Trip Answers

Question answered:13/09/07 Warning! this question is over two years old.

eMedicine (an American online textbook) also has a chapter on cholangiocarcinoma [1], this reports:

 

“Frequency:

 

In the US: Each year, approximately 2500 cases occur, compared to 5000 cases of gallbladder cancer and 15,000 cases of hepatocellular cancer. Average incidence is 1 case per 100,000 persons per year.

 

Internationally: Incidence in most Western countries ranges from 2-6 cases per 100,000 people per year. The highest incidences are in Japan at 5.5 cases per 100,000 people and in Israel at 7.3 cases per 100,000 people.”

 

The eMedicine chapter reports on causes, stating:

 

“Causes: The etiology of most bile duct cancers remains undetermined. Currently, gallstones are not believed to increase the risk of CCC. Chronic viral hepatitis and cirrhosis also do not appear to be risk factors.

 

Infections
 - In Southeast Asia, chronic infections with liver flukes, Clonorchis sinensis, and Opisthorchis viverrini have been causally related to CCC.
 - Other parasites, including Ascaris lumbricoides, have been implicated in the pathogenesis.

 

Inflammatory bowel disease
 - A strong relationship exists between CCC and PSC. CCC generally develops in patients with long-standing ulcerative colitis and PSC.
 - The lifetime risk of developing this cancer in the setting of PSC is 10-20%. At increased risk are patients with ulcerative colitis without symptomatic PSC and a small subset of patients with Crohn disease.

 

Chemical exposures
 - Certain chemical exposures have been implicated in the development of bile duct cancers, primarily among workers in the aircraft, rubber, and wood finishing industries.
 - CCC occasionally has developed years after administration of the radiopaque medium thorium dioxide (ie, thorotrast).

 

Congenital diseases of the biliary tree, including choledochal cysts and Caroli disease, have been associated with CCC.

 

Other conditions rarely associated with CCC include bile duct adenomas, biliary papillomatosis, and alpha1-antitrypsin deficiency.”

 

Patient Plus (aimed at clinicians) also has a chapter [2] and this reports:

 

“Incidence 1–2 per 100,000 population per year in UK and US. In Japan and Israel the incidence is much higher at 5.5 and 7.3 cases per 100,000 people per year. There appears to have been a significant increase in the incidence of cholangiocarcinoma in the UK over recent years (a fifteen-fold increase in age specific mortality rates for those aged >45 from 1968 to 1998. This may be due to better diagnosis or a true increase in the disease's incidence, due to unknown mechanisms. There is a high incidence of cholangiocarcinoma in south-east Asian countries due to chronic endemic parasitisation with liver flukes.

 

Risk Factors

 

 - Most cases occur in those aged >60
 - Male:Female ratio 1.5:14
 - Inflammatory bowel disease – patients with chronic ulcerative colitis who develop primary sclerosing cholangitis are prone to this malignancy. The lifetime risk of developing this cancer is ~10–20% with primary sclerosing cholangitis; a subset of patients with Crohn's disease may also be at risk.
 - Infection with the liver flukes Clonorchis sinensis and Opisthorchis viverrini have been causally linked. Ascaris lumbricoides infection has been implicated
 - industrial chemical exposure – chemicals used in the aircraft, rubber and wood-finishing industries have been implicated
 - Use of the now-defunct radio-opaque contrast medium Thorotrast is associated with cases of cholangiocarcinoma, as are other incidents of thorium exposure
 - Presence of congenital choledochal cysts
 - Caroli disease (rare congenital disorder of intrahepatic bile ducts associated with autosomal recessive polycystic kidney disease where bile ducts become chronically dilated)
 - Recently implicated potential risk factors for intrahepatic form include hepatitis C, HIV, cirrhosis and diabetes”

 

Finally, you may be interested in the 2002 document “Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document” [3], see URL below.

 

References

1) http://www.emedicine.com/med/topic343.htm
2) http://www.patient.co.uk/showdoc/40001906/
3) http://gut.bmj.com/cgi/reprint/51/suppl_6/vi1.pdf
 

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