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March 12, 2026Cancers3 citationsOpen Access

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

WAWaqar ArifSSSanford M. SimonAOAllison Fa. O’Neill

Key Points

  • To confirm the presence of DNAJB1::PRKACA fusion in hepatoblastoma and to assess its diagnostic relevance.
  • Re-examined data using 225 assays.
  • Analyzed 148 hepatoblastoma samples from five institutions.
  • Compared findings with fibrolamellar carcinoma samples.
  • Detected no DNAJB1::PRKACA fusion in any hepatoblastoma cases.
  • Found 286 out of 290 fibrolamellar carcinoma samples positive for the fusion.
  • Confirmed specificity of the fusion to fibrolamellar carcinoma.

Abstract

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.

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Cite This Study

Arif et al. (2026) studied this question.

synapsesocial.com/papers/69b257ec96eeacc4fcec70achttps://doi.org/10.3390/cancers18050877
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Also Consider

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