American Radium Society (ARS) Appropriate Use Criteria (AUC) for Extrahepatic Cholangiocarcinoma

Although uncommon, extrahepatic cholangiocarcinoma (EHCC) is a deadly malignancy, and the treatment approaches remain controversial. While surgery remains the only cure, few patients are candidates for resection up front, and there are high rates of both local and distant failure following resection. Herein, we systematically review the available evidence regarding treatment approaches for patients with EHCC, including surgery, radiation, and chemotherapy. The evidence regarding treatment outcomes was assessed using the Population, Intervention, Comparator, Outcome, and Study design (PICOS) framework. A summary of recommendations based on the available literature is outlined for specific clinical scenarios encountered by providers in the clinic to guide the management of these patients.

Automated summary

Although rare, extrahepatic cholangiocarcinoma (EHCC) is a highly lethal malignancy with controversial treatment options. Surgery is the only potentially curative treatment, but only a few patients are eligible for upfront resection and high rates of both local and distant recurrence are observed post-resection. In this review, we systematically examine the available evidence on treatment approaches for EHCC patients, including surgery, radiation, and chemotherapy, using the Population, Intervention, Comparator, Outcome, and Study design (PICOS) framework. We provide a summary of recommendations based on the existing literature to guide clinical management of EHCC patients.