Cholangiocarcinoma (CCA) is an aggressive malignancy arising from the biliary epithelium. It may occur at any location along the biliary tree with the perihilar area being the most common. Prognosis is poor with 5-year overall survival at less than 10%, typically due to unresectable disease at presentation. Radical surgical resection with clear margins offers a chance of cure in patients with resectable tumours, but is frequently not possible due to locally advanced disease. On the other hand, orthotopic liver transplantation (LT) allows for a radical and potentially curative resection for these patients, but has been historically controversial due to the limited supply of donor grafts and previously poor outcomes. In patients with perihilar CCA, within specific criteria and following the implementation of a protocol combining neoadjuvant chemoradiation and LT, excellent results have been achieved in the last decades, resulting in its increasing acceptance as an indication for LT and the standard of care in several centres with significant experience. However, in intrahepatic CCA, the role of LT remains controversial and owing to dismal previous results it is not an accepted indication. Nevertheless, more recent studies have demonstrated favourable results with LT in early intrahepatic CCA, indicating that, under defined criteria, its role may increase in the future. This review highlights the history and contemporary advances of LT in CCA, with particular focus on the improving outcomes of LT in intrahepatic and perihilar CCA and future perspectives.
Intrahepatic cholangiocarcinoma (iCCA) is an aggressive malignancy with an increasing incidence worldwide and poor prognosis, despite several advances and continuous efforts to develop effective treatments. Complete surgical resection is the mainstay of treatment and offers a potentially curative option, but is only possible in less than a third of patients, owing to advanced disease. Chemotherapy is a well-established treatment in the adjuvant and palliative setting, however, confers limited benefit. Conventional radiotherapy is challenging due to local toxicity. With recent advances in stereotactic ablative radiotherapy (SABR), it is now possible to focus ablative beams of radiotherapy precisely aimed at tumours to minimise damage to surrounding viscera. This review details the history, technical background and application of SABR to iCCA, with directions for future research suggested.
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