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黏液样肝细胞腺瘤,一种具有独特影像学特征的肝细胞腺瘤的罕见亚型:一例病例报告并结合免疫组化和分子分析及文献复习

Myxoid hepatocellular adenoma, a rare variant of hepatocellular adenoma with distinct imaging features: A case report with immunohistochemical and molecular analysis and literature review.

机构信息

Department of Radiology, Ghent University Hospital, Ghent 9000, Belgium.

Centre for Medical Genetics, Ghent University Hospital, Ghent 9000, Belgium.

出版信息

Clin Res Hepatol Gastroenterol. 2021 May;45(3):101478. doi: 10.1016/j.clinre.2020.06.004. Epub 2020 Jun 30.

Abstract

Preoperative imaging and histopathology, immunohistochemistry and molecular analysis after resection of 2 hepatocellular adenomas (HCAs) (20 and 2cm) in a 53-year-old female patient were performed. On imaging, the large lesion resembled a myxoid HCA, while the small lesion resembled a more conventional HCA with a small myxoid/fluid area. On microscopy, the large lesion showed cords and nests of hepatocytes embedded in abundant myxoid matrix, while the small lesion resembled a conventional HCA with small foci of myxoid change and serosities; both consistent with a myxoid HCA. Immunophenotyping and molecular subtyping excluded inflammatory HCA, CTNNB1 mutated HCA and sonic hedgehog HCA, and was consistent with HNF1A mutated HCA. The myxoid change as well as the serosities may allow imaging diagnosis of myxoid HCA. As fluid vacuoles can also be present in ASS1+HCA, sonic hedgehog HCA has to be considered in the differential diagnosis.

摘要

对一名 53 岁女性患者切除的 2 个肝细胞腺瘤(HCA)(20cm 和 2cm)进行了术前影像学检查和组织病理学、免疫组织化学和分子分析。影像学上,大病变类似于黏液样 HCA,而小病变类似于具有小黏液样/液体区域的更传统 HCA。显微镜下,大病变显示肝细胞的索状和巢状结构嵌入丰富的黏液样基质中,而小病变类似于具有小灶黏液样改变和浆液的传统 HCA;均符合黏液样 HCA。免疫表型和分子亚型排除了炎性 HCA、CTNNB1 突变型 HCA 和 sonic hedgehog HCA,与 HNF1A 突变型 HCA 一致。黏液样改变以及浆液可能允许对黏液样 HCA 进行影像学诊断。由于 ASS1+HCA 中也可能存在液泡,因此在鉴别诊断中必须考虑 sonic hedgehog HCA。

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