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.
目前对儿童肝细胞腺瘤(HCA)的认识和分类主要基于成人数据。HCA在儿童中较为罕见,且与成人不同,常伴随综合征或异常背景肝脏出现。将成人分类标准应用于儿童肿瘤时,常导致部分病变被归为“无法分类”类型。尽管通常被视为良性病变,少数病例可能表现出非典型特征,且携带β-连环蛋白突变的肿瘤存在恶变风险。小型病灶可进行随访监测,而较大病灶(>5.0厘米)因症状或出血/破裂风险需手术切除。治疗方案需综合考量性别、年龄、基础肝病、多灶性、病灶大小、组织学亚型及是否存在突变等因素。本文综述了儿童HCA的相关数据,并重点介绍了我们在诊断和治疗方面的实践经验。
Pediatric Hepatocellular Adenomas: What Is Known and What Is New?