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Bile Duct Tumors - Types, Diagnosis and Treatment

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Bile duct tumor refers to the cancer of the bile duct, which passes through the pancreas. Read the article below to learn more about the disease.

Written by

Dr. P. Saranya

Medically reviewed by

Dr. Rajesh Gulati

Published At December 19, 2022
Reviewed AtJune 21, 2023

Introduction:

Bile duct tumor is a rare disease in which cancer cells form in the bile ducts. The liver, gallbladder, and small intestine are connected by tubes called ducts. The ducts carry bile fluid from the liver and gallbladder to the small intestine, where the bile digests fat. Bile duct cancer is also called cholangiocarcinoma. Most people who develop cholangiocarcinomas are over 65 years of age. Cholangiocarcinoma occurs more often in men than women.

What Are the Types of Bile Duct Tumors?

  • Intrahepatic Bile Duct Cancer: This cancer affects the bile ducts inside the liver. They are also called intrahepatic cholangiocarcinomas. They are not commonly found.

  • Extrahepatic Bile Duct Cancer: This cancer affects the bile ducts present outside the liver. The extrahepatic bile duct comprises the hilum and the distal region. Cancer can occur in any of the areas.

  • Perihilar Bile Duct Cancer: This cancer is found in the hilum region. This is where the right and left bile ducts exit from the liver and form the common hepatic duct. Perihilar type of cancer is also known as Klatskin tumor or perihilar cholangiocarcinoma.

  • Distal Bile Duct Cancer: This cancer is found in the distal region. The distal part contains the common bile duct, which passes through the pancreas and ends in the small intestine. It is also called extrahepatic cholangiocarcinoma.

Bile duct tumors are also subdivided based on the microscopic feature of cancer cells. These include sarcomas, lymphomas, small-cell cancers, and cholangiocarcinomas. Nearly all bile duct tumors are cholangiocarcinomas. Other types of cancer are less common. Cholangiocarcinoma is a type of adenocarcinoma in which cancer starts in the gland cells that line the inside of the ducts.

What Are the Factors Which Increase the Risk for Bile Duct Tumors?

The following factors can increase the risk of cholangiocarcinoma:

  • Primary Sclerosing Cholangitis: A condition where the bile ducts get blocked by inflammation and scarring.

  • Chronic Liver Disease: This causes scarring of the bile ducts.

  • Bile Duct Problems at Birth: The common congenital bile duct problems are listed below:

  • A choledochal cyst can result in irregularly dilated bile ducts.

  • Bile duct stones.

  • Liver fluke infection.

  • Cirrhosis.

  • Inflammatory bowel diseases such as ulcerative colitis and Crohn's disease.

  • Chronic ulcerative colitis.

  • Alcohol.

  • Diabetes.

  • Obesity.

  • Viral hepatitis infection.

What Are the Signs and Symptoms of Bile Duct Tumors?

Bile duct tumors usually do not cause any symptoms in their early stages.

Some of the symptoms which occur later include:

  • Jaundice (yellowish discoloration of the skin or whites of the eyes).

  • Abdominal pain.

  • Dark urine.

  • Clay-colored stool.

  • Fever.

  • Itchy skin.

  • Nausea and vomiting.

  • Weakness.

  • Weight loss.

  • Stomach upset.

What Are the Diagnostic Methods for Bile Duct Tumors?

  • Physical Examination: The doctor will ask about the general health, family history of cancer, liver disease, and habits such as smoking and alcohol. Physical examination to check masses, tenderness, or the presence of fluid is done.

  • Blood Tests: Used to find out the levels of bilirubin and to look for tumor markers such as CA 19-9 (cancer antigen 19-9), which is elevated in this tumor.

  • Abdominal Ultrasound: This imaging test is done to look for tumors.

  • Liver Function Test: To measure liver function.

  • Computed Tomography (CT) Scan: It produces detailed pictures of the inside of the body.

  • Magnetic Resonance Imaging (MRI): Uses a magnetic field to show internal organs. It can also determine the size and location of the tumor.

  • Endoscopy: An endoscope with a camera is inserted and is used to view the esophagus, stomach, and part of the intestine.

  • Endoscopic Retrograde Cholangiopancreatography (ERCP): A thin, flexible tube with a camera is passed down the throat into the small intestine. The camera is used to examine the bile ducts. A dye is also injected into the bile ducts to improve imaging results.

  • Magnetic Resonance Cholangiopancreatography (MRCP): This procedure uses an MRI machine to make images of the bile ducts. It is a non-invasive alternative to ERCP. This procedure does not need the use of a dye.

  • Percutaneous Transhepatic Cholangiography (PTC): A needle is inserted through the skin and into a bile duct in the liver. A dye is injected, and an X-ray of that area is taken.

  • Laparoscopy: Anesthesia is given, and the surgeon makes a small cut in the stomach through which a tube with a light and a video camera is inserted to look at the bile duct, gallbladder, liver, and other organs and tissues.

  • Biopsy: The doctor takes a sample tissue of bile duct cells for examination under a microscope. This is done during laparoscopy.

What Is the Treatment of Bile Duct Tumors?

  • Biliary Drainage: If the bile duct is blocked, the doctor performs a bypass by cutting the bile duct and reattaching the duct on the other side of the blockage. A stent is placed into the duct to keep bile flowing.

  • Photodynamic Therapy: An inactive form of a specific medication is injected into the vein, and the drug collects more cancer cells than healthy cells. After a few days, an endoscope is used to aim special light at the tumor, which inactivates the drug and kills the cancer cells.

  • Radiation: This therapy uses high-energy rays or particles to kill cancer cells. It can be used before or after the surgery. Before surgery, it is used to shrink the size of a tumor. After surgery, it is used to kill the remaining cancer cells.

  • Chemotherapy: This uses drugs to kill cancer cells and is used along with radiation therapy. Chemotherapy drugs are taken by mouth or through intravenous infusion.

  • Surgery: A part or the entire bile ducts are removed during surgery. Depending on cancer spread, lymph nodes, liver, pancreas, and small intestine are also removed.

  • Liver Transplant: It is a rare treatment that can sometimes cure bile duct cancer.

  • Targeted Therapy: This procedure uses drugs or other substances to find and kill cancer kills without harming normal cells.

  • Immunotherapy: Uses the patient's immune system to fight cancer.

What Is the Prognosis?

Cancers that develop outside the liver have a better prognosis than those which begin inside the liver. But the outcome is poor if cancer has spread to other body parts.

Conclusion:

Bile duct tumors are a rare disease affecting the bile ducts. Since the bile ducts are deep inside the body, small tumors are not seen or felt during a routine examination and do not show any symptoms in the early stage. Diagnosing and treating promptly can help to control the spread to other organs resulting in a good prognosis.

Frequently Asked Questions

1.

Who Is at Risk of Getting Bile Duct Tumors?

Bile duct tumors are associated with various risk factors, and individuals suffering from these conditions have a higher chance of developing bile duct tumors:
 
-Diseases of the liver.-Diseases of the bile duct.-Type 1 and type 2 diabetes.-Obesity.-Non-alcoholic fatty liver disease.-Advanced age.-Hereditary (passed down from parents to their children genetically).

2.

How Common Is Bile Duct Tumors?

 
Bile duct tumors are not a common condition. Bile duct tumors are diagnosed in about 8,000 patients in the US (United States) each year. Both extrahepatic (found outside the liver) and intrahepatic (found inside the liver) bile duct tumors fall under this category.

3.

What Are the Stages of Cholangiosarcoma?

The American Joint Committee on Carcinoma (AJCC) TNM classification is the primary method for describing the various stages of bile duct cancer. Bile duct cancers (cholangiocarcinoma) can be staged depending on where they first appear. 
 
-Intrahepatic Bile Duct Cancers: These are cancers originating in the liver.-Perihilar Bile Duct Cancers: These cancers originate in the hilum (a fissure that can be found on the liver's underside).-Distal Bile Duct Cancers: These cancers originate away from the bile duct system).

4.

How Does Surgery Treat Bile Duct Tumors?

Intrahepatic Bile Duct Cancer: In this surgery, the doctor removes the part of the liver affected by cancer, and this process is called partial hepatectomy. However, in some cases, a complete lobe of the liver (either right or left) is surgically removed. This procedure of removing one lobe is called a hepatic lobectomy.
Perihilar Bile Duct Cancer: These cancers demand difficult, highly skilled surgeries. The bile duct, gallbladder, lymph nodes surrounding, and occasionally a portion of the pancreas and small intestine are also removed along with a portion of the liver in most cases.
Distal Bile Duct Cancer: The surgeon frequently needs to remove a portion of the pancreas and small intestine in addition to the bile duct and adjacent lymph nodes.

5.

Are Bile Duct Tumors Always Cancerous?

 
A bile duct tumor can be cancerous (malignant) or non-cancerous (benign). However, all types of benign extrahepatic bile duct tumors are uncommon compared to malignant tumors.

6.

Where Does Bile Duct Tumors Spread First?

The lining of the abdomen, the lungs, and the bones are where bile duct cancer initially and most frequently spreads.

7.

How Aggressive Are Bile Duct Tumors?

 
Bile duct tumors are a rare type of cancer. These cancers can be aggressive and spread quickly to different body parts. As the early symptoms are vague, it is frequently detected too late. Only about 10 % of patients survive five years following diagnosis.

8.

Does Chemotherapy Work for Bile Duct Tumors?

 
While awaiting a liver transplant, chemotherapy may be used to manage bile duct tumors. Chemotherapy helps to shrink the tumor enough to increase the chances of successful surgical treatment. This is referred to as neoadjuvant therapy.

9.

Are Bile Duct Tumors Treatable?

 
Bile duct tumors are an aggressive type of tumor, and they can be difficult to treat. However, bile duct tumors can be treated with the help of chemotherapy and surgery. The prognosis depends on the size, location, and type of the tumor.

10.

What Happens in Final Stages of Bile Duct Tumors?

The symptoms of the final stages of bile duct tumors include:
 
-Jaundice.-Unusual weight loss.-Pain in the abdominal region.-Dark-colored urine.

11.

Can Bile Duct Tumors Spread to the Brain?

 
Bile duct tumors generally spread to the liver and nearby lymph nodes, and their spread to distant body parts is very rare. The spread of bile duct tumors to the brain is rare. However, It is entirely unknown how often bile duct tumors metastasize to the brain.

12.

Is Bile Duct Tumors the Same as Liver Cancer?

Not all types of bile duct tumors can be considered liver cancer. Intrahepatic bile duct tumors develop in parts of the bile ducts inside the liver and can be classified as a kind of liver cancer.

13.

Can Bile Duct Tumors Be Misdiagnosed?

Bile duct tumors are often misdiagnosed because the initial symptoms of the tumor are very non-specific, and the diagnosis is often delayed. Moreover, these tumors are often incidentally discovered during routine or other medical examinations.

14.

How Long Does Bile Duct Tumors Surgery Take?

 
Different types of surgeries are used to treat bile duct tumors (partial hepatectomy or lobectomy), and these surgical procedures take variable amounts of time to complete. On average, these procedures take around three to five hours.

15.

Can Alcohol Cause Bile Duct Tumors?

 
Excessive alcohol consumption affects an individual's overall health, including liver health. Alcohol not only increases the risk of bile duct tumors but also increases the risk of other liver conditions as well. 
Source Article IclonSourcesSource Article Arrow
Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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