RecruitingNCT02402244
Oncology
Desmoid Tumor
Also known as aggressive fibromatosis, desmoid-type fibromatosis, CTNNB1/APC mutation
Desmoid tumours are rare, locally invasive fibroblastic neoplasms that do not metastasise but cause significant morbidity through infiltrative growth into adjacent structures, nerves, and viscera. They arise from somatic activating mutation
ORPHA:873 ↗Gene CTNNB1Gene APCPrevalence 2–4 in 1,000,000Onset Young to middle-aged adult; FAP-associated tumours may present in 3rd decadeSporadic (somatic CTNNB1 mutation) or familial (germline APC mutation in FAP)
19
studies recruiting now
as of 7 Sept 2026
77
studies registered in total
as of 7 Sept 2026
12
countries with a recruiting site
as of 7 Sept 2026
16 Sept 2025
most recent study posted
among recruiting studies
Recruiting trials
RecruitingPhase 1 / Phase 2NCT04851119
Tegavivint for the Treatment of Recurrent or Refractory Solid Tumors, Including Lymphomas and Desmoid Tumors
RecruitingPhase 1 / Phase 2NCT05919264
FOG-001 in Locally Advanced or Metastatic Solid Tumors
RecruitingPhase 4NCT07176689
Nirogacestat in Premenopausal Females With Desmoid Tumor/Aggressive Fibromatosis (DT/AF)
RecruitingPhase 2NCT05879146
Evaluation of the Response and Non-response of Nirogacestat in Desmoid Tumors- Clinical Study
Showing the 5 most recently updated recruiting studies, as recorded 7 Sept 2026. Live status on each study page.
See all 19 recruiting studiesWhere recruiting studies are running
Countries with at least one recruiting site among the studies above, 7 Sept 2026. Tap a country to search trials there.
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About Desmoid Tumor
Desmoid tumours are rare, locally invasive fibroblastic neoplasms that do not metastasise but cause significant morbidity through infiltrative growth into adjacent structures, nerves, and viscera. They arise from somatic activating mutations in CTNNB1 (encoding beta-catenin) in approximately 90% of sporadic cases, or in the setting of familial adenomatous polyposis (FAP) due to germline APC mutations that result in constitutive WNT/beta-catenin signalling. The clinical behaviour is highly unpredictable — some tumours spontaneously stabilise or regress, while others progress rapidly and become unresectable, necessitating systemic therapy.
Common clinical features
Palpable, firm, painless or mildly painful soft tissue mass most commonly in the abdominal wall, mesentery, or extremityMesenteric desmoids (common in FAP) causing bowel obstruction, ureteric compression, or mesenteric ischaemiaProgressive pain and restricted range of motion when tumours involve extremity musculature or nervesPeripheral neuropathy from perineural tumour infiltrationBowel fistula or perforation from mesenteric desmoid erosion into adjacent intestinal loopsChest wall or thoracic desmoids causing dyspnoea or chest pain from pleural or mediastinal involvementRapid growth during pregnancy due to oestrogen sensitivity in some tumours
From Orphanet’s phenotype annotations (CC BY 4.0). Not a complete list.
Treatments being studied
1 approved treatment and 15 in clinical development, from Open Targets (CC BY 4.0). Not medical advice.
Approved: Nirogacestat
Phase 3Doxorubicin (Adriblastina)
Phase 3Sodium Chloride (Aqsia (balanced salt soln))
Phase 3Sorafenib Tosylate (Nexavar)
Phase 2Aminolevulinic Acid
Phase 2Hydroxyurea (Droxia)
Phase 2Imatinib
Phase 2Methotrexate (Ebetrex)
Phase 2Tamoxifen Citrate (Emblon)
+ 7 more in development
Before you apply
Things trial teams commonly ask about for Desmoid Tumor. Not eligibility rules; those are set by each study.
- Molecular subtyping by CTNNB1 exon 3 mutation status (T41A, S45F, S45P) or APC germline testing is increasingly used for risk stratification and may be an eligibility or stratification criterion in trials.
- A watch-and-wait observation period is now standard of care for non-progressive disease; trials may require documented progression within a specified prior timeframe — provide dated imaging series demonstrating progression.
- FAP-associated desmoid tumours may have different biology and eligibility criteria from sporadic tumours; clarify FAP status and provide relevant genetic and colonoscopy documentation.
Related conditions
Information, not medical advice. Trial listings are shown as recorded on ClinicalTrials.gov; whether any study is right for you is a decision for you and your clinicians, and eligibility is decided by each research team. Disease information from Orphanet (CC BY 4.0).