背景:肝腺瘤(HA)是良性的,肝脏实质性病变,有出血和恶性转化的风险。在这篇评论文章中,我们重点介绍了HA的诊断和管理方面的进展。
方法:使用MEDLINE/PubMed和WebofScience数据库进行了全面审查,检索期为2023年9月30日。使用PubMed,术语“肝细胞,“肝”,搜索了“腺瘤”和“腺瘤”。
结果:根据分子病理学将HA分为至少8种亚型,每个都表现出独特的组织病理学特征,临床考虑,和恶性转化的风险。最常见的亚型是炎性HA(IHA),其次是HNF1α灭活的HA(HHA),β-连环蛋白外显子3突变的HA(βex3-HA),β-连环蛋白外显子7或8突变的HA(βex7,8-HA),声波刺猬HA(shHA),和未分类的HA。MRI是诊断的最佳影像学方法,可以根据脂肪和毛细血管扩张症的病理特征区分HA亚型。恶性转化的风险因分子亚型而异,从<1%到近50%不等。高达42%的HA出现自发性肿瘤内出血和腹膜出血。一般来说,只有15-20%的患者需要手术。大于5cm的HA更容易并发出血和恶变,不管子类型,通常应该切除。特别是,βex3-HA具有很高的恶性转化风险,可以认为是真正的癌前病变。
结论:HAs的管理基于多学科方法。临床决策应整合性别信息,肿瘤大小,和HA亚型。在未来,HA患者将受益于针对个体分子亚型定制的新型药物治疗.
BACKGROUND: Hepatic adenomas (HAs) are benign, solid liver lesions, which carry a risk of hemorrhage and malignant transformation. This review article highlights the advances in the diagnosis and management of HAs.
METHODS: A comprehensive review was performed using MEDLINE/PubMed and Web of Science databases with a search period ending on September 30, 2023. Using PubMed, the terms \"hepatocellular,\" \"hepatic,\" and \"adenoma\" were searched.
RESULTS: HA has been classified into at least 8 subtypes based on molecular pathology, each exhibiting unique histopathologic features, clinical considerations, and risk of malignant transformation. The most common subtype is inflammatory HA, followed by hepatocyte nuclear factor 1α-inactivated HA, β-catenin exon 3-mutated HA (βex3-HA), β-catenin exon 7- or 8-mutated HA, sonic hedgehog HA, and unclassified HA. Magnetic resonance imaging is the best imaging method for diagnosis and can distinguish among HA subtypes based on fat and telangiectasia pathologic characteristics. The risk of malignant transformation varies among molecular subtypes, ranging from <1% to approximately 50%. Up to 42% of HAs present with spontaneous intratumoral hemorrhage and peritoneal hemorrhage. In general, only 15% to 20% of patients require surgery. HA larger than 5 cm are more likely to be complicated by bleeding and malignant transformation, regardless of subtype, and should generally be resected. In particular, βex3-HA carries a high risk of malignant transformation and can be considered a true precancerous lesion.
CONCLUSIONS: The management of HAs is based on a multidisciplinary approach. Clinical decision-making should integrate information on gender, tumor size, and HA subtyping. In the future, patients with HA will benefit from novel medical therapies tailored to the individual molecular subtypes.