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dc.contributor.authorDominguez-Valentin, Mev
dc.contributor.authorSeppälä, Toni T.
dc.contributor.authorSampson, Julian R.
dc.contributor.authorMacrae, Finlay
dc.contributor.authorWinship, Ingrid
dc.contributor.authorEvans, D. Gareth
dc.contributor.authorScott, Rodney J.
dc.contributor.authorBurn, John
dc.contributor.authorMöslein, Gabriela
dc.contributor.authorBernstein, Inge
dc.contributor.authorPylvänäinen, Kirsi
dc.contributor.authorRenkonen-Sinisalo, Laura
dc.contributor.authorLepistö, Anna
dc.contributor.authorLindblom, Annika
dc.contributor.authorPlazzer, John-Paul
dc.contributor.authorTjandra, Douglas
dc.contributor.authorThomas, Huw
dc.contributor.authorGreen, Kate
dc.contributor.authorLalloo, Fiona
dc.contributor.authorCrosbie, Emma J.
dc.contributor.authorHill, James
dc.contributor.authorCapella, Gabriel
dc.contributor.authorPineda, Marta
dc.contributor.authorNavarro, Matilde
dc.contributor.authorVidal, Joan Brunet
dc.contributor.authorRønlund, Karina
dc.contributor.authorNielsen, Randi Thyregaard
dc.contributor.authorYilmaz, Mette
dc.contributor.authorElvang, Louise Laurberg
dc.contributor.authorKatz, Lior
dc.contributor.authorNielsen, Maartje
dc.contributor.authorten Broeke, Sanne W.
dc.contributor.authorNakken, Sigve
dc.contributor.authorHovig, Eivind
dc.contributor.authorSunde, Lone
dc.contributor.authorKloor, Matthias
dc.contributor.authorKnebel Doeberitz, Magnus v.
dc.contributor.authorAhadova, Aysel
dc.contributor.authorLindor, Noralane
dc.contributor.authorSteinke-Lange, Verena
dc.contributor.authorHolinski-Feder, Elke
dc.contributor.authorMecklin, Jukka-Pekka
dc.contributor.authorMøller, Pål
dc.identifier.citationDominguez-Valentin, M., Seppälä, T. T., Sampson, J. R., Macrae, F., Winship, I., Evans, D. G., Scott, R. J., Burn, J., Möslein, G., Bernstein, I., Pylvänäinen, K., Renkonen-Sinisalo, L., Lepistö, A., Lindblom, A., Plazzer, J.-P., Tjandra, D., Thomas, H., Green, K., Lalloo, F., . . . Møller, P. (2019). Survival by colon cancer stage and screening interval in Lynch syndrome : a prospective Lynch syndrome database report. <i>Hereditary Cancer in Clinical Practice</i>, <i>17</i>, Article 28. <a href="" target="_blank"></a>
dc.description.abstractBackground. 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.publisherBioMed Central Ltd
dc.relation.ispartofseriesHereditary Cancer in Clinical Practice
dc.rightsCC BY 4.0
dc.subject.otherlynch syndrome
dc.subject.othercancer stage
dc.subject.othercolon cancer
dc.titleSurvival by colon cancer stage and screening interval in Lynch syndrome : a prospective Lynch syndrome database report
dc.contributor.laitosLiikuntatieteellinen tiedekuntafi
dc.contributor.laitosFaculty of Sport and Health Sciencesen
dc.rights.copyright© 2019 the Authors
dc.subject.ysoLynchin oireyhtymä
jyx.fundinginformationTTS and J-PM are supported by the Emil Aaltonen Foundation, the Finnish Medical Foundation, the Instrumentarium Science Foundation, Sigrid Juselius Foundation, The Finnish Cancer Foundation, Jane and Aatos Erkko foundation and State Research Funding.

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