Thapar Manish, Grapp Oleg, Fisher Constantine
Department of Medicine, Drexel University College of Medicine, Philadelphia, PA, 19142, USA,
Curr Gastroenterol Rep. 2015 Mar;17(3):12. doi: 10.1007/s11894-015-0434-4.
Hepatic adenomatosis (HeAs) is a rare clinical entity defined by the presence of 10 or more hepatic adenomas (HA) within the background of an otherwise normal liver parenchyma, in the absence of glycogen storage disease or anabolic steroid use. HA is a benign tumor associated with oral contraceptive use. Recent advances in pathogenesis and classification of HA have questioned the distinction between these two diseases. HA are currently classified into four different subtypes with genotypic and phenotypic correlation: HNF-1a inactivated HA, B-catenin activated HA, inflammatory HA, and undetermined subtype. The clinical presentation of HA depends on the lesion size and the subtype. MRI using hepatospecific contrast agents is helpful in diagnosing the most common subtypes. When diagnosis is uncertain, biopsy with immunohistochemistry is used to diagnose and classify the lesions. Management is governed by the molecular subtype and tumor size. Pregnancy is not routinely discouraged but management is individualized.
肝腺瘤病（HeAs）是一种罕见的临床病症，其定义为在肝脏实质正常的背景下存在10个或更多的肝腺瘤（HA），且不存在糖原贮积病或使用合成代谢类固醇的情况。HA是一种与口服避孕药使用相关的良性肿瘤。HA发病机制和分类方面的最新进展对这两种疾病之间的区别提出了质疑。目前，HA被分为四种不同的亚型，具有基因型和表型相关性：HNF-1a失活型HA、β-连环蛋白激活型HA、炎症型HA和未确定亚型。HA的临床表现取决于病变大小和亚型。使用肝脏特异性造影剂的MRI有助于诊断最常见的亚型。当诊断不确定时，采用免疫组织化学活检来诊断和分类病变。治疗方案取决于分子亚型和肿瘤大小。通常不常规劝阻妊娠，但治疗方案需个体化。
