Cholangiocarcinoma

Cholangiocarcinoma (CCA) is a rare primary hepatic neoplasm that arises from malignant transformation of the biliary epithelium. Chronic inflammation of the biliary tree as in primary sclerosing cholangitis (PSC), is a risk factor for the development of CCA. Hilar CCA usually presents with obstructive jaundice. Surgical resection and liver transplantation in carefully selected patients with extrahepatic CCA are the only curative modalities currently available. Unfortunately, most tumors are unresectable at the time of presentation. Biliary stenting, chemotherapy, radiation therapy, and photodynamic therapy (PDT) are palliative therapies for CCA. Liver transplantation following neoadjuvant therapy has emerged as an effective therapy for patients with unresectable hilar cholangiocarcinoma or hilar cholangiocarcinoma arising in PSC. Pre-transplant evaluation includes an extensive evaluation for metastatic disease. Pre-liver transplant care includes managing side effects of neoadjuvant therapy, maintaining adequate biliary drainage and promptly treating episodes of acute cholangitis. Post-liver transplant care is similar to that for transplantation for other diseases except for monitoring for recurrent CCA and vigilance for the development of late vascular complications due to neoadjuvant therapy.