Bile duct carcinoma (cholangiocellular carcinoma) is a malignant cancer of the bile ducts that occurs rather rarely. In the western world, one to two people per 100,000 inhabitants are diagnosed with cholangiocellular carcinoma every year.
Here you will find all the information and selected specialists for the diagnosis and treatment of cholangiocarcinoma.
A characteristic feature of cholangiocellular carcinoma is that the tumor grows very slowly. For this reason, in many cases the first symptoms only appear at later stages of the disease. This is one reason why the disease is often diagnosed too late.
The tumors, also known as cholangiocarcinomas, belong to the class of adenocarcinomas. This means that the malignant changes originate from the glandular tissue of the bile ducts. Depending on their anatomical location, cholangiocarcinomas are divided into extrahepatic, intrahepatic and perihilar cholangiocarcinomas.
With a share of 60 to 70 percent, perihilar carcinoma, the so-called Klatskin tumor, is the most common bile duct carcinoma. The tumor is located in the area of the bile duct bifurcation, where the left and right common bile ducts converge. While intrahepatic tumors are found in the bile ducts of the liver, extrahepatic carcinomas are located outside the liver. Distal tumors occur in the last section of the bile duct up to the mouth of the duodenum.
As with many cancers, the exact causes of cholangiocellular carcinoma are unknown. However, there are various risk factors that can promote the development of the tumor. One of these risk factors is primary sclerosing cholangitis (PSC). This is a chronic inflammation of the bile ducts. It often occurs in connection with the chronic inflammatory bowel disease ulcerative colitis.
People with primary sclerosing cholangitis are up to 15 percent more likely to develop the disease. It is not yet known exactly why primary sclerosing cholangitis can develop into cholangiocarcinoma. However, it is probably related to the inflammatory conditions.
Various parasitic diseases of the liver and bile ducts also increase the risk of degeneration of the tissue in the bile ducts. However, this risk factor is negligible in the western world. The parasites that cause the disease are mainly found in countries such as Thailand, Laos, Malaysia, Japan and Korea.
The following diseases can promote cancer of the bile ducts:
A connection between the occurrence of gallstones and cholangiocellular carcinoma is suspected. However, this has not yet been scientifically proven.
Various liver diseases, such as liver cirrhosis, can promote the development of cholangiocellular carcinoma.
Patients with cholangiocellular carcinoma do not experience any symptoms for a long period of time. Symptoms such as yellowing of the skin (icterus) only occur in more advanced stages. This yellowing is caused by an increased concentration of bilirubin in the blood. Bilirubin is a breakdown product of hemoglobin (red blood pigment).
Normally, bilirubin is discharged into the intestine with the bile and excreted in the stool. In the case of advanced bile duct carcinoma, however, the tumor can block the bile duct. This results in impaired bile outflow and the bilirubin passes into the blood. As a result, less bilirubin enters the intestine.
As the substance normally gives the stool its brownish color, the stool is now discolored. Increased excretion via the kidneys results in brownish urine. Other typical symptoms of cholangiocarcinoma are
The prognosis of cholangiocellular carcinoma depends on whether the tumor can be completely removed during surgery. If complete surgical removal is not possible, the carcinoma of the bile ducts is considered incurable. It then usually leads to death within a short time. Tumors that can be completely removed surgically have a significantly better prognosis.
If necessary, not only the bile ducts but also the gallbladder and parts of the liver are removed during the operation. These are usually major liver resections. In order to ensure that bile can continue to flow from the liver into the intestine, the surgeon may create new connections there.
The benefits of so-called neoadjuvant therapy are still controversial. Patients receive chemotherapy or radiotherapy before the operation to shrink the tumor. Subsequent chemotherapy or radiotherapy is particularly useful if complete removal of the tumor was not successful and/or to reduce the risk of recurrence.
In many cases, bile duct carcinoma is unfortunately inoperable. In this case, palliative chemotherapy with or without radiotherapy is usually given. This is intended to improve the patient's quality of life and prolong their life.
If necessary, patients are given a so-called stent (plastic or metal tube) to ensure bile drainage. This is inserted into the bile duct. The stent keeps the bile ducts open so that the bile can drain better. It is also possible to drain bile to the outside by inserting a drainage tube.
Overall, the prognosis for cholangiocellular carcinoma is rather poor. For a large proportion of patients, only palliative therapy is possible. Five years after diagnosis, only 10 to 20 percent of those affected are still alive.
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