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以背部皮下肿块为表现的伴有缺失的肉瘤样恶性间皮瘤转移:一例报告并文献复习

Metastasis of Sarcomatoid Malignant Mesothelioma With Deletion Manifested as a Subcutaneous Mass in the Back: A Case Report and Review of Literature.

作者信息

Chen Kai-Bo, Huang Ya-Jing, Huang Yi, Wu Zhi-Wei, Jin Xiao-Li, Zhang Hang, Xiang Xue-Ping, Chen Lin, Chen Li

机构信息

Second Affiliated Hospital, 89681Zhejiang University School of Medicine, Hangzhou, China.

Equal contributors.

出版信息

Int J Surg Pathol. 2021 Dec;29(8):856-863. doi: 10.1177/10668969211005094. Epub 2021 Mar 17.

Abstract

Sarcomatoid malignant mesothelioma (MM) is a rare and aggressive disease, and its diagnosis is challenging. A 60-year-old man presented with a recurrent subcutaneous mass in his right back after the initial resection. A chest computed tomography (CT) scan found right pleural thickening, nodular pleural thickening, pleural effusion, mediastinal, and right infraclavicular lymph nodes enlargement, which indicated a right pleura MM. Immunohistochemical stains of the resected mass showed sarcomatous atypical spindle cells, which were positive for pan-CKs (clone Anti-cytokeratin cocktail AE1/AE3), cytokeratin 5/6 (CK5/6), Wilm's tumor 1, podoplanin, vimentin and programmed death-ligand 1 (PD-L1), and negative for Napsin A, thyroid transcription factor 1, CDX 2, calretinin and desmin, and fluorescent in situ hybridization detected homozygous p16/cyclin-dependent kinase inhibitor 2A () deletion. The association of the chest CT features and the pathological assessment confirmed metastatic MM in the subcutaneous layer of the back. Moreover, positron emission tomography-CT showed multiple metastases in his brain. He developed massive right pleural effusion and chest tightness soon, and the mass kept growing despite local and systemic treatments. The patient die of pulmonary failure in 3 months.

摘要

肉瘤样恶性间皮瘤(MM)是一种罕见且侵袭性强的疾病,其诊断具有挑战性。一名60岁男性在初次切除后右背部出现复发性皮下肿块。胸部计算机断层扫描(CT)发现右胸膜增厚、结节状胸膜增厚、胸腔积液、纵隔及右锁骨下淋巴结肿大,提示右胸膜MM。切除肿块的免疫组织化学染色显示肉瘤样非典型梭形细胞,其对泛细胞角蛋白(克隆抗细胞角蛋白鸡尾酒AE1/AE3)、细胞角蛋白5/6(CK5/6)、威尔姆斯瘤1、足板蛋白、波形蛋白和程序性死亡配体1(PD-L1)呈阳性,而对Napsin A、甲状腺转录因子1、CDX 2、钙视网膜蛋白和结蛋白呈阴性,荧光原位杂交检测到纯合性p16/细胞周期蛋白依赖性激酶抑制剂2A()缺失。胸部CT特征与病理评估相结合证实背部皮下层存在转移性MM。此外,正电子发射断层扫描-CT显示其脑部有多处转移。他很快出现大量右胸腔积液和胸闷,尽管进行了局部和全身治疗,肿块仍持续生长。患者在3个月后死于呼吸衰竭。

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