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. |
