Clin Orthop Surg.  2020 Mar;12(1):113-119. 10.4055/cios.2020.12.1.113.

Factors Associated with Disease Stabilization of Desmoid-Type Fibromatosis

• Kim Y
• Rosario MS
• Cho HS
• Han I

• KMID: 2470059
• DOI: http://doi.org/10.4055/cios.2020.12.1.113

Abstract

BACKGROUND
Spontaneous disease stabilization of desmoid-type fibromatosis (DF) has been demonstrated in many reports, and the watchful waiting approach without any frontline treatment is becoming popular as an initial management strategy. In this study, we aimed to assess the disease stabilization rate and identify predictive factors for disease stabilization of DF in patients with conservative treatment.
METHODS
We reviewed 76 patients with sporadic extra-abdominal DF who were managed with frontline conservative treatment in our institute. The minimum follow-up was 12 months. Stabilization was defined as radiological evidence of no change or continuous decrease in size of the tumor for six months or more. The primary endpoint was stabilization of DF. Possible patient-, disease-, and treatment-related factors predictive of disease stabilization were analyzed with multivariate analysis.
RESULTS
At final follow-up, 54 of the 76 tumors (71%) were stable, and mean time to stabilization was 30.4 months (range, 7 to 112 months). On Kaplan-Meier survival analysis, the spontaneous stabilization rate was 25.4% at one year, 52.7% at two years, and 70.9% at three years. The mean time to spontaneous stabilization was longer in patients with ≤ 40 years of age (p = 0.022) or recurrence (p = 0.041). On multivariate analysis with the Cox proportional hazard method, recurrence (hazard ratio [HR], 1.79; p = 0.041) and younger age (HR, 2.04; p = 0.022) were identified as independent prognostic factors for longer time to disease stabilization.
CONCLUSIONS
Frontline conservative treatment seems to be the optimal treatment for most patients with DF. Younger patients or those with recurrence may require longer time to spontaneous disease stabilization.

Keyword

Desmoid; Fibromatosis; Aggressive; Stabilization; Watchful waiting

MeSH Terms

Fibroma*
Fibromatosis, Aggressive
Follow-Up Studies
Humans
Methods
Multivariate Analysis
Recurrence
Watchful Waiting

Figure

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  Fig. 1 Algorithm of patient selection. DF: desmoid-type fibromatosis, FU: follow-up, RT: radiation therapy, CTx: chemotherapy.

Reference

1. MacFarlane J. Clinical reports of the surgical practice of the Glasgow Royal Infirmary. Glasgow: Robertson;1832. 1:p. XVI-314.

2. Otero S, Moskovic EC, Strauss DC, et al. Desmoid-type fibromatosis. Clin Radiol. 2015; 70(9):1038–1045.

3. Nieuwenhuis MH, Casparie M, Mathus-Vliegen LM, Dekkers OM, Hogendoorn PC, Vasen HF. A nation-wide study comparing sporadic and familial adenomatous polyposis-related desmoid-type fibromatoses. Int J Cancer. 2011; 129(1):256–261.

4. Stoeckle E, Coindre JM, Longy M, et al. A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases. Eur J Surg Oncol. 2009; 35(2):129–134.

5. Bonvalot S, Eldweny H, Haddad V, et al. Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol. 2008; 34(4):462–468.

6. Yoon GW, Kim JD, Chung SH. The analysis of treatment of aggressive fibromatosis using oral methotrexate chemotherapy. Clin Orthop Surg. 2014; 6(4):439–442.

7. 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.

8. Prodinger PM, Rechl H, Keller M, et al. Surgical resection and radiation therapy of desmoid tumours of the extremities: results of a supra-regional tumour centre. Int Orthop. 2013; 37(10):1987–1993.

9. Zeng WG, Zhou ZX, Liang JW, et al. Prognostic factors for desmoid tumor: a surgical series of 233 patients at a single institution. Tumour Biol. 2014; 35(8):7513–7521.

10. Wang YF, Guo W, Sun KK, et al. Postoperative recurrence of desmoid tumors: clinical and pathological perspectives. World J Surg Oncol. 2015; 13:26.

11. Mueller C, Croner R, Klein P, Grutzmann R, Vassos N. Primary and recurrent sporadic desmoids: Prognostic factors influencing recurrence-free survival after complete gross resection. Int J Surg. 2016; 31:63–70.

12. Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol. 2009; 16(9):2587–2593.

13. Bertani E, Testori A, Chiappa A, et al. Recurrence and prognostic factors in patients with aggressive fibromatosis: the role of radical surgery and its limitations. World J Surg Oncol. 2012; 10:184.

14. Gronchi A, Colombo C, Le Pechoux C, et al. Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group. Ann Oncol. 2014; 25(3):578–583.

15. Huang K, Wang CM, Chen JG, et al. Prognostic factors influencing event-free survival and treatments in desmoidtype fibromatosis: analysis from a large institution. Am J Surg. 2014; 207(6):847–854.

16. Colombo C, Miceli R, Le Pechoux C, et al. Sporadic extra abdominal wall desmoid-type fibromatosis: surgical resection can be safely limited to a minority of patients. Eur J Cancer. 2015; 51(2):186–192.

17. van Broekhoven DL, Verhoef C, Elias SG, et al. Local recurrence after surgery for primary extra-abdominal desmoidtype fibromatosis. Br J Surg. 2013; 100(9):1214–1219.

18. Mitchell G, Thomas JM, Harmer CL. Aggressive fibromatosis: evidence for a stable phase. Sarcoma. 1998; 2(3-4):149–154.

19. Barbier O, Anract P, Pluot E, et al. Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only. Orthop Traumatol Surg Res. 2010; 96(8):884–889.

20. Gronchi A, Raut CP. Optimal approach to sporadic desmoid tumors: from radical surgery to observation: time for a consensus? Ann Surg Oncol. 2012; 19(13):3995–3997.

21. Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 1999; 86(10):2045–2052.

22. Sri-Ram K, Haddo O, Dannawi Z, et al. The outcome of extra-abdominal fibromatosis treated at a tertiary referral centre. Eur J Surg Oncol. 2012; 38(8):700–705.

23. He XD, Zhang YB, Wang L, et al. Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: Analysis of 114 patients at a single institution. Eur J Surg Oncol. 2015; 41(8):1013–1019.

24. Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am. 1984; 66(9):1369–1374.

25. Dalen 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):455–459.

26. Leithner A, Gapp M, Leithner K, et al. Margins in extra-abdominal desmoid tumors: a comparative analysis. J Surg Oncol. 2004; 86(3):152–156.

27. Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF. The enigma of desmoid tumors. Ann Surg. 1999; 229(6):866–872.

28. Shin SH, Ko KR, Cho SK, Choi YL, Seo SW. Surgical outcome of desmoid tumors: adjuvant radiotherapy delayed the recurrence, but did not affect long-term outcomes. J Surg Oncol. 2013; 108(1):28–33.

29. Cates JM. Prognostic factors for second recurrence after surgical resection of recurrent desmoid-type fibromatosis. Pathol Oncol Res. 2015; 21(4):1085–1090.

30. Villalobos VM, Hall F, Jimeno A, et al. Long-term follow-up of desmoid fibromatosis treated with PF-03084014, an oral gamma secretase inhibitor. Ann Surg Oncol. 2018; 25(3):768–775.

31. Kasper B, Baumgarten C, Garcia J, et al. 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.