Desmoid Tumor Ally Educational Program

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Care options for desmoid tumors

desmoid tumor tendril

A multidisciplinary team can help you determine your treatment goals

Once an accurate diagnosis has been obtained, there are several treatment approaches that may be recommended depending on the size, location, aggressiveness, and discomfort of the tumor among other factors.1,2

An open, honest discussion about what your treatment goals are can be helpful in developing your care plan. It is recommended that your care team include specialists across different fields of medicine who have experience managing your condition.2 Desmoid tumors are rare—so most healthcare providers may not have experience with diagnosing or treating this type of tumor.3

A first step to finding experienced providers may be to find a sarcoma center.* If there is not a sarcoma center close to you, it may be possible for your local doctors to work with desmoid tumor experts to create a treatment plan for you.3

SpringWorks Therapeutics is providing this link to help patients find a sarcoma specialist by region, but SpringWorks had no role in developing this list and inclusion on this list does not represent an endorsement or a recommendation from SpringWorks for any center or physician.

Andrea’s Care Team

Hear about Andrea’s experience—She found a care team she trusts, with professionals who listen to her and make her feel supported in her desmoid tumor journey.

Andrea, a real person living with a desmoid tumor

When medical experts from different specialties are able to share knowledge, together they can cover the different aspects of your care. Your care team may include:3

  • Medical oncologist
  • Sarcoma specialist
  • Surgeon/surgical oncologist
  • Radiologist and/or radiation oncologist
  • Pathologist
  • Nurses

Your primary care physician, mental health professionals, and other healthcare professionals may be involved in your continuing care.4

Care Options Guide

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Progression and thinking beyond your scans

Progression is a word used to describe how a medical condition advances, or gets worse.5
An increase in the size of the tumor, as seen in a CT scan or MRI, may be one way to see whether a desmoid tumor is progressing.2,6

There may be other ways you can track the impact of a desmoid tumor on your health that are just as important. You don’t have to wait until the next scans are scheduled. You can take the lead and let your doctor and care team know how you feel:

  • Pain levels.7-9 Are you experiencing more pain day to day? Is the pain getting worse? Are you feeling pain in other parts of your body?
  • A change in symptoms.2,6 Are there new symptoms you are experiencing? Or have certain symptoms gotten worse?
  • Effect on your daily activities.2,6 Are some simple activities or movements becoming more challenging for you? Is your ability to work affected?

When a desmoid tumor progresses, managing pain may become more of a concern. Let your care team know about any pain you experience. They can recommend options for pain relief or refer you to a doctor who specializes in pain management.10 One way to help your care team understand your needs is to track your symptoms.

“For anybody who’s diagnosed now: please research all of your treatment options.”

DeAnn, a real person living with a desmoid tumor

Current care options for desmoid tumors

In the latest NCCN Guidelines for Patients®, desmoid tumor experts no longer recommend surgery as the first option to treat most desmoid tumors. Other care options may be considered first based on the location of the tumor and other factors.3

Results and side effects can vary from person to person. It may be helpful to talk about what to expect with your care team. Together, you and your care team can come up with strategies to help handle side effects if you experience them.

Active Surveillance

The first step for some people may be observation (“watch and wait”). This may include regular doctor check-ups and frequent imaging tests.2 This will give doctors a chance to understand if your symptoms are changing and see if or how fast the tumor is growing.11-13

If the tumor grows quickly, symptoms get worse, or if the tumor is in a critical location, your doctor may decide to begin treatment.2

Medical Therapy

Medical therapy, also known as systemic therapy, describes treatment with medicines that travel through the bloodstream and can affect cells throughout the body.14 Systemic therapy may be given as pills or IV infusions.3

Currently, there are no FDA-approved treatment options indicated for desmoid tumors.15

In the NCCN Guidelines for Patients 2023, medical experts on desmoid tumors recommend the following systemic options:3

  • Tyrosine kinase inhibitors (TKIs, may include sorafenib, pazopanib, and imatinib)
  • Chemotherapy (options may include methotrexate and vinblastine/vinorelbine, doxorubicin-based regimens)
  • Nonsteroidal anti-inflammatory drugs (NSAIDs, for pain)

Antihormonal therapies (tamoxifen or toremifene, with or without NSAIDs) do not have guideline recommendations.2,6

A Medical Oncologist is often the leader of your care team. This expert specializes in treating tumors using systemic therapy. They can create a treatment plan tailored to your needs.3,16

Surgery

Guidelines from 2 highly respected groups of desmoid tumor experts* do not recommend surgery as the first option for treating desmoid tumors in most locations.3,6

A team of experts should agree on whether surgery is appropriate. They will assess the tumor size and location, and how symptoms may be affecting daily life.3 There are other important factors to consider:

  • During surgery, the surgeon tries to remove the entire desmoid tumor and some surrounding healthy tissue to make sure they removed it all. Doctors call this surrounding area a “clear margin” where no tumor cells are visible6
    • Unfortunately, even with a clear margin, sometimes desmoid tumors still come back (recurrence)17-20
  • Completely removing a desmoid tumor and getting a clear margin may be difficult. That’s because desmoid tumors have far-reaching “tendril-like” growths that radiate outward.21,22
    • If any part of the desmoid tumor is left after surgery (called a “positive margin”), there is a high risk of recurrence 19,22
  • Desmoid tumors can come back after surgery 24%-77% of the time.23,24 The risk may be higher for people who:
    • Are diagnosed at a younger age17
    • Have tumors in certain locations (intra-abdominal, chest wall or limbs)17
    • Have a larger tumor size17
    • Have already had a desmoid tumor come back after surgery23
  • There is a likelihood of additional surgeries for people who have had desmoid tumor surgery. For some, multiple procedures have led to short- or long-term problems with doing daily activities and symptoms such as pain, weakness, or decreased movement22,25

The National Comprehensive Cancer Network (NCCN) and The Desmoid Tumor Working Group (DTWG).

The Surgeon on your care team is the expert who removes desmoid tumors—if surgery is appropriate, based on tumor location and other factors.3

“Mine grows and it takes over. I have a whole bunch of empty spaces because they had to take out a bunch of muscle. I just wish that there was something that explained it easily.”

Amy, a real person living with a desmoid tumor

Knowledge is power

What questions should you ask your doctor? What are your options or next steps for desmoid tumor care? These are important aspects of making informed decisions for your health. These guides can help:

Doctor Discussion Guide

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Care Options Guide

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Radiation therapy

Radiation therapy is only recommended for desmoid tumors in the arms, legs, outer torso, head, and neck.3,9 Radiation therapy can be used where surgery would not be feasible and medications are not available.1

If radiation therapy is an option for you, a Radiation Oncologist is the expert who will prescribe and plan the therapy to treat your desmoid tumor.3

Cryoablation

Cryoablation uses extreme cold to destroy tumor tissue.3,26 It can be used for small to medium tumors that are not in the abdomen (belly).13 A cold gas freezes the tumor tissue. It is pumped into the tumor through a thin needle. The tissue thaws and the freezing and thawing are repeated several times to destroy the cells.26 There are other ablation therapies that your doctor may recommend as well.3

Clinical Trials

Treatments are being studied for desmoid tumors. Talk to your doctor to see if you may be a good candidate for a clinical trial.

“There’s no one-size-fits-all treatment”

Melony, a real person living with a desmoid tumor

  1. Gronchi A, et al. Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020;127:96-107.
  2. 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 August 25, 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.
  3. Referenced with permission from the NCCN Guidelines for Patients® for Soft Tissue Sarcoma 2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed July 24, 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.
  4. Cancer.Net. The oncology team. Accessed February 12, 2023. https://www.cancer.net/navigating-cancer-care/cancerbasics/cancer-care-team/oncology-team
  5. NCI (National Cancer Institute). Dictionary of cancer terms: progression. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/progression
  6. Gronchi A et al. Desmoid Tumor Working Group. The management of desmoid tumours: a joint global consensus-based guideline approach for adult and paediatric patients [supplementary appendix]. Eur J Cancer. 2020;127:96-107.
  7. Cuomo P, Scoccianti G, Schiavo A, et al. Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study. BMC Cancer. 2021;21:437.
  8. Penel N, Bonvalot S, Bimbai AM, et al. Pain in desmoid fibromatosis (DF) patient: prevalence, associated factors and prognosis. Presented at the European Society of Medical Oncology Annual Meeting, 2022. Abstract 1512P.
  9. Quintini C, Ward G, Shatnawei A, et al. Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis. Ann Surg. 2012;255:511-516.
  10. NCI (National Cancer Institute). Pain management for people with cancer. Accessed August 8, 2023. https://www.cancer.gov/about-cancer/treatment/side-effects/pain
  11. Cancer.Net. Desmoid tumor: types of treatment. Accessed February 12, 2023. https://www.cancer.net/cancer-types/desmoid-tumor/types-treatment
  12. Kasper B, Baumgarten C, Bonvalot S, et al. Desmoid Working Group. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise—a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer. 2015;51(2):127-136.
  13. Kasper B, Baumgarten C, Garcia J, et al. Desmoid Working Group. An update on the management of sporadic desmoid-type fibromatosis: a 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.
  14. NCI (National Cancer Institute). Dictionary of cancer terms: systemic therapy. Accessed July 24, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/systemic-therapy
  15. Riedel RF. Agulnik M. Evolving strategies for management of desmoid tumor. Cancer. 2022;128(16):3027-3040.
  16. Cancer.Net Types of oncologists. Accessed February 12, 2023. https://www.cancer.net/navigating-cancer-care/cancer-basics/cancer-care-team/types-oncologists
  17. Crago AM, Denton B, Salas S, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. 2013;258(2):347-353.
  18. Peng PD, Hyder O, Mavros MN, et al. Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients. Ann Surg Oncol. 2012;19(13):4036-4042.
  19. Janssen ML, van Broekhoven DL, Cates JM, et al. Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis. Br J Surg. 2017;104(4):347-357.
  20. Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer. 2000;88(7):1517-1523. 
  21. Ballo MT, Zagars GK, Pollack A, Pisters PWT, Pollock RA. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17(1):158-167.
  22. Tsagozis P, Stevenson JD, Grimer R, Carter S. Outcome of surgery for primary and recurrent desmoid-type fibromatosis. A retrospective case series of 174 patients. Ann Med Surg (Lond). 2017;17:14-19.
  23. Easter DW, Halasz NA. Recent trends in the management of desmoid tumors. Summary of 19 cases and review of the literature. Ann Surg. 1989;210(6)765-769.
  24. Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964.
  25. Fernandez M, Bell T, Tumminello B, Khan S, Zhou S, Oton A. Disease and economic burden of surgery in desmoid tumors: a review. Expert Rev Pharmacoecon Outcomes Res. 2023;23(6):607-618. doi: 10.1080/14737167.2023.2203915
  26. NCI (National Cancer Institute). Dictionary of cancer terms: cryoablation. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/cryoablation