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Bile Duct Cancer 

Introduction

Bile duct cancer, also called cholangiocarcinoma, is a rare form of cancer that occurs in the duct that carries bile from the liver to the small intestine.  Bile duct cancer is relatively slow growing.  Its main symptom is jaundice (yellowing of skin and eyes).  Surgery is the treatment of choice for bile duct cancer.  Radiation and chemotherapy is commonly used before surgery to reduce the size of a tumor or as a follow-up treatment after surgery.

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Anatomy

The bile duct begins as many small channels in your liver that meet to form the hepatic duct.  The hepatic duct is joined by the cystic duct from the gallbladder, and their union forms the common bile duct.  The common bile duct continues to the duodenum, the first part of your small intestine.

Your gallbladder works with your liver and pancreas to produce bile and digestive enzymes.  Bile is a fluid that breaks down fat in food for digestion.  Bile is produced in the liver and stored in the gallbladder until it is needed.  When you eat high-fat or high-cholesterol foods, your gallbladder sends bile to your duodenum via the common bile duct.

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Causes

Bile duct cancer is a rare form of cancer.  Most bile duct cancers are adenocarcinomas.  The majority of cases are slow growing and late to metastasize.  Cancer that has spread to other parts of the body is termed metastasized.  However, by the time most bile duct cancers are diagnosed, they are too advanced for surgical removal.
 
Researchers do not know the cause of most bile duct cancers.  Cancer occurs when cells grow abnormally and out of control, instead of dividing in an orderly manner.  Most bile duct cancer arises from the mucus glands that line the duct.  It appears that chronic irritation of the bile duct, by inflammation or parasitic infection, is the top risk factor associated with bile duct cancer formation. 

Cancer can develop in any part of the bile duct.  The cancer is classified based on its location.  The majority of bile duct cancer develops in the hepatic duct at the site where the small channels in the liver join together.  Cancers in this area are called perihilar cancers or Klatskin tumors.  Distal bile duct cancers form in the common bile duct near the small intestine.  A small percentage of bile duct cancers form in the channels within the liver and are called intrahepatic bile duct cancers.

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Symptoms

Symptoms occur when the bile ducts become blocked.  Jaundice is the most common symptom of bile duct cancer.  Jaundice is a condition caused by an excess of bilirubin.  Symptoms of jaundice include yellowing of the eyes and skin, dark urine and pale-colored stools.  You may also experience fever, nausea, vomiting, chills, and itching.  You may lose your appetite and lose weight.  You may feel pain in your right upper abdomen that may spread to your back.

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Diagnosis
Your doctor can begin to diagnose bile duct cancer through a series of tests, examinations, and by reviewing your medical history.  Your doctor will feel your abdomen for masses or enlarged organs.  Blood tests will evaluate your liver function and bilirubin.  Your blood may be tested for tumor markers, which some people produce in the presence of bile duct cancer. 

Imaging tests are used to identify the location and size of tumors and blockages. Common imaging tests include ultrasound, computed tomography (CT) scans, magnetic resonance imaging (MRI) scans, endoscopic retrograde cholangiopancreatography (ERCP), positron emission tomography (PET) scans, cholangiography, and angiography.  Ultrasound uses sound waves to produce images of internal organs and detect abnormal tissues.  An ultrasound device may be placed over the abdomen area, inserted through the mouth and into the stomach (endoscopic ultrasound), or through an incision in the side of the body (laparoscopic ultrasound).

CT scans take cross-sectional images of the body.  They may be used with a contrast agent or dye to take pictures of your bile duct and nearby organs.  CT scans are useful for determining if cancer has metastasized.  MRI scans produce even more detailed images and can outline the exact site of bile duct blockage. 

An ERCP uses an endoscope to view the biliary system.  An endoscope is a thin tube with a light and viewing instrument at the end of it.  After you are sedated, the thin tube is passed through your mouth and into your small intestine.  An endoscope is used to take tissue samples with biliary brushing.  It can administer dye to enhance views. 

A cholangiography can determine the exact location of bile duct cancer.  It is helpful for determining if the cancer can be treated with surgery.  For this procedure, contrast dye is injected into the bile duct before X-rays are taken. 
 
Angiography is used to show surgeons the location of blood vessels that are near the bile duct cancer for surgical planning.  Angiography involves inserting a small tube into a blood vessel to inject contrast dye near the suspected site before X-rays are taken.
 
A PET scan is an imaging test that uses a radioactive sugar substance.  A PET scan determines how quickly the cells metabolize the sugar.  Cancer cells and normal cells metabolize sugar at different rates.

A laparoscopy is a procedure used to view the bile duct, gallbladder, liver, and other internal organs.  It uses a thin-lighted instrument, a laparoscope, which is inserted through an incision in the abdomen.  A laparoscope can take a biopsy.  A biopsy is a tissue sample that is taken for evaluation of cancer cells.  A CT scan is used to guide needle biopsies.   

If you have bile duct cancer, your doctor will assign your cancer a classification stage based on the results of all of your tests.  Staging describes the cancer and how it has metastasized. Staging is helpful for treatment planning and recovery prediction. 
 
There is more than one type of staging system for cancer, and you should make sure that you and your doctor are referring to the same one.  Generally, lower numbers in a classification system indicate a less serious cancer, and higher numbers indicate a more serious cancer.  The stages may be subdivided into grades or classifications that use letters and numbers.

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Treatment

Surgery is the treatment of choice to remove bile duct cancer.  Chemotherapy or radiation may be used if all of the cancer cannot be removed with surgery.  The type of treatment that you receive depends on many factors, including the location and stage of your cancer.

Intrahepatic surgery is used for bile duct cancer that originates in the liver.  This procedure removes the part of the liver that contains cancer.  Surgery for perihilar cancer usually includes removing the bile duct, gallbladder, and part of the pancreas, small intestine, and liver.  The remaining bile ducts are connected to the small intestine.  Part of the pancreas and small intestine is usually removed during surgical treatment of distal bile duct cancer.  A Whipple procedure removes the bile ducts, part of the stomach, duodenum, pancreas, gallbladder, and lymph nodes.  In select cases, a complete liver transplantation may be necessary to treat bile duct cancer.  If all of the cancer cannot be removed, a bypass surgery is used to relieve symptoms of bile duct obstruction.  Bypass surgery creates a new route from the bile duct to the small intestine.

Chemotherapy, radiation therapy, or both may be used to reduce the size of a tumor before surgery or as a follow-up treatment after surgery.  Radiation therapy uses high-energy rays to eliminate cancer cells.  Chemotherapy uses cancer-fighting drugs to destroy cancer cells.  There are different types of chemotherapy and radiation therapy.

Even with treatment, some cases of bile duct cancer may return.  This is termed “recurrent bile duct cancer.”  The cancer may come back near the site of the original cancer or in other parts of the body.  Your doctor can explain your risk for recurrent bile duct cancer and possible treatments if it does recur.

The experience of bile duct cancer and cancer treatments can be an emotional process for people with cancer and their loved ones.  It is important that you receive support from a positive source.  Some people find comfort in their family, friends, counselors, co-workers, and faith.  Cancer support groups are another good option.  They can be a source of information and support from people who understand what you are experiencing.  Ask your doctor for cancer support group locations in your area.

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Prevention
You may prevent bile duct cancer by reducing the risk factors that you can control.  It may be helpful to avoid the hazardous chemicals associated with bile duct cancer.  You can prevent hepatitis B with a vaccine and hepatitis C by avoiding blood-borne or sexually transmitted infections.  Stopping alcohol abuse may prevent liver cirrhosis.  When travelling in Asia, it is important to avoid contact with liver flukes.

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Am I at Risk

Risk factors may increase your likelihood of developing bile duct cancer, although some people that experience this cancer may not have any risk factors.  People with all of the risk factors may never develop bile duct cancer; however, the likelihood increases with the more risk factors you have.  You should tell your doctor about your risk factors and discuss your concerns. 


Risk factors for bile duct cancer:


_____ Long-term inflammation of the bile duct is associated with an increased risk of developing bile cancer. 
_____ Sclerosing cholangitis is a type of bile duct inflammation that leads to scar tissue formation and is associated with an increased risk of bile duct cancer.
_____ Ulcerative colitis is an inflammation of the large intestine that can lead to sclerosing cholangitis.
_____ Smoking increases the risk of bile duct cancer for people with sclerosing cholangitis.
_____ Stones in the bile duct increase the risk for developing bile duct cancer.
_____ Diseases of the liver and bile duct increase the risk for bile duct cancer. Such conditions include polycystic liver disease, choledochal cysts, congenital dilation of the intrahepatic bile ducts, and cirrhosis.
_____ In Asian countries, parasites called liver flukes are a major cause of bile duct cancer. 
_____ Aging increases the risk for bile duct cancer.  Bile duct cancer occurs most frequently in people over the age of 65.
_____ Radioactive chemicals, including Thorotrast (thorium dioxide) which was used years ago during X-rays, are associated with an increased risk for bile duct cancer development.
_____ Certain chemicals may be associated with bile duct cancer formation.  These chemicals include dioxin, nitrosamines, and polychlorinated biphenyls (PCBs).
_____ Viral hepatitis B or C is linked to intrahepatic bile duct cancer.  The link is greater for hepatitis C.
_____ An association with both diabetes and HIV have been suggested but not proven.

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Advancements
Scientists are studying the genetic changes associated with bile duct cancer.  They hope to use such information to better prevent, diagnose, and treat the disease.  Researchers are studying immunotherapy to boost the immune system’s response to fight cancer.  Photodynamic therapy is being investigated as a treatment method that uses medication and special light rays to cause cancer cells to die. 

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This information is intended for educational and informational purposes only. It should not be used in place of an individual consultation or examination or replace the advice of your health care professional and should not be relied upon to determine diagnosis or course of treatment.

The iHealthSpot patient education library was written collaboratively by the iHealthSpot editorial team which includes Senior Medical Authors Dr. Mary Car-Blanchard, OTD/OTR/L and Valerie K. Clark, and the following editorial advisors: Steve Meadows, MD, Ernie F. Soto, DDS, Ronald J. Glatzer, MD, Jonathan Rosenberg, MD, Christopher M. Nolte, MD, David Applebaum, MD, Jonathan M. Tarrash, MD, and Paula Soto, RN/BSN. This content complies with the HONcode standard for trustworthy health information. The library commenced development on September 1, 2005 with the latest update/addition on April 13th, 2016. For information on iHealthSpot’s other services including medical website design, visit www.iHealthSpot.com.