Two prospective and eight retrospective cohort studies that analyzed the cumulative incidence of CRC based on the causative gene in patients with Lynch syndrome found that the cumulative incidence of CRC by the age of 80 years was 46%–61% for MLH1, 33%–52% for MSH2 and EPCAM, 10%–44% for MSH6, and 8.7%–20% for PMS2, indicating variability in the incidence of CRC depending on the causative gene [10, 335, 410, 429–433]. Here, MLH1 is linked to colorectal carcinoma.