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Uptake and outcomes of small intestinal and urinary tract cancer surveillance in Lynch syndrome

World Journal of Clinical Oncology 2021年第11期12卷 1023-1036页

作者： jeshua dejesse Ravy K Vajravelu Christina Dudzik Gillain Constantino Jessica M Long Kirk J Wangensteen Kathleen D Valverde Bryson W Katona Department of Genetics University of PennsylvaniaPhiladelphiaPA 19104United States Division of Gastroenterology and Hepatology University of Pennsylvania Perelman School of MedicinePhiladelphiaPA 19104United States Division of Hematology and Oncology University of PennsylvaniaPhiladelphiaPA 19104United States

BACKGROUND Lynch syndrome(LS)is a hereditary cancer predisposition syndrome associated with increased risk of multiple *** colorectal cancer surveillance decreases mortality in LS and is recommended by guidelines,there is lack of evidence for the efficacy of surveillance for extra-colonic cancers associated with LS,including small intestinal cancer(SIC)and urinary tract cancer(UTC).Given the limited evidence,guidelines do not consistently recommend surveillance for SIC and UTC,and it remains unclear how often individuals will choose to undergo and follow through with extra-colonic surveillance *** To study factors associated with SIC and UTC surveillance uptake and outcomes in *** This is an IRB-approved retrospective analysis of individuals with LS seen at a tertiary care referral *** individuals had a pathogenic or likely pathogenic variant in MLH1,MSH2,MSH6,PMS2,or EPCAM,or were a confirmed obligate carrier,and had at least one documented visit to our *** regarding SIC and UTC surveillance was captured for each individual,and detailed personal and family history was obtained for individuals who had an initial LS management visit in our center’s dedicated high-risk LS clinic between January 1,2017 and October 29,*** these initial management visits,all patients had in-depth discussions of SIC and UTC surveillance with 1 of 3 providers experienced in LS management to promote informed decision-making about whether to pursue SIC and/or UTC *** analysis using Pearson’s chi-squared test and Wilcoxon rank-sum test was completed to understand the factors associated with pursuit and completion of SIC and UTC surveillance,and a P value below 0.05 was deemed statistically *** Of 317 individuals with LS,86(27%)underwent a total of 105 SIC surveillance examinations,with 5 leading to additional work-up and no SICs ***,99(31%)patients underwent a total of 3

关键词： Lynch syndrome Urinary tract cancer Intestinal neoplasms Early diagnosis of cancer Patient preference Gastrointestinal surgical procedure

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