Bile Duct Cancer

1 What is Bile Duct Cancer?

Bile duct cancer, also called cholangiocarcinoma is a rare type of cancer that develops in the slender tubes (bile ducts) that carry the digestive fluid– bile to your small intestine. It is common among people older than the age of 50.

It is an aggressive cancer that progresses very quickly, and is hard to treat. It can occur in the bile ducts that are either outside or inside the liver, but cancer of the bile duct outside the liver is most common. It causes signs and symptoms such as yellowing of the skin and eyes (jaundice), intense itchiness of the skin, fever, pain in the abdomen, and white-colored stools.

The treatment options include radiation therapy, chemotherapy, and surgery.

2 Symptoms

The signs and symptoms caused by bile duct cancer include the following:

  • Jaundice (yellowish discoloration of the skin or sclera of the eyes)
  • Dark colored urine
  • Greasy clay colored stool
  • Pain in the abdomen
  • Nausea and vomiting
  • Fever
  • Itchy skin
  • Weight loss for no apparent reasons

3 Causes

Although the exact cause of bile duct cancer is not known, researchers have found a link between certain risk factors such as irritation or inflammation of the bile ducts and bile duct cancer.

Inflammation may lead to changes in the DNA of cells, which results in abnormal growth of cells and development of cancers. There are some genes that control the growth of cells, cell division, and cell death. Genes that help in cell growth, division, and keeping them alive are called oncogenes. Genes that retard division of cells or help in death of cells at the right time are called tumor suppressor genes. Mutations in the DNA that cause activation of oncogenes and deactivation of tumor suppressor genes result in cancers. Actually, for a cancer to develop, there should be changes occurring in different genes.

Gene mutations that cause bile duct cancers are acquired during ones life rather than being inherited from parents. Acquired changes in the TP53 tumor suppressor gene are the reason for most bile duct cancers. Other genes that play a role in bile duct cancers include KRAS, HER2, and MET. 

4 Making a Diagnosis

In order to plan for an appropriate treatment, the following tests and procedures are performed to detect diagnose, and stage bile duct cancer:

  • Physical examination and history: Your doctor will perform a careful physical examination to check for general signs of health, including the signs of disease, such as lumps or anything that is abnormal. A history of your past illnesses and treatments will also be taken.
  • Liver function tests: Your blood sample will be tested for the amounts of bilirubin and alkaline phosphatase that gets released from the liver. An elevated level of these substances is a sign of liver disease that is caused by bile duct cancer.
  • Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker test: In this test, a sample of blood, urine, or other tissues are checked to assess the amount of certain substances produced by organs, tissues, or tumor cells in the body. These substances are called tumor markers, and are linked with specific types of cancer when found in increased amounts in the body. Elevated levels of carcinoembryonic antigen (CEA) and CA 19-9 indicate a bile duct cancer.
  • CT scan (CAT scan): This scan makes a series of detailed images of internal organs of our body, such as the abdomen, which is taken from different angles. The pictures are created with the help of a computer linked to an X-ray machine. A special dye may be injected into your vein or swallowed in order to help make the organs or tissues appear more clearly.
  • MRI (magnetic resonance imaging): A procedure that makes use of a magnet, radio waves, and a computer to create a series of detailed images of the internal organs of our body. 
  • MRCP (magnetic resonance cholangiopancreatography): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of organs such as the liver, bile ducts, gallbladder, pancreas, and pancreatic duct.
  • Biopsy: During biopsy, a sample of cells and tissues are removed and viewed under a microscope to check for signs of cancer. Various procedures such as laparoscopy, percutaneous transhepatic cholangiography (PTC), endoscopic retrograde cholangiopancreatography (ERCP) are used to obtain these samples and diagnose bile duct cancer, and the type of procedure depends on how well you can tolerate surgery.

Getting Ready for Your Appointment

  Prepare a list of questions you may want to ask your cancer care team. For example, you can consider these questions:

  • Where exactly is my cancer located?
  • Has cancer spread beyond the bile ducts?
  • What is the stage of my cancer, and what does that mean?
  • What tests do I need before considering the treatment options?
  • What treatment options do I have?
  • What do you suggest and why?
  • What risks or side effects are involves with the treatments you suggest?
  • How long will be the course of treatment? 
  • What are the chances of my cancer being cured with these treatments?
  • What type of follow-up care might be required after treatment?

5 Treatment

There are three standard treatment options for patients with bile duct cancer. These include:

Surgery: The surgical procedures used to treat bile duct cancer include:

  • Surgical removal of the bile duct: This is indicated if the tumor is small and confined to the bile duct only. During the procedure, lymph nodes are removed and tissue from the lymph nodes is viewed under a microscope to check if there is cancer.
  • Partial hepatectomy: In this procedure, the portion of the liver with cancer is removed. The resected part may in the form of a wedge if tissue, an entire lobe, or larger part of the liver along with some normal tissue.

   Whipple procedure: In this surgical procedure, the head of the pancreas, the gallbladder, portion of the stomach and small intestine is removed along with the bile duct. 

Some patients may be given chemotherapy or radiation therapy after the surgery to destroy any remaining cancer cells. Treatment given after the surgery, to lower the risk of recurrence is called adjuvant therapy.

Palliative surgical procedures performed to relieve the symptoms caused by a blocked bile duct and improve your quality of life are:

Biliary bypass: A surgical procedure which involves connecting the part of the bile duct before the blockage to the part of the bile duct past the blockage or to the small intestine. This makes bile to flow easily to the gallbladder or small intestine.

Stent placement: This involves placing a stent (a thin, flexible tube or metal tube) in the bile duct so as to open it, and allow bile to flow into the small intestine or through a catheter that is connected to a collection bag outside the body.

Percutaneous transhepatic biliary drainage: A thin needle is inserted through the skin just below the ribs so that it reaches the liver. A special dye is injected into the liver or bile ducts and an X-ray is taken. If there is blockage in the bile duct, a thin, flexible tube called a stent is left in the liver to drain off bile into the small intestine.

Radiation therapy: This type of treatment uses high-energy X-rays or other types of radiation to destroy the cancer cells or prevent their growth. The two types of radiation therapy include:

   External radiation therapy: It uses a machine placed outside the body to send radiation towards the cancer. 

   Internal radiation therapy: It uses radioactive substances sealed in objects such as needles, seeds, wires, or catheters that are placed directly into or close to the cancer.

In unresectable, metastatic, or recurrent bile duct cancers, new methods that improve the effect of external radiation therapy on cancer cells are being studied.

    Hyperthermia therapy: In this therapy, the body tissue is exposed to high temperatures so that the cancer cells become more sensitive to the effects of radiation therapy and certain anticancer drugs.

    Radiosensitizers: These are drugs that make the cancer cells more sensitive to radiation. Combining radiation therapy with radiosensitizers may help to kill more cancer cells.

 Chemotherapy: Chemotherapy is a type of cancer treatment that makes use of drugs to stop the uncontrolled growth of cancer cells, either by killing these cells or by preventing their division. There are two routes to administer anticancer drugs. These include:

   Systemic chemotherapy: The drugs are either taken by mouth or injected into a vein or muscle. Once drugs enter the blood circulation, they can reach cancer cells all throughout the body. Therefore, it is used to treat unresectable, metastatic, or recurrent bile duct cancer. 

   Regional chemotherapy: The drugs are injected directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen. These drugs can affect the cancer cells in those areas.

6 Lifestyle and Coping

There are different ways to adapt your lifestyle in coping with bile duct cancer.

 Treatment in some people with bile duct cancer may completely remove or destroy the cancer. Completion of treatment may be both exciting as well as stressful. You may find it difficult to handle the fear of cancer coming back. This is a common concern in cancer survivors. It may take some time for your fears to disappear.

 In people with cancer that has not resolved completely, regular treatment with chemotherapy, radiation therapy, or other therapies is suggested to keep the cancer under control and help alleviate your symptoms. Coping with the uncertainty of cancer that does not go away may be hard and stressful.

Follow-up care

 Even if you have completed your cancer treatment, your doctors may want to keep you under close observation. It is very important to go to your follow-up appointments to doctor. During these visits, your doctors will examine you and may order laboratory tests, X-rays, and scans to check for signs of cancer or side effects of treatment.

7 Risks and Complications

There are many risk factors that may attribute to the development of bile duct cancer.

The following factors make a person more likely to develop bile duct cancer:

  • Diseases affecting the liver or bile ducts: People with chronic inflammation of the bile ducts are at a higher risk of developing bile duct cancer. Conditions that cause inflammation of bile ducts are primary sclerosing cholangitis, bile duct stones, choledochal cysts, cirrhosis, infection with hepatitis B virus or hepatitis C virus.
  • Inflammatory bowel disease: It includes ulcerative colitis and Crohn’s disease, and people with these diseases are at an increased risk of developing bile duct cancer. 
  • Older age: Older people in thier 60's and 70's are more likely to get bile duct cancer.
  • Ethnic background and geographic regions: In the US, the risk of bile duct cancer is higher among Hispanic Americans and Native Americans. In the rest of the world, bile duct cancer is more common in Southeast Asia and China, mainly due to the high rate of liver fluke infection in these areas.
  • Obesity: Being overweight or obese can increase your risk of developing cancer of the bile ducts. This is because obesity causes changes in certain hormones, and increases the risk of bile duct stones. 
  • Alcohol abuse: People who drink alcohol are at an increased risk of getting intrahepatic bile duct cancer. The risk is much higher if the person has liver problems.

Other possible risk factors include:

  • Smoking
  • Pancreatitis (inflammation of the pancreas)
  • HIV infection 
  • Exposure to asbestos, radon or other radioactive chemicals, dioxin, nitrosamines, or polychlorinated biphenyls (PCBs)

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