Primary monophasic synovial sarcoma of the pleura - Five cases confirmed by the presence of SYT-SSX fusion transcript
Primary monophasic synovial sarcoma of the pleura - Five cases confirmed by the presence of SYT-SSX fusion transcript
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DOI：
10.1097/00000478-200106000-00009
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发表时间：
2001-06-01
影响因子：
5.6
通讯作者：
Tazelaar, HD
中科院分区：
文献类型：
--
作者：
Aubry, MC;Bridge, JA;Tazelaar, HD
本研究报告了5例原发性胸膜单相滑膜肉瘤，并评估了SYT-SSX融合转录物在鉴别诊断中的作用。患者平均年龄47岁，无性别偏好。临床表现为胸痛和胸膜肿块伴积液。每个病人都接受了完整的手术切除肿块。4例患者平均随访9个月。他们都还活着，没有患病的迹象。组织学上，肿瘤由密集排列的梭状细胞组成，局部与较少的细胞区交替。苏木精和伊红水平未见上皮分化。角蛋白和上皮膜抗原反应性是局灶性的，分别存在于4个和2个肿瘤中。对CD34无免疫反应性。RT-PCR检测SYT-SSX1或SYT-SSX2融合转录物在所有肿瘤中均呈阳性。相比之下，10例局部纤维性肿瘤角蛋白和上皮膜抗原免疫组化阴性，CD34阳性。SYT-SSX融合转录物在5个局部纤维性肿瘤中均未被发现。鉴定滑膜肉瘤特异性嵌合转录物（SYT-SSX1或SYT-SSX2），结合免疫过氧化物酶研究，可以非常有助于识别胸膜单相滑膜肉瘤病例。
This study reports five cases of primary pleural monophasic synovial sarcomas and assesses the role of the SYT-SSX fusion transcript in the differential diagnosis. Patients had a mean age of 47 years with no gender predilection. Chest pain and pleural-based masses with effusions characterized the clinical presentations. Each patient underwent a complete surgical resection of the mass. The mean follow-up was 9 months, available in four patients. They were all alive, with no evidence of disease. Histologically, neoplasms were composed of densely packed fusiform cells focally alternating with less cellular areas. No epithelial differentiation was identified at the hematoxylin and eosin level. Keratin and epithelial membrane antigen reactivity was focal and present in four and two tumors, respectively. There was no immunoreactivity for CD34. RT-PCR studies for the presence of a SYT-SSX1 or SYT-SSX2 fusion transcript were positive in every tumor. In comparison, 10 localized fibrous tumors were immunohistochemically negative for keratin and epithelial membrane antigen and positive for CD34. A SYT-SSX fusion transcript was not identified in any of five localized fibrous tumors tested. Identification of the synovial sarcoma-specific chimeric transcript (SYT-SSX1 or SYT-SSX2), in conjunction with immunoperoxidase studies, can be extremely helpful in identifying cases of pleural monophasic synovial sarcoma.