Background: Fibrolamellar liver carcinoma (FLC) is a rare subtype of liver cancer with an incompletely understood etiology. There is limited information in the literature regarding the most appropriate staging system for FLC, and data on the prognostic significance of clinical factors in patients with FLC remain contradictory and require further investigation.

Aim: To evaluate the applicability of staging systems used for operable hepatocellular carcinoma (TNM/AJCC and BCLC) in patients with fibrolamellar carcinoma and to assess the influence of clinical factors (disease stage, sex, age, and tumor size) on long-term treatment outcomes.

Materials and Methods: This retrospective study included 34 patients with FLC who underwent radical surgical treatment as the initial therapeutic approach at the N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of the Russian Federation, between 2005 and 2020.

Results: Assessment of disease extent according to the TNM-8/AJCC classification demonstrated a significant correlation between disease stage and prognosis. The longest recurrence-free survival (RFS) was observed in patients with stages II–IIIB disease, whereas the shortest RFS was observed in patients with stage IVB disease (p = 0.002 and p < 0.001, respectively). Similarly, the highest overall survival (OS) was achieved in patients with stages II–IIIB disease, while the lowest OS was observed in the stage IVB group (p = 0.050 and p < 0.001, respectively). Overall survival was not associated with patient sex. Tumor size was identified as an independent prognostic factor for both RFS and OS.

Conclusions: The TNM-8/AJCC classification appears to be the most appropriate staging system for predicting surgical outcomes in patients with FLC. The use of the Barcelona Clinic Liver Cancer (BCLC) staging system for this subtype of liver cancer appears to be of limited value. Primary tumor size, as a component of the FLC staging system, was identified as an independent prognostic factor. Patient age ≥20 years was associated with poorer overall survival.

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