·                                 Primary sclerosing cholangitis.

·                                 Chronic ulcerative colitis.

·                                 Choledochal cysts.

·                                 Infection with a Chinese liver fluke parasite.

Possible signs of extrahepatic bile duct cancer include jaundice and pain.

These and other symptoms may be caused by extrahepatic bile duct cancer or by other conditions. A doctor should be consulted if any of the following problems occur:

·                                 Jaundice (yellowing of the skin or whites of the eyes).

·                                 Pain in the abdomen.

·                                 Fever.

·                                 Itchy skin.

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For more information about each treatment option, select from the following list:

1.                                surgery

2.                                radiation therapy

3.                                chemotherapy

4.                                biological therapy

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Gallbladder cancer may result from a complication of gallstones. However, this inference rests on circumstantial evidence from postmortem examinations: 60% to 90% of gallbladder cancer patients also have gallstones, but postmortem data from patients with gallstones show gallbladder cancer in only 0.5%.

The predominant tissue type in gallbladder cancer is adenocarcinoma, 85% to 95%; squamous cell, 5% to 15%. Mixed-tissue types are rare.

Lymph node metastasis is present in 25% to 70% of patients at diagnosis. Direct extension to the liver is common (in 46% to 89%); direct extension to both the cystic and the common bile ducts, stomach, colon, duodenum, and jejunum also occurs and produces obstructions. Metastasis also spreads by portal or hepatic veins to the peritoneum, ovaries, and lower lung lobes.

The cause of extrahepatic bile duct cancer isn’t known; however, statistics report an unexplained increased incidence of this cancer in patients with ulcerative colitis. This association may be due to a common cause — perhaps an immune mechanism, or chronic use of certain drugs by the colitis patient.

Extrahepatic bile duct cancer is the cause of approximately 3% of all cancer deaths in the United States. It occurs in both males and females (incidence is slightly higher in males) between ages 60 and 70. The usual site is at the bifurcation in the common duct. Cancer at the distal end of the common duct is commonly confused with cancer of the pancreas. Characteristically, metastatic spread occurs to local lymph nodes, the liver, lungs, and the peritoneum.

Clinically, gallbladder cancer is almost indistinguishable from cholecystitis — pain in the epigastrium or right upper quadrant, weight loss, anorexia, nausea, vomiting, and jaundice. However, chronic, progressively severe pain in an afebrile patient suggests malignancy. In patients with simple gallstones, pain is sporadic. Another telling clue to malignancy is palpable gallbladder (right upper quadrant), with obstructive jaundice. Some patients may also have hepatosplenomegaly.

Progressive profound jaundice is commonly the first sign of obstruction due to extrahepatic bile duct cancer. The jaundice is usually accompanied by chronic pain in the epigastrium or the right upper quadrant, radiating to the back. Other common signs or symptoms, if associated with active cholecystitis, include pruritus, skin excoriations, anorexia, weight loss, chills, and fever.

No test or procedure, by itself, can diagnose gallbladder cancer. However, the following laboratory tests support the diagnosis when they suggest hepatic dysfunction and extrahepatic biliary obstruction:

❑baseline studies — complete blood count, routine urinalysis, electrolyte studies, enzymes

❑ liver function tests — typically reveal elevated serum bilirubin, urine bile and bilirubin, and urobilinogen levels in more than 50% of patients as well as consistently elevated serum alkaline phosphatase levels

❑ occult blood in stools — linked to the associated anemia

❑ cholecystography — may show calculi or calcification

❑ cholangiography — may locate the site of common duct obstruction

❑ magnetic resonance imaging — detects tumors.

The following tests help compile data that confirm extrahepatic bile duct cancer:

❑ liver function studies — indicate biliary obstruction: elevated levels of bilirubin [5 to 30 mg/dl], alkaline phosphatase, and blood cholesterol as well as prolonged prothrombin time

❑ endoscopic retrograde cannulization of the pancreas — identifies the tumor site and allows access for obtaining a biopsy specimen.

Surgical treatment of gallbladder cancer is essentially palliative and includes various procedures, such as cholecystectomy, common bile duct exploration, T-tube drain-age, and wedge excision of hepatic tissue.

If the cancer invades gallbladder musculature, the survival rate is less than 5%, even with massive resection. Although some cases of long-term survival (4 to 5 years) have been reported, few patients survive longer than 6 months after surgery for gallbladder cancer.

Surgery is normally indicated to relieve obstruction and jaundice that result from extrahepatic bile duct cancer. The procedure used to relieve obstruction depends on the cancer site. Such procedures may include cholecystoduodenostomy or T-tube drainage of the common duct.

Other palliative measures for both kinds of cancer include radiation, radiation implants (mostly used for local and incisional recurrences), and chemotherapy (with combinations of fluorouracil, irinotecan, and gemcitabine). All of these treatment measures have limited effects.

After biliary resection:

❑Monitor vital signs.

❑Use strict sterile technique when caring for the incision and the surrounding area.

❑Place the patient in low Fowler’s position.

❑Prevent respiratory problems by encouraging deep breathing and coughing. The high incision makes the patient want to take shallow breaths; using analgesics and splinting his abdomen with a pillow or an abdominal binder may aid in greater respiratory efforts.

❑Monitor bowel sounds and bowel movements. Observe the patient’s tolerance to diet.

❑Provide pain control.

ELDER TIP Check intake and output carefully. Watch for electrolyte imbalance; monitor I.V. solutions to avoid overloading the cardiovascular system, especially in older patients.

❑Monitor the nasogastric tube, which will be in place for 24 to 72 hours postoperatively to relieve distention, and the T tube. Record amount and color of drainage each shift. Secure the T tube to minimize tension on it and prevent its being pulled out.

❑Help the patient and his family cope with their initial fears and reactions to the diagnosis by offering information and support.

❑Before discharge, teach the patient how to manage the biliary catheter.

❑Advise the patient of the adverse effects of both chemotherapy and radiation therapy, and monitor him for these effects.

❑When all treatments have failed, concentrate on keeping the patient comfortable and free from pain and provide as much psychological support as possible. If the patient is going home, discuss continuing care needs with the caregiver or refer the patient to an appropriate home health care or hospice agency. Encourage the patient and caregiver to express their feelings and concerns. 

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Nov. 1, 1999 — The American football legend Walter Payton died today at his home near Chicago at age 45. Mr. Payton was the all-time leading rusher in the history of the National Football League.

It was well known that he had a progressive liver disease called primary sclerosing cholangitis. However, the cause of Mr. Payton’s death was bile duct cancer, said Dr. Greg Gores, his physician at the Mayo Clinic in Rochester, Minnesota.

During a news conference in Chicago on Feb. 2, Mr. Payton had revealed that he had primary sclerosing cholangitis. He said doctors had told him he would need a liver transplant within two years. Only a week later, this time table was revised and he was told that he needed a transplant by the end of 1999.

Dr. Gores indicated that Mr. Payton had received chemotherapy and radiation treatment after the bile duct cancer was diagnosed but that the cancer had progressed to where “transplantation was no longer a viable option.”

The purpose of this article is to provide perspectives on both of Mr. Payton’s illnesses: bile duct cancer and primary sclerosing cholangitis.

Bile Duct Cancer

Bile duct cancer is a rare disease in which there are malignant cells in the tissues of the extrahepatic bile duct. The bile duct is a tube that connects the liver and the gallbladder to the small intestine. The part of the bile duct that is outside the liver is called the extrahepatic bile duct.

A fluid called bile, which is made by the liver and breaks down fats during digestion, is stored in the gallbladder. When food is being broken down in the intestines, bile is released from the gallbladder through the bile duct to the first part of the small intestine.

The symptoms of bile duct cancer include yellowing of the skin (jaundice), pain in the abdomen, fever, and itching.

If there are symptoms, tests are usually ordered to see if there is cancer. These may include an ultrasound (a test that uses sound waves to find tumors), a CT (a computed tomographic) scan, a special type of x-ray that uses a computer to make a picture of the inside of the abdomen) and an MRI (magnetic resonance imaging which uses magnetic waves to make a picture of the inside of the abdomen).

A test called an ERCP (endoscopic retrograde cholangiopancreatography) may be done. A flexible tube is put down the throat, through the stomach, and into the small intestine. The doctor can see through the tube and inject dye into the drainage tube (duct) of the pancreas so that the area can be seen more clearly on an x-ray.

PTC (percutaneous transhepatic cholangiography) is another test that can help find cancer of the extrahepatic bile duct. During this test, a thin needle is put into the liver through the right side of the patient. Dye is injected through the needle into the bile duct in the liver so that blockages can be seen on x-rays.

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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. 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.

Having colitis or certain liver diseases can increase the risk of developing extrahepatic bile duct cancer.

Anything that increases your risk of getting a disease is called a risk factor. Risk factors include having any of the following disorders:

                               Primary sclerosing cholangitis.

                               Chroniculcerative colitis.

                               Choledochal cysts.

                               Infection with a Chinese liver fluke parasite.

Possible signs of extrahepatic bile duct cancer include jaundice and pain.

These and other symptoms may be caused by extrahepatic bile duct cancer or by other conditions. A doctor should be consulted if any of the following problems occur:

                               Jaundice (yellowing of the skin or whites of the eyes).

                               Pain in the abdomen.

                               Fever.

                               Itchy skin.

Tests that examine the bile duct and liver are used to detect (find) and diagnose extrahepatic bile duct cancer.

The following tests and procedures may be used:

                               Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

                               Ultrasound: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

                               CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A spiral or helical CT scan makes detailed pictures of areas inside the body using an x-ray machine that scans the body in a spiral path.

                               MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

                               ERCP (endoscopic retrograde cholangiopancreatography): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes bile duct cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth, esophagus, and stomach into the first part of the small intestine. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs of cancer.

                               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 an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body.

                               Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The sample may be taken using a fine needle inserted into the duct during an x-ray or ultrasound. This is called needle biopsy or fine-needle aspiration biopsy. The biopsy is usually done during PTC or ERCP. Tissue may also be removed during surgery.

                               Liver function tests: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign of liver disease that may be caused by extrahepatic bile duct cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

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

                               The stage of the cancer (whether it affects only the bile duct or has spread to other places in the body).

                               Whether the tumor can be completely removed by surgery.

                               Whether the tumor is in the upper or lower part of the duct.

                               Whether the cancer has just been diagnosed or has recurred (come back).

Treatment options may also depend on the symptoms caused by the tumor. Extrahepatic bile duct cancer is usually found after it has spread and can rarely be removed completely by surgery. Palliative therapy may relieve symptoms and improve the patient’s quality of life.

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1. Surgery (taking out the cancer or taking steps to relieve symptoms caused by the cancer)
2. Radiation therapy (using high-dose x-rays to kill cancer cells)
3. Other treatments for extrahepatic bile duct cancer are being studied in clinical trials. These include:
4. Chemotherapy (using drugs to kill cancer cells)
5. Biological therapy (using the body’s immune system to fight cancer)

Surgery is a common treatment of extrahepatic bile duct cancer. If the cancer is small and is only in the bile duct, a doctor may remove the whole bile duct and make a new duct by connecting the duct openings in the liver to the intestine. The doctor will also remove lymph nodes and look at them under the microscope to see if they contain cancer. If the cancer has spread outside the bile duct, a surgeon may remove the bile duct and the tissues around it.

If the cancer has spread and it cannot be removed, the doctor may do surgery to relieve symptoms. If the cancer is blocking the small intestine and bile builds up in the gallbladder, the doctor may do surgery to go around (bypass) all or part of the small intestine. During this operation, the doctor will cut the gallbladder or bile duct and sew it to the small intestine. This is called biliary bypass. Surgery or x-ray procedures may also be done to put in a tube (catheter) to drain bile that has built up in the area. During these procedures, the doctor may make the catheter drain through a tube to the outside of the body or the catheter may go around the blocked area and drain the bile to the small intestine. In addition, if the cancer is blocking the flow of food from the stomach, the stomach may be sewn directly to the small intestine so the patient can continue to eat normally.

Radiation therapy is the use of high-energy x-rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes into the area where the cancer cells are found (internal radiation therapy).

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by inserting a needle into a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells outside the bile duct.

Biological therapy tries to get the body to fight cancer. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body’s natural defenses against disease. Biological therapy is sometimes called biological response modifier (BRM) therapy or immunotherapy. This treatment is currently only being given in clinical trials.

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There are different types of treatment for patients with extrahepatic bile duct cancer.

Different types of treatment are available for patients with extrahepatic bile duct cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. Before starting treatment, patients may want to think about taking part in a clinical trial. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

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The part of the bile duct that is outside the liver is called the extrahepatic bile duct. A fluid called bile, which is made by the liver and stored in the gallbladder, breaks down fats during digestion. When food is being broken down in the intestines, bile is released from the gallbladder through the bile duct into the first part of the small intestine.

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