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Malignant solitary fibrous tumor occurring in the mediastinal pleura showing NAB2ex4‐STAT6ex2 fusion and negative STAT6 immunohistochemistry: A case report

机译:纵隔胸膜发生恶性孤立性纤维性肿瘤显示NAB2ex4-STAT6ex2融合STAT6免疫组化阴性:一例

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摘要

Solitary fibrous tumor (SFT) is a rare clinical tumor, defined as a mesenchymal tumor of fibroblastic origin. A classic SFT is benign in most cases, but its clinical behavior is unpredictable. Lately, molecular analyses has discovered that almost all SFTs harbor an NAB2‐STAT6 fusion gene, which is considered specific to this tumor type. Recent studies have suggested that nuclear STAT6 immunoreactivity is a highly sensitive and specific marker of SFTs and can be helpful when diagnosis is inconclusive by conventional methods. We herein report the case of a rare malignant solitary fibrous tumor occurring in the mediastinal pleura. An 82‐year‐old Chinese man with intermittent breathlessness was referred to our hospital. Chest CT showed a significantly enhanced irregular huge soft tissue mass in the anterior mediastinal area. After radical resection, the immunohistochemistry staining results of the sample showed that STAT6 was negative. The final diagnosis was confirmed by qualitative endpoint reverse transcriptase‐polymerase chain reaction technique, showing positive NAB2ex4‐STAT6ex2 fusion.
机译:孤立性纤维性肿瘤(SFT)是一种罕见的临床肿瘤,定义为成纤维细胞来源的间充质肿瘤。经典SFT在大多数情况下是良性的,但其临床行为是不可预测的。最近,分子分析发现几乎所有SFT都带有一个NAB2-STAT6融合基因,该基因被认为对这种肿瘤类型具有特异性。最近的研究表明,核STAT6免疫反应性是SFT的高度敏感和特异的标志物,当通过常规方法不能确定诊断时可能会有所帮助。我们在此报告了在纵隔胸膜中发生罕见的恶性孤立性纤维性肿瘤的情况。一位82岁的中国男子间歇性呼吸困难被转诊到我们医院。胸部CT显示在纵隔前部区域明显增强了不规则的巨大软组织肿块。根治性切除后,样品的免疫组织化学染色结果显示STAT6为阴性。定性终点逆转录酶-聚合酶链反应技术证实了最终诊断,显示NAB2ex4-STAT6ex2融合阳性。

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