Cholangiocarcinoma: risk factors and clinical presentation.

Cholangiocarcinoma (CCA), a cancer originating from the neoplastic transformation of the biliary epithelium, is characterized by a progressive increase in incidence and prevalence. A number of risk factors have been identified including primary sclerosing cholangitis, liver fluke infestation, and hepatolithiasis. More recently, hepatitis viruses (HCV, HBV) have been taken into consideration as risk factors for the intrahepatic CCA and this could explain the increased incidence seen in the last two decades. All these risk factors induce chronic inflammation in the biliary epithelium together with partial bile obstruction. These two conditions are considered the background (chronic inflammation) favouring the cancer development. The only effective treatment is the radical surgical resection but, this is applicable in less than 40% of the patients since CCA is mostly diagnosed at an advanced stage. This mainly occurs because, in the majority of the cases, CCA is clinically silent, with symptoms only developing at an advanced stage but also for the lack of effective biomarkers to be used for a screening purpose. A number of serum and bile biomarkers have been recently proposed for the diagnosis of CCA but, their impact on the early diagnosis is still under the evaluation.