The cumulative risk of CRC by the age of 70 years is higher in Lynch patients with pathogenic germline mutations in MSH2 (48–77 %), MLH1 (41–79 %) or deletions affecting the 3′ exon of EPCAM (69–75 %), compared to carriers of pathogenic germline mutations in MSH6 (12-50 %) or PMS2 (15–20 %) [21–23]. Here, MSH2 is linked to colorectal carcinoma.