Cholangiocarcinoma – present and future

Key messages

Cholangiocarcinoma is anatomically classified as intrahepatic, perihilar, and distal
Mixed hepatocellular-cholangiocellular carcinoma is a subtype of intrahepatic neoplasm that shows markers of hepatocellular carcinoma and cholangiocarcinoma differentiation simultaneously and is associated with worse prognosis compared with hepatocellular carcinoma
Cirrhosis and hepatitis B and C are recently identified risk factors for intrahepatic cholangiocarcinoma
All intrahepatic lesions in cirrhosis should be investigated to rule out the possibility of intrahepatic cholangiocarcinoma
Fluorescence in-situ hybridisation improves performance of cytological evaluation of biliary brushings for the diagnosis of perihilar cholangiocarcinoma
Proliferative and inflammatory gene signature classes have been described in intrahepatic cholangiocarcinoma; FGFR2 gene fusion and IDH1 and IDH2 mutations are newly identified targetable derangements in cholangiocarcinoma
Surgical resection is a first-line therapy in patients with intrahepatic or perihilar cholangiocarcinoma who are good surgical candidates and have no evidence of disease progression beyond regional lymph nodes
Surgical techniques for perihilar cholangiocarcinoma are improved by extended resection, portal vein embolisation, and associating liver partition and portal vein ligation for staged hepatectomy
The best outcomes are observed in highly selected patients with perihilar cholangiocarcinoma treated with liver transplantation coupled with neoadjuvant chemoradiation
Locoregional therapies can be considered for intrahepatic cholangiocarcinoma
Gemcitabine and cisplatin combination is an acceptable standard of practice for advanced intrahepatic cholangiocarcinoma; for perihilar disease the effectiveness remains less proven
Elucidation of cholangiocarcinoma molecular pathogenesis could guide early diagnosis, prevention, and individualised treatment

Read text here:
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4069226/