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Current updates on the molecular genetics and magnetic resonance imaging of focal nodular hyperplasia and hepatocellular adenoma

机译:局灶性结节性增生和肝细胞腺瘤的分子遗传学和磁共振成像的最新进展

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摘要

AbstractFocal nodular hyperplasia (FNH) and hepatocellular adenomas (HCAs) constitute benign hepatic neoplasms in adults. HCAs are monoclonal neoplasms characterised by an increased predilection to haemorrhage and also malignant transformation. On the other hand, FNH is a polyclonal tumour-like lesion that occurs in response to increased perfusion and has an uneventful clinical course. Recent advances in molecular genetics and genotype-phenotype correlation in these hepatocellular neoplasms have enabled a new classification system. FNHs are classified into the typical and atypical types based on histomorphological and imaging features. HCAs have been categorised into four subtypes: (1) HCAs with HNF-1α mutations are diffusely steatotic, do not undergo malignant transformation, and are associated with familial diabetes or adenomatosis. (2) Inflammatory HCAs are hypervascular with marked peliosis and a tendency to bleed. They are associated with obesity, alcohol and hepatic steatosis. (3) HCAs with β-catenin mutations are associated with male hormone administration and glycogen storage disease, frequently undergo malignant transformation and may simulate hepatocellular carcinoma on imaging. (4) The final type is unclassified HCAs. Each of these except the unclassified subtype has a few distinct imaging features, often enabling reasonably accurate diagnosis. Biopsy with immunohistochemical analysis is helpful in difficult cases and has strong implications for patient management.
机译:摘要成年人的结节性结节增生(FNH)和肝细胞腺瘤(HCA)构成了良性肝肿瘤。 HCA是单克隆肿瘤,其特征在于对出血的倾向增加以及恶性转化。另一方面,FNH是一种多克隆肿瘤样病变,可因灌注增加而发生,并且临床过程平稳。这些肝细胞肿瘤中的分子遗传学和基因型-表型相关性的最新进展使新的分类系统成为可能。根据组织形态和影像学特征,FNHs可分为典型和非典型类型。 HCA已分为四个亚型:(1)具有HNF-1α突变的HCA具有弥漫性脂肪变性,不会发生恶性转化,并与家族性糖尿病或腺瘤病相关。 (2)炎性HCA是血管过度的,有明显的骨盆病和出血的趋势。它们与肥胖,酒精和肝脂肪变性有关。 (3)具有β-catenin突变的HCA与男性激素给药和糖原贮积病相关,经常发生恶性转化,并可能在影像学上模拟肝细胞癌。 (4)最终类型是未分类的HCA。除未分类的亚型外,每一种都具有一些独特的影像学特征,通常可以进行合理准确的诊断。免疫组织化学分析活检对困难病例有帮助,对患者管理有重要意义。

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