Disease · MONDO_0021038

Ewing sarcoma/peripheral primitive neuroectodermal tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. Ewing sarcoma/peripheral primitive neuroectodermal tumor. Diseases, record MONDO_0021038. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0021038

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: Ewing sarcoma/peripheral primitive neuroectodermal tumor; Open Targets
Open Targets ID: MONDO_0021038; Open Targets
Other names: EFTs, Ewing family of tumors, Ewing family of tumours, Ewing sarcoma family of tumors, Ewing sarcoma family of tumours, Ewing sarcoma/peripheral PNET, 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; Open Targets
NCI Thesaurus: C27291; NCI Thesaurus
Highest stage of its drugs: Phase 2, for 24 of its 27 drugs, from ClinicalTrials.gov; Open Targets

Drugs27

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	4	4	4	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 2	ClinicalTrials.gov	4	4	4	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Phase 2	ClinicalTrials.gov	4	4	4	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
IFOSFAMIDE	CHEMBL1024	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
IRINOTECAN	CHEMBL481	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
MELPHALAN	CHEMBL852	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
TOPOTECAN	CHEMBL84	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
ALDESLEUKIN	CHEMBL1201438	Protein	Phase 2	ClinicalTrials.gov	1	1	0	Drugs and targets
BEVACIZUMAB	CHEMBL1201583	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 27

See all 27 drugs

Targets37

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
TOP1	DNA topoisomerase 1	3	DNA topoisomerase I inhibitor	Phase 2	https://conductscience.org/library/genes/7150	Drugs and targets
CSF3R	Granulocyte colony-stimulating factor receptor	2	Granulocyte colony stimulating factor receptor agonist	Phase 2	https://conductscience.org/library/genes/1441	Drugs and targets
KIT	Mast/stem cell growth factor receptor Kit	2	Stem cell growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3815	Drugs and targets
PDGFRB	Platelet-derived growth factor receptor beta	2	Platelet-derived growth factor receptor beta inhibitor	Phase 2	https://conductscience.org/library/genes/5159	Drugs and targets
TOP2A	DNA topoisomerase 2-alpha	2	DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitor	Phase 2	https://conductscience.org/library/genes/7153	Drugs and targets
TUBA1A	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7846	Drugs and targets
TUBA1B	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/10376	Drugs and targets
TUBA1C	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/84790	Drugs and targets
TUBA3C	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7278	Drugs and targets
TUBA3E	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/112714	Drugs and targets

1 to 10 of 37

See all 37 targets

Trials17

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT02454972	Clinical Trial of Lurbinectedin (PM01183) in Selected Advanced Solid Tumors	Phase 2	345	2021-11-22	Advanced Solid Tumors	LURBINECTEDIN	Its record names the disease	ClinicalTrials.gov
NCT00001566	A Pilot Study of Autologous T-Cell Transplantation With Vaccine Driven Expansion of Anti-Tumor Effectors After Cytoreductive Therapy in Metastatic Pediatric Sarcomas	Phase 2	42	2012-06-12	Ewing's Sarcoma, Rhabdomyosarcoma	INDINAVIR SULFATE	Open Targets' reading of its record	ClinicalTrials.gov
NCT00618813	Two Regimens of Combination Chemotherapy in Treating Younger Patients With Newly Diagnosed Localized Ewing Sarcoma Family of Tumors	Not applicable	35	2014-02-10	Ewing Sarcoma of Bone, Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor	CYCLOPHOSPHAMIDE, TOPOTECAN HYDROCHLORIDE, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT01946529	Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors	Phase 2	24	2016-10-19	Desmoplastic Small Round Cell Tumor, Ewing Sarcoma of Bone or Soft Tissue, Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor	BEVACIZUMAB, CYCLOPHOSPHAMIDE, DOXORUBICIN, ETOPOSIDE, IFOSFAMIDE, IRINOTECAN, SORAFENIB, TEMOZOLOMIDE, TEMSIROLIMUS, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT01222767	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	Phase 2	17	2021-10-29	Ewing's Sarcoma, Primitive Neuroectodermal Tumor (PNET), Askin's Tumor of the Chest Wall, Extraosseous Ewing's Sarcoma (EOE)	PM-00104	Its record names the disease	ClinicalTrials.gov
NCT00357396	Busulfan, Melphalan, and Thiotepa Followed By a Donor Stem Cell Transplant in Treating Patients With High-Risk Ewing's Tumors	Phase 2	10	2015-11-25	Sarcoma	BUSULFAN, MELPHALAN, THIOTEPA	Open Targets' reading of its record	ClinicalTrials.gov
NCT00003745	Chemotherapy in Treating Children With Recurrent or Refractory Sarcomas	Phase 2	125		Sarcoma	TOPOTECAN	Its record names the disease	ClinicalTrials.gov
NCT00001335	New Therapeutic Strategies for Patients With Ewing's Sarcoma Family of Tumors, High Risk Rhabdomyosarcoma, and Neuroblastoma	Phase 2	90		Ewing's Sarcoma, Neuroblastoma, Rhabdomyosarcoma	CYCLOPHOSPHAMIDE, DOXORUBICIN, ETOPOSIDE, IFOSFAMIDE, MELPHALAN, TOPOTECAN, VINCRISTINE	Open Targets' reading of its record	ClinicalTrials.gov
NCT00503295	Safety and Efficacy Study of REOLYSIN® in the Treatment of Bone and Soft Tissue Sarcomas Metastatic to the Lung	Phase 2	53		Osteosarcoma, Ewing Sarcoma Family Tumors, Malignant Fibrous Histiocytoma, Sarcoma, Synovial, Fibrosarcoma, Leiomyosarcoma	PELAREOREP	Open Targets' reading of its record	ClinicalTrials.gov
NCT00062205	Imatinib Mesylate in Treating Patients With Recurrent Ewing's Family of Tumors or Desmoplastic Small Round-Cell Tumor	Phase 2	40		Sarcoma	IMATINIB MESYLATE	Its record names the disease	ClinicalTrials.gov

1 to 10 of 17

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Comparisons in its trials1

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial
Patients With HIGH RISK EWING'S SARCOMA FAMILY TUMOR vs Related Donors: serious adverse events	4 of 6	0 of 4	10	NCT00357396

Source Trial comparisons

Papers from its trials11

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
18676758	RESULT	1	PubMed
16435380	RESULT	1	PubMed
37556023	DERIVED	1	PubMed
36037567	DERIVED	1	PubMed
35830841	DERIVED	1	PubMed
35486638	DERIVED	1	PubMed
34739582	DERIVED	1	PubMed
33096421	DERIVED	1	PubMed
32224306	DERIVED	1	PubMed
31401903	DERIVED	1	PubMed

1 to 10 of 11

See all 11 papers from its trials

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0021038

GET https://conductscience.org/api/diseases/v1/MONDO_0021038/drugs?page=2

API reference For AI assistants