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Review
. 2022 Feb;7(1):100378.
doi: 10.1016/j.esmoop.2021.100378. Epub 2022 Jan 13.

How I treat biliary tract cancer

A Lamarca  1 , J Edeline  2 , L Goyal  3
Affiliations
Review

How I treat biliary tract cancer

A Lamarca et al. ESMO Open. 2022 Feb.

Abstract

Management of biliary tract cancers (BTCs) is rapidly evolving. Curative management relies on surgical resection followed by adjuvant capecitabine for cholangiocarcinoma and gallbladder cancers. Unfortunately relapse rate remains high, and better adjuvant strategies are urgently required. A majority of patients are diagnosed with advanced disease, when chemotherapy with cisplatin and gemcitabine followed by second-line 5-FU and oxaliplatin /irinotecan is the cornerstone of treatment for most patients in the absence of targetable alterations. Targeted therapies, including therapies for tumours with fibroblast growth factor receptor-2 (FGFR-2) fusions, isocitrate dehydrogenase-1 (IDH-1) mutations, B-Raf proto-oncogene serine/threonine kinase (BRAF) V600E mutations, neurotrophic tyrosine receptor kinase (NTRK) fusions, Human epidermal growth factor-2 (HER-2) amplifications, and/or microsatellite instability are rapidly changing the treatment paradigm for many patients with advanced BTC, especially for patients with intrahepatic cholangiocarcinoma. Because of this, molecular profiling should be considered early on patients pathway to allow adequate planning of therapy. Ongoing research is likely to clarify the role of immunotherapy, liver-directed therapy, and liver transplant for BTCs in the future.

Keywords: ampullary cancer; biliary tract cancer; chemotherapy; cholangiocarcinoma; gallbladder cancer; surgery; targeted; treatment.

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Conflict of interest statement

Disclosure AL has received travel and educational support from Ipsen, Pfizer, Bayer, AAA, Sirtex, Novartis, Mylan, and Delcath; speaker honoraria from Merck, Pfizer, Ipsen, Incyte, AAA, QED, Servier, and EISAI; advisory honoraria from EISAI, Nutricia Ipsen, QED, Roche, Servier, and Boston Scientific; is a member of the Knowledge Network and NETConnect Initiatives funded by Ipsen. JE has received honoraria from Servier, Incyte, Basilea, Boston Scientific, MSD, AstraZeneca, Roche, Eisai, Ipsen, and Bayer. LG reports research funding (to the institution) from Adaptimmune, Bayer, Merck, MacroGenics, Genentech, Novartis, Incyte, Loxo Oncology, Relay Therapeutics, QED, Taiho Oncology, Leap Therapeutics, Bristol Myers Squibb, NuCana, and Servier; honoraria (to self) for serving on scientific advisory committees or as a consultant from Alentis Therapeutics AG, Black Diamond, Basilea, Genentech, Exelixis, H3Biomedicine, Incyte Corporation, QED Therapeutics, Sirtex Medical Ltd, The Servier Group, Sirtex, and Taiho Oncology and participation on data safety monitoring boards for AstraZeneca.

Figures

Figure 1
Figure 1
Biliary tract cancers (BTCs) are a heterogeneous group of malignancies. 5-FU, 5-fluorouracil; eCCA, extrahepatic cholangiocarcinoma; FOLFOX, 5-fluorouracil and oxaliplatin; FOLFIRI, 5-fluorouracil and irinotecan; iCCA, intrahepatic cholangiocarcinoma; nal-IRI, nanoliposomal irinotecan; PEI, pancreatic exocrine insufficiency. Adapted from Lamarca A, Frizziero M, McNamara MG, Valle JW. Clinical and translational research challenges in biliary tract cancers. Curr Med Chem. 2020;27(29):4756-4777.
Figure 2
Figure 2
Biliary tract cancers (BTCs): Diagnosis and management algorithm. BRAF, B-Raf proto-oncogene serine/threonine kinase; CCA, cholangiocarcinoma; dMMR, deficient DNA mismatch repair; eCCA, extrahepatic cholangiocarcinoma; ECOG PS, Eastern Cooperative Oncology Group performance status; FGFR, fibroblast growth factor receptor; GBC, gallbladder cancer; HER-2, human epidermal growth factor-2; iCCA, intrahepatic cholangiocarcinoma; IDH-1, isocitrate dehydrogenase-1; MEK, mitogen-activated extracellular signal-regulated kinase; MSI, microsatellite instability; nal-IRI, nanoliposomal irinotecan; NTRK, neurotrophic tyrosine receptor kinase; TMB, tumour mutational burden; TRK, tyrosine receptor kinase. Adapted from.
See this image and copyright information in PMC

References

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