临床与实验病理学杂志2019，Vol.35Issue(1)：33-37,5.DOI:10.13315/j.cnki.cjcep.2019.01.008
RELA融合基因阳性室管膜瘤11例临床病理分析
RELA fusion-positive ependymoma: a clincopathologic analysis of eleven cases
摘要
Abstract
Purpose To investigate the clinicopathologic features of RELA fusion-positive ependymoma. Methods The clinical manifestations, radiological appearance, pathological and immunohistochemical findings and immunophenotype in 11 cases of RELA fusion-positive ependymoma were retrospectively analyzed, and the related literature was reviewed. Results Eleven cases of RELA fusion-positive ependymomas occured in the supratentorial region. There were 6 males and 5 females ranged in age from 3-56 years (median = 27). Imaging showed the occupying lesion in the supratentorial region. Histologically, a distinctive branching network of delicate capillaries and (true) ependymal rosettes/pseudorosettes were observed. Immunohistochemically, the tumor cells were diffusely positive for GFAP, L1 CAM and Cyclin D1, and EMA staining with dot-like cytoplasmic reactivity were observed. Most of the cases showed a positive for nestin, but all of the cases showed negative for Olig-2. Conclusion RELA fusion-positive ependymomas usually affect the supratentorial region of young people, which have unique immunophenotype and gene phenotype, and are characterized by poor prognosis. This type of ependymoma should be identified in the diagnosis and differential diagnosis.关键词
室管膜瘤/RELA融合基因/L1CAM/免疫组织化学Key words
ependymoma/RELA fusion gene/L1CAM/immunohistochemistry分类
医药卫生引用本文复制引用
张安莉,孙思柏,李恒,吴海波..RELA融合基因阳性室管膜瘤11例临床病理分析[J].临床与实验病理学杂志,2019,35(1):33-37,5.基金项目
中央高校基本科研业务费专项资金(WK9110000042) （WK9110000042）
