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Ref Type

Journal Article

PMID

(39864891)

Authors

Vogel A, Ducreux M, ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org

Title

ESMO Clinical Practice Guideline interim update on the management of biliary tract cancer.

Journal	Vol	Issue	Date	Pages	Journal Short Title
ESMO open	10	1	2025 Jan	104003	ESMO Open

URL

Abstract Text

• This ESMO Clinical Practice Guideline update addresses new developments in the management of biliary tract cancer. • Recommendations are given for first-line treatment with immune checkpoint inhibitors. • Key recommendations are also provided for second-line treatment with targeted therapies. • The update also covers the latest developments in molecular testing and intra-arterial therapies. • A management algorithm for early-stage, locally advanced and advanced/metastatic disease is provided.

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Molecular Profile	Indication/Tumor Type	Response Type	Therapy Name	Approval Status	Evidence Type	Efficacy Evidence	References
BRAF V600E	biliary tract cancer	sensitive	Dabrafenib + Trametinib	Guideline	Actionable	Tafinlar (dabrafenib) in combination with Mekinist (trametinib) is included in guidelines as second or later-line therapy for patients with biliary tract cancer harboring BRAF V600E (PMID: 39864891; ESMO.org).	detail... 39864891
FGFR2 fusion	cholangiocarcinoma	sensitive	Pemigatinib	Guideline	Actionable	Pemazyre (pemigatinib) is included in guidelines as second or later-line therapy for patients with cholangiocarcinoma harboring FGFR2 fusions or rearrangements (PMID: 39864891; ESMO.org).	39864891 detail...
FGFR2 fusion	cholangiocarcinoma	sensitive	Futibatinib	Guideline	Actionable	Lytgobi (futibatinib) is included in guidelines as second or later-line therapy for patients with cholangiocarcinoma harboring FGFR2 fusions and rearrangements (PMID: 39864891; ESMO.org).	detail... 39864891
FGFR2 rearrange	cholangiocarcinoma	sensitive	Futibatinib	Guideline	Actionable	Lytgobi (futibatinib) is included in guidelines as second or later-line therapy for patients with cholangiocarcinoma harboring FGFR2 fusions and rearrangements (PMID: 39864891; ESMO.org).	detail... 39864891
FGFR2 rearrange	cholangiocarcinoma	sensitive	Pemigatinib	Guideline	Actionable	Pemazyre (pemigatinib) is included in guidelines as second or later-line therapy for patients with cholangiocarcinoma harboring FGFR2 fusions or rearrangements (PMID: 39864891; ESMO.org).	detail... 39864891
IDH1 mutant	cholangiocarcinoma	sensitive	Ivosidenib	Guideline	Actionable	Tibsovo (ivosidenib) is included in guidelines as second or later-line therapy for patients with cholangiocarcinoma harboring IDH1 mutations (PMID: 39864891; ESMO.org).	detail... 39864891
MLH1 negative	biliary tract cancer	sensitive	Pembrolizumab	Guideline	Actionable	Keytruda (pembrolizumab) is included in guidelines as second or late-line therapy for biliary tract cancer patients with high microsatellite instability (MSI-H) or deficient mismatch repair (dMMR, often defined by the loss of MLH1, PMS2, MSH2, or MSH6 expression by IHC) who have not received first-line immunotherapy (PMID: 39864891; ESMO.org).	detail... 39864891
MSH6 negative	biliary tract cancer	sensitive	Pembrolizumab	Guideline	Actionable	Keytruda (pembrolizumab) is included in guidelines as second or late-line therapy for biliary tract cancer patients with high microsatellite instability (MSI-H) or deficient mismatch repair (dMMR, often defined by the loss of MLH1, PMS2, MSH2, or MSH6 expression by IHC) who have not received first-line immunotherapy (PMID: 39864891; ESMO.org).	detail... 39864891
PALB2 inact mut	biliary tract cancer	sensitive	Rucaparib	Guideline	Actionable	Rubraca (rucaparib) is included in guidelines as second or later-line therapy for patients with biliary tract cancer harboring PALB2 mutations (PMID: 39864891; ESMO.org).	39864891 detail...
RET fusion	biliary tract cancer	sensitive	Selpercatinib	Guideline	Actionable	Retevmo (selpercatinib) is included in guidelines as second or later-line therapy for patients with biliary tract cancer harboring RET fusions (PMID: 39864891; ESMO.org).	detail... 39864891

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