DelveInsight’s “Desmoid Tumors Market Insight, Epidemiology, and Market Forecast 2034” report provides a comprehensive analysis of desmoid tumors, including historical and forecasted epidemiology, current treatment practices, emerging therapies, and market trends across the United States, EU4 (Germany, France, Italy, and Spain), the United Kingdom, and Japan.
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Some of the Key Facts of the Desmoid Tumors Market Report
• The desmoid tumors market across the 7MM was valued at approximately USD 89 million in 2023 and is projected to grow at a significant CAGR through the 2024–2034 forecast period.
• The United States held the largest share of the desmoid tumors market, accounting for roughly 68% of the overall 7MM market in 2023, with a market size of approximately USD 60 million.
• Total incident cases of desmoid tumors across the 7MM were estimated at around 3,400 in 2023, with the United States contributing the highest share of cases.
• CTNNB1 gene mutations accounted for the vast majority of mutation-specific cases (~28,400), far outnumbering APC gene mutation-driven cases (~3,000) in 2023.
• OGSIVEO (nirogacestat), from SpringWorks Therapeutics, remains the only US FDA-approved therapy for adult patients with progressing desmoid tumors requiring systemic treatment; it received FDA approval in November 2023, and its EU Marketing Authorization Application was validated in February 2024.
• In November 2024, SpringWorks reported long-term Phase III DeFi trial data showing continued tumor-size reduction, higher objective response rates, and sustained symptom improvement with extended nirogacestat treatment.
• In March 2024, Immunome completed its acquisition of AL102 (and related candidate AL101) from Ayala Pharmaceuticals; AL102 holds FDA Fast Track and Orphan Drug designations and is in Phase II/III development (RINGSIDE study).
• Emerging candidates such as AL102, Tegavivint (BC2059), and E7386 are expected to expand and diversify the desmoid tumors treatment landscape over the forecast period.
Key Desmoid Tumors Companies
• SpringWorks Therapeutics
• Immunome / Ayala Pharmaceuticals
• Iterion Therapeutics
• Apollomics
• Eisai
• PRISM BioLab
• Others
Key Desmoid Tumors Therapies
• OGSIVEO (nirogacestat)
• AL102
• Tegavivint (BC2059)
• E7386
• Others
The desmoid tumors market is expected to expand due to rising disease incidence, improved diagnostic capabilities, growing awareness supported by desmoid tumor-specific ICD-10 coding, and a shift away from aggressive surgery toward active surveillance and systemic therapy. A diversifying pipeline of gamma secretase inhibitors, TBL1 inhibitors, and Wnt/β-catenin pathway inhibitors is expected to drive market growth through 2034.
Desmoid Tumors Overview
Desmoid tumors, also known as aggressive fibromatosis, are rare mesenchymal neoplasms arising from fibroblast cells in connective tissue. They occur in an estimated 2 to 6 cases per million annually and are characterized by local invasiveness without metastatic potential. More than 90% of cases are sporadic and linked to CTNNB1 mutations, while a smaller subset occurs in patients with germline APC mutations associated with familial adenomatous polyposis (FAP).
Desmoid tumors commonly develop:
• Sporadically, tied to CTNNB1 mutation
• In association with FAP/Gardner syndrome via APC mutation
• At sites of prior surgery or trauma
• More frequently in females, particularly during or after pregnancy
Diagnosis is often delayed, with misdiagnosis reported in 30–40% of cases and diagnostic delays exceeding one year in over half of patients. MRI is the preferred imaging modality, and immunohistochemistry (β-catenin positivity, CTNNB1/APC mutation testing) supports confirmation.
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Desmoid Tumors Treatment Landscape
Management of desmoid tumors has shifted from aggressive surgical resection toward active surveillance and systemic therapy, reflecting the tumor’s unpredictable, non-metastatic but locally invasive course.
Current standard-of-care strategies include:
• Active surveillance (“watch and wait”) for asymptomatic patients
• Surgery, primarily for abdominal wall tumors, with R0 resection as the goal
• Radiation therapy as an adjuvant option when margins are positive or surgery isn’t feasible
• Systemic therapy for rapidly progressing tumors or those threatening vital structures
• Chemotherapy regimens (e.g., doxorubicin-based combinations) for select patients
The emerging pipeline is expanding non-invasive, targeted options:
• OGSIVEO (nirogacestat) (SpringWorks Therapeutics) – An oral gamma secretase inhibitor and the first FDA-approved disease-specific therapy for progressing desmoid tumors.
• AL102 (Immunome/Ayala Pharmaceuticals) – An investigational oral gamma secretase inhibitor targeting Notch signaling, in Phase II/III development.
• Tegavivint (BC2059) (Iterion Therapeutics/Apollomics) – A first-in-class TBL1 inhibitor disrupting Wnt/β-catenin signaling.
• E7386 (Eisai/PRISM BioLab) – A CBP/β-catenin interaction inhibitor in early-phase development.
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Desmoid Tumors Market Strengths
• Presence of an approved, disease-specific oral therapy (OGSIVEO) with demonstrated long-term benefit.
• Growing clinical evidence base supporting targeted Wnt/Notch pathway inhibition over surgery.
• Increasing diagnostic accuracy through ICD-10-specific coding and multidisciplinary evaluation.
• Active late-stage pipeline with multiple mechanisms of action in development.
Desmoid Tumors Market Opportunities
• Reduction of diagnostic delays through improved awareness and registries.
• Expansion of oral, non-invasive treatment options reducing reliance on surgery.
• Advancing Phase II/III candidates including AL102, with topline RINGSIDE Part B data anticipated.
• Strong unmet need in FAP-associated (Gardner syndrome) desmoid tumors requiring more intensive management.
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Scope of the Desmoid Tumors Market Report
Study Period
2020–2034
Forecast Period
2024–2034
Coverage
• United States
• Germany
• France
• Italy
• Spain
• United Kingdom
• Japan
Key Companies
• SpringWorks Therapeutics
• Immunome / Ayala Pharmaceuticals
• Iterion Therapeutics
• Apollomics
• Eisai
• PRISM BioLab
• Others
Key Therapies
• OGSIVEO (nirogacestat)
• AL102
• Tegavivint (BC2059)
• E7386
• Others
Analysis Includes
• KOL Views
• SWOT Analysis
• Conjoint Analysis
• Reimbursement Analysis
• Unmet Needs Assessment
• Market Drivers and Barriers
• Epidemiology and Patient Burden Analysis
To learn more about companies developing desmoid tumors therapies
Table of Contents
- Desmoid Tumors Market Report Introduction
- Executive Summary
- SWOT Analysis
- Market Overview
- Disease Background and Overview
- Market Segmentation and Analysis
- Regional Market Analysis
- Country-Specific Market Analysis
- Competitive Landscape
- Key Company Profiles
- Unmet Needs
- Emerging Therapies and Pipeline
- Desmoid Tumors Market Outlook
- Desmoid Tumors Market Access and Reimbursement
- Desmoid Tumors Market Drivers
- Desmoid Tumors Market Barriers
- Recent Developments
- Appendix
- Report Methodology
- DelveInsight Capabilities
- Disclaimer
About DelveInsight
DelveInsight is a leading healthcare business consulting and market research firm focused exclusively on the life sciences industry. The company provides end-to-end market intelligence and strategic consulting services to pharmaceutical, biotechnology, medical device, and diagnostics organizations. Through expertise in epidemiology, competitive intelligence, forecasting, and healthcare consulting, DelveInsight helps clients identify emerging opportunities, accelerate business growth, and make informed strategic decisions across global healthcare markets.
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