2006
DOI: 10.1007/s10689-005-5668-0
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Surveillance and management of upper gastrointestinal disease in Familial Adenomatous Polyposis

Abstract: Almost all patients affected by Familial Adenomatous polyposis (FAP) will develop foregut as well as hindgut polyps, and following prophylactic colectomy duodenal cancer constitutes one of the leading causes of death in screened populations. Without prophylactic colectomy, FAP patients predictably develop colorectal cancer, but the lifetime risk of upper gastrointestinal cancer is lower, estimated at approximately 5%. Management of the upper gastrointestinal cancer risk is one of the greatest challenges facing… Show more

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Cited by 65 publications
(44 citation statements)
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References 122 publications
(170 reference statements)
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“…Duodenal adenomas occur in up to 90% of patients with FAP, most commonly at the ampulla, peri-ampullary region or distal duodenum, which is thought to be in part due to the exposure of the duodenal mucosa to bile in a predisposed patient [Bulow et al 2004;Gallagher et al 2006;Spigelman et al 1989]. The lifetime risk of duodenal cancer in patients with FAP is estimated to be 3-5% [Bulow et al 2004;Groves et al 2002;Lepisto et al 2009;Vasen et al 1997].…”
Section: Duodenal Adenomas In Familial Adenomatous Polyposismentioning
confidence: 99%
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“…Duodenal adenomas occur in up to 90% of patients with FAP, most commonly at the ampulla, peri-ampullary region or distal duodenum, which is thought to be in part due to the exposure of the duodenal mucosa to bile in a predisposed patient [Bulow et al 2004;Gallagher et al 2006;Spigelman et al 1989]. The lifetime risk of duodenal cancer in patients with FAP is estimated to be 3-5% [Bulow et al 2004;Groves et al 2002;Lepisto et al 2009;Vasen et al 1997].…”
Section: Duodenal Adenomas In Familial Adenomatous Polyposismentioning
confidence: 99%
“…In view of this high risk of duodenal cancer in patients with advanced duodenal polyposis, upper gastrointestinal endoscopic surveillance has been proposed, with examinations beginning from age 25-30 [Cairns et al 2010;Gallagher et al 2006]. Forward and side viewing endoscopes should be used to provide adequate visualization of all the duodenal mucosa [Bulow et al 2004;Groves et al 2002].…”
Section: Surveillance Of Duodenal Familial Adenomatous Polyposismentioning
confidence: 99%
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“…2 While prophylactic proctocolectomy has decreased the proportion of colorectal cancer as a cause of death in FAP patients, duodenal cancer has become a more important cause of death for these patients. 3,4 Therefore, endoscopic surveillance is important for the early detection of duodenal cancer and can improve the prognosis of FAP patients. 5 The stomach is also known as a common site of polyps in FAP patients.…”
Section: Introductionmentioning
confidence: 99%
“…Return to normal histology (34%) and downgrading in the Spigelman stage III and IV in either periampullary or extra-ampullary adenomas have been reported (68,69). However, only longer-term follow up will determine whether repeated endoscopic intervention, either alone or in combination with pharmacological therapy, may be offer to selected patients.…”
Section: Extracolonic Tumor Surveillancementioning
confidence: 99%