Hilar cholangiocarcinoma

Hilar cholangiocarcinoma may not cause symptoms at first. Symptoms typically happen when the cancer grows and blocks the bile ducts, preventing bile from draining from the liver into the intestine. Signs and symptoms of hilar cholangiocarcinoma may include:

• Yellowing of the skin and the whites of the eyes, known as jaundice.
• Itching.
• Dark urine.
• Clay-colored stools.
• Stomach pain.
• Fatigue.
• Weight loss.

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Diagnosing hilar cholangiocarcinoma, also called perihilar cholangiocarcinoma or Klatskin tumor, often begins with a physical exam. Your healthcare professional also will likely ask about your health history. You may have imaging tests and blood tests. A sample of tissue may be taken for lab testing.

Imaging tests

Imaging tests make pictures of the inside of the body. They help your healthcare team see the location, size and spread of a hilar cholangiocarcinoma.

• Ultrasound is often the first test. It can show whether the bile ducts are blocked or enlarged.
• CT scans take detailed pictures of the liver and bile ducts, especially the blood vessels going into the liver. They help show if the cancer has spread or can be taken out with surgery.
• MRI gives even more detailed pictures of the liver and bile ducts.
• Magnetic resonance cholangiopancreatography (MRCP) is a special type of MRI that makes clear images of the bile ducts, liver, gallbladder and pancreas to help find the exact spot of the blockage or tumor.
• Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). Many people with hilar cholangiocarcinoma develop jaundice because the tumor blocks the bile ducts. When imaging shows a blockage, procedures such as ERCP or PTC may be used to look at the bile ducts, obtain tissue samples and define the extent of the tumor. These procedures can also help relieve the blockage by placing stents or drainage tubes to allow bile to flow, which often improves symptoms and helps prevent infection.

Liver function tests

Blood tests to measure your liver function can give your healthcare team clues about what’s causing your symptoms.

Tumor marker tests

A tumor marker test looks for substances called tumor markers. These markers are often made by cancer cells or healthy cells in response to cancer. Checking the level of the tumor markers in your blood may give your healthcare team more clues about your diagnosis.

Tumor markers for hilar cholangiocarcinoma include carbohydrate antigen 19-9, also called CA 19-9, and carcinoembryonic antigen, also called CEA. These are proteins that are overproduced by cholangiocarcinoma cells. Then levels of these tumor markers are high, it may mean a more advanced cancer.

Having high levels of CA 19-9 and CEA in your blood doesn’t always mean you have hilar cholangiocarcinoma. This also can happen with other bile duct conditions, such as bile duct inflammation and obstruction.

Biopsy

A biopsy is a procedure to remove a sample of tissue for testing in a lab. To get a sample of tissue from a hilar cholangiocarcinoma, a healthcare professional might use a procedure called endoscopic retrograde cholangiopancreatography (ERCP). During this procedure, a health professional puts a thin tube with a camera down the throat. The tube, called an endoscope, goes through the stomach and into the small intestine to get to the bile duct. The endoscope is used to put contrast material into the bile duct to help it show more clearly on X-rays. The X-ray images help guide the endoscope to the right place. A brush tool can extend from the endoscope to collect cells from the cancer.

Treatment for hilar cholangiocarcinoma depends on the location and extent of the cancer. Surgery is the main treatment and offers the only chance to cure the disease if all the cancer can be taken out. For some people, a liver transplant also may be an option.

If surgery or transplant is not possible, other treatments are used to control the cancer and ease symptoms. These may include radiation therapy, radiofrequency ablation and medicines. Medicines used to treat hilar cholangiocarcinoma include chemotherapy, targeted therapy and immunotherapy.

Your healthcare team considers many factors when creating a treatment plan. These factors may include your overall health, the type and stage of your cancer, and your preferences.

Surgery

The goal of surgery for hilar cholangiocarcinoma is to take out all of the cancer. But it may not be an option for everyone. This cancer grows near many important structures, which can make surgery difficult. Less than half of people can have surgery when they are first diagnosed because many already have cancer that has spread too far.

When surgery is possible, options may include:

• Surgery to remove the cancer. Removing the cancer often includes taking out and rebuilding part of the bile duct and nearby structures, such as the surrounding ducts and parts of the liver and gallbladder.
• Removing nearby lymph nodes. Lymph nodes may be taken out to be tested for cancer. Removing cancerous lymph nodes may help prevent the spread of the cancer.
• Liver transplant. Sometimes, using surgery to take out just the cancer is not possible. If the cancer can’t be removed with surgery but has not spread outside the liver, radiation therapy can be combined with a liver transplant. After radiation therapy, a surgeon removes the cancer and all of the liver. Then the surgeon puts a healthy liver from a donor into the body.
• Biliary drainage. Biliary drainage uses a procedure to restore the flow of bile if cancer blocks a bile duct. This procedure might involve placing a thin tube into the bile duct to drain the bile. Sometimes a surgeon does a bypass surgery to route the bile duct around the cancer. Another kind of procedure might involve placing stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve symptoms of hilar cholangiocarcinoma.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

For hilar cholangiocarcinoma, radiation therapy is sometimes combined with chemotherapy to kill cancer cells that remain after surgery. Radiation therapy also may be combined with chemotherapy to treat cancer that has spread to the lymph nodes.

Sometimes the healthcare team uses other procedures to place radiation close to the cancer. One way to do this is with a procedure called radioembolization. It involves putting small beads that hold radiation into a blood vessel that leads to the cancer. The beads travel through the blood vessel and block the blood supply to the cancer. The beads give off radiation directly to the cancer.

Chemotherapy

Chemotherapy treats cancer with strong medicines. Many chemotherapy medicines exist. Most chemotherapy medicines are given through a vein. Some come in pill form.

For hilar cholangiocarcinoma, chemotherapy may be used after surgery to kill any remaining cancer cells. For those with advanced cancer that can’t be taken out with surgery, chemotherapy may be an option to slow the growth of the cancer.

Chemotherapy is sometimes combined with radiation therapy after surgery to kill any remaining cancer cells. Chemotherapy also may be combined with radiation to treat cancer that has spread to the lymph nodes.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in the cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die. For those with advanced hilar cholangiocarcinoma that can’t be taken out with surgery, targeted therapy may be an option. Some cancer cells might be tested to see if targeted therapy is likely to work on the cancer.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body’s immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells, such as cancer cells, that shouldn’t be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells. For those with advanced hilar cholangiocarcinoma that can’t be taken out with surgery, immunotherapy may be an option.

Palliative care

Palliative care is a special type of healthcare that helps you feel better when you have a serious illness. If you have cancer, palliative care can help ease pain and other symptoms. A healthcare team that may include doctors, nurses and other specially trained health professionals provides palliative care. The care team’s goal is to improve quality of life for you and your family.

Palliative care specialists work with you, your family and your care team. They provide an extra layer of support while you have cancer treatment. You can have palliative care at the same time you’re getting strong cancer treatments, such as surgery, chemotherapy, immunotherapy, targeted therapy or radiation therapy.

The use of palliative care with other treatments can help people with cancer feel better and live longer.