Classification, Diagnosis, and Management of Cholangiocarcinoma

Abstract

Cholangiocarcinomas (CCAs) are tumors that develop along the biliary tract. Depending on their site of origin, they have different features and require specific treatments. Classification of CCAs into intrahepatic, perihilar, and distal subgroups has helped standardize the registration, treatment, and study of this lethal malignancy. Physicians should remain aware that cirrhosis and viral hepatitis B and C are predisposing conditions for intrahepatic CCA. Treatment options under development include locoregional therapies and a chemotherapy regimen of gemcitabine and cisplatin. It is a challenge to diagnose perihilar CCA, but an advanced cytologic technique of fluorescence in situ hybridization for polysomy can aid in diagnosis. It is important to increase our understanding of the use of biliary stents and liver transplantation in the management of perihilar CCA, as well as to distinguish distal CCAs from pancreatic cancer, because of different outcomes from surgery. We review advances in the classification, diagnosis, and staging of CCA, along with treatment options.

High throughput molecular profiling reveals differential mutation patterns in intrahepatic cholangiocarcinomas arising in chronic advanced liver diseases

“…Of these, intrahepatic cholangiocarcinomas are the second most commonly occurring primary liver malignancy worldwide after hepatocellular carcinoma. 7 Furthermore, their incidence has been variably increasing not only in Western countries but also in Asia where biliary diseases are prevalent. 7 Intrahepatic cholangiocarcinomas usually arise in the normal liver but some are associated with chronic biliary diseases, such as primary sclerosing cholangitis, and hepatolithiasis.…”

“…7 Furthermore, their incidence has been variably increasing not only in Western countries but also in Asia where biliary diseases are prevalent. 7 Intrahepatic cholangiocarcinomas usually arise in the normal liver but some are associated with chronic biliary diseases, such as primary sclerosing cholangitis, and hepatolithiasis. 8 A minority of intrahepatic cholangiocarcinomas occurs in the setting of non-biliary chronic liver diseases, associated with HCV-and HBV-related hepatitis, leading to cirrhosis.…”

Comprehensive management of cholangiocarcinoma: Part I. Diagnosis

“…24,26 ERCP has been the mainstay in PCC or DCC where a dominant stricture, a polypoid intraductal mass or significant dilation of the proximal duct may be seen. 31 In differentiating benign from malignant biliary strictures, ERCP has been shown to have similar sensitivity, specificity and accuracy to MRCP (74% vs 70%, 76% vs 74%, and 70% vs 73%). 65 Due to the highly desmoplastic nature of CC resulting in the presence of fibrous tissue and the relatively small number of available malignant cells, endoscopic sampling is subject to errors and false negative results.…”

“…Due to its anatomic location, it is difficult to differentiate from pancreatic carcinoma. 30,31 DCC are well to moderately differentiated adenocarcinomas and are classified into intraductal papillary and biliary intraepithelial neoplasms. MF tumors are uncommon 4,32 The DCC has similar clinical presentation to the PCC, with painless jaundice, pruritus, weight loss, decreased appetite, and abdominal pain, but less incidence of fever.…”

Cholangiocarcinoma: from molecular biology to treatment

“…Considering that the vast majority of CCs at diagnosis are unresectable, patients are subjected to other therapeutic modalities with minimal success [5,11,53]. Therefore, chemotherapy is only used to control the disease and improve the quality of life in patients with unresectable CC, in patients with tumor recurrence and metastatic CC.…”