项一宁,陈佳,黄淞崧,钟愉,出良钊
XIANG Yining1,CHEN Jia2,HUANG Songsong2,ZHONG Yu2,CHU Liangzhao3 (1.Department of Pathology

• 1:贵阳医学院病理学教研室
• 2:贵阳医学院附院病理科
• 3:贵阳医学院附院神经外科

摘要(Abstract)：

目的:探讨非典型性畸胎样瘤-横纹肌样瘤(AT/RT)的临床病理学特征。方法:对1例AT/RT患儿进行临床表现、组织病理学、免疫组织化学和超微结构的观察,并对患者进行随访。结果:患者主要临床表现为头痛和右侧脑室前角占位,组织病理学特点主要有上皮结构、原始神经外胚叶肿瘤(PNET)结构、髓母细胞瘤结构、横纹肌样瘤结构及间叶组织来源肿瘤结构;免疫组织化学在不同的组织区域免疫表型不同,弥漫表达Vimen-tin和局灶表达EMA、CD99、GFAP、Syn、SMA及NSE;超微结构发现肿瘤细胞的胞浆中出现大量特征性的旋涡状的中间丝;患儿出院1周后死亡。结论:AT/RT是胚胎性高度恶性肿瘤,有复杂的组织学结构和免疫组织化学特点及特征性的超微结构。
Objective:To investigate the clinical and pathological characteristic features of atypical teratoid/rhabdoid tumors(AT/RT).Method:Clinical manifestation,histopathology characteristics,immunohistochemical and ultrastructural features of 1 case of AT/RT were observed,and the patient was followed up.Results:The main clinical symptom of the patient was headache,and an occupational mass was found in right anterior horn of lateral ventricle.The histopathology features of the tumor were complex.There were five main structures:epithelial,primitive neural epiblast tumor-like,medulloblastoma-like,striped muscle-like and mesenchymal structures.The immunohistochemical features were different in different histo-region.The case diffusely expressed vimentin,and focally expressed EMA,CD99,GFAP,Syn,SMA and NSE.A great number of distinctive gyrate interzonal fibers were found in tumor cell cytoplasm.The patient died in a week after leaving hospital.Conclusion:AT/RT which usually happen in children is a highly aggressive tumor.It has complex histopathology structure,immunohistochemical feature,and characteristic ultrastructure.

关键词(KeyWords)： 非典型性畸胎样瘤-横纹肌样瘤;儿童;病理学;免疫组织化学
atypical teratoid /rhabdoid tumor;children;pathology;immunohistochemistry

Abstract:

Keywords:

基金项目(Foundation):

作者(Author): 项一宁,陈佳,黄淞崧,钟愉,出良钊
XIANG Yining1,CHEN Jia2,HUANG Songsong2,ZHONG Yu2,CHU Liangzhao3 (1.Department of Pathology

DOI: 10.19367/j.cnki.1000-2707.2011.04.013

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