Systemic chemotherapy for advanced biliary tract cancers: present and future

Biliary tract cancers, with fewer than 2,000 new cases per year in France, must be regarded as orphan tumors. There is currently no evidence of the benefit of neoadjuvant or adjuvant chemotherapy and/or radiotherapy in resectable biliary tract cancers. In unresectable disease, best supportive care is the mainstay of treatment, especially effective biliary drainage. Gemcitabinecisplatin combination (GEMCIS regimen) is the first standard for first-line palliative chemotherapy in patients with advanced biliary tract cancer and correct performance status. Despite the absence of randomized controlled trials comparing GEMCIS and gemcitabine-oxaliplatin (GEMOX) regimens, the latter represents an interesting option. Currently, no data allow to define a standard for second-line and further therapies. Future therapeutic advances will likely come from the combination of targeted agents with chemotherapy, with selection of patients on tumor oncogenic alterations of interest.