In our cohort, variants were found in well-known CRC predisposition genes such as APC and MUTYH, as well as in newly emerging candidate genes for cancer predisposition, such as MSH3, EXO1 and FAN1. Since patients with biallelic mutations in MUTYH were previously discarded in our LLS series [19,31], only three heterozygous MUTYH carriers were found (current study and [10]). Here, EXO1 is linked to colorectal carcinoma.