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Bile Duct Cancer

What is the bile duct?

The bile duct is long tube-like structure that connects the liver to the intestine and transports bile from the liver to the intestine. The top half of the bile duct is associated with the liver while the bottom half of the bile duct is associated the pancreas. The bile duct enters the part of the intestine called the duodenum into a structure called the Ampulla. Blockage of the bile duct causes build up of the bile in the blood since the bile can no longer go into the intestine. This condition is called jaundice and the skin becomes yellow from the accumulated bile in the blood.

What is bile duct cancer

Bile duct cancer or cholangiocarcinoma are tumors that occur in the bile duct. These are uncommon tumors and about 4,000 new cases are diagnosed in the United States each year. Bile duct cancer usually develops in patients older than 65 years old.

What symptoms are present in patients with bile duct cancer

The patient usually presents with the following symptoms

  • jaundice (yellowness of the skin)
  • abnormal liver tests
  • weight loss
  • abdominal pain
  • poor appetite
  • weakness and fatigue.

Types of bile duct tumors

Two major types of bile duct tumors are found

  • Distal bile duct tumors: tumors affecting the bottom half of the bile duct
  • Klatskin's tumors: Tumors affecting the upper part of the bile duct

This separation between the two locations is important since the treatment for the tumors in the two locations is different. For tumors affecting the bottom half of the bile duct, the cancer is removed with a Whipple operation. For tumors in the top half of the bile duct, surgical treatment often requires removal of the tumor together with a liver resection (removal) in an attempt to provide a surgical cure.

Distal bile duct tumors

These tumors arise in the bottom half of the bile duct and are often intimately associated with the pancreas since the bottom inch of the bile duct goes through the head of the pancreas as it enters into the duodenum.

Periampullary cancers

Patients with distal bile duct cancer presents with symptoms that are similar to that of pancreatic cancer and ampullary cancer. Distal bile duct tumors, pancreatic cancer and ampullary cancer are often called periampullary tumors because all three present with similar symptoms. It is important to recognize bile duct cancer and ampullary cancer since the outcome of the two cancers is better than that for pancreatic cancer when patients are compared stage for stage.

Diagnosis and staging of distal bile duct cancers

Once the diagnosis of bile duct cancer is made, the patient requires a work up for surgical removal of the tumor. The work up for distal bile duct cancer is similar to that for pancreatic cancer. This link will guide you to a detailed description of the work up at USC for bile duct cancer.

Treatment of distal bile duct cancer

Complete removal of the tumor is the only effective and potentially curative treatment for cancers of the lower bile duct. The treatment usually requires a Whipple operation. Distal bile duct cancer is more amenable to complete removal compared to pancreatic cancer. Five-year survival rates of up to 40% are found after complete removal of distal bile duct cancer.

Unresectable distal bile duct cancer

In patients with advanced distal bile duct cancer that is not surgically removable, the goal of treatment is palliation. The most important palliative measure is the relief of jaundice. We do not recommend surgical treatment to relieve the jaundice. A wall stent placed by a gastroenterologist provides adequate biliary drainage to relieve the obstruction and relieve jaundice. Surgically unresectable distal bile duct cancers do not usually respond very well to chemotherapy and radiation therapy therefore treatment options are limited.

Klatskin's tumors

Bile duct cancer of the upper part of the bile duct is also called Klatskin's tumor. Klatskin's tumors involve the upper part of the bile duct as divides to enter the right and the left parts of the liver. The bile ducts in the liver are called right and left hepatic ducts. The tumor may involve one or both right and left sides of the hepatic ducts as they enter the liver. The hepatic ducts are closely associated with the blood vessels that supply blood to the liver. Klatskin’s tumors are closely associated with liver and as they grow invasion into the blood vessels that supply blood to the liver is often found.

Diagnosis and staging of Klatskin's tumors

The patient usually presents with jaundice (yellowness of the skin) and/or abnormal liver tests. The diagnosis is usually made when the medical work up for the jaundice shows the presence of a tumor in the bile duct. The diagnosis is made by an ERCP or a PTC. These procedures also allow placement of stent to relieve the jaundice. A CT scan is then obtained to stage the tumor.Once diagnosis of a Klatskin’s tumor is made then the goal of the work up is to assess for possible removal of the tumor by surgery.

The liver is made up of two lobes: a right lobe and a left lobe. One of the two lobes can be safely removed at surgery. Klatskin’s tumors often invade the blood vessels called hepatic artery and the portal vein, that supply blood flow to the liver. The goal of staging prior to surgery is to assess whether the blood vessels of the liver are free of the tumor. The location of proximal bile duct tumors sometimes makes this evaluation difficult and often the final decision regarding surgery is made at the time of exploratory surgery.

Treatment of Klatskin's tumor

Complete removal of the tumor is the only effective and potentially curative treatment for cancers of the upper bile duct. The treatment usually requires a surgical procedure to remove the tumor in the bile duct together with one side of the liver due to the high frequency with which the tumor invades blood vessels of the liver.

Klatskin's tumors are removable if:

  • Blood supply to one side of the liver is not affected by the tumor: Klatskin’s tumors are closely associated with liver and as they grow invasion into the blood vessels that supply blood to the liver is often found. If the blood supply to one side of the liver is free of tumor then the portion of the liver invaded by the tumor can be removed.
  • The bile duct to one side of the liver is free of tumor: Klatskin's tumors affect the portion of the bile duct in the liver. One or both side of the bile ducts in the liver may be affected

Unresectable Klatskin's tumor

Klatskin's tumor is unresectable if it invades the blood supply to both sides of the liver and/or the hepatic duct to the both sides of the livertherapy treatments are used. In general these tumors respond poorly to treatment. At USC clinical protocols are available for advanced Klatskin’s tumors.

 

 

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