腹膜后恶性外周神经鞘膜瘤13例临床病理分析  

Retroperitoeal malignant peripheral nerve sheath tumors:a clinicopathologic study of 13 cases

作　　者：张笑盈 任玉波[1] 杨绍敏 苗成利[3] 饶晓松 ZHANG Xiao-ying;REN Yu-bo;YANG Shao-min;MIAO Cheng-li;RAO Xiao-song(Department of Pathology;3.Department of Pathology,Peking University Health Science Center/Peking University Third Hospital,Beijing 102206,China;2.Department of Retroperitoneal Tumor,Peking University International Hospital,Beijing 100083,China)

机构地区：[1]北京大学国际医院病理科,北京102206 [2]北京大学医学部病理学系/北京大学第三医院病理科,北京100083 [3]北京大学国际医院腹膜后肿瘤科,北京102206

出　　处：《临床与实验病理学杂志》2018年第6期627-631,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨腹膜后恶性外周神经鞘膜瘤(malignant peripheral nerve sheath tumors,MPNST)的临床病理学特征,及H3K27me3在MPNST中的表达。方法回顾性分析13例MPNST的临床病理特征及预后情况;应用免疫组化法检测MPNST、滑膜肉瘤、去分化脂肪肉瘤及平滑肌肉瘤中H3K27me3的表达,并复习相关文献。结果 13例MPNST均为高级别,肿瘤平均直径20 cm;2年生存率约60%;5年生存率约30%;与NF-1相关型及散发型相比,放疗(radiation therapy,RT)相关型具有较差的预后(P<0.05);复发及远处转移患者具有较差的预后(P<0.05);年龄对患者生存率无明显影响(P>0.05)。免疫组化结果显示13例MPNST中11例H3K27me3表达缺失;且H3K27me3在MPNST中的表达与在滑膜肉瘤、去分化脂肪肉瘤及平滑肌肉瘤中的表达相比差异有统计学意义(P<0.05)。结论腹膜后MPNST以高级别常见,肿瘤体积相对较大,预后较差;RT相关型、复发及远处转移是影响疾病生存率的重要因素;H3K27me3缺失更常见于高级别MPNST,可能是MPNST的有效标志物。Purpose To investigate the clinicopathology and the expression of H3 K27 me3 in retroperitoeal malignant peripheral nerve sheath tumors（MPNST）.Methods The clinicopathology and prognosis of 13 cases MPNST were analyzed.Immunohistochemical analysis was used to detect H3 K27 me3 in MPNST,synovial sarcoma,dedifferentiated liposarcoma and leiomyosarcoma.Results 13 cases of MPNST were high-grade.The mean diameter of tumors was 20 cm.2-year survival rate of MPNST was about 60%.5-year survival rate of MPNST was about 30%.Compared to NF-1 associated and sporadic MPNST（P〈0.05）,the RT-induced MPNST had a poor prognosis.Recurrence and distant metastasis patient had a poor prognosis（P〈0.05）.Age had no significant effect on patient survival.In addition,immunohistochemical staining showed that the expression of H3 k27 me3 was absent in 11 of 13 cases of MPNST.And compared with the expression of H3 K27 me3 in synovial sarcoma,dedifferentiated liposarcoma and leiomyosarcoma,it had statistical significance of that expression in MPNST（P〈0.05）.Conclusion Retroperitoeal MPNST is common at high-grade.Tumor volume is relatively large and prognosis is poor.RT-induced,recurrence and distant metastasis play an important role in survival rate of MPNST.H3 K27 me3 which is more common absence in high-grade could be an effective marker of MPNST.

关 键 词：恶性外周神经鞘膜瘤 腹膜后肿瘤 H3K27me3 

分 类 号：R735.4[医药卫生—肿瘤]