cho·lan·gi·o·car·ci·no·ma

An adenocarcinoma of the intrahepatic bile ducts.

Cholangiocarcinoma is an adenocarcinoma of the biliary duct system. It is a rare cancer with an incidence of 1-2:100,000. Its risk factors may include environmental exposures such as polyvinyl chloride or Thorotrast (thorium dioxide); however, this is controversial. It is also associated with the parasite opisthorchis viverrini and clonorchiasis - liver fluke. Other risk factors include hepatolithiasis, congenital liver disorders, thorotrast and ulcerative colitis - commonly when associated with primary sclerosing cholangitis. It is NOT associated with hepatitis B or liver cirrhosis.

Cholangiocarcinoma is considered curable only by surgical resection, and very often the disease is discovered too late for successful surgery. Chemotherapy has traditionally been seen as largely ineffective, but the chemotherapy agent gemcitabine (Gemzar) is often prescribed. Recently, there has been some success with the GFLIP protocol (Gemzar, 5-FU, leucovorin, irinotecan, and cisplatin or oxalyplatin), a protocol first developed and shown to be useful for pancreatic cancer.

A bile duct is any of a number of long tube-like structures that carry bile. Bile, required for the digestion of food, is excreted by the liver into passages that carry bile toward the hepatic duct, which joins with the cystic duct (carrying bile to and from the gallbladder) to form the common bile duct, which opens into the intestine. The biliary tree (see below) is the whole network of various sized ducts branching through the liver.

The top half of the common bile duct is associated with the liver, while the bottom half of the common bile duct is associated with the pancreas, through which it passes on its way to the intestine. It opens in the part of the intestine called the duodenum into a structure called the ampulla of Vater.

Blockage of the bile duct by a cancer, gallstones, or scarring from injury prevents the bile from being transported to the intestine and the active ingredient in the bile (bilirubin) instead accumulates in the blood. This condition is called jaundice and the skin and eyes become yellow from the bilirubin in the blood. This condition also causes severe itchiness from the bilirubin deposited in the tissues.

Descriptions and images are borrowed from answers.com and wikipedia