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.
出处
《中国现代医学杂志》
CAS
CSCD
北大核心
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)
关键词
ASKIN瘤
胸壁肿瘤
临床病理
诊断治疗
免疫组化
预后
Askin tumor
chest wall tumors
clinical pathology
diagnosis therapy
immunohistochemistry
prognosis
