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Cancers of the bile ducts are relatively uncommon in the United States. About 10,000 cases are diagnosed each year, mostly in people over the age of 70. Since most bile duct cancers are diagnosed in more advanced stages, the current five-year survival rate is only 10% to 30%, depending upon the type of cancer.
Bile duct cancer can be divided into two main categories: Intrahepatic and extrahepatic.
Intrahepatic bile duct cancer
This cancer occurs in the bile ducts that are within the liver. It is often misdiagnosed as liver cancer. Although uncommon, the incidence of intrahepatic bile duct cancer is increasing.
Extrahepatic bile duct cancers
These diseases occur in bile ducts outside the liver. There are three types of extrahepatic bile duct cancers:
- Perihilar bile duct cancer: Perihilar bile duct cancer is the most common type of extrahepatic bile duct cancer. It occurs at the junction where the bile ducts exit the liver. These tumors account for 40 to 60% of all bile duct cancer cases. Twenty to 30% originate in the lower bile duct, and about 10% arise in bile ducts within the liver. This disease is sometimes called hilar cancer or Klatskin tumors.
- Distal bile duct cancer: This disease arises near the small intestine, at the farthest reach of the bile ducts.
- Gallbladder cancer: A cancer that arises in the wall of the gallbladder.
Bile Duct Cancer Risk Factors
There are several medical conditions that increase the risk for bile duct cancer, which is typically found in the older population.
Risk factors include:
- Diseases of the liver, including cirrhosis, bile duct stones and cholangitis
- Age: Most cases in the United States are diagnosed in men and women over the age of 70
- Hepatitis B or hepatitis C infection
- Inflammatory bowel diseases, including Crohn’s disease and ulcerative colitis
- Ethnicity: Bile duct cancer is much more common in Asian countries, where a liver parasite is common. In the U.S., Native Americans and Hispanics are more likely to get bile duct cancers.
Excessive consumption of alcohol increases the risk of bile duct cancer, especially among people who have alcohol-associated liver damage.
Bile duct cancer is often discovered during a CT scan or ultrasound for unrelated health reasons. It can also be found during routine gallbladder surgery.
The most common symptoms of biliary cancer include:
- Jaundice: Yellowing of the skin and whites of the eyes
- Itching is a common symptom. It is caused by excess bilirubin in the blood reaching the skin.
- Enlarged abdominal mass: As the tumor grows and interferes with nearby organs, it can cause a visible mass.
- Abdominal pain and/or bloating can occur in advanced stages of bile duct cancer.
- Lack of appetite or unexplained weight loss
- Nausea and/or fever: These symptoms are often caused by abnormal liver function, infection caused by the blockage of bile ducts or an increase in bilirubin.
- Light-colored stools with dark colored urine
Having one or more of these symptoms does not necessarily mean you have bile duct cancer. However, it’s crucial to discuss these conditions with your doctor so that the disease can be diagnosed as early as possible.
Because bile ducts are deep within the body, they typically don’t cause any visible signs or symptoms until the disease is well advanced. Only a very small number of bile duct cancers are diagnosed before they have spread to other areas of the body.
Tools for diagnosing bile duct cancer may include:
- Blood tests to determine liver and gallbladder function
- Imaging tests, including CT scan, X-ray, MRI, and abdominal ultrasound
- Endoscopy (ERCP)
- Tumor markers: CEA, CA 19-9
Bile Duct Cancer Staging
The staging system for bile duct cancer is the TNM system, developed by the American Joint Committee on Cancer (AJCC).
Each type of bile duct cancer has its own staging system, based on where the tumor starts.
Perihilar Bile Duct Cancer Stages
(source: American Joint Committee on Cancer)
- TX: No description of the tumor’s extent is possible because of incomplete information.
- T0: There is no evidence of a primary tumor.
- Tis: Cancer cells are only in the mucosa (the innermost layer of the bile duct) and have not invaded deeper layers of the bile duct. This stage is also known as intramucosal carcinoma and was previously called carcinoma in situ.
- T1: The cancer has grown into deeper layers of the bile duct wall, such as the muscle layer or the fibrous tissue layer.
- T2: The tumor has grown through the wall of the bile duct and into nearby tissue.
- T2a: The tumor has grown through the wall of the bile duct and into surrounding fat.
- T2b: The tumor has grown through the wall of the bile duct and into nearby liver tissue.
- T2a: The tumor has grown through the wall of the bile duct and into surrounding fat.
- T3: The cancer is growing into branches of the main blood vessels of the liver (the portal vein and/or the hepatic artery) on one side (left or right).
- T4: The cancer is growing into the main blood vessels of the liver (the portal vein and/or the common hepatic artery) or into branches of these vessels on both sides (left and right), OR the cancer is growing directly into other bile ducts while part of the tumor is growing into one of the main blood vessels.
- NX: Nearby (regional) lymph nodes cannot be assessed.
- N0: The cancer has not spread to nearby lymph nodes.
- N1: The cancer has spread to nearby lymph nodes, such as those along the cystic duct, the common bile duct, the hepatic artery, and the portal vein.
- N2: The cancer has spread to lymph nodes farther away from the tumor, such as those around the major blood vessels of the abdomen (the aorta, the vena cava, the celiac artery, and the superior mesenteric artery).
- M0: The cancer has not spread to tissues or organs far away from the bile duct.
- M1: The cancer has spread to tissues or organs far away from the bile duct.
Once the T, N, and M categories have been determined, this information is combined in a process called stage grouping.
Stage 0 (Tis, N0, M0): Cancer cells are only in the innermost layer of the bile duct and have not grown into deeper layers (Tis). Cancer has not spread to nearby lymph nodes (N0) or distant sites (M0).
Stage I (T1, N0, M0): The cancer has grown into deeper layers of the bile duct wall, such as the muscle layer or the fibrous tissue layer (T1). It has not spread to nearby lymph nodes (N0) or distant sites (M0).
Stage II (T2, N0, M0): The tumor has grown through the wall of the bile duct and into surrounding fat (T2a) or liver tissue (T2b). Cancer has not spread to nearby lymph nodes (N0) or distant sites (M0).
Stage III: Has 2 substages:
Stage IIIA (T3, N0, M0): The cancer is growing into branches of the main blood vessels of the liver (the portal vein and/or the hepatic artery) on one side (T3). Cancer has not spread to nearby lymph nodes (N0) or distant sites (M0).
Stage IIIB (T1 to T3, N1, M0): The cancer has grown into deeper layers of the bile duct wall (T1) and may have grown through the wall and into nearby fat or liver tissue (T2). The cancer may be growing into branches of the main blood vessels of the liver on one side (T3). Cancer cells are found in nearby lymph nodes (N1), but the cancer has not spread to distant sites (M0).
Stage IV: Has 2 substages:
Stage IVA (T4, N0-1, M0): The cancer is growing into the main blood vessels of the liver (the portal vein and/or the common hepatic artery), is growing into branches of these vessels on both sides, or part of the cancer is growing directly into other bile ducts while another part of the tumor is growing into one of the main blood vessels (T4). The cancer may have spread to nearby lymph nodes (N0 or N1), but it has not spread to distant sites (M0).
Stage IVB (Any T, N2, M0) or (Any T, any N, M1): The cancer has either spread to lymph nodes away from the tumor (N2) or it has spread to distant sites (tissues or organs away from the bile duct) such as the lungs or bones (M1).
Choosing the right treatment for bile duct cancer depends upon disease stage, the patient’s physical condition and tumor characteristics. Our treatment approach is individualized for every patient.
MD Anderson has experts with years of experience treating bile duct cancer with surgery, radiation and chemotherapy.
Complete removal of the tumor is the most effective surgery for biliary tract cancers. Bile duct cancer surgery is a complicated operation. Only specialized surgeons with significant experience should attempt bile duct cancer surgery.
Surgery is only performed on patients with early-stage disease who are in good physical condition. Depending on the location of the tumor along the bile duct, complete removal of the tumor can be performed in up to 56% of patients.
For all patients with intrahepatic tumors and nearly all patients with perihilar tumors, surgical treatment requires removal of portions of the liver (hepatectomy). Tumors at the far end of the bile ducts typically require removal of portions of the pancreas and small intestine. This is sometimes called Whipple’s procedure.
Portal Vein Embolization
If bile duct cancer patients will need to have part of their liver removed, doctors must determine whether enough liver remains to function properly before surgery takes place. If precise calculations indicate that the remaining liver will not be large enough, procedures to induce liver growth are needed.
MD Anderson is a leader in using portal vein embolization (PVE). This procedure involves the injection of tiny plastic pellets through a needle into the blood vessel closest to the tumor. The pellets block the blood vessel, which tricks the other side of the liver to grow to make up for the loss. A surgical approach that includes a team of specialized liver anesthesiologists and nurses, dedicated radiologists and interventional radiologists is critical in achieving optimal outcomes for bile duct cancer patients.
Our radiation oncologists are recognized as leaders in the oncology field for biliary cancers, and have pioneered the use of advanced technologies to deliver higher doses of radiation to treat these challenging diseases. Radiation can improve survival and provide a realistic chance of cure in patients who are not candidates for surgery.
MD Anderson offers several radiation therapy options for patients with biliary cancers. These include:
Stereotactic body radiation therapy (SBRT): This involves delivery of very high doses of radiation in the span of about one or two weeks. SBRT is best for smaller tumors that are not near the gastrointestinal tract.
Intensity modulated radiation therapy (IMRT) and 3D conformal radiation therapy: These techniques use high-energy photons to treat tumors. The radiation beams are controlled by changing beam intensity, by using customized shielding to shape the beams, or both. These options are used when the tumor is near the gastrointestinal tract.
Proton therapy: This radiation treatment uses high energy particles to precisely target tumors without harming nearby organs. This option may help to spare the normal liver better than IMRT in some cases.
MD Anderson uses state-of-the-art image guidance to monitor delivery of radiation and to make sure it doesn’t harm the stomach, small bowel, colon, and healthy parts of the liver. X-rays or CT scans are used in various ways during radiation treatment to make sure the patient is properly positioned for the most effective dose.
Most biliary cancers are discovered at an advanced stage, when surgical treatment options are limited. Chemotherapy is typically used at an advanced metastatic stage to control the spread of the cancer, alleviate symptoms and improve overall survival. Gemcitabine, cisplatin, 5-FU, oxaliplatin, irinotecan are some of the commonly used chemotherapies for bile duct cancer.
Clinical trials of new agents are available at MD Anderson to improve the prognosis and survival of metastatic biliary cancers. In certain cases of locally advanced cancers, pre-operative (or neoadjuvant) chemotherapy is given to improve the chances of successful surgery. In others, chemotherapy may be recommended after surgical resection (adjuvant therapy) to decrease chances of recurrence.
An important area of research in biliary cancer is targeted therapy. Several genetic mutations have been identified for bile duct cancer that provide effective treatment targets. Targeted therapies are created to seek out and destroy these abnormalities within cancer cells. Targeted therapies are given orally or by IV injection. MD Anderson is testing several promising targeted therapies for bile duct cancer in clinical trials.
Management of Biliary Obstruction
Obstruction of the bile ducts and infection (cholangitis) can be life-threatening complications of bile duct cancers. A gastroenterology team with expertise is needed to manage biliary obstruction. Stents are tubes made of plastic or wire mesh that are surgically inserted into the bile duct to keep it open. Stents are crucial in preventing or correcting obstructions and complications, such as infection.
Bile duct cancer or therapies used to treat it can cause complications that must be managed. A multidisciplinary approach that includes palliative care, gastroenterology, interventional radiology and pain management is needed to address these issues.
Complications from bile duct cancer include:
- Obstruction to the stomach (gastric outlet obstruction)
- Slowing of the stomach (gastroparesis)
- Weight loss
- Fluid buildup in the abdomen (ascites)
- Abdominal pain
Why choose MD Anderson for bile duct cancer care?
At MD Anderson's Gastrointestinal Center, we customize your care for bile cancer. This means we treat you as an individual, not a statistic. Your treatment plan is personalized to include the most advanced therapies, while focusing on your quality of life.
Cancer of the biliary tract (cholangiocarcinoma and gallbladder cancer) can be aggressive and its management requires world-class multi-disciplinary expertise.
MD Anderson is working to improve survival rates for bile duct cancer by providing a range of innovative treatments, including targeted therapies, surgical techniques and high-dose radiation therapy.
Bile duct cancer survivors often have to deal with side effects. Our expert health care team provides supportive care and management of bile duct obstruction, malnutrition and digestive issues.