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伴有β-连环蛋白激活的色素沉着性肝细胞腺瘤:病例报告及文献复习

Pigmented hepatocellular adenoma with β-catenin activation: case report and literature review.

作者信息

Coelho Rosa, Gonçalves Regina, Carneiro Fátima, Fernandes Margarida, Lopes Joanne, Guimarães Susana, Macedo Guilherme

机构信息

Gastroenterology Department, Centro Hospitalar São João, Porto. Portugal.

Pathology Department, Centro Hospitalar São João, Porto. Portugal; IPATIMUP, Institute of Molecular Pathology and Immunology of the University of Porto, Porto. Portugal.

出版信息

Ann Hepatol. 2016 Jul-Aug;15(4):598-603.

Abstract

Hepatocellular adenomas (HCAs) are benign liver tumors recently characterized into 4 different types according to genetic, pathological and clinical features. The prognosis is not well established yet and malignant transformation has been recently associated with β-catenin activation. We aimed to describe a case of a pigmented HCA with β-catenin nuclear expression and inflammatory features and to review the cases of pigmented HCAs in the literature. We report a case of a young female patient without contraceptive use, with a liver tumor diagnosis. Liver biopsy revealed diffuse expression of β-catenin and a partial hepatic resection was performed. The histologic analysis revealed a hepatocellular tumor composed of uniform trabeculae of hepatocytes and solid areas, the later with a significant amount of black pigment highlighted by Masson-Fontana stain. Immunohistochemistry showed co-expression of C-reactive protein and serum amyloid A in the tumor. Literature review revealed that pigmented HCAs, previously reported as dark adenomas, are rare tumors. In HCAs, the presence of β-catenin activation should be searched for due to the higher risk of malignant transformation in hepatocarcinoma. We describe a pigmented HCA with β-catenin nuclear expression and inflammatory features being the fifth case reported so far.

摘要

肝细胞腺瘤(HCAs)是一种良性肝脏肿瘤,最近根据基因、病理和临床特征被分为4种不同类型。其预后尚未完全明确,且恶性转化最近被认为与β-连环蛋白激活有关。我们旨在描述一例具有β-连环蛋白核表达和炎症特征的色素沉着型HCA病例,并回顾文献中色素沉着型HCA的病例。我们报告一例年轻女性患者,未使用避孕药,被诊断为肝脏肿瘤。肝脏活检显示β-连环蛋白弥漫性表达,并进行了部分肝切除术。组织学分析显示,肝细胞肿瘤由均匀的肝细胞小梁和实性区域组成,后者经Masson-Fontana染色显示有大量黑色色素。免疫组化显示肿瘤中C反应蛋白和血清淀粉样蛋白A共表达。文献回顾显示,色素沉着型HCA,以前称为深色腺瘤,是罕见肿瘤。在HCA中,由于肝细胞癌发生恶性转化的风险较高,应寻找β-连环蛋白激活的存在情况。我们描述了一例具有β-连环蛋白核表达和炎症特征的色素沉着型HCA,这是迄今为止报道的第五例。

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