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Perihilar and Distal Cholangiocarcinoma

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Three Types of Cholangiocarcinoma

Cholangiocarcinoma (co-LAN-gee-oh-car-see-NO-mah) is a rare form of cancer. It starts, or originates, in the cells that line the liver’s bile ducts.

Cholangio refers to the cells that line the bile ducts. Carcinoma means cancer.

There are three types of cholangiocarcinoma:

1. Perihilar. This cancer arises from large bile ducts. It involves the ducts and theliver. This condition is typically treated with surgery, a liver transplant, a stent, or chemotherapy. Peri refers to the area where the bile duct and vessels connect to the liver.

2. Distal. This cancer arises from the large common bile duct. It may be found on ornear the pancreas. This is typically treated with a stent, surgery or chemotherapy. Distal refers to distant, meaning away from the liver.

3. Intrahepatic.This cancer arises from the small bile ducts inside the liver. (Intra means inside.) It forms a growth or a lesion. It is typically treated with surgical resection or a form of therapy: radiofrequency ablation, chemoembolization or chemotherapy.

Perihilar and distal cholangiocarcinomas are more common than intrahepatic cholangiocarcinoma.

Perihilar and distal cholangiocarcinoma are often called extrahepatic cholangiocarcinoma. Extra means outside and hepatic refers to the liver.

How the Liver Normally Works

The liver is the largest solid organ in the body. It sits in the upper right side of the chest, under the ribs. (See Figure 2.) The liver’s biggest job is to produce bile. Bile helps you digest food. it is very helpful in digesting fats.

Your liver has a system of tubes called bile ducts. Bile ducts collect and drain the bile from the liver and carry it into the intestine. There, it meets with your food.

The largest duct in the liver is called the common bile duct. This duct is connected — by the cystic duct — to the gallbladder. Bile ducts are lined with small cells called cholangiocytes. Cholangiocytes secrete fluid into the bile as it passes into the intestine.They also absorb nutrients from the bile.

What Is Cholangiocarcinoma?

Cholangiocarcinoma is a cancer that comes from the cholangiocytes. Cholangiocytes are the small cells that line the inside of the bile ducts.

Perihilar and distal cholangiocarcinomas start in the ductal structures between the liver and the intestine. (See Figure 3.) The tumors block the bile duct so the duct can’t carry bile to the intestines. A tumor also may be called a mass.

These tumors may or may not be easy to see on an imaging test like an MRI or CT scan. (See also “Diagnosing cholangiocarcinoma.”)

These specific types of cancer often cause skin to turn yellow. This condition is called jaundice.

Risk Factors and Symptoms

Risk factors

Certain conditions, called risk factors, may increase the risk for getting perihilar or distal cholangiocarcinoma, including:

• Chronic inflammatory diseases of the bile ducts, such as primary sclerosing cholangitis (PSC).

• Stones in the bile duct.

• Cystic diseases of the bile ducts.

• Infections from parasites. These are mainly a problem in Asia.

For people who do not have one of those conditions, there is no known cause for cholangiocarcinoma. There is no known link between cholangiocarcinoma and diet or other lifestyle choices. This disease does not run in families.

Symptoms

Having a yellow tint to the skin, a condition called jaundice, is the most common symptom of perihilar and distal cholangiocarcinomas. It happens when there is a build-up of bilirubin in the blood. Most bilirubin is excreted in bile. Some bilirubin is excreted in urine.

Other symptoms

• Dark urine. This happens when too much bilirubin is excreted into the urine.

• Light-colored stools. This is caused by not having enough (or any) bile in the stool.

• Itchy skin. This is a reaction to bile in the blood.

Bacterial infection of the bile ducts is common, especially after a procedure is done to relieve blockage in the bile ducts. These infections may cause fever, chills and pain.

Diagnosing Cholangiocarcinoma

If your physician believes that you have perihilar or distal cholangiocarcinoma, you will have blood tests. These tests check for tumor markers and enzymes that increase when the bile ducts are blocked. The blood-test results may identify the need for other tests.

Some of the tests used to diagnose cholangiocarcinoma are listed here. You may not need all of these tests.

• Cholangiogram. A cholangiogram is an X-ray of the bile ducts, the pancreatic ducts or both. A thin, flexible tube is inserted through your mouth into the stomach and then into the part of the intestine where the bile duct drains into the bowel. Dye is injected into the bile duct, and X-rays are taken.

This is also called an endoscopic retrograde cholangiopancreatography, or ERCP.

A cholangiogram also may be done by inserting a needle directly into a bile duct in the liver. Dye is injected, and X-rays are taken. This is also known as a percutaneous transhepatic cholangiography, or PTHC.

If the ERCP shows a blocked duct, during the ERCP your gastroenterologist may place a small stent in the bile duct in an attempt to help the bile drain. This may lessen the jaundice in your skin. During this procedure, your gastroenterologist also may remove stones or perform other therapies and look at the inside of your stomach and the upper part of your small intestine, the duodenum. (See Figure 4.)

• Ultrasound with Doppler (ultrasonography). This test uses sound waves to make pictures of internal organs. The test also shows blood flow in the liver. An ultrasound may give information about the shape, texture and makeup of tumors. And it may show how close tumors are to the liver’s blood vessels.

• Computed tomography (also called CT scan or CAT scan). This test uses X-rays to produce cross-sectional images of your body.

• Magnetic resonance imaging (MRI). An MRI creates images using a magnetic field and radio waves. Contrast dye also may be used.

Using FISH

“FISH” stands for fluorescent in situ hybridization. This is a test that counts chromosomes. One of the signs of cancer is to have too many chromosomes in your cells. FISH is another way to get results from biopsy samples.

Treating Cholangiocarcinoma

Treatment depends on many issues, including:

• Location and size of the tumor.

• Whether the tumor has spread to other organs or nearby blood vessels.

• Other specific health-related issues, such as your age and overall health.

Treatment may be done to help cure you of cancer. This is called curative treatment. Or it may be done to make you feel better when there is no chance for cure. This is called palliative treatment.

Surgery

Surgery offers the best chance for a cure. It may be done to remove the tumor or, for some conditions, to transplant a new liver.

Surgery may be done through one large incision in the abdomen. This is called an open surgery. Or it may be done using a few, smaller incisions. This is called laparoscopic surgery. Laparoscopic surgery is not an option for some people.

During surgery, part of the liver may be removed with the bile ducts. The part of the liver that is removed can regrow. This is called regeneration. Losing part of the liver usually does not lead to long-term health problems.

In addition, some of the bile ducts may be removed during surgery. Since the bile still needs to drain from the liver, your surgeon may re-route the bile’s path. This is done by sewing part of the intestine to the bile ducts that are still in the liver.

Risks and possible complications of surgery

Possible problems, or complications, of surgery include:

• Bile leakage into the abdomen. You may need another procedure to find and correct this condition.

• Infection. Antibiotic medication typically is used to treat an infection.

• Jaundice and a longer hospital stay. These may happen if your liver doesn’t regrow (regenerate) or it regrows very slowly.

• Hernia at the incision site(s). An incisional hernia happens when soft tissue, of the intestines, pushes through a weak spot in your abdominal wall. You may need another procedure to correct a hernia.

There is also the risk that tumors may come back after surgery. You may talk to your health care provider about this if it happens to you.

Another risk is a condition called cholangitis. See the boxed text for information about this life-threatening condition.

Cholangitis: A life-threatening complication

Cholangitis is an infection of a bile duct. It is usually caused by bacteria that move up from the place where a bile duct meets the first part of your small intestine. The infection usually happens as a result of something blocking a bile duct.

Early signs can include:

• A fever (can be either low or high-grade).

• Uncontrollable shaking.

• Dark-colored urine or light-colored stools.

• Yellowing of the skin and whites of the eyes, called jaundice.

• Abdominal pain on your upper right side and above your belly button. Most people describe the pain as dull but severe.

Later signs can include:

• Confusion.

• Feeling faint or lightheaded.

If you have symptoms of cholangitis, seek emergency medical care immediately. Treatment may include antibiotics, an outpatient procedure or surgery depending on your needs. Do not delay getting help.

Liver transplantation

If the cancer can’t be completely removed and it has not spread outside the liver, a liver transplant may be an option for some people.

Radiation therapy and chemotherapy would be done before the transplant. Also, you may have surgery to see whether the disease has spread outside the liver. For some, rare people, a liver transplant may cure the condition. However, it’s also true that tumors may come back after a transplant.

Risks and complications of transplantation

Some of the problems that may come with transplant surgery are the same as with any surgery: infection, bleeding and blood clots. Rare complications include heart attack, stroke and death. After liver transplant, other problems may include organ rejection, blocked blood vessels and blocked or leaking bile ducts. You may develop high blood pressure or high blood sugar.

Each of these possible problems is treated differently.

Chemotherapy

Chemotherapy uses powerful drugs to kill cancer cells. It is somewhat effective as a treatment for cancer. However, chemotherapy is not a treatment that could cure cancer.

Chemotherapy may be used throughout the body. This is called systemic treatment.

Or it may be done just in the area of the cancer. This is called regional treatment.

Chemotherapy may be used alone or with other treatment. Gemcitabine-cisplatin is a common drug combination used to treat this disease. Another drug, 5-fluorouracil, or 5-FU, also is commonly used.

Risks and complications of chemotherapy

Possible problems, or complications, of chemotherapy include:

• Fatigue and an overall ill feeling for a few days following chemotherapy treatment.

• A decrease in blood counts for your white blood cells, platelets or both.

• Nerve tingling, called neuropathy.

Biliary stents

Cholangiocarcinoma tumors may block, or obstruct, the bile ducts. This can lead to itchy skin, jaundice, dark urine, and poor absorption of nutrients. A blocked bile also damages the liver.

To treat the blockage, your gastroenterologist may put a thin, hollow tube in the duct, moving it through or across the tumor. This tube is called a stent. (See Figure 5.)

If your condition cannot be cured, placing a biliary stent may help relieve some of the symptoms, such as jaundice and itchy skin. This palliative treatment may help you feel more comfortable.

Risks and complications of biliary stents

There is a risk for a bile-duct infection. Antibiotic medication may be used to prevent or treat an infection.

Also, the stent could become clogged or need to be replaced for another reason. Stents in the bile ducts often get clogged because bile is fairly thick. You would need another procedure to replace a stent.

Questions and Answers

What is my life expectancy if the tumor cannot be removed?

How long you live depends on your overall health, how much of the tumor was removed during surgery and how well you respond to treatment.

For people with advanced cancer who are treated, a two-year survival is fairly common. If no treatment is done, the survival time is usually less than 18 months.

Are there any experimental treatments that could help?

The cause for cholangiocarcinoma is not known. But medical research has helped health care providers understand more about the disease. In the future, clinical trials may be available to test new therapies. If you would like more information about this, talk to your health care provider.

Is there any special diet that I should follow?

There is no special diet that will improve the course of your disease. However, you may feel better if you eat a few small meals per day instead of three large meals.

What symptoms will I have as the disease progresses?

As the disease continues, most people notice that they aren’t very hungry, lose weight and have less energy than they used to have. This disease usually is not painful.

Are members of my family at risk for cholangiocarcinoma?

Cholangiocarcinoma does not run in families. You cannot “catch” this disease or give it to anyone by hugging, kissing or having sexual relations.

For More Information

Your Midas providers have walked this journey with many people.

Please call us if you have any questions, and let us know how we may help you.