Cholangiocarcinoma (patient information)

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Editor-in-Chief: C. Michael Gibson, M.S.,M.D. [mailto:mgibson@perfuse.org] Phone:617-632-7753; Associate Editor-In-Chief: Jinhui Wu, M.D.

Overview
The bile duct is a thin tube which reaches from the liver to the small intestine. Its function is to transport bile from the liver and gallbladder to the small intestine to help digest the fats in foods. Cholangiocarcinoma is a cancerous growth in one of the ducts. Cholangiocarcinoma doesn't have any early symptoms. With the development of the cancer, frequent symptoms include jaundice, itching, abdominal pain and ther symptoms due to blockage of the bile duct. Treatments include surgery, radiation, chemotherapy or a combination of them.

What are the symptoms of Cholangiocarcinoma?
Early cholangiocarcinoma does not have any symptoms. As the tumor grows larger, people may notice one or more of the following symptoms:


 * Jaundice


 * Itching


 * Abdominal pain


 * Loss of appetite/weight loss


 * Fever


 * Light colored stools/dark urine


 * Nausea or vomiting

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

What causes Cholangiocarcinoma?
Cancerous tumors of the bile ducts are usually slow-growing and do not spread (metastasize) quickly. However, many of these tumors are already advanced by the time they are found.

A cholangiocarcinoma may start anywhere along the bile ducts. These tumors block off the bile ducts.

They affect both men and women. Most patients are older than 65.

Risks for this condition include:


 * Bile duct (choledochal) cysts
 * Chronic biliary irritation
 * History of infection with the parasitic worm, liver flukes
 * Primary sclerosing cholangitis
 * Ulcerative colitis

Cholangiocarcinoma is rare. It occurs in approximately 2 out of 100,000 people.

Who is at highest risk?
Clinical data has suggested that the development of cholangiocarcinoma is related to several factors.


 * Chronic inflammation of the bile duct: Clinical data suggest patients who have chronic inflammation of the bile duct such as primary sclerosing cholangitis, ulcerative colitis, bile duct stones, choledochal cysts and cirrhosis have high risk of developing cholangiocarcinoma.


 * Liver fluke infections: Liver fluke infection is a major cause of cholangiocarcinoma in some Asian countries.


 * Exposure to thorotrast: Thorotrast, widely used as a contrast agent for x-rays until 1950s, was found to lead to hepatic cancer and cholangiocarcinoma.


 * Obesity: Epidemiological survey show being overweight or obese can increase the risk of developing cancers of cholangiocarcinoma.


 * Age: Clinical data show old people are more likely to developing cholangiocarcinoma.

When to seek urgent medical care?
Call your health care provider if symptoms of cholangiocarcinoma develop. If you experience either of the following symptoms, seeking urgent medical care as soon as possible:
 * Severe abdominal pain
 * Hyperpyrexia

Diagnosis

 * Tests of liver and gallbladder function: This is a blood test ordered to detect whether your liver or gallbladder is abnormal or not.


 * Tumor markers: High blood levels of CEA and CA-199 suggest that there is cancer. But attention false positive and false negative. Patients are suggested more tests, especially image tests.


 * Ultrasound: Ultrasound may be the first imaging test done in people who have symptoms. The mechanism of echoes is that echos produced by tumors issue are different from those of normal tissue. During the procedure, you only need to lie on a bed while the doctor places the transducer on the skin over the part of your body.


 * Endoscopic retrograde cholangiopancreatography (ERCP) and biopsy: During this procedure, a thin, lighted and flexible tube is passed down your throat, through the esophagus and stomach, then into the first part of the small intestine. A small amount of contrast material is then injected through the tube into the common bile duct and x-rays are taken. The x-ray images can show narrowing or blockage of these ducts that might be due to cholangiocarcinoma. At the same time the doctor doing this test can also put a small brush through the tube to remove cells for a biopsy.


 * Magnetic resonance cholangiopancreatography (MRCP): This is a less invasive way to image the bile ducts using magnetic fields.


 * Computed tomography (CT) scan and biopsy: CT scans are often used to diagnose cholangiocarcinoma. It can confirm the location of the cancer and show the organs near the bile ducts, as well as lymph nodes and distant organs where the cancer might have spread. These are helpful for determining the stage of the cancer and in determining whether surgery is a good treatment option. CT scans can also be used to guide biopsy and a biopsy sample is usually removed and looked at under a microscope.
 * Magnetic resonance imaging (MRI): An MRI uses magnetic fields but it is a different type of image than what is produced by computed tomography (CT) and produces detailed images of the body. Like computed tomography (CT), a contrast agent may be injected into a patient’s vein to create a better picture.
 * Positron emission tomography (PET) scan: When doing this test, a small amount of a radioactive medium is injected into your body and absorbed by the organs or tissues. This radioactive substance gives off energy which in turn is used to produce the images. PET can provide more helpful information than either CT or MRI scans. It is useful to see if the cancer has spread to the lymph nodes and it is also useful for your doctor to locate where the cancer has spread.

Treatment options
Patients with cholangiocarcinoma have many treatment options. The selection depends on the stage of the tumor. The options are surgery, radiation therapy, chemotherapy, or a combination of these methods. Before treatment starts, ask your health care team about possible side effects and how treatment may change your normal activities. Because cancer treatments often damage healthy cells and tissues, side effects are common. Side effects may not be the same for each person, and they may change from one treatment session to the next.


 * Surgery: Surgery for cholangiocarcinoma is very complex. It includes potentially curative surgery and palliative surgery.


 * Radiation therapy: This therapy does not usually work well for kidney cancer. In some cases, hormone treatments may reduce the growth of the tumor. It includes external beam radiation therapy (EBRT), three-dimensional conformal radiation therapy (3D-CRT) and brachytherapy (internal radiation therapy).


 * Chemotherapy: The treatment is to use drugs to stop the growth of cancer cells either by killing the cells or by stopping them from dividing. Usual used drugs are 5-fluorouracil (5-FU), gemcitabine and mitomycin C.

Diseases with similar symptoms
Other health problems may also cause similar symptoms. Go to see your doctor to verify your diseases as early as possible. Diseases with similar symptoms are listed in the following:


 * Gallbladder cancer


 * Hepatocellular Carcinoma


 * Pancreatic cancer


 * Cholecystitis and choledochitis


 * Liver fluke infections

Where to find medical care for Cholangiocarcinoma?
Directions to Hospitals Treating cholangiocarcinoma

Prevention of Cholangiocarcinoma
Epidemiology data show the following intervention may help to reduce your risk of cholangiocarcinoma:


 * Getting vaccinated against the hepatitis B


 * Taking precautions to avoid blood-borne or sexually transmitted infections by other viruses


 * Keeping a healthy lifestyle: Quitting smoking and alcohol, controlling weight.


 * Avoiding exposure to the certain chemicals such as Thorotrast

Possible complications

 * Infection
 * Liver failure
 * Spread (metastasis) of tumor to other organs

What to expect (Outlook/Prognosis)?
The prognosis of cholangiocarcinoma is not good and it depends on the following:
 * The location of tumor in bile duct
 * Whether or not the tumor can be removed by surgery
 * The stage of the cancer
 * The patient’s general health
 * Whether the cancer has just been diagnosed or has recurred