Liver transplantation

Hilar cholangiocarcinoma (CCA) is a rare but devastating malignancy that presents late and is notoriously difficult to diagnose due to lack of effective screening tests. Treatment of hilar CCA is similarly challenging because of the lack of effective adjuvant therapy, aggressive infiltrative and longitudinal growth pattern of CCA, and location of the tumor in close proximity to vital structures. Historically, the usual management for these patients was palliative despite the absence of distant metastasis at initial presentation and the prognosis has been poor. The development and evolution of liver surgery including orthotopic liver transplantation (OLT) over the past four decades has significantly improved the surgical management of CCA. A complete extirpation of tumor including all microscopically detectable disease (R0 resection) offers the only possibility of long-term survival in patients with CCA. Unfortunately, many patients present with unresctable hilar CCA due to the presence of advanced liver disease and/or tumor extension to hepatic parenchyma and/or major vessels (hepatic artery and portal vein) of both right and left hemilivers, metastasis to regional lymph nodes or insufficient future liver remnant volume (Table 25.1).