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.
作者
蒋洪棉
朱世龙
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)
出处
《中国社区医师》
2020年第33期132-133,共2页
Chinese Community Doctors
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
Bone and soft tissue
Peripheral primitive neuroectodermal tumor
Diagnosis
Differential diagnosis
