外周原始神经外胚叶肿瘤的临床病理

目的探讨外周原始神经外胚叶肿瘤(pPNET)的病理学特征、诊断及鉴别诊断。方法应用免疫组化方法,对我院1996年~2006年间10例病理诊断为外周原始神经外胚叶肿瘤患者的年龄、性别、发病部位、病理特征及免疫组化表型进行回顾性临床病理分析。结果pPNET好发于儿童及青年,肿瘤沿身体长轴分布,细胞呈小圆形,部分为梭形,密集呈巢状排列,由纤维结缔组织分割成分叶状或腺泡状,常形成Homer-Wrigh菊形团结构,血管丰富,易出血及坏死。免疫组化CD99及至少2项神经性标记阳性。结论pPNET是来源于神经外胚叶组织的小细胞恶性肿瘤,免疫组化染色对诊断和鉴别诊断是必不可少的。

Clinic Pathological of Peripheral Primitive Neuroectodermal Tumor

Objective To study the clinic opathological features of peripheral primitive neuroectodermal tu-mor(pPNET),and improve the diagnosis and differential diagnosis of pPNET. Methods 10 cases of pPNET in our hos-pital from 1996 to 2006.The age,gender,the location of affection,clinical symptoms pathological characteristics and ex-pression of immunohistochemistry were analyzed retrospectively. Results pPNET usually occurs in bone or soft tissue,it was found in children and young adults.The affection usually distributes along the long axis of the body.Histologically,the tumor is consisted of sheets of undifferentiated small round cells or oval cells forming abortive Homer-Wright rosettes.I-mmunohistochemical study revealed that the tumor cells expressed positive CD99. Conclusion pPNET is a malignant small cell tumor origin from neuroectoderm.Making diagnosis of pPNET depends on its typical pathological characteris-tics,histological and immunohistochemical features,it is important to distinguish other small round tumors.