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dc.contributor.authorMøller, Pal
dc.contributor.authorSeppälä, Toni T.
dc.contributor.authorAhadova, Aysel
dc.contributor.authorCrosbie, Emma J.
dc.contributor.authorHolinski-Feder, Elke
dc.contributor.authorScott, Rodney
dc.contributor.authorHaupt, Saskia
dc.contributor.authorMöslein, Gabriela
dc.contributor.authorWinship, Ingrid
dc.contributor.authorBajwa-ten Broeke, Sanne W.
dc.contributor.authorKohut, Kelly E.
dc.contributor.authorRyan, Neil
dc.contributor.authorBauerfeind, Peter
dc.contributor.authorThomas, Laura E.
dc.contributor.authorEvans, D. Gareth
dc.contributor.authorAretz, Stefan
dc.contributor.authorSijmons, Rolf H.
dc.contributor.authorHalf, Elizabeth
dc.contributor.authorHeinimann, Karl
dc.contributor.authorHorisberger, Karoline
dc.contributor.authorMonahan, Kevin
dc.contributor.authorEngel, Christoph
dc.contributor.authorCavestro, Giulia Martina
dc.contributor.authorFruscio, Robert
dc.contributor.authorAbu-Freha, Naim
dc.contributor.authorZohar, Levi
dc.contributor.authorLaghi, Luigi
dc.contributor.authorBertario, Lucio
dc.contributor.authorBonanni, Bernardo
dc.contributor.authorTibiletti, Maria Grazia
dc.contributor.authorLino-Silva, Leonardo S.
dc.contributor.authorVaccaro, Carlos
dc.contributor.authorDella Valle, Adriana
dc.contributor.authorRossi, Benedito Mauro
dc.contributor.authorda Silva, Leandro Apolinário
dc.contributor.authorde Oliveira Nascimento, Ivana Lucia
dc.contributor.authorRossi, Norma Teresa
dc.contributor.authorDębniak, Tadeusz
dc.contributor.authorMecklin, Jukka-Pekka
dc.contributor.authorBernstein, Inge
dc.contributor.authorLindblom, Annika
dc.contributor.authorSunde, Lone
dc.contributor.authorNakken, Sigve
dc.contributor.authorHeuveline, Vincent
dc.contributor.authorBurn, John
dc.contributor.authorHovig, Eivind
dc.contributor.authorKloor, Matthias
dc.contributor.authorSampson, Julian R.
dc.contributor.authorDominguez-Valentin, Mev
dc.identifier.citationMøller, P., Seppälä, T. T., Ahadova, A., Crosbie, E. J., Holinski-Feder, E., Scott, R., Haupt, S., Möslein, G., Winship, I., Bajwa-ten Broeke, S. W., Kohut, K. E., Ryan, N., Bauerfeind, P., Thomas, L. E., Evans, D. G., Aretz, S., Sijmons, R. H., Half, E., Heinimann, K., . . . Dominguez-Valentin, M. (2023). Dominantly inherited micro-satellite instable cancer – the four Lynch syndromes : an EHTG, PLSD position statement. <i>Hereditary Cancer in Clinical Practice</i>, <i>21</i>, Article 19. <a href="" target="_blank"></a>
dc.description.abstractThe recognition of dominantly inherited micro-satellite instable (MSI) cancers caused by pathogenic variants in one of the four mismatch repair (MMR) genes MSH2, MLH1, MSH6 and PMS2 has modified our understanding of carcinogenesis. Inherited loss of function variants in each of these MMR genes cause four dominantly inherited cancer syndromes with different penetrance and expressivities: the four Lynch syndromes. No person has an “average sex “or a pathogenic variant in an “average Lynch syndrome gene” and results that are not stratified by gene and sex will be valid for no one. Carcinogenesis may be a linear process from increased cellular division to localized cancer to metastasis. In addition, in the Lynch syndromes (LS) we now recognize a dynamic balance between two stochastic processes: MSI producing abnormal cells, and the host’s adaptive immune system’s ability to remove them. The latter may explain why colonoscopy surveillance does not reduce the incidence of colorectal cancer in LS, while it may improve the prognosis. Most early onset colon, endometrial and ovarian cancers in LS are now cured and most cancer related deaths are after subsequent cancers in other organs. Aspirin reduces the incidence of colorectal and other cancers in LS. Immunotherapy increases the host immune system’s capability to destroy MSI cancers. Colonoscopy surveillance, aspirin prevention and immunotherapy represent major steps forward in personalized precision medicine to prevent and cure inherited MSI cancer.en
dc.publisherBiomed Central
dc.relation.ispartofseriesHereditary Cancer in Clinical Practice
dc.rightsCC BY 4.0
dc.titleDominantly inherited micro-satellite instable cancer – the four Lynch syndromes : an EHTG, PLSD position statement
dc.contributor.laitosLiikuntatieteellinen tiedekuntafi
dc.contributor.laitosFaculty of Sport and Health Sciencesen
dc.rights.copyright© The Author(s) 2023
dc.subject.ysoperinnölliset taudit
jyx.fundinginformationPM, EH and MD-V received a grant from The Norwegian Cancer Society, Contract 194751–2017. TTS received grants from Cancer Society Finland, Sigrid Juselius Foundation, Jane and Aatos Erkko Foundation, Relander Foundation and the Academy of Finland. EJC and DGE are funded by the Manchester National Institute for Health Research (NIHR) Biomedical Research Centre (ISBRC-1215–20007), IS-BRC-1215–20007. JRS received grant support previously from Health and Care Research Wales for the Wales Gene Park.

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