- 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
- 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, according to the NCCN Guidelines2
- Desmoid tumors have infiltrative, tendril-like growths and clear surgical margins can be challenging to achieve3,4
- Local recurrence rates range from 24% to 77% after surgical resection of desmoid tumors4,5,†
- Treatment considerations, in addition to reduction of tumor growth, include reduction in symptoms, improvement in functioning, and improvement in overall quality of life6-8
- 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.
Based on retrospective, observational data. Factors associated with local recurrence postsurgery include tumor location, age of the participant, and tumor size.9
Identify desmoid tumor progression early to help impact patient outcomes with this 1-page resource endorsed by desmoid tumor experts.
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.6-8
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 CT1,2,10
- Worsening of symptoms1,2,10
- Worsening impact on daily living (eg, functional impairment)1,2,10
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.11 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 [category 2A])
- Chemotherapy (methotrexate and vinblastine/vinorelbine, doxorubicin-based regimens [category 2A])
- NSAIDs (sulindac or other NSAIDs, including celecoxib for pain [category 2A])
Hear from a desmoid tumor expert oncologist
In most tumor locations, surgery is no longer the recommended first-line treatment option for desmoid tumors.1 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, according to the NCCN Guidelines.2 Active surveillance is now preferred and recommended by the Desmoid Tumor Working Group Guideline for initial management of all asymptomatic patients.1,12
When active treatment becomes necessary, the Desmoid Tumor Working Group Guideline recommends treatment modalities other than surgery if morbidity or positive microscopic margins can be anticipated.1 For abdominal wall desmoid tumors, surgery is still recommended as the first option in case of progression.1
Desmoid tumors are invasive and clear margins are challenging to achieve
Desmoid tumors can infiltrate surrounding organs and neurovascular structures, which can decrease quality of life and in some cases may be life-threatening.13,14
Gross tumor specimen post-resection showing invasive tendrils (arrows)15
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.3,4,16,17
- At 3 major US surgical centers, 40.1% (67/167) of patients with desmoid tumors had positive margins after undergoing surgery with curative intent18
- In a retrospective analysis of 174 patients with desmoid tumors who were treated with surgery, only 4% were able to achieve wide, clear margins19
- 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 margins20
Gross tumor specimen post-resection showing ill defined borders (arrows)21
Recurrence is common after surgery for desmoid tumors
Regardless of whether the margins are positive or negative, recurrence rates can be high after desmoid tumor surgery.
- 24-77% local recurrence rates dependent on tumor location4,5
- Extremity, chest wall, and intra-abdominal tumors have the highest recurrence risk9
- Younger patient age and larger tumor size can be associated with higher recurrence risk following complete resection9
- Median time to recurrence after surgery was 7.2 months (range: 1.9 to 34.9 months) in one study of 135 patients with extra-abdominal desmoid tumors22
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.3 More aggressive local treatment may be associated with poorer long-term functional outcomes.3,15,23,24
- 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 operations25
- Following surgery for extra-abdominal desmoid tumors, 73% of patients reported similar or worse pain and 80% of patients reported similar or worse levels of weakness17,‡
- Based on a prospective study of 206 patients with extremity desmoid tumors, 24% of those with recurrent tumors (22/91) would have required amputation to achieve definitive resection16
Retrospective study of 15 patients with desmoid tumors treated at a US academic institution.
Hear from a desmoid tumor expert surgeon
Radiation therapy, cryoablation, and high-frequency ultrasound are sometimes used to help treat desmoid tumors.1,2,12,26
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.1,3,12 Caution should be exercised as to use of radiotherapy for abdominal wall tumors and in young patients given the risk of secondary malignancy.3,12
The utility of cryoablation is limited to small and moderately sized tumors in extra-abdominal locations.12
Enrollment in a clinical trial may be considered an option for appropriate patients.13
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.
- 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.
- Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.1.2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed March 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.
- 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.
- Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964.
- 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.
- Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16(5):682-693.
- 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.
- 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.
- 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.
- 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.
- Riedel RF, Agulnik M. Evolving strategies for management of desmoid tumor. Cancer. 2022;128(16):3027-3040.
- 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.
- Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274.
- Constantinidou A, Scurr M, Judson I, Litchman C. Clinical presentation of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012:chap 2. Accessed February 22, 2023. https://www.researchgate.net/publication/226455135.
- Foo W, Lazar AJ. Pathology of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012. Accessed February 22, 2023. https://doi.org/10.1007/978-94-007-1685-8_3.
- Lewis JJ, Boland PJ, Leung DHY, et al. The enigma of desmoid tumors. Ann Surg. 1999;229(6):866-872.
- Dafford K, Kim D, Nelson A, Kline D. Extraabdominal desmoid tumors. Neurosurg Focus. 2007;22(6):E21.
- 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.
- 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.
- 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.
- Obeidin F, Alexiev B. Fibromatosis-desmoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosisdeep.html. Accessed February 22, 2023.
- Minami Y, Matsumoto S, Ae K, et al. The clinical features of multicentric extra-abdominal desmoid tumors. Cancer Diagn Progn. 2021;1(4):339-343.
- 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.
- 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.
- Lev D, Kotilingam D, Wei C, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25(13):1785-1791.
- 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.