{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T15:34:14Z","timestamp":1787758454228,"version":"build-2784847793"},"reference-count":90,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T00:00:00Z","timestamp":1785283200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T00:00:00Z","timestamp":1785283200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Current literature estimates that 10% of pediatric cancers are caused by pathogenic or likely pathogenic (P\/LP) germline variants in cancer predisposition genes (CPGs). Variants in CPGs thought to increase cancer risk exclusively during adulthood are referred to as \u201cadult\u2010onset\u201d CPGs (aoCPGs). Because next\u2010generation sequencing (NGS)\u2010based molecular tumor profiling, whole genome sequencing (WGS), and whole exome sequencing (WES) are used increasingly in patient assessments, P\/LP likely germline aoCPG variants are identified frequently in pediatric patients. However, whether those variants drive cancer development remains an open question. The prevalence of P\/LP germline aoCPG variants in pediatric cancers is unclear due to differences in bioinformatics pipelines employed, variability in the aoCPGs analyzed, predominance of small cohort sizes, and a limited number of cancer subtypes analyzed. This review provides a summary of the current literature on aoCPGs in pediatric cancers, identifies gaps in our knowledge, and highlights areas of focus for future research.<\/jats:p>","DOI":"10.1002\/1545-5017.70465","type":"journal-article","created":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T03:46:00Z","timestamp":1785383160000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Role of \u201cAdult\u2010Onset\u201d Cancer Predisposition Genes in Pediatric Cancer: A Comprehensive Review"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-9652-759X","authenticated-orcid":false,"given":"Maria","family":"Rozo","sequence":"first","affiliation":[{"name":"Division of Pediatric Hematology, Oncology, Neuro\u2010Oncology &amp; Stem Cell Transplantation Ann &amp; Robert H. 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