Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments

Abstract

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a malignant liver tumor which is thought to be a variant of conventional hepatocellular carcinoma (HCC). It accounts for a small proportion of HCC cases and occurs in a distinctly different group of patients which are young and usually not in the setting of chronic liver disease. The diagnosis of FL-HCC requires the integration of clinical information, imaging studies, and histology. In terms of the treatment options, the only potentially curative treatment option for patients who have resectable disease is surgery either liver resection (LR) or liver transplantation (LT). When performed in a context of aggressive therapy, long-term outcomes after surgery, particularly liver resection for FL-HCC, were favorable. The clinical outcome of patients with unresectable disease is suboptimal with median survival of less than 12 months. The aim of this review is to update the available evidence on diagnosis, treatment options, outcome predictors, and recent developments of patients with this rare disease and to provide a summarized overview of the available literature.

Progression after Immunotherapy for Fibrolamellar Carcinoma

“…In advanced stages, prognosis is very poor with a median survival of 12 months and only minor advances in FLC-specific systemic therapies in recent years [6, 17]. As there are only few patients with this rare disease, controlled studies regarding the treatment of advanced FLC are limited and therapeutic approaches are therefore either adapted from typical HCC or based on small treatment series or case reports.…”

“…There are no specific recommendations or guidelines. While several studies show a benefit of different systemic chemotherapies, these reports are based on small case series and have shown only limited long-term efficiency [17]. Targeted tumor therapies have not been investigated in FLC and a retrospective analysis of 10 patients receiving sorafenib indicated limited efficiency [13].…”

Favorable response to mammalian target of rapamycin inhibition in a young patient with unresectable fibrolamellar carcinoma of the liver

“…Current treatment approaches to FLC with a potentially curative intention in the resectable stage are based on surgery. (2) In the palliative setting, attributed to the rarity of this tumor entity and thus the lack of large clinical trials, treatment approaches are often chosen based on data derived from studies including conventional hepatocellular carcinoma (HCC). However, recent genomic profiling has revealed fundamental mechanistic differences between FLC and conventional HCC, suggesting FLC being a unique tumor entity rather than a subtype of HCC.…”

Mujer joven con hepatocarcinoma fibrolamelar metastásico; citorreducción y HIPEC. A propósito de un caso

“…El tratamiento descrito en la literatura del hepatocarcinoma fibrolamelar se ha basado en el mismo tratamiento de hepatocarcinoma hepatocelular. El tratamiento quirúrgico con resección hepática es un tratamiento seguro principalmente en hígados no cirróticos y es la única opción curativa, por tal motivo, actualmente es el tratamiento de elección en estos casos 6,7 .…”