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The Desmoid Tumor Research Foundation

2026 Lay Summary of Global Consensus Paper

Initiatives

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As a member group of the Sarcoma Patient Advocacy Global Network (SPAGN), the DTRF is excited to share the new 2026 Lay Summary of the Global Consensus Paper on the management of desmoid tumors, originally published in JAMA Oncology in 2024.

While the scientific article (only accessible with a JAMA Oncology subscription) was written for clinicians and researchers, the new lay summary translates its key messages into clear, accessible language for patients, families, and advocates.

Highly technical medical publications are essential for advancing care, but they are often difficult for non-specialists to interpret. Creating a lay summary helps bridge this gap by explaining complex medical information in a way that supports better understanding, informed conversations with healthcare teams, and stronger patient advocacy.

We are grateful to SPAGN for their dedication and collaboration on this impactful resource. Special thanks to DTRF Medical Advisory Board Member, Professor Dr. Bernd Kasper for his careful review and commitment to finalizing this invaluable tool for the desmoid community.

At the DTRF, we encourage patients and caregivers to review, download, and share this important document with your medical care team. 

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Last year, we came together to correct a critical misconception that desmoid tumors are NOT benign. This year, we are building on that momentum to go one step further and make sure the correct classification of desmoid tumors is front and center.

The medical community, research publications, and other sources often use differing terms when describing desmoid tumors. This confusion can lead to misunderstandings about treatment and minimizes the potentially serious consequences of this rare disease.

How are desmoid tumors classified?
Desmoid tumors (also known as aggressive fibromatosis, desmoid fibromatosis, and desmoid-type fibromatosis) do not metastasize. They are classified by the World Health Organization (WHO) as  “Intermediate (locally aggressive)” soft tissue tumors. They do not fall under “Benign” or “Malignant” classifications.

Learn more about the World Health Organization’s (WHO) classification of desmoid tumors, which is listed under Table II: Fibroblastic/Myofibroblastic Tumours.

How can you help Spread the Word?

  • Engage with us by sharing our all the campaign content with your social media networks.
  • Watch a recording of our Spread the Word educational webinar: “How Desmoid Tumors are Classified and Why It Matters for Patients.”
  • Amplify patient voices by watching and sharing our live event recording—featuring desmoid warrior, Morgan B.
  • Educate your community by sharing trusted resources from DTRF.org.
  • Learn more by visiting our “What is a desmoid tumor?” page.
  • Contact our team if your current medical institution or clinician uses an incorrect classification on their website or within their practice.
  • Support our mission by making a donation!

Join us today. Use your voice and help us Spread the Word on behalf of the desmoid patient community!

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A growing national conversation around the urgent needs of rare disease patients is shining a spotlight on the realities desmoid tumor patients know all too well: research moves slowly, but desmoid tumors don’t.

In a recent Newsweek article titled “Research Moves Slowly. Rare Diseases Don’t—So Patients Aren’t Waiting,” rare disease advocates shared how systemic barriers delay progress, while patients face aggressive, life-altering conditions with limited treatment options. Desmoid tumors—classified by the World Health Organization as “intermediate (locally aggressive)” soft tissue tumors—fit this reality exactly. They don’t metastasize, but they can be debilitating, invasive, and unpredictable.

The Desmoid Tumor Research Foundation (DTRF) has long been at the forefront of disrupting this status quo. Founded in 2005, DTRF is the leading foundation in the world focused on desmoid tumors, advancing innovative research and uniting a global network of patients, clinicians, and scientists.

From launching the only virtual tumor board dedicated to desmoid tumor cases, to funding high-impact translational research, and advocating for drug development—DTRF’s efforts reflect the urgency of the disease and the resilience of its community.

As the rare disease space evolves, DTRF continues to build momentum. The Foundation invites patients, researchers, clinicians, and industry partners to join its mission and help close the gap between diagnosis and cure.

Read Full Article

Media Inquiries

Chaney Pope
Communications Manager, DTRF
chaney@DTRF.org

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Become a DTRF Ambassador! DTRF Ambassadors are volunteers who extend the ability of the Desmoid Tumor Research Foundation (DTRF) to serve patients and caregivers across the U.S., and help to strengthen and support the desmoid tumor patient population in their local communities.

NOTE:  After a program agreement, ambassadors will begin the onboarding process with a DTRF team member. The following list of responsibilities may change based on the needs of the DTRF community.

Responsibilities can include:

Patient and Caregiver Support
  • Using your experience with desmoid tumors to provide empathy and compassion for others on their journey
  • Connect patients and caregivers with local support organizations, healthcare professionals, and resources* that can assist in their desmoid tumor journey

*DTRF does not directly endorse, support, or recommend any products or services.

Educational Materials and Resources
  • Become educated about the programs and resources the DTRF makes available to the desmoid tumor community 
  • Engage virtually (or in-person) with patients by sharing DTRF resources, services and educational materials 
  • Gather information on local helpful patient resources to contribute web content to the DTRF knowledgebase
Medical Outreach
  • Assist DTRF staff in promoting and distributing educational materials to doctors and medical centers
  • Identify local doctors treating desmoid tumors for possible inclusion in the DTRF’s Doctor Directory (internal document)
  • Encourage local doctors and medical centers to refer desmoid tumor patients to the DTRF for information and support

Optional activities: 

  • Host local or regional informal meet-ups for patients and caregivers
  • Work together with DTRF staff to plan regional community conferences
  • Identify potential funders or major donors who may be interested in supporting the DTRF’s mission

Expectations

  • Take initiative to become educated about the programs and resources the DTRF makes available to the desmoid tumor community.
  • Maintain regular contact with a designated DTRF staff member, and keep them informed of activities performed on behalf of DTRF.
  • Respect the privacy of patients and caregivers; do not share patient identities or health information with anyone other than your designated DTRF staff contact.
  • Clearly represent your role as a volunteer for the DTRF when communicating in your capacity as an Ambassador.
  • Never attempt to give medical advice or opinions on how a patient should be treated; instead, share facts or educational resources and allow them to make their own decisions.
  • Act in the best interest of the DTRF by operating with integrity and high ethical standards, using your best judgement to avoid any activities that could create risk or reputational harm to the Foundation.
  • Proactively disclose to DTRF staff any potential personal or professional conflicts of interest. 
  • Maintain confidentiality of non-public information obtained from the DTRF.
  • Willingly accept training, coaching, and feedback from DTRF staff.

Support

The DTRF staff will support and direct the work of Ambassadors in the following ways:

  • Work with Ambassadors to identify activities based on the Foundation’s goals as well as the needs and opportunities of the Ambassador’s local community
  • Provide DTRF materials and collateral as needed
  • Provide training and oversight appropriate to the Ambassador’s activities
  • Provide introductions to patients, families, and medical professionals, as appropriate
  • Handle logistics of DTRF professional meetings, as needed

To learn more or to express interest in joining the program, please contact info@dtrf.org. We look forward to hearing from you!

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At the DTRF, we want to “Spread the Word” far and wide that desmoid tumors are NOT benign.

The medical community, research publications, and other sources often use differing terms when describing desmoid tumors; one of those terms is the word “benign.” Over the years, we’ve heard countless stories that this is confusing to patients as well as clinicians. This confusion can lead to misunderstandings about treatment and minimizes the potentially serious consequences of this rare disease. 

How are desmoid tumors classified?
Desmoid tumors (also known as aggressive fibromatosis, desmoid fibromatosis, and desmoid-type fibromatosis) do not metastasize. They are classified by the World Health Organization (WHO) as  “Intermediate (locally aggressive)” soft tissue tumors. They do not fall under “Benign” or “Malignant” classifications.

Learn more about the World Health Organization’s (WHO) classification of desmoid tumors, which is listed under Table II: Fibroblastic/Myofibroblastic Tumours.

To make a significant impact on behalf of the patient community, we invite you to connect with the DTRF, share our social content, and spread the word that desmoid tumors are NOT benign.

Thank you for helping raise awareness and educate the community about the classification of desmoid tumors!

Watch the Spread the Word Facebook Live conversation with desmoid tumor patient, Amy Beitz.

Watch the Spread the Word Webinar, “Understanding the Classification of Desmoid Tumors.”

View and follow our campaign posts on Facebook.

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(Pittsburgh, PA) – November 27, 2023 – The U.S. Food and Drug Administration (FDA) announced approval today of OGSIVEO (nirogacestat) for the treatment of adults with progressing desmoid tumors. This is the first FDA-approved medical therapy in this rare disease. FDA approval follows positive results from the SpringWorks Therapeutics, Inc. (Nasdaq: SWTX) DeFi (NCT03785964) phase 3 clinical trial, a global, randomized, double-blind, placebo-controlled trial evaluating nirogacestat in adult patients with desmoid tumors. Results from the DeFi trial were published on March 9, 2023, in The New England Journal of Medicine in the article, “Nirogacesat, a γ-Secretase Inhibitor for Desmoid Tumors,” by Drs. Gounder, Ratan, Kummar, Kasper et al.

OGSIVEO (nirogacestat) is an oral gamma secretase inhibitor. For more information, visit www.ogsiveo.com.

“The significance of FDA approval of OGSIVEO (nirogacestat) to desmoid tumor patients cannot be overstated. This is an extraordinary victory in the area of rare diseases, and demonstrates the power of collaboration among patients, industry and researchers.”

Jeanne Whiting, Executive Director Emeritus, Desmoid Tumor Research Foundation

“SpringWorks is grateful to the Desmoid Tumor Research Foundation, as well as the patients and investigators who participated in our trials, whose enduring commitment and enthusiasm helped us get to this point. We are honored to provide the first FDA-approved therapy and committed to offering ongoing support to the desmoid tumor community throughout their treatment journey on OGSIVEO.”

Saqib Islam, Chief Executive Officer, SpringWorks Therapeutics

The collaborative path to approval

The Desmoid Tumor Research Foundation (DTRF) has been instrumental in the nearly 15-year path to approval of OGSIVEO (nirogacestat). The drug was first studied in patients with a range of solid tumors including seven desmoid tumor patients. A poster presentation of the promising results of this early work in desmoid tumors caught the eye of members of the National Cancer Institute’s (NCI) Rare Tumors Initiative, Shivaani Kummar, MD and Abby Sandler, PhD. The pair were interested in a phase 2 trial, but due to the rare nature of the disease, concluded that in order to be successful they needed the support of a patient advocacy organization. Synergistic collaboration between the DTRF and NCI brought the phase 2 trial forward, and with the DTRF’s outreach efforts, filled it in less than five months. 

Despite promising results that were coming out of the phase 2 study, a business decision resulted in halting the development of the asset. Determined not to let this potential treatment stall, the DTRF once again galvanized its resources and continued discussions with Pfizer staff. Supportive clinicians, as well as encouraging staff at Pfizer, supported the effort to move the drug forward. Eventually SpringWorks Therapeutics was created to license nirogacestat and other assets that showed potential in underserved patient populations.

Under newly-formed SpringWorks, the phase 3 DeFi trial for nirogacestat opened. During the conduct of the DeFi trial, and with the help of DTRF’s patient outreach, the trial was fully enrolled in July 2020. In September 2022, trial data were presented in a Presidential Symposium during the ESMO Congress and at the annual DTRF International Desmoid Tumor Research Workshop. In December 2022, SpringWorks filed a New Drug Application for nirogacestat which was accepted for Priority Review on February 27, 2023. Today, FDA approval of OGSIVEO (nirogacestat), makes it the first FDA-approved drug for the treatment of adults with desmoid tumors.

“This breakthrough represents the power of patient advocacy. The Desmoid Tumor Research Foundation’s role in OGSIVEO’s path to approval represents collaboration at its finest. Were it not for their advocacy work to shepherd the drug through the trial phases, facilitate communication among stakeholders, and use their outreach to help fill the trial, we may not be here today. I’m honored to be part of the process that brings the first-ever FDA-approved medical therapy to desmoid tumor patients.”

Mrinal Gounder, MD, Memorial Sloan Kettering Cancer Center

About desmoid tumors

Desmoid tumors (also known as aggressive fibromatosis) are rare, invasive, soft tissue tumors that form in the connective tissues of the body. They affect 5-6 per 1 million people per year. Although they do not metastasize, desmoid tumors can be locally aggressive and severely damage surrounding tissues and vital structures as they grow. They are highly recurrent and can be multifocal. These tumors may occur anywhere in the body (abdominal, intraabdominal, and extra-abdominal locations) and approximately 5-10% arise in the context of a rare genetic condition known as Familial Adenomatous Polyposis (F.A.P.). Previously, there have been no approved therapies for desmoid tumors.

About The Desmoid Tumor Research Foundation

The Desmoid Tumor Research Foundation (DTRF) was co-founded in 2005 by Jeanne Whiting and Marlene Portnoy, with the mission of facilitating and funding desmoid tumor research and supporting patients with information. The DTRF’s mission is to aggressively fund research to accelerate the development of improved therapies, and ultimately find a cure for desmoid tumors. The DTRF collaborates with dedicated researchers and clinicians worldwide to facilitate research and improve the lives of patients through education, awareness and support. Every year the foundation’s progress has gained momentum with new clinical trials, emerging therapies and important new discoveries about the disease coming to fruition. The DTRF is a recipient of the National Organization of Rare Disorders Abby S. Meyers Leadership Rare Impact Award, and the Global Genes’ Champions of Hope Rare Champion in Advocacy Award. For more information, please visit www.DTRF.org.

For more information about this important milestone for desmoid tumor patients, read the FDA’s and SpringWorks’ press releases linked below.

SpringWorks Therapeutics Press Release

U.S. FDA Press Release

DTRF Press Release PDF

Media Inquiries

Maggie Cameron
Communications Manager, DTRF
Maggie@DTRF.org