In a meta-analysis of 11 studies incorporating comprehensive germline testing for children and adolescents with cancer, a P/LP germline variant in one of the MMR genes was reported in 19 of 3975 patients including 9 patients with PMS2 variants (brain tumors n = 4, nonbrain solid tumors n = 4, hematologic neoplasms n = 1) [12]. This evidence concerns the gene MRC1 and hematopoietic and lymphoid system neoplasm.