Disease · MONDO_0017387

epithelioid sarcoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. epithelioid sarcoma. Diseases, record MONDO_0017387. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0017387

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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: epithelioid sarcoma; Open Targets
Open Targets ID: MONDO_0017387; Open Targets
Other names: ES, epithelioid cell sarcoma; Open Targets
Disease Ontology: DOID:6193; Disease Ontology
NCI Thesaurus: C3714; NCI Thesaurus
Orphanet: ORPHA:293202; Orphanet
Highest stage of its drugs: Phase 2, for 4 of its 10 drugs, from ClinicalTrials.gov; Open Targets

Drugs10

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
TAZEMETOSTAT	CHEMBL3414621	Small molecule	Phase 2	ClinicalTrials.gov	5	5	5	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
CEMIPLIMAB	CHEMBL4297723	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
UBAMATAMAB	CHEMBL4594595	Antibody	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
NIVOLUMAB	CHEMBL2108738	Antibody	Phase 1/2	ClinicalTrials.gov	2	2	2	Drugs and targets
IPILIMUMAB	CHEMBL1789844	Antibody	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets
SIROLIMUS	CHEMBL413	Small molecule	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets
CATEQUENTINIB	CHEMBL4303201	Small molecule	Unknown	ClinicalTrials.gov	1	1	1	Drugs and targets
IRINOTECAN	CHEMBL481	Small molecule	Unknown	ClinicalTrials.gov	1	1	1	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Unknown	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets25

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
PDCD1	Programmed cell death protein 1	2	Programmed cell death protein 1 inhibitor	Phase 2	https://conductscience.org/library/genes/5133	Drugs and targets
CTLA4	Cytotoxic T-lymphocyte protein 4	1	Cytotoxic T-lymphocyte protein 4 inhibitor	Phase 1/2	https://conductscience.org/library/genes/1493	Drugs and targets
EZH2	Histone-lysine N-methyltransferase EZH2	1	Histone-lysine N-methyltransferase EZH2 inhibitor	Phase 2	https://conductscience.org/library/genes/2146	Drugs and targets
FKBP1A	Peptidyl-prolyl cis-trans isomerase FKBP1A	1	FK506-binding protein 1A inhibitor	Phase 1/2	https://conductscience.org/library/genes/2280	Drugs and targets
FLT4	Vascular endothelial growth factor receptor 3	1	Vascular endothelial growth factor receptor 3 inhibitor	Unknown	https://conductscience.org/library/genes/2324	Drugs and targets
KDR	Vascular endothelial growth factor receptor 2	1	Vascular endothelial growth factor receptor 2 inhibitor	Unknown	https://conductscience.org/library/genes/3791	Drugs and targets
KIT	Mast/stem cell growth factor receptor Kit	1	Stem cell growth factor receptor inhibitor	Unknown	https://conductscience.org/library/genes/3815	Drugs and targets
PDGFRB	Platelet-derived growth factor receptor beta	1	Platelet-derived growth factor receptor beta inhibitor	Unknown	https://conductscience.org/library/genes/5159	Drugs and targets
TOP1	DNA topoisomerase 1	1	DNA topoisomerase I inhibitor	Unknown	https://conductscience.org/library/genes/7150	Drugs and targets
TOP2A	DNA topoisomerase 2-alpha	1	DNA topoisomerase II alpha inhibitor	Phase 2	https://conductscience.org/library/genes/7153	Drugs and targets

1 to 10 of 25

See all 25 targets

Trials8

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
NCT02601950	A Study of Tazemetostat in Adult Participants With Soft Tissue Sarcoma	Phase 2	267	2025-03-11	Malignant Rhabdoid Tumors (MRT), Rhabdoid Tumors of the Kidney (RTK), Atypical Teratoid Rhabdoid Tumors (ATRT), Selected Tumors With Rhabdoid Features, Synovial Sarcoma, INI1-negative Tumors, Malignant Rhabdoid Tumor of Ovary, Renal Medullary Carcinoma, Epithelioid Sarcoma, Poorly Differentiated Chordoma (or Other Chordoma With Sponsor Approval), Any Solid Tumor With an EZH2 GOF Mutation	DOXORUBICIN, TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT02601937	EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma	Phase 1	109	2024-10-03	Rhabdoid Tumors, INI1-negative Tumors, Synovial Sarcoma, Malignant Rhabdoid Tumor of Ovary	TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT03190174	Nivolumab (Opdivo®) Plus ABI-009 (Nab-rapamycin) for Advanced Sarcoma and Certain Cancers	Phase 1/2	34	2025-02-17	Ewing Sarcoma, PEComa, Epithelioid Sarcoma, Desmoid Tumor, Chordoma, Non Small Cell Lung Cancer, Urothelial Carcinoma, Melanoma, Renal Cell Carcinoma, Squamous Cell Carcinoma, Hepatocellular Carcinoma, Classical Hodgkin Lymphoma, Colorectal Cancer, MTOR Activating Mutation	NIVOLUMAB, SIROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT05407441	Tazemetostat+Nivo/Ipi in INI1-Neg/SMARCA4-Def Tumors	Phase 1/2	49		Atypical Teratoid Rhabdoid Tumor, INI1 (SMARCB1)-Deficient Primary CNS Malignant Tumors, SMARCA4-deficient Primary CNS Malignant Tumors, Malignant Rhabdoid Tumor (MRT), Rhabdoid Tumor of the Kidney (RTK), Epithelioid Sarcoma, Chordoma	IPILIMUMAB, NIVOLUMAB, TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT06444880	Phase II Trial of Ubamatamab Alone or in Combination With Cemiplimab in MUC16-Expressing SMARCB1-Deficient Malignancies	Phase 2	40		SMARCB1-Deficient Malignancies, Epithelioid Sarcoma, Renal Medullary Carcinoma	CEMIPLIMAB, UBAMATAMAB	Its record names the disease	ClinicalTrials.gov
NCT04204941	Tazemetostat in Combination With Doxorubicin as Frontline Therapy for Advanced Epithelioid Sarcoma	Phase 1	25		Advanced Soft-tissue Sarcoma, Advanced Epithelioid Sarcoma	DOXORUBICIN, TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov
NCT05656222	Irinotecan and Anlotinib for Epithelioid Sarcoma	Not applicable	0		Objective Response, Overall Survival	CATEQUENTINIB, IRINOTECAN, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT04225429	Tazemetostat Expanded Access Program for Adults With Epithelioid Sarcoma				Epithelioid Sarcoma	TAZEMETOSTAT	Its record names the disease	ClinicalTrials.gov

Comparisons in its trials0

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.

None in this release.

Papers from its trials6

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
24089446	RESULT	1	PubMed
40749151	DERIVED	1	PubMed
38501690	DERIVED	1	PubMed
38310286	DERIVED	1	PubMed
33035459	DERIVED	2	PubMed
31401903	DERIVED	1	PubMed

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_0017387

GET https://conductscience.org/api/diseases/v1/MONDO_0017387/targets?page=2

API reference For AI assistants