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What is Cholangiocarcinoma

What Cholangiocarcinoma Is

Cholangiocarcinoma (also called cholangioma or bile duct cancer) is a rare, malignant tumor of the bile duct, accounting for fewer than 1% of all cancers. Cholangiocarcinoma is a type of adenocarcinoma ("adeno" for gland) that originates in the mucous glands of the epithelium, or surface layer, of the bile ducts.

Diagram showing the network of bile ducts and associated organs
DThe liver, pancreas, gallbladder, and
network of bile ducts (Click to enlarge)

Bile Ducts

Bile ducts are channels that carry bile or "gall" from the liver where it is secreted to the gall bladder for storage. The bile is discharged in the duodenum (the first 1/3 of the small intestine) primarily to aid digestion by emulsifying fats.  The bile ducts start as small tributaries in the liver and merge into larger ducts. These ultimately lead to the common hepatic duct. The common hepatic duct then leaves the liver and attaches to the gall bladder via the cystic duct, which then empties into the duodenum via the common bile duct. The pancreatic duct, which delivers pancreatic enzymes to the intestines to help digestion, also enters the duodenum at the same site. It sometimes merges with the common bile duct before entering the duodenum.

Where Cholangiocarcinoma Begins

Cholangiocarcinomas can therefore arise within the liver (i.e. intra-hepatic) or in the bile ducts just outside the liver.  The disease can spread along the bile ducts, thereby involving both the liver and either the pancreas, gall bladder or duodenum.  They can also spread to adjacent lymph nodes, spread to liver or lungs via the blood, or simply "seed" the abdomen with tumor cells landing on other abdominal organs (e.g. bowel, bladder, rectum, ovaries).  This "seeding" process is called intra-peritoneal dissemination and is quite common with this disease.
Some cholangiocarcinomas are true primary liver tumors in that they may originate in the liver, while others may spread there or to adjacent organs (including pancreas) through the bile ducts. 

More than two thirds of cholangiocarcinomas are located in either the common hepatic duct (CHD) or the common bile duct (CBD). The CHD -- where the right and left ducts from the liver meet to form one channel on to the duodenum -- is the most likely location for a cholangiocarcinoma, and the CBD -- the channel from the gallbladder formed by union of the hepatic duct and the cystic duct -- is the second most common location for a cholangiocarcinoma.

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