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Approaches to Patient Management

Guidelines recommend first-line systemic therapy for progressive or symptomatic disease
desmoid tumor tendril

Key Takeaways

  • Patients with desmoid tumors seek both disease control and symptom improvement
  • The Desmoid Tumor Working Group* Guideline recommends systemic treatment first-line instead of surgery for most tumor locations1
  • According to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®): In general, surgery is not considered a first-line treatment option for desmoid tumors, except in certain situations if agreed upon by a multidisciplinary tumor board2
  • Although they do not metastasize, desmoid tumors are associated with local recurrence rates ranging from 24% to 77% after surgical resection, regardless of margin status3,4,†
    • Desmoid tumor recurrence risk remains high even with clean margins: various independent studies report desmoid tumor recurrence rates after complete surgical resection of 15% to 30%.5-8 Some studies suggest that the majority of tumors recur within 5 years5,6,9
  • The NCCN Guidelines® and Desmoid Tumor Working Group consensus guideline recommend engaging a multidisciplinary care team with experience in desmoid tumors1,2

The Desmoid Tumor Working Group includes more than 50 sarcoma experts from different disciplines, patients, and patient advocates from Europe, North America, and Japan.1

Based on retrospective, observational data. Factors associated with local recurrence postsurgery include tumor location, age of the participant, tumor size, margin status, and prior recurrence.5,10

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Guidelines recommend consulting a desmoid tumor expert

The NCCN Guidelines and Desmoid Tumor Working Group* Guideline recommend an initial evaluation and management by a multidisciplinary team with expertise and experience in desmoid tumors.1,2 Consulting with desmoid tumor experts can help support more accurate patient assessment, the prompt initiation of treatment when appropriate, and individualized management of symptoms.11,12

Hear Andrea’s Story

She found a care team she trusts, with professionals who listen to her and make her feel supported in her desmoid tumor journey.

SpringWorks is providing this link to assist in finding a specialist by region, but had no role in developing the list and inclusion does not represent an endorsement or a recommendation from SpringWorks for any center or physician.

Management Options

NCCN Guidelines recommend that patients having tumors that are progressing, symptomatic, or impairing or threatening in function be offered therapy with the decision based on the location of the tumor and the potential morbidity of the therapeutic option.2 In addition to reduction of tumor growth, treatment considerations should include reducing symptoms and improving functioning and quality of life.12-14

Active Surveillance  (also known as “Watch and Wait”)

For patients who have desmoid tumors that are asymptomatic and not progressing or morbid, active surveillance may be an appropriate option.1 However, it is recommended that they stay in close contact with their multidisciplinary team along the way for intervention as needed.1

NCCN Guidelines recommend continued observation with MRI or CT scan. Imaging is recommended every 3 months, but optimal frequency depends on tumor location, risk of progression, and symptoms. More frequent imaging may be indicated in symptomatic patients.2

Desmoid tumor progression can be symptomatic as well as radiographic. In order to help improve patient outcomes, healthcare providers should assess for progression as early as possible with at least one of the following:

  • Tumor growth documented by MRI or CT1,2,15
  • Worsening of symptoms1,2,15
  • Worsening impact on daily living (eg, functional impairment)1,2,15

“Worsening symptoms may be a sign of disease progression, even in the absence of radiographic changes. I try to be vigilant in preserving quality of life for patients affected by desmoid tumors.”

Dr. Riedel, MD Medical Oncologist
Hear more from Dr. Riedel

Systemic Therapy

For most tumor locations, the Desmoid Tumor Working Group Guideline recommends systemic treatment first-line instead of surgery.1 Currently, there are no FDA-approved treatment options indicated for desmoid tumors.16 However, some systemic therapies are used off-label. NCCN Guidelines list agents and regimens with activity in desmoid tumors:2

  • Tyrosine kinase inhibitors (sorafenib [category 1]; imatinib, pazopanib [both category 2A])
  • Chemotherapy (methotrexate and vinblastine/vinorelbine, select doxorubicin-based regimens [all category 2A])
  • NSAIDs (sulindac or other NSAIDs, including celecoxib for pain [all category 2A])

Hear from a desmoid tumor expert oncologist

Desmoid Tumor Management for Medical Oncologists

Watch Dr. Riedel, medical oncologist from Duke Health and Duke Cancer Institute, discuss treatment guidelines, desmoid tumor pathophysiology, and currently available systemic therapies.

Surgery

According to the NCCN Guidelines: In general, surgery is not considered a first-line treatment option for desmoid tumors, except in certain situations if agreed upon by a multidisciplinary tumor board.2

Desmoid tumors are invasive and clear margins are challenging to achieve

Due to the infiltrative nature of desmoid tumors, clear margins can be difficult to achieve and may require extensive resection that can lead to additional morbidity.4,17-19

  • At 3 major US surgical centers, 40.1% (67/167) of patients with desmoid tumors had positive margins after undergoing surgery with curative intent6
  • In a meta-analysis of 894 patients with desmoid tumors, positive margins were found to increase the risk of local recurrence nearly 2-fold vs R0 margins7
Image adapted with permission from Foo W, Lazar AJ. Pathology of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012. Accessed August 2, 2023. https://doi.org/10.1007/978-94-007-1685-8_3. Arrows added.

Gross tumor specimen post-resection showing invasive tendrils (arrows)20

Recurrence is common after surgery for desmoid tumors

  • Although they do not metastasize, desmoid tumors are associated with local recurrence rates ranging from 24% to 77% after surgical resection, regardless of margin status3,4,§
    • Desmoid tumor recurrence risk remains high even with clean margins: various independent studies report desmoid tumor recurrence rates after complete surgical resection of 15% to 30%.5-8 Some studies suggest that the majority of tumors recur within 5 years5,6,9
  • Extremity, chest wall, and intra-abdominal tumors have the highest recurrence risk5
  • Younger patient age and larger tumor size can be associated with higher recurrence risk following complete resection5

Based on retrospective, observational data. Factors associated with local recurrence postsurgery include tumor location, age of the participant, tumor size, margin status, and prior recurrence.5,10

Gross tumor specimen post-resection showing ill defined borders (arrows)21

Image adapted with permission from Obeidin F, Alexiev B. Fibromatosis-desmoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosisdeep.html. Accessed August 2, 2023. Arrows added.

Surgery can cause additional morbidity for patients with desmoid tumors

Resection needed for clear margins is often large and may require radical surgery, which can lead to functional impairment or morbidity.5 More aggressive local treatment may be associated with poorer long-term functional outcomes.17,20,22,23

  • Data from a prospective soft tissue database showed that 12% (18/146) of patients who received surgery for desmoid tumors experienced complications requiring prolonged hospital stays and/or additional operations24
  • The economic burden of surgery—including potential complications, repeat surgeries, and amputation—should be considered25

Hear from a desmoid tumor expert surgeon

Desmoid Tumor Management for Surgeons

Watch Dr. Randall, orthopedic surgeon from the University of California Davis, review treatment guidelines and discuss important considerations relating to surgery for the management of desmoid tumors.

Locoregional Therapy

Radiation therapy, cryoablation, and high-frequency ultrasound are sometimes used to help treat desmoid tumors.1,2,26,27

The Desmoid Tumor Working Group Guideline does not recommend perioperative radiotherapy.1 Moderate-dose radiotherapy can be considered to provide local control for unresectable, progressive, extra-abdominal desmoid tumors if medical therapies are not available or not active.1,17,26 Caution should be exercised as to use of radiotherapy for abdominal wall tumors and in young patients given the risk of secondary malignancy.17,26

The utility of cryoablation is limited to small and moderately sized tumors in extra-abdominal locations.26

Clinical Trials

Enrollment in a clinical trial may be considered an option for appropriate patients.28

To help with the diagnosis and management of desmoid tumors, explore the additional resources, clinical papers, and downloadable discussion tips in the Resources section.

Next: Resources & Expert Videos

CT, computed tomography; FDA, Food and Drug Administration; MRI, magnetic resonance imaging; NCCN, National Comprehensive Cancer Network® (NCCN®); NSAIDs, nonsteroidal anti-inflammatory drugs.

  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 2, 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. 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. 
  4. Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964. 
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. Ballo MT, Zagars GK, Pollack A, et al. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999;17(1):158-167.
  10. 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.
  11. Joglekar SB, Rose PS, Sim F, Okuno S, Petersen I. Current perspectives on desmoid tumors: the Mayo Clinic approach. Cancers (Basel). 2011;3(3):3143-3155.
  12. Husson O, Younger E, Dunlop A, et al. Desmoid fibromatosis through the patients’ eyes: time to change the focus and organisation of care? Support Care Cancer. 2019;27(3):965-980.
  13. Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16(5):682-693.
  14. Timbergen MJM, van de Poll-Franse LV, Grünhagen DJ, et al. Identification and assessment of health-related quality of life issues in patients with sporadic desmoid-type fibromatosis: a literature review and focus group study. Qual Life Res. 2018;27(12):3097-3111.
  15. 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.
  16. Riedel RF, Agulnik M. Evolving strategies for management of desmoid tumor. Cancer. 2022;128(16):3027-3040.
  17. Bonvalot S, Desai A, Coppola S, et al. The treatment of desmoid tumors: a stepwise clinical approach. Ann Oncol. 2012;23(suppl 10):x158-x166.
  18. Lewis JJ, Boland PJ, Leung DHY, et al. The enigma of desmoid tumors. Ann Surg. 1999;229(6):866-872.
  19. Dafford K, Kim D, Nelson A, Kline D. Extraabdominal desmoid tumors. Neurosurg Focus. 2007;22(6):E21.
  20. Foo W, Lazar AJ. Pathology of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012. Accessed August 2, 2023. https://doi.org/10.1007/978-94-007-1685-8_3.
  21. Obeidin F, Alexiev B. Fibromatosis-desmoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosisdeep.html. Accessed August 2, 2023.
  22. Penel N, Cesne AL, Bonvalot S, et al. Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French Sarcoma Group. Eur J Cancer. 2017;83:125-131.
  23. Newman ET, Lans J, Kim J, et al. PROMIS function scores are lower in patients who underwent more aggressive local treatment for desmoid tumors. Clin Orthop Relat Res. 2020;478(3):563-577.
  24. Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25(13):1785-1791.
  25. Fernandez MM, Bell T, Tumminello B, et al. Disease and economic burden of surgery in desmoid tumors: a review. Expert Rev Pharmacoecon Outcomes Res. 2023;23(6):607-618.
  26. 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.
  27. Ghanouni P, Dobrotwir A, Bazzocchi A, et al. Magnetic resonance-guided focused ultrasound treatment of extra-abdominal desmoid tumors: a retrospective multicenter study. Eur Radiol. 2017;27(2):732-740.
  28. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;28(10):2399-2408.