Desmoid Tumors Market Outlook 2034 - Clinical Trials, Market Size, Medication, Prevalence, Companies by DelveInsight

Desmoid Tumors Companies are 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
Desmoid Tumors Market Summary

In 2023, the desmoid tumors market across the 7 major markets (7MM) was valued at approximately USD 89 million and is expected to grow significantly over the forecast period. The United States dominated the market, accounting for nearly 68% of the total value. There were around 3,400 incident cases in the 7MM, with the highest number reported in the US.

CTNNB1 gene mutations represented the majority of mutation-specific cases (~28,400), far exceeding APC mutations (~3,000). Currently, OGSIVEO (nirogacestat) is the only FDA-approved therapy for progressing desmoid tumors requiring systemic treatment, alongside chemotherapy and other supportive approaches. The treatment landscape is evolving, shifting from surgical interventions to active surveillance and systemic therapies. Several companies, including SpringWorks Therapeutics, Immunome/Ayala, Iterion, and Eisai, are advancing novel candidates that are expected to further drive market growth from 2024 to 2034.

DelveInsight's report, "Desmoid Tumors Market Insights, Epidemiology, and Market Forecast-2034," delivers a comprehensive analysis of Desmoid Tumors, covering both historical and projected epidemiology as well as market dynamics across the United States, EU4 (Germany, Spain, Italy, France), the United Kingdom, and Japan. The report provides detailed insights into current treatment approaches, emerging therapies, individual drug market shares, and market size projections from 2020 to 2034. Additionally, it examines existing treatment algorithms, key market drivers and challenges, and the prevailing unmet needs-identifying strategic opportunities and evaluating the overall growth potential within the Desmoid Tumors market.

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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 are rare, non-metastasizing but locally aggressive soft tissue neoplasms that arise from connective tissue, particularly in the muscles, fascia, or aponeuroses. Desmoid Tumors are also known as aggressive fibromatosis and can occur anywhere in the body, though they are most commonly found in the abdomen, shoulders, upper arms, and thighs. Desmoid Tumors often affect individuals between the ages of 15 and 40 and are more prevalent in females.

Desmoid Tumors are associated with mutations in the CTNNB1 gene or the APC gene, especially in individuals with familial adenomatous polyposis (FAP). Desmoid Tumors may present as a painless or painful mass and can cause symptoms depending on their location and size, such as bowel obstruction, nerve compression, or organ dysfunction.

Desmoid Tumors are diagnosed through imaging studies like MRI or CT scans, followed by biopsy for histological confirmation. Desmoid Tumors do not spread to distant organs but can recur locally after treatment. Desmoid Tumors treatment options include active surveillance, surgery, radiation therapy, and systemic therapies such as non-steroidal anti-inflammatory drugs, hormonal therapy, chemotherapy, or targeted agents like tyrosine kinase inhibitors.

Desmoid Tumors require individualized treatment plans, and ongoing research is focused on improving outcomes through novel therapies.

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Desmoid Tumors Epidemiology

Desmoid tumors are rare, representing less than 3% of all soft tissue tumors, with an annual incidence of just 1 in 250,000 to 1 in 500,000 people. In the United States, approximately 1,000 to 1,650 new cases are diagnosed each year. Across the EU4 and the UK, there were an estimated 4,500 male and 8,300 female 10-year prevalent cases in 2023. In Japan, extra-abdominal desmoid tumors were nearly three times more common than intra-abdominal cases. Over 90% of desmoid tumors are sporadic and linked to mutations in the CTNNB1 (-catenin) gene, while a smaller proportion arises in individuals with germline APC mutations associated with familial adenomatous polyposis (FAP). Patients often experience delayed or incorrect diagnoses due to limited awareness among both healthcare providers and the public, and the disease course can be highly unpredictable.

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

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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/-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/-catenin inhibitor that blocks downstream Wnt signaling by disrupting CBP--catenin interactions, including in APC or -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.
* AL102: Ayala Pharmaceuticals
* Tegavivint: Iterion Therapeutics
* Sirolimus: Pfizer
* Imatinib: Novartis
* Nab-Rapamycin: Aadi Bioscience, Inc.
* Fentanyl sublingual spray: INSYS Therapeutics Inc
* REC-4881: Recursion Pharmaceuticals Inc.

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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/-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

* Rising Awareness & Improved Diagnosis: Increased recognition of desmoid tumors among clinicians and patients is leading to earlier detection and higher diagnosis rates.
* Shift Toward Systemic & Targeted Therapies: Movement away from aggressive surgery toward less invasive drug-based treatment boosts demand for novel therapeutics.
* Regulatory Support: Orphan Drug and Fast Track designations for emerging therapies (e.g., nirogacestat, AL102) are accelerating development and commercialization.
* Expanding Pipeline: Multiple late-stage candidates from companies like SpringWorks, Immunome/Ayala, Iterion, and Eisai promise competitive market growth.
* Increasing Healthcare Spending: Higher investment in rare disease treatment globally supports market expansion.

Desmoid Tumors Market Barriers

* Low Prevalence & Diagnostic Delays: As a rare and often misdiagnosed condition, patient identification remains limited.
* High Treatment Costs: Novel targeted therapies may pose affordability and reimbursement challenges.
* Limited Clinical Data & Long-Term Outcomes: Lack of robust comparative data may slow physician adoption of newer treatments.
* Relapse & Treatment Resistance: High recurrence rates create uncertainty in therapeutic effectiveness.
* Awareness Gaps Outside the U.S. and EU: Limited infrastructure and knowledge in emerging markets may constrain growth geographically.

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

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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 Us

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