Disease · MONDO_0007608

desmoid tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. desmoid tumor. Diseases, record MONDO_0007608. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0007608

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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: desmoid tumor; Open Targets
Open Targets ID: MONDO_0007608; Open Targets
Other names: aggressive fibromatosis, deep fibromatosis, deep fibromatosis/desmoid tumor, deep fibromatosis/desmoid tumour, desmoid fibromatosis, desmoid type fibromatosis; Open Targets
Disease Ontology: DOID:0080366; Disease Ontology
MeSH: D018222; MeSH
NCI Thesaurus: C9182; NCI Thesaurus
Orphanet: ORPHA:873; Orphanet
Highest stage of its drugs: Approval, for 1 of its 29 drugs, from EMA Human Drugs; Open Targets

Drugs29

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
NIROGACESTAT	CHEMBL1770916	Small molecule	Approval	EMA Human Drugs	9	8	8	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
SORAFENIB TOSYLATE	CHEMBL1200485	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
IMATINIB	CHEMBL941	Small molecule	Phase 2	ClinicalTrials.gov	5	5	5	Drugs and targets
VINBLASTINE	CHEMBL159	Small molecule	Phase 2	ClinicalTrials.gov	3	3	3	Drugs and targets
METHOTREXATE	CHEMBL34259	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
NIVOLUMAB	CHEMBL2108738	Antibody	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
TOREMIFENE	CHEMBL1655	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
VACTOSERTIB	CHEMBL3260567	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
AMINOLEVULINIC ACID	CHEMBL601	Small molecule	Phase 2	ClinicalTrials.gov	1	1	0	Drugs and targets

1 to 10 of 29

See all 29 drugs

Targets54

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
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	3	Vascular endothelial growth factor receptor 3 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/2324	Drugs and targets
KDR	Vascular endothelial growth factor receptor	3	Vascular endothelial growth factor receptor 2 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/3791	Drugs and targets
ABL1	Bcr/Abl fusion protein	2	Bcr/Abl fusion protein inhibitor; Tyrosine-protein kinase ABL inhibitor	Phase 2	https://conductscience.org/library/genes/25	Drugs and targets
BCR	Bcr/Abl fusion protein	2	Bcr/Abl fusion protein inhibitor	Phase 2	https://conductscience.org/library/genes/613	Drugs and targets
ESR1	Estrogen receptor	2	Estrogen receptor alpha modulator; Estrogen receptor modulator	Phase 2	https://conductscience.org/library/genes/2099	Drugs and targets
FLT1	Vascular endothelial growth factor receptor	2	Vascular endothelial growth factor receptor inhibitor	Phase 3	https://conductscience.org/library/genes/2321	Drugs and targets
PDCD1	Programmed cell death protein 1	2	Programmed cell death protein 1 inhibitor	Phase 2	https://conductscience.org/library/genes/5133	Drugs and targets
TUBA1A	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7846	Drugs and targets

1 to 10 of 54

See all 54 targets

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. 1 more is not shown, since the checks do not confirm it.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT03785964	Nirogacestat for Adults With Desmoid Tumor/Aggressive Fibromatosis (DT/AF)	Phase 3	142	2024-06-12	Desmoid Tumor, Aggressive Fibromatosis	NIROGACESTAT	Its record names the disease	ClinicalTrials.gov
NCT02066181	Sorafenib Tosylate in Treating Patients With Desmoid Tumors or Aggressive Fibromatosis	Phase 3	87	2019-09-20	Desmoid Fibromatosis	SORAFENIB TOSYLATE	Its record names the disease	ClinicalTrials.gov
NCT01876082	PAZOPANIB Efficacy and Tolerance in Desmoids Tumors	Phase 2	72	2021-03-05	Progressive Desmoids Tumors	PAZOPANIB, VINBLASTINE	Open Targets' reading, confirmed by NLM's indexing of its papers	ClinicalTrials.gov
NCT00068419	Sulindac and Tamoxifen in Treating Patients With Desmoid Tumor	Phase 2	70	2013-12-04	Desmoid Tumor	SULINDAC, TAMOXIFEN	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
NCT02145741	Weekly Intravenous Administrations of BI 836845 in Japanese Patients With Advanced Solid Tumours	Phase 1	21	2025-06-19	Neoplasms	XENTUZUMAB	Its record names the disease	ClinicalTrials.gov
NCT01981551	Phase II Trial of the Gamma-Secretase Inhibitor PF-03084014 in Adults With Desmoid Tumors/Aggressive Fibromatosis	Phase 2	17	2020-02-05	Desmoid Tumors, Aggressive Fibromatosis	NIROGACESTAT	Its record names the disease	ClinicalTrials.gov
NCT01265030	A Pilot Study Evaluating the Use of mTor Inhibitor Sirolimus in Children and Young Adults With Desmoid-Type Fibromatosis	Phase 1/2	9	2023-06-26	Desmoid Tumor	SIROLIMUS	Its record names the disease	ClinicalTrials.gov
NCT03886311	Talimogene Laherparepvec, Nivolumab and Trabectedin for Sarcoma	Phase 2	200		Sarcoma	NIVOLUMAB, TALIMOGENE LAHERPAREPVEC, TRABECTEDIN	Its record names the disease	ClinicalTrials.gov
NCT04851119	Tegavivint for the Treatment of Recurrent or Refractory Solid Tumors, Including Lymphomas and Desmoid Tumors	Phase 1/2	147		Colorectal Carcinoma, Endometrial Carcinoma, Melanoma, Neuroblastoma, Ovarian Carcinoma, Pancreatic Ductal Adenocarcinoma, Recurrent Desmoid Fibromatosis, Recurrent Ewing Sarcoma, Recurrent Hepatoblastoma, Recurrent Hepatocellular Carcinoma, Recurrent Malignant Solid Neoplasm, Recurrent Non-Hodgkin Lymphoma, Recurrent Osteosarcoma, Refractory Desmoid Fibromatosis, Refractory Ewing Sarcoma, Refractory Hepatoblastoma, Refractory Hepatocellular Carcinoma, Refractory Malignant Solid Neoplasm, Refractory Non-Hodgkin Lymphoma, Refractory Osteosarcoma, Solid Pseudopapillary Neoplasm of the Pancreas, Wilms Tumor	TEGAVIVINT	Open Targets' reading of its record	ClinicalTrials.gov

1 to 10 of 34

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Comparisons in its trials4

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
Double-Blind Phase - Nirogacestat vs Double-Blind Phase - Placebo: deaths from any cause	0 of 69	1 of 72	141	NCT03785964	Trial comparisons
Double-Blind Phase - Nirogacestat vs Double-Blind Phase - Placebo: serious adverse events	14 of 69	8 of 72	141	NCT03785964	Trial comparisons
PAZOPANIB vs Vinblastine and Methotrexate: deaths from any cause	1 of 48	0 of 22	70	NCT01876082	Trial comparisons
PAZOPANIB vs Vinblastine and Methotrexate: serious adverse events	20 of 48	10 of 22	70	NCT01876082	Trial comparisons

Papers from its trials17

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
28350521	RESULT	1	PubMed
24089446	RESULT	1	PubMed
20622000	RESULT	1	PubMed
17290057	RESULT	1	PubMed
41903360	DERIVED	1	PubMed
41115259	DERIVED	1	PubMed
37347393	DERIVED	1	PubMed
36884323	DERIVED	1	PubMed
34870878	DERIVED	1	PubMed
34590610	DERIVED	1	PubMed

1 to 10 of 17

See all 17 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_0007608

GET https://conductscience.org/api/diseases/v1/MONDO_0007608/drugs?page=2

API reference For AI assistants