Surgical resection and radiation therapy of desmoid tumours of the extremities: results of a supra-regional tumour centre

Abstract

Purpose Desmoid tumours of the extremity have a high recurrence rate. The purpose of this study was to analyse the outcome after resection of these tumours with special emphasis on recurrent disease and adjuvant therapeutic strategies. Methods In this retrospective study we evaluated prognostic factors for recurrence-free survival after surgical treatment of desmoid tumours of the extremity in 27 patients with an average age of 41 years treated from 1997 to 2009. Adjuvant radiotherapy (50-60 Gy) was given in five cases with primary and in nine patients with recurrent disease. The average follow-up was 64 months. Results The five-year recurrence-free survival in patients with primary disease was 33 %. Patients with negative resection margins tended to have a better outcome than patients with positive resection margins, but the difference between both groups was not significant (56 vs 14 %, p=0.145). In patients with positive margins, adjuvant radiotherapy did not significantly improve recurrence-free survival (40 vs 14 %, p=0.523). Patients with local recurrence had a five-year further recurrencefree survival of 47 %. In those patients further recurrence-free survival was significantly better after adjuvant radiation (89 vs 25 %, p=0.015). Two thirds of all patients suffered moderate or severe complications due to the treatment regimen. Conclusions Compared to desmoids of the trunk or the head and neck region, desmoids affecting the limbs show by far the worst outcome in terms of relapse or treatment-related morbidity. The importance of negative resection margins is still not clear. Particularly in recurrent desmoids adjuvant radiotherapy appears to reduce the further recurrence rate. Therefore, a general use of radiation should be considered for this high-risk group.

Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis

“…Sufficient data for analysis were published in two articles; authors from the other 50 articles were contacted and requested to provide the additional data needed. Of these, 14 responded, bringing the total number of included studies to 16 ( Fig. ).…”

“…Baumert et al 36 Bertani et al 37 Bonvalot et al 11 Cates and Stricker 24 Eastley et al 38 Gronchi et al 22 Huang et al 39 Ihalainen et al 40 Kriz et al 41 Pignatti et al 42 Prodinger et al 43 Salas et al 25 Stoeckle et al 44 van Broekhoven et al 23 Wang et al 45 Wilkinson et al 46 Total Heterogeneity: 37 Bonvalot et al 11 Cates and Stricker 24 Eastley et al 38 Gronchi et al 22 Huang et al 39 Ihalainen et al 40 Kriz et al 41 Pignatti et al 42 Prodinger et al 43 Salas et al 25 Stoeckle et al 44 van Broekhoven et al 23 Wang et al 45 Wilkinson et al 46 Total Heterogeneity: χ 2 = 7·97, 3 d.f., P = 0·05; I 2 = 62% Test for overall effect: Z = 2·50, P = 0·01 Bertani et al 37 Bonvalot et al 11 Cates and Stricker 24 Eastley et al 38 Gronchi et al 22 Huang et al 39 Ihalainen et al 40 Kriz et al 41 Pignatti et al 42 Prodinger et al 43 Salas et al 25 Stoeckle et al 44 van Broekhoven et al 23 Wang et al…”

Risk factors for local recurrence of fibromatosis

“…However, there is no consensus in this regard, since there are desmoid tumours that have been observed to stop growing spontaneously. 3,7,13,14 It does appear clear that as we increase therapeutic measures, in addition to increasing monitoring of the disease, we enhance the functional consequences, those of radical surgery and radiotherapy in particular. 13,14 We must remember, in our series, that treatment by periodic monitoring only took place in patients who had already developed local recurrence, therefore we do not know what would have happened if we had not operated the primary tumours.…”

Four different treatment strategies in aggressive fibromatosis: A systematic review

“…Finally, after reading the full text, 37 studies were included in the analysis ( Fig. 1 ) [1] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] .

Fig.

…”