Journal of Carcinogenesis & Mutagenesis

Abstract

Genetic Advances in Fibrolamellar Hepatocellular Carcinoma: Comparison with Focal Nodular Hyperplasia of the Liver

Kazumi Fujioka*

Fibrolamellar Hepatocellular Carcinoma (FL-HCC) is more frequent in non-cirrhotic young adult populations and surgically complete resection is regarded as one of the only curative therapy options. Pathologically the central stellate scar and conspicuous fibrous tissue may be observed in up to 75% of FL-HCC, while characteristic central stellate scar with radiating fibrous septa is shown in Focal Nodular Hyperplasia (FNH) suggesting that the differential diagnosis of two entities is very important. The recent report described the trends in FNH along with oxaliplatin- induced this entity. In this article, the current knowledge and trends of genetic advances in FL-HCC along with comparison with FNH of the liver have been reviewed. Additionally, the author described the differential diagnosis of two entities emphasizing on the central stellate scar. It is plausible that patients with atypical FNH on radiological features should be examined at a specialized hepatobiliary center. The new technology, Super-Resolution Ultrasonography (US) without contrast agent may be a potential tool of detection of spoke wheel sign in FNH suggesting that this finding may contribute to the differential diagnosis of two entities. FL-HCC is associated with DNAJB1-PRKACA gene fusion having tumorigenesis, while unique endothelial cell expressed SOST of fibrous septa in FNH with no therapy may contribute to promote the fibrosis process through PDGFB/PDGFRB pathway suggesting the different genetic nature of two entities. For immunotherapy, based on the evidence, personalized DNAJB1-PRKACA-derived peptide vaccine may be an effective treatment in a single FL-HCC patient. Though further studies are needed to validate for immunotherapy in FL-HCC.

Published Date: 2023-09-25; Received Date: 2023-08-23