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Region Hovedstaden - en del af Københavns Universitetshospital
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Incidence of and survival after subsequent cancers in carriers of pathogenic MMR variants with previous cancer: a report from the prospective Lynch syndrome database

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

  • Pål Møller
  • Toni Seppälä
  • Inge Bernstein
  • Elke Holinski-Feder
  • Paola Sala
  • D Gareth Evans
  • Annika Lindblom
  • Finlay Macrae
  • Ignacio Blanco
  • Rolf Sijmons
  • Jacqueline Jeffries
  • Hans Vasen
  • John Burn
  • Sigve Nakken
  • Eivind Hovig
  • Einar Andreas Rødland
  • Kukatharmini Tharmaratnam
  • Wouter H de Vos Tot Nederveen Cappel
  • James Hill
  • Juul Wijnen
  • Mark Jenkins
  • Kate Green
  • Fiona Lalloo
  • Lone Sunde
  • Miriam Mints
  • Lucio Bertario
  • Marta Pineda
  • Matilde Navarro
  • Monika Morak
  • Laura Renkonen-Sinisalo
  • Ian M Frayling
  • John-Paul Plazzer
  • Kirsi Pylvanainen
  • Maurizio Genuardi
  • Jukka-Pekka Mecklin
  • Gabriela Möslein
  • Julian R Sampson
  • Gabriel Capella
  • Mallorca Group (http://mallorca-group.org)
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OBJECTIVE: Today most patients with Lynch syndrome (LS) survive their first cancer. There is limited information on the incidences and outcome of subsequent cancers. The present study addresses three questions: (i) what is the cumulative incidence of a subsequent cancer; (ii) in which organs do subsequent cancers occur; and (iii) what is the survival following these cancers?

DESIGN: Information was collated on prospectively organised surveillance and prospectively observed outcomes in patients with LS who had cancer prior to inclusion and analysed by age, gender and genetic variants.

RESULTS: 1273 patients with LS from 10 countries were followed up for 7753 observation years. 318 patients (25.7%) developed 341 first subsequent cancers, including colorectal (n=147, 43%), upper GI, pancreas or bile duct (n=37, 11%) and urinary tract (n=32, 10%). The cumulative incidences for any subsequent cancer from age 40 to age 70 years were 73% for pathogenic MLH1 (path_MLH1), 76% for path_MSH2 carriers and 52% for path_MSH6 carriers, and for colorectal cancer (CRC) the cumulative incidences were 46%, 48% and 23%, respectively. Crude survival after any subsequent cancer was 82% (95% CI 76% to 87%) and 10-year crude survival after CRC was 91% (95% CI 83% to 95%).

CONCLUSIONS: Relative incidence of subsequent cancer compared with incidence of first cancer was slightly but insignificantly higher than cancer incidence in patients with LS without previous cancer (range 0.94-1.49). The favourable survival after subsequent cancers validated continued follow-up to prevent death from cancer. The interactive website http://lscarisk.org was expanded to calculate the risks by gender, genetic variant and age for subsequent cancer for any patient with LS with previous cancer.

OriginalsprogEngelsk
TidsskriftGut
Vol/bind66
Udgave nummer9
Sider (fra-til)1657-1664
ISSN0017-5749
DOI
StatusUdgivet - sep. 2017

ID: 49150856