The EWS-WT1 gene fusion in desmoplastic small round cell tumor

被引：90

作者：

Gerald, WL

Gerald, A

机构：

[1] Mem Sloan Kettering Canc Ctr, Dept Pathol, New York, NY 10021 USA

[2] Massachusetts Gen Hosp, Ctr Canc, Charlestown, MA 02129 USA

[3] Harvard Univ, Sch Med, Charlestown, MA 02129 USA

来源：

SEMINARS IN CANCER BIOLOGY | 2005年 / 15卷 / 03期

关键词：

desmoplastic small round cell tumor; DSRCT; EWS-WT1; chromosomal translocation;

D O I：

10.1016/j.semcancer.2005.01.005

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Desmoplastic small round cell tumor (DSRCT) is a poorly understood neoplasm with distinctive clinical, histologic and immunophenotypic features. It is associated with a novel, specific chromosomal abnormality, t(11;22)(p13;q12) that fuses EWS with WT1 leading to production of a chimeric protein with transcriptional regulatory activity. This chimeric transcription factor has unique DNA-binding properties and regulates expression of specific target genes. Several of these have been identified and their biological role characterized. The dysregulated expression of EWS-WT1 targets contribute to the malignant phenotype of DSRCT and provide valuable insight regarding the molecular mechanisms underlying the development and progression of this distinct translocation associated tumor. (c) 2005 Elsevier Ltd. All rights reserved.

引用

页码：197 / 205

页数：9

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