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Cholangiocarcinoma (Bile Duct Cancer)

A rare cancer that forms in the bile ducts. A bile duct is a tube that carries bile (fluid made by the liver) between the liver and gallbladder and the small intestine.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Bile Duct Cancer (Cholangiocarcinoma)

Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts.

A network of tubes, called ducts, connects the liver, gallbladder, and small intestine. This network begins in the liver where many small ducts collect bile (a fluid made by the liver to break down fats during digestion). The small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct, and cystic duct and is stored in the gallbladder.

When food is being digested, bile stored in the gallbladder is released and passes through the cystic duct to the common bile duct and into the small intestine.

Bile duct cancer is also called cholangiocarcinoma... Read more about Cholangiocarcinoma

What works? Research summarized

Evidence reviews

Systematic review and meta-analysis of the role of vascular resection in the treatment of hilar cholangiocarcinoma

BACKGROUND: The management of hilar cholangiocarcinoma has evolved over time and extended liver resection, including the caudate lobe, and major vascular resection and extended lymphadenectomy have become established practice. The benefit of vascular resection has not been investigated.

Serum carbohydrate antigen (CA) 19-9 as a prognostic factor in cholangiocarcinoma: a meta-analysis

This study was performed to determine the prognostic role of preoperative serum carbohydrate antigen (CA) 19-9 levels in the survival of patients with cholangiocarcinoma. Articles published up to June 1(st), 2010 that evaluated preoperative CA19-9 levels and the prognosis of cholangiocarcinoma were collected for meta-analysis. The required information for calculating individual relative risk (RR) was extracted from the studies, and a combined overall RR was estimated. Nine eligible studies were included. One study dealt with extra-hepatic cholangiocarcinoma, while the other eight studies analyzed intra-hepatic cholangiocarcinoma. The mean methodological quality score was 74.1%, ranging from 65.5% to 82.5%. The overall RR for the nine studies was 1.28 (95% confidence interval = 1.10-1.46), and the Z-score for overall effect was 13.83 (P<0.001). The association between serum CA19-9 level and lymph node involvement was also assessed. The combined RR was 1.471 (95% confidence interval = 0.411-5.264) and Z-score for overall effect was 0.59 (P = 0.553). CA19-9 levels were associated significantly with the prognosis of patients with cholangiocarcinoma. This meta-analysis shows that elevation of preoperative CA19-9 levels is correlated with a poor prognosis of patients with cholangiocarcinoma. However, larger scale and randomized studies are needed to draw a more substantive conclusion.

Management of lymph nodes during resection of hepatocellular carcinoma and intrahepatic cholangiocarcinoma: a systematic review

The role of lymph node dissection (LND) in the treatment of patients with hepatocellular carcinoma (HCC) or intrahepatic cholangiocarcinoma (ICC) remains controversial. We sought to systematically review all available evidence to determine the role of LND in patients with HCC and ICC. Studies that reported on LND, lymph node metastasis (LNM), and short- and long-term outcomes for patients with HCC or ICC survival were identified from PubMed, Cochrane, Embase, Scopus, and Web of Science databases. Data were extracted, synthesized, and analyzed using standard techniques. A total of 603 and 434 references were identified for HCC and ICC, respectively. Among HCC patients, the overall prevalence of LND was 51.6 % (95 % confidence interval (CI) 19.7-83.5) with an associated LNM incidence of 44.5 % (95 % CI 27.4-61.7). LNM was associated with a 3- and 5-year survival of 27.5 and 20.8 %, respectively. Among ICC patients, most patients 78.5 % (95 % CI 76.2-80.7) underwent LND; 45.2 % (95 % CI 39.2-51.2) had LNM. Three and 5-year survival among ICC patients with LNM was 0.2 % (95 % CI 0-0.7) and 0 %, respectively. While there are insufficient data to recommend a routine LND in all patients with HCC or ICC, the potential prognostic value of LND suggests that LND should at least be considered at the time of surgery.

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Summaries for consumers

Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®): Patient Version

Expert-reviewed information summary about the treatment of bile duct cancer.

Insufficient evidence to support or refute glucocorticosteroids for primary sclerosing cholangitis

Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and, ultimately, liver cirrhosis. Patients with primary sclerosing cholangitis are at higher risk of cholangiocarcinoma as well as of colonic neoplasia, since primary sclerosing cholangitis is associated with inflammatory bowel disease in more than 80% of patients. Several therapeutic modalities have been proposed for primary sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis with liver cirrhosis.

No evidence to support or refute endoscopic retrograde cholangiopancreaticography (ERCP) with stenting in patients with malignant pancreaticobiliary diseases, awaiting surgery

Pancreatico‐biliary malignancy includes cancers of pancreas, ampulla, duodenum, and cholangiocarcinoma. There is significant morbidity and mortality related to surgery in these patients. Studies have claimed the beneficial role of biliary decompression, which can be performed via endoscopic retrograde cholangiopancreaticography (ERCP) with stent insertion pre‐surgically. The review found that pre‐surgical biliary stenting via ERCP did not improve the morbidity and mortality in patients with pancreatico‐biliary malignancy. Further evidence about its efficiency is needed.

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Terms to know

Adenocarcinoma
Cancer that begins in glandular (secretory) cells. Glandular cells are found in tissue that lines certain internal organs and makes and releases substances in the body, such as mucus, digestive juices, or other fluids. Most cancers of the breast, pancreas, lung, prostate, and colon are adenocarcinomas.
Bile
Fluid made by the liver and stored in the gallbladder that helps break down fats and get rid of wastes in the body.
Bile Ducts
Tubes that carry bile from the liver to the gallbladder for storage and to the small intestine for use in digestion.
Distal
In medicine, refers to a part of the body that is farther away from the center of the body than another part. For example, the fingers are distal to the shoulder. The opposite is proximal.
Gallbladder
The organ that stores the bile made in the liver and that is connected to the liver by bile ducts. The gallbladder can store about 2 tablespoons of bile. Eating signals the gallbladder to empty the bile through the bile ducts to help the body digest fats.
Hepatic
Related to the liver.
Hilum
A notch in or opening from a bodily part.
Liver
The largest abdominal organ. The liver carries out many important functions, such as making important blood proteins and bile, changing food into energy, and cleaning alcohol and poisons from the blood.
Perihilar
Surrounding the hilum.
Small Intestine
The organ where most digestion occurs. It measures about 20 feet and includes the duodenum, jejunum, and ileum.

More about Cholangiocarcinoma

Photo of an adult

Also called: Bile duct adenocarcinoma, Bile duct carcinoma, Cholangiocellular carcinoma

See Also: Extrahepatic Bile Duct Cancer, Intrahepatic Bile Duct Cancer

Other terms to know: See all 10
Adenocarcinoma, Bile, Bile Ducts

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