Pancreatic cancer and biliary tract tumors in individuals with lynch syndrome.

Lynch syndrome (LS) is an autosomal dominant disorder leading to increased risk of cancer, especially colorectal and endometrial cancers. However, there is very limited available data for other gastrointestinal tumors such as pancreatic and biliary tract cancers. The aim of this study was to evaluate pancreatic cancer (PC), cholangiocarcinoma (CCA) and hepatocellular carcinoma (HCC) incidence among LS carriers. The retrospective study comprised 425 patients, with a median age of 54 (IQR 43-65) years old, where the most frequent tumor was colorectal cancer followed by endometrial cancer. Four patients (0.94 %) were diagnosed with pancreatic cancer at average age of 53.5 years (IQR 41.75-64.5), and 8 (1.9 %) with cholangiocarcinoma at average age of 62.25 (IQR 51.75-75.25) and no cases of HCC. Age-Standardized Incidence Rate (ASR) for CCA was 210.6 (95 % CI: 63.43-357.77), 46.8 times more frequent in LS carriers. ASR for PC was 119.59 (95 % CI:1.22-240.4), 6.3 times more frequent in LS carriers but without significant statistical differences. The risk of developing PC and CCA in LS patients is higher than in general population with an earlier age of onset, mainly from CCA.