Fibrolamellar hepatocellular carcinoma – Prolonged survival with multimodality therapy

AimWe report the clinical outcome for a series of ten patients with fibrolamellar hepatocellular treated with resection followed by close surveillance and aggressive management of relapse.MethodsThe case notes for all patients treated at this institution since 1982 were reviewed and details of initial stage and management were extracted along with investigations and treatment of relapse. Time to relapse, overall survival and post-relapse survival were analysed.ResultsRelapse occurred in all ten cases at a median of 2.2 (95% CI 0.9–2.7) years but, with a combination of re-resection, systemic chemotherapy and radiotherapy, the overall median survival was 9.3 (95% CI 3.0–18.5) years. One patient was disease free eight years after two resections for recurrent disease. Two of nine patients had a partial response to cisplatin and fluorouracil while three had stable disease. FDG-PET was positive for recurrence in three of four cases of relapse, and in one case detected recurrence in advance of CT.ConclusionThe early detection of relapse combined with multimodality therapy results in prolonged survival. Further improvements in systemic therapy are required to improve the prognosis in this disease.