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
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
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.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.
- 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.
- Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16(5):682-693.
- 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.
- 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.
- 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.
- 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.
- 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.
- Obeidin F, Alexiev B. Fibromatosis-desmoid. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuefibromatosisdeep.html. Accessed August 2, 2023.
- 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.
- 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.
- 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.
- 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.
- Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;28(10):2399-2408.