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

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. 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. 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 recurrence-free 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. 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.