UCLA Radiological Sciences Proceedings

Alagille Syndrome is a multisystem genetic disorder characterized by paucity of intrahepatic bile ducts, which can lead to early cirrhosis and an increase in the risk of developing focal liver lesions, including hepatocellular carcinoma. Therefore, patients with Alagille syndrome are often followed with routine imaging surveillance. However, it can be challenging to distinguish incidentally found benign liver tumors from their malignant counterparts in this subset of patients. Here we present the case of a giant regenerative hepatic nodule in a 17-year-old woman with Alagille syndrome. The case is presented with associated imaging studies that favored a diagnosis of benign hyperplasia, confirmed by the results of histologic examination.