Living With Desmoid Tumors

Learn about desmoid tumors and find links to advocacy groups.

Facts & Stats on Desmoid Tumors

Explore the following information about desmoid tumors to help make informed decisions with your care team.

Desmoid tumors are dense, soft-tissue tumors. They can grow aggressively and affect nearby tissues and organs. A desmoid tumor is also called aggressive fibromatosis. Only 3% of soft tissue tumors are desmoid tumors. It is a rare tumor type.1-3

In the US, up to 1650 people are diagnosed with desmoid tumors every year.4-6

Women are more likely to be diagnosed with desmoid tumors, but men are at risk as well5,7,8

The majority of people with desmoid tumors are diagnosed in young adulthood5

Graphic stating that up to 77% of desmoid tumors may recur after surgery.

After surgery, desmoid tumors may come back—based on the tumor location and other factors8,9

Desmoid tumors can affect each person differently depending on where the tumor is in the body, how fast it grows, and other factors.

The risk of desmoid tumors rises for people with a family history of familial adenomatous polyposis (FAP).10 If a person has FAP, doctors may recommend a colectomy to help prevent colon cancer. However, for some, this surgery can increase the risk of developing desmoid tumors.3

Desmoid tumors can develop anywhere in the body where there is soft connective tissue.1 For example: tumors may form in tissues inside the abdomen (intra-abdominal), the chest, legs and feet, arms and hands, or the head and neck.3

Desmoid tumors do not spread to other parts of the body (metastasize). Although they are not malignant, they can cause serious health problems as they grow. They can affect and damage surrounding organs, blood vessels, and nerves.1

Desmoid tumor symptoms can vary based on where the tumor is located

A tumor within the abdominal area may cause digestive system blockages, pain, malaise, abdominal swelling, weight loss, or muscle wasting.3,11,12

Tumors in the arms or legs may cause limited movement, deformity, pain, tiredness, weakness, or muscle problems.3,13,14

Identifying progression

It’s important to know if your tumor is progressing, or getting worse.15 The NCCN* Guidelines for Patients® suggest starting treatment for desmoid tumors if:19

You’re experiencing symptoms

This tumor is affecting how you function

Scans show the tumor is growing

The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit alliance of leading cancer centers devoted to patient care, research, and education.

I went from doctor to doctor and specialist to specialist trying to find someone who could give me answers.
Christina, a desmoid tumor patient
Watch Christina’s full story and hear other patient voices.
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Individual results may vary.

Regularly monitoring and reporting your symptoms to your healthcare providers can help them to create a more personalized treatment plan

  • Listen to your body. New or changing symptoms, such as new lumps you can see or feel, or increased pain, could signal progression and may indicate it’s time to discuss a treatment adjustment2,15
  • Keep track of your symptoms. Track your symptoms with helpful tools like the OGSIVEO Treatment Tracker
  • Don’t delay. Don’t wait to talk to your doctor, advocate for yourself, and don’t miss your regular scans

Digital symptom trackers are also available

The OGSIVEO Digital Companion on the Medisafe app has features for logging desmoid tumor symptoms and allows you to get reminders for medications and doctor’s appointments to help you stay on track with your treatment.

There are also various health apps you can use to keep track of your symptoms on your smartphone such as Apple Health™. WebMD® also offers a tracking feature called Symptom Checker.

Apple App Store download icon, Google Play Store download icon
App Store download icon

These applications are provided for informational purposes only and do not represent an endorsement or a recommendation from SpringWorks Therapeutics, Inc. The applications listed are independent of SpringWorks and SpringWorks has no control over the content of these applications. The applications are linked to other websites that have different terms of use and privacy policies. Please refer to those applications and websites for the applicable terms of use and privacy policies.

Guideline Recommendation

The NCCN Guidelines for Patients recommend systemic therapy as a preferred treatment option for desmoid tumors that are growing and/or causing symptoms.19

Nirogacestat (OGSIVEO) is recommended by the NCCN Guidelines for Patients as a treatment option for desmoid tumors that are growing and/or causing symptoms.19

The National Comprehensive Cancer Network® (NCCN®) is a not-for-profit alliance of leading cancer centers devoted to patient care, research and education.

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Frequently Asked Questions

How are desmoid tumors diagnosed?

Your doctor may perform a physical exam and ask about your medical and family history.
These tests can also help with diagnosis:

  • Biopsy: Typically, a tiny sample of the tumor tissue is taken out with a needle and analyzed by a pathologist to confirm if it is a desmoid tumor16,17
  • Imaging Scans (CT or MRI16,17 ) can also help your doctor see the location and size of the tumor
Can desmoid tumors be mistaken for other conditions?

Because they are so rare, desmoid tumors may sometimes be misdiagnosed as:7,18

  • Hypertrophic scars or keloids
  • Low-grade sarcomas or gastrointestinal stromal tumor (GIST)
  • Benign growths such as nodular fasciitis, lipoma, fibroma, schwannoma, or leiomyoma
Where can I learn more about desmoid tumors?

You may ask your doctor or care team for additional information. Explore online resources through advocacy groups and communities focused on desmoid tumor education, including:

For more information, the National Comprehensive Cancer Network® (NCCN®) also has helpful resources, including the NCCN Guidelines for Patients®: Soft Tissue Sarcoma.19

SpringWorks Therapeutics, Inc. is providing these links to help patients find more information about desmoid tumors, but inclusion on this list does not represent an endorsement or a recommendation from SpringWorks for any group or organization. The organizations listed are independent of SpringWorks Therapeutics.

Which healthcare professionals can help with treating or managing desmoid tumors?

Your care team may include desmoid tumor specialists, such as:19,20

  • Medical oncologist
  • Surgeon, orthopedic or surgical oncologist
  • Radiation oncologist
  • Pathologist

Your care team may also include nurses, nurse practitioners, and physician assistants.

Mental health and other healthcare professionals may also be involved in your care.20

If a local desmoid tumor specialist isn’t available, they may still be able to collaborate with a local community oncologist to manage your care closer to home.19

A multidisciplinary care team can help you:21

  • Decide on your individualized care plan based on the advice of multiple healthcare professionals, including a desmoid tumor specialist or expert
  • Coordinate your care for a faster time to treatment
  • Use the team’s shared knowledge to help make informed treatment decisions

Sarcoma Centers

You can find a registry of sarcoma centers on the website for Sarcoma Alliance for Research through Collaboration (SARC), a non-profit organization.*

SARC is independent of SpringWorks Therapeutics, Inc. SpringWorks Therapeutics is providing this resource to help patients find healthcare professionals by location who have experience treating adults with desmoid tumors. Inclusion of a healthcare setting in this registry does not represent an endorsement, referral, or recommendation from SpringWorks Therapeutics and is not intended as medical advice. The healthcare professionals or institutions included in this locator directory do not necessarily prescribe or endorse any SpringWorks Therapeutics products. This registry is not comprehensive and other providers with experience treating adults with desmoid tumors may be available.

Find a Center

Advocacy Groups

The following advocacy organizations can provide additional support and information for patients with desmoid tumors

The Desmoid Tumor Research Foundation logo
The Desmoid Tumor Research Foundation (DTRF)
Rein in Sarcoma logo
Rein in Sarcoma
Sarcoma Alliance for Research through Collaboration (SARC) logo
Sarcoma Alliance for Research through Collaboration (SARC)
NCCN logo
NCCN Guidelines for Patients®
Northwest Sarcoma Foundation logo
Northwest Sarcoma Foundation
Sarcoma Foundation of America
Sarcoma Foundation of America
National Organization for Rare Disorders (NORD) logo
National Organization for Rare Disorders (NORD)
Global Genes logo
Global Genes

The organizations listed are independent of SpringWorks Therapeutics. SpringWorks Therapeutics, Inc. is providing these links to help patients find more information about desmoid tumors, but inclusion on this list does not represent an endorsement or a recommendation from SpringWorks for any group or organization.

Glossary

What is Ogsiveo?
Important Safety Information

What is OGSIVEO?  

OGSIVEO is a prescription medication used to treat adults with progressing desmoid tumors who require a medicine by mouth or injection (systemic therapy). It is not known if OGSIVEO is safe and effective in children.

Important Safety Information  

Before taking OGSIVEO tell your healthcare provider about all of your medical conditions, including if you:

  • Have liver problems.
  • Are pregnant or plan to become pregnant. OGSIVEO can harm your unborn baby. Tell your healthcare provider if you become pregnant or think you may be pregnant during treatment.
  • Females who are able to become pregnant:
    • Your healthcare provider will give you a pregnancy test before you start treatment with OGSIVEO.
    • You should use effective birth control during treatment and for 1 week after the last dose. Talk to your healthcare provider about methods that may be right for you.
    • Stop taking OGSIVEO and tell your healthcare provider right away if you become pregnant.
  • Males with female partners who are able to become pregnant should use effective birth control during treatment with OGSIVEO and for 1 week after the last dose.
  • Are breastfeeding or plan to breastfeed. It is not known if OGSIVEO passes into your breast milk. Do not breastfeed during treatment with OGSIVEO and for 1 week after the last dose.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

You should avoid taking proton pump inhibitors (PPIs) and H2 blockers during treatment with OGSIVEO. Ask your healthcare provider if you are not sure if you take one of these medicines. 

How should I take OGSIVEO? 

  • Take OGSIVEO exactly as your healthcare provider tells you to take it.
  • Your healthcare provider may change your dose, temporarily stop, or permanently stop treatment with OGSIVEO if you develop side effects.
  • Take OGSIVEO 2 times a day with or without food.
  • Swallow OGSIVEO tablets whole. Do not break, crush, or chew.
  • If you take an antacid medicine, take OGSIVEO 2 hours before or 2 hours after the antacid.
  • If you vomit after taking a dose or miss a dose of OGSIVEO, take your next dose at your regular time. Do not take 2 doses of OGSIVEO to make up the dose.

What should I avoid while taking OGSIVEO? 

Avoid eating or drinking grapefruit products, Seville oranges, and starfruit during treatment with OGSIVEO.  

What are the possible side effects of OGSIVEO? 

OGSIVEO can cause serious side effects, including:

  • Diarrhea. Diarrhea is common with OGSIVEO and may sometimes be severe. Your healthcare provider may tell you to drink more fluids or to take antidiarrheal medicines. Tell your healthcare provider right away if you have diarrhea that lasts longer than a few days and does not get better after taking antidiarrheal medicines.
  • Ovarian problems. Females who are able to become pregnant may have ovarian problems and changes in their menstrual cycle during treatment. OGSIVEO may affect fertility which may affect your ability to have a child. Tell your healthcare provider if you have any changes in your menstrual cycle or hot flashes, night sweats, or vaginal dryness during treatment.
  • Liver problems. OGSIVEO can increase liver enzymes. Your healthcare provider will do blood tests to check your liver function before you start and during treatment with OGSIVEO.
  • New non-melanoma skin cancers. Your healthcare provider will do skin exams before and during treatment with OGSIVEO if you are at risk for skin cancer. Tell your healthcare provider if you have any new or changing skin lesions.
  • Electrolyte (salt) problems. Your healthcare provider will do blood tests to check your phosphate and potassium levels during treatment and may give you medicines to treat low phosphate or low potassium if needed. Tell your healthcare provider if you develop any muscle pain or weakness.

Tell your healthcare provider right away if you have any side effect that bothers you or that does not go away. Your healthcare provider may change your dose, temporarily stop, or permanently stop treatment with OGSIVEO.  

The most common side effects of OGSIVEO are: 

  • rash 
  • nausea 
  • tiredness 
  • mouth sores 
  • headache 
  • stomach (abdominal) pain 
  • cough 
  • hair loss 
  • upper respiratory infection 
  • shortness of breath 

OGSIVEO can affect fertility in females and males, which may affect your ability to have a child. Talk to your healthcare provider if this is a concern for you.
These are not all of the possible side effects of OGSIVEO. Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Please see full Prescribing Information and Patient Information for OGSIVEO.

What is OGSIVEO?  

OGSIVEO is a prescription medication used to treat adults with progressing desmoid tumors who require a medicine by mouth or injection (systemic therapy). It is not known if OGSIVEO is safe and effective in children.

Please see full Prescribing Information and Patient Information for OGSIVEO.

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