.2 S100B ENO2
45 pulmonary artery leiomyosarcoma 10.2 MYOD1 ENO2
46 optic nerve glioma 10.2 S100B ENO2
47 malignant melanocytic neoplasm of the peripheral nerve sheath 10.2 SYP ENO2
48 myxopapillary ependymoma 10.2 SYP S100B
49 ureter small cell carcinoma 10.2 SYP ENO2
50 sertoli-leydig cell tumor 10.2 SYP ENO2

Graphical network of the top 20 diseases related to Extraosseous Ewing Sarcoma:



Diseases related to Extraosseous Ewing Sarcoma

Symptoms & Phenotypes for Extraosseous Ewing Sarcoma

Drugs & Therapeutics for Extraosseous Ewing Sarcoma

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

(show all 39)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Histamine Approved, Investigational Phase 3 51-45-6 774
2
Cyproheptadine Approved Phase 3 129-03-3 2913
3 Gastrointestinal Agents Phase 3
4 Neurotransmitter Agents Phase 3
5 Dermatologic Agents Phase 3
6 Antipruritics Phase 3
7 Histamine H1 Antagonists Phase 3
8 Serotonin Agents Phase 3
9 Histamine Antagonists Phase 3
10
Histamine Phosphate Phase 3 51-74-1 65513
11 Serotonin Antagonists Phase 3
12 Anti-Allergic Agents Phase 3
13
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
14
Topotecan Approved, Investigational Phase 2 119413-54-6, 123948-87-8 60700
15
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
16
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
17
Glycerol Approved, Investigational Phase 2 56-81-5 753
18
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
19
Epirubicin Approved Phase 2 56420-45-2 41867
20
Dactinomycin Approved, Investigational Phase 2 50-76-0 2019 457193
21
Ifosfamide Approved Phase 2 3778-73-2 3690
22
Lenograstim Approved, Investigational Phase 2 135968-09-1
23
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
24
Etoposide Approved Phase 2 33419-42-0 36462
25 topoisomerase I inhibitors Phase 2
26 Topoisomerase Inhibitors Phase 2
27 Immunologic Factors Phase 2
28 Alkylating Agents Phase 2
29 Anti-Bacterial Agents Phase 2
30 razoxane Phase 2
31 Antibiotics, Antitubercular Phase 2
32
Liposomal doxorubicin Phase 2 31703
33 Immunosuppressive Agents Phase 2
34 Antirheumatic Agents Phase 2
35 Protective Agents Phase 2
36 Antimitotic Agents Phase 2
37
Isophosphamide mustard Phase 2 0
38 Anti-Infective Agents Phase 2
39 Etoposide phosphate Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
2 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
3 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
4 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 Extraosseous Ewing Sarcoma

Genetic Tests for Extraosseous Ewing Sarcoma

Anatomical Context for Extraosseous Ewing Sarcoma

MalaCards organs/tissues related to Extraosseous Ewing Sarcoma:

40
Bone, Kidney, Lung, Prostate, Pineal, Spinal Cord, Thyroid

Publications for Extraosseous Ewing Sarcoma

Articles related to Extraosseous Ewing Sarcoma:

(show top 50) (show all 175)
# Title Authors PMID Year
1
The first report of extraosseous Ewing's sarcoma in the rectovaginal septum. 54 61
12400989 2002
2
A novel chimera gene between EWS and E1A-F, encoding the adenovirus E1A enhancer-binding protein, in extraosseous Ewing's sarcoma. 54 61
8605035 1996
3
Myogenic regulatory protein (MyoD1) expression in childhood solid tumors: diagnostic utility in rhabdomyosarcoma. 54 61
2260621 1990
4
Primary Ewing sarcoma of the adrenal gland: A rare cause of abdominal mass. 61
31737137 2020
5
Primary Extraosseous Ewing Sarcoma of the Maxillary Sinus in an Adult-A Rare Case Report. 61
31857985 2019
6
Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature. 61
31688622 2019
7
Primary Ewing sarcoma of the larynx with distant metastasis: a case report and review of the literature. 61
31548827 2019
8
An Extraosseous Ewing Sarcoma of the Index Finger Masquerading as a Benign Tumor. 61
31327500 2019
9
Ewing Sarcoma Presenting as a Congenital Scalp Mass. 61
29989837 2019
10
Primary intracranial extraosseous Ewing's sarcoma. 61
30353305 2019
11
[Primary extra-osseous Ewing sarcoma of the dorsal spine with spinal cord compression at diagnosis: Case report]. 61
30652454 2019
12
Primary intradural Ewing's sarcoma of the spine: a systematic review of the literature. 61
30579048 2019
13
Extraosseous Ewing's Sarcoma of the Parapharyngeal Space - A Rare Entity - with Review of Literature. 61
30783599 2019
14
[Extraosseous Ewing sarcoma]. 61
31284263 2019
15
Rhabdomyosarcoma and Extraosseous Ewing Sarcoma. 61
30544742 2018
16
Augmented reality in computer-assisted interventions based on patient-specific 3D printed reference. 61
30464847 2018
17
Extraosseous Ewing sarcoma arising in a chronically lymphedematous limb. 61
30148241 2018
18
The Successful Treatment of Metastatic Extraosseous Ewing Sarcoma with Pazopanib. 61
29780156 2018
19
Extra-Osseous Ewing Sarcoma of Sciatic Nerve Masquerading as Benign Nerve Sheath Tumor and Presented as Lumbar Radiculopathy: Case Report and Review of Literature. 61
29673820 2018
20
Peripheral primitive neuroendocrine tumor of the chest wall-A case report with pathological correlation. 61
29904480 2018
21
Extraosseous Ewing sarcoma arising at the papilla of Vater. 61
29557408 2018
22
Lower Extremity Weakness in an Adolescent Female - A Rare Presentation of Ewing Sarcoma. 61
30559628 2018
23
Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature. 61
29804110 2018
24
Extraosseous Ewing's Sarcoma of the Pancreas: An Uncommon but Treatable Disease. 61
29387511 2017
25
Extraosseous Ewing sarcoma: Expanding the differential diagnosis of supraclavicular fossa tumors. 61
28122109 2017
26
Extraosseous Ewing sarcoma in the lumbar epidural space: an uncommon malignant tumor mimicking a spinal schwannoma. 61
26968238 2016
27
The Importance of Surgery as Part of Multimodal Therapy in Rapid Progressive Primary Extraosseous Ewing Sarcoma of the Cervical Intra- and Epidural Space. 61
28176976 2016
28
Primary intradural extraosseous Ewing's sarcoma in a young child. 61
26780782 2016
29
Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management. 61
27186040 2016
30
Adult Primary Spinal Epidural Extraosseous Ewing's Sarcoma: A Case Report and Review of the Literature. 61
27610254 2016
31
High EZH2 expression is correlated to metastatic disease in pediatric soft tissue sarcomas. 61
27471434 2016
32
Primary Ewing Sarcoma Presenting as a Vulvar Mass in an Adolescent: Case Report and Review of Literature. 61
26211932 2015
33
Extraosseous Ewing sarcoma in the mesentery: the first report of cases in children. 61
26280743 2015
34
Intradural extramedullary extraosseous Ewing sarcoma/PNET of foramen magnum. 61
25092552 2015
35
[Primary presentation of extraosseous Ewing's sarcoma]. 61
25002348 2015
36
Primary extraosseous Ewing sarcoma of the lumbar spine presenting with left leg weakness. 61
25724431 2015
37
Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma. 61
26266019 2015
38
Extraskeletal Ewing's Sarcoma Arising from the Sciatic Nerve: A Diagnostic Challenge. 61
26236533 2015
39
Pazopanib for recurrent extraosseous Ewing's sarcoma of the retroperitoneum. 61
25040171 2014
40
Evaluation of outcome and prognostic factors in extraosseous Ewing sarcoma. 61
25132242 2014
41
Extraosseous Ewing's sarcoma arising in the perineal region (Rectovaginal Septum): a case report with respect to the usefulness of cytological examination. 61
24106051 2014
42
Appearance of extraosseous pelvic Ewing sarcoma on triphasic bone scan. 61
24566405 2014
43
Primary Ewing's Sarcoma of the Lung. 61
24570867 2014
44
Two Cases of Spinal, Extraosseous, Intradural Ewing's sarcoma/Peripheral Neuroectodermal Tumor: Radiologic, Pathologic, and Molecular Analysis. 61
24678438 2014
45
Primary intradural extraosseous Ewing's sarcoma of the lumbar spine presenting with acute bleeding. 61
23472623 2013
46
A rare case of extraosseous Ewing sarcoma primarily arising in the ovary. 61
24286435 2013
47
Role of color-Doppler US in the evaluation of scrotal edema. 61
24432179 2013
48
An Unusual Location of Extraosseous Ewing's Sarcoma. 61
23898272 2013
49
Primary pancreatic Ewing's sarcoma with portal vein tumor thrombosis. 61
23192427 2013
50
Primary extraosseous Ewing sarcoma of the orbit. 61
23247035 2013

Variations for Extraosseous Ewing Sarcoma

Expression for Extraosseous Ewing Sarcoma

Search GEO for disease gene expression data for Extraosseous Ewing Sarcoma.

Pathways for Extraosseous Ewing Sarcoma

Pathways related to Extraosseous Ewing Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.07 EWSR1 ETV4 ETV1

GO Terms for Extraosseous Ewing Sarcoma

Biological processes related to Extraosseous Ewing Sarcoma according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 cellular response to estradiol stimulus GO:0071392 9.49 MYOG MYOD1
2 positive regulation of muscle cell differentiation GO:0051149 9.48 MYOG MYOD1
3 skeletal muscle fiber development GO:0048741 9.46 MYOG MYOD1
4 positive regulation of myoblast differentiation GO:0045663 9.43 MYOG MYOD1
5 positive regulation of myoblast fusion GO:1901741 9.4 MYOG MYOD1
6 regulation of neuronal synaptic plasticity GO:0048168 9.37 SYP S100B
7 muscle organ development GO:0007517 9.33 MYOG MYOD1 ETV1
8 myoblast differentiation GO:0045445 9.32 MYOG MYOD1
9 myotube differentiation GO:0014902 9.26 MYOG MYOD1
10 positive regulation of skeletal muscle fiber development GO:0048743 8.96 MYOG MYOD1
11 muscle cell fate commitment GO:0042693 8.62 MYOG MYOD1

Molecular functions related to Extraosseous 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 9.26 MYOG MYOD1 ETV4 ETV1
2 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 8.92 MYOG MYOD1 ETV4 ETV1

Sources for Extraosseous Ewing Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
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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