圆形细胞肉瘤分类的演变是由其分子学研究进展驱动的。具有EWSR1-PATZ1基因融合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，我们报道2例EWSR1-PATZ1基因融合阳性的梭形和圆形细胞肉瘤，它们具有交叉的组织学特征和多向分化特点。患者分别为31岁和53岁女性，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，呈片状和巢状排列，细胞染色质均匀，核仁小而明显，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）检测显示，肿瘤的EWSR1基因位点发生重排。基于二代测序的RNA融合分析显示，2例肿瘤均有EWSR1-PATZ1基因融合。EWSR1-PATZ1基因融合阳性的梭形和圆形细胞肉瘤表现为丰富的肿瘤内纤维增生以及多向分化特征，因此需鉴别一系列其他肿瘤，包括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准确分类以及它们与尤文肉瘤家族肿瘤的关系有待进一步明确。
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©2018 Wolters Kluwer Health
The material is published by Wolters Kluwer Health with the permission of American Journal of Surgical Pathology.No part of this publication may be reproduced in any form,stored in a retrieval system or transmitted in any form,by any means,without prior written permission from Wolters Kluwer Health.Opinions expressed by the authors and advertisers are not necessarily those of the American Journal of Surgical Pathology, its affiliates,or of the Publisher.The American Journal of Surgical Pathology,its affiliates,and the Publisher disclaim any liability to any party for the accuracy,completeness,efficacy,or availability of the material contained in this publication (including drug dosages) or for any damages arising out of the use or non-use of any of the material contained in this publication.
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【本文经《美国外科病理学杂志》授权发布，其他媒体转载或引用须经《美国外科病理学杂志》同意 ，否则追究法律责任；所有文章仅供公益交流，不代表本站立场。欢迎提供素材、资料等，投稿邮箱： tougao@91360.com，一经采纳将给予稿费】
©2018 Wolters Kluwer Health
The material is published by Wolters Kluwer Health with the permission of American Journal of Surgical Pathology.No part of this publication may be reproduced in any form,stored in a retrieval system or transmitted in any form,by any means,without prior written permission from Wolters Kluwer Health.Opinions expressed by the authors and advertisers are not necessarily those of the American Journal of Surgical Pathology, its affiliates,or of the Publisher.The American Journal of Surgical Pathology,its affiliates,and the Publisher disclaim any liability to any party for the accuracy,completeness,efficacy,or availability of the material contained in this publication (including drug dosages) or for any damages arising out of the use or non-use of any of the material contained in this publication.
Although advertising material is expected to conform to ethical (medical) standards,inclusion in this publication does not constitute a guarantee or endorsement of the quality or value of such product or of the claims made of it by its manufacturer.
【本文经《美国外科病理学杂志》授权发布，其他媒体转载或引用须经《美国外科病理学杂志》同意 ，否则追究法律责任；所有文章仅供公益交流，不代表本站立场。欢迎提供素材、资料等，投稿邮箱： tougao@91360.com，一经采纳将给予稿费】
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