Outcome of surgery for primary and recurrent desmoid-type fibromatosis. A retrospective case series of 174 patients

•Primary excision with clear margins is associated with superior local control.•Localization in the extremities is associated with higher recurrence rate.•Surgery for recurrent disease carries a very high risk of local relapse, irrespective of surgical margins.•Adjuvant radiotherapy or chemotherapy do not affect local control rate of recurrent disease.

BackgroundThe best management of relapsing desmoid-type fibromatosis, a benign but locally infiltrative soft-tissue tumour, is largely undecided. Our aim was to investigate the incidence and the factors influencing local relapse after surgery for primary and recurrent disease of the trunk and extremities.Patients and MethodsRetrospective analysis of 174 patients who had surgical treatment for desmoid-type fibromatosis. The quality of the surgical margins and use of adjuvant radiotherapy or chemotherapy were analysed regarding local recurrences in primary and recurrent disease.ResultsClear margins were achieved in 41% of cases. 10-year local control rate was 58% for clear primary resections as compared to 37% with intralesional primary resections (p = 0.030). Extremity tumours had a higher risk of local recurrence compared to trunk and pelvic ones (p < 0.001). Attempted resection of recurrent disease was associated with an approximately 90% incidence of relapse after each procedure, despite the quality of the surgical margins being equivalent to primary resections. Quality of surgical margins was not important for local control of recurrent lesions. Adjuvant treatments (radiotherapy and chemotherapy) had a no significant effect on the local control rate of recurrent disease (odds ratio 0.693 and 0.969 respectively).ConclusionsA complete primary excision is the best window of opportunity to achieve local control of desmoid-type fibromatosis. Once the disease relapses, surgical intervention is accompanied with a high risk of failure, irrespective of the quality of the margins and adjuvant treatment given.