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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。
Objective To analyze the comprehensive therapeutic effect and related factors that affect the prognosis of patients with primitive neuroectodermal tumor (PNET). Methods A retrospective analysis of 41 cases of postoperative PNET patients admitted to our hospital from June 2008 to December 2014, including 24 males and 17 females, aged from 4 to 65 years, with a median age of 24 years. There were 6 cases of central PNET, 35 cases of peripheral PNET, 8 cases of primary tumor in limbs, 10 cases of intracranial and head and face, 4 cases of intraspinal and paravertebral, 5 cases of chest, 7 cases of abdominal cavity and 7 cases of pelvic cavity. Simple surgery in 2 cases, surgery + chemotherapy in 22 cases, surgery + radiotherapy in 4 cases, surgery + chemotherapy + radiotherapy in 13 cases. Results The median overall survival was 38 months. The 1-year, 3-year and 5-year overall survival rates were 87.8%, 51.9% and 37.1% respectively. The 1-year and 2-year progression-free survival rates were 41.3% and 29% . 26 cases of local recurrence (63.4%). Univariate analysis showed that local recurrence and incomplete surgical resection were the adverse factors that affected the prognosis of PNET patients. Local radiotherapy could improve the prognosis of PNET patients. Multivariate analysis showed that the combined operation of complete resection and surgery + chemotherapy plus radiotherapy was an independent influencing factor for the long-term survival of PNET patients (P = 0.006,0.013). Conclusions PNET treatment is still recommended comprehensive treatment of surgery + chemotherapy + radiotherapy. Postoperative local recurrence seriously affects the prognosis of patients. Patients with complete resection of the surgery have a better prognosis.