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Cholangiocarcinoma

Orpha number ORPHA70567
Prevalence of rare diseases 1-5 / 10 000
Inheritance
  • Sporadic
Age of onset Adulthood
ICD 10 code
  • C22.1
MIM number -
Synonym(s) Bile duct cancer

Summary

Cholangiocarcinoma is a primary tumour of the extra- or intrahepatic biliary ducts. The prevalence is estimated at 2 in 100 000 and the tumour is slightly more frequent in males. The disease progresses slowly, with symptoms only becoming apparent during the later stages of the disease. The principle manifestations are abdominal pain, and jaundice (which may or may not be accompanied by pain) often associated with puritus. Treatment is usually surgical, but palliative care may be sufficient in some cases, and aims to re-establish bile flow to the intestine. The prognosis is severe, as the cancer is usually at an advanced stage at the time of diagnosis. *Author: Orphanet (January 2006)*.

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