Recent updates to the National Comprehensive Cancer Network (NCCN) guidelines for the evaluation and management of patients with biliary tract cancers (BTCs) (gallbladder cancer, intrahepatic cholangiocarcinoma, and extrahepatic cholangiocarcinoma) were published in July 2023 in the Journal of the NCCN (JNCCN). Select recommendations for molecular testing and for systemic therapy are outlined below.
The NCCN recommends comprehensive molecular profiling for those with unresectable or metastatic BTCs, such as NTRK gene fusion, microsatellite instability-high/mismatch repair deficient (MSI-H/dMMR) status, tumor mutational burden-high (TMB-H) status, BRAF V600E mutation, FGFR2 fusion or rearrangement, IDH1 mutation, HER2 (ERBB2) overexpression and/or amplification, and/or RET gene fusion.
Consider cell-free DNA (cfDNA) to identify gene mutations.
Germline testing in hepatobiliary cancers
Insufficient evidence exists for definitive recommendations about specific criteria to guide genetic risk assessment in hepatobiliary cancers or for universal germline testing in these tumors.
Primary treatment for unresectable and metastatic disease
Preferred regimen: Durvalumab plus gemcitabine plus cisplatin. This regimen is also recommended as an option for those with recurrent disease that develops longer than 6 months postoperatively with curative intent as well as longer than 6 months post adjuvant therapy completion.
Subsequent-line therapy for BTCs that progress
Preferred regimen: Folinic acid plus fluorouracil plus oxaliplatin (FOLFOX)
For more Clinical Practice Guidelines, please go to Guidelines.
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Cite this: Biliary Tract Cancers Clinical Practice Guidelines (NCCN, 2023) - Medscape - Aug 16, 2023.