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伴有EWSR1基因重排的间叶性肿瘤

Mesenchymal Tumors with EWSR1 Gene Rearrangements.

作者信息

Thway Khin, Fisher Cyril

机构信息

Sarcoma Unit, Royal Marsden Hospital, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK.

Department of Musculoskeletal Pathology, Royal Orthopaedic Hospital NHS Foundation Trust, Robert Aitken Institute for Clinical Research, University of Birmingham, Birmingham B15 2TT, UK.

出版信息

Surg Pathol Clin. 2019 Mar;12(1):165-190. doi: 10.1016/j.path.2018.10.007.

DOI:10.1016/j.path.2018.10.007
PMID:30709442
Abstract

Among the various genes that can be rearranged in soft tissue neoplasms associated with nonrandom chromosomal translocations, EWSR1 is the most frequent one to partner with other genes to generate recurrent fusion genes. This leads to a spectrum of clinically and pathologically diverse mesenchymal and nonmesenchymal neoplasms, variably manifesting as small round cell, spindle cell, clear cell or adipocytic tumors, or tumors with distinctive myxoid stroma. This review summarizes the growing list of mesenchymal neoplasms that are associated with EWSR1 gene rearrangements.

摘要

在与非随机染色体易位相关的软组织肿瘤中可发生重排的各种基因中,EWSR1是与其他基因配对产生复发性融合基因最常见的一个。这导致了一系列临床和病理上多样的间叶性和非间叶性肿瘤,表现为小圆形细胞、梭形细胞、透明细胞或脂肪细胞肿瘤,或具有独特黏液样基质的肿瘤。本综述总结了与EWSR1基因重排相关的间叶性肿瘤不断增加的列表。

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