While mutations in MLH1 were mostly indicative of an increased risk of CRC, MSH2 and MSH6 mutations were seen to predispose to extracolonic malignancies such as endometrial and urothelial cancers in addition to CRC and PMS2 to a lower life time cancer risk and more atypical malignancies [63,64]. Here, MSH6 is linked to colorectal carcinoma.