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Overview

Bile ducts are a network of tubes that connects the liver, gall bladder, and small intestine. The liver is responsible for the formation of bile which is stored in the gall bladder. The function of the bile duct is to carry bile from the liver and gall bladder to the small intestine. The bile helps in the breakdown of fats in the food and improves digestion [1] . Bile duct cancer, also known as cholangiocarcinoma, is a rare form of cancer in which cancer cells are formed in the bile ducts. It is the sixth leading cause of cancer death around the world.

Types of Cholangiocarcinoma Cancer

Based on the region, where the cancer cells start to develop in the bile duct there are two types of bile duct cancer. They are:

Intrahepatic bile duct cancer

It is also known as intrahepatic cholangiocarcinoma. In this type of cancer, the cancerous cells are formed in the bile ducts inside the liver.

Extrahepatic bile duct cancer:

In this type of cancer, the cancerous cells are formed in the bile ducts outside the liver.

Causes

Bile duct cancer is caused by acquired (somatic) gene mutations rather than inherited (germline) gene mutations. In most bile duct cancers, acquired genetic mutation occurs in the TP53 tumor suppressor gene. Other mutations in genes such as HER2, ALK, and KRAS may result in bile duct cancer. Also, chronic inflammation may cause changes in the genes that will further lead to bile duct cancer.

Risk Factors

Some of the risk factors identified that may be responsible for developing bile duct cancer are:

Primary sclerosing cholangitis (PSC)

In this particular condition, scar tissue is formed due to the inflammation of bile ducts. This condition increases the risk of developing bile duct cancer.

Liver fluke infections

The fluke resides in the bile duct and is responsible for causing bile duct cancer.

Bile duct stones:

This causes inflammation of the bile duct and further increases the risk of developing bile duct cancer.

Hepatitis B or C virus infections:

Increased risk of intrahepatic bile duct cancer is associated with hepatitis B or C infection.

Cirrhosis

It also increases the risk of developing bile duct cancer.

Choledochal cyst:

It is a rare disease in which sacs are filled with bile along the bile ducts and this leads to inflammation of the duct walls. This inflammation may further cause bile duct cancer to develop.

Inflammatory bowel disease

People with ulcerative colitis and Crohn’s diseases are at a higher risk of bile duct cancer.

Age

The chances of developing bile duct cancer are higher in the older age group i.e. 60-70 years of age than in younger people.

Overweight/ obesity:

It increases the risk of developing bile duct cancer due to hormonal changes or bile duct stones.

Others:

Include HIV infections, exposure to asbestos, radon, nitrosamines, dioxin, or polychlorinated biphenyls, smoking, and chronic pancreatitis.

Signs & symptoms

Signs & symptoms observed in bile duct cancer are:

Jaundice

Dark coloured urine and pale stool

Fever

Abdominal pain and itchy skin

Loss of weight, nausea, and vomiting

Diagnosis

Some of the tests performed to diagnose cancer are

Liver function tests: The amount of bilirubin and alkaline phosphatase released into the blood by the liver is measured in the blood sample. A higher amount found in the blood sample may be caused by bile duct cancer.

Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker test: The higher amount of carcinoembryonic antigen (CEA) and CA 19-9 may point to bile duct cancer.

MRCP (magnetic resonance cholangiopancreatography): Detailed pictures of the liver, bile ducts, pancreas, gall bladder, and pancreatic duct can be obtained in this specialized imaging technique.

Laparoscopy: A surgical procedure to look at the organs inside the abdomen, such as the bile ducts and liver, to check for signs of cancer.

Percutaneous transhepatic cholangiography (PTC): A procedure used to visualize the liver and bile ducts. A sample of tissue is removed and checked for signs of cancer.

Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to see the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes bile duct cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice.

Cholangiocarcinoma Cancer