Extrahepatic Bile Duct Cancer Center

What is Extrahepatic Bile Duct Cancer?

Extrahepatic bile duct cancer is a rare disease in which malignant (cancerous) cells form in the tissues of the extrahepatic bile duct.

Part of the biliary system, the bile duct is a tube that connects the liver and the gallbladder to the small intestine. The right and left hepatic bile ducts lead out of the liver and join to become the common hepatic duct. The extrahepatic bile duct is the part of the common hepatic duct that is outside the liver.

Fluid called bile, which is made by the liver and breaks down fats during digestion, is stored in the gallbladder. When food is being digested, bile is released from the gallbladder through the common bile duct to the first part of the small intestine. The bile ducts and gallbladder make up the biliary tract.

Types of Bile Duct Cancers

Bile duct carcinomas can develop inside the liver (intrahepatic bile duct cancer) and outside the liver (extrahepatic bile duct cancer). 

The majority of bile duct cancers are adenocarcinomas. Bile duct adenocarcinomas, or cholangiocarcinomas, develop from the mucus glands that line the inside of the duct. The term "cholangiocarcinoma" is sometimes used to refer to any primary cancer of the biliary system. However, its use is often restricted to intrahepatic tumors and, therefore, it is not included in this overview.

Extrahepatic Bile Duct Cancer Risk Factors

Carcinoma of the extrahepatic bile duct is slightly more common in males, and most often occurs in between the ages of 50 and 60.

The following are some possible risk factors for extrahepatic bile duct cancer:

• Having chronic ulcerative colitis or certain diseases of the liver and bile duct
• History of Primary Sclerosing Cholangitis (PSC):  This is thought to be an autoimmune disorder, one in which the body's own inflammatory cells attack the bile ducts. PSC causes progressive scarring and narrowing of the bile ducts, which block bile from reaching the intestines. Many patients eventually develop liver failure, necessitating a liver transplant. About 10 to 20 percent of patients with PSC will develop bile duct carcinoma. It is thought that the progressive epithelial injury and subsequent regeneration predisposes patients with PSC to carcinoma. More than half of patients with PSC have a history of another autoimmune disorder, idiopathic inflammatory bowel disease.
• Congenital abnormalities (abnormalities one is born with) of the bile ducts:  These include choledochal cysts (dilation of the common bile duct) and Caroli's disease (dilation of the intrahepatic bile ducts). It is thought that prolonged sludging of bile in these dilated spaces, and subsequent infection, predispose patients to carcinoma through progressive epithelial injury and repair. The overall lifetime risk of cholangiocarcinoma in these patients is about 10 percent.
• Benign tumors of the bile ducts:  This includes bile duct adenomas (a single tumor) or bile duct papillomatosis (multifocal)
• Hepatobiliary parasitic infection:  These cases are most often seen in the Far East and include Clonorchis sinensis (most prevalent in Japan, Korea, and Vietnam) and Opisthorchis viverrini (most prevalent in Thailand, Laos, and Malaysia). Clonorchis is acquired when humans eat fresh water fish that harbor the Clonorchis cyst. The cysts develop into flukes (flatworms) in the friendly confines of the human intestine, and ascend from the duodenum (the first part of the intestine) into the common bile duct, where they mature. Carriage of this worm imparts a 25 to 50 risk of developing biliary tract carcinoma.
• Toxic exposures:  Thorium dioxide (Thorotrast), which was used as a contrast dye in radiologic procedures between 1930 and 1950, has been shown to promote cancers in the liver and bile ducts.

Symptoms of Extrahepatic Bile Duct Cancer

Though symptoms of extrahepatic bile duct cancer vary from person to person, some of the most common symptoms include the following:

• Jaundice (yellowing of the skin or whites of the eyes)
• Abdominal pain (possibly accompanied by loss of weight and/or appetite)
• Itching
• Fever

Extrahepatic Bile Duct Cancer Treatment Options

Extrahepatic bile duct cancer treatment options depend on many factors, such as the progression of the illness, as well as your age, overall health, and personal preferences. The most common bile duct cancer treatment options are surgery, radiation therapy and chemotherapy.  If the tumor cannot be removed surgically, bypass procedures may be performed to prevent obstruction of the gastrointestinal and biliary tracts and to relieve symptoms.

At Cancer Treatment Centers of America (CTCA), we use many tools to help you fight extrahepatic bile duct cancer on all fronts. Here, a multidisciplinary care team will work with you to develop a treatment plan that is tailored to your needs. Our cancer experts provide a powerful combination of traditional and new, innovative therapies. The following are some extrahepatic bile duct cancer treatment options:

• Surgery is a common procedure used to treat extrahepatic bile duct cancer whenever practical. Surgery may also help to relieve symptoms or prevent future complications, such as blockage or bleeding.
• Intensity Modulated Radiation Therapy (IMRT) employs a powerful, advanced computer program that plans a precise dose of radiation in three dimensions based on individual tumor size, shape and location. Remarkably, IMRT allows for higher radiation doses than traditional radiotherapy methods, while helping to spare more of the surrounding healthy tissue.
• Metronomic (Fractionated-Dose) Chemotherapy divides a powerful dose of anticancer drugs into smaller doses, administered over several days. This approach may expose cancer cells to the drugs for a longer period of time, while also helping to reduce the unpleasant side effects often experienced with larger doses.
• Biotherapy / Immunotherapy is a treatment that is sometimes used for extrahepatic bile duct cancer. Immunotherapy causes the body's own natural defenses (immune system) to attack the cancer.

Extrahepatic Bile Duct Cancer Quality of Life Therapies

In addition the therapies described above, CTCA enriches your treatment by offering complementary medicine therapies, such as such as naturopathic medicine, nutrition therapy, oncology rehabilitation, mind-body medicine, and spiritual support.  These therapies may help reduce side effects and improve your quality of life throughout extrahepatic bile duct cancer treatment. At CTCA, you receive integrated, patient-centered cancer care for the whole person, not just the disease.

Next Topic: Conventional Treatments for Extrahepatic Bile Duct Cancer