Diagnosis, Staging, and Treatment of Cholangiocarcinoma.

Attention has to be focused on earlier detection of cholangiocarcinoma, especially in high-risk patients such as those with primary sclerosing cholangitis (PSC). Enhanced techniques for diagnosing this disease include imaging studies such as positron emission tomography (PET) scanning, and cytologic analysis including digital image analysis (DIA) and fluorescent in situ hybridization (FISH). Magnetic resonance imaging and endoscopic ultrasonography have also improved and simplified preoperative staging. Despite recent advances in the endoscopic management of cholangiocarcinoma, this neoplastic disease is still fatal in the majority of patients. Surgical resection or liver transplantation offers the only possibility for curing this disease in its early stages. However, most patients present with advanced disease that is not amenable to such therapy. For those patients presenting with unresectable carcinoma above the cystic duct without intrahepatic or extrahepatic metastases, orthotopic liver transplantation combined with preoperative irradiation and chemotherapy is available and demonstrates improved survival on the basis of a recent study conducted at the Mayo Clinic. In the future, chemopreventive strategies aimed at blocking the links between inflammation (eg, nitric oxide synthase and cylcooxygenase 2 inhibitors) and carcinogenesis may help prevent this often fatal disease in high-risk patients (eg, patients with PSC).