MCID: EWN003
MIFTS: 64

Ewing Sarcoma

Categories: Genetic diseases, Rare diseases, Neuronal diseases, Bone diseases, Cancer diseases

Aliases & Classifications for Ewing Sarcoma

MalaCards integrated aliases for Ewing Sarcoma:

Name: Ewing Sarcoma 54 12 50 25 56 71 13 14
Neuroepithelioma 54 50 56 52 69
Ewing's Tumor 12 50 25 71
Ewing's Sarcoma 50 25 29
Ewings Sarcoma 12 52 69
Localized Peripheral Primitive Neuroectodermal Tumor 12 69
Ewings Sarcoma-Primitive Neuroectodermal Tumor 12 69
Peripheral Primitive Neuroectodermal Tumor 12 56
Peripheral Neuroepithelioma 71 52
Askin Tumor 71 42
Ewing Tumor 50 25
Localized Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Extraosseous Ewings Sarcoma-Primitive Neuroepithelial Tumor 69
Ewing's Sarcoma/peripheral Primitive Neuroectodermal Tumor 12
Neuroectodermal Tumors, Primitive, Peripheral 42
Neuroectodermal Tumor, Primitive 69
Pnet of Thoracopulmonary Region 12
Primitive Neuroectodermal Tumor 71
Ewing's Family Localized Tumor 12
Ewing Sarcoma Family of Tumors 71
Neuroepithelioma, Peripheral 69
Localized Ewing's Sarcoma 12
Tumor of the Ewing Family 25
Extraosseous Ewing Tumor 71
Localized Ewing Sarcoma 12
Localized Ewing's Tumor 12
Ewing Family of Tumors 25
Pnet of the Chest Wall 71
Sarcoma, Ewing's 50
Peripheral Pnet 56
Sarcoma, Ewing 42
Askin's Tumor 69
Ppnet 56
Pnet 71
Esft 71
Pne 71
Es 71

Characteristics:

Orphanet epidemiological data:

56
ewing sarcoma
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe),1-9/100000 (Europe); Age of onset: Childhood;
neuroepithelioma
Inheritance: Not applicable; Age of onset: Childhood;

HPO:

32
ewing sarcoma:
Inheritance somatic mutation


Classifications:



External Ids:

OMIM 54 612219
Disease Ontology 12 DOID:3369
UMLS via Orphanet 70 C0553580 C0027828 C0684337
MESH via Orphanet 43 D018241
SNOMED-CT via HPO 65 124975008 76909002

Summaries for Ewing Sarcoma

NIH Rare Diseases : 50 ewing sarcoma is a malignant (cancerous) bone tumor that affects children. it can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. the tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. it may also develop in the skull or the flat bones of the trunk. there are few symptoms. the most common is pain and occasionally swelling at the site of the tumor. fever may also be present. the tumor often spreads (metastasis) to the lungs and other bones. the cause of ewing sarcoma is unknown. most cases are thought to occur randomly and many involved a reciprocal translocation between chromosomes 11 and 22. treatment depends upon a number of factors, but may include chemotherapy, radiation and/or surgical interventions. last updated: 2/13/2016

MalaCards based summary : Ewing Sarcoma, also known as neuroepithelioma, is related to supratentorial primitive neuroectodermal tumor and cerebral primitive neuroectodermal tumor, and has symptoms including ewing's sarcoma and fever. An important gene associated with 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 Afinitor and Erbitux have been mentioned in the context of this disorder. Affiliated tissues include bone, lung and endothelial, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and cellular

UniProtKB/Swiss-Prot : 71 Ewing sarcoma: A highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue that affects children and adolescents. It belongs to the Ewing sarcoma family of tumors, a group of morphologically heterogeneous neoplasms that share the same cytogenetic features. They are considered neural tumors derived from cells of the neural crest. Ewing sarcoma represents the less differentiated form of the tumors.

Genetics Home Reference : 25 Ewing sarcoma is a cancerous tumor that occurs in bones or soft tissues, such as cartilage or nerves. There are several types of Ewing sarcoma, including Ewing sarcoma of bone, extraosseous Ewing sarcoma, peripheral primitive neuroectodermal tumor (pPNET), and Askin tumor. These tumors are considered to be related because they have similar genetic causes. These types of Ewing sarcoma can be distinguished from one another by the tissue in which the tumor develops. Approximately 87 percent of Ewing sarcomas are Ewing sarcoma of bone, which is a bone tumor that usually occurs in the thigh bones (femurs), pelvis, ribs, or shoulder blades. Extraosseous (or extraskeletal) Ewing sarcoma describes tumors in the soft tissues around bones, such as cartilage. pPNETs occur in nerve tissue and can be found in many parts of the body. A type of pPNET found in the chest is called Askin tumor.

OMIM : 54
The Ewing sarcoma family of tumors (primitive neuroectodermal tumors; PNET) comprise morphologically heterogeneous tumors that are characterized by nonrandom chromosomal translocations involving the EWS gene on chromosome 22q12 and one of several members of the ETS family of transcription factors. The tumors include Ewing sarcoma, peripheral neuroepithelioma, and Askin tumor. In approximately 90% of cases of ESFT, the FLI1 gene (193067) on chromosome 11 is the fusion partner of EWS; in approximately 10%, the EWS fusion partner is the ERG gene (165080) on chromosome 22. Many other ETS family members have been identified as fusion partners of EWS, but these cases are rare (Khoury, 2005). (612219)

Disease Ontology : 12 A bone cancer that has_material_basis in neural crest cells derives from undeveloped, undifferentiated neuroectoderm.

Wikipedia : 72 Ewing sarcoma or Ewing\'s sarcoma (/ˈjuːɪŋ/) is a malignant small, round, blue cell tumor. It is a rare... more...

Related Diseases for Ewing Sarcoma

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

(show top 50) (show all 169)
id Related Disease Score Top Affiliating Genes
1 supratentorial primitive neuroectodermal tumor 35.1 CD99 ENO2
2 cerebral primitive neuroectodermal tumor 12.3
3 intracranial primitive neuroectodermal tumor 12.2
4 primitive neuroectodermal tumor of the cervix uteri 12.2
5 extraskeletal ewing sarcoma 12.1
6 supratentorial primitive neuroectodermal tumors, childhood 12.1
7 primitive neuroectodermal tumor of the corpus uteri 12.1
8 extraosseous ewings sarcoma-primitive neuroepithelial tumor 11.9
9 soft tissue peripheral neuroepithelioma 11.9
10 bone peripheral neuroepithelioma 11.8
11 pancreatic neuroendocrine tumor 11.6
12 dissociative seizures 11.6
13 pineoblastoma 11.4
14 vitreous syneresis 11.2 CD99 EWSR1
15 exertional headache 11.2 EWSR1 FLI1 KIT
16 thyroid cancer, anaplastic 11.1 CD99 KIT
17 anal canal adenocarcinoma 11.1 ENO2 EWSR1 PTPRC
18 bone ewing's sarcoma 11.1
19 ovarian cystic teratoma 11.1 KIT POU5F1 PTPRC
20 liposarcoma of bone 11.1 ENO2 EWSR1 WT1
21 pleomorphic xanthoastrocytoma 11.1 ENO2 EWSR1 WT1
22 brain stem infarction 11.1 ENO2 KIT PTPRC
23 intellectual disability-polydactyly-uncombable hair syndrome 11.1 ENO2 PTPRC
24 filamentary keratitis 11.1 KIT PTPRC
25 chondroid chordoma 11.1 ENO2 EWSR1 NCAM1
26 epithelioid trophoblastic tumor 11.1 KIT WT1
27 brown-vialetto-van laere syndrome 11.1 ENO2 EWSR1 KIT POU5F1
28 submandibular adenitis 11.1 ENO2 EWSR1 FUS
29 gingival hypertrophy 11.1 CD99 WT1
30 central nervous system disease 11.1 CD99 ENO2 KIT POU5F1
31 arteriosclerosis obliterans 11.0 ENO2 NCAM1
32 uveal melanoma 11.0 CD99 WT1
33 parkinsonism with dementia of guadeloupe 11.0 ATF1 EWSR1
34 lung leiomyosarcoma 11.0 ENO2 KIT WT1
35 ethmoid sinus schneiderian papilloma 11.0 ENO2 NCAM1 PTPRC
36 glomus tumor 11.0 ENO2 KIT NCAM1
37 thymus lipoma 11.0 NCAM1 WT1
38 mediastinum rhabdomyosarcoma 11.0 ENO2 EWSR1 KIT WT1
39 nasal cavity squamous cell carcinoma 11.0 ENO2 KIT WT1
40 verruciform xanthoma of skin 11.0 ENO2 NCAM1
41 vaginal yolk sac tumor 11.0 CD99 KIT NCAM1
42 pericoronitis 11.0 KIT PTPRC
43 encephalitozoonosis 11.0 ENO2 KIT NCAM1
44 pancreatic agenesis 11.0 ENO2 KIT NCAM1
45 urethral villous adenoma 11.0 ENO2 KIT NCAM1
46 cholesteatoma 11.0 KIT NCAM1 PTPRC
47 hydrarthrosis 11.0 CD99 KIT NCAM1
48 hypohidrosis 11.0 ERG EWSR1 FLI1 FUS KIT
49 heel spur 11.0 EWSR1 PTPRC
50 lung benign neoplasm 11.0 CD99 ENO2 EWSR1 PTPRC WT1

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



Diseases related to Ewing Sarcoma

Symptoms & Phenotypes for Ewing Sarcoma

Clinical features from OMIM:

612219

Human phenotypes related to Ewing Sarcoma:

32
id Description HPO Frequency HPO Source Accession
1 ewing's sarcoma 32 HP:0012254

UMLS symptoms related to Ewing Sarcoma:


fever

GenomeRNAi Phenotypes related to Ewing Sarcoma according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-104 9.96 IMPG2
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-109 9.96 MYCN PTPRC
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-119 9.96 PTPRC
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-153 9.96 IMPG2
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-165 9.96 IMPG2 KIT MYCN PTPRC
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-173 9.96 MYCN
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-18 9.96 KIT
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-213 9.96 PTPRC
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-26 9.96 IMPG2
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-33 9.96 IMPG2
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-34 9.96 IMPG2
12 Decreased shRNA abundance (Z-score < -2) GR00366-A-55 9.96 IMPG2
13 Decreased shRNA abundance (Z-score < -2) GR00366-A-61 9.96 KIT
14 Decreased shRNA abundance (Z-score < -2) GR00366-A-68 9.96 PTPRC
15 Decreased shRNA abundance (Z-score < -2) GR00366-A-87 9.96 IMPG2 PTPRC
16 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.87 WT1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-102 9.87 KIT
18 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.87 EWSR1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.87 WT1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.87 EWSR1
21 Increased shRNA abundance (Z-score > 2) GR00366-A-137 9.87 EWSR1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-140 9.87 EWSR1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.87 EWSR1
24 Increased shRNA abundance (Z-score > 2) GR00366-A-151 9.87 IMPG2
25 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.87 IMPG2 KIT WT1 EWSR1
26 Increased shRNA abundance (Z-score > 2) GR00366-A-157 9.87 KIT
27 Increased shRNA abundance (Z-score > 2) GR00366-A-164 9.87 EWSR1
28 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.87 IMPG2
29 Increased shRNA abundance (Z-score > 2) GR00366-A-170 9.87 KIT
30 Increased shRNA abundance (Z-score > 2) GR00366-A-178 9.87 IMPG2
31 Increased shRNA abundance (Z-score > 2) GR00366-A-19 9.87 EWSR1
32 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.87 WT1
33 Increased shRNA abundance (Z-score > 2) GR00366-A-23 9.87 WT1
34 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.87 KIT WT1
35 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.87 WT1
36 Increased shRNA abundance (Z-score > 2) GR00366-A-42 9.87 EWSR1 KIT
37 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.87 WT1
38 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.87 WT1
39 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.87 EWSR1
40 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.87 EWSR1
41 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.87 KIT WT1
42 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.87 KIT
43 Increased shRNA abundance (Z-score > 2) GR00366-A-99 9.87 KIT EWSR1

MGI Mouse Phenotypes related to Ewing Sarcoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.18 ENO2 ERG ETV1 FLI1 IGF1R KIT
2 embryo MP:0005380 9.97 ERG ETV4 FLI1 IGF1R KIT MYCN
3 liver/biliary system MP:0005370 9.87 FLI1 IGF1R KIT MYCN POU5F1 PTPRC
4 muscle MP:0005369 9.8 ETV1 ETV4 FLI1 IGF1R KIT MYCN
5 nervous system MP:0003631 9.73 ERG ETV1 ETV4 FLI1 IGF1R KIT
6 neoplasm MP:0002006 9.7 WT1 ERG ETV4 FLI1 IGF1R KIT
7 respiratory system MP:0005388 9.17 ENO2 ERG IGF1R KIT MYCN PTPRC

Drugs & Therapeutics for Ewing Sarcoma

FDA approved drugs:

(show all 6)
id Drug Name Active Ingredient(s) 17 Company Approval Date
1
Afinitor 17 46 EVEROLIMUS Novartis March 2009
2
Erbitux 17 46 CETUXIMAB Imclone, Bristol-Myers Squibb February 2004
3
Evista 17 46 RALOXIFENE HYDROCHLORIDE Eli Lilly September 2007
4
Istodax 17 46 ROMIDEPSIN Gloucester Pharmaceuticals November 2009
5
Sutent 17 46 SUNITINIB MALATE Pfizer May 2011/ January 2006
6
Treanda 17 46 BENDAMUSTINE HYDROCHLORIDE Cephalon October 2008

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

(show top 50) (show all 253)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1,Early Phase 1 50-18-0, 6055-19-2 2907
2
Dactinomycin Approved Phase 3,Phase 2 50-76-0 2019 457193
3
Doxorubicin Approved, Investigational Phase 3,Phase 2,Phase 1 23214-92-8 31703
4
Etoposide Approved Phase 3,Phase 2,Phase 1 33419-42-0 36462
5
Ifosfamide Approved Phase 3,Phase 2,Phase 1 3778-73-2 3690
6
Mesna Approved Phase 3,Phase 2,Phase 1 3375-50-6 598
7
Vincristine Approved, Investigational Phase 3,Phase 2,Phase 1 2068-78-2, 57-22-7 5978
8
Busulfan Approved, Investigational Phase 3,Phase 2,Phase 1 55-98-1 2478
9
Mechlorethamine Approved Phase 3,Phase 2,Phase 1 51-75-2 4033
10
Melphalan Approved Phase 3,Phase 2,Phase 1 148-82-3 4053 460612
11
Lenograstim Approved Phase 3,Phase 2,Phase 1 135968-09-1
12
Carboplatin Approved Phase 3,Phase 1,Phase 2 41575-94-4 10339178 498142 38904
13
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
14
Epirubicin Approved Phase 3,Phase 2 56420-45-2 41867
15
Topotecan Approved, Investigational Phase 3,Phase 2,Phase 1 119413-54-6, 123948-87-8 60700
16
Zoledronic acid Approved Phase 3,Phase 1 118072-93-8 68740
17
Cyproheptadine Approved Phase 3 129-03-3 2913
18
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
19
Doxil Approved June 1999 Phase 3,Phase 2,Phase 1 31703
20 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
21
Treosulfan Investigational Phase 3 299-75-2 9296
22 Alkylating Agents Phase 3,Phase 2,Phase 1,Early Phase 1
23 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1,Early Phase 1
24 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
25 Anti-Infective Agents Phase 3,Phase 2,Phase 1,Early Phase 1
26 Antimitotic Agents Phase 3,Phase 2,Phase 1
27 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Phase 1
28 Antirheumatic Agents Phase 3,Phase 2,Phase 1,Early Phase 1
29
Cobalt Phase 3,Phase 2 7440-48-4 104729
30 Etoposide phosphate Phase 3,Phase 2,Phase 1
31 Immunosuppressive Agents Phase 3,Phase 2,Phase 1,Early Phase 1
32 Isophosphamide mustard Phase 3,Phase 2,Phase 1
33 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2,Phase 1
34 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1
35 Adjuvants, Immunologic Phase 3,Phase 2,Phase 1
36 Liver Extracts Phase 3,Phase 2,Phase 1
37 Peripheral Nervous System Agents Phase 3,Phase 2,Phase 1
38 Antineoplastic Agents, Hormonal Phase 2, Phase 3,Phase 1
39 topoisomerase I inhibitors Phase 3,Phase 1,Phase 2
40 Gastrointestinal Agents Phase 3,Phase 2,Phase 1
41 Protective Agents Phase 3,Phase 2,Phase 1
42 razoxane Phase 3,Phase 2
43 Adjuvants, Anesthesia Phase 3
44 Analgesics Phase 3,Phase 2,Phase 1
45 Analgesics, Opioid Phase 3
46 Anesthetics Phase 3
47 Anesthetics, General Phase 3
48 Anesthetics, Intravenous Phase 3
49 Central Nervous System Depressants Phase 3,Phase 1
50 Narcotics Phase 3

Interventional clinical trials:

(show top 50) (show all 246)

id Name Status NCT ID Phase Drugs
1 Combination Chemotherapy Plus Surgery and Radiation Therapy in Treating Patients With Ewing's Sarcoma Unknown status NCT00002516 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;mesna;vincristine sulfate
2 Combination Chemotherapy With or Without Peripheral Stem Cell Transplantation, Radiation Therapy, and/or Surgery in Treating Patients With Ewing's Sarcoma Unknown status NCT00020566 Phase 3 busulfan;doxorubicin hydrochloride;etoposide;ifosfamide;melphalan;vincristine sulfate
3 Comparison of Combination Chemotherapy Regimens in Treating Patients With Ewing's Sarcoma or Neuroectodermal Tumor Completed NCT00006734 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
4 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
5 Music Therapy or Book Discussion in Improving Quality of Life in Young Patients Undergoing Stem Cell Transplant Completed NCT00305851 Phase 3
6 Surgery Followed by Chemotherapy in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
7 Efficacy of Dose Intensification in Patients With Non-metastatic Ewing Sarcoma Recruiting NCT02063022 Phase 3 Standard treatment (as per protocol ISG SSG III);Intensified chemotherapy
8 Study in Localized and Disseminated Ewing Sarcoma Recruiting NCT00987636 Phase 3 Zoledronic acid;Busulfan;Treosulfan
9 Combination Chemotherapy With or Without Ganitumab in Treating Patients With Newly Diagnosed Metastatic Ewing Sarcoma Recruiting NCT02306161 Phase 3 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Ifosfamide;Vincristine Sulfate
10 Co-treatment With GnRH Analogs on the Ovarian Reserve in Young Women Treated With Alkylating Agents for Cancer Recruiting NCT02856048 Phase 2, Phase 3 Triptorelin (GnRHa) + Chemotherapy
11 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
12 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
13 Post-operative Radiotherapy in Poor Responders Ewing's Sarcoma Patients Withdrawn NCT01734863 Phase 3
14 Combination Chemotherapy With or Without Topotecan in Treating Patients With Newly Diagnosed Localized Ewing's Sarcoma Withdrawn NCT00334867 Phase 3 cyclophosphamide;dexrazoxane hydrochloride;doxorubicin hydrochloride;etoposide;ifosfamide;topotecan hydrochloride;vincristine sulfate
15 Study of Intensive Chemotherapy, Surgery and Radiotherapy to Treat Ewing's Sarcoma in Children and Young Adults Unknown status NCT01696669 Phase 2 Chemotherapy
16 Hematopoietic Stem Cell Transplantation From Human Leukocyte Antigen (HLA) Compatible Donor in Ewing Sarcomas and Soft Tissues Sarcomas Unknown status NCT00998361 Phase 2
17 Study of Vinorelbine and Cyclofosfamide Among Patients With Refractory Tumours or in Relapse Unknown status NCT00180947 Phase 2 Vinorelbine, cyclofosfamide
18 Tandem Peripheral Blood Stem Cell (PBSC) Rescue for High Risk Solid Tumors Unknown status NCT00179816 Phase 1, Phase 2 High-Dose Chemotherapy with Tandem PBSC Rescue.
19 Umbilical Cord Blood for Stem Cell Transplantation in Treating Young Patients With Malignant or Nonmalignant Diseases Unknown status NCT00084695 Phase 2 busulfan;cyclophosphamide;fludarabine phosphate;melphalan;methylprednisolone
20 Olaparib in Adults With Recurrent/Metastatic Ewing's Sarcoma Completed NCT01583543 Phase 2 Olaparib
21 Study Of CP-751,871 In Patients With Ewing's Sarcoma Family Of Tumors Completed NCT00560235 Phase 1, Phase 2 CP-751,871
22 Vinblastine, Celecoxib, and Combination Chemotherapy in Treating Patients With Newly-Diagnosed Metastatic Ewing's Sarcoma Family of Tumors Completed NCT00061893 Phase 2 celecoxib;cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;vinblastine sulfate;vincristine sulfate;MESNA;Filgrastim
23 Aerosol L9-NC and Temozolomide in Ewing's Sarcoma Completed NCT00492141 Phase 1, Phase 2 Temozolomide;L9-NC
24 Eurosarc Trial of Linsitinib in Advanced Ewing Sarcoma Completed NCT02546544 Phase 2 Linsitinib
25 Irinotecan in Treating Patients With Newly Diagnosed Ewing's Sarcoma Completed NCT00276692 Phase 2 irinotecan hydrochloride
26 Cytarabine in Treating Young Patients With Recurrent or Refractory Ewing's Sarcoma Completed NCT00470275 Phase 2 cytarabine
27 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
28 New Therapeutic Strategies for Patients With Ewing's Sarcoma Family of Tumors, High Risk Rhabdomyosarcoma, and Neuroblastoma Completed NCT00001335 Phase 2 ADR-529;Topotecan;G-CSF
29 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
30 Gemcitabine and Docetaxel in Treating Patients With Recurrent Osteosarcoma (Closed to Accrual as of 12/21/06) or Ewing's Sarcoma or Unresectable or Locally Recurrent Chondrosarcoma Completed NCT00073983 Phase 2 docetaxel;gemcitabine hydrochloride
31 Combination Chemotherapy in Treating Patients With Newly Diagnosed Metastatic Ewing's Sarcoma or Primitive Neuroectodermal Tumor Completed NCT00002643 Phase 2 amifostine trihydrate;cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;topotecan hydrochloride;vincristine sulfate
32 Holmium Ho 166 DOTMP Followed by Peripheral Stem Cell Transplantation in Treating Patients With Metastatic Ewing's Sarcoma or Rhabdomyosarcoma That Has Spread to the Bone Completed NCT00006234 Phase 1, Phase 2
33 Exatecan Mesylate in Treating Patients With Ewing's Sarcoma, Primitive Neuroectodermal Tumor, or Desmoplastic Small Round Cell Tumor Completed NCT00055952 Phase 2 exatecan mesylate
34 Combination Chemotherapy and Radiation Therapy in Treating Patients With Peripheral Neuroectodermal Tumors, Ewing's Sarcoma, Wilms' Tumor, or Bone Cancer Completed NCT00002466 Phase 2 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
35 Busulfan, Melphalan, and Thiotepa Followed By a Donor Stem Cell Transplant in Treating Patients With High-Risk Ewing's Tumors Completed NCT00357396 Phase 2 busulfan;melphalan;thiotepa
36 Trabectedin in Treating Young Patients With Recurrent or Refractory Soft Tissue Sarcoma or Ewing's Family of Tumors Completed NCT00070109 Phase 2 trabectedin
37 Imatinib Mesylate in Treating Patients With Recurrent Ewing's Family of Tumors or Desmoplastic Small Round-Cell Tumor Completed NCT00062205 Phase 2 imatinib mesylate
38 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
39 A Five-Tier, Open-Label Study of IMC-A12 in Advanced Sarcoma Completed NCT00668148 Phase 2
40 A Study of R1507 in Participants With Recurrent or Refractory Sarcoma Completed NCT00642941 Phase 2 RG1507
41 A Pilot Study of Tumor-Specific Peptide Vaccination and IL-2 With or Without Autologous T Cell Transplantation in Recurrent Pediatric Sarcomas Completed NCT00001564 Phase 2 EF-1 Peptide;EF-2 Peptide;PXFK Peptide;E7 Peptide;IL-2;IL-4;GM-CSF;CD40 Ligand
42 A Pilot Study of Autologous T-Cell Transplantation With Vaccine Driven Expansion of Anti-Tumor Effectors After Cytoreductive Therapy in Metastatic Pediatric Sarcomas Completed NCT00001566 Phase 2 indinavir sulfate
43 A Study of Pemetrexed in Children With Recurrent Cancer Completed NCT00520936 Phase 2 pemetrexed
44 Intensive Chemo-Radiotherapy With Peripheral Blood Progenitor Cell Rescue for Children With Advanced Neuroblastoma and Sarcomas Completed NCT00165139 Phase 2 Vincristine;Cyclophosphamide;Adriamycin;Etoposide (VP-16);Cisplatin;Carboplatin;Melphalan;Ifosfamide;G-CSF (granulocyte-colony stimulating factor);Mesna
45 A Combined Study in Pediatric Cancer Patients for Dose Ranging and Efficacy/Safety of Plerixafor Plus Standard Regimens for Mobilization Versus Standard Regimens Alone Completed NCT01288573 Phase 1, Phase 2 plerixafor;plerixafor;plerixafor
46 Efficacy and Safety Assessment of Oral LBH589 in Adult Patients With Advanced Soft TIssue Sarcoma After Pre-treatment Failure Completed NCT01136499 Phase 2 LBH589 (Panobinostat®)
47 Rebeccamycin Analogue in Treating Children With Solid Tumors or Non-Hodgkin's Lymphoma Completed NCT00006102 Phase 2 becatecarin
48 Cixutumumab in Treating Patients With Relapsed or Refractory Solid Tumors Completed NCT00831844 Phase 2
49 Stem Cell Transplantation in Patients With High-Risk and Recurrent Pediatric Sarcomas Completed NCT00043979 Phase 2 F-18 Fluorodeoxyglucose;cyclophosphamide;cyclosporine;doxorubicin hydrochloride;etoposide;fludarabine phosphate;melphalan;prednisone;sirolimus;tacrolimus;vincristine sulfate;Filgrastim
50 QUILT-3.025: A Phase 2 Study of AMG 479 in Relapsed or Refractory Ewing's Family Tumor and Desmoplastic Small Round Cell Tumors Completed NCT00563680 Phase 2 AMG 479

Search NIH Clinical Center for Ewing Sarcoma

Inferred drug relations via UMLS 69 / NDF-RT 48 :


Cochrane evidence based reviews: sarcoma, ewing

Genetic Tests for Ewing Sarcoma

Genetic tests related to Ewing Sarcoma:

id Genetic test Affiliating Genes
1 Ewing's Sarcoma 29

Anatomical Context for Ewing Sarcoma

MalaCards organs/tissues related to Ewing Sarcoma:

39
Bone, Lung, Endothelial, Kidney, Breast, T Cells, Brain

Publications for Ewing Sarcoma

Articles related to Ewing Sarcoma:

(show top 50) (show all 439)
id Title Authors Year
1
Quantitative diffusion-weighted magnetic resonance imaging assessment of chemotherapy treatment response of pediatric osteosarcoma and Ewing sarcoma malignant bone tumors. ( 28806574 )
2017
2
Microscopic Infiltration of Cryopreserved Ovarian Tissue in 2 Patients With Ewing Sarcoma. ( 28816797 )
2017
3
Extra-skeletal Ewing sarcoma of the lumbosacral region in an adult pregnant patient: a case report. ( 28435928 )
2017
4
Inadvertently boarding a pirate ship: disease progression in a paediatric patient with relapsed metastatic Ewing sarcoma receiving treatment at a centre for alternative therapy in Mexico. ( 28062429 )
2017
5
Ewing sarcoma of the sternal manubrium: first report of a case in Australasia and review of the literature. ( 28419662 )
2017
6
Bone Marrow Involvement in Metastatic Pediatric Ewing Sarcoma. ( 28903844 )
2017
7
Development of Secondary Acute Myeloid Leukemia in a Pediatric Patient Concurrently Receiving Primary Therapy for Ewing Sarcoma. ( 28816792 )
2017
8
Intracardiac Low-grade Sarcoma Following Treatment for Ewing Sarcoma. ( 28060130 )
2017
9
Ewing Sarcoma of the Bone With EWS/FLI1 Translocation After Successful Treatment of Primary Osteosarcoma. ( 27918348 )
2017
10
Quality of Survivorship in a Rare Disease: Clinicofunctional Outcome and Physical Activity in an Observational Cohort Study of 618 Long-Term Survivors of Ewing Sarcoma. ( 28355116 )
2017
11
Advantages in Prognosis of Adult Patients with Ewing Sarcoma: 11-years Experiences and Current Treatment Management. ( 28803261 )
2017
12
Primary Ewing Sarcoma of Sphenoid Bone with Intracranial Extension: A Common Tumour at an Uncommon Location. ( 28384876 )
2017
13
Pulmonary Nodule Size <5 mm Still Warrants Investigation in Patients With Osteosarcoma and Ewing Sarcoma. ( 28060128 )
2017
14
DNA methylation profiling identifies PTRF/Cavin-1 as a novel tumor suppressor in Ewing sarcoma when co-expressed with caveolin-1. ( 27894957 )
2017
15
Prognostic value of FDG-PET indices for the assessment of histological response to neoadjuvant chemotherapy and outcome in pediatric patients with Ewing sarcoma and osteosarcoma. ( 28841702 )
2017
16
Modeling Stroma-Induced Drug Resistance in a Tissue-Engineered Tumor Model of Ewing Sarcoma. ( 27923328 )
2017
17
EWS-FLI1 Retargets BAF Chromatin Remodeling Complexes in Ewing Sarcoma. ( 28864478 )
2017
18
Primary Bone Anaplastic Large Cell Lymphoma Masquerading as Ewing Sarcoma: Diagnosis by Anchored Multiplex PCR. ( 28452858 )
2017
19
Entering the Double Digits-Ewing Sarcoma In Adult Maxilla. ( 28472564 )
2017
20
High-throughput RNAi screen in Ewing sarcoma cells identifies leucine rich repeats and WD repeat domain containing 1 (LRWD1) as a regulator of EWS-FLI1 driven cell viability. ( 27760381 )
2017
21
Transcriptomic and proteomic insight into the effects of a defined European mistletoe extract in Ewing sarcoma cells reveals cellular stress responses. ( 28454538 )
2017
22
Post-Chemotherapy Neuroblastoma-like Differentiation in Ewing Sarcoma of Humerus: Report of a Rare Case with Review of Literature. ( 28818170 )
2017
23
CIC-DUX4 Induces Small Round Cell Sarcomas Distinct from Ewing Sarcoma. ( 28404587 )
2017
24
EWS-FLI1 positively regulates autophagy by increasing ATG4B expression in Ewing sarcoma cells. ( 28902354 )
2017
25
Results for patients with sarcoma not otherwise specified and other diagnoses than Ewing sarcoma treated according to the Euro-EWING 99 trial. ( 28436593 )
2017
26
Adoptive natural killer cell therapy is effective in reducing pulmonary metastasis of Ewing sarcoma. ( 28507811 )
2017
27
DNA methylation profiling opens a new phase in the search of targeted therapy against Ewing sarcoma. ( 28832268 )
2017
28
Eribulin alone or in combination with the PLK1 inhibitor BI 6727 triggers intrinsic apoptosis in Ewing sarcoma cell lines. ( 28477025 )
2017
29
Primary Ewing Sarcoma / Primitive Neuroectodermal Tumor of the Kidney: A Clinicopathologic Study of 23 Cases. ( 28429277 )
2017
30
Eribulin alone or in combination with the PLK1 inhibitor BI 6727 triggers intrinsic apoptosis in Ewing sarcoma cell lines. ( 28881742 )
2017
31
Rare presentation of Ewing sarcoma metastasis to the sella and suprasellar cistern. ( 27816880 )
2017
32
Reactivation of TWIST1 contributes to Ewing sarcoma metastasis. ( 28873262 )
2017
33
Immunohistochemical analysis of NKX2.2, ETV4, and BCOR in a large series of genetically confirmed Ewing sarcoma family of tumors. ( 28864350 )
2017
34
DNA methylation heterogeneity defines a disease spectrum in Ewing sarcoma. ( 28134926 )
2017
35
Outcome of resectable pediatric Ewing sarcoma of the ribs. ( 28462848 )
2017
36
Second malignancies in Ewing sarcoma survivors. ( 28837218 )
2017
37
Bone adaptation of a biologically reconstructed femur after Ewing sarcoma: Long-term morphological and densitometric evolution. ( 28447127 )
2017
38
Biomarkers of Osteosarcoma, Chondrosarcoma, and Ewing Sarcoma. ( 28439237 )
2017
39
Reply to Second malignancies in Ewing sarcoma survivors. ( 28837220 )
2017
40
Combinatorial Drug Screening Identifies Ewing Sarcoma-specific Sensitivities. ( 28062706 )
2017
41
Ewing Sarcoma and Atypical Teratoid Rhabdoid Tumor. ( 28382842 )
2017
42
Rare Ileal Ewing Sarcoma/Primitive Neuroectodermal Tumor on 18F-FDG PET/CT. ( 28806254 )
2017
43
Morbidity and mortality after treatment of Ewing sarcoma: A single-institution experience. ( 28417551 )
2017
44
Role for the EWS domain of EWS/FLI in binding GGAA-microsatellites required for Ewing sarcoma anchorage independent growth. ( 28847958 )
2017
45
Primary Ewing sarcoma of the kidney: a case report and treatment review. ( 28509141 )
2017
46
(18)F-FDG PET/CT as an Indicator of Survival in Ewing Sarcoma of Bone. ( 28928879 )
2017
47
A Comparison of Pediatric vs. Adult Patients with the Ewing Sarcoma Family of Tumors. ( 28534008 )
2017
48
Matrix screen identifies synergistic combination of PARP inhibitors and nicotinamide phosphoribosyltransferase (NAMPT) inhibitors in Ewing sarcoma. ( 28899971 )
2017
49
Prognostic Value of SUVmax Measured by Pretreatment Fluorine-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in Patients with Ewing Sarcoma. ( 27100297 )
2016
50
First Case of Primary Sellar/Suprasellar-Intraventricular Ewing Sarcoma: Case Report and Review of the Literature. ( 28017744 )
2016

Variations for Ewing Sarcoma

ClinVar genetic disease variations for Ewing Sarcoma:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 RDH12 NM_152443.2(RDH12): c.806_810delCCCTG (p.Ala269Glyfs) deletion Pathogenic rs386834261 GRCh37 Chromosome 14, 68196055: 68196059
2 ABCC6 NM_001171.5(ABCC6): c.3389C> T (p.Thr1130Met) single nucleotide variant Pathogenic/Likely pathogenic rs63750459 GRCh37 Chromosome 16, 16256967: 16256967
3 VPS13B NM_017890.4(VPS13B): c.2591C> A (p.Ser864Ter) single nucleotide variant Pathogenic/Likely pathogenic rs140936527 GRCh37 Chromosome 8, 100286501: 100286501
4 CNGA3 NM_001298.2(CNGA3): c.1688G> A (p.Arg563His) single nucleotide variant Pathogenic/Likely pathogenic rs552069173 GRCh37 Chromosome 2, 99013321: 99013321
5 CNGB3 NM_019098.4(CNGB3): c.1285delT (p.Ser429Leufs) deletion Pathogenic/Likely pathogenic GRCh37 Chromosome 8, 87645015: 87645015
6 ABCC6 NM_001171.5(ABCC6): c.4104delC (p.Asp1368Glufs) deletion Pathogenic rs72664237 GRCh38 Chromosome 16, 16154732: 16154732
7 IMPG2 NM_016247.3(IMPG2): c.1680T> A (p.Tyr560Ter) single nucleotide variant Pathogenic rs758291149 GRCh37 Chromosome 3, 100963495: 100963495

Cosmic variations for Ewing Sarcoma:

9
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM520 KRAS soft tissue,lung,sarcoma,NS c.35G>T p.G12V 32

Copy number variations for Ewing Sarcoma from CNVD:

7 (show top 50) (show all 157)
id CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 13324 1 1 124300000 Deletion Ewing''s sarcoma
2 13741 1 1 27800000 Deletion Ewing''s sarcoma
3 16432 1 124300000 247249719 Gain Ewing''s sarcoma
4 16433 1 124300000 247249719 Gain Ewing''s sarcoma
5 16640 1 1286916 1536363 Loss Ewing''s sarcoma
6 17338 1 142400000 247249719 Gain Ewing''s sarcoma
7 17361 1 142565191 158296814 Gain AG1 Ewing''s sarcoma
8 20077 1 15300000 154800000 Gain Ewing''s sarcoma
9 20877 1 154978607 154979115 Gain HDGF Ewing''s sarcoma
10 21389 1 158408551 189951958 Gain FREB Ewing''s sarcoma
11 27410 1 208096981 208097464 Gain C1orf107 Ewing''s sarcoma
12 27922 1 215848737 215849264 Copy number GPATCH2 Ewing''s sarcoma
13 28256 1 220611046 220683633 Loss FBXO28 Ewing''s sarcoma
14 28623 1 224110806 224111244 Gain TMEM63A Ewing''s sarcoma
15 29204 1 228895774 228896323 Gain COG2 Ewing''s sarcoma
16 29247 1 229426179 229426672 Copy number C1orf131 Ewing''s sarcoma
17 29294 1 2300000 27800000 Deletion Ewing''s sarcoma
18 29735 1 234815741 234816376 Gain HEATR1 Ewing''s sarcoma
19 31804 1 3118000 5001000 Deletion Ewing''s sarcoma
20 31805 1 3118000 5001000 Deletion Ewing''s sarcoma
21 34220 1 53642491 53911866 Loss DMRTB1 Ewing''s sarcoma
22 35775 1 67181498 104009233 Loss SLC35D1 Ewing''s sarcoma
23 37996 1 9778925 10706826 Deletion APITD1 Ewing''s sarcoma
24 37997 1 9778925 10706826 Deletion CLSTN1 Ewing''s sarcoma
25 37998 1 9778925 10706826 Deletion CORT Ewing''s sarcoma
26 37999 1 9778925 10706826 Deletion CTNNBIP1 Ewing''s sarcoma
27 38000 1 9778925 10706826 Deletion DFFA Ewing''s sarcoma
28 38001 1 9778925 10706826 Deletion LZIC Ewing''s sarcoma
29 38002 1 9778925 10706826 Deletion NMNAT1 Ewing''s sarcoma
30 38003 1 9778925 10706826 Deletion PEX14 Ewing''s sarcoma
31 38004 1 9778925 10706826 Deletion PGD Ewing''s sarcoma
32 38005 1 9778925 10706826 Deletion RBP7 Ewing''s sarcoma
33 38141 10 1 135374737 Deletion Ewing''s sarcoma
34 41153 10 138206 135293404 Gain Ewing''s sarcoma
35 48491 11 1 52900000 Gain Ewing''s sarcoma
36 55430 11 52900000 134452384 Gain Ewing''s sarcoma
37 59789 11 777331 821974 Loss BM88 Ewing''s sarcoma
38 60445 11 86456481 127953177 Loss Ewing''s sarcoma
39 61420 12 1 132349534 Gain Ewing''s sarcoma
40 61533 12 1 35400000 Gain Ewing''s sarcoma
41 62737 12 111075038 111075556 Gain TRAFD1 Ewing''s sarcoma
42 63302 12 116955326 116955682 Copy number WSB2 Ewing''s sarcoma
43 64487 12 12873623 12874134 Gain DDX47 Ewing''s sarcoma
44 65323 12 1570852 1571348 Copy number FBXL14 Ewing''s sarcoma
45 66228 12 2670999 2671234 Deletion CACNA1C Ewing''s sarcoma
46 67041 12 35400000 132349534 Gain Ewing''s sarcoma
47 67472 12 41121456 41121979 Gain PPHLN1 Ewing''s sarcoma
48 68463 12 49967 52715434 Gain IQSEC3 Ewing''s sarcoma
49 68787 12 51501640 51502010 Copy number KRT79 Ewing''s sarcoma
50 69068 12 52981304 57130106 Gain Ewing''s sarcoma

Expression for Ewing Sarcoma

Search GEO for disease gene expression data for Ewing Sarcoma.

Pathways for Ewing Sarcoma

GO Terms for Ewing Sarcoma

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

id Name GO ID Score Top Affiliating Genes
1 transcription, DNA-templated GO:0006351 9.91 ATF1 ERG ETV1 ETV4 EWSR1 FLI1
2 regulation of transcription, DNA-templated GO:0006355 9.85 ATF1 ERG ETV1 ETV4 EWSR1 FLI1
3 positive regulation of transcription from RNA polymerase II promoter GO:0045944 9.56 ATF1 ERG ETV1 ETV4 FLI1 MYCN
4 megakaryocyte development GO:0035855 9.32 FLI1 KIT
5 transcription from RNA polymerase II promoter GO:0006366 9.17 ATF1 ERG ETV1 ETV4 FLI1 POU5F1

Molecular functions related to Ewing Sarcoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 DNA binding GO:0003677 9.96 ATF1 ERG ETV1 ETV4 FLI1 FUS
2 transcription factor activity, sequence-specific DNA binding GO:0003700 9.76 ATF1 ERG ETV1 ETV4 FLI1 MYCN
3 RNA polymerase II transcription factor activity, sequence-specific DNA binding GO:0000981 9.67 ERG ETV4 FLI1 POU5F1
4 RNA polymerase II core promoter proximal region sequence-specific DNA binding GO:0000978 9.62 ERG ETV1 ETV4 FLI1
5 sequence-specific DNA binding GO:0043565 9.5 ATF1 ERG ETV1 ETV4 FLI1 POU5F1
6 transcriptional activator activity, RNA polymerase II core promoter proximal region sequence-specific binding GO:0001077 9.02 ERG ETV1 ETV4 FLI1 WT1
7 protein binding GO:0005515 10.33 ATF1 CD99 ENO2 ERG ETV1 ETV4

Sources for Ewing Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 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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