Askin瘤6例报告并文献复习  

6 cases of Askin tumor report and review of the literatures

作　　者：张曙光[1] 宋平平[1] 张为迪[1] 刘希斌[1] 申玉春 董印军[1] 张百江[1] 

机构地区：[1]山东省肿瘤医院外科第六病区,山东济南250117 [2]山东省嘉祥县二院,山东嘉祥272404

出　　处：《中国现代医学杂志》2013年第19期44-48,共5页China Journal of Modern Medicine

基　　金：山东省自然科学基金(No:Y2008C150;ZR2011HM089)

摘　　要：目的探讨Askin瘤的临床特点、影像学和病理特征、诊断、治疗及预后状况。方法复习国内发表的经病理证实的45例Askin瘤病例,结合该院近期收治的6例Askin瘤病例进行分析。结果 Askin瘤是发生于胸壁的原始神经外胚层肿瘤,临床特征不典型,特征性病理形态是镜下有典型的Homer-Wright菊形团结构,电镜检查显示的胞浆内神经内分泌颗粒为诊断本病的重要依据。免疫组化以CD99、S-100、NSE阳性表达为特征。治疗多采用以手术为主的综合治疗,治疗效果差,30例患者的平均生存期仅为7个月。结论 Askin瘤是一种发生于胸壁、罕见的神经内分泌瘤,高度恶性,易复发、转移,目前主张综合治疗,预后差。【Objective】To investigate clinical features,imaging and pathological features,diagnosis,therapy,prognosis of Askin tumor.【Methods】Six cases of askin tumor were presented and 45 cases reported in literatures published in China's Mainland were reviewed.【Results】Askin tumor is a primitive neuroectodermal tumor in thoracic region,clinical features is not typical,the typical pathological form is Homer-Wright rosettes under microscope,the important basis of diagnosing disease is intracytoplasm neuroendocrine granules under electron microscope.Features are positive expression of immunohistochemical reactivity for CD99,S-100,NSE.The comprehensive treatment based on operation is important,the therapeutic effect was poor,the mean survival time of 30 patients was only 7 months.【Conclusion】Askin tumor is a rare neuroendocrine tumor in thoracic region,high malignant tumor,recurrence and metastasis were likely to happen,comprehensive treatments are the claimed at present,but the prognosis remains very poor.

关 键 词：ASKIN瘤 胸壁肿瘤 临床病理 诊断治疗 免疫组化 预后 

分 类 号：R734.4[医药卫生—肿瘤]