BACKGROUND Hepatocellular adenoma (HCA) in the pediatric population is very rare and there are only limited studies, especially with molecular characterization of the tumors. Main HCA subtypes recognized in the current WHO classification include HNF1A-inactivated HCA (H-HCA), inflammatory HCA (IHCA), β-catenin-activated HCA (b-HCA), and β-catenin-activated IHCA (b-IHCA) and sonic hedgehog HCA (shHCA) is reported as an emerging subtype. METHODS Clinical history, pathological information, and molecular studies for a series of 2 cases of pediatric HCA were reviewed. RESULTS Case 1 was a b-HCA characterized by somatic CTNNB1 S45 mutation in a 11-year-old male with Abernethy malformation. Case 2 was a H-HCA characterized by germline HNF1A variant (c.526+1G>A) in a 15-year-old male associated with maturity-onset diabetes of the young type 3 (MODY3). CONCLUSION Our findings highlight the rarity of these 2 cases associated with adenomatosis, and the contribution of molecular/genetic analysis for proper sub-typing, prognosis and family surveillance.

中文翻译：

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肝细胞腺瘤：2 例报告强调了儿科人群表型-基因型相关性的相关性。

背景 儿科人群中的肝细胞腺瘤（HCA）非常罕见，而且研究也很有限，特别是在肿瘤的分子特征方面。目前WHO分类中认可的主要HCA亚型包括HNF1A灭活HCA（H-HCA）、炎性HCA（IHCA）、β-连环蛋白激活HCA（b-HCA）和β-连环蛋白激活IHCA（b-IHCA）据报道，音猬因子 HCA (shHCA) 是一种新兴亚型。方法 对 2 例儿童 HCA 病例的临床病史、病理学信息和分子研究进行回顾。结果 病例 1 是一名患有 Abernethy 畸形的 11 岁男性，其特征为体细胞 CTNNB1 S45 突变。病例 2 是一名 15 岁男性的 H-HCA，其特征为种系 HNF1A 变异 (c.526+1G>A)，与青少年 3 型成年发病糖尿病 (MODY3) 相关。结论 我们的研究结果强调了这 2 例与腺瘤病相关的病例的罕见性，以及分子/遗传分析对正确分型、预后和家庭监测的贡献。