Department of Laboratories, Seattle Children's Hospital.
Departments of Laboratory Medicine and Pathology.
Am J Surg Pathol. 2021 Dec 1;45(12):1641-1647. doi: 10.1097/PAS.0000000000001763.
Hepatocellular adenomas are rare in children. A large study focused on pediatric patients has not been undertaken. A natural language search was performed at 5 institutions for hepatocellular adenomas in patients younger than 21 years old. Clinical characteristics as well as immunohistochemical staining profile was reviewed and adenomas subtyped per standard classification. Patients were divided into prepubescent and postpubescent age group. Thirty-one patients were included. Eleven (35%) were male and 10 (32%) were prepubescent. Fifteen (54%) of 28 patients with known clinical histories had adenomas associated with a syndrome. The percentage of the different adenoma subtypes was: 16% β-catenin activated, 10% combined inflammatory and β-catenin activated, 29% HFN1α-inactivated, 35% inflammatory, and 10% unclassified subtype by immunohistochemical staining. Interestingly 53% of patients with syndromes were male, while 85% of patients in the nonsyndromic group were female. The total number of β-catenin activated tumors was greater in the syndromic group (5/15, 33%) and prepubescent group (5/10, 50%) than in the nonsyndromic group (2/13, 16%) and postpubescent group (3/21, 14%), P=0.4 and 0.07, respectively. Inflammatory type adenoma was more frequent in the postpubescent (10/21, 48%) than in the prepubescent group (1/10, 10%), P=0.06, trending toward significance. Pediatric patients with hepatocellular adenomas frequently have syndromes, especially in the prepubescent group. In patients with syndromes a greater percentage of adenomas were β-catenin activated. In patients without a known syndrome the distribution of hepatocellular adenoma subtypes appears similar to adults.
儿童肝细胞腺瘤较为罕见。目前尚未开展针对儿科患者的大型研究。在 5 家机构中，使用自然语言搜索了年龄小于 21 岁的肝细胞腺瘤患者。对临床特征及免疫组织化学染色特征进行了回顾，并按照标准分类对腺瘤进行了亚型划分。将患者分为青春期前和青春期后两组。共纳入 31 例患者。其中 11 例（35%）为男性，10 例（32%）为青春期前。28 例已知临床病史的患者中，有 15 例（54%）的腺瘤与综合征相关。不同腺瘤亚型的比例为：β-连环蛋白激活型 16%，炎症合并β-连环蛋白激活型 10%，HFN1α失活型 29%，炎症型 35%，免疫组织化学染色未分类型 10%。有趣的是，综合征组中男性占 53%（5/15），而无综合征组中女性占 85%（13/15）。综合征组（5/15，33%）和青春期前组（5/10，50%）β-连环蛋白激活型肿瘤总数多于无综合征组（2/13，16%）和青春期后组（3/21，14%），P=0.4 和 0.07。炎症型腺瘤在青春期后组（10/21，48%）比青春期前组（1/10，10%）更常见，P=0.06，有显著趋势。患有肝细胞腺瘤的儿科患者常伴有综合征，尤其是在青春期前。在有综合征的患者中，更多的腺瘤为β-连环蛋白激活型。在无已知综合征的患者中，肝细胞腺瘤亚型的分布与成人相似。
