Overview of Cholangiocarcinoma
Cholangiocarcinoma is a rare and often fatal cancer that affects
the bile ducts.
The bile ducts are a series of tubes that transport digestive
juices called bile from your liver, where it’s made, to your gallbladder, where
it’s stored. From the gallbladder, ducts carry bile to your gut, where it helps
to break down fats in the foods you eat.
In most cases, cholangiocarcinoma arises in those parts of the
bile ducts that lie outside the liver. Rarely, the cancer can develop in ducts
that are located within the liver.
Types of Cholangiocarcinoma
Most often, cholangiocarcinomas are part of the family of tumors
known as adenocarcinomas, which originate in glandular tissue.
Less commonly, they’re squamous cell carcinomas, which develop in
the squamous cells lining your digestive tract.
Tumors that develop outside of your liver tend to be fairly
small, while those in the liver can be either small or large.
What Are the Symptoms of Cholangiocarcinoma?
Your symptoms may vary depending on the location of your tumor,
but they can include the following:
- The most
common symptom is jaundice, which is yellowing of the skin. This may
develop at an early or late stage, depending on the tumor site.
- Dark urine and pale stools may develop.
may occur, and it can be caused by jaundice or by the cancer.
- You can
have pain in your abdomen that penetrates to your back. This tends
to occur as the cancer progresses.
Additional rare but serious side effects may include enlargement
of your liver, spleen, or gallbladder.
You may also have more general symptoms, such as:
- a fever
- loss of appetite
- weight loss
What Causes Cholangiocarcinoma?
Doctors don’t understand why cholangiocarcinoma develops, but it’s
thought that chronic inflammation of the bile ducts and chronic parasitic
infections can play a part.
Who Is at Risk for Cholangiocarcinoma?
You’re more likely to develop cholangiocarcinoma if you’re male
or older than 65 years of age. Certain conditions can increase your risk for
this type of cancer, including:
- liver fluke, or parasitic flatworm, infections
- bile duct infections or chronic inflammation
- ulcerative colitis
- exposure to chemicals used in industries such as
- rare conditions, such as primary sclerosing
cholangitis, hepatitis, lynch syndrome, biliary papillomatosis
How Is Cholangiocarcinoma Diagnosed?
Your doctor will perform a physical examination and may take
blood samples. Blood tests can
check how well your liver is functioning and can be used to look for substances
called tumor markers. Levels of tumor markers might rise in people with
You may also need imaging
scans such as an ultrasound, CT scan, and MRI scan. These provide
pictures of your bile ducts and the areas around them, and they can reveal
tumors. Imaging scans can also help to guide your surgeon’s movements if they
decide to remove a sample of tissue to check for cancer cells. This is called a
A procedure known as an endoscopic retrograde
cholangiopancreatography (ERCP) is sometimes done. Your surgeon will pass a
long tube with a camera down your throat and into the part of your gut where
the bile ducts open. Your surgeon may inject dye into the bile ducts. This
helps the ducts show up clearly on an X-ray, revealing any blockages. In some
cases, they’ll also pass a probe that takes ultrasound pictures into the area
of your bile ducts. This is called an endoscopic ultrasound scan.
In the test known as percutaneous
transhepatic cholangiography (PTC), your doctor takes X-rays after
injecting dye into your liver and bile ducts. In this case, they inject the dye
straight into your liver through the skin of your abdomen.
How Is Cholangiocarcinoma Treated?
Your treatment will vary according to the location and size of
your tumor, whether it has spread, or metastasized, and the state of your
Surgical treatment is the only option that offers a cure,
especially if your cancer has been caught early and hasn’t spread beyond your
liver or bile ducts. Sometimes, if a tumor is still confined to the bile ducts,
you may only need to have the ducts removed. If the cancer has spread beyond
the ducts and into your liver, a section of the liver, or even your entire
liver, may have to be removed. If your entire liver must be removed, you’ll
need a liver transplant to replace it.
If your cancer has invaded nearby organs, a Whipple procedure may be carried
out. In this procedure, your surgeon will remove:
- the bile ducts
- the gallbladder
- the pancreas
- sections of your stomach and gut
Even if your cancer cannot be cured, you can have surgery to
treat the blocked bile ducts and relieve some of your symptoms. Typically, the
surgeon either inserts a tube to hold the duct open or creates a bypass. This
can help to treat your jaundice. A blocked section of the gut can also be
You might need to receive chemotherapy or radiation treatments
following your surgery.
What Is the Long-Term Outlook for People
If it’s possible to remove your tumor completely, you have a
chance of being cured. Your outlook is generally better if the tumor isn’t in
Many people aren’t eligible for surgery to remove the tumor by
removing all or part of the liver or bile duct. This can be because the cancer
is too advanced, has already metastasized, or is in an inoperable location.