ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2015
DNAJB1-PRKACA is specific for fibrolamellar carcinoma.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 94 papers.
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Who cites it
94 citing papers in PubMed, 181 citations in OpenAlex.
- Fibrolamellar Carcinoma in the Molecular Era: From DNAJB1::PRKACA Biology to Precision Therapeutic Strategies.Journal of gastrointestinal cancer · 2026Review
- Clinical guideline for the diagnosis and treatment of fibrolamellar carcinoma.Hepatology (Baltimore, Md.) · 2026Article
- Hepatocellular carcinoma in a child with hereditary tyrosinemia type I despite early NTBC therapy: When should we suspect it?Radiology case reports · 2026Article
- Cancer-associated fusion transcripts: mechanisms, functional roles, and clinical implications.Clinical and experimental medicine · 2026Review
- Global Re-Analysis Confirms Absence of theCancers · 2026Article
- Exceptionally Long Recurrence-Free Survival in a Male Patient With Fibrolamellar Hepatocellular Carcinoma.Case reports in hepatology · 2026Article
- Serum Procalcitonin: A Novel Tumor Biomarker for Diagnosis and Follow-Up in Fibrolamellar Hepatocellular Carcinoma.medRxiv : the preprint server for health sciences · 2025Article
- β-Catenin-Cohesin Ring-CEGRs/ALCDs Axis Activation Contributes to the Development of Hepatoblastoma and Fibrolamellar HCC.Molecular cancer research : MCR · 2025Article
- Fibrolamellar hepatocellular carcinoma: Advances, challenges and opportunities in a rare malignancy.World journal of gastrointestinal surgery · 2025Review
- Chemical Evolution of Aplithianine Class of Serine/Threonine Kinase Inhibitors.Journal of medicinal chemistry · 2025Article
- Liver cancer multiomics reveals diverse protein kinase A disruptions convergently produce fibrolamellar hepatocellular carcinoma.Nature communications · 2024Article
- Distinct phases of cellular signaling revealed by time-resolved protein synthesis.Nature chemical biology · 2024Article
- Organoid as a promising tool for primary liver cancer research: a comprehensive review.Cell & bioscience · 2024Review
- Article
- Models of fibrolamellar carcinomas, tools for evaluation of a new era of treatments.Frontiers in immunology · 2024Review
- Article
- Liver-directed therapies for fibrolamellar carcinoma: A single-center experience.Oncology research · 2024Article
- Article
- Recurrent Somatic Copy Number Alterations and Their Association with Oncogene Expression Levels in High-Grade Ovarian Serous Carcinoma.Life (Basel, Switzerland) · 2023Article
- The mystery of phospho-Drp1 with four adaptors in cell cycle: when mitochondrial fission couples to cell fate decisions.Cell cycle (Georgetown, Tex.) · 2023Article
34 more citing papers are in PubMed but not listed here.
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13 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Fibrolamellar carcinoma is a distinct subtype of hepatocellular carcinoma that predominantly affects young patients without underlying cirrhosis. A recurrent DNAJB1-PRKACA fusion has recently been reported in fibrolamellar carcinomas. To determine the specificity of this fusion and to develop routinely available clinical methods of detection, we developed an RT-PCR assay for paraffin-embedded tissues and a FISH probe for detection of the rearrangements of the PRKACA locus. We also developed an RNA in situ hybridization assay to assess expression levels of the total chimeric transcript and wild-type transcripts. A total of 106 primary liver tumors were studied by RT-PCR, including 26 fibrolamellar carcinomas (4 of which were metastases to the abdominal wall or lymph nodes), 25 conventional hepatocellular carcinomas, 25 cholangiocarcinomas, 25 hepatic adenomas, and 5 hepatoblastomas. RT-PCR was successful in 92% of tested fibrolamellar carcinoma cases (24/26) and the DNAJB1-PRKACA fusion transcript was found in all fibrolamellar carcinomas but not in other tumor types. FISH was tested in 19 fibrolamellar carcinomas and in 6 scirrhous hepatocellular carcinomas, which can closely mimic fibrolamellar carcinoma. Rearrangements of the PRKACA locus was seen in all 19 fibrolamellar carcinoma specimens, but in none of the scirrhous hepatocellular carcinomas. Finally, a RNA in situ hybridization strategy was positive in 7/7 successfully hybridized cases, and showed mRNA over-expression in all of the fibrolamellar carcinomas. In addition, the stromal cells embedded in the characteristic intratumoral fibrosis of fibrolamellar carcinomas and the background liver tissues were negative for the DNAJB1-PRKACA fusion by all tested methods. In conclusion, detection of DNAJB1-PRKACA is a very sensitive and specific finding in support of the diagnosis of fibrolamellar carcinoma.
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