TY - JOUR AU - Dominguez-Valentin, Mev AU - Haupt, Saskia AU - Seppälä, Toni T. AU - Sampson, Julian R. AU - Sunde, Lone AU - Bernstein, Inge AU - Jenkins, Mark A. AU - Engel, Christoph AU - Aretz, Stefan AU - Nielsen, Maartje AU - Capella, Gabriel AU - Balaguer, Francesc AU - Evans, Dafydd Gareth AU - Burn, John AU - Holinski-Feder, Elke AU - Bertario, Lucio AU - Bonanni, Bernardo AU - Lindblom, Annika AU - Levi, Zohar AU - Macrae, Finlay AU - Winship, Ingrid AU - Plazzer, John-Paul AU - Sijmons, Rolf AU - Laghi, Luigi AU - Della Valle, Adriana AU - Heinimann, Karl AU - Dębniak, Tadeusz AU - Fruscio, Robert AU - Lopez-Koestner, Francisco AU - Alvarez-Valenzuela, Karin AU - Katz, Lior H. AU - Laish, Ido AU - Vainer, Elez AU - Vaccaro, Carlos AU - Carraro, Dirce Maria AU - Monahan, Kevin AU - Half, Elizabeth AU - Stakelum, Aine AU - Winter, Des AU - Kennelly, Rory AU - Gluck, Nathan AU - Sheth, Harsh AU - Abu-Freha, Naim AU - Greenblatt, Marc AU - Rossi, Benedito Mauro AU - Bohorquez, Mabel AU - Cavestro, Giulia Martina AU - Lino-Silva, Leonardo S. AU - Horisberger, Karoline AU - Tibiletti, Maria Grazia AU - Nascimento, Ivana do AU - Thomas, Huw AU - Rossi, Norma Teresa AU - Apolinário da Silva, Leandro AU - Zaránd, Attila AU - Ruiz-Bañobre, Juan AU - Heuveline, Vincent AU - Mecklin, Jukka-Pekka AU - Pylvänäinen, Kirsi AU - Renkonen-Sinisalo, Laura AU - Lepistö, Anna AU - Peltomäki, Päivi AU - Therkildsen, Christina AU - Madsen, Mia Gebauer AU - Burgdorf, Stefan Kobbelgaard AU - Hopper, John L. AU - Win, Aung Ko AU - Haile, Robert W. AU - Lindor, Noralane AU - Gallinger, Steven AU - Le Marchand, Loïc AU - Newcomb, Polly A. AU - Figueiredo, Jane AU - Buchanan, Daniel D. AU - Thibodeau, Stephen N. AU - von Knebel Doeberitz, Magnus AU - Loeffler, Markus AU - Rahner, Nils AU - Schröck, Evelin AU - Steinke-Lange, Verena AU - Schmiegel, Wolff AU - Vangala, Deepak AU - Perne, Claudia AU - Hüneburg, Robert AU - Redler, Silke AU - Büttner, Reinhard AU - Weitz, Jürgen AU - Pineda, Marta AU - Duenas, Nuria AU - Vidal, Joan Brunet AU - Moreira, Leticia AU - Sánchez, Ariadna AU - Hovig, Eivind AU - Nakken, Sigve AU - Green, Kate AU - Lalloo, Fiona AU - Hill, James AU - Crosbie, Emma AU - Mints, Miriam AU - Goldberg, Yael AU - Tjandra, Douglas AU - ten Broeke, Sanne W. AU - Kariv, Revital AU - Rosner, Guy AU - Advani, Suresh H. AU - Thomas, Lidiya AU - Shah, Pankaj AU - Shah, Mithun AU - Neffa, Florencia AU - Esperon, Patricia AU - Pavicic, Walter AU - Torrezan, Giovana Tardin AU - Bassaneze, Thiago AU - Martin, Claudia Alejandra AU - Moslein, Gabriela AU - Moller, Pål TI - Mortality by age, gene and gender in carriers of pathogenic mismatch repair gene variants receiving surveillance for early cancer diagnosis and treatment: a report from the prospective Lynch syndrome database JO - EClinicalMedicine VL - 58 SN - 2589-5370 CY - Amsterdam PB - Elsevier M1 - DKFZ-2023-00849 SP - 101909 PY - 2023 AB - Background: The Prospective Lynch Syndrome Database (PLSD) collates information on carriers of pathogenic or likely pathogenic MMR variants (path_MMR) who are receiving medical follow-up, including colonoscopy surveillance, which aims to the achieve early diagnosis and treatment of cancers. Here we use the most recent PLSD cohort that is larger and has wider geographical representation than previous versions, allowing us to present mortality as an outcome, and median ages at cancer diagnoses for the first time. Methods: The PLSD is a prospective observational study without a control group that was designed in 2012 and updated up to October 2022. Data for 8500 carriers of path_MMR variants from 25 countries were included, providing 71,713 years of follow up. Cumulative cancer incidences at 65 years of age were combined with 10-year crude survival following cancer, to derive estimates of mortality up to 75 years of age by organ, gene, and gender. Findings: Gynaecological cancers were more frequent than colorectal cancers in path_MSH2, path_MSH6 and path_PMS2 carriers [cumulative incidence: 53.3 LB - PUB:(DE-HGF)16 DO - DOI:10.1016/j.eclinm.2023.101909 UR - https://inrepo02.dkfz.de/record/275750 ER -