Published online Nov 15, 2021. doi: 10.4251/wjgo.v13.i11.1696
Peer-review started: March 16, 2021
First decision: May 3, 2021
Revised: May 30, 2021
Accepted: September 14, 2021
Article in press: September 14, 2021
Published online: November 15, 2021
Processing time: 240 Days and 14.7 Hours
Cancer of the biliary confluence also known as hilar cholangiocarcinoma (HC) or Klatskin tumor, is a rare type of neoplastic disease constituting approximately 40%-60% of intrahepatic malignancies, and 2% of all cancers. The prognosis is extremely poor and the majority of Klatskin tumors are deemed unresectable upon diagnosis. Most patients with unresectable bile duct cancer die within the first year after diagnosis, due to hepatic failure, and/or infectious complications secondary to biliary obstruction. Curative treatments include surgical resection and liver transplantation in highly selected patients. Nevertheless, very few patients are eligible for surgery or transplant at the time of diagnosis. For patients with unresectable HC, radiotherapy, chemotherapy, photodynamic therapy, and liver-directed minimally invasive procedures such as percutaneous image-guided ablation and intra-arterial chemoembolization are recommended treatment options. This review focuses on currently available treatment options for unresectable HC and discusses future perspectives that could optimize outcomes.
Core Tip: Most patients with hilar cholangiocarcinoma (HC) are not candidates for surgery or liver transplant at the time of diagnosis. Recently, several options for the management of unresectable HC have emerged and due to the complexity of this disease, a multi-disciplinary approach with multimodal treatment is recommended, including surgery, medical oncology, radiation oncology, diagnostic radiology, interventional radiology, gastroenterology, and pathology. Recent data suggest an improvement in overall survival, better response rates, and tumor control in patients with unresectable HC can be achieved by combining chemotherapy and minimal invasive ablatives strategies.