原始神经外胚层肿瘤预后因素分析

Survival and prognostic factors analysis of primitive neuroectodermal tumor

目的探讨原始神经外胚层肿瘤（PNET）的预后及其影响因素。方法收集1998年2月1日至2017年2月1日北京协和医院收治的99例PNET患者的临床和随访资料，应用Kaplan－Meier和Log rank检验进行单因素生存分析，采用Cox回归模型进行多因素生存分析。结果99例患者中，外周性PNET 81例，中枢性PNET 18例。仅行穿刺活检16例，R0切除61例，R1切除4例，R2切除18例。合并化疗72例，合并放疗48例。单纯手术12例，单纯化疗9例，单纯放疗1例。99例患者的中位总生存时间（OS）为14.0个月，中位无进展生存时间（PFS）为8.0个月。1、3、5年总生存率分别为79.2%、63.9%和56.1%，1、3、5年无进展生存率分别为42.7%、25.7%和19.8%。单因素分析显示，淋巴转移情况、手术切除程度、是否合并化疗、化疗周期、放疗剂量与PNET患者的OS有关，性别、年龄、淋巴结转移情况、分期、化疗周期与PNET患者的PFS有关（均P〈0.05）。多因素分析显示，手术切除程度、化疗周期、放疗剂量是PNET患者OS的独立影响因素（RR分别为1.856、0.398和0.408，均P〈0.05），性别是PNET患者PFS的独立影响因素（RR＝0.494，P〈0.05）。结论综合治疗是PNET的主要治疗方法，手术切除程度、化疗周期、以及放疗剂量是影响PNET患者总生存时间的独立预后因素。

ObjectiveTo investigate the clinical features and prognosis of primitive neuroectodermal tumor （PNET）.MethodsThe clinical data of 99 patients with PNET from February 1, 1998 to February 1, 2017 were retrospectively analyzed. Univariate analysis was performed using Kaplan-Meier and Log rank test. Multiviate Cox regression was applied to analyzed independent prognostic factor for patient survival.ResultsAmong the 99 patients, 81 were peripheral PNET（pPNET） and 18 were central PNET （cPNET） . Biopsy was performed exclusively in 16 cases, with R0 resection in 61 cases, with R1 resection in 4 cases, and with R2 resection in 18 cases. Twelve patients underwent surgery only, nine had chemotherapy only, and one received radiotherapy only. There were 72 patients who had combined treatment including chemotherapy, and 48 patients had combined therapy including radiotherapy. The one-year, three-year and five-year overall survival（OS） rates of the 99 PNET patients were 79.2%, 63.9% and 56.1% respectively, and median OS time was 14.0 months. The one-year, three-year and five-year progression free survival （PFS） rates of these patients were 42.7%, 25.7% and 19.8% respectively, and median PFS time was 8.0 months. The univariate analysis revealed that lymph node metastasis, surgical resection, numbers of cycles of chemotherapy and radiotherapy dose were the main factors affecting the OS （all P〈0.05）. Gender, age, lymph node metastasis, staging, and chemotherapy cycles were related to PFS in PNET patients （P〈0.05）. Multivariate analysis showed that the degree of surgical resection, chemotherapy cycle, and radiotherapy dosage were independent influencing factors of OS in PNET patients （risk ratio=1.856, 0.398, and 0.408, respectively, all P〈0.05）, and gender was an independent factor influencing PFS in PNET patients （risk ratio=0.494, P〈0.05）.ConclusionsComprehensive therapy is the main therapy for PNET patients. The surgical resection, cycles of chemotherapy and radiotherapy dosage are independent prognostic factors for patient′s OS.