Hepatic adenosquamous carcinoma with sarcomatous transformation: a case report and review of the literature.

IF 1.2 4区医学 Q3 PATHOLOGY

Medical Molecular Morphology Pub Date : 2024-10-07 DOI:10.1007/s00795-024-00406-y

Quynh Thi Nguyen, Hiep Canh Nguyen, Kenta Takahashi, Kaori Yoshimura, Hiroko Ikeda, Kazuto Kozaka, Zihan Li, Dong Thanh Le, Rui Yang, Shintaro Yagi, Kenichi Harada

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Abstract

Adenosquamous carcinoma (ASC) with the presence of a sarcomatous component is exceptionally uncommon in intrahepatic cholangiocarcinoma (iCCA). We report a case of hepatic ASC with rhabdoid transformation, one variation of sarcomatous change. A 72-year-old man was admitted to our hospital after being diagnosed with a 45 mm-diameter neoplastic lesion in the right hepatic duct on abdominal computed tomography. Laboratory findings showed increases in AST, ALT, ALP, gamma-GT, CA19-9 and DUPAN-II. The patient then underwent an extended right hepatectomy. Histopathologically, the tumor was composed of an ASC component within an abundant fibrous stroma and a sarcomatoid carcinoma component. By immunohistochemistry, keratin 7 and keratin 19 were expressed by all tumor cells. Expression of keratin 5/6, p40 and p63 was restricted to the squamous component. The sarcomatoid component was immunoreactive for vimentin with no loss of INI1 expression. This component also showed a loss of membranous E-cadherin expression and a reduction of membranous β-catenin expression. Staining for desmin, myoglobin and HepPar1 was negative in any tumor cells. The patient died of liver failure 3 months after surgery. This report aims to provide a better understanding of the clinicopathological characteristics and disease progression of the rare variants of iCCA to aid diagnosis and treatment.

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肝腺鳞癌伴肉瘤变：病例报告和文献综述。

在肝内胆管癌（iCCA）中，伴有肉瘤成分的腺鳞癌（ASC）并不常见。我们报告了一例肝ASC伴横纹肌样变的病例，横纹肌样变是肉瘤变的一种变异。一名 72 岁的男性在腹部计算机断层扫描中被诊断为右肝管直径 45 毫米的肿瘤性病变，随后被送入我院。实验室检查结果显示，患者的 AST、ALT、ALP、γ-GT、CA19-9 和 DUPAN-II 均有所升高。患者随后接受了右肝扩大切除术。从组织病理学角度看，肿瘤由位于丰富纤维基质内的 ASC 成分和肉瘤样癌成分组成。免疫组化结果显示，所有肿瘤细胞均表达角蛋白 7 和角蛋白 19。角蛋白5/6、p40和p63的表达仅限于鳞状细胞。肉瘤成分对波形蛋白有免疫反应，但 INI1 的表达没有丧失。该成分还显示膜E-cadherin表达缺失，膜β-catenin表达减少。任何肿瘤细胞中的 desmin、肌红蛋白和 HepPar1 染色均为阴性。患者在术后 3 个月死于肝功能衰竭。本报告旨在让人们更好地了解罕见变异型iCCA的临床病理特征和疾病进展情况，以帮助诊断和治疗。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Medical Molecular Morphology 医学-病理学

CiteScore

2.90

自引率

5.60%

发文量

审稿时长

>12 weeks

期刊介绍： Medical Molecular Morphology is an international forum for researchers in both basic and clinical medicine to present and discuss new research on the structural mechanisms and the processes of health and disease at the molecular level. The structures of molecules, organelles, cells, tissues, and organs determine their normal function. Disease is thus best understood in terms of structural changes in these different levels of biological organization, especially in molecules and molecular interactions as well as the cellular localization of chemical components. Medical Molecular Morphology welcomes articles on basic or clinical research in the fields of cell biology, molecular biology, and medical, veterinary, and dental sciences using techniques for structural research such as electron microscopy, confocal laser scanning microscopy, enzyme histochemistry, immunohistochemistry, radioautography, X-ray microanalysis, and in situ hybridization. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been reviewed by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in an appropriate version of the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted.