Desmoid tumour in familial adenomatous polyposis patients: responses to treatments.

No guidelines for desmoid tumors (DT) management are available and DT have now become the first cause of death in FAP patients who had restorative proctocolectomy. We aimed to assess the results of the different treatments used for DT in Familial Adenomatous Polyposis (FAP) patients. All patients followed for FAP who developed a DT between 1970 and 2010 were collated. We analysed separately the history of DT according to location: mesenteric, parietal or mixed. 79 FAP patients [45 females (56 %); mean age 33.3 ± 12.5] presented 149 DT and were included; 16(20 %) had a DT diagnosed during or before first abdominal surgery and 47 (59 %) had isolated mesenteric DT. 11 patients had only surgical treatment, 17 only medical treatments, 31 had combined treatment and 20 had no treatment with spontaneous DT regression or stabilization. Overall, 80 treatment lines were administered to patients with a progression free or regression rate of 43 % (34/80). Response rates were: chemotherapy 77 % (10/13); Sulindac + tamoxifen 50 % (6/12); Tamoxifen 40 % (6/15); Imatinib 36 % (4/11); Sulindac 28 % (8/29). Among the 42 surgical procedures, an R0 resection was performed in 26 (62 %) allowing the absence of recurrence for 54 %. After a median follow-up of 81 months, 8 patients died of their DT and 6 died of other cause. Overall and DT-specific survival at 20 years were 52 and 79 %, respectively. Chemotherapy was the most efficient treatment. For intra-abdominal DT, we consider it as a first choice treatment and reserve surgery when it is impossible or when DT are life threatening.