Women who carry an MSH2, MLH1 or MSH6 germline mutation and who present with CRC in the absence of distant metastases will present an extraordinarily high lifetime risk for carcinoma of the endometrium and/or ovary, therefore prophylactic hysterectomy and oophorectomy may also be considered for female patients with LS [61,69]. Here, MSH6 is linked to colorectal carcinoma.