Cholangiocarcinoma
ICD-10 C22.1 · ICD-11 2C12.0Y

Treatment of Unresectable or Metastatic Extrahepatic Cholangiocarcinoma with No Targetable Molecular Alteration

Patients with extrahepatic cholangiocarcinoma (eCCA) whose disease is unresectable or metastatic, and in whom molecular profiling identifies no actionable alteration, represent a defined clinical population with a specific evidence-based first-line strategy.

Unresectable or metastatic extrahepatic cholangiocarcinoma confirmed on molecular profiling to harbour no targetable molecular alteration.

Current evidence supports a first-line combination of platinum-based chemotherapy with immune checkpoint inhibition. Molecular profiling is integral to the initial workup to confirm the absence of actionable alterations and to support clinical trial eligibility. The complete regimen, individualisation criteria, and subsequent-line options are detailed in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jhep.2025.03.007

Gemcitabine and cisplatin in combination with either durvalumab or pembrolizumab should be considered standard of care for the first-line systemic treatment of patients with unresectable or metastatic eCCA (LoE 2, strong recommendation, consensus).

Patients with unresectable or metastatic eCCA should receive molecular profiling to identify and therapeutically address actionable alterations and to support inclusion into clinical trials (LoE 2, strong recommendation, strong consensus).

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