When the hilum is affected, a resection of extrahepatic bile ducts combined with a formal/extended liver resection gives a better result than an isolated resection of bile ducts. Segment one should be removed to ensure that the procedure is radical. It is also possible that a routine resection of the portal vein, especially on the right side, will give a better result.
An isolated resection of bile ducts does not improve long-term survival (> 5 years).
Mortality related to this procedure is relatively high. Higher than normal morbidity is also reported after the surgical procedure. It is therefore recommended that surgical treatment is centralized.
A selected group of patients with bile duct cancer can be offered a liver transplantation accordance with the Mayo Protocol (8,9). This protocol combines preoperative external radiation therapy and brachytherapy together with chemotherapy (5-FU) before the liver transplant. The results show that patients with hilar cholangiocarcinomas without extrahepatic metastases have a similar five year survival as patients with benign liver diseases. A liver transplantation program for this selected group of cholangiocarcinomas started in 2009 in Norway. Patients to be considered must satisfy the following criteria:
- Diagnosis of irresectable hilar cholangiocarcinoma with intraluminal brush cytology, intraluminal biopsy, or carcinoma antigen (CA) 19.9 higher than 100 ng/ml combined with malignant stricture detected radiologically. Percutaneous biopsy is contraindicated.
- Largest diameter of tumor < 30 mm
- No sign of local local recurrence determined by CT or MRI taken within 3 weeks before assessment at transplant meeting.
- No sign of extrahepatic tumor growth assessed with CT or MRI and PET (thorax, abdomen, pelvis) taken within 3 weeks before assessment of transplant meeting. Skeletal scintigraphy should be performed.
- Age 18–65 years
- Good performance status, ECOG 0 or 1
- Satisfactory blood tests Hb > 9 g/dl, neutrophiles > 1.0 trc > 50, creatinine < 1.25 x upper reference value.
- A negative staging laparoscopy entailing retrieval of lymph nodes from the hepatoduodenal ligament for histological examination. This must be completed before start of radiation therapy.
- Completed work-up and approval for liver transplantation.
- No sign of lung metastasis assessed by CT of thorax immediately before the transplantation.
- No sign of extrahepatic tumor growth at peroperative lymph node dissection.
For tumors in stage 1 (T1a), a cholecystectomy is sufficient treatment to cure the disease.
For stage 2 tumors (T1b), long-term survival varies if only a cholecystectomy is performed. The gallbladder wall is often thin and it may be difficult to determine whether there is invasion. It is therefore recommended to perform an extended cholecystectomy for this condition. An extended cholecystectomy includes:
- Liver resection (segment 4b and 5)
- Extrahepatic resection of bile ducts with removal of lymph nodes in the hepatoduodenal ligament
- A hepatico-jejunostomy Roux-Y is installed with confluence from the right and left bile duct
For stage 3, an extended cholecystectomy is also performed. As in stage 2, an extrahepatic resection of bile ducts with removal of lymph nodes is performed in the hepatoduodenal ligament and a hepatico-jejunostomy is prepared.
For stage 4a, the same treatment plan is followed as in stage 3. A formal or extended liver resection is added.
For stage 4b, the disease is too advanced for surgical treatment to prolong survival.
Gallbladder cancer diagnosed at the histological examination
Patients who had their cancer diagnosis at the histological examination of the cholecystectomy specimen, should be assessed for reoperation. The depth of the tumor should be determined microscopically and the treatment must be determined according to this. Before the operation, the patient must be assessed by image diagnostics. If the cystic duct is not free of tumor growth, it is recommended to remove the extrahepatic bile ducts with installation of a hepatico-jejunostomy Roux-Y at the confluence from the bile ducts. At the same time, lymph node removal in the hepatoduodenal ligament should be carried out. There is consensus that if the gallbladder is removed by laparoscopy without a bag, the incision through the abdominal wall must be excised. Otherwise, all of the port sites are excised, however the benefit of this has not been conclusively documented.