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Desmoid Tumor Education

Refresh your knowledge about desmoid tumors, and find links to key advocacy groups.

Facts & Stats on Desmoid Tumors

Knowledge of desmoid tumors may help you move forward and make informed decisions with your doctor.

Desmoid tumors are dense, soft-tissue tumors. They can grow aggressively and affect nearby tissues and organs. They can often return, even after surgery or other treatments. 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

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.

Family tree Icon

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.11

Human body icon showing locations of desmoid tumors

Desmoid tumors can develop anywhere in the body where there is soft, connective tissue.2 For example: tumors may form in tissues within the abdominal area, the chest, legs and feet, arms and hands, or the head and neck.

Desmoid tumor damaging surrounding areas icon

Desmoid tumors do not spread to other parts of the body (metastasize). However, even if they are not malignant, they can cause serious health problems as they grow. They can affect and damage surrounding organs, blood vessels, and nerves.1,2

Human body in pain icon

Desmoid tumor symptoms can vary based on where the tumor is located. For example: A tumor within the abdominal area may cause digestive system blockages, pain, malaise, abdominal swelling, weight loss, or muscle wasting.11-13 Tumors in the arms or legs may cause limited movement, deformity, pain, tiredness, weakness or muscle problems.11,14,15

How can I track my symptoms?

There are various health apps, such as Apple HealthTM, you can use to keep track of your symptoms on your smart phone. WebMD® also offers a tracking feature called Symptom Checker. Monitoring your symptoms regularly and reporting them to your healthcare provider may help track your progress and manage your care plan.

These applications are provided for informational purposes only and do not represent an endorsement or a recommendation from SpringWorks Therapeutics. 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.

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 tumor18
Can desmoid tumors be mistaken for other medical conditions?

Because they are so rare, desmoid tumors may sometimes be misdiagnosed as7,19:

  • Hypertrophic scars or keloids
  • Low-grade sarcomas or gastrointestinal stromal tumor (GIST)
  • Benign growths such as nodular fasciitis, lipoma, fibroma, schwannoma, or leiomyoma
Which healthcare professionals can help with treating or managing desmoid tumors?

The care team for a person with a desmoid tumor may include:20,21

  • Medical oncologist
  • Sarcoma specialist
  • Surgeon or surgical oncologist
  • Radiologist
  • Radiation oncologist
  • Pathologist
  • Nurse, nurse practitioner, and physician assistant

Mental health and other healthcare professionals may also be involved in your care.21

A multidisciplinary care team can help you:22

  • 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
Where can I learn more about desmoid tumors?

For more information about desmoid tumors, the National Comprehensive Cancer Network® (NCCN®) has helpful resources. Find the NCCN Guidelines for Patients®: Soft Tissue Sarcoma at NCCN.org.

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

SpringWorks Therapeutics 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.

Advocacy Organizations for Information and Support

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

SpringWorks Therapeutics 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, Inc.

Glossary

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 click here for full Prescribing Information including Patient Information.

Important Safety Information

References
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  2. NIH. National Cancer Institute Center for Cancer Research. Desmoid tumor. Accessed October 16, 2023. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoid-tumor
  3. Kasper B, Baumgarten C, Garcia J, et al. Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG). Ann Oncol. 2017;28(10):2399-2408.
  4. Orphanet Report Series: Rare Diseases Collection. Prevalence and incidence of rare diseases: bibliographic data. Number 1, January 2022. Accessed October 16, 2023. https://www.orpha.net/orphacom/cahiers/docs/GB/ Prevalence_of_rare_diseases_by_alphabetical_list.pdf.
  5. van Broekhoven DLM, Grünhagen DJ, den Bakker MA, van Dalen T, Verhoef C. Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: population-based study. Ann Surg Oncol. 2015;22(9):2817-2823.
  6. U.S. Department of Commerce. News Blog. U.S. population estimated at 332,403,650 on Jan. 1, 2022. Accessed October 16, 2023. https://www.commerce.gov/news/blog/2022/01/us-population-estimated-332403650-jan-1-2022
  7. Penel N, Coindre JM, Bonvalot S, et al. Management of desmoid tumours: nationwide survey of labelled reference centre networks in France. Eur Cancer. 2016;58:90-96.
  8. Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964.
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  12. Shinagare AB, Ramaiya NH, Jagannathan JP, et al. to of desmoid tumors. AJR Am Roentgenol. 2011;197(6):W1008-W1014.
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  16. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.2.2023. ©National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed October 16, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.
  17. Gronchi A, et al. Desmoid Tumor Working Group. The management of desmoid tumours: joint global consensus-based guideline approach for adult and paediatric patients [supplementary appendix]. Eur Cancer. 2020;127:96-107.
  18. Cancer.Net. Desmoid tumor: diagnosis. Accessed October 16, 2023. https://www.cancer.net/cancer-types/desmoid-tumor/diagnosis
  19. Huss S, Nehles J, Binot E, Wardelmann E, et al. b-Catenin (CTNNB1) mutations and clinicopathological features of mesenteric desmoid-type fibromatosis. Histopathology. 2013;62:294-304. doi: 10.1111/j.1365-2559.2012.04355.
  20. Referenced with permission from the NCCN Guidelines for Patients® for Soft Tissue Sarcoma, 2023. ©National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed October 16, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use, or application, and disclaims any responsibility for their application or use in any way.
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