Biliary tract cancer (BTC) is a rare but highly aggressive malignancy that includes intrahepatic cholangiocarcinoma (ICC), extrahepatic cholangiocarcinoma, and gallbladder cancer (GBC). While BTC has a low global incidence, its regional variations are notable. Among nations, Korea has the second-highest incidence of BTC globally, with the highest mortality rate worldwide, underscoring the need for a deeper understanding of this cancer. Liver fluke infection and hepatitis B virus infection are key risk factors unique to Korea, contributing to regional differences in BTC incidence. Additionally, genomic alterations in Korean patients with BTC differ from those in other populations, including lower frequencies of IDH1 mutations and FGFR2 fusions in ICC and a higher prevalence of ERBB2 amplification in GBC. Recognizing the clinical significance of these alterations, ivosidenib and pemigatinib have been approved in Korea for BTC patients with IDH1 mutations and FGFR2 fusions, respectively. This review explores the epidemiology, risk factors, and molecular features of BTC, along with corresponding targeted therapies. Furthermore, we compare the unique characteristics of BTC in Korea with global data to inform future research and clinical practice.
Citations
Citations to this article as recorded by
Global, regional, and national burden of gallbladder and biliary tract cancer among adults aged 55 years and older, 2010–2021 Mingjuan Li, Jiaguang Li, Shuangjiang Li, Minmin Zhang, Shuang Li, Jiahui Zhao, Tao Gan, Min Wu, Shunwen Luo, Yunying Liang, Qiuyun Li, Guangdong Pan, Jianqing Yang, Guoqing Ouyang Frontiers in Nutrition.2025;[Epub] CrossRef
Changing patterns of cholangiocarcinoma and gallbladder cancer: A regional perspective from Northeastern Italy Duygu Kirkik, Ayse Naz Ozabaci, Sevgi Kalkanli Tas World Journal of Gastrointestinal Oncology.2025;[Epub] CrossRef
Gallbladder large cell neuroendocrine carcinoma in a patient with multiple myeloma: a case report and literature review Yuhan Yin, Xinjie Dai, Dan Li, Xinyu Yang, Nan Ming, Kang Xiang, Dengding Wu, Kong Ai, Rucheng Yao, Jun Hu Frontiers in Oncology.2025;[Epub] CrossRef
A 53-year-old female patient visited our hospital complaining of intermittent right upper quadrant pain for 6 months. Computed tomography (CT) scan revealed an irregular shaped tumor at segment 4 of the liver with biliary tumor thrombi extending into the common bile duct. Percutaneous transhepatic biliary drainage was done for decompression of bile duct dilatation. The patient underwent 6 sessions of transarterial chemoembolization (TACE). Partial response was obtained shortly after TACE. However, regrowth of intraductal tumor resulted in an obstructive jaundice. After a slight decompression of the obstructive jaundice, the patient underwent TACE. Jaundice temporarily worsened following the TACE, but improved, and follow-up CT demonstrated some shrinkage of the intraductal mass. This case indicates that obstructive-type jaundice may not be a contraindication for TACE, and aggressive TACE may improve prognoses of patients with hepatocellular carcinoma and biliary tumor thrombi.