A related syndrome is CMMRD discussed above, a much less frequent pediatric autosomal recessive disease where children inherit bi-allelic MLH1, MSH2, MSH6 or PMS2 mutations that drive aggressive cancer predisposition and are affected with cancers as often as every 2-3 years in early life and commonly perish from brain, GI, and hematopoietic malignancies (50). Here, MSH6 is linked to cancer.