Current Advances in the Treatment of Fibrolamellar Carcinoma of Liver

Abstract

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.

Fibrolamellar hepatocellular carcinoma: a case report and gene analysis

“…In our case, lymph node recurrence also occurred after hepatectomy, resulting in lymphadenectomy. Gummadi et al stated that lymph node dissection may contribute to improved prognosis [ 19 ]. Based on these reports, we suggest that the surgical strategy should consider whether lymph node dissection should be performed in addition to hepatectomy.…”

cAMP-PKA/EPAC signaling and cancer: the interplay in tumor microenvironment

“…cAMP signaling has complex roles in liver tumorigenesis. While DNAJB1-PRKACA fusion drives oncogenic pathways and induces FLC [ 105 ], the mixed-FLC/HCC tumors with inactivating mutations or translocations in the gene encoding BRCA1-associated protein-1 (BAP1) also harbor a chromosome gain of PRKACA and a loss of the inhibitory regulatory subunit PRKAR2A, thus exhibiting addiction to PKA activation [ 106 ]. Moreover, GNAS-activating mutation promotes liver tumorigenesis by upregulating cAMP/JAK/STAT3 signaling [ 107 ].…”

Long-term survival and remarkably durable treatment response in a case of inoperable fibrolamellar carcinoma of the liver — a comprehensive analysis of an 8-year chemotherapeutic journey

“…Several studies described locoregional therapies, such as transarterial chemoembolization (TACE) and transarterial radioembolization (TARE), as viable options in disease treatment, but these recommendations are based on low-grade evidence [15][16][17]. Liver transplant ought to be considered in patients with unresectable tumors and without extrahepatic disease [18]. Systemic treatment with HCC regimens is considered a viable option for advanced disease [18].…”

“…Liver transplant ought to be considered in patients with unresectable tumors and without extrahepatic disease [18]. Systemic treatment with HCC regimens is considered a viable option for advanced disease [18]. There are few data on the routine use of radiation therapy in FLHCC.…”