Cholangiocarcinoma

Cholangiocarcinoma is a rare cancer that starts in your bile ducts. It’s also called bile duct cancer. Bile ducts are tubes that bring bile from your liver and gallbladder to your small intestine. Bile is a fluid that helps you digest fats. 

This disease is aggressive, which means it spreads fast. It doesn’t often cause symptoms in the early stages, when surgery might cure it. Most people don’t find out they have it until it’s already advanced. At this point, it’s challenging to treat.

Still, no two diagnoses are the same. Your treatment plan and outlook depend on lots of factors that your healthcare provider will discuss with you.

​Types

There are four types:

• Intrahepatic cholangiocarcinoma: The tumor forms in bile ducts inside your liver.
• Perihilar (hilar) cholangiocarcinoma: The tumor starts just outside your liver where the smaller bile ducts from inside your liver merge to form the common hepatic duct. It’s the most common form of bile duct cancer. Another name for it is a Klatskin tumor.
• Distal cholangiocarcinoma: The tumor starts outside your liver, in the ducts closer to your small intestine.
• Gallbladder cancer: The tumor starts in your gallbladder, which is also made of bile duct cells.

Symptoms of cholangiocarcinoma

Symptoms don’t usually start until the tumor blocks a bile duct. At this point, it’s usually advanced. Symptoms include:

• Abdominal (belly) pain
• Fever
• Fatigue
• Itchy skin
• Dark pee or pale stools
• Nausea and vomiting
• Signs of jaundice, like yellowing in your skin or in the whites of your eyes
• Unexplained weight loss

Cholangiocarcinoma causes

Experts don’t know what causes cholangiocarcinoma. But conditions that cause long-term inflammation in your bile ducts may play a role.

Ongoing damage from inflammation can cause changes in cell DNA. DNA contains the instructions that tell cells how to work properly. But damaged DNA can cause cells to keep growing and dividing when they shouldn’t. The cells can cluster together to form tumors that damage tissue.

Risk factors

Cholangiocarcinoma is most common in Southeast Asia. This is because it’s sometimes a complication of an infection from a Chinese liver fluke parasite called clonorchiasis.

Most people with this disease don’t have any known risk factors. But experts have found some conditions that may make you more likely to develop it, such as:

• Scarring in your bile ducts, called primary sclerosing cholangitis
• Bile duct stones or cysts
• Inflammatory bowel disease, especially ulcerative colitis
• Infections, including clonorchiasis and hepatitis B and C
• Liver disease, including types related to alcohol use, obesity and diabetes
• Genetic conditions, including Caroli disease, Lynch syndrome and cystic fibrosis

How doctors diagnose this condition

Your healthcare provider will consider your symptoms and medical history. You may need:

• Blood tests: High enzyme levels in your blood may mean liver or bile duct damage. Signs of a tumor may include high levels of CA 19-9 or carcinoembryonic antigen (CEA).
• Imaging tests: Imaging helps providers see tumors inside your body. Tests include ultrasounds, CT scans, MRIs and MRCP. MRCP uses dye and specialized images to enhance the clarity of your bile ducts during an MRI. 
• Endoscopic ultrasound: This test uses a scope with a camera to show your bile ducts up close. The scope goes from your mouth to your small intestine. The image shows up on an ultrasound.
• Endoscopic X-rays: Endoscopic retrograde cholangiopancreatography (ERCP) uses a specialized scope and a dye to make images of your bile ducts on an X-ray. Providers can also use tools inside the scope to drain a blocked bile duct.
• Percutaneous transhepatic cholangiography (PTC): Places a drain in your bile ducts if they’re blocked, and an ERCP isn’t an option. The drain tube connects to a bag outside of your body.
• Biopsy: Your provider may take a tissue sample to test for cancer cells.
• Genetic tests: If results show cancer, your provider will look for gene changes in the cancer cells. If they’re present, you may be able to get treatments that target these cells for destruction.
• Liquid biopsy: Your provider may test blood if the tumor’s location makes taking a large enough sample for gene testing difficult. This is called a liquid biopsy.

Staging

Providers use your test results to stage the disease, or learn how advanced it is. Staging systems vary based on the type of cholangiocarcinoma.

But in general, they rank the disease from stage 0 to stage IV (4). Stage 0, or carcinoma in situ, is the earliest stage. This means you have abnormal cells that could become cancer, but haven’t yet. Stage IV is the most advanced. At this stage, it’s spread beyond your bile ducts to distant organs.

How is cholangiocarcinoma treated?

Surgery can treat bile duct cancers that haven’t spread. Other treatments can make surgery more effective. Or they can help you live longer without surgery. As this disease doesn’t always respond to current treatments, your healthcare provider may suggest you take part in a clinical trial to access new therapies.

Surgery

Surgery is the main treatment for earlier-stage disease. But it’s only an option when your surgeon can remove the tumor without harming your liver or key structures that drain your bile ducts. Only about 2 to 3 out of every 10 people diagnosed with bile duct cancer meet these criteria.

A liver transplant is a possible cure for some types of the disease. This procedure replaces your liver with one from a donor.

Chemotherapy

This treatment sends drugs throughout your body to destroy cancer cells. You may need it after surgery to get rid of any remaining cancer cells. Chemo can sometimes shrink tumors so they’re small enough to remove with surgery. Or it may help you live longer with fewer symptoms.

Special types of chemotherapy allow providers to send the treatment directly to a tumor. They include:

• Transarterial chemoembolization (TACE)
• Drug-eluting bead transarterial chemoembolization (DEB-TACE)
• Hepatic artery chemo-infusion (HAI)

Radiation therapy

This treatment uses X-rays from a machine to kill cancer cells or shrink tumors. Providers often use it alongside chemotherapy. Sometimes, providers place tiny beads of radiation in the blood vessels supplying a tumor to shrink it. This is called transarterial radioembolization (TARE).

Targeted therapy

This treatment can destroy cancer cells that have certain gene errors. They include genes linked to several types of cancers, including FGFR2, HER2 and BRAF. The errors cause cancer cells to make proteins that fuel their growth. Targeted therapies block the proteins.

Immunotherapy

This treatment helps your body fight cancer. Some cancer cells contain a protein that prevents your immune cells from attacking. Immunotherapy disables this protein so immune cells can fight the cancer.

Other treatments

Your provider may suggest lesser-known cancer treatments that can provide symptom relief. In some cases, they help people live longer. They include:

• Biliary drainage: Uses a stent (small, hollow tube) to drain a blocked bile duct that’s causing symptoms
• Radiofrequency ablation: Uses extreme heat to destroy cancer cells
• Photodynamic therapy: Uses light to activate a chemical reaction that kills cancer cells

What can I expect if I have this condition?

Bile duct cancer is an aggressive and often fatal disease. It can be cured if surgery removes all of it. But surgery isn’t always successful. The tumor can come back. Your provider will monitor you to ensure you get the treatment you need if it does.

Most cancers aren’t diagnosed until they’re too advanced for surgery. At this point, the focus shifts from curing the cancer to slowing its progression. Palliative care is important, too. It can help you manage the physical and emotional challenges of living with this disease.

Survival rate for cholangiocarcinoma

Fewer than 2 out of 10 people diagnosed with this disease survive past five years. 

Still, it’s important to remember that lots of things affect your life expectancy. And new treatments are helping people with cancer live longer with fewer symptoms. Plus, survival rates report on numbers from previous years. They don’t reflect the advancements.

This is why it’s a good idea to talk to your healthcare provider about your prognosis based on your situation.