Bile duct cancer, or cholangiocarcinoma, is a malignant tumor of the bile ducts within the liver (intrahepatic), or leading from the liver to the small intestine (extrahepatic). It is a rare tumor with poor outcome for most patients.
Bile is a substance manufactured by the liver that aids in the digestion of food. Bile ducts are channels that carry the bile from the liver to the small intestine. Like the tributaries of a river, the small bile ducts in the liver converge into two large bile ducts called the left and right hepatic ducts.
These exit the liver and join to form the common hepatic duct. The gallbladder, which concentrates and stores the bile, empties into the common hepatic duct to form the common bile duct. Finally, this large duct connects to the small intestine where the bile can help digest food. Collectively, this network of bile ducts is called the biliary tract.
Bile duct cancer originates from the cells that line the inner surface of the bile ducts. A tumor may arise anywhere along the biliary tract, either within or outside of the liver. Bile duct tumors are typically slowgrowing tumors that spread by local invasion of neighboring structures and by way of lymphatic channels.
Bile duct cancer is an uncommon malignancy. In the United States, approximately one case arises per 100,000 people per year, but it is more common in Southeast Asia. It occurs in men only slightly more often than in women and it is most commonly diagnosed in people in their 50s and 60s. In fact, about 65% of patients with bile duct cancer are over age 65.
A number of risk factors are associated with the development of bile duct cancer:
Jaundice is the first symptom in 90% of patients. This occurs when the bile duct tumor causes an obstruction in the normal flow of bile from the liver to the small intestine. Bilirubin, a component of bile, builds up within the liver and is absorbed into the bloodstream in excess amounts. This can be detected in a blood test, but it can also manifest as yellowish discoloring of the skin and eyes. The bilirubin in the bloodstream also makes the urine appear dark. Additionally, the patient may experience generalized itching due to the deposition of bile components in the skin. Normally, a portion of the bile is excreted in stool; bile actually gives stool its brown color. But when the biliary tract is obstructed by tumor, the stools may appear pale.
Abdominal pain, fatigue, weight loss, and poor appetite are less common symptoms. Occasionally, if obstruction of the biliary tract causes the gallbladder to swell enormously yet without causing pain, the physician may be able to feel the gallbladder during a physical examination. Sometimes the biliary tract can become infected, but this is normally a rare consequence of invasive tests. Infection causes fever, chills, and pain in the right upper portion of the abdomen.
Certain laboratory tests of the blood may aid in the diagnosis. The most important one is the test for elevated bilirubin levels in the bloodstream. Levels of alkaline phosphatase and CA 19-9 may also be elevated.
When symptoms, physical signs, and blood tests point toward an abnormality of the biliary tract, the next step involves radiographic exams. Ultrasound, computed tomography (CT scan), and magnetic resonance imaging (MRI) are noninvasive and rapid. In recent years, MRI has become the favored imaging choice for initial diagnosis of cholangiocarcinoma when the exam is available and affordable or covered by insurance. These tests can often detect the actual tumor as well as dilatation of the obstructed biliary tract. If these tests indicate the presence of a tumor, cholangiography is required. This procedure involves injecting dye into the biliary tract to obtain anatomic images of the bile ducts and the tumor. The specialist that performs this test can also insert small tubes, or stents, into a partially obstructed portion of the bile duct to prevent further obstruction by growth of the tumor. This is vitally important since itmay be the only intervention that is possible in certain patients. Cholangiography is an invasive test that carries a small risk of infection of the biliary tract. The objective of these radiological tests is to determine the size and location of the tumor, as well as the extent of spread to nearby structures.
The treatment of bile duct tumors is usually not affected by the specific type of cancer cells that comprise the tumor. For this reason, some physicians forego biopsy of the tumor.
The treatment is with surgical resection (removal) of the tumor and all involved structures. Unfortunately, sometimes the cancer has already spread too far when the diagnosis is made. Thus, in the treatment of bile duct cancer, the first question to answer is if the tumormay be safely resected by surgery with reasonable benefit to the patient. If the cancer involves certain blood vessels or has spread widely throughout the liver, resection may not be possible. Sometimes further invasive testing is required.
Angiography can determine if the blood vessels are involved. Laparoscopy is a surgical procedure that allows the surgeon to directly assess the tumor and nearby lymph nodes without making a large incision in the abdomen. Only about 45% of bile duct cancers are ultimately resectable.
If the tumor is resectable, and the patient is healthy enough to tolerate the operation, the specific type of surgery performed depends on the location of the tumor. For tumors within the liver or high up in the biliary tract, resection of part of the liver may be required. Tumors in the middle portion of the biliary tract can be removed alone. Tumors of the lower end of the biliary tract may require extensive resection of part of the pancreas, small intestine, and stomach to ensure complete resection.
Unfortunately, sometimes the cancer appears resectable by all the radiological and invasive tests, but is found to be unresectable during surgery. In this scenario, a bypass operation can relieve the biliary tract obstruction, but does not remove the tumor itself. This does not produce a cure but it can offer a better quality of life for the patient.
Prognosis depends on the stage and resectability of the tumor. If the patient cannot undergo surgical resection, the survival rate is commonly less than one year. If the tumor is resected, the survival rate improves, with 20% of these patients surviving past five years.
Other than the avoidance of infections caused by liver flukes, there are no known preventions for this cancer.