Table of ContentsView AllTable of ContentsTypesSymptomsCausesDiagnosisTreatmentSurvival
Table of ContentsView All
View All
Table of Contents
Types
Symptoms
Causes
Diagnosis
Treatment
Survival
Bile duct cancer, also known ascholangiocarcinoma, is a rare type of cancer that affects the bile ducts—the thin tubes in the digestive system that run from the liver andgallbladderto thesmall intestine.
This cancer is usually incurable, in part because symptoms usually develop after 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.
FatCamera / Getty
Types of Bile Duct Cancer
The biliary system is a network of organs and ducts that produce, store, and transport a digestive fluid calledbile. This includes the liver which produces bile and the gallbladder which stores and releases bile when needed.
The system starts with theright andleft hepatic ductsinside the liver that join to form thecommon hepatic ductoutside the liver. The common hepatic duct then connects to thecystic ductextending from the gallbladder to form thecommon bile ductthat delivers bile to the small intestine.
What Are Common Symptoms of Bile Duct Cancer?
These can include:
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 not cause symptoms at all.
Itchy Skin as a Cancer Warning Sign
Causes and Risk Factors of Bile Duct Cancer
The cause of bile duct cancer is unknown, but there are risk factors associated with this rare form of cancer affecting roughly 8,000 people in the United States each year.
Risk factors for bile duct cancer include:
There are also numerous diseases and disorders linked to bile duct cancer, including:
Common Causes and Risk Factors of Cancer
How Is Bile Duct Cancer Diagnosed and Staged?
If your healthcare provider is concerned that you could have bile duct cancer, you will have a medical history and physical examination. From there, they may order blood tests, imaging studies, and other procedures to determine the cause of your symptoms.
A diagnosis of bile duct cancer is confirmed with abiopsy.
Blood Tests
Blood tests used to diagnose bile duct cancer include:
Imaging Tests
The following imaging tests can help visualize the tumor and any obstruction in the biliary flow:
Biopsy
A biopsy is the extraction of tissues for evaluation in the pathology lab. It provides the definitive diagnosis of solid tumor cancers like bile duct cancer.
The biopsy may be performed during the following procedures:
What Is Stage 3 Cancer?
Cancer Staging
Once bile duct cancer is diagnosed, the biopsy along with other tests can stage the cancer. Staging is used to determine how advanced the cancer is based on theTNM classification system. By determining what stage the cancer is—from stage 0 to stage 4—the healthcare provider can ensure the appropriate course of treatment.
The TNS system is based on the size and characteristics of the tumor (T), the number of local lymph nodes with cancer cells in the (N), and whether the cancer hasmetastasized(spread) or not (M).
There are slight variations in how intrahepatic, perihilar, and distal bile duct cancers are staged.
Source: American Cancer Society
How Is Bile Duct Cancer Treated
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 for surgery, most people will undergo exploratory surgery to determine ifsurgical resection(removal) is possible.
This is usually performed with laparoscopy rather thanopen 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.
Some early-stage intrahepatic tumors are inoperable but can still be treated with aliver transplant. In such cases,chemotherapyandradiationmay 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 radiation therapy are considered adjuvant therapies.
It is unclear how effective these therapies are in preventing cancer from returning, and there is debate as to whether adjuvant chemotherapy or radiation therapy can extend survival times even for people with early-stage bile duct cancer.
Even so, healthcare providers often recommend adjuvant therapy, since there is always a chance of remaining cancer cells after surgery.
Targeted Therapy and Immunotherapy
Option include:
Palliative Therapy
Palliative therapyis 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:
Prognosis and Survival Rates
Five-year survival is a common measure used to determine what percentage of people with a disease will be aliveat leastfive years following the initial diagnosis.
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.
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.
What Your Prognosis Means
Summary
Bile duct cancer is a rare, aggressive form of cancer that is rarely diagnosed in its early stages. Late-stage symptoms depend on where the tumor is located and often mimic hepatitis. 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. In addition, treatments (chemo, radiation) are often used to prevent recurrence. When surgery is not an option, 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.
Tips on How to Survive Cancer
17 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Cancer Society.Survival rates for bile duct cancer.American Cancer Society.What is bile duct cancer?.American Cancer Society.Signs and symptoms of bile duct cancer.American Cancer Society.Key statistics for bile duct cancer.MedlinePlus.Cholangiocarcinoma.Labib PL, Goodchild G, Pereira SP.Molecular pathogenesis of cholangiocarcinoma.BMC Cancer.2019;19(1):185. doi:10.1186/s12885-019-5391-0Tshering 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.17-0879Olthof SC, Othman A, Clasen S, Schraml C, Nikolaou K, Bongers M.Imaging of cholangiocarcinoma.Visc Med. 2016;32(6):402-410. doi:10.1159/000453009National Cancer Institute.Bile duct cancer (cholangiocarcinoma) treatment.American Cancer Society.Staging of intrahepatic bile duct cancers.American Cancer Society.Staging of perihilar bile duct cancers.American Cancer Society.Staging distal bile duct cancers.Cillo U, Fondevila C, Donadon M, et al.Surgery for cholangiocarcinoma.Liver Int. 2019;39(S1):143-155. doi:10.1111/liv.14089Wang K, Zhang H, Xia Y, Liu J, Shen F.Surgical options for intrahepatic cholangiocarcinoma.Hepatobiliary Surg Nutr.2017;6(2):79-90. doi:10.21037/hbsn.2017.01.06Radtke A, Königsrainer A.Surgical therapy of cholangiocarcinoma.Visc Med. 2016;32(6):422-426. doi:10.1159/000452921Sapisochín G, Fernández de Sevilla E, Echeverri J, Charco R.Liver transplantation for cholangiocarcinoma: current status and new insights.World J Hepatol.2015;7(22):2396. doi:10.4254/wjh.v7.i22.2396Ma KW, Cheung TT, Leung B, et al.Adjuvant chemotherapy improves oncological outcomes of resectable intrahepatic cholangiocarcinoma: a meta-analysis.Medicine.2019;98(5):e14013. doi:10.1097/MD.0000000000014013
17 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Cancer Society.Survival rates for bile duct cancer.American Cancer Society.What is bile duct cancer?.American Cancer Society.Signs and symptoms of bile duct cancer.American Cancer Society.Key statistics for bile duct cancer.MedlinePlus.Cholangiocarcinoma.Labib PL, Goodchild G, Pereira SP.Molecular pathogenesis of cholangiocarcinoma.BMC Cancer.2019;19(1):185. doi:10.1186/s12885-019-5391-0Tshering 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.17-0879Olthof SC, Othman A, Clasen S, Schraml C, Nikolaou K, Bongers M.Imaging of cholangiocarcinoma.Visc Med. 2016;32(6):402-410. doi:10.1159/000453009National Cancer Institute.Bile duct cancer (cholangiocarcinoma) treatment.American Cancer Society.Staging of intrahepatic bile duct cancers.American Cancer Society.Staging of perihilar bile duct cancers.American Cancer Society.Staging distal bile duct cancers.Cillo U, Fondevila C, Donadon M, et al.Surgery for cholangiocarcinoma.Liver Int. 2019;39(S1):143-155. doi:10.1111/liv.14089Wang K, Zhang H, Xia Y, Liu J, Shen F.Surgical options for intrahepatic cholangiocarcinoma.Hepatobiliary Surg Nutr.2017;6(2):79-90. doi:10.21037/hbsn.2017.01.06Radtke A, Königsrainer A.Surgical therapy of cholangiocarcinoma.Visc Med. 2016;32(6):422-426. doi:10.1159/000452921Sapisochín G, Fernández de Sevilla E, Echeverri J, Charco R.Liver transplantation for cholangiocarcinoma: current status and new insights.World J Hepatol.2015;7(22):2396. doi:10.4254/wjh.v7.i22.2396Ma KW, Cheung TT, Leung B, et al.Adjuvant chemotherapy improves oncological outcomes of resectable intrahepatic cholangiocarcinoma: a meta-analysis.Medicine.2019;98(5):e14013. doi:10.1097/MD.0000000000014013
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
American Cancer Society.Survival rates for bile duct cancer.American Cancer Society.What is bile duct cancer?.American Cancer Society.Signs and symptoms of bile duct cancer.American Cancer Society.Key statistics for bile duct cancer.MedlinePlus.Cholangiocarcinoma.Labib PL, Goodchild G, Pereira SP.Molecular pathogenesis of cholangiocarcinoma.BMC Cancer.2019;19(1):185. doi:10.1186/s12885-019-5391-0Tshering 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.17-0879Olthof SC, Othman A, Clasen S, Schraml C, Nikolaou K, Bongers M.Imaging of cholangiocarcinoma.Visc Med. 2016;32(6):402-410. doi:10.1159/000453009National Cancer Institute.Bile duct cancer (cholangiocarcinoma) treatment.American Cancer Society.Staging of intrahepatic bile duct cancers.American Cancer Society.Staging of perihilar bile duct cancers.American Cancer Society.Staging distal bile duct cancers.Cillo U, Fondevila C, Donadon M, et al.Surgery for cholangiocarcinoma.Liver Int. 2019;39(S1):143-155. doi:10.1111/liv.14089Wang K, Zhang H, Xia Y, Liu J, Shen F.Surgical options for intrahepatic cholangiocarcinoma.Hepatobiliary Surg Nutr.2017;6(2):79-90. doi:10.21037/hbsn.2017.01.06Radtke A, Königsrainer A.Surgical therapy of cholangiocarcinoma.Visc Med. 2016;32(6):422-426. doi:10.1159/000452921Sapisochín G, Fernández de Sevilla E, Echeverri J, Charco R.Liver transplantation for cholangiocarcinoma: current status and new insights.World J Hepatol.2015;7(22):2396. doi:10.4254/wjh.v7.i22.2396Ma KW, Cheung TT, Leung B, et al.Adjuvant chemotherapy improves oncological outcomes of resectable intrahepatic cholangiocarcinoma: a meta-analysis.Medicine.2019;98(5):e14013. doi:10.1097/MD.0000000000014013
American Cancer Society.Survival rates for bile duct cancer.
American Cancer Society.What is bile duct cancer?.
American Cancer Society.Signs and symptoms of bile duct cancer.
American Cancer Society.Key statistics for bile duct cancer.
MedlinePlus.Cholangiocarcinoma.
Labib PL, Goodchild G, Pereira SP.Molecular pathogenesis of cholangiocarcinoma.BMC Cancer.2019;19(1):185. doi:10.1186/s12885-019-5391-0
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
Olthof SC, Othman A, Clasen S, Schraml C, Nikolaou K, Bongers M.Imaging of cholangiocarcinoma.Visc Med. 2016;32(6):402-410. doi:10.1159/000453009
National Cancer Institute.Bile duct cancer (cholangiocarcinoma) treatment.
American Cancer Society.Staging of intrahepatic bile duct cancers.
American Cancer Society.Staging of perihilar bile duct cancers.
American Cancer Society.Staging distal bile duct cancers.
Cillo U, Fondevila C, Donadon M, et al.Surgery for cholangiocarcinoma.Liver Int. 2019;39(S1):143-155. doi:10.1111/liv.14089
Wang K, Zhang H, Xia Y, Liu J, Shen F.Surgical options for intrahepatic cholangiocarcinoma.Hepatobiliary Surg Nutr.2017;6(2):79-90. doi:10.21037/hbsn.2017.01.06
Radtke A, Königsrainer A.Surgical therapy of cholangiocarcinoma.Visc Med. 2016;32(6):422-426. doi:10.1159/000452921
Sapisochín G, Fernández de Sevilla E, Echeverri J, Charco R.Liver transplantation for cholangiocarcinoma: current status and new insights.World J Hepatol.2015;7(22):2396. doi:10.4254/wjh.v7.i22.2396
Ma KW, Cheung TT, Leung B, et al.Adjuvant chemotherapy improves oncological outcomes of resectable intrahepatic cholangiocarcinoma: a meta-analysis.Medicine.2019;98(5):e14013. doi:10.1097/MD.0000000000014013
Meet Our Medical Expert Board
Share Feedback
Was this page helpful?Thanks for your feedback!What is your feedback?OtherHelpfulReport an ErrorSubmit
Was this page helpful?
Thanks for your feedback!
What is your feedback?OtherHelpfulReport an ErrorSubmit
What is your feedback?
By clicking “Accept All Cookies”, you agree to the storing of cookies on your device to enhance site navigation, analyze site usage, and assist in our marketing efforts.Cookies SettingsAccept All Cookies
By clicking “Accept All Cookies”, you agree to the storing of cookies on your device to enhance site navigation, analyze site usage, and assist in our marketing efforts.
Cookies SettingsAccept All Cookies
