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The aim of this retrospective study was to analyse outcome in 31 European patients operated on for peripheral cholangiocarcinoma between 1988 and 1995 (hilar cholangiocarcinoma was excluded). Before 1992, in addition to conventional resection, patients with irresectable tumour and with no extrahepatic metastasis underwent total hepatectomy with liver transplantation. Mild abdominal pain was the most frequent presenting clinical sign (11 of 31 patients) and jaundice was present in only four patients. Preoperative histological findings were available for 20 patients but the diagnosis was correct in only eight of these. Nineteen patients had curative surgery: 17 underwent conventional resection and two had total hepatectomy with liver transplantation. Major hepatectomy was performed in 12 of 17 cases, extended to the caudate lobe in five, to the bile duct confluence in four and to the retrohepatic vena cava in one. Postoperative mortality and morbidity rates were three and seven of 19 patients respectively, median survival was 15 months, and actuarial 1-, 2- and 5-year survival rates were 67, 40 and 32 per cent respectively. Twelve patients had only exploratory laparotomy because of the presence of extrahepatic metastasis or irresectable tumour. The invasive nature of peripheral cholangiocarcinoma can explain the limited number of resectable cases and the particularly unfavourable prognosis. Total hepatectomy does not provide survival benefit. Conventional surgery remains the only effective treatment, even for patients with advanced stage tumours.

The results in this highly selected group seem to justify an attempt at reresection whenever possible. Long-term results may be improved by using adjuvant treatment.