Current Advances in the Treatment of Fibrolamellar Carcinoma of Liver

Abstract

Jyotsna Gummadi,1 Xin Wang,2 Changqing Xie2,3 1Department of Medicine, MedStar Franklin Square Medical Center, Baltimore, MD, 21237, USA; 2Thoracic and GI Malignancies Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, 20814, USA; 3NCI CCR Liver Cancer Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, 20814, USACorrespondence: Changqing Xie, National Institute of Health, 10 Center Drive, Building 10 3B43, Bethesda, MD, 20892, USA, Email [email protected]: 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.Keywords: fibrolamellar carcinoma of liver, immunotherapy, target therapy

Keywords

• fibrolamellar carcinoma of liver
• immunotherapy
• target therapy