Meaning of cholangiocarcinoma: Location drives almost everything here, since a tumor deep inside the liver behaves and is assessed quite differently from one near the intestine.

Definition of cholangiocarcinoma

Cholangiocarcinoma is a cancer that begins in the cells lining the bile ducts, the tubes carrying bile from the liver to the small intestine. It is uncommon, and most cases are adenocarcinomas, meaning they arise from gland-forming lining cells. Tumors can develop in the fine ducts inside the liver or in the larger ducts outside it.

How cholangiocarcinoma is built

chol angio carcinoma cholangiocarcinoma
  • chol/e, chol/o Combining form · Greek bile, gall
  • angi/o Combining form · Greek vessel
  • -carcinoma Suffix · Greek cancer arising in epithelial tissue

A medical term is read from its ending backwards: the suffix first, then the prefix, then the root. See all 223 word parts.

How is it classified?

TypeWhere it arisesHow it usually declares itself
IntrahepaticThe small bile ducts inside the liverOften a mass found on imaging, with vague pain or weight loss
PerihilarWhere the right and left hepatic ducts join, just outside the liverBlocked bile flow and yellowing of the skin
DistalThe lower duct, closer to the small intestineBlocked bile flow and yellowing of the skin

A perihilar tumor is also called a Klatskin tumor, and this group is the most common of the three. The division is not a formality: the three differ in how they are staged, in what an operation would involve, and in which neighboring structures a tumor tends to reach first.

Why does jaundice appear so often?

Bile carries a yellow pigment called bilirubin out of the liver. A tumor growing in or around a duct narrows the channel, and once flow is obstructed the pigment backs up into the blood. The result is jaundice: yellowing of the skin and of the whites of the eyes, dark urine, pale stool and often intense itching. A tumor inside the liver has many alternative drainage routes around it, so it can grow considerably before producing any of this, which is part of why intrahepatic tumors are frequently found late. A single duct outside the liver has no such redundancy, so even a small tumor there produces obvious signs early.

What raises the risk?

Long-standing inflammation of the bile ducts is the common thread. Primary sclerosing cholangitis, a chronic scarring disease of the ducts, carries a markedly increased risk. So do congenital cysts of the bile duct, chronic infection with liver flukes in regions where those parasites are common, and repeated stones within the ducts. Cirrhosis and chronic viral hepatitis contribute as well, particularly for tumors arising inside the liver. Most cases, however, occur in people with none of these, so the absence of a known risk factor does not argue against the diagnosis.

Also known as

bile duct cancer

Used in a sentence

Imaging demonstrated bile duct dilation consistent with an obstructing cholangiocarcinoma.

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Good to know

Cholangiocarcinoma arises from the epithelial lining of the bile duct, which distinguishes it from the far rarer cholangiosarcoma arising from the duct's connective tissue, though both can produce the same symptoms of bile duct obstruction.

Word origin

Where “cholangiocarcinoma” comes from

Formed from chole-, Greek for bile, angio-, meaning vessel or duct, and carcinoma, from Greek karkinoma, meaning cancer.

For learning, not diagnosis. This glossary provides general educational information and is not a substitute for advice from a qualified healthcare professional. If you may be experiencing a medical emergency, contact local emergency services.