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Identifying a CPD remains challenging, as traditional approaches rely on clinical features and family history. Additionally, the contribution of de novo germline pathogenic variants (GPVs) to RMS etiology remains uncharacterized, and the presence of de novo GPVs, variable expressivity, or incomplete penetrance may limit clinical recognition of at\u2010risk individuals. The McGill Interactive Pediatric OncoGenetic Guidelines (MIPOGG) is a tool proposed to help clinicians identify patients with increased likelihood of a CPD by incorporating tumor\u2010specific referral criteria. The Children's Oncology Group (COG) Genetics of Embryonal and Alveolar Rhabdomyosarcoma Study (GEARS; AEPI15N1) enrolled families of 299 children and young adults with RMS. In addition to collection of questionnaires, germline samples were collected from probands and family members for whole genome sequencing (WGS). GPVs in CPD genes were identified in 10.6% of probands and more commonly in patients with embryonal versus alveolar tumors (12.0% vs. 7.8%, respectively;\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.372). Through WGS analysis of proband\u2010parent trios, 30.0% of GPVs were classified as de novo. The MIPOGG algorithm correctly identified 85.7% of children with RMS and a GPV, but specificity was limited (43.0%). Germline CPD identification has important clinical implications, and these findings highlight the utility and limitations of decision\u2010support tools like MIPOGG. This emphasizes the need to refine CPD screening methods to inform risk assessments and guide genetic evaluation and counseling in children with RMS.\n                  <\/jats:p>","DOI":"10.1002\/ijc.70705","type":"journal-article","created":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T05:17:15Z","timestamp":1788931035000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Germline Cancer Predisposition and De Novo Contributions in Pediatric Rhabdomyosarcoma: A Report From the Children's Oncology Group"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1644-6093","authenticated-orcid":false,"given":"Taylor M.","family":"Luckie","sequence":"first","affiliation":[{"name":"Texas Children's Cancer and Hematology Center, Texas Children's Hospital  Houston Texas USA"},{"name":"Department of Pediatrics, Division of Hematology and Oncology Baylor College of Medicine  Houston Texas 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Hospital  Houston Texas USA"},{"name":"Department of Pediatrics, Division of Hematology and Oncology Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olga","family":"Taylor","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Aflac Cancer and Blood Disorders Center at Children's Healthcare of Atlanta  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2516-0222","authenticated-orcid":false,"given":"Logan G.","family":"Spector","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Epidemiology and Clinical Research University of Minnesota  Minneapolis Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael E.","family":"Scheurer","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Aflac Cancer and Blood Disorders Center at Children's Healthcare of Atlanta  Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas S.","family":"Hawkins","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology\/Oncology Seattle Children's Hospital  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajkumar","family":"Venkatramani","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Center, Texas Children's Hospital  Houston Texas USA"},{"name":"Department of Pediatrics, Division of Hematology and Oncology Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chad D.","family":"Huff","sequence":"additional","affiliation":[{"name":"Department of Epidemiology University of Texas MD Anderson Cancer Center  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon E.","family":"Plon","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Center, Texas Children's Hospital  Houston Texas USA"},{"name":"Department of Pediatrics, Division of Hematology and Oncology Baylor College of Medicine  Houston Texas USA"},{"name":"Department of Molecular and Human Genetics Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0978-5863","authenticated-orcid":false,"given":"Philip J.","family":"Lupo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Emory University School of Medicine  Atlanta Georgia USA"},{"name":"Aflac Cancer and Blood Disorders Center at Children's Healthcare of Atlanta  Atlanta Georgia 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