Disease: Bile Duct Cancer
(Cholangiocarcinoma)

    What is bile duct cancer (cholangiocarcinoma)? What are causes and risk factors for bile duct cancer?

    Bile duct cancer is a type of cancer that arises from the cells that line the bile ducts, the drainage system for bile of the liver. Bile ducts collect the bile from the liver and allow it to drain into the small intestine. The bile within the intestine helps with food digestion. Bile duct cancer is also called cholangiocarcinoma.

    Bile duct cancer is a rare form of cancer, with less than 3,000 new cases diagnosed in the United States each year. There are three general locations where this type of cancer arises within the bile drainage system:

    • Within the liver (intrahepatic): Affecting the bile ducts located within the liver
    • Just outside of the liver (extrahepatic): also called perihilar, located at the notch of the liver where the bile ducts exit
    • Far outside of the liver (distal extrahepatic): near where the bile ducts enter the intestine (called the ampulla of Vater)

    Bile duct cancers are most commonly found just outside of the liver in the perihilar area and least commonly within the liver.

    The incidence of bile duct cancer increases with age. It is a slow-growing cancer that invades local structures and for that reason, the diagnosis is often made late in the disease process when the bile ducts become blocked. This prevents bile drainage from the liver into the intestine. Depending upon where the blockage occurs, this can lead to inflammation of the liver (hepatitis) and/or pancreas (pancreatitis).

    Most patients who develop bile duct cancer have no risk to do so. However, chronic inflammation of the bile ducts may be a risk factor for this cancer. Diseases that can cause this type of chronic inflammation include primary sclerosing cholangitis and chronic parasite infections. Patients with chronic liver disease, including hepatitis B, hepatitis C, chronic alcoholic hepatitis, and cirrhosis may also be at risk for developing bile duct cancer.

    Gallstones are not a risk factor for developing bile duct cancer, but stones within the liver pose an increased risk. Liver stones are not often seen in the North American population but are more common in Asian countries.

    There are rare congenital diseases that increase the risk of bile duct cancer, including Lynch II syndrome (hereditary nonpolyposis colorectal cancer associated with biliary tree and other cancers) and Caroli's syndrome (portal hypertension, hepatic fibrosis, and biliary tree cysts).

    Native Americans are six times more likely to develop bile duct cancer. Asian Americans may also be at higher risk.

    What are bile duct cancer symptoms and signs?

    The initial symptoms of bile duct cancer occur because of inability for bile to drain, causing liver inflammation (hepatitis). Symptoms include yellow coloring of the skin (jaundice), itching, abdominal pain, bloating, and weight loss. Low-grade fever can be present, and there can be darkening in the color of urine and stool.

    Unfortunately, bile duct tumors may not cause any symptoms until they have grown in size and the cancer has spread (metastasized) from beyond its original location. Abdominal pain is often a late symptom and is usually located in the right upper quadrant of the belly and may be associated with a tender, enlarged liver.

    How is bile duct cancer diagnosed?

    History and physical examination are key clues for the diagnosis of bile duct cancer. Painless jaundice (yellow/orange coloring of the skin) may be the only initial clue. The history often includes reviewing alcohol use, drug use or recent illnesses that can be associated with hepatitis, or inflammation of the liver. Other symptoms may be weight loss, loss of appetite, weakness, loss of energy, and easy bruising or bleeding.

    The physical examination may be useful in detecting tenderness in the abdomen, especially in the right upper quadrant beneath the ribs (where the liver is located). A quarter of patients with bile duct cancer will have an enlarged liver that can be palpated or felt on exam. On general exam, the patient is often jaundiced, having yellow-tinged skin. This may be seen most easily in the white portion (sclerae) of the eyes or under the tongue.

    Blood tests are often ordered to assess liver function. Liver enzymes (AST, ALT, GGT), bilirubin levels, complete blood count, electrolytes, BUN and creatinine, and INR/PTT (international normalized ratio/partial thromboplastin time) and PT (prothrombin time).

    There is no blood test that can specifically diagnose bile duct cancer. The diagnosis is confirmed by tissue sample obtained by biopsy and pathology examination of the cells obtained by that biopsy sample.

    Imaging may be used including ultrasound, CT scan, and MRI to look for a tumor and its location

    ERCP (endoscopic retrograde cholangiopancreatography) is a specialized test used to examine the bile duct as it enters the duodenum. ERCP is performed by a gastroenterologist using a fiberoptic camera at the end of a flexible viewing tube. The tube is passed through the mouth and passed through the stomach into the first part of the small intestine where the common bile duct enters from the gallbladder. This is used to detect diseases that affect the bile ducts including bile duct cancer, gallstones stuck in the bile duct, and abnormal narrowing of the bile duct. Dye can be injected to outline the bile ducts and detect obstruction. Biopsies or cell washings can be obtained to look for cancer cells. If a blockage is found, the gastroenterologist may be able to place a stent during the same procedure.

    Sometimes, an interventional radiologist may be needed to obtain a tissue biopsy by threading a needle through the skin into the liver.

    Once the diagnosis of bile duct cancer is made, it is important to stage the cancer to help direct potential treatment. The three parts of TNM staging include the following:

    • T is for the primary tumor and how much it has grown locally and invaded other structures. For a bile duct tumor, this includes the liver, gallbladder, pancreas, stomach, and intestine.
    • N is for the lymph nodes that are involved. The more nodes involved and the farther the distance from the bile duct, the more severe the cancer.
    • M is for metastasis. Has the tumor spread to other parts of the body?

    Cancer can be staged from 0 to 4, where 0 is no tumor, 1 is local tumor with no spread to lymph nodes or other parts of the body, and 4 is significant local growth and lymph node involvement and spread to other parts of the body.

    While staging is important, as well as detecting tumor spread beyond the liver and bile duct, often the critical staging questions can only be answered at surgery. Then it an assessment can be made as to whether or not the whole tumor be resected or removed. Survival rates are markedly improved if complete resection is possible.

    What is the treatment for bile duct cancer?

    Treatment for bile duct cancer depends upon where the cancer is located and whether it is possible to be completely removed by surgery. Unfortunately, those afflicted with this cancer tend to be older and may be unable to tolerate and recover from a significant operation. The decision regarding surgery needs to be individualized for the specific patient and their situation.

    Other treatment options tend to be palliative, not curative, and are meant to preserve quality of life. Chemotherapy and radiation therapy have their place. Photodynamic therapy is another alternative to help shrink the tumor and control symptoms.

    ERCP may be used to stent the bile duct, keeping it open to allow bile drainage from the liver and gallbladder into the intestine. This is often very helpful in controlling symptoms but does not treat the tumor itself.

    As with all cancers, the treatment is individualized for the patient. Discussion between the patient, health-care professional, and family are important to help understand treatment options, including cure versus palliation or symptom control and quality of life. The patient's wishes are key.

    What is the prognosis for bile duct cancer? What is the life expectancy for bile duct cancer?

    How well a patient does after the diagnosis of bile duct cancer depends upon many factors, including where the tumor is located, if and how much it has spread, and the patient's underlying general health. Typically, bile duct cancers have a poor prognosis and the five-year survival rate for a cancer that can be completely resected is less than 50%.

    National Cancer Institute statistics list the following 5-year survival rates:

    Intrahepatic (Within the Liver) Bile Duct CancerStage5-Year Relative SurvivalLocalized (Stage 1)15%Regional spread (Stage 2, 3)6%Distant spread (Stage 4)2%
    Extrahepatic (Outside the Liver) Bile Duct CancerStage5-Year Relative SurvivalLocalized (Stage 1)30%Regional spread (Stage 2, 3)24%Distant spread (Stage 4)2%

    What are bile duct cancer symptoms and signs?

    The initial symptoms of bile duct cancer occur because of inability for bile to drain, causing liver inflammation (hepatitis). Symptoms include yellow coloring of the skin (jaundice), itching, abdominal pain, bloating, and weight loss. Low-grade fever can be present, and there can be darkening in the color of urine and stool.

    Unfortunately, bile duct tumors may not cause any symptoms until they have grown in size and the cancer has spread (metastasized) from beyond its original location. Abdominal pain is often a late symptom and is usually located in the right upper quadrant of the belly and may be associated with a tender, enlarged liver.

    How is bile duct cancer diagnosed?

    History and physical examination are key clues for the diagnosis of bile duct cancer. Painless jaundice (yellow/orange coloring of the skin) may be the only initial clue. The history often includes reviewing alcohol use, drug use or recent illnesses that can be associated with hepatitis, or inflammation of the liver. Other symptoms may be weight loss, loss of appetite, weakness, loss of energy, and easy bruising or bleeding.

    The physical examination may be useful in detecting tenderness in the abdomen, especially in the right upper quadrant beneath the ribs (where the liver is located). A quarter of patients with bile duct cancer will have an enlarged liver that can be palpated or felt on exam. On general exam, the patient is often jaundiced, having yellow-tinged skin. This may be seen most easily in the white portion (sclerae) of the eyes or under the tongue.

    Blood tests are often ordered to assess liver function. Liver enzymes (AST, ALT, GGT), bilirubin levels, complete blood count, electrolytes, BUN and creatinine, and INR/PTT (international normalized ratio/partial thromboplastin time) and PT (prothrombin time).

    There is no blood test that can specifically diagnose bile duct cancer. The diagnosis is confirmed by tissue sample obtained by biopsy and pathology examination of the cells obtained by that biopsy sample.

    Imaging may be used including ultrasound, CT scan, and MRI to look for a tumor and its location

    ERCP (endoscopic retrograde cholangiopancreatography) is a specialized test used to examine the bile duct as it enters the duodenum. ERCP is performed by a gastroenterologist using a fiberoptic camera at the end of a flexible viewing tube. The tube is passed through the mouth and passed through the stomach into the first part of the small intestine where the common bile duct enters from the gallbladder. This is used to detect diseases that affect the bile ducts including bile duct cancer, gallstones stuck in the bile duct, and abnormal narrowing of the bile duct. Dye can be injected to outline the bile ducts and detect obstruction. Biopsies or cell washings can be obtained to look for cancer cells. If a blockage is found, the gastroenterologist may be able to place a stent during the same procedure.

    Sometimes, an interventional radiologist may be needed to obtain a tissue biopsy by threading a needle through the skin into the liver.

    Once the diagnosis of bile duct cancer is made, it is important to stage the cancer to help direct potential treatment. The three parts of TNM staging include the following:

    • T is for the primary tumor and how much it has grown locally and invaded other structures. For a bile duct tumor, this includes the liver, gallbladder, pancreas, stomach, and intestine.
    • N is for the lymph nodes that are involved. The more nodes involved and the farther the distance from the bile duct, the more severe the cancer.
    • M is for metastasis. Has the tumor spread to other parts of the body?

    Cancer can be staged from 0 to 4, where 0 is no tumor, 1 is local tumor with no spread to lymph nodes or other parts of the body, and 4 is significant local growth and lymph node involvement and spread to other parts of the body.

    While staging is important, as well as detecting tumor spread beyond the liver and bile duct, often the critical staging questions can only be answered at surgery. Then it an assessment can be made as to whether or not the whole tumor be resected or removed. Survival rates are markedly improved if complete resection is possible.

    What is the treatment for bile duct cancer?

    Treatment for bile duct cancer depends upon where the cancer is located and whether it is possible to be completely removed by surgery. Unfortunately, those afflicted with this cancer tend to be older and may be unable to tolerate and recover from a significant operation. The decision regarding surgery needs to be individualized for the specific patient and their situation.

    Other treatment options tend to be palliative, not curative, and are meant to preserve quality of life. Chemotherapy and radiation therapy have their place. Photodynamic therapy is another alternative to help shrink the tumor and control symptoms.

    ERCP may be used to stent the bile duct, keeping it open to allow bile drainage from the liver and gallbladder into the intestine. This is often very helpful in controlling symptoms but does not treat the tumor itself.

    As with all cancers, the treatment is individualized for the patient. Discussion between the patient, health-care professional, and family are important to help understand treatment options, including cure versus palliation or symptom control and quality of life. The patient's wishes are key.

    What is the prognosis for bile duct cancer? What is the life expectancy for bile duct cancer?

    How well a patient does after the diagnosis of bile duct cancer depends upon many factors, including where the tumor is located, if and how much it has spread, and the patient's underlying general health. Typically, bile duct cancers have a poor prognosis and the five-year survival rate for a cancer that can be completely resected is less than 50%.

    National Cancer Institute statistics list the following 5-year survival rates:

    Intrahepatic (Within the Liver) Bile Duct CancerStage5-Year Relative SurvivalLocalized (Stage 1)15%Regional spread (Stage 2, 3)6%Distant spread (Stage 4)2%
    Extrahepatic (Outside the Liver) Bile Duct CancerStage5-Year Relative SurvivalLocalized (Stage 1)30%Regional spread (Stage 2, 3)24%Distant spread (Stage 4)2%

    Source: http://www.rxlist.com

    History and physical examination are key clues for the diagnosis of bile duct cancer. Painless jaundice (yellow/orange coloring of the skin) may be the only initial clue. The history often includes reviewing alcohol use, drug use or recent illnesses that can be associated with hepatitis, or inflammation of the liver. Other symptoms may be weight loss, loss of appetite, weakness, loss of energy, and easy bruising or bleeding.

    The physical examination may be useful in detecting tenderness in the abdomen, especially in the right upper quadrant beneath the ribs (where the liver is located). A quarter of patients with bile duct cancer will have an enlarged liver that can be palpated or felt on exam. On general exam, the patient is often jaundiced, having yellow-tinged skin. This may be seen most easily in the white portion (sclerae) of the eyes or under the tongue.

    Blood tests are often ordered to assess liver function. Liver enzymes (AST, ALT, GGT), bilirubin levels, complete blood count, electrolytes, BUN and creatinine, and INR/PTT (international normalized ratio/partial thromboplastin time) and PT (prothrombin time).

    There is no blood test that can specifically diagnose bile duct cancer. The diagnosis is confirmed by tissue sample obtained by biopsy and pathology examination of the cells obtained by that biopsy sample.

    Imaging may be used including ultrasound, CT scan, and MRI to look for a tumor and its location

    ERCP (endoscopic retrograde cholangiopancreatography) is a specialized test used to examine the bile duct as it enters the duodenum. ERCP is performed by a gastroenterologist using a fiberoptic camera at the end of a flexible viewing tube. The tube is passed through the mouth and passed through the stomach into the first part of the small intestine where the common bile duct enters from the gallbladder. This is used to detect diseases that affect the bile ducts including bile duct cancer, gallstones stuck in the bile duct, and abnormal narrowing of the bile duct. Dye can be injected to outline the bile ducts and detect obstruction. Biopsies or cell washings can be obtained to look for cancer cells. If a blockage is found, the gastroenterologist may be able to place a stent during the same procedure.

    Sometimes, an interventional radiologist may be needed to obtain a tissue biopsy by threading a needle through the skin into the liver.

    Once the diagnosis of bile duct cancer is made, it is important to stage the cancer to help direct potential treatment. The three parts of TNM staging include the following:

    • T is for the primary tumor and how much it has grown locally and invaded other structures. For a bile duct tumor, this includes the liver, gallbladder, pancreas, stomach, and intestine.
    • N is for the lymph nodes that are involved. The more nodes involved and the farther the distance from the bile duct, the more severe the cancer.
    • M is for metastasis. Has the tumor spread to other parts of the body?

    Cancer can be staged from 0 to 4, where 0 is no tumor, 1 is local tumor with no spread to lymph nodes or other parts of the body, and 4 is significant local growth and lymph node involvement and spread to other parts of the body.

    While staging is important, as well as detecting tumor spread beyond the liver and bile duct, often the critical staging questions can only be answered at surgery. Then it an assessment can be made as to whether or not the whole tumor be resected or removed. Survival rates are markedly improved if complete resection is possible.

    Source: http://www.rxlist.com

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