About Bile Duct Cancer
Overview and Types
If you've been diagnosed with bile duct cancer or are worried about it, you likely have a
lot of questions. Learning some basics is a good place to start.
● What Is Bile Duct Cancer?
Research and Statistics
See the latest estimates for new cases of bile duct cancer and deaths in the US and
what research is currently being done.
● Key Statistics for Bile Duct Cancer
● What's New in Bile Duct Cancer Research?
What Is Bile Duct Cancer?
Cancer starts when cells in the body start to grow out of control. Cells in nearly any part
of the body can become cancer, and can spread to other areas of the body. To learn
more about how cancers start and spread, see What Is Cancer?
Bile duct cancer starts in a bile duct. To understand this cancer, it helps to know about
the bile ducts and what they normally do.
About the bile ducts
The bile ducts are a series of thin tubes that go from the liver to the small intestine.
Their major job is to move a fluid called bile from the liver and gallbladder into the small
intestine, where it helps digest the fats in food.
Different parts of the bile duct system have different names. In the liver it begins as
many tiny tubes (called ductules) where bile collects from the liver cells. The ductules
come together to form tubes called small ducts. These merge into larger ducts and then
the left and right hepatic ducts. All of these ducts within the liver are called intrahepatic
bile ducts.
The left and right hepatic ducts exit the liver and join to form the common hepatic duct in
an area called the hilum. Lower down, the gallbladder (a small organ that stores bile) is
joined to the common hepatic duct by a small duct called the cystic duct. This
combined duct is called the common bile duct. The common bile duct passes through
part of the pancreas before it joins with the pancreatic duct and empties into the first
part of the small intestine (the duodenum) at the ampulla of Vater.
Types of bile duct cancers by location
Cancer can start in any part of the bile duct system. Based on where the cancers are
(see the picture below), they're grouped into 3 types:
● Intrahepatic bile duct cancers
● Perihilar (also called hilar) bile duct cancers
●Distal bile duct cancers
Another name for bile duct cancer is cholangiosarcoma.
Cholangiosarcomas in these different groups cause different symptoms.
Intrahepatic bile duct cancers
These cancers start in the smaller bile duct branches inside the liver. Sometimes they're
confused with cancers that start in the liver cells, which are called hepatocellular
carcinomas, which are often treated the same way.
Perihilar (also called hilar) bile duct cancers
These cancers start at the hilum, where the left and right hepatic ducts have joined and
are just leaving the liver. These are also called Klatskin tumors. These cancers are
grouped with distal bile duct cancers as extrahepatic bile duct cancers.
Distal bile duct cancers
These cancers are found further down the bile duct, closer to the small intestine. Like
perihilar cancers, these are extrahepatic bile duct cancers because they start outside of
the liver.
Types of bile duct cancer by cell type
Bile duct cancers can also be divided into types based on how the cancer cells look
under the microscope.
Nearly all bile duct cancers or cholangiocarcinomas are adenocarcinomas, which are
cancers that start in glandular cells. Bile duct adenocarcinomas start in the mucous
gland cells that line the inside of the ducts.
Other types of bile duct cancers are much less common. These include sarcomas,
lymphomas, and small cell cancers. Our information does not cover these other types of
bile duct cancer.
Benign bile duct tumors
Not all bile duct tumors are cancer. Bile duct hamartomas and bile duct adenomas are
examples of benign (non-cancer) tumors.
Other cancers in the liver
The most common type of cancer that starts in the liver – much more common than
cholangiosarcoma – is hepatocellular carcinoma, which starts in cells that form theliver.
Cancers that start in other organs can spread to the liver. These are called liver
metastases or metastatic cancer to the liver. Their outlook and treatment are not the
same as cancer that starts in the liver (such as hepatocellular carcinoma) or bile ducts
(like cholangiocarcinoma), but instead depend on where the cancer started. For this
reason, it’s important to know whether a tumor in the liver started in bile ducts (is a
cholangiocarcinoma), or whether it's made up of cancer cells that started in another
organ (like the colon) and spread to the liver (is metastatic cancer).
●References
Abou-Alfa GK, Jarnagin W, Lowery M, et al. Liver and bile duct cancer. In: Neiderhuber
JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical
Oncology. 5th ed. Philadelphia, PA. Elsevier; 2014:1373-1395.
National Cancer Institute. Bile Duct Cancer (Cholangiocarcinoma) Symptoms, Tests,
Prognosis, and Stages (PDQ®)–Patient Version. March 22, 2018. Accessed at
[Link]/types/liver/patient/about-bile-duct-cancer-pdq on June 18, 2018.
Patel T, Borad MJ. Carcinoma of the biliary tree. In: DeVita VT, Lawrence TS,
Rosenberg SA, eds. DeVita, Hellman, and Rosenberg’s Cancer: Principles and Practice
of Oncology. 10th ed. Philadelphia, PA. Lippincott Williams & Wilkins; 2015:715-735.
See all references for Bile Duct Cancer
Last Medical Review: July 3, 2018 Last Revised: July 3, 2018
American Cancer Society medical information is copyrighted material. For reprint
requests, please see our Content Usage Policy.
Key Statistics for Bile Duct Cancer
Bile duct cancer (cholangiocarcinoma) is rare. About 8,000 people in the United States
are diagnosed with it each year. This includes both intrahepatic (inside the liver) and
extrahepatic (outside the liver) bile duct cancers. But the actual number of cases is
likely to be higher, because these cancers can be hard to diagnose, and some might be
misclassified as other types of cancer.
Bile duct cancer is more common in Southeast Asia, mostly because a parasitic
infection that can cause bile duct cancer is much more common there.
Bile duct cancer can occur at younger ages, but it's seen mainly in older people. The
average age of people in the US diagnosed with cancer of the intrahepatic bile ducts is
70, and for cancer of the extrahepatic bile ducts it's 72.
The chances of survival for patients with bile duct cancer depend to a large extent on its
location and how advanced it is when it's found. For more on this, see Survival Statistics
for Bile Duct Cancers.
Visit the American Cancer Society’s Cancer Statistics Center for more key statistics.
● References
American Cancer Society. Cancer Facts & Figures 2018. Atlanta, Ga: American Cancer
Society; 2018.
Antwi SO, Mousa OY, Patel T. Racial, ethnic, and age disparities in incidence and
survival of intrahepatic cholangiocarcinoma in the United States: 1995-2014. Annals of
Hepatology. 2018;17(2):274-285.
Howlader N, Noone AM, Krapcho M, Miller D, Bishop K, Kosary CL, Yu M, Ruhl J,
Tatalovich Z, Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER
Cancer Statistics Review, 1975-2014, National Cancer Institute. Bethesda, MD.
Accessed at [Link] based on November 2016 SEER
data submission, posted to the SEER web site, April 2017.
See all references for Bile Duct Cancer
Last Medical Review: July 3, 2018 Last Revised: July 3, 2018
American Cancer Society medical information is copyrighted material. For reprint
requests, please see our Content Usage Policy.
What's New in Bile Duct Cancer
Research?
Because bile duct cancer is rare, it's been hard to study. Most experts agree that
treatment in a clinical trial should be considered for any type or stage of bile duct
cancer. This way people can get the best treatment available now and may also get the
treatments that are thought to be even better.
Research on bile duct cancer is taking place in many university hospitals, medical
centers, and other institutions around the world. Each year, scientists find out more
about what causes the disease, how to prevent it, and how to better treat it. The new
and promising treatments discussed here tend to only be available in clinical trials.
Surgery
Doctors are constantly improving the surgical techniques used to treat bile duct cancers
and looking for ways to make surgery possible for more people.
For instance, sometimes surgery to remove the cancer might be possible, but it can’t be
done because it might not leave enough healthy liver behind. One option being studied
is to cut off the blood supply to the part of the liver that’s going to be removed (known as
portal vein embolization). As this part of the liver shrinks, the other part of the liver
grows to compensate. Over time, there might be enough healthy liver to go ahead with
the operation to remove the part of the liver with the tumor.
Better ways to use laparoscopic surgery are also being tested and compared to open
surgery. Adjuvant and neoadjuvant treatments, those used before and after surgery, are
also active areas of research interest. Doctors are looking for ways to combine other
treatments with surgery to improve outcomes.
Radiation therapy
Researchers are looking at better ways to use radiation therapy. One example is using
a different type of radiation called proton beam radiation therapy. This form of radiation
uses proton beams instead of the usual photon or electron beams. Protons are parts of
atoms that cause little damage to tissues they pass through but are very good at killing
cells at the end of their path. This means that proton beam radiation may be able to
deliver more radiation to the tumor while reducing side effects on normal tissues.
Other ways to use radiation therapy are also being studied. For example, doctors are
looking at whether radioactive stents placed inside bile ducts might help shrink tumors
and keep the ducts open longer than standard stents.
Another approach being widely studied is the injection of tiny radioactive beads into the
hepatic artery, which carries blood to the area of the cancer. The beads lodge in the
blood vessels near the tumor, where they give off small amounts of radiation. This is
called transarterial radioembolization or TARE. So far, studies have shown good
results with TARE, but more research is needed.
Chemotherapy
In general, the effects of chemo against bile duct cancer have been found to be limited,
but new drugs and new combinations of drugs are being tested. Studies are also
looking for better ways to combine chemo with other treatments, like surgery, radiation,
and liver transplant.
There's a lot of research interest in combining chemotherapy and targeted therapy.
Targeted therapy
Drugs have been developed that work differently from standard chemo drugs. These
drugs can target specific changes in cancer cells that help them grow and survive. They
can also change certain proteins made by the cancer cells to cause the cells to die.
Targeted drugs can work with the immune system to help it find and kill cancer cells,
too. Many of these drugs are being tested for use in treating bile duct cancer.
Many other kinds of cancers are already treated with targeted therapy. As researchers
learn more about the changes in bile duct cells that cause them to become cancer,
they're looking to use targeted drugs that focus on killing the cells with these changes.
For instance, some of these drugs target tumor blood vessels. Bile duct tumors need
new blood vessels to grow beyond a certain size. Bevacizumab (Avastin®), erlotinib
(Tarceva®), ramuciruman (Cyramza ®), and regorafenib (Stivarga®) are examples of
drugs that target blood vessel growth and are being studied against bile duct cancer.
Other drugs have different targets. For example, EGFR, a protein that helps cells grow,
is found in high amounts on some cancer cells. Drugs that target EGFR have shown
some benefit against many types of cancer. Some of these drugs, such as cetuximab
(Erbitux®) and panitumumab (Vectibix®) are now being studied for use in people with
bile duct cancer, often in combination with chemotherapy or other targeted drugs.
Other types of targeted therapy, such as MEK inhibitors (like tivantinib ) and anti-PD1
drugs (like pembrolizumab [Keytruda®]), are also being studied for use against bile duct
cancer.
Again, this is an active area of research, but a lot more research is needed to find out if
targeted therapy works and which drugs work best in treating bile duct cancer.
● References
Bréchon M, Dior M1 Dréanic J, et al. Addition of an antiangiogenic therapy,
bevacizumab, to gemcitabine plus oxaliplatin improves survival in advanced biliary tract
cancers. Invest New Drugs. 2018;36(1):156-162.
Deipolyi AR, Zhang YS, Khademhosseini A, et al. Portal Vein Embolization: Impact of
Chemotherapy and Genetic Mutations. J Clin Med. 2017;6(3).
Ha S, Alshahrani AA, Hwang S. ALPPS in a patient with periductal infiltrating
intrahepatic cholangiocarcinoma. Ann Hepatobiliary Pancreat Surg. 2017;21(4):223-
227. Hong TS, Wo JY, Yeap BY, et al. Multi-Institutional Phase II Study of High-Dose
Hypofractionated Proton Beam Therapy in Patients With Localized, Unresectable
Hepatocellular Carcinoma and Intrahepatic Cholangiocarcinoma. J Clin Oncol.
2016;34(5):460-468. Lu J, Guo JH, Zhu HD, et al. Palliative treatment with radiation-
emitting metallic stents in unresectable Bismuth type III or IV hilar cholangiocarcinoma.
ESMO Open. 2017;2(4):e000242.
Rizvi S, Khan SA, Hallemeier CL, Kellek RK, Gores GJ. Cholangiosarcoma -- evolving
concepts and therapeutic strategies. Nat Rev Clin Oncol. 2018;15(2):95-111.
Valle JW, Lamarca A, Goyal L, Barriuso J, Zhu AX. New Horizons for Precision
Medicine in Biliary Tract Cancers. Cancer Discov. 2017;7(9):943-962.
Zhu A. Future directions in the treatment of cholangiocarcinoma. Best Pract Res Clin
Gastroenterol. 2015;29(2):355-361.
See all references for Bile Duct Cancer
Last Medical Review: July 3, 2018 Last Revised: July 3, 2018
American Cancer Society medical information is copyrighted material. For reprint
requests, please see our Content Usage Policy.
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