The sister (ID_315) was also a carrier and developed an EC at 62 years, which demonstrated loss of MSH2/MSH6 expression and a somatic second hit in MSH2. The second exception was an EC diagnosed at 53 years from ID_156 who carried the MSH6 c.3556 + 5_3556 + 9delins variant that was MSI-H/dMMR from tumor sequencing, showed solitary loss of MSH6 expression but no somatic mutation in MSH6; however, a CRC diagnosed at 61 years in ID_156 also showed a solitary loss of MSH6 expression with a second hit in MSH6. This evidence concerns the gene MSH2 and colorectal carcinoma.