Multimodal treatment of desmoid tumours: the significance of local control.

INTRODUCTION: Desmoid tumours are a rare group of tumours arising in the deep musculoaponeurotic structures and although they have no metastatic potential they can be locally aggressive with relapse rates of between 23-40%. Three sub-sites are reported: extra-abdominal, abdominal wall and intra-abdominal. The purpose of this study was to analyze patients with these tumours treated and followed at our institution and to determine factors influencing disease free survival. MATERIAL AND METHODS: We conducted a retrospective study of 20 patients treated between 1997 and 2009. Data was compiled to include age, gender, surgical history, familial adenomatous polyposis (FAP), contraceptives, tumour site, first-line treatment, positive margins and adjuvant radiotherapy. A descriptive and survival statistical analysis was also performed. RESULTS: Most patients were women, with a median age of 36 years, with abdominal wall involvement and treated with complete surgery without adjuvant radiotherapy. With a median follow-up of 35 months (range 0-188), local control at 5 years for any kind of treatment was 80%. Overall survival (OS) and 5-year progression-free survival (PFS) were 100% and 86%, respectively. CONCLUSION: Desmoid tumours are group of rare tumours. Although complete surgical resection remains the cornerstone of treatment for resectable lesions, there is still substantial risk of recurrence. Our outcomes are comparable to those reported in the few series published to date.