Today most patients with Lynch syndrome survive their first cancer.
There is limited information on the incidences and outcome of subsequent cancers.
Dr Pål Møller and colleagues addressed 3 questions, including the cumulative incidence of a subsequent cancer, in which organs subsequent cancers occur, and the survival following these cancers is.
Information was collated on prospectively organized surveillance and prospectively observed outcomes in patients with Lynch syndrome who had cancer prior to inclusion and analysed by age, gender and genetic variants.
The team followed up 1273 patients with Lynch syndrome from 10 countries for 7753 observation years.
The researchers found that 26% of patients developed 341 first subsequent cancers, including colorectal, upper GI, pancreas or bile duct and urinary tract.
|26% of patients developed 341 first subsequent cancers|
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 the cumulative incidences were 46%, 48% and 23%, respectively.
The research team observed that crude survival after any subsequent cancer was 82%, and 10-year crude survival after colorectal cancer was 91%.
Dr Møller's team concludes, "Relative incidence of subsequent cancer compared with incidence of first cancer was slightly but insignificantly higher than cancer incidence in patients with Lynch syndrome without previous cancer."
"The favorable 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 Lynch syndrome with previous cancer."