Early detection and treatment of cholangiocarcinoma

Key Points. 1. The major identified risk factor for the development of cholangiocarcinoma in Western countries is primary sclerosing cholangitis (PSC). 2. The diagnosis of cholangiocarcinoma in PSC requires a high index of suspicion because endoscopic brush cytology and/or biopsies and imaging studies are often negative for malignancy. Positron emission tomography is a promising imaging modality for the diagnosis of cholangiocarcinoma, even in patients with PSC. 3. A serum carbohydrate antigen 19-9 value greater than 100 U/mL has a sensitivity and specificity for cholangiocarcinoma of approximately 75% and 80%, respectively. 4. Liver transplantation is a viable therapeutic option for selected patients with early-stage cholangiocarcinoma. Outcomes are optimized by using preoperative radiation and chemotherapy and ensuring the absence of metastases by an exploratory laparotomy.