Surgical treatment of 32 patients with peripheral intrahepatic cholangiocarcinoma

BACKGROUND: Peripheral intrahepatic cholangiocarcinoma (PIC) is an intrahepatic primary liver neoplasm which is clinicopathologically distinct from hepatocellular carcinoma and major duct cholangiocarcinoma. The clinical outcome after resection of these rare tumours is not well documented. METHODS: Review of the hepatic database and tumour registry at Memorial Sloan-Kettering Cancer Center identified 32 cases of PIC resected for cure over a 23-year period. Intrahepatic cholangiocarcinomas with major bile duct involvement were excluded from this analysis. Demographics, pathological features, biochemical markers, operative results and survival were analysed. RESULTS: The majority of patients presented with abdominal pain (n=19). Only two patients had pathological evidence of hepatic cirrhosis. Serum marker levels included 7-fetoprotein (AFP; median 3.7 (range 0-225) ng/ml) and carcinoembryonic antigen (CEA; median 1-6 (range 0-30) ng/ ml). Type of hepatic resection included: wedge (n=2), lobectomy (n=14) and extended lobectomy (n=16). There was one postoperative death. Median follow-up time was 27 months. Median survival was 59 months with an actuarial 5-year survival of 42 per cent. Vascular invasion and intrahepatic satellite lesions were predictors of worse survival (P < 0.05). CONCLUSION: PIC is a rare hepatic primary tumour, which usually presents in non-cirrhotic livers with a normal serum AFP and CEA level. In selected patients, complete surgical resection can be performed safely and is associated with long-term survival.