Straub Beate K, Müller Lukas, Duret Diane S, Tóth Marcell, Mittler Jens, Schirmacher Peter
Institut für Pathologie, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland.
Klinik und Poliklinik für Diagnostische und Interventionelle Radiologie, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland.
Pathologie (Heidelb). 2025 Jun 5. doi: 10.1007/s00292-025-01444-8.
Hepatocellular adenomas (HCAs) are rare benign hepatocellular neoplasia that typically occur in a non-cirrhotic liver in young women on contraceptive therapy or in metabolic liver disease. HCAs may be subtyped radiologically and histologically, controlled under discontinuation of contraceptives, and resected in the case of malignant transformation potential or an HCA size of more than 5 cm.Histologically, HCAs present as well-differentiated hepatocellular neoplasms, which in contrast to focal nodular hyperplasia (FNH) lack portal tract-like structures. Prognostically relevant morphomolecular HCA subtypes have been described. HNF1A-inactivated HCAs often show a prominent steatosis and loss of L‑FABP. Inflammatory HCAs (IHCAs) are characterized morphologically by a prominent inflammatory infiltrate and ectatic sinusoids and show a positive immune reaction with antibodies against serum amyloid A and CRP. In contrast to other HCAs, β‑catenin-activated HCAs due to CTNNB1 mutation in exon 3 occur relatively more frequently in men (for example after intake of anabolic steroids) and have a significantly increased risk of transformation in a hepatocellular carcinoma (HCC) in comparison to CTNNB1 mutations in exons 7 and 8. CTNNB1 mutations may also occur in IHCAs (b-IHCA). Sonic hedgehog-activated HCAs show increased ASS1 expression and have a high risk of rupture and bleeding.Concerning differential diagnosis, it is important to distinguish HCAs from FNH, which cover a clinically similar patient group, and from highly differentiated HCC, which occur more frequently in men at an increased patient age and in chronic liver disease.
肝细胞腺瘤(HCAs)是一种罕见的良性肝细胞肿瘤,通常发生在接受避孕治疗的年轻女性或患有代谢性肝病的非肝硬化肝脏中。HCAs可通过影像学和组织学进行亚型分类,停用避孕药后病情可得到控制,若有恶变潜能或HCA大小超过5厘米则需进行切除。组织学上,HCAs表现为高分化肝细胞肿瘤,与局灶性结节性增生(FNH)不同,缺乏类似门静脉的结构。已经描述了与预后相关的形态分子HCA亚型。HNF1A失活的HCAs通常表现为明显的脂肪变性和L-FABP缺失。炎症性HCAs(IHCAs)在形态上的特征是有明显的炎症浸润和扩张的血窦,并且对血清淀粉样蛋白A和CRP抗体呈现阳性免疫反应。与其他HCAs不同,由于外显子3中的CTNNB1突变导致的β-连环蛋白激活的HCAs在男性中相对更常见(例如在服用合成代谢类固醇后),并且与外显子7和8中的CTNNB1突变相比,其转变为肝细胞癌(HCC)的风险显著增加。CTNNB1突变也可能发生在IHCAs(b-IHCA)中。音猬因子激活的HCAs显示ASS1表达增加,并且有很高的破裂和出血风险。关于鉴别诊断,将HCAs与FNH(其覆盖临床相似的患者群体)以及与高分化HCC(在年龄较大的男性和慢性肝病患者中更常见)区分开来很重要。