Disease · MONDO_0019373

desmoplastic small round cell tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. desmoplastic small round cell tumor. Diseases, record MONDO_0019373. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0019373

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: desmoplastic small round cell tumor; Open Targets
Open Targets ID: MONDO_0019373; Open Targets
Other names: DSRCT, Desmoplas. small round cell tumor, Desmoplas. small round cell tumour, Desmoplastic small round cell tumour, Desmoplastic small round-cell neoplasm, Polyphenotypic small round cell tumor, Polyphenotypic small round cell tumour; Open Targets
Disease Ontology: DOID:6785; Disease Ontology
MeSH: D058405; MeSH
NCI Thesaurus: C8300; NCI Thesaurus
Orphanet: ORPHA:83469; Orphanet
Highest stage of its drugs: Phase 2, for 32 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
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	5	5	5	Drugs and targets
IRINOTECAN	CHEMBL481	Small molecule	Phase 2	ClinicalTrials.gov	5	5	5	Drugs and targets
TEMOZOLOMIDE	CHEMBL810	Small molecule	Phase 2	ClinicalTrials.gov	4	4	4	Drugs and targets
BEVACIZUMAB	CHEMBL1201583	Antibody	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
OMBURTAMAB	CHEMBL4297233	Antibody	Phase 2	ClinicalTrials.gov	3	2	2	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
IMATINIB MESYLATE	CHEMBL1642	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
SORAFENIB	CHEMBL1336	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets

1 to 10 of 41

See all 41 drugs

Targets69

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	4	Vascular endothelial growth factor receptor 2 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3791	Drugs and targets
FKBP1A	Peptidyl-prolyl cis-trans isomerase FKBP1A	3	FK506-binding protein 1A inhibitor	Phase 2	https://conductscience.org/library/genes/2280	Drugs and targets
FLT1	Vascular endothelial growth factor receptor	3	Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/2321	Drugs and targets
FLT4	Vascular endothelial growth factor receptor	3	Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/2324	Drugs and targets
KIT	Mast/stem cell growth factor receptor Kit	3	Stem cell growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3815	Drugs and targets
PDGFRB	Platelet-derived growth factor receptor	3	Platelet-derived growth factor receptor beta inhibitor; Platelet-derived growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/5159	Drugs and targets
TOP1	DNA topoisomerase 1	3	DNA topoisomerase I inhibitor	Phase 2	https://conductscience.org/library/genes/7150	Drugs and targets
TUBA1A	Tubulin	3	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7846	Drugs and targets
TUBA1B	Tubulin	3	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/10376	Drugs and targets
TUBA1C	Tubulin	3	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/84790	Drugs and targets

1 to 10 of 69

See all 69 targets

Trials27

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
NCT00642941	A Study of R1507 in Participants With Recurrent or Refractory Sarcoma	Phase 2	317	2021-02-03	Sarcoma	TEPROTUMUMAB	Its record names the disease	ClinicalTrials.gov
NCT00043979	Stem Cell Transplantation in Patients With High-Risk and Recurrent Pediatric Sarcomas	Phase 2	60	2013-09-13	Sarcoma	CYCLOPHOSPHAMIDE, CYCLOSPORINE, DOXORUBICIN, ETOPOSIDE, FILGRASTIM, FLUDARABINE, MELPHALAN, PREDNISONE, SIROLIMUS, TACROLIMUS ANHYDROUS, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT03034200	Phase 2 Study of ONC201 in Neuroendocrine Tumors	Phase 2	30	2024-11-26	Recurrent Neuroendocrine Tumor, Metastatic Neuroendocrine Tumor	DORDAVIPRONE	Its record names the disease	ClinicalTrials.gov
NCT04145349	CAMPFIRE: A Study of Ramucirumab (LY3009806) in Children and Young Adults With Desmoplastic Small Round Cell Tumor	Phase 1/2	30	2025-07-20	Desmoplastic Small Round Cell Tumor	CYCLOPHOSPHAMIDE, RAMUCIRUMAB, VINORELBINE	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
NCT01277744	Pilot Study of Hyperthermic Peritoneal Perfusion (HIPEC) for Adolescent and Young Adults With Desmoplastic Small Round Cell Tumor	Phase 2	22	2019-10-01	Advanced Cancers, Sarcoma	CISPLATIN	Its record names the disease	ClinicalTrials.gov
NCT05131386	Multicohort Trial of Trabectedin and Low-dose Radiation Therapy in Advanced/Metastatic Sarcomas	Phase 2	85		Soft Tissue Sarcoma, Bone Tumors, Small Round-cell Sarcomas	TRABECTEDIN	Open Targets' reading of its record	ClinicalTrials.gov
NCT06986811	CEB-01 in Pediatrics With Locally Resectable Abdominal Tumors	Phase 2	80		Locally Resectable Pediatrics Tumors	7-ETHYL-10-HYDROXYCAMPTOTHECIN	Its record names the disease	ClinicalTrials.gov
NCT05004116	A Study of Repotrectinib in Combination With Chemotherapy in Children and Young Adults With Solid Tumor Cancer	Phase 1/2	77		Advanced Cancer, Metastatic Solid Tumor	IRINOTECAN, REPOTRECTINIB, TEMOZOLOMIDE	Its record names the disease	ClinicalTrials.gov
NCT03275818	Trial of Nab-paclitaxel in Patients With Desmoid Tumors and Multiply Relapsed/Refractory Desmoplastic Small Round Cell Tumors and Ewing Sarcoma	Phase 2	69		Tumor, Desmoplastic Small Round Cell, Adult, Tumor, Desmoplastic Small Round Cell, Childhood, Sarcoma, Ewing, Sarcoma, Desmoid	PACLITAXEL	Its record names the disease	ClinicalTrials.gov

1 to 10 of 27

See all 27 trials

Comparisons in its trials3

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	Source
Ramucirumab + Cyclophosphamide + Vinorelbine vs Cyclophosphamide + Vinorelbine: deaths from any cause	12 of 20	7 of 10	30	NCT04145349	Trial comparisons
Ramucirumab + Cyclophosphamide + Vinorelbine vs Cyclophosphamide + Vinorelbine: serious adverse events	6 of 20	3 of 10	30	NCT04145349	Trial comparisons
Ramucirumab + Cyclophosphamide + Vinorelbine vs Cyclophosphamide + Vinorelbine: Number of Participants With Treatment-Emergent Anti-Drug Antibodies (TE-ADA)	0 of 13	0 of 5	18	NCT04145349	Trial comparisons

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
35022321	RESULT	1	PubMed
21896345	RESULT	1	PubMed
40934647	DERIVED	1	PubMed
33119478	DERIVED	1	PubMed
22682017	DERIVED	1	PubMed
22025149	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_0019373

GET https://conductscience.org/api/diseases/v1/MONDO_0019373/drugs?page=2

API reference For AI assistants