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Desmoid tumors

Desmoid tumors are rare, locally aggressive tumors that form in the connective tissues of the body.1,2 Connective tissue is found throughout the body, meaning desmoid tumors can grow anywhere in the body. Sometimes referred to as desmoid fibromatosis, desmoid tumors can cause severe pain, limited function, loss of mobility, disfigurement and fatigue.1-6 Desmoid tumors are challenging to manage due to their unpredictable nature and high recurrence rate, which can significantly and sometimes debilitatingly impact individuals' quality of life.2,3,4,7,8 These invasive, tendril-like growths can wrap around nearby tissue and compress vital organs, muscles, vessels, and nerves.2,3,9 For some, a desmoid tumor may be life-threatening, depending on the tumor size, where and how it grows and the person's overall health.2,9

There are approximately 1,300-2,300 new cases of desmoid tumors diagnosed annually in the EU.10,11,12

References:

  1. Sbaraglia M, Bellan E, Dei Tos AP. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Pathologica. 2021;113(2):70-84. doi:10.32074/1591- 951X-213.
  2. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol. 2017;29(4):268-274. doi:10.1097/CCO.0000000000000374.
  3. Constantinidou A, Scurr M, Judson I, Litchman C. Clinical presentation of desmoid tumors. In: Litchman C, ed. Desmoid Tumors. Springer; 2012:chap 2. Accessed 12th June, 2025. https://www.researchgate.net/publication/226455135_Clinical_Presentation_of_Desmoid_Tumors.
  4. Bektas, M, et al. Desmoid Tumors: A Comprehensive Review. Adv Therapeutics 2023. doi.org/10.1007/s12325-023-02592-0.
  5. 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. doi:10.1007/s00520-018-4386-8.
  6. Gounder MM, Maddux L, Paty J, Atkinson TM. Prospective development of a patient-reported outcomes instrument for desmoid tumors or aggressive fibromatosis. Cancer. 2020;126(3):531-539. doi:10.1002/cncr.32555.
  7. Skubitz KM. Biology and treatment of aggressive fibromatosis or desmoid tumor. Mayo Clin Proc. 2017;92(6):947-964. doi:10.1016/j.mayocp.2017.02.012.
  8. 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. doi:10.1097/00000658-198912000-00012.
  9. 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. doi:10.3390/cancers3033143.
  10. van Broekhoven DLM, Grünhagen DJ, den Bakker MA, van Dalen T, Verhoef C. Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study. Ann Surg Oncol. 2015;22(9):2817-2823. doi:10.1245/s10434-015-4632-y
  11. 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. doi:10.1016/j.ejca.2019.11.013.
  12. Eurostat. Population structure and ageing. European Commission. Accessed 12th June, 2025. https://ec.europa.eu/eurostat/statistics-explained/index. php?title=Population_structure_and_ageing.