Skip to main navigation menu Skip to main content Skip to site footer

Original article

Vol. 149 No. 2930 (2019)

Extra-abdominal desmoid tumours – further evidence for the watchful waiting policy

  • Andreas H. Krieg
  • Christian Wirth
  • Ulrich Lenze
  • Christoph Kettelhack
  • Michael Coslovsky
  • Daniel Baumhoer
  • Frank M. Klenke
  • Klaus A. Siebenrock
  • G. Ulrich Exner
  • Beata Bode-Lesniewska
  • Bruno Fuchs
  • Stéphane Cherix
  • Fritz Hefti
Cite this as:
Swiss Med Wkly. 2019;149:w20107



Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres.


The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0–40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities.


The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0–11.5 years).


Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.


  1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds. WHO Classification of Tumours of Soft Tissue and Bone, Fourth edition. Lyons: IARC Press; 2013.
  2. Okuno S. The enigma of desmoid tumors. Curr Treat Options Oncol. 2006;7(6):438–43. doi:.
  3. Escobar C, Munker R, Thomas JO, Li BD, Burton GV. Update on desmoid tumors. Ann Oncol. 2012;23(3):562–9. doi:.
  4. Kasper B, Ströbel P, Hohenberger P. Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist. 2011;16(5):682–93. doi:.
  5. Nakayama T, Tsuboyama T, Toguchida J, Hosaka T, Nakamura T. Natural course of desmoid-type fibromatosis. J Orthop Sci. 2008;13(1):51–5. doi:.
  6. Kasper B, Baumgarten C, Bonvalot S, Haas R, Haller F, Hohenberger P, et al.; Desmoid Working Group. Management of sporadic desmoid-type fibromatosis: a European consensus approach based on patients’ and professionals’ expertise - a sarcoma patients EuroNet and European Organisation for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer. 2015;51(2):127–36. doi:.
  7. Dalén BP, Bergh PM, Gunterberg BU. Desmoid tumors: a clinical review of 30 patients with more than 20 years’ follow-up. Acta Orthop Scand. 2003;74(4):455–9. doi:.
  8. Higaki S, Tateishi A, Ohno T, Abe S, Ogawa K, Iijima T, et al. Surgical treatment of extra-abdominal desmoid tumours (aggressive fibromatoses). Int Orthop. 1995;19(6):383–9. doi:.
  9. Pignatti G, Barbanti-Bròdano G, Ferrari D, Gherlinzoni F, Bertoni F, Bacchini P, et al. Extraabdominal desmoid tumor. A study of 83 cases. Clin Orthop Relat Res. 2000;(375):207–13. doi:.
  10. Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984;66(9):1369–74. doi:.
  11. Shido Y, Nishida Y, Nakashima H, Katagiri H, Sugiura H, Yamada Y, et al. Surgical treatment for local control of extremity and trunk desmoid tumors. Arch Orthop Trauma Surg. 2009;129(7):929–33. doi:.
  12. Gronchi A, Casali PG, Mariani L, Lo Vullo S, Colecchia M, Lozza L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 2003;21(7):1390–7. doi:.
  13. Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PW, et al. Optimizing treatment of desmoid tumors. J Clin Oncol. 2007;25(13):1785–91. doi:.
  14. Salas S, Dufresne A, Bui B, Blay JY, Terrier P, Ranchere-Vince D, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol. 2011;29(26):3553–8. doi:.
  15. Ballo MT, Zagars GK, Pollack A. Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 1998;42(5):1007–14. doi:.
  16. Baumert BG, Spahr MO, Von Hochstetter A, Beauvois S, Landmann C, Fridrich K, et al. The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network. Radiat Oncol. 2007;2(1):12. doi:.
  17. Huang K, Wang CM, Chen JG, Du CY, Zhou Y, Shi YQ, et al. Prognostic factors influencing event-free survival and treatments in desmoid-type fibromatosis: analysis from a large institution. Am J Surg. 2014;207(6):847–54. doi:.
  18. Crago AM, Denton B, Salas S, Dufresne A, Mezhir JJ, Hameed M, et al. A prognostic nomogram for prediction of recurrence in desmoid fibromatosis. Ann Surg. 2013;258(2):347–53. doi:.
  19. Mavrogenis AF, Angelini A, Pala E, Calabro T, Bianchi G, Casadei R, et al. Radiation-induced sarcomas. J Long Term Eff Med Implants. 2011;21(3):233–40. doi:.
  20. Garbay D, Le Cesne A, Penel N, Chevreau C, Marec-Berard P, Blay JY, et al. Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG). Ann Oncol. 2012;23(1):182–6. doi:.
  21. Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G. High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer. 2004;100(3):612–20. doi:.
  22. Briand S, Barbier O, Biau D, Bertrand-Vasseur A, Larousserie F, Anract P, et al. Wait-and-see policy as a first-line management for extra-abdominal desmoid tumors. J Bone Joint Surg Am. 2014;96(8):631–8. doi:.
  23. Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009;16(9):2587–93. doi:.
  24. Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008;34(4):462–8. doi:.
  25. Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF. The enigma of desmoid tumors. Ann Surg. 1999;229(6):866–72, discussion 872–3. doi:.

Most read articles by the same author(s)