Definition and Overview

Cholangiocarcinoma of the biliary tract, or bile duct cancer, is a rare type of cancer caused by malignant cells within the bile ducts.

Bile ducts are a network of tubes that connect the small intestine, gallbladder, and liver. They are responsible for carrying bile from the gallbladder and liver through the pancreas and the first segment of the small intestine (duodenum).

In many cases, the cancer starts from the lining of the bile ducts and can affect any part of the biliary tract. The exact location of the tumor and the type of cells involved determine the type of bile duct cancer.

  • Intrahepatic cholangiocarcinoma – This is the rare form of biliary tract cancer that accounts for just about 5%-10% of all biliary cancer cases. It begins in the liver and is thus often misdiagnosed as liver cancer.

  • Extrahepatic bile duct cancer – This develops outside the liver and accounts for up to 90% of bile duct cancer cases. This type of cancer can be either hilar or Klatskin tumor (forms where the hepatic duct branches leave the liver) or distal (normally occurs near the small intestine).

Tumors of the bile ducts can cause various symptoms as they tend to block the flow of bile. This causes bile to flow back into the body tissues and blood.

Causes of Condition

The exact causes of bile duct cancers are yet to be determined. However, there are factors that can increase one’s risk of developing it. These include:

  • Bile ducts cysts

  • Certain liver diseases

  • Chronic bile duct inflammation

  • Chronic inflammatory bowel conditions, such as ulcerative colitis

  • Congenital abnormalities of the biliary tract

  • Infection caused by a parasite called liver fluke

  • Jaundice

Bile Duct Cancer Symptoms

Bile duct cancer does not cause any noticeable signs or symptoms during the first stages. This makes it difficult to diagnose when it is most responsible to treatment. When the condition causes symptoms, it is usually because the bile duct is already blocked. These symptoms include:

  • Abdominal pain – This symptom is normally present when biliary tract cancer has reached advanced stages. Patients typically complain of pain below their right ribs.

  • Fever

  • Itching and dark urine caused by elevated levels of bilirubin in the blood

  • Light-colored stools – If bile ducts are blocked and the flow of bile is obstructed, the patient will not be able to digest fatty foods causing stool to become unusually pale

  • Nausea and vomiting

  • Unexplained weight loss and loss of appetite

Who to See and Types of Treatments Available

Patients suffering from symptoms of cholangiocarcinoma of the biliary tract must consult a physician right away. The earlier the condition is diagnosed, the more treatment options they have.

The initial consultation with the doctor typically involves the review of the patient’s medical history, risk factors, symptoms, and the first time the patient has observed them. A physical examination that focuses on the abdomen is then performed to check for any fluid build-up, tenderness, and lumps.

If the results of initial tests point to biliary tract cancer, additional confirmatory lab and imaging tests will be performed. These include:

  • Angiogram

  • Biopsy

  • Computed tomography (CT) scan

  • Endoscopic retrograde cholangiopancreatography (ERCP)

  • Endoscopic ultrasound scan (EUS)

  • Magnetic resonance imaging (MRI) scan

  • Percutaneous transhepatic cholangiography (PTC)

Additional procedures will then be performed to determine the stage of cancer, which is factored in when formulating the best treatment plan for the patient.

Treatment of biliary tract cancer follows the same protocols used in treating any type of cancer. The main treatment is surgery in which the malignant tumor is removed. If the condition is caught early, the surgical procedure will be performed to remove only the bile ducts where the cancer was found. The remaining ducts will then be joined in the liver to the small bowel to restore the flow of bile. However, in cases where the cancer has spread to the liver, partial liver resection will also be performed.

If cancer has spread into nearby structures, Whipple’s procedure is considered. This is more complicated and requires post-operation intensive care. The surgery involves the removal of the pancreas, parts of the small bowel and stomach, bile ducts, and surrounding lymph nodes. Surgery is often followed by chemotherapy and radiotherapy.

Bile duct cancer survival rate depends on the type of cancer the patient has. The survival rate for patients with intrahepatic bile duct cancer is relatively low when compared to those with extrahepatic bile duct cancer. The five-year survival rate for the former is 15% for localized cancer, 6% for regional cancer, and 2% for distant cancer or when the cancer has metastasized to distant organs.

Meanwhile, the five-year survival rate for patients with extrahepatic bile duct cancer is 30% for localized cancer, 24% for regional cancer, and 2% for distant cancer.

Bile duct cancer prognosis is affected by several factors including the stage of cancer, the patient’s overall health condition, and whether or not the patient is suffering from other medical conditions.


  • The American Cancer Society
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