Abstract

Background: Ewing sarcoma (ES) can be confirmed by identifying the EWSR1-FLI1 fusion transcript. This study is to investigate whether immunostaining (IHC) of PRKCB—a protein directly regulated by EWSR1-FLI1 is a surrogate maker for diagnosing ES in routine practice. Methods: Microarray gene expression analyses were conducted. RKCB IHC was applied to 69 ES confirmed by morphology and molecular methods, and 41 non-Ewing small round cell tumors. EWSR1 rearrangement, EWSR1-FLI1 fusion or t(11;22)(q24;q12) were identified by fluorescence in situ hybridization, reverse transcriptase polymerase chain reaction, or cytogenetic analysis, respectively. Results: Gene array analyses showed significant overexpression of the PRKCB in ES. PRKCB IHC was positive in 19 cases of ES with EWSR1-FLI1 fusion, 3 cases with cytogenetic 11:22 translocation and 59 cases with EWSR1 rearrangement while negative in only one EWSR1 rearranged case. PRKCB IHC is sensitive (98%) and specific (96%) in detecting EWSR1 rearranged ES. Conclusions: PRKCB is a reliable antibody for diagnosing ES in routine practice.

摘要

背景：可以通过鉴定EWSR1-FLI1融合转录本来确认尤文肉瘤 (ES)。本研究旨在调查 PRKCB（一种由EWSR1-FLI1直接调节的蛋白质）的免疫染色 (IHC) 是否是常规实践中诊断 ES 的替代指标。方法：进行微阵列基因表达分析。RKCB IHC 应用于 69 例经形态学和分子学方法证实的 ES 和 41 例非尤文小圆细胞肿瘤。EWSR1 重排、EWSR1-FLI1 融合或 t(11;22)(q24;q12) 分别通过荧光原位杂交、逆转录酶聚合酶链反应或细胞遗传学分析进行鉴定。结果：基因阵列分析显示ES 中PRKCB的显着过度表达。PRKCB IHC 在 19 例 EWSR1-FLI1 融合 ES、3 例细胞遗传学 11:22 易位和 59 例 EWSR1 重排中呈阳性，而只有 1 例 EWSR1 重排呈阴性。PRKCB IHC 在检测EWSR1重排 ES时敏感 (98%) 和特异性 (96%) 。结论： PRKCB 是常规实践中诊断 ES 的可靠抗体。