MCID: EXT065
MIFTS: 33

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 15
Extraosseous Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Extraosseous Ewing's Sarcoma 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/peripheral primitive neuroectodermal tumor, 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, lung and kidney.

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 256)
# Related Disease Score Top Affiliating Genes
1 extraosseous ewings sarcoma-primitive neuroepithelial tumor 33.7 SYP EWSR1 ENO2 CD99
2 extraskeletal ewing sarcoma 32.7 SYP EWSR1 ENO2 CD99
3 ewing sarcoma 31.3 SYP S100B MYOG EWSR1 ETV4 ETV1
4 spindle cell sarcoma 31.2 S100B MYOG
5 supratentorial primitive neuroectodermal tumor 30.3 SYP S100B CD99
6 embryonal sarcoma 30.1 S100B MYOG MYOD1
7 exophthalmos 30.1 SYP ENO2
8 sarcoma 29.8 S100B MYOG MYOD1 EWSR1 ETV1 ENO2
9 connective tissue benign neoplasm 29.7 EWSR1 ENO2
10 medulloblastoma 28.9 SYP S100B MYOG MYOD1 ENO2
11 askin's tumor 28.4 TSFM SYP EWSR1 ETV4 ETV1 ENO2
12 rhabdomyosarcoma 28.3 SYP S100B MYOG MYOD1 EWSR1 ENO2
13 extraskeletal mesenchymal chondrosarcoma 10.4 EWSR1 CD99
14 pulmonary vein leiomyosarcoma 10.4 MYOD1 CD99
15 conventional central osteosarcoma 10.3 EWSR1 CD99
16 cellular ependymoma 10.3 SYP CD99
17 primary hepatic neuroendocrine carcinoma 10.3 SYP S100B
18 hypoganglionosis 10.3 SYP S100B
19 cerebral primitive neuroectodermal tumor 10.3 SYP S100B
20 retroperitoneal neuroblastoma 10.3 ENO2 CD99
21 rosette-forming glioneuronal tumor 10.3 SYP S100B
22 angiocentric glioma 10.3 SYP S100B
23 kidney clear cell sarcoma 10.3 EWSR1 CD99
24 ectopic cushing syndrome 10.3 SYP S100B
25 anaplastic ependymoma 10.2 SYP S100B
26 pleomorphic liposarcoma 10.2 S100B EWSR1
27 intracranial cysts 10.2 S100B ENO2
28 bednar tumor 10.2 S100B ENO2
29 kidney rhabdoid cancer 10.2 EWSR1 ENO2
30 chordoid meningioma 10.2 SYP S100B
31 bone osteosarcoma 10.2 EWSR1 CD99
32 head injury 10.2 S100B ENO2
33 meningeal melanocytoma 10.2 S100B ENO2
34 chordoid glioma 10.2 SYP S100B
35 akinetic mutism 10.2 S100B ENO2
36 clear cell meningioma 10.2 SYP S100B
37 post-cardiac arrest syndrome 10.2 S100B ENO2
38 ossifying fibromyxoid tumor 10.2 S100B ENO2
39 traumatic brain injury 10.2 S100B ENO2
40 brain injury 10.2 S100B ENO2
41 myxopapillary ependymoma 10.2 SYP S100B
42 rhabdoid cancer 10.2 MYOG EWSR1
43 malignant melanocytic neoplasm of the peripheral nerve sheath 10.2 SYP ENO2
44 anal neuroendocrine tumor 10.2 SYP ENO2
45 breast neuroendocrine neoplasm 10.2 SYP ENO2
46 middle cerebral artery infarction 10.2 S100B ENO2
47 sinonasal undifferentiated carcinoma 10.2 SYP ENO2
48 papillary tumor of the pineal region 10.2 SYP ENO2
49 small cell carcinoma of the bladder 10.2 SYP ENO2
50 bile duct mucinous adenocarcinoma 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 37)
# 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 Neurotransmitter Agents Phase 3
4 Histamine Antagonists Phase 3
5 Anti-Allergic Agents Phase 3
6
Histamine Phosphate Phase 3 51-74-1 65513
7 Histamine H1 Antagonists Phase 3
8 Dermatologic Agents Phase 3
9 Gastrointestinal Agents Phase 3
10
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
11
Topotecan Approved, Investigational Phase 2 123948-87-8, 119413-54-6 60700
12
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
13
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
14
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
15
Glycerol Approved, Investigational Phase 2 56-81-5 753
16
Dactinomycin Approved, Investigational Phase 2 50-76-0 457193 2019
17
Etoposide Approved Phase 2 33419-42-0 36462
18
Epirubicin Approved Phase 2 56420-45-2 41867
19
Lenograstim Approved, Investigational Phase 2 135968-09-1
20
Ifosfamide Approved Phase 2 3778-73-2 3690
21
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
22 topoisomerase I inhibitors Phase 2
23 Antibiotics, Antitubercular Phase 2
24 Immunologic Factors Phase 2
25
Liposomal doxorubicin Phase 2 31703
26 razoxane Phase 2
27 Cardiotonic Agents Phase 2
28 Immunosuppressive Agents Phase 2
29 Alkylating Agents Phase 2
30 Tubulin Modulators Phase 2
31 Protective Agents Phase 2
32 Antirheumatic Agents Phase 2
33 Antimitotic Agents Phase 2
34 Anti-Bacterial Agents Phase 2
35 Etoposide phosphate Phase 2
36 Anti-Infective Agents Phase 2
37
Isophosphamide mustard Phase 2 0

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, Lung, Kidney, Thyroid, Adrenal Gland, Bone Marrow, Brain

Publications for Extraosseous Ewing Sarcoma

Articles related to Extraosseous Ewing Sarcoma:

(show top 50) (show all 73)
# Title Authors PMID Year
1
An Extraosseous Ewing Sarcoma of the Index Finger Masquerading as a Benign Tumor. 61
31327500 2020
2
Primary Ewing sarcoma of the adrenal gland: A rare cause of abdominal mass. 61
31737137 2020
3
A Nonpediatric Extraosseous Ewing Sarcoma of the Pancreas: Differential Diagnosis and Therapeutic Strategies. 61
32082662 2020
4
Extraosseous Ewing Sarcoma Presenting with Inferior ST-Elevation Myocardial Infarction and Systemic Emboli due to Tumor Thrombus and Invasion of the Left Atrium. 61
32047685 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
Ewing Sarcoma Presenting as a Congenital Scalp Mass. 61
29989837 2019
9
[Primary extra-osseous Ewing sarcoma of the dorsal spine with spinal cord compression at diagnosis: Case report]. 61
30652454 2019
10
[Extraosseous Ewing sarcoma]. 61
31284263 2019
11
Rhabdomyosarcoma and Extraosseous Ewing Sarcoma. 61
30544742 2018
12
The Successful Treatment of Metastatic Extraosseous Ewing Sarcoma with Pazopanib. 61
29780156 2018
13
Extraosseous Ewing sarcoma arising in a chronically lymphedematous limb. 61
30148241 2018
14
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
15
Extraosseous Ewing sarcoma arising at the papilla of Vater. 61
29557408 2018
16
Primary Spinal Intradural Extraosseous Ewing Sarcoma in a Pediatric Patient: Case Report and Review of the Literature. 61
29804110 2018
17
Lower Extremity Weakness in an Adolescent Female - A Rare Presentation of Ewing Sarcoma. 61
30559628 2018
18
Extraosseous Ewing sarcoma: Expanding the differential diagnosis of supraclavicular fossa tumors. 61
28122109 2017
19
Extraosseous Ewing sarcoma in the lumbar epidural space: an uncommon malignant tumor mimicking a spinal schwannoma. 61
26968238 2016
20
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
21
Extraosseous Ewing Sarcoma: Diagnosis, Prognosis and Optimal Management. 61
27186040 2016
22
High EZH2 expression is correlated to metastatic disease in pediatric soft tissue sarcomas. 61
27471434 2016
23
Primary Ewing Sarcoma Presenting as a Vulvar Mass in an Adolescent: Case Report and Review of Literature. 61
26211932 2015
24
Extraosseous Ewing sarcoma in the mesentery: the first report of cases in children. 61
26280743 2015
25
Intradural extramedullary extraosseous Ewing sarcoma/PNET of foramen magnum. 61
25092552 2015
26
Primary extraosseous Ewing sarcoma of the lumbar spine presenting with left leg weakness. 61
25724431 2015
27
Clinical Activity of Pazopanib in Metastatic Extraosseous Ewing Sarcoma. 61
26266019 2015
28
Evaluation of outcome and prognostic factors in extraosseous Ewing sarcoma. 61
25132242 2014
29
Appearance of extraosseous pelvic Ewing sarcoma on triphasic bone scan. 61
24566405 2014
30
A rare case of extraosseous Ewing sarcoma primarily arising in the ovary. 61
24286435 2013
31
Primary extraosseous Ewing sarcoma of the orbit. 61
23247035 2013
32
Extraosseous Ewing sarcoma and peripheral primitive neuroectodermal tumor of the thyroid gland: Case report and review. 61
23599116 2013
33
Extraosseous Ewing sarcoma of the vagina: a rare entity. 61
23563009 2013
34
Primary extraosseous Ewing sarcoma of the lung: Case report and literature review. 61
27330628 2013
35
Retraction: Primary extraosseous Ewing sarcoma of the lung in children. 61
23858324 2013
36
Primary extraosseous Ewing sarcoma of the lung in children. 61
23653672 2013
37
Primary pediatric endobronchial Ewing sarcoma family of tumors. 61
23569566 2013
38
Analysis of prognostic factors in extraosseous Ewing sarcoma family of tumors: review of St. Jude Children's Research Hospital experience. 61
22739653 2012
39
Extraosseous Ewing sarcoma with foci of neuroblastoma-like differentiation associated with EWSR1(Ewing sarcoma breakpoint region 1)/FLI1 translocation without prior chemotherapy. 61
22703924 2012
40
Extraosseous ewing sarcoma of the tonsil. 61
22935188 2012
41
IVC tumor thrombus: an advanced case of rare extraosseous Ewing sarcoma of the adrenal gland. 61
22656426 2012
42
Primary intradural extraosseous Ewing sarcoma of the spine: case report and literature review. 61
21572359 2011
43
Giant extraosseous Ewing sarcoma of the lung in a young adolescent female--a case report. 61
21667743 2011
44
A primary extraosseous Ewing sarcoma in the cerebellopontine angle of a child: review of relevant literature and case report. 61
21107154 2010
45
Extraosseous Ewing sarcoma of the thyroid gland. 61
19690849 2009
46
Primary extraosseous ewing sarcoma of the kidney with level III inferior vena cava thrombus. 61
19815490 2009
47
Primary pediatric intraspinal sarcomas. Report of 3 cases. 61
19772405 2009
48
Primary vaginal extraosseous Ewing sarcoma/primitive neuroectodermal tumor with cranial metastasis. 61
19541570 2009
49
Primary spinal epidural Ewing sarcoma: a case report and review of the literature. 61
18773677 2008
50
Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma. 61
18345719 2008

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 11.08 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 MYOD1 MYOG
2 positive regulation of muscle cell differentiation GO:0051149 9.48 MYOD1 MYOG
3 skeletal muscle fiber development GO:0048741 9.46 MYOD1 MYOG
4 positive regulation of myoblast differentiation GO:0045663 9.43 MYOD1 MYOG
5 regulation of neuronal synaptic plasticity GO:0048168 9.4 S100B SYP
6 positive regulation of myoblast fusion GO:1901741 9.37 MYOD1 MYOG
7 muscle organ development GO:0007517 9.33 ETV1 MYOD1 MYOG
8 myoblast differentiation GO:0045445 9.32 MYOD1 MYOG
9 myotube differentiation GO:0014902 9.26 MYOD1 MYOG
10 positive regulation of skeletal muscle fiber development GO:0048743 8.96 MYOD1 MYOG
11 muscle cell fate commitment GO:0042693 8.62 MYOD1 MYOG

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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