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目的:分析中国人群中嗅神经母细胞瘤(esthesioneuroblastoma,ENB)的治疗疗效及预后因素。方法:以“嗅神经母细胞瘤”为关键字在CNKI和万方数据库中进行检索,检索时限为1981-2015年。从病例报道和病例分析研究中共收集187例ENB病例,全部按Kadish分期标准进行分期,Kaplan-Meier法计算生存率。结果:研究共纳入187位ENB患者,中位随访时间为24个月(1~204个月),患者的2年总生存率(overall survival,OS)为75.4%,2年无病生存率(disease-free survival,DFS)为52.9%。Kadish临床分期A期、B期、C期患者的2年OS分别为92%、96%、62.5%(χ2=21.724,P=0.000),2年DFS分别为72%、66%、42.9%(χ2=14.874,P=0.000)。以手术联合放疗为主的综合治疗模式和其他治疗模式(包括单纯放疗、单纯手术、放疗+化疗、姑息治疗)的2年OS分别为84.5%和60.6%(χ2=15.283,P=0.000),2年DFS分别为63.8%和35.2%(χ2=15.907,P=0.003)。有颈部淋巴结转移(N+)和无颈部淋巴结转移(N0)的2年OS分别为53.1%和80%(χ2=12.614,P=0.000),2年DFS分别为34.4%和56.8%(χ2=8.722,P=0.003)。复发是治疗失败的主要模式,其中以远处转移预后最差。在187例患者中共有64例患者出现复发,复发率为34.2%,其中C期49例(43.8%),增加化疗并不能减少C期患者的复发率,但颈部淋巴结清扫能减少N+患者的复发率。结论:手术联合放疗为主的综合治疗仍是目前ENB最佳的治疗模式,影响生存的主要因素为Kadish分期、治疗方式、复发和淋巴结情况,化疗的影响仍需进一步深入研究。
Objective: To analyze the curative effect and prognostic factors of esthesioneuroblastoma (ENB) in Chinese population. Methods: We searched the CNKI and Wanfang databases with the keywords “olfactory neuroblastoma”, and the retrieval time was from 1981 to 2015. A total of 187 cases of ENB were collected from case reports and case studies. All cases were staged by Kadish staging and Kaplan-Meier method was used to calculate the survival rate. Results: A total of 187 ENB patients were recruited. The median follow-up time was 24 months (range, 1 to 204 months). The 2-year overall survival (OS) was 75.4% and the 2-year disease- disease-free survival (DFS) was 52.9%. The 2-year OS of Kadish patients with clinical stage A, B and C were 92%, 96% and 62.5% respectively (χ2 = 21.724, P = 0.000). The 2-year DFS were 72%, 66% and 42.9% χ2 = 14.874, P = 0.000). The 2-year OS was 84.5% and 60.6% (χ2 = 15.283, P = 0.000) for the combined treatment modalities and other treatment modalities including radiotherapy, surgery alone, radiotherapy + chemotherapy and palliative treatment. The 2-year DFS was 63.8% and 35.2%, respectively (χ2 = 15.907, P = 0.003). The 2-year OS was 53.1% and 80% respectively (χ2 = 12.614, P = 0.000) with two cases of neck lymph node metastasis (N +) and no neck lymph node metastasis (N0) = 8.722, P = 0.003). Recurrence is the main mode of treatment failure, of which the prognosis of the worst distant metastasis. Of the 187 patients, a total of 64 patients had a relapse with a recurrence rate of 34.2%, of which 49 (43.8%) had stage C chemotherapy. Chemotherapy did not reduce the recurrence rate in patients with stage C disease, but cervical lymph node dissection reduced N Recurrence rate. Conclusion: The combination of surgery and radiotherapy is still the best treatment mode for ENB at present. The main factors influencing survival are Kadish staging, treatment modality, recurrence and lymph node status. The influence of chemotherapy remains to be further studied.