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Cholangiocarcinoma (Bile Duct Cancer) (6702)

Cholangiocarcinoma (Bile Duct Cancer) (6702) - Clinical Hub, Patient Education, Health and Nutrition Facts For You, Cancer, BMT, Hematology


Cholangiocarcinoma (Bile Duct Cancer)

The Bile Duct System (Biliary Tract)

A network of bile ducts (tubes) connects the liver and the gallbladder to the small intestine.
This network begins in the liver where many small ducts collect bile, a fluid made by the liver to
break down fats during digestion. The small ducts come together to form the right and left
hepatic bile ducts, which lead out of the liver. The two ducts join outside the liver to become the
common hepatic duct. Ducts within the liver are called intrahepatic ducts. The part of the
common hepatic duct that is outside the liver is called the extrahepatic bile duct. The
extrahepatic bile duct is joined by a duct from the gallbladder (which stores bile) to form the
common bile duct. Bile is released from the gallbladder through the common bile duct into the
small intestine when food is being digested.


Bile Duct Cancer (Cholangiocarcinoma)

Bile duct cancer, a rare cancer in the United
States, starts in the lining of the bile ducts
(epithelial cells). Bile duct cancers can start
in the bile ducts within the liver. These are
called intrahepatic cholangiocarcinomas.
Or they can start in the bile ducts outside of
the liver. These are called extrahepatic

Risk Factors

The following disorders are risk factors for
developing bile duct cancer:

ξ Primary sclerosing cholangitis
ξ Chronic ulcerative colitis
ξ Choledochal (common bile duct)
ξ Infection with a Chinese liver
fluke parasite


Bile duct cancers usually cause symptoms
when the cancer blocks (obstructs) the
biliary drainage system causing yellow skin
or whites of the eyes (jaundice). Itching
(pruritis) often occurs due to the deposits of
bile salts in the skin. Other symptoms
include pain, often in the right upper
abdomen, fever, clay-colored stools, and
dark urine.


The prognosis (chance of recovery) and
treatment options depend on:

ξ The stage of the cancer (whether
it affects only the bile duct or has
spread to other places in the
ξ Whether the cancer can be
completely removed by surgery.
ξ Whether the cancer is in the
upper or lower part of the duct.
ξ Whether the cancer is newly
diagnosed or has recurred (come

Treatment options may also depend on the
symptoms caused by the cancer. Bile duct
cancer found after it has spread may not be
completely removed by surgery
(unresectable). If the cancer has spread,
palliative treatment may improve the
patient’s quality of life by controlling
symptoms and complications of the disease.

Diagnosis and Staging

These tests and procedures may be used to
diagnose bile duct cancers and determine the
stage of disease (extent of the cancer). The
stage of the disease is important to know in
order to make a treatment plan.

ξ Physical exam and complete
history of health habits, past
illnesses, and treatments.
ξ Ultrasound – a radiology
procedure that bounces high-
energy sound waves (ultrasound)
off tissues or organs to form a
picture called a sonogram
ξ CT scan (CAT scan) – detailed
picture of the inside of the body
taken by a special x-ray machine
that is attached to a computer
ξ MRI (magnetic resonance
imaging) – a radiology procedure
that uses a magnet, radio waves,
and a computer to make detailed
pictures of the inside of the body


ξ ERCP (endoscopic retrograde
cholangiopancreatography) – a
procedure performed by a
gastroenterologist (a doctor who
specializes in digestive tract
diseases) where a small lighted
tube (scope) is passed through
the mouth, esophagus, stomach,
and first part of the small
intestine. A smaller tube or
catheter is passed into the ducts,
a dye is injected and x-rays
taken. If the ducts are blocked, a
small flexible tube (stent) may be
inserted into the duct to unblock
it. Tissue samples (biopsies)
may be taken.
ξ PTC (percutaneous transhepatic
cholangiography) – a procedure
used to x-ray the liver and bile
ducts. A thin needle is inserted
through the skin below the ribs
and into the liver. Dye is
injected into the liver or bile
ducts and x-rays are taken. If a
blockage is found, a flexible tube
or stent is sometimes left in the
liver to drain bile into either the
small intestine or to a collection
bag outside the body. Tissue
samples or biopsies may also be
ξ Biopsy – the removal of cells or
tissues to be examined under the
microscope to check for cancer.
Tissue can be removed during an
ERCP, a PTC, or surgery.
ξ Liver function tests – blood
tests that measure the amounts of
certain substances released into
the blood by the liver. Higher
than normal amounts can be a
sign of liver disease that may be
caused by bile duct cancer.

ξ Laparoscopy – surgery to look
at the organs inside the abdomen
to check for signs of disease. A
small lighted tube (scope) is
inserted through a small incision
in the abdomen. Tissue samples
(biopsies) may be taken through
the scope. A laparoscopy helps
determine if the cancer can be
removed surgically or if it has
spread to other organs in the

Stages of Bile Duct Cancer

Stage 0 (Carcinoma in situ) –
cancer is found only in the innermost
layer of cells lining the bile duct.

Stage I is divided into stage IA and
stage IB.
ξ Stage IA – cancer is found in
the bile duct only
ξ Stage IB – cancer has spread
through the wall of the bile

Stage II is divided into stage IIA and
stage IIB.
ξ Stage IIA – cancer has
spread to the liver,
gallbladder, pancreas, and/or
to either the right or left
branches of the hepatic artery
or to the right or left branches
of the portal vein


ξ Stage IIB – cancer has
spread to nearby lymph nodes
o is found in the bile
duct; or
o has spread through
the wall of the bile
duct; or
o has spread to the
liver, gallbladder,
pancreas, and/or the
right or left branches
of the hepatic artery
or portal vein

Stage III – cancer has spread:
ξ to the portal vein or to both
right and left branches of the
portal vein; or
ξ to the hepatic artery; or
ξ to other nearby organs or
tissues, such as the colon,
stomach, small intestine, or
abdominal wall; or
ξ perhaps to nearby lymph

Stage IV – cancer has spread to
lymph nodes and/or organs far away
from the bile duct.

Treatment Groups

ξ Localized (and resectable) – the
cancer is in a limited area and can be
completely removed by surgery.
ξ Unresectable – the cancer has
spread to nearby lymph nodes, blood
vessels or other organs and cannot be
removed completely by surgery.

Methods of Treatment


These types of surgery are used to treat bile
duct cancer.

ξ Removal of the bile duct – If-the
tumor is small and only in the bile
duct, the entire bile duct may be
removed. A new duct is made by
connecting the duct openings in the
liver to the intestine. Lymph nodes
are removed and examined under the
microscope to see if they contain
ξ Partial hepatectomy – Removal of
the part of the liver where cancer is
found. The part removed may be a
wedge of tissue, an entire lobe, or a
larger part of the liver, along with
some normal tissue (margin) around
ξ Whipple procedure - A surgery in
which the head of the pancreas, the
gallbladder, part of the stomach, part
of the small intestine, and the bile
duct are removed. Enough of the
pancreas is left to produce digestive
juices and insulin.
ξ Surgical biliary bypass – If the
tumor cannot be removed but is
blocking the small intestine and
causing bile to build up in the
gallbladder, a biliary bypass may be
done. During this operation, the
gallbladder or bile duct will be cut
and sewn to the small intestine to
create a new pathway around the
blocked area. This procedure helps
to relieve jaundice caused by the
build-up of bile.


ξ Stent placement – If the tumor is
blocking the bile duct, a stent (thin
tube) may be placed in the duct to
drain bile that has built up in the
area. The stent may drain to the
outside of the body or it may go
around the blocked area and drain
the bile into the small intestine. A
stent may be place during an ERCP,
a PTC, or surgery.

Radiation Therapy

Radiation therapy is a cancer treatment that
uses high-energy x-rays or other types of
radiation to kill cancer cells. There are two
types of radiation therapy. External beam
radiation uses a machine outside the body to
send radiation toward the cancer. Internal
beam radiation uses a radioactive substance
sealed in needles, seeds, wires, or catheters
that are placed directly into or near the
cancer. The way the radiation therapy is
given depends on the type and stage of the
cancer being treated.


Chemotherapy uses drugs to stop the growth
of cancer cells, either by killing the cells or
by stopping the cells from dividing. Unlike
surgery and radiation therapy, chemotherapy
is a systemic treatment that can reach cancer
cells throughout the body. Chemotherapy is
sometimes used along with radiation therapy
to make the radiation therapy more

Clinical Trials

Clinical trials, exploring ways of improving
local control may be available using
chemotherapy with or without radiation.

This information has been reproduced with
permission of the National Cancer Institute.
For more information please visit their
website at HUwww.nci.nih.gov U

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