Biliary cancer: intrahepatic cholangiocarcinoma vs. extrahepatic cholangiocarcinoma vs. gallbladder cancers: classification and therapeutic implications

Abstract

Biliary cancers (BCs) are a diverse group of tumors that arise from the bile duct epithelium and are divided into cholangiocarcinomas of the intrahepatic and extrahepatic cholangiocarcinoma (EHCC) and cancer of the gallbladder. Despite improvements in treatment and diagnosis, BCs are often diagnosed at an advanced stage and associated with poor prognosis and limited treatment options. Recent discoveries have allowed us to have a better understanding of the genomic diversity in BC, and identify genes that are likely contributing to its pathogenesis, proliferation and treatment resistance. Additionally, these advances have allowed us to reason that each anatomic group within BC behave as distinct diseases, with differences in prognosis and outcomes. Based on this knowledge, recent advances have allowed us to identify actionable mutations that form rational therapeutic targets with novel agents, where their relevance will be better understood through the completion of prospective clinical trials.

Causes of hOCT1‐Dependent Cholangiocarcinoma Resistance to Sorafenib and Sensitization by Tumor‐Selective Gene Therapy

“…At present, sorafenib is the reference drug used in the pharmacological treatment of HCC. The high proportion of genetic alterations in critical signaling pathways involved in cell proliferation in CCA has led to an interest in the development of clinical trials investigating targeted therapies including sorafenib . However, data on the effectiveness of sorafenib, and other TKIs, in patients with CCA are controversial, reporting both beneficial effect and poor activity against this type of liver cancer .…”

“…Biliary tract cancers are a type of heterogeneous tumors that include cholangiocarcinoma (CCA), both intrahepatic (iCCA) and extrahepatic (eCCA), and gallbladder cancer, with diverse phenotypic characteristics . CCA, whose incidence is increasing worldwide, is currently the second most frequent primary hepatic malignancy after hepatocellular carcinoma (HCC).…”

ID3 Promotes Stem Cell Features and Predicts Chemotherapeutic Response of Intrahepatic Cholangiocarcinoma

“…Their results are inconsistent with ours for the following reasons. First, BTC is a heterogeneous group of diseases consisting of intrahepatic cholangiocarcinoma, extrahepatic cholangiocarcinoma, and gallbladder cancer; each anatomic group within BTC behaves as a distinct disease, with different prognoses and outcomes . Second, Harder included only the advanced BTC (unresectable ICC patients), which is quite different from our study cohort (resectable ICC patients following hepatectomy).…”

Pattern of distant metastases in primary extrahepatic bile‐duct cancer: A SEER‐based study

“…Compared with its counterpart occupied in the liver (roughly 5%‐10% of total cholangiocarcinomas), which is currently classified as primary liver cancer . EBDC is unique not only from the perspective of anatomy, but also from epidemiology, pathogenesis and its further development compared with other malignancies . Over 12 000 cases of extrahepatic biliary tract cancers are diagnosed annually in the United States, more than 60% are gallbladder cancers.…”

“…3,4 EBDC is unique not only from the perspective of anatomy, but also from epidemiology, pathogenesis and its further development compared with other malignancies. 5,6 Over 12 000 cases of extrahepatic biliary tract cancers are diagnosed annually in the United States, more than 60% are gallbladder cancers. The rest, approximately 3000 cases per year, are extrahepatic cholangiocarcinomas.…”