Abstract
Fibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer that affects adolescents and young adults without underlying liver disease. Surgery remains the mainstay of therapy; however, most patients are either not surgical candidates or suffer from recurrence. There is no approved systemic therapy and the overall survival remains poor. Historically classified as a subtype of hepatocellular carcinoma (HCC), FLC has a unique clinical, histological, and molecular presentation. At the genomic level, FLC contains a single 400kB deletion in chromosome 19, leading to a functional DNAJB1-PRKACA fusion protein. In this review, we detail the recent advances in our understanding of the molecular underpinnings of FLC and outline the current knowledge gaps.
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
MeSH terms
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Animals
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Antineoplastic Agents / therapeutic use
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Biomarkers, Tumor / antagonists & inhibitors
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Biomarkers, Tumor / genetics*
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Biomarkers, Tumor / metabolism
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Carcinoma, Hepatocellular / enzymology
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Carcinoma, Hepatocellular / genetics*
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Carcinoma, Hepatocellular / pathology
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Carcinoma, Hepatocellular / therapy
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Cell Transformation, Neoplastic / genetics*
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Cell Transformation, Neoplastic / metabolism
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Cell Transformation, Neoplastic / pathology
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Chromosomes, Human, Pair 19
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / antagonists & inhibitors
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / genetics
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits / metabolism
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Gene Fusion
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Genetic Predisposition to Disease
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HSP40 Heat-Shock Proteins / genetics
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Humans
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Molecular Targeted Therapy
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Neoplasm Recurrence, Local
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Phenotype
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Protein Kinase Inhibitors / therapeutic use
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Treatment Outcome
Substances
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Antineoplastic Agents
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Biomarkers, Tumor
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DNAJB1 protein, human
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HSP40 Heat-Shock Proteins
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Protein Kinase Inhibitors
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Cyclic AMP-Dependent Protein Kinase Catalytic Subunits
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PRKACA protein, human
Supplementary concepts
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Fibrolamellar hepatocellular carcinoma