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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

Combined portal vein resection for hilar cholangiocarcinoma: a meta-analysis of comparative studies

Hilar cholangiocarcinoma (HCCA) frequently invades into the adjacent portal vein, and portal vein resection (PVR) is the only way to manage this condition and achieve negative resection margins. However, the safety and effectiveness of PVR is controversial. Studies analyzing the effect of PVR on the surgical and pathological outcomes in the management of HCCA with gross portal vein involvement were considered eligible for this meta-analysis. The outcome variables analyzed included postoperative morbidity, mortality, survival rate, proportion of R0 resection, lymph node metastasis, microscopic vascular invasion, and perineural invasion. From 11 studies, 371 patients who received PVR and 1,029 who did not were identified and analyzed. Data from patients who received combined PVR correlated with higher postoperative death rates (OR = 2.31; 95 % CI, 1.21-4.43; P = 0.01) and more advanced tumor stage. No significant difference was detected in terms of morbidity, proportion of R0 resection, or 5-year survival rate. Subgroup analysis demonstrated that in centers with more experience or studies published after 2007, combined PVR did not cause significantly higher postoperative death. No strong evidence could suggest that combined PVR leads to more morbidity or mortality for patients with HCCA when the portal vein is grossly involved. In addition, combined PVR is oncologically valuable because R0 resection and 5-year survival did not differ significantly between two cohorts, despite the fact that the PVR cohort consisted of patients with more advanced HCCA.

Association between praziquantel and cholangiocarcinoma in patients infected with Opisthorchis viverrini: a systematic review and meta-analysis

BACKGROUND: The liver fluke, Opisthorchis viverrini, and the associated incidence of subsequent cholangiocarcinoma (CCA) are still a public health problem in Thailand, and praziquantel (PZQ) remains the antihelminthic drug of choice for treatment. Evidence in hamsters shows that repeated infection and PZQ treatments could increase the risk of CCA. However, the existing evidence in humans is inconclusive regarding increased risk of CCA with frequency of PZQ intake.

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.

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Summaries for consumers

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.

Bile acids for primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterised by progressive inflammation and scarring of liver bile ducts. Destruction of bile ducts leads to incidence of bile flow to the gut, resulting in the development of biliary cirrhosis and end‐stage liver disease. PSC is most common in young males and its aetiology is still not fully understood. The disease is usually classified as an autoimmune disorder, but other aetiological factors cannot be excluded. There is a strong association of PSC with inflammatory bowel diseases, particularly ulcerative colitis, which coexists in approximately 70% of patients. Besides its progressive and irreversible nature, PSC is also associated with an increased risk for cholangiocarcinoma, which contributes to an even higher morbidity and mortality of this disease.

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Terms to know

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.
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.
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.
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.
Related to the liver.
A notch in or opening from a bodily part.
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.
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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