Bile duct tumours are potentially curable by resection, although overall only 30% with intrahepatic and perihilar cholangiocarcinoma can undergo curative-intent resection, and 40% with distal disease. Careful evaluation by accurate preoperative staging is essential. Early consultation with those familiar with their management is advised.

Tumours involving the bifurcation and below can often be resected. If an unsuspected, but potentially resectable, bile duct cancer is found at operation, the unprepared surgeon should terminate the procedure and immediately refer the patient. Dissection of the area of the porta hepatis should be kept to a minimum. The liver should be closely examined for the presence of metastases and any enlarged lymph nodes in the area should be aspirated for cytology.

Perihilar tumours require biliary resection ± major liver resection ± major vascular and biliary reconstruction. Operative mortality is generally in the 5-10% range, and median survival is 24 months. Five-year survival is seen in approximately 25%.

Pancreaticoduodenectomy with lymph node dissection is the procedure of choice for distal cholangiocarcinomas and is associated with a better prognosis than that seen for pancreatic adenocarcinoma with a median survival of 22-33 months, and 5 year survival of 14-40%.

Partial hepatectomy is required in intrahepatic cholangiocarcinomas. Available outcome data are sparse, but median survival ranges from 12-59 months depending on the case series.

Many patients with apparently advanced disease can enjoy a prolonged period of palliation by combined surgical and radiologic procedures. Stenting, either by ERCP or percutaneously, can be particularly beneficial.

Many American centres use neo-adjuvant or adjuvant chemotherapy, radiotherapy or chemo-radiotherapy (usually with 5-fluorouracil) for node positive or margin positive disease. The value of these practices is unknown since they have not been subjected to well designed clinical trials, and there are no compelling data from the available case series.

The role of chemotherapy overall remains undefined. There have been no well-designed adjuvant chemotherapy trials. The experience with palliative chemotherapy is largely confined to small Phase II studies; a number agents appear to have some activity, most notably gemcitabine, cisplatin and 5-fluorouracil. There is one small randomised trial that suggests that chemotherapy with 5-fluorouracil and leucovorin can provide some palliative benefit in patients with advanced disease.¹

The role of radiotherapy (including endoluminal brachytherapy) in the palliative setting is unclear.

Reference:

1. Glimelius B, Hoffman K, Sjoden P.O, Chemotherapy improves survival and quality of life in advanced pancreatic and biliary cancer. Ann Oncol 1996;7: 593.