{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T09:20:48Z","timestamp":1770888048339,"version":"3.50.1"},"reference-count":50,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,10,20]],"date-time":"2023-10-20T00:00:00Z","timestamp":1697760000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>An estimated 8.6% of all pediatric patients with central nervous system tumors (CNSTs) have underlying hereditary cancer predisposition (HCP). Identifying HCP affects risk assessment and medical management options for the patients and their family members. However, there is a lack of consensus on the optimal germline genetic testing (GT) approach for pediatric patients with CNSTs. As a first step in addressing the need for consensus, we surveyed oncology and genetics providers from 47 institutions in professional organizations across the United States. We investigated their current practice (e.g., GT decisions and ordering practices) when assessing pediatric patients with CNSTs for HCP. We received 60 responses from 21 pediatric oncologists, 10 neuro\u2010oncologists, 28 genetics providers, and one neuro\u2010oncologist\/geneticist. Results demonstrate genetic counselors, followed by oncologists, most often facilitated consent, ordered testing, and selected which test to order. The most ordered test was a multi\u2010gene panel (60%). Of 18 CNST diagnoses, choroid plexus carcinoma (CPC) was the diagnosis for which most providers (78%) reported they would offer GT. For medulloblastoma, 56% overall reported they would offer GT (64% of genetics providers, 62% of neuro\u2010oncologists, 20% of pediatric oncologists; <jats:italic>p<\/jats:italic>\u00a0=\u00a0.050). Findings suggest that even for the CNSTs most commonly known to be associated with HCP regardless of family history, there was variability in providers\u2019 decisions to offer GT. The lack of consensus in GT decisions in our study indicates inconsistencies in the genetics care of pediatric patients with CNSTs, demonstrating a need for consensus guidelines to promote consistent genetics care.<\/jats:p>","DOI":"10.1002\/pbc.30725","type":"journal-article","created":{"date-parts":[[2023,10,20]],"date-time":"2023-10-20T07:59:19Z","timestamp":1697788759000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Current practice of cancer predisposition testing in pediatric patients with CNS tumors in the United States"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-8669-0980","authenticated-orcid":false,"given":"Baylee","family":"Roy","sequence":"first","affiliation":[{"name":"Division of Human Genetics Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics College of Medicine University of Cincinnati  Cincinnati Ohio USA"},{"name":"Leadership Education in Neurodevelopmental and related Disabilities University of Cincinnati and Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Knapke","sequence":"additional","affiliation":[{"name":"Division of Human Genetics Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Cancer and Blood Diseases Institute Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natasha","family":"Pillay\u2010Smiley","sequence":"additional","affiliation":[{"name":"Cancer and Blood Diseases Institute Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xue","family":"Zhang","sequence":"additional","affiliation":[{"name":"Division of Human Genetics Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kate","family":"Queen","sequence":"additional","affiliation":[{"name":"Cancer and Blood Diseases Institute Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rebecca","family":"Sisson","sequence":"additional","affiliation":[{"name":"Division of Human Genetics Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"Department of Pediatrics College of Medicine University of Cincinnati  Cincinnati Ohio USA"},{"name":"Cancer and Blood Diseases Institute Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,10,20]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2014.09.011"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2010.04.003"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ncl.2018.04.009"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2019.08.004"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s43018\u2010021\u201000172\u20101"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1508054"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/jncics\/pkaa018"},{"key":"e_1_2_9_9_1","unstructured":"HowladerN NooneAM KrapchoM et\u00a0al.SEER cancer statistics review 1975\u20132018.National Cancer Institute (NCI);2021. Accessed on May 1 2022.https:\/\/seer.cancer.gov\/csr\/1975_2018\/"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.3322\/caac.21219"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noab106"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470\u20102045(18)30242\u20100"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/nop\/npv051"},{"issue":"18","key":"e_1_2_9_14_1","first-page":"5358","article-title":"A cancer family syndrome in twenty\u2010four kindreds","volume":"48","author":"Li FP","year":"1988","journal-title":"Cancer Res"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40291\u2010013\u20100020\u20100"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.CCR\u201017\u20100590"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.CCR\u201017\u20100595"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10689\u2010016\u20109905\u20105"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470\u20102045(16)30249\u20102"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.CCR\u201017\u20100834"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.6004\/jnccn.2022.0062"},{"key":"e_1_2_9_22_1","unstructured":"DalyMB PalT AlHilliZ et\u00a0al.Genetic\/familial high\u2010risk assessment: breast ovarian and pancreatic version 3.2023 NCCN Clinical Practice Guidelines in Oncology.NCCN; published2023. Accessed on January 1 2023.https:\/\/www.nccn.org\/professionals\/physician_gls\/pdf\/genetics_bop.pdf"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1245\/s10434\u2010019\u201007549\u20108"},{"key":"e_1_2_9_24_1","unstructured":"GuptaS WeissJ AxellL et\u00a0al.Genetic\/familial high\u2010risk assessment: colorectal version 2.2022. NCCN Clinical Practice Guidelines in Oncology.NCCN; published2022. Accessed on October 1 2022.https:\/\/www.nccn.org\/professionals\/physician_gls\/pdf\/genetics_colon.pdf"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2016.71.0012"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12837"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2017.09.048"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1101\/mcs.a003863"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.canlet.2017.06.018"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noac079.389"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoncol.2020.6252"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1200\/PO.21.00516"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381\u2010020\u201004712\u20105"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978\u20100\u2010444\u201062702\u20105.00010\u2010X"},{"key":"e_1_2_9_35_1","volume-title":"Von Hippel\u2013Lindau Syndrome","author":"Mikhail MI","year":"2022"},{"key":"e_1_2_9_36_1","volume-title":"GeneReviews","author":"Van Leeuwaarde RS","year":"2000"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2005.01.026"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2008.16.6959"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.23349"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2009.26.8169"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1097\/CCO.0000000000000423"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.751784"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.586288"},{"key":"e_1_2_9_44_1","volume-title":"GeneReviews","author":"Northrup H","year":"1999"},{"key":"e_1_2_9_45_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2017.1406"},{"key":"e_1_2_9_46_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29982"},{"key":"e_1_2_9_47_1","doi-asserted-by":"publisher","DOI":"10.1002\/jgc4.1339"},{"key":"e_1_2_9_48_1","doi-asserted-by":"publisher","DOI":"10.1093\/nop\/npab031"},{"key":"e_1_2_9_49_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073815587944"},{"key":"e_1_2_9_50_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10897\u2010017\u20100158\u20108"},{"key":"e_1_2_9_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2021.08.008"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.30725","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T13:31:55Z","timestamp":1702042315000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.30725"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,20]]},"references-count":50,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,1]]}},"alternative-id":["10.1002\/pbc.30725"],"URL":"https:\/\/doi.org\/10.1002\/pbc.30725","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,20]]},"assertion":[{"value":"2023-05-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-06","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e30725"}}