Cholangiocarcinomas should be resected if there are no distant metastases and no irreparable involvement of the hepatic artery and portal vein. Lower third tumours can be treated by a standard pancreatico-duodenectomy and middle third tumours by resection of the bile duct. It is advisable to perform a full lymph-node dissection, including the retro-pancreatic and para-aortic stations, as cure has been reported even with hilar nodal metastases.

The results of surgery are good, with microscopically curative resections possible in over 75% of the patients, and five-year survival of approximately 50% in the best series.

In cases of unresectable tumours, cholestatic jaundice requires surgical, endoscopic, or percutaneous palliation. Cholangiocarcinomas often grow slowly, and in young patients surgical bypass is preferred. A gastroenterostomy can also be performed to prevent duodenal obstruction. Endoscopic stenting is less invasive but the failure rate is high and the prostheses often need to be replaced (polyethylene tubes) or unblocked (Wallstent). The percutaneous transhepatic route is generally superior because of better access to negotiate the obstruction.

The results of trials comparing the different modalities are inconclusive. Percutaneous transhepatic stenting is definitely preferred in cases of recurrence after surgery. Although cholangiocarcinomas are not generally considered chemosensitive or radiosensitive, responses to chemotherapy with cisplatin and 5-fluorouracil and folinic acid have been observed, occasionally allowing resection of apparently unresectable tumours. Newer agents such as gemcitabine and irinote-can are under investigation.

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