{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T10:09:57Z","timestamp":1790244597879,"version":"4.1.0"},"reference-count":11,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T00:00:00Z","timestamp":1766361600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,12,22]],"date-time":"2025-12-22T00:00:00Z","timestamp":1766361600000},"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 & Cancer"],"published-print":{"date-parts":[[2026,2]]},"abstract":"ABSTRACT<\/jats:title>\n \n Anaplastic sarcoma of the kidney (ASK) is a\n DICER1<\/jats:italic>\n \u2010associated malignant tumor presumed to arise in a benign precursor, pediatric cystic nephroma (PCN). However, the initial oncogenic alteration(s) associated with malignant transformation are unknown. Here, we report genomic characterization of a PCN harboring focal anaplasia consistent with early sarcomatous transformation. Multi\u2010region sequencing of PCN and anaplastic areas revealed an\n NRAS<\/jats:italic>\n Q61R mutation exclusively in the latter. Germline and somatic\n DICER1<\/jats:italic>\n alterations were common to both. This is the first report of an oncogenic alteration in incipient sarcomatous transformation in a PCN and supports the histogenetic association between PCN and ASK.\n <\/jats:p>","DOI":"10.1002\/1545-5017.70068","type":"journal-article","created":{"date-parts":[[2025,12,23]],"date-time":"2025-12-23T07:02:28Z","timestamp":1766473348000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Oncogenic\n NRAS<\/i>\n Mutation in Incipient Sarcomatous Transformation of Cystic Nephroma From a Patient With\n DICER1<\/i>\n \u2010Related Tumor Predisposition Syndrome"],"prefix":"10.1002","volume":"73","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1704-9284","authenticated-orcid":false,"given":"Nahir","family":"Cortes\u2010Santiago","sequence":"first","affiliation":[{"name":"Department of Pathology Texas Children's Hospital Houston Texas USA"},{"name":"Department of Pathology & Immunology Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah B.","family":"Whittle","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Center Texas Children's Hospital Houston Texas USA"},{"name":"Division of Hematology and Oncology Department of Pediatrics Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2017-0245","authenticated-orcid":false,"given":"Sarah","family":"Scollon","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Center Texas Children's Hospital Houston Texas USA"},{"name":"Division of Hematology and Oncology Department of Pediatrics 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"}]},{"given":"Sharon E.","family":"Plon","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Center Texas Children's Hospital Houston Texas USA"},{"name":"Division of Hematology and Oncology Department of Pediatrics 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"}]},{"given":"Sanjeev","family":"Vasudevan","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery M.E. DeBakey Department of Surgery Texas Children's Hospital Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bryony J.","family":"Lucas","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Center Texas Children's Hospital Houston Texas USA"},{"name":"Division of Hematology and Oncology Department of Pediatrics Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angshumoy","family":"Roy","sequence":"additional","affiliation":[{"name":"Department of Pathology Texas Children's Hospital Houston Texas USA"},{"name":"Department of Pathology & Immunology Baylor College of Medicine Houston Texas USA"},{"name":"Texas Children's Cancer and Hematology Center Texas Children's Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,12,22]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31804d43a4"},{"key":"e_1_2_6_3_1","unstructured":"Texas Children's Hospital Department of Pathology Cancer Genomics Laboratory \u201cPediatric Solid Tumor Mutation Panel \u201dTexas Children's Hospital Department of Pathology Cancer Genomics Laboratory https:\/\/www.lucidoc.com\/cgi\/doc\u2010gw.pl?ref=tch:16746."},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.31090"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2016.03.002"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25959"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2017.100"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1200\/PO.21.00554"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1200\/PO.17.00113"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/onc.2014.150"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1177\/10935266211043861"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41379\u2010020\u20100516\u20101"}],"container-title":["Pediatric Blood & Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/1545-5017.70068","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/1545-5017.70068","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/1545-5017.70068","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T09:37:11Z","timestamp":1790242631000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/1545-5017.70068"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,22]]},"references-count":11,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,2]]}},"alternative-id":["10.1002\/1545-5017.70068"],"URL":"https:\/\/doi.org\/10.1002\/1545-5017.70068","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,22]]},"assertion":[{"value":"2025-08-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-01","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-22","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70068"}}