摘要：鼻腔鼻窦低级别腺癌（sinonasal adenocarcinomas，low-grade SNAC）是指一组具有低组织形态学异质性的肿瘤。本文报道3例以ETV6基因重排为特点的低级别SNAC独特亚型病例。患者包括2位女性（32岁和88岁）和1位男性（75岁），均只行手术治疗。随访9~170个月，1例局部复发2次，无区域或远处转移。肿瘤由胞浆丰富的柱状和立方形嗜酸性细胞组成，呈管状和小梁状排列，核位于基底部 。免疫组化结果显示，全部3例CK7、DOG1、GCDFP-15和SOX10均阳性，2例波形蛋白阳性。散在单个或少部分肿瘤细胞S-100阳性。1例局灶、弱表达GATA3，乳球蛋白阴性。2例病例有ETV6-NTRK3基因融合，其余1例ETV6基因与未知伙伴基因融合。相似的形态学表现、免疫表型和遗传学改变提示本组 3 例病例为同类肿瘤。既往鼻腔鼻窦基因易位相关癌是指涎腺型肿瘤，本文报道首个以特定基因重排和独特表型为特征、鼻腔鼻窦原发、未见发生于大涎腺的肿瘤，暂称此类肿瘤为伴 ETV6 基因重排的低级别 SNAC。确诊新病例有助于充分了解此类肿瘤的组织形态学和生物学谱系。
关键词：鼻腔鼻窦腺癌，ETV6，ETV6-NTRK3，ETV6基因重排鼻腔鼻窦低级别腺癌，ETV6-X
来源：Am J Surg Pathol 2017;41:1552-1560
美国外科病理学杂志中文版2018年第一期全文No.5
（岳常丽 翻译 刘红刚 审校）
The American Journal of Surgical Pathology中文版声明：
©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】
【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】
苏公网安备 32011402011742