Cholangiocarcinoma: Epidemiology, Risk Factors, Pathogenesis, and Diagnosis

Abstract

Cholangiocarcinoma (CCA) is a rare tumor arising from the epithelium of the intrahepatic or the extrahepatic bile ducts. It is rarely diagnosed before 40 years of age except in patients with primary sclerosing cholangitis. CCA is usually clinically silent until the tumor obstructs the bile ducts. Carbohydrate antigen 19-9 is the most commonly used tumor marker, and magnetic resonance cholangiopancreatography is the best available imaging modality for CCA. Endoscopic retrograde cholangiopancreatography and cholangioscopy allow tissue acquisition. Positron emission tomography may play a role in identifying occult metastases. Tissue diagnosis is obtained by brush cytology or bile duct biopsy.

The prognostic role of baseline CEA and CA 19-9 values and their time-dependent variations in advanced colorectal cancer patients submitted to first-line therapy

“…We agree with this fascinating hypothesis. However, in our study we observed the same independent prognostic role for CA 19-9, a glycoprotein expressed in several neoplastic disease especially gastrointestinal 18,19 , and in inflammatory processes such as Hashimoto thyroiditis or viral hepatitis 20,21 . To our knowledge, CA 19-9 has never been associated to cell adhesion and/or tumor growth and metastatization.…”

K‐Ras mutation and amplification status is predictive of resistance and high basal pAKT is predictive of sensitivity to everolimus in biliary tract cancer cell lines

“…BTCs arise as a result of epithelial cells lining the biliary tract (cholangiocytes) undergoing dysplasia, hyperplasia, carcinoma in situ , and finally invasive carcinoma. The incidence of the disease varies around the world, with highest rates in north‐eastern Thailand and neighbouring Laos and Cambodia where liver fluke infestations ( Opisthorchis viverrini ) are endemic (Charbel and Al‐Kawas, 2011; Geynisman and Catenacci, 2012). In the United States, there are approximately 12 000 new cases of BTC annually, with the majority (80–90%) presenting with advanced disease (Charbel and Al‐Kawas, 2011; Hezel et al ., 2010).…”

“…The incidence of the disease varies around the world, with highest rates in north‐eastern Thailand and neighbouring Laos and Cambodia where liver fluke infestations ( Opisthorchis viverrini ) are endemic (Charbel and Al‐Kawas, 2011; Geynisman and Catenacci, 2012). In the United States, there are approximately 12 000 new cases of BTC annually, with the majority (80–90%) presenting with advanced disease (Charbel and Al‐Kawas, 2011; Hezel et al ., 2010). The incidence of BTC, particularly intrahepatic cholangiocarcinoma, is increasing in the Western world for reasons that are presently unknown (Hezel et al ., 2010).…”

Mutational landscape of intrahepatic cholangiocarcinoma

“…For example, ICC affects 96 per 100,000 people in Thailand, an incidence 100-fold higher than the worldwide average 8 . In addition to the increasing incidence, the prognosis of ICC is devastating and the mortality rate is high 1 , because patients diagnosed with ICC are usually at advanced stages because of lack of appropriate markers for early conclusive diagnosis 9 . Currently, surgical removal is considered the only choice of treatment of ICC 1 .…”