Cholangiocarcinoma: a site-specific update on the current state of surgical management and multi-modality therapy

Biliary tract cancers (BTC) are rare, heterogeneous malignancies that include cholangiocarcinoma and gallbladder cancer (GBC). Cholangiocarcinoma subtypes differ by anatomic location and molecular profile. Currently, resection with lymphadenectomy is the only curative treatment of locally advanced cholangiocarcinoma. Given the high risk of recurrence, multi-modality therapy spanning surgery, chemotherapy, and radiation therapy should be considered. Current data is discordant and there is limited prospective data to support an optimal treatment regimen, though recent studies have demonstrated the utility of adjuvant chemotherapy and chemoradiation in specific settings and patient populations. There is a potential role for neoadjuvant chemotherapy in patients with resectable disease or chemoradiation in select patients with unresectable, locally advanced disease. Randomized clinical trials are necessary to establish the effectiveness of therapies specific to disease sites, especially with the emerging role of immunotherapy and targeted therapy to actionable mutations.