Department of Cardiothoracic Surgery, Tianjin Medical University General Hospital, Tianjin, China.
Thorac Cancer. 2020 May;11(5):1344-1349. doi: 10.1111/1759-7714.13395. Epub 2020 Mar 20.
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.
孤立性纤维瘤（SFT）是一种罕见的临床肿瘤，定义为源自成纤维细胞的间叶性肿瘤。大多数情况下，经典 SFT 为良性，但临床行为不可预测。最近的分子分析发现，几乎所有 SFT 都存在 NAB2-STAT6 融合基因，这被认为是该肿瘤类型的特异性标志物。最近的研究表明，核 STAT6 免疫反应性是 SFT 的高度敏感和特异性标志物，当常规方法诊断不明确时，可能会有所帮助。本文报告了一例罕见的发生在纵隔胸膜的恶性孤立性纤维瘤。一名 82 岁的中国男性间歇性呼吸困难，被转至我院。胸部 CT 显示前纵隔区明显增强的不规则巨大软组织肿块。根治性切除后，样本的免疫组织化学染色结果显示 STAT6 阴性。最终诊断通过定性终点逆转录-聚合酶链反应技术确认，显示 NAB2ex4-STAT6ex2 融合阳性。
