UCLA Radiological Sciences Proceedings

Alagille Syndrome is a multisystem genetic disorder characterized by paucity of interlobular bile ducts, which can lead to early cirrhosis and increase the risk of developing focal liver lesions including hepatocellular carcinoma. As a result, routine imaging surveillance is often performed in these patients.  However, it can be challenging to distinguishing 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 female with Alagille syndrome, and the associated imaging characteristics that favored a benign diagnosis, with benign pathology confirmed by image-guided core needle biopsy.