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色素性毛细血管扩张性腺瘤中发生的肝细胞癌,核 β-连环蛋白和谷氨酰胺合成酶阳性:病例报告及文献复习。

Hepatocellular carcinoma arising in a pigmented telangiectatic adenoma with nuclear β-catenin and glutamine synthetase positivity: case report and review of the literature.

机构信息

Department of Pathology, Mount Sinai School of Medicine, One Gustave L. Levy Place, New York, NY 10029, USA.

出版信息

Am J Surg Pathol. 2011 Jun;35(6):927-32. doi: 10.1097/PAS.0b013e318218ca3f.

DOI:10.1097/PAS.0b013e318218ca3f
PMID:21572265
Abstract

Telangiectatic hepatocellular adenoma is a rare, recently recognized subtype of hepatocellular adenoma that is often underrecognized by pathologists. We report a case of hepatocellular carcinoma arising within a pigmented telangiectatic hepatocellular adenoma in a noncirrhotic man with diffuse glutamine synthetase and nuclear β-catenin positivity. This case highlights malignant transformation of telangiectatic adenomas, and describes a previously unreported association between pigment deposition and telangiectatic adenoma. Radiology, gross pathology, and histopathology are shown. Review of the literature with attention to β-catenin and glutamine synthetase staining, malignant transformation, patient characteristics, the presence of Dubin-Johnson-like pigment, and genetic characteristics of telangiectatic adenomas are discussed.

摘要

毛细血管扩张性肝细胞腺瘤是一种罕见的、最近才被认识的肝细胞腺瘤亚型,常被病理学家所忽视。我们报告了一例非肝硬化患者的肝细胞癌在色素性毛细血管扩张性肝细胞腺瘤中发生,该腺瘤弥漫性表达谷氨酰胺合成酶和核β-连环蛋白阳性。该病例强调了毛细血管扩张性腺瘤的恶性转化,并描述了色素沉着与毛细血管扩张性腺瘤之间以前未报道的关联。本文展示了影像学、大体病理和组织病理学表现。本文还对文献进行了复习,重点讨论了β-连环蛋白和谷氨酰胺合成酶染色、恶性转化、患者特征、Dubin-Johnson 样色素的存在以及毛细血管扩张性腺瘤的遗传学特征。

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