Cholangiocarcinoma: Advances in Pathogenesis, Diagnosis, and Treatment
By B. Blechacz, Gregory J Gores. Hepatology, 2008
678 citations recorded by the source corpus.
Cholangiocarcinoma (CCA) is an epithelial cancer originating from the bile ducts with features of cholangiocyte differentiation.1 CCA is the second most common primary hepatic malignancy, and epidemiologic studies suggest its incidence is increasing in Western countries.2 Advanced CCA has a devastating prognosis, with a median survival of <24 months.3 The only curative therapy is surgical extirpation or liver transplantation, but unfortunately the majority of patients present with advanced stage disease, which is not amenable to surgical therapies. Anatomically, CCA is classified into extrahepatic and intrahepatic forms of the disease. The extrahepatic form is more common, accounting for 80% to 90% of CCAs. It is further divided into proximal or perihilar and distal subsets depending on the location of the cancer within the extrahepatic biliary system. Perihilar disease is also frequently referred to as a Klatskin tumor. Three different growth patterns of extrahepatic CCA can be observed: (1) periductal infiltrating, (2) papillary or intraductal, and (3) mass forming.4 Intrahepatic CCA typically presents as an intrahepatic mass. In addition to their distinct morphology and clinical presentations, intrahepatic and extrahepatic CCAs differ in etiopathogenesis, molecular signatures, and management. In the last several years there have been significant new insights into the molecular pathogenesis of CCA. New diagnostic and therapeutic modalities have also been developed, resulting in improved detection rates and outcomes. In addition, we have now entered the era of targeted therapies for human cancers. Therefore, it is timely and topical to review these advances with a focus on promising targeted therapies for this disease. An additional goal is to stimulate further interest in this disease with the hope of improving outcomes for this still highly lethal malignancy.
Subfields: Pulmonary and Respiratory Medicine, Surgery
View Paper: https://www.semanticscholar.org/paper/b5276beae22480b728222377af2a4918f8a009b0
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In Pulmonary and Respiratory Medicine
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Citations in the corpus
- Clinical diagnosis and staging of cholangiocarcinoma (2011, background): “In addition, 90% of patients with perihilar cholangiocarcinoma clinically present with biliary problems such as painless jaundice, and 10% of patients present with cholangitis”
- Pathogenesis, Diagnosis, and Management of Cholangiocarcinoma (2013, background): “Serum levels of carbohydrate antigen 19-9 (CA19-9), a tumor biomarker, can aid in diagnosis, but this assay detects iCCA with only 62% sensitivity and 63% specificity.(133) Moreover, increased levels of CA19-9 also have been observed in patients with benign diseases such as bacterial cholangitis or ”