Survival by colon cancer stage and screening interval in Lynch syndrome : a prospective Lynch syndrome database report

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dc.contributor University of Helsinki, HUS Abdominal Center en
dc.contributor University of Helsinki, HUS Abdominal Center en
dc.contributor University of Helsinki, HUS Abdominal Center en
dc.contributor.author Dominguez-Valentin, Mev
dc.contributor.author Seppälä, Toni T.
dc.contributor.author Sampson, Julian R.
dc.contributor.author Macrae, Finlay
dc.contributor.author Winship, Ingrid
dc.contributor.author Evans, D. Gareth
dc.contributor.author Scott, Rodney J.
dc.contributor.author Burn, John
dc.contributor.author Moeslein, Gabriela
dc.contributor.author Bernstein, Inge
dc.contributor.author Pylvaenaeinen, Kirsi
dc.contributor.author Renkonen-Sinisalo, Laura
dc.contributor.author Lepistö, Anna
dc.contributor.author Lindblom, Annika
dc.contributor.author Plazzer, John-Paul
dc.contributor.author Tjandra, Douglas
dc.contributor.author Thomas, Huw
dc.contributor.author Green, Kate
dc.contributor.author Lalloo, Fiona
dc.contributor.author Crosbie, Emma J.
dc.contributor.author Hill, James
dc.contributor.author Capella, Gabriel
dc.contributor.author Pineda, Marta
dc.contributor.author Navarro, Matilde
dc.contributor.author Vidal, Joan Brunet
dc.contributor.author Ronlund, Karina
dc.contributor.author Nielsen, Randi Thyregaard
dc.contributor.author Yilmaz, Mette
dc.contributor.author Elvang, Louise Laurberg
dc.contributor.author Katz, Lior
dc.contributor.author Nielsen, Maartje
dc.contributor.author ten Broeke, Sanne W.
dc.contributor.author Nakken, Sigve
dc.contributor.author Hovig, Eivind
dc.contributor.author Sunde, Lone
dc.contributor.author Kloor, Matthias
dc.contributor.author Doeberitz, Magnus v Knebel
dc.contributor.author Ahadova, Aysel
dc.contributor.author Lindor, Noralane
dc.contributor.author Steinke-Lange, Verena
dc.contributor.author Holinski-Feder, Elke
dc.contributor.author Mecklin, Jukka-Pekka
dc.contributor.author Moller, Pal
dc.date.accessioned 2019-11-08T12:32:03Z
dc.date.available 2019-11-08T12:32:03Z
dc.date.issued 2019-10-14
dc.identifier.citation Dominguez-Valentin , M , Seppälä , T T , Sampson , J R , Macrae , F , Winship , I , Evans , D G , Scott , R J , Burn , J , Moeslein , G , Bernstein , I , Pylvaenaeinen , K , Renkonen-Sinisalo , L , Lepistö , A , Lindblom , A , Plazzer , J-P , Tjandra , D , Thomas , H , Green , K , Lalloo , F , Crosbie , E J , Hill , J , Capella , G , Pineda , M , Navarro , M , Vidal , J B , Ronlund , K , Nielsen , R T , Yilmaz , M , Elvang , L L , Katz , L , Nielsen , M , ten Broeke , S W , Nakken , S , Hovig , E , Sunde , L , Kloor , M , Doeberitz , M V K , Ahadova , A , Lindor , N , Steinke-Lange , V , Holinski-Feder , E , Mecklin , J-P & Moller , P 2019 , ' Survival by colon cancer stage and screening interval in Lynch syndrome : a prospective Lynch syndrome database report ' , Hereditary Cancer in Clinical Practice , vol. 17 , no. 1 , 28 . https://doi.org/10.1186/s13053-019-0127-3 en
dc.identifier.issn 1731-2302
dc.identifier.other PURE: 127994254
dc.identifier.other PURE UUID: 86f6f2fc-ff38-462a-b191-05e1362106b8
dc.identifier.other WOS: 000490141300001
dc.identifier.other ORCID: /0000-0002-5866-4059/work/64323933
dc.identifier.other ORCID: /0000-0002-4940-3498/work/64325718
dc.identifier.uri http://hdl.handle.net/10138/306836
dc.description.abstract Background We previously reported that in pathogenic mismatch repair (path_MMR) variant carriers, the incidence of colorectal cancer (CRC) was not reduced when colonoscopy was undertaken more frequently than once every 3 years, and that CRC stage and interval since last colonoscopy were not correlated. Methods The Prospective Lynch Syndrome Database (PLSD) that records outcomes of surveillance was examined to determine survival after colon cancer in relation to the time since previous colonoscopy and pathological stage. Only path_MMR variants scored by the InSiGHT variant database as class 4 or 5 (clinically actionable) were included in the analysis. Results Ninety-nine path_MMR carriers had no cancer prior to or at first colonoscopy, but subsequently developed colon cancer. Among these, 96 were 65 years of age or younger at diagnosis, and included 77 path_MLH1, 17 path_MSH2, and 2 path_MSH6 carriers. The number of cancers detected within <1.5, 1.5-2.5, 2.5-3.5 and at > 3.5 years after previous colonoscopy were 9, 43, 31 and 13, respectively. Of these, 2, 8, 4 and 3 were stage III, respectively, and only one stage IV (interval 2.5-3.5 years) disease. Ten-year crude survival after colon cancer were 93, 94 and 82% for stage I, II and III disease, respectively (p <0.001). Ten-year crude survival when the last colonoscopy had been <1.5, 1.5-2.5, 2.5-3.5 or > 3.5 years before diagnosis, was 89, 90, 90 and 92%, respectively (p = 0.91). Conclusions In path_MLH1 and path_MSH2 carriers, more advanced colon cancer stage was associated with poorer survival, whereas time since previous colonoscopy was not. Although the numbers are limited, together with our previously reported findings, these results may be in conflict with the view that follow-up of path_MMR variant carriers with colonoscopy intervals of less than 3 years provides significant benefit. en
dc.format.extent 6
dc.language.iso eng
dc.relation.ispartof Hereditary Cancer in Clinical Practice
dc.rights en
dc.subject Lynch syndrome en
dc.subject Survival en
dc.subject Colonoscopy en
dc.subject Surveillance en
dc.subject Cancer stage en
dc.subject Colon cancer en
dc.subject 3122 Cancers en
dc.title Survival by colon cancer stage and screening interval in Lynch syndrome : a prospective Lynch syndrome database report en
dc.type Article
dc.description.version Peer reviewed
dc.identifier.doi https://doi.org/10.1186/s13053-019-0127-3
dc.type.uri info:eu-repo/semantics/other
dc.type.uri info:eu-repo/semantics/publishedVersion
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