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 is made, to your gallbladder, where it is stored. From there, the 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. In about 10 percent of cases, they are 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 Causes Cholangiocarcinoma?
Doctors do not understand why cholangiocarcinoma develops, but it is thought that chronic inflammation of the bile ducts and chronic parasitic infections can play a part. Each year, there are approximately 2,500 cases of cholangiocarcinoma in the United States.
Who Is at Risk for Cholangiocarcinoma?
You are more likely to develop cholangiocarcinoma if you are male or older than 65 years of age. Other conditions can increase your risk for this type of cancer, including:
- liver fluke (parasitic flatworm) infections
- bile duct infections or inflammation
- ulcerative colitis
- exposure to chemicals used in industries such as aircraft manufacturing
What Are the Symptoms of Cholangiocarcinoma?
Your symptoms may vary depending on the location of your tumor, but they can include:
- jaundice (yellowing of the skin) — the most common sign, which may develop at an early or late stage, depending on the tumor site
- dark urine and pale stools
- itching -- this can be caused by jaundice or by the cancer itself
- pain in your abdomen that can penetrate to your back — this tends to occur as the cancer progresses
An additional rare but serious symptom is the enlargement of your liver, spleen, or gallbladder.
You may also have more general symptoms, such as:
- chills and fever
- loss of appetite
- weight loss
Your doctor will examine you 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 cholangiocarcinoma.
You may also need imaging scans such as an ultrasound, CT (computerized tomography) scan, and MRI (magnetic resonance imaging). These provide pictures of your bile ducts and the areas around them, and can reveal tumors when they are present. Imaging scans can also help to guide the surgeons’ movements if they decide to remove a sample of tissue (biopsy) to check for cancer cells.
A procedure known as an ERCP (endoscopic retrograde cholangiopancreatography) is sometimes carried out, in which a long tube with a camera is passed down your throat and into the part of your gut where the bile ducts open. The 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, a probe that takes ultrasound pictures is also passed down into the area of your bile ducts. This is called an endoscopic ultrasound scan.
In the test known as PTC (percutaneous transhepatic cholangiography), X-rays are taken after injecting dye into your liver and bile ducts. In this case, the dye is injected straight into your liver through the skin of your abdomen.
Your treatment will vary according to the location and size of your tumor, whether it has spread (metastasized), and the state of your overall health.
Surgical treatment is the only option that offers a cure, especially if your cancer has been caught early and has not 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, a liver transplant will be required to replace it.
If your cancer has invaded nearby organs, a Whipple’s procedure may be carried out. Here, the bile ducts, gallbladder, pancreas, and sections of your stomach and gut are removed.
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 treated surgically.
You might need to receive chemotherapy or radiation treatments following your surgery.
What Can Be Expected in the Long Term?
If it is possible to remove your tumor completely, you have a chance of being cured.
Up to 40 percent of people who have their tumors removed will survive for five years or more. Your outlook is generally better if your tumor is not located in your liver.
Unfortunately, close to 90 percent of patients are not eligible for surgery to remove all or part of the liver or bile duct to remove the tumor because their cancer has already metastasized, or is in an inoperable location. In general, cholangiocarcinoma patients survive for about 12 to18 months after diagnosis.