Ewing sarcoma/peripheral primitive neuroectodermal tumor
diseaseOn this page
Also known as EFTsEwing family of tumorsEwing family of tumoursEwing sarcoma family of tumorsEwing sarcoma family of tumoursEwing sarcoma/peripheral PNETEwing's family of tumorsEwing's family of tumoursEwing's sarcoma/peripheral primitive neuroectodermal tumorEwing's sarcoma/peripheral primitive neuroectodermal tumourtumors of Ewing's familytumors of the Ewing's familytumours of Ewing's familytumours of the Ewing's family
Summary
Ewing sarcoma/peripheral primitive neuroectodermal tumor (MONDO:0021038) is a cancer (an umbrella term covering 5 Mondo subtypes) and 41 clinical trials. Top therapeutic interventions include irinotecan, larotrectinib, and thiotepa. A subtype of embryonal neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Umbrella term: 5 Mondo subtypes
- Clinical trials: 41
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | Ewing sarcoma/peripheral primitive neuroectodermal tumor |
| Mondo ID | MONDO:0021038 |
| NCIT | C27291 |
| UMLS | C3536893 |
| MedGen | 760735 |
| Is cancer (heuristic) | yes |
Also known as: EFTs · Ewing family of tumors · Ewing family of tumours · Ewing sarcoma family of tumors · Ewing sarcoma family of tumours · Ewing sarcoma/peripheral PNET · Ewing sarcoma/peripheral primitive neuroectodermal tumor · Ewing’s family of tumors · Ewing’s family of tumours · Ewing’s sarcoma/peripheral primitive neuroectodermal tumor · Ewing’s sarcoma/peripheral primitive neuroectodermal tumour · tumors of Ewing’s family · tumors of the Ewing’s family · tumours of Ewing’s family · tumours of the Ewing’s family
Data availability: 8 cell lines.
Disease family
This is a subtype of embryonal neoplasm. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › embryonal neoplasm › Ewing sarcoma/peripheral primitive neuroectodermal tumor
Related subtypes (11): notochordal tumor, rhabdoid tumor, primitive neuroectodermal tumor, blastoma, Wilms tumor, testicular embryonal carcinoma, medulloblastoma, pineoblastoma, congenital mesoblastic nephroma, intraocular medulloepithelioma, hepatoblastoma
Subtypes (5): Ewing sarcoma, central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor, peripheral primitive neuroectodermal tumor, extraskeletal Ewing sarcoma/peripheral primitive neuroectodermal tumor, Ewing sarcoma/peripheral primitive neuroectodermal tumor of bone
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 41.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 18 |
| Not specified | 11 |
| PHASE1 | 7 |
| PHASE1/PHASE2 | 3 |
| PHASE3 | 1 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT03495921 | PHASE3 | TERMINATED | A Trial For Participants With Ewing’s Sarcoma Treated With Vigil in Combination With Irinotecan and Temozolomide |
| NCT01946529 | PHASE2 | ACTIVE_NOT_RECRUITING | Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors |
| NCT03210714 | PHASE2 | ACTIVE_NOT_RECRUITING | Erdafitinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With FGFR Mutations (A Pediatric MATCH Treatment Trial) |
| NCT03213652 | PHASE2 | ACTIVE_NOT_RECRUITING | Ensartinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With ALK or ROS1 Genomic Alterations (A Pediatric MATCH Treatment Trial) |
| NCT03213704 | PHASE2 | ACTIVE_NOT_RECRUITING | Larotrectinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions (A Pediatric MATCH Treatment Trial) |
| NCT00004078 | PHASE2 | COMPLETED | Irinotecan in Treating Children With Refractory Solid Tumors |
| NCT00030667 | PHASE2 | COMPLETED | Imatinib Mesylate in Treating Patients With Relapsed or Refractory Solid Tumors of Childhood |
| NCT00091182 | PHASE2 | COMPLETED | Oxaliplatin in Treating Young Patients With Recurrent Solid Tumors That Have Not Responded to Previous Treatment |
| NCT00112463 | PHASE2 | COMPLETED | Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma |
| NCT00330421 | PHASE2 | COMPLETED | Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07) |
| NCT00331643 | PHASE2 | COMPLETED | Ixabepilone in Treating Young Patients With Refractory Solid Tumors |
| NCT00516295 | PHASE2 | COMPLETED | Vincristine Sulfate, Topotecan Hydrochloride, and Cyclophosphamide With or Without Bevacizumab in Treating Young Patients With Refractory or First Recurrent Extracranial Ewing Sarcoma |
| NCT01154452 | PHASE1/PHASE2 | COMPLETED | Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma |
| NCT01154816 | PHASE2 | COMPLETED | Alisertib in Treating Young Patients With Recurrent or Refractory Solid Tumors or Leukemia |
| NCT01614795 | PHASE2 | COMPLETED | Cixutumumab and Temsirolimus in Treating Younger Patients With Recurrent or Refractory Sarcoma |
| NCT02011126 | PHASE2 | WITHDRAWN | Imetelstat Sodium in Treating Younger Patients With Relapsed or Refractory Solid Tumors |
| NCT02116777 | PHASE1/PHASE2 | COMPLETED | Talazoparib and Temozolomide in Treating Younger Patients With Refractory or Recurrent Malignancies |
| NCT02304458 | PHASE1/PHASE2 | COMPLETED | Nivolumab With or Without Ipilimumab in Treating Younger Patients With Recurrent or Refractory Solid Tumors or Sarcomas |
| NCT03011528 | PHASE2 | COMPLETED | First-line Treatment of Ewing Tumours With Primary Extrapulmonary Dissemination in Patients From 2 to 50 Years (CombinaiR3) |
| NCT03213665 | PHASE2 | COMPLETED | Tazemetostat in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With EZH2, SMARCB1, or SMARCA4 Gene Mutations (A Pediatric MATCH Treatment Trial) |
| NCT03233204 | PHASE2 | COMPLETED | Olaparib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Defects in DNA Damage Repair Genes (A Pediatric MATCH Treatment Trial) |
| NCT04530487 | PHASE2 | TERMINATED | Donor Stem Cell Transplant After Chemotherapy for the Treatment of Recurrent or Refractory High-Risk Solid Tumors in Pediatric and Adolescent-Young Adults |
| NCT00004241 | PHASE1 | COMPLETED | 17-N-Allylamino-17-Demethoxygeldanamycin in Treating Patients With Advanced Epithelial Cancer, Malignant Lymphoma, or Sarcoma |
| NCT00012181 | PHASE1 | COMPLETED | Flavopiridol in Treating Children With Relapsed or Refractory Solid Tumors or Lymphomas |
| NCT00093821 | PHASE1 | COMPLETED | Tanespimycin in Treating Young Patients With Recurrent or Refractory Leukemia or Solid Tumors |
| NCT00101270 | PHASE1 | COMPLETED | Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas |
| NCT00609141 | PHASE1 | COMPLETED | IMC-A12 in Treating Young Patients With Relapsed or Refractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor or Other Solid Tumor |
| NCT00638898 | PHASE1 | COMPLETED | Busulfan, Melphalan, Topotecan Hydrochloride, and a Stem Cell Transplant in Treating Patients With Newly Diagnosed or Relapsed Solid Tumor |
| NCT02736565 | PHASE1 | COMPLETED | Pbi-shRNA™ EWS/FLI1 Type 1 LPX in Subjects With Advanced Ewing’s Sarcoma |
| NCT01825902 | EARLY_PHASE1 | COMPLETED | 18F-FLT Positron Emission Tomography and Diffusion-Weighted Magnetic Resonance Imaging in Planning Surgery and Radiation Therapy and Measuring Response in Patients With Newly Diagnosed Ewing Sarcoma |
| NCT03496402 | Not specified | ACTIVE_NOT_RECRUITING | Biological Characterisation of High Risk CHildhood Cancer in Children, Adolescents and Young Adults (MICCHADO) |
| NCT06997211 | Not specified | NOT_YET_RECRUITING | Fatigue, Anxiety, and Mother-Baby Bonding and Emotional Freedom Technique |
| NCT00048984 | Not specified | COMPLETED | Diagnostic Study of Tumor Characteristics in Patients With Ewing’s Sarcoma |
| NCT00536601 | Not specified | COMPLETED | High-Dose Chemotherapy With or Without Total-Body Irradiation Followed by Autologous Stem Cell Transplant in Treating Patients With Hematologic Cancer or Solid Tumors |
| NCT00674193 | Not specified | COMPLETED | Evaluating Dactinomycin and Vincristine in Young Patients With Cancer |
| NCT00899990 | Not specified | COMPLETED | Collecting and Storing Biological Samples From Patients With Ewing Sarcoma |
| NCT01795430 | Not specified | WITHDRAWN | Whole-Body Radiation Therapy, Systemic Chemotherapy, and High-Dose Chemotherapy Followed By Stem Cell Rescue in Treating Patients With Poor-Risk Ewing Sarcoma |
| NCT01876303 | Not specified | COMPLETED | Genetic Biomarkers in Saliva Samples From Patients With Ewing Sarcoma |
| NCT02159443 | Not specified | COMPLETED | Pretreatment Anti-Therapeutic Antibodies (PATA) in Patients Treated With hu14.18K322A Antibody |
| NCT03542097 | Not specified | COMPLETED | Assessment of MGMT Promoter Methylation in Advanced Ewing Sarcoma Treated With Temozolomide and Irinotecan |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| IRINOTECAN | 4 | 6 |
| LAROTRECTINIB | 4 | 2 |
| THIOTEPA | 4 | 2 |
| ENSARTINIB | 4 | 1 |
| ERDAFITINIB | 4 | 1 |
| IMATINIB | 4 | 1 |
| IMETELSTAT SODIUM | 4 | 1 |
| IXABEPILONE | 4 | 1 |
| ROMIDEPSIN | 4 | 1 |
| SORAFENIB | 4 | 1 |
| TALAZOPARIB | 4 | 1 |
| TAZEMETOSTAT | 4 | 1 |
| TEMOZOLOMIDE | 4 | 1 |
| TOPOTECAN HYDROCHLORIDE | 4 | 1 |
| VISMODEGIB | 4 | 1 |
| ALVOCIDIB | 3 | 3 |
| TANESPIMYCIN | 3 | 2 |
| ALISERTIB | 3 | 1 |
| CIXUTUMUMAB | 2 | 2 |
| CHEMBL4786163 | 0 | 2 |
| CHEMBL4228794 | 0 | 1 |
| CHEMBL4248195 | 0 | 1 |
| CHEMBL4066465 | 0 | 1 |
| CHEMBL3764816 | 0 | 1 |
| CHEMBL5724798 | 0 | 1 |
| CHEMBL5398431 | 0 | 1 |