Disease · MONDO_0002142

undifferentiated pleomorphic sarcoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. undifferentiated pleomorphic sarcoma. Diseases, record MONDO_0002142. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0002142

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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: undifferentiated pleomorphic sarcoma; Open Targets
Open Targets ID: MONDO_0002142; Open Targets
Other names: MFH, Storiform-pleomorphic MFH, Storiform-pleomorphic fibrous histiocytoma, Storiform-pleomorphic malignant fibrous histiocytoma, UPS, Unclassified Pleomorphic sarcoma (formerly "MFH"), Unclassified Pleomorphic sarcoma (formerly "malignant fibrous histiocytoma"), adult malignant fibrous histiocytoma, adult unclassified pleomorphic sarcoma, adult undifferentiated pleomorphic sarcoma, fibrous histiocytoma, malignant, fibrous histiocytoma, malignant (morphologic abnormality), fibroxanthosarcoma, fibroxanthosarcoma (morphologic abnormality), histiocytoma, fibrous, malignant, malignant fibrous cytoma, malignant fibrous histiocytoma, malignant fibrous histiocytoma of soft tissue and bone, malignant fibrous histiocytoma of the soft tissue and bone, malignant fibroxanthoma, unclassified pleomorphic sarcoma, undifferentiated pleomorphic soft tissue sarcoma; Open Targets
Disease Ontology: DOID:1907; Disease Ontology
MeSH: D051677; MeSH
NCI Thesaurus: C114541; NCI Thesaurus
NCI Thesaurus: C4247; NCI Thesaurus
Orphanet: ORPHA:2023; Orphanet
Highest stage of its drugs: Phase 3, for 14 of its 41 drugs, from ClinicalTrials.gov; Open Targets

Drugs41

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
PEMBROLIZUMAB	CHEMBL3137343	Antibody	Phase 3	ClinicalTrials.gov	11	11	11	Drugs and targets
CISPLATIN	CHEMBL11359	Small molecule	Phase 3	ClinicalTrials.gov	3	3	2	Drugs and targets
GEMCITABINE	CHEMBL888	Small molecule	Phase 3	ClinicalTrials.gov	3	3	3	Drugs and targets
IFOSFAMIDE	CHEMBL1024	Small molecule	Phase 3	ClinicalTrials.gov	3	3	3	Drugs and targets
DACARBAZINE	CHEMBL476	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
EPIRUBICIN	CHEMBL417	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
ERIBULIN	CHEMBL1683590	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
TRABECTEDIN	CHEMBL450449	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
BRIGIMADLIN	CHEMBL5314455	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 41

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Targets64

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
KDR	Vascular endothelial growth factor receptor	6	Vascular endothelial growth factor receptor 2 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/3791	Drugs and targets
PDCD1	Programmed cell death protein 1	6	Programmed cell death protein 1 antagonist; Programmed cell death protein 1 inhibitor	Phase 3	https://conductscience.org/library/genes/5133	Drugs and targets
KIT	Mast/stem cell growth factor receptor Kit	5	Stem cell growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/3815	Drugs and targets
PDGFRB	Platelet-derived growth factor receptor	5	Platelet-derived growth factor receptor beta inhibitor; Platelet-derived growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/5159	Drugs and targets
FLT4	Vascular endothelial growth factor receptor	4	Vascular endothelial growth factor receptor 3 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/2324	Drugs and targets
CSF1R	Macrophage colony-stimulating factor 1 receptor	3	Macrophage colony stimulating factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/1436	Drugs and targets
FLT1	Vascular endothelial growth factor receptor	3	Vascular endothelial growth factor receptor 1 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/2321	Drugs and targets
PDGFRA	Platelet-derived growth factor receptor	3	Platelet-derived growth factor receptor alpha inhibitor; Platelet-derived growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/5156	Drugs and targets
TOP2A	DNA topoisomerase 2-alpha	3	DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitor	Phase 3	https://conductscience.org/library/genes/7153	Drugs and targets
TUBA1A	Tubulin	3	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7846	Drugs and targets

1 to 10 of 64

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Trials34

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
NCT02301039	SARC028: A Phase II Study of the Anti-PD1 Antibody Pembrolizumab (MK-3475) in Patients With Advanced Sarcomas	Phase 2	144	2020-09-29	Soft Tissue Sarcoma, Bone Sarcoma	PEMBROLIZUMAB	Its record names the disease	ClinicalTrials.gov
NCT02879162	Durvalumab and Tremelimumab in Patients With Advanced Rare Tumours	Phase 2	140	2025-02-17	Advanced Rare Tumours	DURVALUMAB, TREMELIMUMAB	Its record names the disease	ClinicalTrials.gov
NCT02815995	Multi-Arm Study to Test the Efficacy of Immunotherapeutic Agents in Multiple Sarcoma Subtypes	Phase 2	57	2025-06-12	Advanced and/or Metastatic Sarcoma	DURVALUMAB, TREMELIMUMAB	Its record names the disease	ClinicalTrials.gov
NCT03899805	A Phase II Study of Eribulin and Pembrolizumab in Soft Tissue Sarcomas	Phase 2	57	2025-08-28	Sarcoma, Liposarcoma, Leiomyosarcoma, Undifferentiated Pleomorphic Sarcoma	ERIBULIN, PEMBROLIZUMAB	Its record names the disease	ClinicalTrials.gov
NCT00400569	Phase II Study of Sunitinib Malate for Metastatic and/or Surgically Unresectable Soft Tissue Sarcoma	Phase 2	48	2012-08-28	Liposarcoma, Leiomyosarcoma, Fibrosarcoma, Malignant Fibrous Histiocytoma	SUNITINIB MALATE	Its record names the disease	ClinicalTrials.gov
NCT02888665	Pembrolizumab and Doxorubicin Hydrochloride in Treating Patients With Sarcoma That is Metastatic or Cannot Be Removed by Surgery	Phase 1/2	37	2021-05-26	Sarcoma	DOXORUBICIN HYDROCHLORIDE, PEMBROLIZUMAB	Its record names the disease	ClinicalTrials.gov
NCT03123276	Pembrolizumab and Gemcitabine Chemotherapy in Leiomyosarcoma and Undifferentiated Pleomorphic Sarcoma	Phase 1/2	25	2026-02-13	Sarcoma	GEMCITABINE, PEMBROLIZUMAB	Its record names the disease	ClinicalTrials.gov
NCT05017103	Sintilimab for the Treatment of Locally Advanced, Metastatic, Recurrent, or Unresectable Undifferentiated Pleomorphic Sarcoma, SiARa Cancer Study	Phase 2	6	2024-11-12	Locally Advanced Undifferentiated Pleomorphic Sarcoma, Metastatic Undifferentiated Pleomorphic Sarcoma, Recurrent Undifferentiated Pleomorphic Sarcoma, Unresectable Undifferentiated Pleomorphic Sarcoma	SINTILIMAB	Its record names the disease	ClinicalTrials.gov
NCT01710176	Localized High-Risk Soft Tissue Sarcomas Of The Extremities And Trunk Wall In Adults: An Integrating Approach Comprising Standard Vs Histotype-Tailored Neoadjuvant Chemotherapy	Phase 3	550		Localized High-risk Soft Tissue Sarcomas of the Extremities and Trunk Wall in Adults	DACARBAZINE, DOCETAXEL, EPIRUBICIN, ETOPOSIDE, GEMCITABINE, IFOSFAMIDE, TRABECTEDIN	Its record names the disease	ClinicalTrials.gov
NCT06422806	Measuring if Immunotherapy Plus Chemotherapy is Better Than Chemotherapy Alone for Patients With Aggressive Poorly Differentiated Sarcomas	Phase 3	365		Metastatic Dedifferentiated Liposarcoma, Metastatic Undifferentiated Pleomorphic Sarcoma, Stage III Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8, Stage IV Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8, Unresectable Dedifferentiated Liposarcoma, Unresectable Undifferentiated Pleomorphic Sarcoma	DOXORUBICIN, PEMBROLIZUMAB	Its record names the disease	ClinicalTrials.gov

1 to 10 of 34

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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
Phase 1 Cohort 1 vs Phase 1 Cohort 2: Maximum Tolerated Dose (MTD) of Doxorubicin Hydrochloride Plus Pembrolizumab According to the National Cancer Institute (NCI) Common Terminology for Adverse Events (CTCAE) Version 4.0 (Phase I)	0 of 3	0 of 3	6	NCT02888665

Source Trial comparisons

Papers from its trials24

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
39737219	RESULT	1	PubMed
22067397	RESULT	1	PubMed
19451433	RESULT	1	PubMed
42051923	DERIVED	1	PubMed
41836677	DERIVED	1	PubMed
41559089	DERIVED	1	PubMed
39575028	DERIVED	1	PubMed
39547252	DERIVED	1	PubMed
39356980	DERIVED	1	PubMed
38579194	DERIVED	1	PubMed

1 to 10 of 24

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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_0002142

GET https://conductscience.org/api/diseases/v1/MONDO_0002142/drugs?page=2

API reference For AI assistants