Fibrolamellar carcinoma (FLC), a truly unique and uncommon variation of hepatocellular carcinoma (HCC), accounting for barely 1%–9% of all HCC cases. Fibrolamellar carcinoma, an unclear malignancy, appears to be more commonly observed in youthful individuals without any preexisting liver conditions. The nomenclature "fibrolamellar" originates from the presence of dense fibrous collagen bands enveloping the neoplastic cells. Contrary to hepatocellular carcinoma (HCC), cirrhosis and viral hepatitis infection do not serve as predisposing factors for fibrolamellar carcinoma (FLC). Additionally, FLC is not typically associated with increased levels of serum alpha-fetoprotein. Patients with FLC frequently manifest with nonspecific abdominal discomfort, queasiness, general discomfort, and decreased in body mass. Surgical intervention, specifically resection or liver transplantation, serves as the cornerstone of treatment and represents the sole potentially curative avenue. FLCs, or fibrolamellar carcinomas, have historically exhibited lower responsiveness to chemotherapy compared to conventional hepatocellular carcinomas (HCC). Nevertheless, it is important to note that in cases of advanced FLCs, the utilization of multi-modality treatments has shown promising effectiveness. The purpose of this review is to explain the clinical characteristics, diagnostic techniques, and therapeutic approaches for this uncommon tumor in order to enhance the knowledge of healthcare professionals.
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