Cholangiocarcinoma (CCA) is a rare tumor arising from the epithelium of the intrahepatic or the extrahepatic bile ducts. It is rarely diagnosed before 40 years of age except in patients with primary sclerosing cholangitis. CCA is usually clinically silent until the tumor obstructs the bile ducts. Carbohydrate antigen 19-9 is the most commonly used tumor marker, and magnetic resonance cholangiopancreatography is the best available imaging modality for CCA. Endoscopic retrograde cholangiopancreatography and cholangioscopy allow tissue acquisition. Positron emission tomography may play a role in identifying occult metastases. Tissue diagnosis is obtained by brush cytology or bile duct biopsy.

Cholangiocarcinoma (CCA) is a tumor of the bile ducts that usually presents with biliary obstruction and has a poor prognosis. The treatment of CCA is challenging as the tumor is usually diagnosed late and the treatments are not very effective except when complete surgical resection is possible. In carefully selected patients, liver transplant can be a curative therapy. In the majority of cases, complete surgical resection is not possible and palliation is the mainstay of treatment. Stenting, using plastic or metallic stents, allows for biliary drainage. Photodynamic therapy plays a role in palliation and might play a role in adjuvant or neoadjuvant therapy. While radiation and chemotherapy can be beneficial, newer ablative techniques and targeted chemotherapies are promising.

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