18例乳腺原始神经外胚层肿瘤临床分析  

Clinical analysis of 18 cases of breast primitive neuroectodermal tumor

作　　者：方斌 李文涛[1] 翟保平[1] 刘继全 陈海军[1] 刘高秀 汪操 鲁鹤臻 密雪芳 吴丹婷 Fang Bin;Li Wentao;Zhai Baoping;Liu Jiquan;Chen Haijun;Liu Gaoxiu;Wang Cao;Lu Hezhen;Mi Xuefang;Wu Danting(Department of Breast Surgery,Henan Provincial People’s Hospital,Zhengzhou 450003,China;Department of Pathology,Henan Provincial People’s Hospital,Zhengzhou 450003,China)

机构地区：[1]河南省人民医院乳腺外科,450003 [2]河南省人民医院病理科,450003

出　　处：《中华内分泌外科杂志》2019年第2期113-118,共6页Chinese Journal of Endocrine Surgery

基　　金：河南省医学科技攻关计划项目省部共建项目(201601013);河南省科技攻关项目(172102310097).

摘　　要：目的探讨乳腺原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)的临床病理特点及预后分析。方法在知网、Pubmed、Europe PMC等数据库中检索1980年1月至2016年12月文献报道的乳腺PNET患者,结合我院一例乳腺PNET患者对其临床资料进行回顾性分析。结果18例患者以无痛性迅速增长肿块为主要临床特点。肿瘤病理形态学上均表现为小圆细胞肿瘤,PAS染色阳性;免疫组化中CD99、Fli-1特征性表达是诊断乳腺PNET的主要指标,Vimentin、NSE、Syn的阳性表达及CK、EMA、Desmin、CgA、LCA、S-100的阴性表达对于诊断乳腺PNET也有重要作用;遗传学指标EWSRI阳性表达是诊断乳腺PNET的金标准。肿瘤大小、有无手术治疗、淋巴结是否转移、有无远处转、有无化疗是影响患者预后的重要因素。患者1年生存率为71.4%,3年生存率为33.3%。结论乳腺PNET是一种预后较差的罕见恶性肿瘤,确诊高度依赖病理,手术能显著提高患者预后,应采取手术为主,放疗、化疗为辅的综合治疗,目前研究未支持对乳腺PNET患者采用内分泌及靶向药物治疗。Objective To investigate the clinical pathological characteristics and prognosis of primitive neuroectodermal tumor (PNET) of breast. Methods Patients with breast PNET were retrieved from CNKI, Pubmed, Europe PMC and other databases from Jan. 1980 to Dec. 2016. The clinical data of one patient with breast PNET in our hospital were analyzed retrospectively. Results 18 cases had painless, rapid growth mass as the main clinical features. The pathological morphology showed small round cell tumors, PAS staining positive. Immunohistochemistry CD99 and Fli-1 characteristic expression were the main indexes for the diagnosis of breast PNET. The positive expression of Vimentin, NSE, Syn and negative expression of CK, EMA, Desmin, CgA, LCA, S-100 also played an important role in the diagnosis of breast PNET. The positive expression of genetic marker EWSRI was the golden standard for diagnosis of breast PNET. The size of the tumor, surgical treatment, lymph node metastasis, distant metastasis and chemotherapy were the important factors that affect the prognosis of the PNET. The survival rates of 1 and 3 years were 71.4% and 33.3% respectively. Conclusions Breast PNET is a rare tumor with poor prognosis, and its diagnosis is highly dependent on pathology. Surgery can significantly improve the prognosis of the patients. Surgery should be the main treatment, combined with radiotherapy and chemotherapy. The current study does not show evidence of effectiveness in terms of endocrine or targeted drug therapy for breast PNET patients.

关 键 词：乳腺 原始神经外胚层肿瘤 临床病理特点 预后分析 

分 类 号：R737.9[医药卫生—肿瘤]