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Bile Duct Cancer (Cholangiocarcinoma)

What is cholangiocarcinoma?

Cholangiocarcinoma is a cancer that arises from the cells within the bile ducts; both inside and outside the liver. When the tumors arise within the liver, the tumors can be small or large. The terms cholangiocarcinoma and bile duct cancer are often used interchangeably. This form of cancer is slightly more common in males than females and usually affects patients who are between 50-70 years of age.

Intrahepatic Cholangiocarcinoma
Intrahepatic Cholangiocarcinoma

In contrast, when the tumors arise along the bile ducts that enter the liver, the tumors tend to be smaller.

Extrahepatic tumor, intrahepatic tumor resulting in a biliary duct dilation.
A, extrahepatic tumor; B, intrahepatic tumor resulting in a biliary duct dilation

These tumors can be slow-growing tumors that spread locally via the lymphatic system, as well as involve local structures such as the blood vessels feeding the liver.

What are the symptoms of cholangiocarcinoma?

Symptoms of cholangiocarcinoma (bile duct cancer) include:

  • Jaundice
  • Abdominal pain
  • Fever
  • Weight loss
  • Weakness
  • Itching

How is cholangiocarcinoma diagnosed?

When you see your physician you will have a routine examination. Your doctor will ask you questions about your general health and your family history of cancer and liver disease. You will also be asked about your lifestyle and habits, including drinking and smoking.

Your physician may order the following tests:

  • Blood work. Blood tests may include a complete blood count, hematocrit, platelet count, liver function tests, Carcinoembriogenic antigen (CEA) and Carbohydrate antigen 19-9 (CA19-9), which may be elevated in patients with bile duct cancer.
  • Abdominal ultrasound. This test helps the doctor see the tumor.
  • CT scan. This test identifies the tumor(s) and pinpoints their size and location in the liver, as well as their relation to the vascular / biliary structures. It also helps the doctor to determine the overall health of the liver.
Comparison of radiographic images showing cholangiocarcinoma; A computed tomography (CT) image; B, cholangiogram (ERCP) image. Arrows designate the tumor.
Comparison of radiographic images showing cholangiocarcinoma; A, computed tomography (CT) image; B, cholangiogram (ERCP) image.  Arrows designate the tumor.
  • MRI. This test identifies the tumor(s) and pinpoints their size and location in the liver, as well as their relation to the vascular / biliary structures. It also helps the doctor to determine the overall health of the liver.  A doctor will determine whether to do a CT scan, an MRI or both.
  • Endoscopy. Endoscopy is visual instrument that allows your physician to see the inside of the esophagus, stomach and beginning of the lower intestine without surgery.
Room set-up and patient positioning for endoscopic retrograde cholangiopancreatography (ERCP).
Room setup and patient positioning for endoscopic retrograde cholangiopancreatography (ERCP)
  • Endoscopic Retrograde Cholangiopancreatography (ERCP). Endoscopic retrograde cholangiopancreatography is an endoscopic procedure that involves the use of fiberoptic endoscopes. You will be lightly sedated and your doctor will insert an endoscope through the mouth, down the esophagus, and into the stomach and small bowel.  A smaller tube or catheter is passed through the endoscope and into the bile ducts.  Dye is injected into the ducts, and the doctor takes X-rays that can show whether a tumor is present in the bile ducts.
A,B, Position of the endoscope in the duodenum during ERCP.
A, B, Position of the endoscope in the duodenum during ERCP
  • Percutaneous (through the skin) Transhepatic (through the liver) Cholangiogram.  In this procedure, a thin needle is inserted through the skin and into the bile ducts. A dye is injected through the needle so that a contrast image will show up on X-rays. By looking at the X-rays, the doctor may be able to see whether there is a tumor in the bile ducts.
A, technique of transhepatic percutaneous cholangiography; B, corresponding percutaneous cholangiograph (after catheter is introduced).
A, technique of transhepatic percutaneous cholangiography; B, corresponding percutaneous cholangiograph (after catheter is introduced)

What is the treatment for cholangiocarcinoma?

Treatment for bile duct cancer may include a combination of the following:

  • Surgery to remove the bile duct, and sometimes part of the liver 
  • Chemotherapy
  • Radiation
  • Stent placement. A stent is a tube that allows the bile, which is made by the liver, to drain more easily into the intestine if a tumor is blocking the bile duct.
A, point of percutaneous approach on a patient; B, right and left percutaneous transhepatic biliary drainage catheters restore patency around a hilar tumor.
A, point of percutaneous approach on a patient; B, right and left percutaneous transhepatic biliary drainage catheters restore patency around a hilar tumor

Surgical Therapy
Removing biliary tract tumors surgically is the treatment of choice in cholangiocarcinoma as it is the only therapeutic option that offers the potential for cure. Surgical approaches have become increasingly sophisticated over the last decade. It has become apparent that curative treatment depends on expertise that often involves the removal of a major section of the liver. The objective is complete removal of the bile duct and tumor.

A,B, surgical technique for unilateral hepatojejunostomy with Roux-en-Y anastomosis and left hepatic lobectomy.
A, B, surgical technique for unilateral hepatojejunostomy with Roux-en-Y anastomosis and left hepatic lobectomy

What is the prognosis for cholangiocarcinoma?

Your team of physicians will create an individualized treatment plan for you. 

Additional Information

Johns Hopkins Medicine Kimmel Cancer Center

Johns Hopkins Medicine Gastroenterology & Hepatology

 

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