{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:58:48Z","timestamp":1787497128622,"version":"build-2736575974"},"reference-count":16,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:00:00Z","timestamp":1773014400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"THRIVE EU Horizon funding Mission Cancer","award":["EU-HORIZON-MISS-2023-CANCER-01"],"award-info":[{"award-number":["EU-HORIZON-MISS-2023-CANCER-01"]}]},{"name":"SIRIC CARPEM"},{"DOI":"10.13039\/501100004099","name":"Ligue Nationale contre le Cancer","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004099","id-type":"DOI","asserted-by":"crossref"}]},{"name":"CisMutHep InCa High-Risk High-Gain"},{"name":"NIH\/NCI","award":["P50CA210964"],"award-info":[{"award-number":["P50CA210964"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Fleifil et al. recently reported the DNAJB1::PRKACA fusion in 70% of hepatoblastomas (HB), suggesting its diagnostic relevance beyond fibrolamellar carcinoma (FLC). Here, an international consortium re-examined this claim using 225 independent assays across 148 HB samples from five institutions. We found no evidence of the DNAJB1::PRKACA fusion in any HB case, in contrast to 286\/290 FLC samples. These findings demonstrate that the fusion remains specific to FLC and underscore the need for rigorous validation before clinical interpretation of molecular findings in pediatric liver tumors.<\/jats:p>","DOI":"10.3390\/cancers18050877","type":"journal-article","created":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T15:49:42Z","timestamp":1773071382000},"page":"877","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Global Re-Analysis Confirms Absence of the DNAJB1::PRKACA Fusion in Hepatoblastoma. Comment on Fleifil et al. DNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways. Cancers 2025, 17, 83"],"prefix":"10.3390","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9252-0200","authenticated-orcid":false,"given":"Waqar","family":"Arif","sequence":"first","affiliation":[{"name":"Department of Oncology, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8615-4224","authenticated-orcid":false,"given":"Sanford M.","family":"Simon","sequence":"additional","affiliation":[{"name":"Laboratory of Cellular Biophysics, The Rockefeller University, New York, NY 10065, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8957-3870","authenticated-orcid":false,"given":"Allison F.","family":"O\u2019Neill","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02115, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3487-4641","authenticated-orcid":false,"given":"Juan","family":"Putra","sequence":"additional","affiliation":[{"name":"Department of Pathology, Boston Children\u2019s Hospital, Boston, MA 02115, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6584-420X","authenticated-orcid":false,"given":"Dolores H.","family":"L\u00f3pez-Terrada","sequence":"additional","affiliation":[{"name":"Department of Pathology, Texas Children\u2019s Hospital, Houston, TX 77030, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4401-0748","authenticated-orcid":false,"given":"Mark","family":"Yarchoan","sequence":"additional","affiliation":[{"name":"Department of Oncology, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, MD 21231, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5687-0334","authenticated-orcid":false,"given":"Jessica","family":"Zucman-Rossi","sequence":"additional","affiliation":[{"name":"Centre de Recherche des Cordeliers, Universit\u00e9 Paris Cit\u00e9, Sorbonne Universit\u00e9, INSERM, 75006 Paris, France"},{"name":"H\u00f4pital Europ\u00e9en Georges Pompidou, Assistance Publique-H\u00f4pitaux de Paris, APHP, 75015 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4428-2997","authenticated-orcid":false,"given":"Theo Z.","family":"Hirsch","sequence":"additional","affiliation":[{"name":"Centre de Recherche des Cordeliers, Universit\u00e9 Paris Cit\u00e9, Sorbonne Universit\u00e9, INSERM, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,9]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1002\/1097-0142(19800715)46:2<372::AID-CNCR2820460227>3.0.CO;2-S","article-title":"Fibrolamellar Carcinoma of the Liver: A Tumor of Adolescents and Young Adults with Distinctive Clinico-Pathologic Features","volume":"46","author":"Craig","year":"1980","journal-title":"Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1126\/science.1249484","article-title":"Detection of a Recurrent DNAJB1-PRKACA Chimeric Transcript in Fibrolamellar Hepatocellular Carcinoma","volume":"343","author":"Honeyman","year":"2014","journal-title":"Science"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1038\/modpathol.2015.4","article-title":"DNAJB1-PRKACA Is Specific for Fibrolamellar Carcinoma","volume":"28","author":"Graham","year":"2015","journal-title":"Mod. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1093\/annonc\/mdw135","article-title":"A Genomic Case Study of Mixed Fibrolamellar Hepatocellular Carcinoma","volume":"27","author":"Griffith","year":"2016","journal-title":"Ann. Oncol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1053\/j.gastro.2019.10.028","article-title":"Recurrent Rearrangements in PRKACA and PRKACB in Intraductal Oncocytic Papillary Neoplasms of the Pancreas and Bile Duct","volume":"158","author":"Singhi","year":"2019","journal-title":"Gastroenterology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1080\/15592294.2015.1076955","article-title":"Methylome Sequencing for Fibrolamellar Hepatocellular Carcinoma Depicts Distinctive Features","volume":"10","author":"Malouf","year":"2015","journal-title":"Epigenetics"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1016\/j.jhep.2019.12.006","article-title":"BAP1 Mutations Define a Homogeneous Subgroup of Hepatocellular Carcinoma with Fibrolamellar-like Features and Activated PKA","volume":"72","author":"Hirsch","year":"2020","journal-title":"J. Hepatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1002\/hep.29719","article-title":"Fibrolamellar Carcinoma in the Carney Complex: PRKAR1A Loss Instead of the Classic DNAJB1-PRKACA Fusion","volume":"68","author":"Graham","year":"2018","journal-title":"Hepatology"},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Fleifil, Y., Gulati, R., Jennings, K., Miethke, A., Bondoc, A., Tiao, G., Geller, J.I., Karns, R., Timchenko, L., and Timchenko, N. (2025). DNAJB1-PKAc Kinase Is Expressed in Young Patients with Pediatric Liver Cancers and Enhances Carcinogenic Pathways. Cancers, 17.","DOI":"10.3390\/cancers17010083"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"E5916","DOI":"10.1073\/pnas.1424894112","article-title":"Transcriptomic Characterization of Fibrolamellar Hepatocellular Carcinoma","volume":"112","author":"Simon","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1001\/jamaoncol.2015.5699","article-title":"Diagnostic Yield of Clinical Tumor and Germline Whole-Exome Sequencing for Children with Solid Tumors","volume":"2","author":"Parsons","year":"2016","journal-title":"JAMA Oncol."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Haines, K., Sarabia, S.F., Alvarez, K.R., Tomlinson, G., Vasudevan, S.A., Heczey, A.A., Roy, A., Finegold, M.J., Parsons, D.W., and Plon, S.E. (2019). Characterization of Pediatric Hepatocellular Carcinoma Reveals Genomic Heterogeneity and Diverse Signaling Pathway Activation. Pediatr. 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Commun."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2626","DOI":"10.1158\/0008-5472.CAN-23-4110","article-title":"Increased Protein Kinase A Activity Induces Fibrolamellar Hepatocellular Carcinoma Features Independent of DNAJB1","volume":"84","author":"Shirani","year":"2024","journal-title":"Cancer Res."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Saltsman, J.A., Hammond, W.J., Narayan, N.J.C., Requena, D., Gehart, H., Lalazar, G., LaQuaglia, M.P., Clevers, H., and Simon, S. (2020). A Human Organoid Model of Aggressive Hepatoblastoma for Disease Modeling and Drug Testing. 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