Abstract 196P
Background
Fibrolamellar carcinoma (FLC), which comprises 1%–9% of hepatocellular carcinoma (HCC), is a very rare liver cancer that displays unique clinical and biological features from conventional HCC. The majority of FLC cases affect young patients with no primary liver disease. There are no identified risk factors for FLC.
Methods
Retrospective, observational study. Included patients with diagnosis of FLC treated at the National Cancer Institute in Mexico City between 2009 and 2022. Statistical analysis required: X2 and T-test, Kaplan-Meier and Cox Regression.
Results
The study included 323 patients diagnosed with HCC. FLC was reported in 11.4% (n=36) of the patients, the median age was 23 (16 – 58) years. Among them 17 patients were males and 19 were females. At diagnosis, 27 patients (75%) had metastatic disease, most frequently to the lymph nodes (36%) lungs (33%), peritoneum (17%) and bone (8%). 12 patients presented metastasis in more than one site. An abdominal palpable mass was the most common symptom at diagnosis (69%). Mean alpha-fetoprotein level was 3 (0-999). Mean tumor size was 12 cm (4-21 cm). 80% had lymph node involvement and 47% had macrovascular invasion. Regarding management, 9 patients were treated with surgery, 4 with radioablation and 19 with systemic treatment (12 patients with fluoropyrimidine or platinum-based doublet, 6 patients with sorafenib and one patient with atezolizumab plus bevacizumab). Only one patient received palliative care. The progression free survival (PFS) was 3.4 months (2-10 months) and the median overall survival (OS) was 16 months. In patients with recurrent disease (7%), the median time to recurrence was 28.3 months with a 5-year OS of 64%. At Cox Regression analysis only surgery (p=0.006; HR 0.54, 95% CI 0.52-0.78) and baseline neutrophil–lymphocyte ratio (NLR) < 3 (p < 0.02; HR 0.58, 95% CI 0.67-0.85) remained as independent predictors of OS.
Conclusions
FLC is a rare cancer; this analysis shows prognostic factors in advanced disease associated with poor survival. The lack of an actionable therapeutic target is an unmet need that requires assessment in the future to improve outcomes in this population.
Legal entity responsible for the study
C. Diaz Romero.
Funding
Has not received any funding.
Disclosure
All authors have declared no conflicts of interest.