Desmoid Tumor Ally Educational Program

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Care options for desmoid tumors

desmoid tumor tendril

Currently, there are no FDA-approved treatment options indicated for desmoid tumors.1 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.2,3

A multidisciplinary team can help you determine your treatment goals

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.3 You can ask your doctor about a multidisciplinary care team approach to determine the best treatment path for you.

Building your treatment plan? Start with good information.

There’s a lot to consider when making decisions about desmoid tumor treatment. This guide outlines current care options available, and how you and your multidisciplinary care team can work together to make decisions about the management of your desmoid tumor.

Discussion Guide

Download Care Options Guide

Progression and thinking beyond your scans

Progression is a word used to describe how a medical condition advances, or gets worse.4
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.3,5

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.6-8 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.3,5 Are there new symptoms you are experiencing? Or have certain symptoms gotten worse?
  • Effect on your daily activities.3,5 Are some simple activities or movements becoming more challenging for you? Is your ability to work affected?

There’s no one-size-fits-all treatment.

Melony, patient with a desmoid tumor

Melony smiling

Current care options for desmoid tumors

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 to “watch and wait.” This may include regular doctor check-ups and frequent imaging tests.3 This will give doctors a chance to understand if your symptoms are changing and see if or how fast the tumor is growing.9-11

If the tumor grows quickly, symptoms get worse, or the tumor is in a critical location, your doctor may decide to begin treatment.3

Medical Therapy

Currently, there are no FDA-approved treatment options indicated for desmoid tumors.1 However, your care team may prescribe medical therapies to help shrink and/or stabilize the tumor and manage symptoms. The recent clinical guidelines recommend the following treatment options:2,3

  • 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.2,3

Surgery

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 visible.2

Surgery used to be the most common treatment for desmoid tumors, but it is generally no longer recommended as a first approach in most tumor locations by desmoid tumor experts.2,11 This is because:

  • surgery may require the removal of large amounts of tissue11
  • desmoid tumors often come back after surgery (recurrence)12
  • desmoid tumors may remain stable or resolve on their own11

Desmoid tumors include the tumor itself and its “tendril-like” growths that radiate outward. These can be far-reaching. Because of this, completely removing the desmoid tumor and getting a clear margin can be difficult. Removing a large amount of tissue could cause some loss of function or changes in appearance.10,13

Desmoid tumors can come back after surgery. It has been reported that they come back about 24% to 77% of the time.14,15 Factors such as the location and size of the tumor, as well as the age of the patient, can influence risk of recurrence.16 So, surgery may not be the best option for everyone.2

Radiation therapy

Radiation therapy is only recommended for desmoid tumors in the arms, legs, outer torso, head, and neck.3,17 Radiation therapy can be used where surgery would not be feasible and medications are not available.2

Cryoablation

Cryoablation uses extreme cold to destroy tumor tissue.17,18 It can be used for small to medium tumors that are not in the abdomen (belly).11 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.17,18

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.

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

DeAnn, patient with a desmoid tumor

DeAnn on a medical exam table

  1. Riedel RF. Agulnik M. Evolving strategies for management of desmoid tumor. Cancer. 2022;128(16):3027-3040.
  2. 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.
  3. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.2.2022. © National Comprehensive Cancer Network, Inc. 2022. All rights reserved. Accessed February 12, 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. NCI (National Cancer Institute). Dictionary of cancer terms: progression. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/progression
  5. 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.
  6. Cuomo P, Scoccianti G, Schiavo A, et al. Extra-abdominal desmoid tumor fibromatosis: a multicenter EMSOS study. BMC Cancer. 2021;21:437.
  7. 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.
  8. 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.
  9. Cancer.Net. Desmoid tumor: types of treatment. Accessed February 12, 2023. https://www.cancer.net/cancer-types/desmoid-tumor/types-treatment
  10. 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.
  11. 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.
  12. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274.
  13. 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.
  14. 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.
  15. Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92)6):947-964.
  16. Crago AM, Denton B, Salas S, et al. A prognostic nomogram for prediction ofrecurrence in desmoid fibromatosis. Ann Surg. 2013;258(2):347-353.
  17. Referenced with permission from the NCCN Guidelines for Patients® for Soft Tissue Sarcoma V.1.2020. © National Comprehensive Cancer Network, Inc. 2020. All rights reserved. Accessed February 12, 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.
  18. NCI (National Cancer Institute). Dictionary of cancer terms: cryoablation. Accessed February 12, 2023. https://www.cancer.gov/publications/dictionaries/cancer-terms/def/cryoablation