Perihilar / Proximal Tumours

These tumours are associated with a very high rate of stage III disease that renders them inoperable. Staging laparoscopy is essential to detect peritoneal deposits and to judge the extent of vascular involvement in the hilum. For the 15-20% of operable patients, resection of the tumour usually with partial hepatectomy and re-establishment of biliary drainage is accomplished by a Roux-en-Y hepatojejunostomy. This is associated with peri-operative mortality of 10%.
Positive margins are a poor prognostic feature (5 year survival < 10%) and occur in a significant number of patients. With negative margins 5 year survival is about 40%. Local failure is significantly more common in perihilar cholangiocarcinoma when compared to tumours of the gallbladder (60% vs 15%).

Adjuvant Therapies

The poor outcomes with positive margins suggests a benefit from additional local treatment. There is not prospective randomised data. Retrospective review of the SEER database demonstrate improved survival with the addition of radiotherapy (median survival 19 months instead of 8); there is likely selection bias affecting these results but radiotherapy is becoming more mainstream in this population.

Newer Therapies

Liver transplant offers a potentially curative option for patients with otherwise unresectable disease due to vascular involvement in the hilum. This has been studied in case series, with patients receiving neoadjuvant chemoradiotherapy and brachytherapy to control disease while waiting for transplant. 5 year survival rates were around 90% with this strategy.

Palliative Treatment

Unresectable cases have a median survival of 6 months. Palliation predominately involves providing a route for bile drainage, either percutaneously or with biliary stents. Other treatment options are being developed but there are no randomised studies at this time; most data comes from case series.


There is no established systemic therapy treatment. Gemcitabine is commonly used, and response rates of about 20% exist.


Patients treated with radiotherapy have a mean survival of 9 months; whether this is due to selection bias is not clear. There are no randomised studies. The usual dose is 45-50.4 Gy delivered to the primary tumour.
Brachytherapy has been used in combination with chemotherapy, external beam radiotherapy and stenting as well as brachytherapy as a single modality. Dose is 30 Gy in 6# (alone) or 20 Gy in 4# (combined). Alone, brachytherapy patients have a median survival of 10 months in reports. Uncontrolled studies show median survival from 12-24 months when brachytherapy is combined with external beam radiotherapy and 5-fluorouracil. A good recommendation would be, for fit patients, 50.4 Gy in 28 fractions with concurrent 5-fluorouracil.

Distal Bile Duct Tumours

In contrast to tumours above the union of the common hepatic and cystic ducts, distal bile duct tumours are often resectable. This is due to less likely involvement of the vessels of the porta hepatis combined with early diagnosis due to biliary obstruction, jaundice and potentially cholangitis.


Surgical resection forms the mainstay of treatment. Often, a pancreatoduodenectomy is performed with a pancreatojejunostomy and hepatojejunostomy.

Adjuvant Therapy

Most cases of periampullary cancers are pancreatic cancer and management seems to follow the guidelines for pancreatic cancer. In general, periampullary tumours have improved survival relative to other sites within the pancreas due to noticeably earlier presentation and improved resectability.