原发性纵隔透明细胞肉瘤：一例报告并文献复习
Primary mediastinal clear cell sarcoma: a case report and review of the literature.
作者信息
Jin Long, Sui Yuxia, Zhu Haili, Chen Zhizhong, Liu Shuguang
机构信息
Department of Pathology, Provincial Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital, Fuzhou, 350001, Fujian Province, People's Republic of China.
Department of Pharmacy, Provincial Clinical Medical College of Fujian Medical University, Fujian Provincial Hospital, Fuzhou, 350001, Fujian Province, People's Republic of China.
出版信息
Diagn Pathol. 2017 Jan 13;12(1):5. doi: 10.1186/s13000-016-0594-z.
BACKGROUND
Clear cell sarcoma (CCS) is a rare malignant soft-tissue neoplasm that displays melanocytic markers and exhibits striking histopathological features. The tumour has a predilection for the lower extremities and rarely presents in the mediastinum.
CASE PRESENTATION
We present a case of primary mediastinal CCS in a 57-year-old man. Computer tomography (CT) revealed a 12 × 12 × 7.5 cm mass in the anterior mediastinum. Microscopically, the tumour mainly consisted of epithelioid cells with oval vesicular nuclei and eosinophilic cytoplasm. Immunohistochemically, the tumour was positive for human melanoma black 45 (HMB-45) and vimentin but negative for S-100 and Melan-A. Fluorescence in situ hybridisation (FISH) showed a translocation involving the EWSR1 gene region.
CONCLUSION
This report will illustrate that the mediastinum is a potential site for primary CCS and FISH plays an important role in making a conclusive diagnosis.
背景
透明细胞肉瘤（CCS）是一种罕见的恶性软组织肿瘤，具有黑素细胞标志物且呈现出显著的组织病理学特征。该肿瘤好发于下肢，很少出现在纵隔。
病例报告
我们报告一例57岁男性的原发性纵隔CCS。计算机断层扫描（CT）显示前纵隔有一个12×12×7.5厘米的肿块。显微镜下，肿瘤主要由具有椭圆形泡状核和嗜酸性细胞质的上皮样细胞组成。免疫组织化学检查显示，肿瘤对人黑素瘤黑色45（HMB-45）和波形蛋白呈阳性反应，但对S-100和Melan-A呈阴性反应。荧光原位杂交（FISH）显示涉及EWSR1基因区域的易位。
结论
本报告将说明纵隔是原发性CCS的一个潜在部位，FISH在做出确定性诊断中起重要作用。
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