骨和软组织Ewings肉瘤/外周原始神经外胚层肿瘤临床病理分析  

Clinical pathology analysis of Ewing sarcoma/peripheral primitive neuroectodermal tumor in the bone and soft tissue

作　　者：蒋洪棉[1] 朱世龙[1] 朱晓菲 罗丽凤 韦芳 Jiang Hongmian;Zhu Shilong;Zhu Xiaofei;Luo Lifeng;Wei Fang(Department of Pathology,the First People's Hospital of Nanning City,Guangxi Nanning 530022)

机构地区：[1]南宁市第一人民医院病理科,广西南宁530022

出　　处：《中国社区医师》2020年第33期132-133,共2页Chinese Community Doctors

摘　　要：目的:探讨骨和软组织尤因肉瘤(EWS)/外周原始神经外胚层肿瘤(pPNET)的临床病理特征、诊断、鉴别诊断及预后。方法:选取2016年2月-2020年2月骨和软组织EWS/pPNET 6例的临床病理特征并复习文献。结果:EWS/pPNET中肿块最大直径1.5~9.3 cm(平均6.2 cm)。低倍镜下肿瘤细胞呈索状或弥漫分布,可见Homer-Wright菊形团或假性菊形团,核分裂象可见,有坏死。免疫表型:肿瘤细胞vimentin、CD99阳性,Ki-67增殖指数30%~50%,6例均行手术、化疗及放疗干预后,患者症状改善显著,病情得到控制。术后随访,患者死亡2例。结论:骨和软组织EWS/pPNET常见于躯干、四肢和中线软组织,确诊主要依赖组织学形态、免疫表型及分子病理学检测,EWS/pPNET以手术完整切除为主要治疗方法,并结合术后化疗、放疗。Objective:To discuss the clinical pathological characteristics,diagnosis,differential diagnosis and surgical prognosis for patients with Ewing sarcoma(EWS)/peripheral primitive neuroectodermal tumor(pPNET)in the bone and soft tissue.Methods:From February 2016 to February 2020,the clinical pathological characteristics about 6 patients with EWS/pPNET in the bone and soft tissue and the literatures were reviewed.Results:The maximum lesion diameter of EWS/pPNET was in the range of 1.5~9.3 cm,with an average value of 6.2 cm.At low magnification,the tumor cells showed cord-shaped or diffuse distribution,Homer-Wright chrysanthemum-shaped or pseudochrysanthemum-shaped,mitotic and necrotic.Immunophenotype:tumor cells were positive for vimentin and CD99,and Ki-67 proliferation index was 30%~50%.After the intervention of operation,chemotherapy and radiotherapy in all the 6 cases,the symptoms of the patients were significantly improved and the condition was controlled.Postoperative follow-up showed that 2 patients died.Conclusion:EWS/pPNET is commonly appeared in the body,limb and soft tissues.Patients are mainly confirmed by histological patterns,immunophenotyping and molecular pathology,and cured by radical resection with chemotherapy and radiotherapy.

关 键 词：骨和软组织 外周性原始神经外胚层肿瘤 诊断 鉴别诊断 

分 类 号：R738[医药卫生—肿瘤]