Bile Duct Cancer Prognosis

The bile duct is a narrow tube that connects the liver to the small intestine. Its function is to carry bile (which helps to digest fat in the food we eat) from the gallbladder and liver to the small intestine. Bile duct cancers (or cholangiocarcinomas) are rare (only about 2,000 cases are diagnosed each year in the U.S.) and occurs most often in the 50- to 70-year age bracket. Tumors generally spread and grow relatively slowly and symptoms are usually mild. The most effective treatment is surgical removal; however, patients are often asymptomatic until later stages when the cancer has already spread and the tumor cannot be completely removed.

Symptoms

• Symptoms are often vague and could be attributed to other medical conditions such as gallstones. Tumors can block the ducts, resulting in loss of appetite, abdominal pain, back pain, fever, chills, itching, clay-colored stools, bloating and jaundice (yellowing of the skin). Jaundice is caused by an accumulation of bilirubin (a component of bile) which normally is transported to the small intestines via the bile ducts. Pain usually occurs in more advanced stages of the disease.

Types

• Cancer can develop in any area of the bile duct system. The most common tumors are called Hilar or Klatskin tumors and are located at the intersection of the right and left hepatic bile ducts. Intrahepatic bile duct cancers develop in the smaller branches within the liver. Distal bile duct cancer is located closer to the small intestine. The majority (more than 95 percent) of cholangiocarcinomas are adenocarcinoma (cancer of glandular cells which develop from the mucous glands lining the ducts).

Diagnosis

• Diagnosis may be made using one (or more) of several procedures.
  • Blood tests
  • Ultrasounds
  • CT scans
  • MRI
  • Fine-Needle Aspiration, which is performed with a local anesthetic and allows your doctor to use a small needle to remove a small sample of the tumor for microscopic study.
  • Cholangiogram (X-ray of the bile ducts often performed as an endoscopy)
  • PET (Positron Emission Tomogram), performed by introducing sugar molecules (radioisotope-labeled molecules) into the bloodstream. Tumors will attract the sugar molecules and the PET scan can pick up any "hot spots" to locate the tumor.

Risk Factors

• Researchers have linked certain risk factors to a higher potential for developing bile duct cancer:
  • Chronic bile duct inflammation (can be caused by conditions such as sclerosing cholangitis, ulcerative colitis, bile duct stones, cirrhosis)
  • Rare liver or bile duct such as Caroli syndrome or polycystic liver disease
  • Family History
  • Exposure to Thorotrast (radioactive substance used in X-rays until the 1950s)
  • Obesity

  Other risk factors may include:
  • Obesity
  • Smoking
  • Pancreatitis
  • Hepatitis B or Hepatitis C
  • Asbestos exposure
  • Radon exposure

Treatment

• Small tumors can be surgically removed. This is the optimal treatment and generally offers the most favorable prognosis. This is referred to as the "Resectable" stage. Radiation is often recommended after surgery, and chemotherapy may also be part of the treatment plan. When the tumor has spread beyond the bile duct (to the liver or lymph nodes) and cannot be removed, it is known as "Unresectable." Chemotherapy and radiation are occasionally used in an effort to alleviate some symptoms, but have not been shown to be effective in treating the cancer.

Additional Treatment Options

• The Mayo Clinic is one of only a few facilities in the world to offer liver transplantation as an option for patients with tumors that are contained within the liver but cannot be completely resected otherwise. Tumors may still recur; however, in some cases the liver transplant cured the cancer.

  Photodynamic therapy is a treatment in which a light-sensitive chemical is injected into the patient. Your doctor then uses a special endoscope to go into the bile duct and direct a laser at the tumor. This only affects cells on the surface, but has shown promise in killing tumor cells.

Prognosis

• Your prognosis will vary depending on your cancer stage and treatment options. It's important to know that since bile duct cancer is rare, accurate survival rates are difficult to determine. For patients with resectable bile duct cancers, the five-year survival rate varies from 10 percent to 40 percent depending of the type and location of the tumor. The five-year survival rate ranges from 10 percent to 30 percent. Unresectable cancers have a lower five-year survival rate.