In 5 patients, germline pathogenic variants were identified in genes for which there are currently no evidence for a causal role in the development of neoplasms in these children (ie, CHEK2 in acute myeloid leukemia, heterozygous PMS2 in Wilms tumor, SDHA in osteosarcoma, MSH6::FBXO11 in diffuse large B-cell lymphoma,8 and Turner syndrome in neuroblastoma). Here, PMS2 is linked to Wilms tumor.