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目的 分析外周型原始神经外胚层肿瘤(pPNET)的治疗及预后.方法 收集2006年3月至2015年4月间治疗的47例pPNET患者临床资料,分析不同治疗方式对pPNET患者生存的影响.结果 全组pPNET患者的中位总生存时间(OS)为23.5个月,5年生存率为33.8%.26例接受根治性手术的pPNET患者中,中位OS为70.4个月,5年生存率为54.4%;中位无病生存时间(DFS)为23.1个月,5年无病生存率为34.4%.有16例患者出现术后复发或转移,术后2年内复发转移13例,占81.2%(13/16).根治性手术后辅助化疗和未辅助化疗患者的中位 OS差异有统计学意义(P=0.04),但中位DFS的差异无统计学意义(P=0.057).根治性手术后辅助放疗和未辅助放疗患者的中位OS和中位DFS差异均无统计学意义(P=0.619, P=0.191),局部复发率差异无统计学意义(P=0.40).34例接受一线姑息化疗患者的中位无进展生存时间(PFS)为3.2个月,中位OS为10.7个月, 1年生存率为48.0%,2年生存率为17.8%.一线姑息化疗的有效率为53.1%,临床获益率为75.0%.含铂方案组患者的中位PFS为3.3个月,中位OS为14.5个月;非铂方案组患者的中位PFS为2.7个月,中位OS为10.3个月,两组患者的PFS和OS差异均无统计学意义(P=0.973,P=0.671).姑息手术及放疗未能改善pPNET患者的生存情况.结论 化疗、放疗和手术在内的综合治疗是早期pPNET患者的标准治疗模式,辅助化疗可明显改善其预后.化疗是晚期pPNET患者的主要治疗方法,铂类药物可作为化疗的一种选择.

Objective To evaluate the treatment and prognosis of peripheral primitive neuroectodermal tumor(pPNET). Methods From March 2006 to April 2015,47 patients with pPNET who had undergone chemotherapy in our hospital were enrolled. The clinical data and survival information of these patients were collected and interpreted retrospectively to analyze the effect of each treatment on the survival of patients. Results The median overall survival(OS)for whole group was 23.5 months,and 5-year survival rate was 33.8%. In the patients who underwent radical surgery,the median OS was 70.4 months,the 5-year survival rate was 54.4%,the median disease-free survival(DFS)was 23.1months,and 5-year DFS rate was 34.4%. Sixteen patients had recurrences or metastasis after surgery. Eighty-one percent of them(13/16) occurred within 2 years after surgery. The difference of median OS between patients who got adjuvant chemotherapy and those who did not was statistically significant(P=0.04). But the difference of median PFS between these two groups was not statistically significant(P= 0.057). There was no statistically significant difference for median OS(P=0.619)and median DFS(P=0.191)between patients who got adjuvant radiotherapy and those who did not. The recurrence rate between these two groups was not statistically significant(P=0.40). The median OS and PFS for 34 patients who received first-line palliative chemotherapy was 10.7 months and 3.2 months.1-year and 2-year survival rates were 48.0% and 17.8%.The response rate and clinical benefit rate for first-line chemotherapy was 53.1% and 75.0%. The median PFS and OS for patients who received platinum-based regimens were 3.3 months and 14.5 months. The median PFS and OS for patients who got non-platinum regimens were 2.7 months and 10.3 months. There was no significant difference of PFS and OS between platinum-based and non-platinum regimens. Palliative surgery and radiotherapy did not improve the OS of pPNET this cohort. Conclusions Comprehensive treatment including chemotherapy,radiotherapy and surgery is the standard treatment model for early pPNET patients. Adjuvant chemotherapy significantly improved the overall survival of early pPNET patients. Chemotherapy is the main treatment for patients with advanced pPNET.Platinum-based chemotherapy seem to be a good option.