外周型原始神经外胚叶瘤临床特征及治疗分析

目的 探讨外周型原始神经外胚叶瘤（peripheral primitive neuroectodermal tumors, pPNET）的临床特征及治疗方法。方法 回顾性分析重庆医科大学附属第一医院2005年11月—2014年4月收治的10例pPNET患者的临床病理特征、治疗方案等资料，并用直接计算法计算其生存率。结果 10例患者中以青少年男性居多，免疫组织化学CD99、NSE表达率高。6例行手术治疗，其中5例行术后放疗+化疗。2例行放疗+化疗，1例行化疗+HIFU治疗，1例行中药治疗。治疗后1、2、5年生存率分别为80.0%（8/10）、71.4%（5/7）、40.0%（2/5）。结论 pPNET的临床表现缺乏特异性，以细胞形态学联合免疫组织化学确诊，采取手术、放疗、化疗为主的综合治疗。

Clinical Characteristics and Treatment Methods on Peripheral Primitive Neuroectodermal Tumors Patients

Objective To investigate the clinical characteristics and treatment methods on peripheral primitive neuroectodermal tumors(pPNET) patients. Methods We analyzed retrospectively the clinicopathological characteristics and treatment methods on 10 pPNET patients treated in the First Affiliated Hospital of Chongqing Medical University from November 2005 to April 2014 and calculated the survival rate by direct method. Results Ten cases were mainly male teenagers. Tumor cells strongly expressed CD99 and NSE. Six patients were treated with surgical resection, among which five patients received postoperative radiotherapy and chemotherapy, two patients received radiotherapy and chemotherapy, one patient received chemotherapy and HIFU and one patient was treated with Chinese medicine. The 1-, 2- and 5-year survival rates of the patients were 80.0%(8/10), 71.4%(5/7) and 40.0%(2/5), respectively. Conclusion The clinical characteristics of pPNET patients are lack of specificity. The diagnosis of pPNET mainly depends on histology and immunohistochemistry. The multimodality therapy of pPNET includes surgery, radiotherapy and chemotherapy.