Pediatric Hepatocellular Adenomas: What Is Known and What Is New?

SIMPLE SUMMARY: Pediatric hepatocellular adenoma (HCA) is a rare tumor and often observed in abnormal livers. Despite advances in adults, there is little progress in understanding the origin and management of pediatric HCAs. Evidence of multifocality, malignant transformation and rupture in a subset of patients warrant a thorough understanding of this rare entity to guide optimum management. We present a concise review of the current literature on HCA, with a focus and comparative assessment on pediatric patients. ABSTRACT: Current understanding and classification of pediatric hepatocellular adenomas (HCA) are largely based on adult data. HCAs are rare in children and, unlike in adults, are often seen in the context of syndromes or abnormal background liver. Attempts to apply the adult classification to pediatric tumors have led to several “unclassifiable” lesions. Although typically considered benign, few can show atypical features and those with beta-catenin mutations have a risk for malignant transformation. Small lesions can be monitored while larger (>5.0 cm) lesions are excised due to symptoms or risk of bleeding/rupture, etc. Management depends on gender, age, underlying liver disease, multifocality, size of lesion, histologic subtype and presence of mutation, if any. In this review, we summarize the data on pediatric HCAs and highlight our experience with their diagnosis and management.