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肝细胞腺瘤更新:诊断、分子分类和临床过程。

Hepatocellular adenoma update: diagnosis, molecular classification, and clinical course.

机构信息

Department of Biomedical Imaging and Image-guided Therapy, Medical University Vienna, Vienna, 1090, Austria.

Department of Radiology, University of Palermo, Palermo, 90133, Italy.

出版信息

Br J Radiol. 2024 Nov 1;97(1163):1740-1754. doi: 10.1093/bjr/tqae180.

Abstract

Hepatocellular adenomas (HCA) are acquired focal liver lesions, that occur mainly in young-to-middle-aged women who are on long-term estrogen-containing contraceptives or young men after prolonged use of anabolic steroids. Furthermore, distinct underlying diseases, such as obesity, metabolic dysfunction-associated steatotic liver disease, glycogen storage disease, etc. are considered risk factors. The 2017 Bordeaux classification, in particular Nault et al, divided HCAs into eight subtypes according to their pheno- and genotypic characteristics. This includes HCAs with hepatocyte-nuclear-factor (HNF1-alpha mutation), HCAs with β-catenin mutation, and HCAs without either of these genetic mutations, which are further subdivided into HCAs with and without inflammatory cells. HCAs should no longer be classified as purely benign without histologic workup since three of the eight subtypes are considered high-risk lesions, requiring adequate management: malignant transformation of the pure (ßex3-HCA) and mixed inflammatory/β-catenin exon 3 (ßex3-IHCA) adenomas, as well as potential bleeding of the sonic hedgehog HCA and pure (ßex7/8-HCA) and mixed inflammatory/β-catenin exon 7/8 (ßex7/8-IHCA). Elective surgery is recommended for any HCA in a male, or for any HCA exceeding 5 cm. Although MRI can classify up to 80% of adenomas, if findings are equivocal, biopsy remains the reference standard for adenoma subtype.

摘要

肝细胞腺瘤(HCA)是获得性局灶性肝脏病变,主要发生在长期服用含雌激素避孕药的年轻至中年女性和长期使用合成代谢类固醇的年轻男性中。此外,明显的基础疾病,如肥胖、代谢功能障碍相关脂肪性肝病、肝糖原贮积病等,被认为是危险因素。2017 年波尔多分类,特别是 Nault 等人,根据其表型和基因型特征将 HCA 分为八个亚型。这包括具有肝细胞核因子(HNF1-α突变)的 HCA、具有β-连环蛋白突变的 HCA 和这两种基因突变均无的 HCA,进一步细分为有炎症细胞和无炎症细胞的 HCA。由于八个亚型中有三个被认为是高危病变,需要进行适当的管理,因此不再将 HCA 单纯归类为良性病变而无需进行组织学检查:纯(ßex3-HCA)和混合炎症/β-连环蛋白外显子 3(ßex3-IHCA)腺瘤的恶变,以及 sonic hedgehog HCA 和纯(ßex7/8-HCA)和混合炎症/β-连环蛋白外显子 7/8(ßex7/8-IHCA)的潜在出血。建议对任何男性的 HCA 或任何超过 5cm 的 HCA 进行选择性手术。尽管 MRI 可以对 80%的腺瘤进行分类,但如果结果不确定,活检仍然是腺瘤亚型的参考标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4cf/11491668/080180efcd87/tqae180f1.jpg

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