成人幕上原始神经外胚叶肿瘤8例临床病理分析  

Adult supratentorial primitive neuroectodermal tumors: clinical and pathological analyses

作　　者：曾英[1] 肖华亮[1] 杜娟[1] 马瑜[1] 钟鹏[1] 林俐[1] 马强[1] 罗清雅[1] 赵连花[1] 牟江洪[1] 

机构地区：[1]第三军医大学大坪医院野战外科研究所病理科,重庆400042

出　　处：《诊断病理学杂志》2016年第10期730-735,共6页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨成人幕上原始神经外胚叶肿瘤（AS-PNET）的临床病理学特征及鉴别诊断要点。方法收集8例AS-PNET的临床及影像学资料,观察其病理学形态及免疫表型,并复习相关文献,探讨该肿瘤的病理诊断和鉴别诊断要点。结果患者男性3例,女性5例,发生部位以颞叶、额叶、顶叶为主。临床表现主要为颅内压增高、头痛、恶心呕吐,意识障碍。CT表现为等密度或高密度影,增强后可强化,肿瘤内可见钙化或微出血。MRI显示肿块界限不清楚或清楚,异质信号,增强后不同程度强化,瘤周出现不同程度水肿。镜下肿瘤组织与周围脑组织边界不清。瘤细胞主要由未分化的小细胞构成,细胞圆形、短梭形,胞质少,核浆比高,核深染,核仁不明显,核分裂象易见。1例可见瘤巨细胞,微血管增生明显,肾小球样血管;2例黏液背景下部分区域可见微囊结构形成,局部可见菊形团;2例局部区域出现少突细胞瘤样分化;2例局部区域出现胶质分化。免疫组化：CD56、Syn、β-tublin、MAP-2、INI-1、Fli-1（4/8）和Olig-2（2/8）（＋）,CD99（3/8）弱（＋）,nestin（7/8）部分细胞（＋）,Neu N（1/8）局灶（＋）;胶质分化区域GFAP（2/8）（＋）,未分化小细胞区域（-）;Cg A、EMA、C-myc（-）;p53（4/8）核弥漫（＋）,Ki-67阳性指数30%～80%。结论 AS-PNET罕见,其确诊需结合临床、影像学、组织病理学及免疫组化等进行综合分析。Objective Investigate clinicopathologic features and differential diagnosis of adult supratentorial primitive neuroectodermal tumors（ AS-PNET）. Methods The clinical and radiological data of eight cases of AS-PNET were collected,their pathological and immunohistochemical features analyzed,and the tumor ＇s pathological diagnosis and differential diagnosis discussed based on related literature review. Results The 3 cases were male and 5 cases were female. The lesions were mostly located in the temporal lobe,frontal lobe and parietal lobe. The most frequent symptoms were increased intracranial pressure, headache, nausea and vomiting, disturbance of consciousness. On computed tomography,these lesions appeared as isodense or hyperdense,enhanced,and calcification or hemorrhage in the tumor. On magnetic resonance imaging（ MRI）,these tumors appeared as well-delimited or ill-defined,inhomogeneous,and variably contrast-enhanced lesions with peritumoral edema. Histologically,the boundary of tumor was ill-defined,the highly cellular tumor consisted of undifferentiated cells with small round to oval hyperchromatic nuclei surrounded by scanty cytoplasm,with some mitoses. Giant cell,significant microvascular proliferation and glomeruloid were seen in one case,mucous background,microcapsule structure,rosettes were seen in two cases,oligodendrocyte cell tumor-like differentiation in 2cases,glial differentiation in 2 cases. There were many cells immunoreactive to Syn,β-Tublin,CD56,MAP-2,INI-1 in all cases. Fli-1 and P53 were positive in four cases,Nestin was positive in seven cases,Olig-2 and GFAP were focally positive in two cases. CD99（ 3 /8） was weakly positive in three cases. Neu N was focally positive in one case. None were immune-reactive to Cg A,EMA,c-Myc. Ki-67 index was 30-80%. Conclusion AS-PNET is a rare tumor,definite diagnosis depends on integration of clinical and radiological data,pathological features and immunohistochemical findings.

关 键 词：中枢神经系统肿瘤 幕上原始神经外胚叶肿瘤 免疫组化 临床病理特征 

分 类 号：R739.4[医药卫生—肿瘤]