Lynch syndrome patients have a risk of developing CRC by age 70 of 40–50% for MLH1 and MSH2 heterozygous carriers, 20% for MSH6 carriers and a significant lower risk for PMS2 carriers [14], according to the most recent findings of the international, multicenter Prospective Lynch Syndrome Database calculations (visit www.plsd.eu (accessed on January 2021) for cancer type, gene, and gender-specific risks). Here, PMS2 is linked to Lynch syndrome.