Fibrolamellar hepatocellular carcinoma at a tertiary centre in South Africa: a case series.

BACKGROUND: Fibrolamellar carcinoma (FLC) is an uncommon malignant tumour of hepatocyte origin that differs from hepatocellular carcinoma (HCC) in aetiology, demographics, condition of the affected liver, and tumour markers. Controversy exists whether FLC demonstrates a more favourable prognosis than typical HCC. A review of existing literature reveals a dearth of FLC data from the African continent. METHODS: We utilised the prospective liver resection database at Groote Schuur Hospital to identify all patients who underwent surgery for FLC between 1990 and 2008. RESULTS: Seven patients (median age 21 years, range 19 - 42, 5 men, 2 women) underwent surgery for FLC. No patient had underlying liver disease or an elevated alpha fetoprotein (AFP) at either initial presentation or recurrence. Six patients had a solitary tumour at diagnosis (mean largest diameter = 12cm), and underwent left hepatectomy (N=2), right hepatectomy (N=1), extended right hepatectomy (N=1), right hepatectomy (N=1), extended right hepatectomy (N=1), and segmentectomies (N=2). Three patients underwent a portal lymphadenectomy for regional lymphatic tumour involvement. One patient with advanced extrahepatic portal nodal metastasis was unresectable. No peri-operative deaths occurred. Recurrence occurred post resection in all 6 patients. Median overall survival was 60 months, and overall 5-year survival was 4 out of 7 (57%). Post-resection survival (N=6) was 61 months, with a 5-year survival rate of 4 out of 6 (67%). The patient with unresectable disease survived 38 months after tumour embolisation with Lipiodol. CONCLUSION: Our series suggests that despite (i) a high resection rate of solitary lesions with clear tumour resection margins, and (ii) absence of underlying liver disease, FLC has a high recurrence rate with an ultimately poor clinical outcome. These findings concur with recent international experience of FLC. experience of FLC.