Treatment of advanced disease

The majority of cases of cholangiocarcinoma present as inoperable (unresectable) disease^[65] in which case patients are generally treated with palliative chemotherapy, with or without radiotherapy. Chemotherapy has been shown in a randomized controlled trial to improve quality of life and extend survival in patients with inoperable cholangiocarcinoma.^[66] There is no single chemotherapy regimen which is universally used, and enrollment in clinical trials is often recommended when possible.^[64] Chemotherapy agents used to treat cholangiocarcinoma include 5-fluorouracil with leucovorin,^[67] gemcitabine as a single agent,^[68] or gemcitabine plus cisplatin,^[69] irinotecan,^[70] or capecitabine.^[71] A small pilot study suggested possible benefit from the tyrosine kinase inhibitor erlotinib in patients with advanced cholangiocarcinoma.^[72]

Photodynamic therapy, an experimental approach in which patients are injected with a light-sensitizing agent and light is then applied endoscopically directly to the tumor, has shown promising results compared to supportive care in two small randomized controlled trials. However, its ultimate role in the management of cholangiocarcinoma is unclear at present.^[73][74]