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
Fleifil et al. recently reported the <i>DNAJB1</i>::<i>PRKACA</i> 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 <i>DNAJB1</i>::<i>PRKACA</i> 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.
Authors
- Waqar Arif (ORCID: https://orcid.org/0000-0002-9252-0200)
- Théo Hirsch
- Sanford M. Simon (ORCID: https://orcid.org/0000-0002-8615-4224)
- Mark Yarchoan (ORCID: https://orcid.org/0000-0003-4401-0748)
- Dolores H. López Terrada (ORCID: https://orcid.org/0000-0002-6584-420X)
- Allison Fa. O’Neill
- Juan Putra
- Jessica Zucman-Rossi
Institutions
- Boston Children's Hospital (US)
- Harvard University (US)
- Johns Hopkins University (US)
- Inserm (FR)
- Université Paris Cité (FR)
- Sorbonne Université (FR)
- Centre de Recherche des Cordeliers (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Hôpital Européen (FR)
- Dana-Farber Cancer Institute (US)
- Texas Children's Hospital (US)
- Sidney Kimmel Comprehensive Cancer Center (US)
- Rockefeller University (US)
Publication Details
- Journal
- Cancers
- Published
- 2026-03-09
- DOI
- https://doi.org/10.3390/cancers18050877
- Citations
- 1
- Primary Topic
- Hepatocellular Carcinoma Treatment and Prognosis
- Type
- article
- Field-Weighted Citation Impact
- 23.38
Funders
- Institut National Du Cancer
- National Institutes of Health
- National Cancer Institute