外周性原始神经外胚层瘤的临床病理及免疫组织化学特征  

Clinicopathological and immunohistochemical features of peripheral primitive neuroectodermal tumors

作　　者：赵尔增[1] 张建中[1] 景青萍[1] 张铭[1] 

机构地区：[1]解放军306医院病理科,北京100101

出　　处：《中华肿瘤防治杂志》2006年第13期994-997,共4页Chinese Journal of Cancer Prevention and Treatment

摘　　要：目的:探讨外周性原始神经外胚层瘤(peripheral primitive neuroectodrmaltumour,pPNET)的临床病理及免疫组织化学特征。方法:对8例pPNET进行组织形态学观察和组化及免疫组化染色,并复习病史。结果:8例pPNET患者临床表现为进行性增大的局部肿块及其继发症状。影像学检查显示病变为密度增高的软组织影或溶骨性骨组织破坏。光镜下,瘤细胞为形态均一的小圆形或卵圆形,被纤维结缔组织所分隔成实性片状或巢状。部分病例可见典型Homer-Wright菊形团及/或菊形团排列趋势,1例伴明显神经细胞分化。免疫组化:8例pPNET中,CD99、Syn和NSE 3种标记阳性率较高,分别为7/8、6/8及7/8,Vim和CgA分别为5/8。Syn和NSE 2项阳性6例,CD99和Syn或NSE 2项阳性7例,3项均阳性6例。结论:pPNET是一种好发于青少年、形态原始和少见的高度恶性肿瘤,CD99、NSE及Syn的联合表达有助于pPNET的诊断和鉴别诊断。OBJECTIVE： To explore the clinicopathological and immunohistochemical features of peripheral primitive neuroectodermal tumors （pPNET）. METHODS：Eight cases of pPNET were studied by light microscopy study, immunohistochemistry staining and clinical data review. RESULTS： Two cases were male and six were female. Eight cases of pPNET presented as progressively growing mas ses and its complication included local pain, functional disorder and pathologic fracture. A soft tissue density mass lesion or osteolytic destructive lesion were showed on the radiographic film. Histopathlogically, tumors were characterized by uniform small round or oval cells and nest-like or dense sheets structures surrounded by sparse fibrovascular stroma. The Homer-Wright rosettes and/or the tendencies were observed in some cases, accompanied with nerve cells differenti ation in some areas of the tumor in 1 case. Immunohistochemically, the tumor cells showed higher positive rates for Syn, NSE and CD99 in （7/8, 6/8 and 7/8 respectively）, lower for vimentin and CgA in （5/8 respectively）. The tumor cells were positive for CD99, Syn and NSE in 6 cases, positive for Syn and NSE in 6 cases and positive for CD99 and Syn or NSE in 7 cases. CONCLUSIONS： The pPNET is a rare, highly malignant, primitive small round-cell sarcoma. Immunohistochemically, the expression of CD99 , Syn and NSE plays an important role in the diagnosis and differential diagnosis of it.

关 键 词：原始神经外胚层瘤 骨/软组织 临床病理 免疫组织化学 

分 类 号：R739.41[医药卫生—肿瘤]