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Desmoid Tumors Market

In 2023, the desmoid tumors market across the seven major markets (7MM) was estimated at around USD 89 million and is projected to expand substantially over the coming years. The United States held the largest share, contributing nearly 68% of the total market value, and also recorded the highest number of incident cases among the ~3,400 reported across the 7MM.

CTNNB1 mutations accounted for the vast majority of mutation-specific cases (~28,400), significantly surpassing APC mutations (~3,000). At present, OGSIVEO (nirogacestat) remains the only FDA-approved therapy for progressing desmoid tumors requiring systemic treatment, supplemented by chemotherapy and other supportive options.

The treatment paradigm is evolving from surgery toward active surveillance and systemic therapies. Multiple companies – such as SpringWorks Therapeutics, Immunome/Ayala, Iterion, and Eisai – are advancing innovative pipeline agents expected to stimulate notable market growth between 2024 and 2034.

DelveInsight’s report, “Desmoid Tumors Market Insights, Epidemiology, and Market Forecast 2034,” offers an in-depth evaluation of Desmoid Tumors, presenting historical trends and future epidemiological projections, along with detailed market dynamics across the United States, EU4 (Germany, Spain, Italy, France), the United Kingdom, and Japan. The analysis highlights current therapeutic practices, upcoming treatment options, drug-specific market shares, and market size forecasts from 2020 to 2034. The report also reviews existing treatment pathways, major market drivers and barriers, and key unmet needs – helping identify strategic opportunities and assess the overall growth outlook for the Desmoid Tumors market.

To Know in detail about the Desmoid Tumors market outlook, drug uptake, treatment scenario and epidemiology trends, Click here; Desmoid Tumors Market Forecast

Some of the key facts of the Desmoid Tumors Market Report:

• The Desmoid Tumors market size was valued ~USD 89 million in 2023 and is anticipated to grow with a significant CAGR during the study period (2020-2034)
• In 2023, the United States held the largest market size for Desmoid Tumors among the seven major markets (7MM), valued at approximately USD 60 million.
• In 2023, there were approximately 3,400 new cases of desmoid tumors across the seven major markets (7MM), with the United States reporting the highest number of these incident cases.
• In 2023, mutation-specific desmoid tumor cases in the seven major markets (7MM) were predominantly driven by CTNNB1 gene mutations, accounting for nearly 28,400 cases, whereas APC gene mutations were associated with around 3,000 cases.
• In 2023, the United States held the largest share of the desmoid tumors market among the seven major markets (7MM), representing about 68% of the total market.
• Desmoid tumors make up less than 3% of all soft tissue tumors, with an estimated annual incidence ranging from 1 in 250,000 to 1 in 500,000.
• In the United States, approximately 1,000 to 1,650 new cases of desmoid tumors are diagnosed each year.
• In 2023, the 10-year prevalence of desmoid tumors in the EU4 and the UK was approximately 4,500 cases among males and 8,300 cases among females.
• Major companies in the desmoid tumors space, including SpringWorks Therapeutics (OGSIVEO [nirogacestat]), Immunome/Ayala Pharmaceuticals (AL102), Iterion Therapeutics and Apollomics (Tegavivint [BC2059]), Eisai and PRISM BioLab (E7386), among others, are currently advancing their lead candidates through various stages of clinical development. These developments are expected to have a significant impact on the desmoid tumors market throughout the forecast period from 2024 to 2034.
• The desmoid tumors pipeline is relatively limited and includes therapies such as Gamma Secretase Inhibitors (GSI), TBL1 inhibitors, CBP/ß-catenin interaction inhibitors, and other treatments.
• Key Desmoid Tumors Companies: SpringWorks Therapeutics, Immunome/Ayala Pharmaceuticals, Iterion Therapeutics, Eisai and PRISM BioLab, MedPacto, Inc., Pfizer, Novartis, Ayala Pharmaceuticals, Iterion Therapeutics, Aadi Bioscience, Inc., INSYS Therapeutics Inc, Recursion Pharmaceuticals Inc, and others
• Key Desmoid Tumors Therapies: OGSIVEO (nirogacestat), AL102, Tegavivint (BC2059), E7386, Vactosertib, Sirolimus, Imatinib, AL102, Tegavivint, Nab-Rapamycin, Fentanyl sublingual spray, REC-4881, and others
• The Desmoid Tumors epidemiology based on gender analyzed that Desmoid Tumors are more common in women as compared to men
• The Desmoid Tumors market is expected to surge due to the disease’s increasing prevalence and awareness during the forecast period. Furthermore, launching various multiple-stage Desmoid Tumors pipeline products will significantly revolutionize the Desmoid Tumors market dynamics.

Desmoid Tumors Overview

Desmoid tumors, also known as aggressive fibromatosis, are rare, locally invasive soft tissue tumors arising from fibroblasts. Although non-metastatic, they can grow aggressively and cause significant morbidity by infiltrating surrounding tissues, nerves, and organs. These tumors may occur sporadically or be associated with genetic conditions such as familial adenomatous polyposis (FAP). Mutations in the CTNNB1 and APC genes are commonly implicated in disease development. Symptoms vary based on tumor location and may include pain, swelling, and functional impairment. Management strategies include active surveillance, systemic therapies, targeted agents, and, in select cases, surgery, with treatment chosen based on tumor behavior and patient-specific factors.

Get a Free sample for the Desmoid Tumors Market Report: Desmoid Tumors Market Dynamics and Trends

Desmoid Tumors Epidemiology

Desmoid tumors are uncommon, accounting for under 3% of all soft tissue tumors, with an annual incidence of roughly 1 in 250,000–500,000 individuals. In the US, about 1,000–1,650 new cases occur each year. In 2023, the EU4 and the UK recorded an estimated 4,500 male and 8,300 female 10-year prevalent cases. In Japan, extra-abdominal desmoid tumors appeared nearly three times more frequently than intra-abdominal forms. Over 90% of cases develop sporadically due to CTNNB1 (β-catenin) mutations, while a smaller subset occurs in people with germline APC mutations related to familial adenomatous polyposis (FAP). Diagnosis is frequently delayed or inaccurate because of low awareness, and the disease often follows an unpredictable course.

Desmoid Tumors Epidemiology Segmentation:

The Desmoid Tumors market report proffers epidemiological analysis for the study period 2020-2034 in the 7MM segmented into:

• Total Prevalence of Desmoid Tumors
• Prevalent Cases of Desmoid Tumors by severity
• Gender-specific Prevalence of Desmoid Tumors
• Diagnosed Cases of Episodic and Chronic Desmoid Tumors

Download the report to understand which factors are driving Desmoid Tumors epidemiology trends @ Desmoid Tumors Epidemiology Forecast

Desmoid Tumors Drugs Uptake and Pipeline Development Activities

AL102 – Immunome/Ayala Pharmaceuticals AL102 is an investigational gamma secretase inhibitor targeting Notch 1–4 signaling, currently in Phase II/III RINGSIDE trials for progressing desmoid tumors. Originally licensed from Bristol Myers Squibb in 2017, it received Fast Track and Orphan Drug Designations from the FDA in 2022 and 2023. In March 2024, Immunome acquired AL102 and its related candidate AL101 from Ayala.

Tegavivint (BC2059) – Iterion Therapeutics Tegavivint is a first-in-class small molecule that blocks TBL1, a key co-factor in the Wnt/β-catenin pathway, disrupting cancer-driving transcription without affecting normal cell membrane functions. A Phase I trial was completed in 2022. Apollomics holds commercialization rights in Greater China under a 2021 partnership.

E7386 – Eisai / PRISM BioLab E7386 is a CBP/β-catenin inhibitor that blocks downstream Wnt signaling by disrupting CBP–β-catenin interactions, including in APC or β-catenin mutant tumors. It achieved clinical proof of concept in 2021 and has been under joint development by Eisai and PRISM since 2011.

Desmoid Tumors Competitive Landscape

• OGSIVEO (nirogacestat): SpringWorks Therapeutics
• AL102: Immunome/Ayala Pharmaceuticals
• Tegavivint (BC2059): Iterion Therapeutics
• E7386: Eisai and PRISM BioLab
• Vactosertib: MedPacto, Inc.
• Sirolimus: Pfizer
• Imatinib: Novartis
• Nab-Rapamycin: Aadi Bioscience, Inc.
• Fentanyl sublingual spray: INSYS Therapeutics Inc
• REC-4881: Recursion Pharmaceuticals Inc.

Discover more about therapies set to grab major Desmoid Tumors market share @ Desmoid Tumors Treatment Market

Desmoid tumors Market Outlook

In 2023, the US held the largest desmoid tumors market (~USD 60M), with France leading in EU4/UK markets. The pipeline remains limited, focusing on GSIs, TBL1 inhibitors, and CBP/β-catenin inhibitors.

Desmoid tumors are localized, non-metastatic neoplasms with unpredictable behavior. Asymptomatic patients may be monitored through active surveillance, while symptomatic cases – especially those linked to Gardner’s syndrome – require systemic therapy. Surgery, hormonal therapy, or NSAIDs are no longer first-line treatments. Targeted therapies, including TKIs like sorafenib and GSIs like nirogacestat, have shown significant efficacy, and Wnt pathway–targeted treatments are in development. Cryoablation is emerging for extra-abdominal tumors.

Treatment strategies depend on tumor location: abdominal wall tumors are often surgically managed, intra-abdominal and critical-structure tumors receive systemic therapy, and extremity or chest wall tumors favor medical management unless low-risk surgery is feasible. FAP-associated desmoid tumors are more aggressive and may require intensive therapy, including TKIs, chemotherapy, NSAIDs, or antihormonal agents.

Desmoid Tumors Market Drivers

1. Rising Awareness and Early Diagnosis

Increased awareness among healthcare providers and patients, coupled with improvements in imaging and diagnostic techniques, is facilitating earlier detection of desmoid tumors, expanding the diagnosed patient population.

2. Advances in Targeted Therapies

The approval of OGSIVEO (nirogacestat) and the development of novel candidates such as AL102, Tegavivint, and E7386 are transforming treatment approaches, creating new growth opportunities.

3. Shift from Surgery to Systemic Therapy

The evolving treatment paradigm—moving from surgical intervention toward active surveillance and systemic therapies—is increasing market adoption of pharmacologic options.

4. Strong R&D Pipeline

Multiple biopharma companies are actively developing innovative therapies, driving future market growth through clinical advancement and pipeline expansion.

Desmoid Tumors Market Barriers

1. Rare Disease with Limited Patient Population

Desmoid tumors are extremely rare, resulting in a small patient base, which can limit commercial potential.

2. High Cost of Treatment

Novel targeted therapies and supportive systemic treatments are expensive, restricting access in certain regions.

3. Clinical and Diagnostic Challenges

Delayed or incorrect diagnoses due to low disease awareness and variable disease presentation can impede timely treatment.

4. Unpredictable Disease Course

The highly variable and often unpredictable progression of desmoid tumors complicates treatment decisions and outcomes.

Scope of the Desmoid Tumors Market Report

• Study Period: 2020-2034
• Coverage: 7MM [The United States, EU5 (Germany, France, Italy, Spain, and the United Kingdom), and Japan]
• Key Desmoid Tumors Companies: SpringWorks Therapeutics, Immunome/Ayala Pharmaceuticals, Iterion Therapeutics, Eisai and PRISM BioLab, MedPacto, Inc., Pfizer, Novartis, Ayala Pharmaceuticals, Iterion Therapeutics, Aadi Bioscience, Inc., INSYS Therapeutics Inc, Recursion Pharmaceuticals Inc., and others
• Key Desmoid Tumors Therapies: OGSIVEO (nirogacestat), AL102, Tegavivint (BC2059), E7386, Vactosertib, Sirolimus, Imatinib, AL102, Tegavivint, Nab-Rapamycin, Fentanyl sublingual spray, REC-4881, and others
• Desmoid Tumors Therapeutic Assessment: Desmoid Tumors current marketed and Desmoid Tumors emerging therapies
• Desmoid Tumors Market Dynamics: Desmoid Tumors market drivers and Desmoid Tumors market barriers
• Competitive Intelligence Analysis: SWOT analysis, PESTLE analysis, Porter’s five forces, BCG Matrix, Market entry strategies
• Desmoid Tumors Unmet Needs, KOL’s views, Analyst’s views, Desmoid Tumors Market Access and Reimbursement

To know more about Desmoid Tumors companies working in the treatment market, visit @ Desmoid Tumors Clinical Trials and Therapeutic Assessment

Table of Contents

1. Desmoid Tumors Market Report Introduction

2. Executive Summary for Desmoid Tumors

3. SWOT analysis of Desmoid Tumors

4. Desmoid Tumors Patient Share (%) Overview at a Glance

5. Desmoid Tumors Market Overview at a Glance

6. Desmoid Tumors Disease Background and Overview

7. Desmoid Tumors Epidemiology and Patient Population

8. Country-Specific Patient Population of Desmoid Tumors

9. Desmoid Tumors Current Treatment and Medical Practices

10. Desmoid Tumors Unmet Needs

11. Desmoid Tumors Emerging Therapies

12. Desmoid Tumors Market Outlook

13. Country-Wise Desmoid Tumors Market Analysis (2020-2034)

14. Desmoid Tumors Market Access and Reimbursement of Therapies

15. Desmoid Tumors Market Drivers

16. Desmoid Tumors Market Barriers

17. Desmoid Tumors Appendix

18. Desmoid Tumors Report Methodology

19. DelveInsight Capabilities

20. Disclaimer

21. About DelveInsight

About DelveInsight

DelveInsight is a leading healthcare-focused market research and consulting firm that provides clients with high-quality market intelligence and analysis to support informed business decisions. With a team of experienced industry experts and a deep understanding of the life sciences and healthcare sectors, we offer customized research solutions and insights to clients across the globe. Connect with us to get high-quality, accurate, and real-time intelligence to stay ahead of the growth curve.

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