Current Advances in the Treatment of Fibrolamellar Carcinoma of Liver. [Review]

MedStar author(s):
Citation: Journal of Hepatocellular Carcinoma. 10:745-752, 2023.PMID: 37215364Institution: MedStar Franklin Square Medical CenterForm of publication: Journal ArticleMedline article type(s): Journal Article | ReviewSubject headings: Year: 2023ISSN:
  • 2253-5969
Name of journal: Journal of hepatocellular carcinomaAbstract: Fibrolamellar carcinoma (FLC) of the liver is a rare type of liver cancer that is prevalent in children and young adults, often less than 40 years old. The etiology is unclear. It presents without underlying liver disease with distinctive histological features such as fibrous collagen bands surrounding the tumor cells. Fusion protein DNAJB1-PRKACA is found in most of the cases. The prognosis of FLC is poor. Even though curative treatment option is surgery for a certain patient population, other treatment modalities including radiation, chemotherapy are currently being used without significant improvement of overall survival. Recently, targeted therapy and immunotherapy have been studied which may provide survival advantage in the future. This review sought to compile data from clinical trials and case reports/series to outline the current state of FLC treatment. Copyright © 2023 Gummadi et al.All authors: Gummadi J, Wang X, Xie CFiscal year: FY2023Digital Object Identifier: ORCID: Date added to catalog: 2023-06-26
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Journal Article MedStar Authors Catalog Article 37215364 Available 37215364

Fibrolamellar carcinoma (FLC) of the liver is a rare type of liver cancer that is prevalent in children and young adults, often less than 40 years old. The etiology is unclear. It presents without underlying liver disease with distinctive histological features such as fibrous collagen bands surrounding the tumor cells. Fusion protein DNAJB1-PRKACA is found in most of the cases. The prognosis of FLC is poor. Even though curative treatment option is surgery for a certain patient population, other treatment modalities including radiation, chemotherapy are currently being used without significant improvement of overall survival. Recently, targeted therapy and immunotherapy have been studied which may provide survival advantage in the future. This review sought to compile data from clinical trials and case reports/series to outline the current state of FLC treatment. Copyright © 2023 Gummadi et al.

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