原标题：具有EWSR1-PATZ1基因交融的梭形和圆形细胞肉瘤
摘要：
圆形细胞肉瘤分类的演化是由其分子学研究进展驱动的。具有EWSR1-PATZ1基因交融阳性的梭形和圆形细胞肉瘤正是这样一种新的肿瘤实体。在此，咱们报导2例EWSR1-PATZ1基因交融阳性的梭形和圆形细胞肉瘤，它们具有穿插的组织学特征和多向分解特色。患者分别为31岁和53岁女人，临床表现为腹腔内肿瘤。2例肿瘤均由圆形至梭形细胞组成，呈片状和巢状摆放，细胞染色质均匀，核仁小而显着，细胞质中等量以及肿瘤细胞间有粗大纤维条带。在免疫组化方面，2例肿瘤均表达CD99、结蛋白、抗体myogenin、MyoD1、S100、Sox10、CD34和GFAP，而角蛋白则呈阴性表达。荧光原位杂交（FISH）检测显现，肿瘤的EWSR1基因位点发作重排。根据二代测序的RNA交融剖析显现，2例肿瘤均有EWSR1-PATZ1基因交融。EWSR1-PATZ1基因交融阳性的梭形和圆形细胞肉瘤表现为丰厚的肿瘤内纤维增生以及多向分解特征，因而需辨别一系列其他肿瘤，包含促结缔组织增生性小圆形细胞肿瘤。这种梭形和圆形细胞肉瘤的精确分类以及它们与尤文肉瘤宗族肿瘤的联系有待进一步清晰。
Am J Surg Pathol 2019;43:220-228
美国外科病理学杂志中文版2019年第三期摘要NO.7
（张晓毅 翻译 樊祥山 审校）
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