目的 分析原始神经外胚层肿瘤 (primitive neuroectodermal tumor, PNET) 术后患者的综合治疗疗效及影响预后的相关因素. 方法 回顾性分析我院 2008 年 6 月-2014 年 12 月收治的 41 例 PNET 术后患者, 其中男 24 例, 女 17 例, 年龄 4~ 65 岁, 中位年龄 24 岁. 中枢性 PNET 6 例, 外周性 PNET 35 例, 肿瘤原发灶位于四肢 8 例, 颅内及头面部 10 例, 椎管内及椎旁 4 例, 胸部 5 例, 腹腔 7 例, 盆腔 7 例. 单纯手术 2 例, 手术+ 化疗 22 例, 手术+ 放疗 4 例, 手术+ 化疗+ 放疗 13 例. 结果 全组中位生存期为 38 个月, 1 年, 3 年, 5 年总生存率分别为 87.8%, 51.9%, 37.1%, 1 年, 2 年无进展生存率分别为 41.3%, 29%. 26 例术后局部复发 (63.4%). 单因素分析显示, 术后局部复发, 手术切除不完整是影响 PNET 患者预后的不良因素; 而局部放疗可以改善 PNET 术后患者的预后. 多因素分析显示, 手术完整切除和手术+ 化疗+ 放疗联合治疗为 PNET 术后患者长期生存的独立影响因素 (P= 0.006, 0.013). 结论 PNET 治疗仍推荐手术+ 化疗+ 放疗的综合治疗方案. 术后局部复发严重影响患者的预后. 手术完整切除的患者预后较好.

Abstract: Objective To evaluate the multimodal treatment outcomes and prognostic factors of patients with primitive neuroectodermal tumor (PNET) after surgery. Methods Clinical data about 41 patients who were diagnosed with PNET in Chinese PLA General Hospital from June 2008 to December 2014 were retrospectively analyzed. There were 24 males and 17 females with the median age of 24 years (ranging from 4 to 65 years). Of the 41 cases, 6 patients were cPNET and 35 patients were pPNET. Original sites included limbs (n= 8), brain and head (n= 10), spinal canal and paravertebral site (n= 4), chest (n= 5), abdomen (n= 7) and pelvis (n= 7). All patients had undergone surgical treatment, including 2 cases with surgery alone, 22 patients with surgery and chemotherapy, 4 patients with surgery and radiotherapy, and 13 patients with surgery, radiotherapy and chemotherapy. Results The overall median survival time was 38 months. The overall survival (OS) rates of 1-year, 3-year and 5-year were 87.8%, 51.9% and 37.1%, respectively. The progression-free survival (PFS) rates of 1-and 2-year were 41.3% and 29%. Twenty-six patients had relapse of this disease (63.4%). Univariate analysis showed that gross tumor resection and recurrence after surgery were negative factors of the prognosis of PNET patients. Local radiotherapy could improve the prognosis of these patients. Multivariate analysis concluded that gross tumor resection and surgery combined with chemoradiotherapy were independent prognostic factors (P= 0.006, P= 0.013). Conclusion Surgery combined with chemoradiotherapy is still a recommended multimodal treatment regimen for PNET patients. Recurrence after surgery affects the survival of PNET patients severely, and radical resection of tumor can improve the prognosis of PNET patients treated with surgery.