Cholangiocarcinoma

Cholangiocarcinoma (CCA) is most strongly associated with risk factors characterized by chronic inflammatory states. However, most CCAs are sporadic, with no identifiable risk factors. In this chapter, we provide a detailed review of the current evaluation and management of CCA, with a particular emphasis on the imaging features. Imaging features of CCA are dependent on the location, size, morphologic growth pattern, and degree of intratumoral fibrosis, necrosis, or mucin content. Ultrasound (US), endoscopic US, computed tomography (CT), magnetic resonance imaging (MRI)/ magnetic resonance cholangiopancreatography (MRCP), and positron emission tomography (PET) are the imaging modalities typically used to diagnose and monitor CCA. Histologically, CCAs are primarily adenocarcinomas. Generally, the more centrally located perihilar or distal CCAs are more likely to have well-formed glands lined by columnar epithelial cells with mucin production, whereas CCAs located at the liver periphery are more likely to grow as irregular, anastomosing tubular structures lined by low cuboidal cells that do not produce mucin. Treatment options for CCA include surgical resection, local image-guided percutaneous thermal and nonthermal ablative therapies, locoregional therapies such as transarterial chemoembolization, transarterial radioembolization and stereotactic body radiation therapy, and systemic therapies, which are increasingly targeted to characteristic oncogenic mutations or fusion proteins.