What Is Bile Duct Cancer?

Table of Contents
View All
Table of Contents

Bile duct cancer, also known as cholangiocarcinoma, is a rare type of cancer that affects the bile ducts—thin tubes that run from the liver and gallbladder to the small intestine.

This cancer is most often incurable, in part because symptoms tend to develop when the tumor has already spread. About 10% of people survive five years or more after diagnosis.

This article covers the symptoms and causes of bile duct cancer. You will also learn how the disease is diagnosed and treated, and what lifestyle changes could help lower your risk.

A cancer patient with her doctor

FatCamera / Getty

Anatomy of Biliary Duct System

Your biliary system is a network of organs, ducts, and other structures that produce, store, and transport bile—a fluid that aids in digestion.

The system starts with ducts in your liver known as intrahepatic bile ducts. These branch off into larger ducts and, finally, the right and left hepatic ducts. These two ducts join outside of your liver and form the common hepatic duct.

Lower down, your cystic duct extends from your gallbladder, which is the organ that stores bile until it's needed for digestion. The common hepatic duct connects to the cystic duct to form the common bile duct, which then carries bile to the small intestine.

Bile duct cancer can develop in any part of this network.

Types of Bile Duct Cancer

The three types of bile duct cancer are based on the location of the tumor:

  • Intrahepatic bile duct cancers: Start in the smaller bile ducts inside the liver
  • Perihilar bile duct cancers: Start in the hepatic hilum, the area in which the left and right hepatic ducts branch off of the liver
  • Distal bile duct cancers: Found further down the bile duct, just below the gallbladder

Perihilar bile duct cancer and distal bile duct cancer are more broadly referred to as extrahepatic bile duct cancers because they develop outside of the liver.

Bile Duct Cancer Symptoms

Bile duct cancer can cause inflammation of the liver known as hepatitis. This leads to increased blood levels of bilirubin, a yellowing pigment caused by the breakdown of red blood cells.

In general, symptoms of bile duct cancer are similar to those of hepatitis and may not appear until late stages. They can include:

  • Jaundice (yellowing of the skin and eyes)
  • Abdominal pain just under the ribs
  • Fever
  • Chalky stools
  • Dark, cola-colored urine
  • Itchy skin
  • Fatigue
  • Nausea
  • Loss of appetite
  • Unintentional weight loss

The severity of symptoms typically depends on the location of the tumor. Tumors that develop outside the liver are likely to cause jaundice, itchy skin, dark urine, and abdominal pain. In some cases, tumors inside the liver may cause no symptoms at all.

Recap

Bile duct cancer often is only diagnosed once it has advanced because symptoms tend to only appear in late stages. They can include yellowing of the skin, chalky stools, pain below the ribs, and more.

Causes

There are numerous diseases and disorders linked to bile duct cancer, including:

  • Primary sclerosing cholangitis: An inflammatory bile duct disease and the most common cause of bile duct cancer in the developing world
  • Inflammatory bowel diseases: Including ulcerative colitis and Crohn's disease, both of which are closely linked to primary sclerosing cholangitis
  • Chronic liver diseases: Including cirrhosis, hepatitis B, hepatitis C, and non-alcoholic fatty liver disease
  • Choledochal cysts: Cysts of the bile duct that block the flow of bile
  • Parasitic liver parasites: Including liver flukes, which are more common in Asia and the developing world
  • Congenital abnormalities of the liver or bile ducts: Including Carroli's syndrome, Lynch syndrome II, and polycystic liver disease—all conditions that are present at birth

Risk Factors

Most people who are diagnosed with bile duct cancer are around 70 years of age. An estimated 8,000 people in the United States are diagnosed with bile duct cancer each year, making this a rare cancer that mostly affects older adults.

Certain groups of people are also at greater risk of bile duct cancer. For reasons not entirely clear, Latinx individuals are more likely to get the disease than other groups in the United States.

Bile duct cancer does not typically run in families, although you may be more at risk if you have a family history of it.

Obesity is also believed to play a key role, in large part because of the extreme inflammatory stress it places on the liver. The same applies to smoking and excessive alcohol use.

Unlike other risk factors, these are considered modifiable. In other words, you may reduce your risk of bile duct cancer if you lose weight, stop smoking, and reduce how much alcohol you drink.

In many cases, the underlying causes of bile duct cancer is never found.

Recap

Bile duct cancer may develop anywhere in your biliary duct system. It may be caused by liver disease, parasitic infection, or conditions that cause bile duct inflammation.

Diagnosis

If your doctor suspects bile duct cancer, they will perform a physical exam and ask you about your personal risk factors. From there, they may order blood tests, imaging studies, and other procedures to reach a diagnosis.

Ultimately, the only way to confirm a diagnosis of bile duct cancer is with a biopsy of the affected tissues.

Blood Tests

Doctors commonly use two blood tests to help diagnose bile duct cancer. Neither is able to confirm the disease, but they can bring doctors closer to a correct diagnosis.

They include:

  • Liver function test (LFT), a panel of tests that can detect if liver enzymes are raised due to liver inflammation: A high level of liver enzymes is a sign of liver disease, but that doesn't necessarily mean cancer.
  • Tumor marker tests that measure carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9: These proteins appear in the blood in response to liver cancer, gallbladder cancer, and other gastrointestinal cancers.

Imaging Tests

Imaging tests can help diagnose bile duct cancer by creating a visual of the tumor and surrounding structures.

A doctor may order any of these studies:

  • Abdominal ultrasound: A procedure that uses high-frequency sound waves to generate images of abdominal organs and structures
  • Computed tomography (CT scan): Multiple X-rays are used to create three-dimensional "slices" of internal organs
  • Magnetic resonance imaging (MRI scan): Powerful magnetic and radio waves create highly detailed images of internal organs, especially soft tissues
  • MRI cholangiopancreatography: A specialized MRI technique using contrast dyes to detect blockages and other problems in the bile ducts, gallbladder, liver, or pancreas

Procedures

Several procedures can help doctors access tumors and obtain tissue samples (biopsies) for lab evaluation. Examination of these samples under a microscope can confirm a bile duct cancer diagnosis.

Common procedures include:

  • Endoscopic retrograde cholangiopancreatography (ERCP): A flexible tube called an endoscope is passed through the mouth and into the small intestine to access the bile duct.
  • Percutaneous transhepatic cholangiography (PTC): A needle is inserted through the abdomen to access tumors in the bile duct.
  • Endoscopic ultrasound: A specialized probe is inserted through the mouth or rectum to examine and take tissue samples from the bile ducts.
  • Laparoscopy: A minimally invasive procedure, this involves making several small incisions in the abdomen to access a tumor.

The tissue sample taken during a biopsy can also be used to determine how advanced the cancer is. This part of the process is known as staging.

Staging

Once bile duct cancer has been diagnosed, the doctor will order an additional round of tests to determine how extensive the cancer is, or its stage.

This may involve imaging studies, such as positron emission tomography (PET). A PET scan uses a slightly radioactive dye that "lights up" in areas where there are active cancer cells.

PET scans can also establish whether the cancer is:

  • Localized: Shows no signs of spreading
  • Regional: Has spread to nearby tissues
  • Distant: Has spread to distant areas of the body (metastasized)

There are give bile duct cancer stages: 0 to 4. Several stages have sub-stages that further define exactly where the cancer has spread. Each advancing stage indicates greater metastasis.

There are slight variations in how intrahepatic, perihilar, and distal bile duct cancers are staged.

Intrahepatic Bile Duct Cancer Stages
 Stage Description
 0 The cancer is only in the bile duct cells. It has not spread to the deep tissues, nearby lymph nodes, or distant areas of the body.
 IA The tumor is no more than 2 inches wide. It has not spread to nearby blood vessels, nearby lymph nodes, or distant areas of the body.
 IB The tumor is more than 2 inches wide. It has not spread to nearby blood vessels, nearby lymph nodes, or distant areas of the body.
 II The tumor has spread to nearby blood vessels, but it has not spread to nearby lymph nodes or distant areas of the body.
—OR—
There are two or more tumors that may or may not have spread to nearby blood vessels, but they have not spread to nearby lymph nodes or distant areas of the body.
 IIIA The cancer has spread to the outer lining of abdominal organs. It has not spread to nearby lymph nodes or distant areas of the body.
 IIIB The cancer has spread outside of the liver and into nearby structures, but it has not spread to nearby lymph nodes or distant areas of the body. —OR— The cancer may or may not have spread outside the bile ducts of the liver or to distant areas of the body, but it has spread to nearby lymph nodes.
 IV The cancer may or may not have spread beyond the bile duct or to nearby lymph nodes, but it has spread to distant areas of the body, such as the bones or lungs.

Source: American Cancer Society

Perihilar Bile Duct Cancer Stages
 Stage Description
 0 The cancer is only in the bile duct cells. It has not spread to the deep tissues, to nearby lymph nodes, or distant areas of the body.
 I The cancer has spread into the deep tissues of the bile duct wall. It has not spread to nearby lymph nodes or distant areas of the body.
 II The tumor has spread through the bile duct wall and into nearby fatty tissue or liver tissue. It has not spread to nearby lymph nodes or distant areas of the body.
 IIIA The cancer has spread to the main blood vessels of the liver. It has not spread to nearby lymph nodes or distant areas of the body.
 IIIB The cancer has spread to the main blood vessels of the liver on one or both sides, but it has not spread to nearby lymph nodes or distant areas of the body.
—OR—
The cancer has spread into other bile ducts on one side,and a main blood vessel on the other side, but it has not spread to nearby lymph nodes or distant areas of the body.
 IIIC The cancer may or may not have spread outside the bile duct or to the blood vessels of the liver. It has spread to 1 to 3 lymph nodes, but it has not spread to distant areas of the body.
 IVA The cancer may or may not have spread outside the bile duct or to the blood vessels of the liver. It has spread to 4 or more lymph nodes, but not to distant areas of the body.
 IVB The cancer may or may not have spread outside the bile duct or to the blood vessels of the liver. It has spread to distant areas of the body, such as the bones, the lungs, or the liver.

Source: American Cancer Society

Distal Bile Duct Cancer Stages
Stage Description
0 The cancer is only in the bile duct cells. It has not spread to the deep tissues, to nearby lymph nodes, or distant areas of the body.
I The cancer has spread less than 1/5 an inch into the bile duct wall. It has not spread to nearby lymph nodes or distant areas of the body.
IIA The cancer has spread up to 1/2 an inch into the bile duct wall. It has not spread to nearby lymph nodes or distant areas of the body.
—OR—
The cancer has spread less than 1/5 an inch into the bile duct wall and to 1 to 3 nearby lymph nodes, but it has not spread to distant areas of the body.
IIB The cancer has spread more than 1/2 an inch into the bile duct wall, but it has not spread to nearby lymph nodes, or distant areas of the body.
—OR—
The cancer has grown at least 1/5 an inch into the bile duct wall, and to 1 to 3 nearby lymph nodes, but it has not spread to distant areas of the body.
IIIA The cancer has spread into the bile duct wall and to at least 4 nearby lymph nodes, but it has not spread to distant areas of the body.
IIIB The cancer has spread to nearby blood vessels. It may or may not have spread to nearby lymph nodes, but it has not spread to distant areas of the body.
IV The cancer has spread into the bile duct wall. It may or may not have spread to nearby blood vessels or lymph nodes. It has spread to distant areas of the body, such as the liver, the lungs, or abdominal lining.

Source: American Cancer Society

Genetic Profiling

Your doctor may order genetic testing to see if you have an underlying gene that causes a treatable mutation of cancer. If so, you may be a candidate for newer targeted therapies that specifically recognize and kill these cancer cells.

Recap

Doctors use blood tests and imaging to look for bile duct cancer, but only a biopsy can confirm the diagnosis.

Once the diagnosis is made, doctors stage the cancer by how far it has spread. The closer a tumor gets to the liver and other organs, the more difficult it becomes to treat.

Treatment

The majority of bile duct cancers are incurable because the disease is typically advanced by the time symptoms appear.

With that said, bile duct cancer is sometimes caught before it has spread and can be treated with surgery and follow-up treatment to destroy all remaining cancer cells.

If the tumor cannot be removed completely, treatment focuses on slowing its spread, reducing symptoms, extending survival, and improving overall quality of life.

Surgery

Unless the cancer is clearly too advanced to be operated on, most people will undergo exploratory surgery to determine if surgical resection (removal) is possible.

This is usually performed with laparoscopy rather than open surgery. With laparoscopy, a probe is inserted through very small incisions in the abdomen to examine affected tissues.

If the tumor is localized or regional with no evidence of spread, resection may be considered based on the general health of the individual and how well their liver is functioning.

The type of surgery used can vary by the location of the tumor:

  • Intrahepatic bile duct cancers: These typically require surgical resection of part of the liver (hepatectomy) along with resection of nearby lymph nodes.
  • Extrahepatic bile duct cancers: These are commonly treated with a Whipple procedure, which involves removing the common bile duct along with a portion of the pancreas and small intestine. The affected extrahepatic bile duct would also be removed.

Some early-stage intrahepatic tumors are inoperable but can still be treated with a liver transplant. In such cases, chemotherapy and radiation may be used to halt the spread of cancer until a donor liver is found.

Adjuvant Therapies

Adjuvant therapies are used after surgery to destroy remaining cancer cells and prevent their return. Common cancer treatments like chemotherapy and external or internal radiation therapies are considered adjuvant therapies.

It is unclear how effective these therapies are in preventing cancer from returning. There is also controversy over when it is appropriate to use them.

Part of that stems from the fact that so few people with bile duct cancer have tumors that can be operated on. Those who do may not respond to adjuvant therapies.

At present, there is no evidence that either adjuvant chemotherapy or radiation therapy can extend survival times, even in people with early-stage bile duct cancer.

Even so, doctors often recommend adjuvant therapy, since there is always a chance of remaining cancer cells after surgery.

Targeted Therapy and Immunotherapy

If genetic testing reveals that you have a specific, treatable mutation of cancer, you may be eligible for targeted therapies or immunotherapies.

Targeted therapies slow cancer growth by attacking cancer cells caused by specific genetic mutations. Immunotherapies strengthen your immune system so that it can fight the cancer cells.

Newer targeted therapies and immunotherapies being used in people whose cancer has specific genetic mutations include:

  • The targeted drugs Tibsovo (ivosidenib) and Pemazyre (pemiganitib), which can stop the growth of cancer
  • Immunotherapeutic agents like Keytruda (pembrolizumab), which can slow disease progression

Palliative Therapy

Palliative therapy is a form of treatment used to alleviate pain and other symptoms of late-stage disease. In people with inoperable bile duct cancer, this can take several forms:

  • Pain medications, including opioid drugs like fentanyl
  • Palliative radiation therapy: Primarily used to reduce the size of a tumor, to unblock a bile duct, or reduce pressure on compressed nerves
  • Palliative chemotherapy delivered to the blocked bile duct via a catheter in a blood vessel in order to shrink the tumor
  • Biliary stenting involving the placement of a tube, called a stent, in a bile duct to improve biliary flow
  • Biliary bypass: A surgical procedure in which the bile duct obstruction is removed and the cut ends are sewn together
  • Percutaneous tumor ablation, in which heat or electrical energy is delivered to the tumor via a needle-like conductor inserted through the skin
  • Percutaneous ethanol injections, in which alcohol is injected into the tumor to shrink it and deaden the nerves that relay pain

Clinical Trials

People diagnosed with bile duct cancer are encouraged to participate in clinical trials. This can provide them access to experimental drugs or therapies that may improve outcomes, particularly if their disease is inoperable.

Recap

Surgery is used to treat bile duct cancer, but most tumors are inoperable due to how far advanced they are when found. In that case, treatment is directed at managing the disease and its symptoms.

Prognosis

Five-year survival is a common measure used to determine what percentage of people with a disease will be alive at least five years following the initial diagnosis.

The five-year survival rate for bile duct cancer is broken down by how far the cancer has spread and whether the tumor is inside the liver or not.

Generally speaking, people with extrahepatic bile duct cancer have better outcomes because the liver is less likely to be affected. The outcome tends to be poor any time cancer spreads to the liver.

Bile Duct Cancer 5-Year Survival Rates by Location
  Intrahepatic Extrahepatic
Localized 15% 30%
Regional 6% 24%
Distant 2% 2%

It is important to remember that the disease can vary from one person to the next and that the five-year survival estimates are just that—estimates. Some people can survive longer based on their general health and the location of the tumor.

Summary

Bile duct cancer is a rare but aggressive form of cancer that is rarely diagnosed in its early stages. Late-stage symptoms often mimic hepatitis, but they depend on where the tumor is located.

Though several tests will be done to help diagnose suspected bile duct cancer, a biopsy is ultimately needed to confirm and stage it.

Bile cancer can be treated with surgery in some cases. If it is, additional treatments (chemo, radiation) are often used to prevent recurrence. When a cancer cannot be operated on, treatments are given to slow progression and improve quality of life.

Even with these efforts, survival five years after diagnosis ranges from 2% to 30%, depending on the type of bile duct cancer and how far it has spread.

A Word From Verywell

Learning that you have bile duct cancer can be overwhelming for you and those who care about you. Be open with loved ones and doctors about the care you wish you to receive and how you wish to spend your time.

No matter what stage of cancer you are diagnosed with, know that you have options. If you are unsure about the diagnosis or a recommended therapy, do not hesitate to seek a second opinion from an oncologist specializing in biliary cancers.

Was this page helpful?
Article Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. American Cancer Society. Survival rates for bile duct cancer. Updated January 2021.

  2. American Cancer Society. What is bile duct cancer?. Updated March 2021.

  3. American Cancer Society. Signs and symptoms of bile duct cancer. Updated July 2018.

  4. Labib PL, Goodchild G, Pereira SP. Molecular pathogenesis of cholangiocarcinoma. BMC Cancer. 2019 Feb;19(1):185. doi:10.1186/s12885-019-5391-0

  5. American Cancer Society. Key statistics for bile duct cancer. Updated July 3, 2018.

  6. Mody K, Antwi SO, Hodge DO, Ailawadhi S, Roberts L, Bekaii-Saab T. A SEER-based multi-ethnic picture of advanced intrahepatic cholangiocarcinoma in the United States pre- and post-the advent of gemcitabine/cisplatin. J Gastrointest Oncol. 2018 Dec;9(6):1063-1073. doi:10.21037/jgo.2018.07.09

  7. U.S. National Library of Medicine. Cholangiocarcinoma. In: Genetic Home Reference. Updated August 2020.

  8. McGee EE, Jackson SS, Petrick JL, et al. Smoking, alcohol, and biliary tract cancer risk: A pooling project of 26 prospective studies. J Natl Cancer Inst. 2019 Dec;111(12):1263-78. doi:10.1093/jnci/djz103

  9. Tshering G, Dorji PW, Chaijaroenkul W, Na-Bangchang K. Biomarkers for the diagnosis of cholangiocarcinoma: A systematic review. Am J Trop Med Hyg. 2018;98(6):1788-97. doi:10.4269/ajtmh.17-0879

  10. Olthof SC, Othman A, Clasen S, Schraml C, Nikolaou K, Bongers M. Imaging of cholangiocarcinoma. Visc Med. 2016 Dec;32(6):402-410. doi:10.1159/000453009

  11. National Cancer Institute. Bile duct cancer (cholangiocarcinoma) treatment. Health professional version. In: PDQ Cancer Information Summaries. Updated July 28, 2020.

  12. American Cancer Society. Staging of intrahepatic bile duct cancers. Updated December 2017.

  13. American Cancer Society. Staging of perihilar bile duct cancers. Updated December 2017.

  14. American Cancer Society. Staging distal bile duct cancers. Updated December 2017.

  15. Andersen JB, Thorgeirsson SS. Genetic profiling of intrahepatic cholangiocarcinoma. Curr Opin Gastroenterol. 2012 May;28(3):266-72. doi:10.1097/MOG.0b013e3283523c7e

  16. Cillo U, Fondevila C, Donadon M, et al. Surgery for cholangiocarcinoma. Liver Int. 2019 Mar;39(1):143-155. doi:10.1111/liv.14089

  17. Wang K, Zhang H, Xia Y, Liu J, Shen F. Surgical options for intrahepatic cholangiocarcinoma. Hepatobiliary Surg Nutr. 2017 Apr;6(2):79-90. doi:10.21037/hbsn.2017.01.06

  18. Radtke A, Königsrainer A. Surgical therapy of cholangiocarcinoma. Visc Med. 2016;32(6):422-426. doi:10.1159/000452921

  19. Sapisochín G, Fernández de Sevilla E, Echeverri J, Charco R. Liver transplantation for cholangiocarcinoma: Current status and new insights. World J Hepatol. 2015 Oct;7(22):2396-403. doi:10.4254/wjh.v7.i22.2396

  20. Wang G, Wang Q, Fan X, Ding L, Dong L. The significance of adjuvant therapy for extrahepatic cholangiocarcinoma after surgeryCancer Manag Res. 2019 Dec;11(1):10871-10882. doi:10.2147/CMAR.S224583

  21. Ma KW, Cheung TT, Leung B, et al. Adjuvant chemotherapy improves oncological outcomes of resectable intrahepatic cholangiocarcinoma: A meta-analysis. Medicine (Baltimore). 2019 Feb;98(5):e14013. doi:10.1097/MD.0000000000014013