BACKGROUND: The lifetime risk of developing @entity5 in @entity1412 ( @entity1412 ) is about 5 per cent. When and to what extent surgical intervention should be undertaken to prevent @entity204 from invasive @entity189 is controversial. The aim of this study was to determine the effectiveness of various surgical treatments for @entity5 and severe duodenal @entity11449 . METHODS: A questionnaire was mailed to the members of the Leeds @entity1412 Group to obtain data on @entity1 with @entity1412 , treated for @entity5 or severe duodenal @entity11449 after 1990. RESULTS: Sixty-nine @entity1 were included. The indication for surgery was @entity3 in 13 @entity1 , of whom six died from metastatic disease. Fifty-six @entity1 were initially treated for severe duodenal @entity11449 , five (9 per cent) of whom died from metastatic disease (P = 0.002). In surviving @entity1 , @entity652 recurred after ampullectomy (six of eight, at mean follow-up of 11 months), after duodenotomy with polypectomy (17 of 21, at mean 29 months) and after pancreatoduodenectomy (six of 25, at mean 47 months). None of six @entity1 who underwent a pancreas-sparing duodenectomy had recurrence of @entity652 (mean follow-up 11 months). CONCLUSION: Surgery for duodenal @entity11449 should take place before endoscopic biopsy reveals @entity3 . Even after extensive surgical procedures, small bowel @entity652 may occur, emphasizing the need for chemoprevention.

Worldwide survey among @entity1412 registries of surgical management of severe duodenal @entity11449 in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity652 :: ('MESH:D000236', 'Disease') :: ['adenoma', 'adenomas'] @entity11449 :: ('MESH:D018255', 'Disease') :: ['adenomatosis'] @entity5 :: ('MESH:D009369', 'Disease') :: ['duodenal cancer', 'cancer'] @entity189 :: ('MESH:D002277', 'Disease') :: ['carcinoma'] @entity3 :: ('MESH:D009362', 'Disease') :: ['invasive cancer'] @entity1412 :: ('MESH:D011125', 'Disease') :: ['familial adenomatous polyposis', 'FAP', 'Castle Polyposis'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death']
@entity1412 :: (MESH:D011125,Disease) :: ['familial adenomatous polyposis']
