外周性原始神经外胚层肿瘤18例

Peripheral primitive neuroectodermal tumors in 18 cases

目的探讨外周性原始神经外胚层肿瘤（pPNET）患者的临床表现、病理学特征、诊断标准、治疗及预后。方法回顾性分析我院18例pPNET患者的临床资料，光镜下：肿瘤细胞呈现形态均一的小圆细胞，被纤维组织分隔成小叶状，部分病例可见典型Homer—Wright菊形团结构；免疫组化：CD99、Syn、NSE、Vim呈高表达状态。结果单纯手术组6例，中位生存期17个月，综合治疗组12例，中位生存期22个月，两组比较差异有统计学意义（P〈0．05）；全组总生存期7～36个月，中位生存期20个月，1年生存率为89％，2年生存率为39％。预后因素中，患者初诊时是否有转移及治疗方法为pPNET患者生存的独立影响因素（P〈0．01）。结论pPNET多发生于青少年，具有一定的病理和免疫组化特点，易复发转移，预后差，尤其是初诊时已发生转移的患者预后更差，采用综合治疗能够提高生存率。

Objective To discuss the clinical and pathological features, diagnostic standards, therapy and prognosis of peripheral primitive neuroeetodermal tumors （pPNET） so as to improve diagnostic and therapeutic levels. Methods Clinical data of 18 cases of pPNET were analyzed retrospectively. Histopathologieally, tumors were characterized by uniform small round or oval cells and nest - like or dense sheets structures surrounded by spare fibrovascular stroma. The Homer - Wright rosettes were observed in some cases. Immunohistochemically, the tumor cells showed higher positive rates for CD99, Syn, NSE and Vim. Results Six patients received surgical alone and twelve patients received chemotherapy and/ or radiotherapy after surgery. The median survival time for two groups was 17 months and 22 months, resneetivelv. The difference was significant （ P 〈 0.01 ）. The overall survival time was 7 - 36 months and the median survival time was 20 months. 1 - year survival rate was 89%, 2 - year survival rate was 39%. Both the ways of treating and metastasis in preliminary diagnosis were important factors affecting the prognosis. Conclusions PNET mainly attacks teengers and has poor prognosis. The clinical pathological features and immunohistoehemical expression can help to diagnose the disease. Surgery combined with chemotherapy and/or radiotherapy was the best choice.