Bile Duct Cancer (Cholangiocarcinoma) - Types and Stages of Bile Duct Cancer

Bile duct cancer develops in the bile duct. The disease may occur at any age, but people over 60 years of age are at increased risk. It is important to consult the doctor if the patient experiences any symptoms, as bile duct carcinoma diagnosed at an advanced stage is difficult to treat.

What Is Bile Duct Cancer?

Bile duct cancer, or bile duct carcinoma, initiates in the bile duct. Bile ducts are tube-like structures that carry the bile from the liver and gallbladder into the digestive system (small intestine). There are several types of bile duct tumors. However, not all of them are cancerous. Bile duct adenomas and bile duct hamartomas are non-cancerous. Almost all bile duct cancers are cholangiocarcinomas, and thus, the term can be used as a synonym for this cancer type. Cholangiocarcinoma is a type of adenocarcinoma, and it begins in the glandular cells. These glandular cells line the inner layer of the bile duct.

How Common is Bile Duct Cancer (Cholangiocarcinoma)?

The incidence and mortality of bile duct carcinoma vary depending on geographical regions, gender, and types of bile duct carcinoma. A few studies have reported the highest incidence in Chile and the lowest in Vietnam. Most countries have had a trend of increasing incidence rates in recent times. Patients aged 75 years or older have 5 to 10 times higher mortality rates compared to the overall mortality rate. Males have a higher incidence and mortality rate for extrahepatic cholangiocarcinoma, ampulla of Vater cancer, and intrahepatic cholangiocarcinoma.

What are Bile Duct Cancer Types?

Depending on the site of origin, bile duct carcinoma is classified into various types. Following are the various bile duct cancer types:

Intrahepatic Cholangiocarcinoma (ICC)

Intrahepatic cholangiocarcinoma is the second most common type of cancer arising from the liver, after hepatocellular carcinoma. ICC accounts for almost 10% of all bile duct cancer types. Only about 15% of the ICC tumors are resectable, and the patients have a median survival period of three years.

Extrahepatic Cholangiocarcinoma

ECC develops outside the liver. ECC may develop at the perihilar and distal anatomical sites in the bile duct. There is a global increase in the incidence of ECC. Surgical resection is the most common treatment for ECC during the early stages. However, when presented as a metastatic or unresectable disease, ECC has a high risk of recurrence that restricts optimal survival.

Hilar Cholangiocarcinoma

Hilar cholangiocarcinoma is a rare condition and is more common in people over 60 years of age. Patients with this condition usually have a poor prognosis. It comprises over 60% of cholangiocarcinoma cases.

What are the Bile Duct Cancer Stages?

Staging is an important aspect of bile duct carcinoma management. It helps doctors understand the extent of the disease and, accordingly, create a treatment plan. The following are the different stages of bile duct cancer (intrahepatic cholangiocarcinoma):


Bile duct cancer is a rare condition characterized by malignant cells in the bile duct. There are several bile duct cancer types, depending on the location of the cancer. These include intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and hilar cholangiocarcinoma. Extrahepatic cholangiocarcinoma is further divided into perihilar bile duct carcinoma and distal cholangiocarcinoma. Lastly, bile duct carcinoma is staged between 0 and 4, depending on the extent of the disease’s spread, with 0 referring to the early stage and 4 referring to the advanced stage of the disease.

Frequently Asked Questions

Bile duct carcinoma starts in the bile ducts of the liver, the bile ducts between the liver and gallbladder, and the bile ducts between the gallbladder and small intestine.

Cholangiocarcinoma may occur at any age. However, people over the age of 60 are at a relatively higher risk of developing it.

Like all cancers, the treatment of bile duct cancer depends on several factors, such as the stage at which the cancer is diagnosed, the age of the patient, their overall health status, and their response to treatment.

Cholangiocarcinoma is a relatively slow-growing cancer. On average, it may take anywhere between 15-20 years for precancerous lesions to form tumors. That said, this duration depends on individual patient circumstances, such as the patient’s age, medical history, and overall health status.

The rate at which bile duct carcinoma grows depends on various parameters. Some bile duct cancer types, such as distal cholangiocarcinoma, progress rapidly, whereas some types, like intrahepatic cholangiocarcinoma, have a slower growth rate.