Synonyms in the broadest sense
Bile duct carcinoma, bile duct tumor, bile duct carcinoma, cholangiocarcinoma (CCC), cholangiocarcinoma, bile system cancer, klatskin tumor, hilary cholangiocarcinoma
Definition
The biliary tumor is caused by degeneration of the bile duct mucosa into an uncontrollably growing, malignant tissue (carcinoma). The bile duct cancer (carcinoma of the bile duct) grows relatively slowly and spreads (metastasizes) relatively late into other tissues. In bile duct cancer, a distinction is made between tumors that develop in bile ducts that lie inside (intrahepatic) or outside (extrahepatic) the liver. Overall, bile duct cancer has a poor prognosis, i.e. it is often incurable at the time of diagnosis. A special form of bile duct carcinoma is the Klatskin tumor, which develops at the junction of the excretory ducts of the right and left liver lobes into the common hepatic bile duct (Ductus hepaticus communis).
Frequency
Bile duct carcinomas are generally very rare. The cancer of the gall bladder is about 3 to 5 times more frequent than the cancer of the bile ducts. The peak of the disease lies beyond the age of 60. Men are more frequently affected by tumors of the bile ducts in contrast to gallbladder cancer, which tends to affect women.
Tumor types and localization
Biliary cancers are histologically mostly adenocarcinomas, which means that the tumors originate from glandular cells of the bile ducts. The tumor develops in a ring around the duct and then longitudinally along the bile ducts. As the disease progresses, the cavity (lumen) of the duct narrows and the bile accumulates in the liver.
This results in the development of jaundice (icterus). Tumors are often found at the bifurcations of the bile ducts, such as the confluence of the left and right lobe of the liver with the large common duct (Ductus hepaticus communis). Bile duct tumors that develop in this area are called Klatskin tumors.
Another predilection site for tumor development is the confluence of the common hepatic duct and the cystic duct of the gallbladder. The development of gallbladder cancer is promoted by various risk factors. Autoimmune diseases such as ulcerative colitis, a chronic inflammatory bowel disease, and primary sclerosing cholangitis (PSC), a chronic inflammatory bile duct disease, which is associated with narrowing (strictures) of the bile ducts due to connective tissue proliferation, correlate with the occurrence of bile duct tumors.
Patients with these diseases have a thirty-fold increased risk of developing biliary carcinoma. Another predictive factor is congenital caroli syndrome, which is associated with bag-like bulges of the bile ducts that lie within the liver (intrahepatic choledochal cysts). Infections of the bile ducts with parasites such as liver flukes and trematodes also contribute to the development of this type of cancer.
In addition, a connection has been observed between bile duct cancer and the chronic consumption of cigarette smoke. In this context, dimethylnitrosamide, which is found in cigarette smoke, is thought to play an important role as a carcinogenic substance. In contrast to gallbladder carcinoma, gallstones do not correlate with the occurrence of bile duct tumors.