No guidelines for @entity765 ( @entity765 ) management are available and @entity765 have now become the first cause of @entity204 in @entity1412 @entity1 who had restorative proctocolectomy. We aimed to assess the results of the different treatments used for @entity765 in @entity1412 ( @entity1412 ) @entity1 . All @entity1 followed for @entity1412 who developed a @entity765 between 1970 and 2010 were collated. We analysed separately the history of @entity765 according to location: mesenteric, parietal or mixed. 79 @entity1412 @entity1 [45 females (56  %); mean age 33.3      12.5] presented 149 @entity765 and were included; 16(20  %) had a @entity765 diagnosed during or before first abdominal surgery and 47 (59  %) had isolated mesenteric @entity765 . 11 @entity1 had only surgical treatment, 17 only medical treatments, 31 had combined treatment and 20 had no treatment with spontaneous @entity765 regression or stabilization. Overall, 80 treatment lines were administered to @entity1 with a progression free or regression rate of 43  % (34/80). Response rates were: chemotherapy 77  % (10/13); Sulindac  +  @entity440 50  % (6/12); @entity440 40  % (6/15); @entity2213 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 @entity1 died of their @entity765 and 6 died of other cause. Overall and @entity765 -specific survival at 20  years were 52 and 79  %, respectively. Chemotherapy was the most efficient treatment. For intra-abdominal @entity765 , we consider it as a first choice treatment and reserve surgery when it is impossible or when @entity765 are life threatening.

Desmoid tumour in XXXX @entity1 : responses to treatments.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity440 :: ('MESH:D013629', 'Chemical') :: ['tamoxifen', 'Tamoxifen'] @entity765 :: ('MESH:D018222', 'Disease') :: ['DT', 'desmoid tumors'] @entity1412 :: ('MESH:D011125', 'Disease') :: ['Familial Adenomatous Polyposis', 'FAP'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity2213 :: ('MESH:C097613', 'Chemical') :: ['Imatinib']
@entity1412 :: (MESH:D011125,Disease) :: ['familial adenomatous polyposis']
