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

Summary

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. 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 81months, 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.