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原发性肝肉瘤样癌:两例报告并文献复习。

Primary liver sarcomatous carcinoma: report of two cases and review of the literature.

机构信息

"F. Addarii" Institute of Oncology and Transplantation Pathology, S. Orsola-Malpighi Hospital, Bologna University, Italy.

出版信息

Pathol Res Pract. 2013 Apr;209(4):249-54. doi: 10.1016/j.prp.2013.01.005. Epub 2013 Feb 26.

Abstract

Primary liver sarcomatous carcinomas (PLSCs) are very aggressive tumors. They are characterized by a fast clinical course, and therefore need a prompt histological diagnosis. Here, we report two cases of PLSC. One arises in a non-cirrhotic liver and the other in cirrhosis, with differences in onset and histological features. Special emphasis is put on the expression of albumin and HCC markers, and their possible usefulness in the diagnosis. The English literature of the last 20 years was revised (92 cases). Immunohistochemistry was performed manually or automatically; in situ hybridization (ISH) technique for albumin mRNA detection was carried out. The sarcomatoid components in both cases were immunoreactive for K8/18, Glutamine Synthetase and EZH2, and negative for Glypican 3, SMA, caldesmon, desmin, DOG-1, CD34, CD31, CD117, CD56, and alpha-fetoprotein. The detection of albumin mRNA by ISH was negative in the sarcomatoid component in both cases. PLSC represents a diagnostic challenge for pathologists, especially in its "pure" form: neither albumin mRNA detection nor HCC markers are useful for the diagnosis: positivity for K8/18 and the negativity for the mesenchymal markers seem to represent the main tools for the histological diagnosis.

摘要

原发性肝脏肉瘤样癌(PLSCs)是非常侵袭性的肿瘤。它们的临床病程进展迅速,因此需要及时进行组织学诊断。在这里,我们报告了两例 PLSC 病例。其中一例发生在非肝硬化肝脏,另一例发生在肝硬化肝脏,其发病和组织学特征存在差异。特别强调了白蛋白和 HCC 标志物的表达及其在诊断中的可能用途。我们复习了过去 20 年的英文文献(92 例)。采用手动或自动免疫组织化学法;进行了白蛋白 mRNA 检测的原位杂交(ISH)技术。两例中的肉瘤样成分均对 K8/18、谷氨酰胺合成酶和 EZH2 呈免疫反应性,而对 Glypican 3、SMA、钙调蛋白、结蛋白、DOG-1、CD34、CD31、CD117、CD56 和甲胎蛋白呈阴性。两例中的肉瘤样成分的 ISH 检测均为白蛋白 mRNA 阴性。PLSC 对病理学家来说是一个诊断挑战,尤其是在其“纯”形式下:白蛋白 mRNA 检测和 HCC 标志物均无助于诊断:K8/18 阳性和间充质标志物阴性似乎是组织学诊断的主要工具。

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