Approaches to Patient Management

desmoid tumor tendril

Currently, there are no FDA-approved treatment options indicated for desmoid tumors.1 Management options include active surveillance, off-label systemic therapy options, surgery, and locoregional therapies.2

Clinical trials for your appropriate patients with unresectable desmoid tumors can be considered when assessing patient management options.3

A multidisciplinary team that includes specialists with expertise in treating desmoid tumors can help determine the best path forward.2,4,5 The NCCN Guidelines and Desmoid Tumor Working Group consensus guideline recommend engaging a multidisciplinary care team with expertise and experience in sarcoma.2,6

Important Management Considerations5

  • Morbidity
  • Tumor location
  • Pain
  • Functional limitation
  • Persistent progression of disease

Management Options

For patients who have desmoid tumors that are asymptomatic and not progressing or morbid, active surveillance may be an appropriate option.2 However, it is recommended that they stay in close contact with their multidisciplinary team along the way for intervention as needed.2

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.6

Currently, there are no FDA-approved treatment options indicated for desmoid tumors.1 However, there are off-label therapy options. The Desmoid Tumor Working Group does not propose a definitive sequence due to the lack of comparative studies.2 NCCN Guidelines list agents and regimens with activity in desmoid tumors:6

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

These regimens do not have an NCCN Guidelines recommendation:

  • Antihormonals +/- NSAIDs (tamoxifen with sulindac, or toremifene)

In most tumor locations, surgery is no longer the recommended first-line treatment option for desmoid tumors.2,7 While historically, surgery was the first-line management approach for desmoid tumors, active surveillance is now preferred and recommended by the Desmoid Tumor Working Group for initial management of all asymptomatic patients.2,7

When active treatment becomes necessary, the Desmoid Tumor Working Group recommends treatment modalities other than surgery if morbidity or positive microscopic margins can be anticipated.2 For abdominal wall desmoid tumors, surgery is still recommended as the first option in case of progression.2

Surgery is associated with local recurrence rates ranging from 24% to 77%, and due to the invasive nature of the tumor’s “tendril-like” growths, clear margins may be difficult to identify.8,9,*

High recurrence rates may be due to growth factors released during wound healing that promote beta-catenin activation.10,11

The tumor site can affect surgical outcomes. Surgery was associated with significantly worse outcomes compared to active surveillance in patients with desmoid tumors in the chest wall, head and neck, and upper limb.12

Resection needed for clear margins is often large and may require radical surgery, which can lead to functional impairment or morbidity.10 For 24% of patients with recurrent extremity desmoid tumors, amputation would be required to achieve definitive resection.13 Due to the invasive nature of desmoid tumors, complete resection is often not achieved.

Based on observational data, factors associated with local recurrence postsurgery include tumor location, age of the participant, and tumor size.14

Gross tumor specimens
Top image reproduced with permission from Foo W, Lazar AJ. Pathology of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012. Accessed October 25, 2022.

Bottom image reproduced with permission from Soft tissue; fibroblastic/myofibroblastic fibromatosis; fibromatosis-desmoid. Accessed October 25, 2022.

Gross tumor specimens post-resection showing invasive tendrils and ill-defined borders (arrows)15,16

Radiation therapy, cryoablation, and high-frequency ultrasound are sometimes used to help treat desmoid tumors.2,7,17

The Desmoid Tumor Working Group does not recommend perioperative radiotherapy.2 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.2,7,10 Caution should be exercised as to use of radiotherapy for abdominal wall tumors and in young patients given the risk of secondary malignancy.7,10

The utility of cryoablation is limited to small and moderately sized tumors in extra-abdominal locations.7

Clinical Trials

Enrollment in a clinical trial may be considered an option for appropriate patients.3

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

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

  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. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274.
  4. 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.
  5. Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16(5):682-693.
  6. 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 May 23, 2022. To view the most recent and complete version of the guideline, go online to 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.
  7. 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.
  8. Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964.
  9. 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.
  10. 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.
  11. Cheon SS, Nadesan P, Poon R, Alman BA. Growth factors regulate beta-catenin-mediated TCF-dependent transcriptional activation in fibroblasts during the proliferative phase of wound healing. Exp Cell Res. 2004;293(2):267-274.
  12. 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.
  13. Lewis JJ, Boland PJ, Leung DHY, et al. The enigma of desmoid tumors. Ann Surg. 1999;229(6):866-872.
  14. 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.
  15. Foo W, Lazar AJ. Pathology of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012. Accessed October 25, 2022.
  16. Soft tissue; fibroblastic/myofibroblastic fibromatosis; fibromatosis-desmoid. Accessed October 25, 2022.
  17. 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.