Cholangiocarcinoma: treatment, survival rates and surgery

Cholangiocarcinoma is a group of cancers that can form in the bile ducts that carry the digestive fluid bile.

We spoke to the highly experienced consultant general surgeon, Mr Martin Sinclair, to find out about the different types of cholangiocarcinoma. He talked us through the treatment options, the survival rate and how dangerous surgery is.

 

What is cholangiocarcinoma?

Cholangiocarcinoma is a rare cancerous tumour of the bile ducts which affects around 900 people annually in the United Kingdom. Bile from the liver drains into the bile ducts, is stored temporarily in the gallbladder, and is released into part of the small intestine called the duodenum. Most people who develop this condition are over 50 years of age although it can affect younger patients if they have underlying conditions such as primary sclerosing cholangitis.
 

There are different types of cholangiocarcinoma:

• Intrahepatic cholangiocarcinoma (within the small ducts inside the liver)
• Hilar cholangiocarcinoma (where the bile duct splits into left and right) – “Klatskin tumour”
• Distal cholangiocarcinoma (just before entering the duodenum, can mimic pancreatic cancer)
   

Surgical removal is the only chance of effective cure but other treatments that may be used if this is not possible include:
 

• Endoscopic stenting (insertion of an expanding metal tube through the blockage) using a telescope via the mouth under sedation
• Stenting through the liver via the skin (using local anaesthetic)
• Radiation
• Chemotherapy
   

What are the most common cholangiocarcinoma symptoms?

Patients often present with a combination of the following symptoms:

• Jaundice (Yellow tinge of skin or whites of the eyes)
• Itching of the skin which can be severe (Caused by bile salts)
• Dark urine
• Pale bowel motions
• Weight loss
• Abdominal pain
• Tiredness
   

What is the survival rate of cholangiocarcinoma?

Between 15 and 50% of patients are suitable for surgical resection depending on the site of the tumour. Patients with tumours towards the bottom of the bile duct, where it enters the intestine, have a greater chance of resection. If surgical removal is clear of cancer a 5-year survival of 30-40% can be achieved. Patients who are not suitable for surgery may be treated with supportive care including stenting, chemotherapy or radiotherapy. These patients will also need palliative care as deterioration can be rapid and survival may be less than one year although some patients survive longer.
 

How is cholangiocarcinoma treated?

Surgery is the main curative treatment for patients with cholangiocarcinoma. These are major operations which may include removal of important adjacent organs including:
 

• Part of the liver
• Duodenum and head of pancreas
• Major blood vessels to the liver
   

Some patients will need stent placement prior to surgery. Other procedures to enlarge part of the liver are sometimes performed. Portal vein embolization blocks the blood supply to the part of the liver that will be removed allowing the remaining liver to enlarge. This ensures enough functioning liver is left after surgery.
 

Chemotherapy and radiotherapy may be needed after surgery in some patients to improve the chances of cure. A multidisciplinary team (MDT) of clinicians including specialist doctors, nurses and allied healthcare professionals will help the patient to decide on the best options.
 

If surgery is not possible and the tumour is not removable, palliative treatment will be considered. The MDT will decide the best options for treating the patient and a shared decision with patient and family will be reached.
 

Patients who are jaundiced will usually benefit from insertion of a metallic expanding stent. This is a tube that is placed through the tumor and allows bile to drain through the tube into the intestine and overcomes the blockage. This gradually expands over a few days making the diameter of the tube larger as the tumour stretches. The stent can be inserted endoscopically or radiologically through the liver if the endoscopic route is not successful.
 

Radiotherapy and / or chemotherapy may be used as palliation in some cases. This has been shown to prolong survival and an oncologist will discuss the pros and cons of these treatments.
 

Other experimental treatments including photodynamic therapy are being trialed in some centres.
 

Is surgery for cholangiocarcinoma dangerous?

Surgery for bile duct cancer is very major surgery. The average risk of procedures for this disease are as follows:
 

Pancreaticoduodenectomy or Whipple’s procedure (removal of pancreas, duodenum, lower bile duct)

Mortality 1.5%

Complications 1:3

Major complications 1:4
 

Liver resection (Hepatectomy) and bowel anastomosis (hepaticojejunostomy)

Mortality 2-3%

Complications 1:3

Major complications 30%
 

Your surgeon will discuss the pros and cons of surgery in your individual case taking into account any underlying health risks you may have. To aid shared decision-making they will usually use a risk scoring system, such as the American College of Surgeons Risk Score, and a frailty assessment, such as the Rockwood Score.
 

For more information on the treatment of cholangiocarcinoma, you may like to get in contact with a skilled consultant general surgeon such as Mr Martin Sinclair . Visit his Top Doctors profile today for more details.