单中心19例复发性颅外恶性生殖细胞肿瘤挽救性治疗经验及回顾性分析

A retrospective single-center study of treatment experience of recurrent extracranial malignant germ cell tumor in 19 children

目的分析复发性颅外恶性生殖细胞肿瘤(MGCTs)患儿的生存率及预后因素,探索复发性颅外MGCTs患儿可行的挽救性治疗手段。方法回顾性研究。研究对象为2010年1月至2020年1月上海儿童医学中心收治的复发性颅外MGCTs患儿,治疗方案包括手术、化疗和放疗。采用Kaplan-Meier生存分析法及Cox风险回归对患儿进行生存率及预后因素分析。结果共收治172例颅外MGCTs患儿,其中21例复发,复发率为12.2%,中位复发时间为治疗结束后11个月,2例复发患儿因不符合入组标准被剔除,最终19例进入统计。19例患儿中男10例,女9例;复发年龄26个月(8~170个月);随访时间57个月(13~122个月)。复发患儿中16例接受挽救性化疗,14例接受手术完整切除,4例患儿接受放疗。复发患儿4年总体生存(4yr-OS)率为(82.5±9.2)%(19例),其中初诊时仅手术未行辅助化疗的患儿,复发后再治疗的4yr-OS率为(92.3±7.4)%(13例);初诊时接受化疗的患儿,复发再治疗后4yr-OS率为(60.0±21.9)%(6例),二者比较差异有统计学意义(P=0.002)。单因素及Cox多因素回归分析均提示,化疗3个疗程后甲胎蛋白水平未恢复正常者明显影响最终生存率。结论颅外MGCTs患儿复发后通过完整手术切除、化疗、放疗等综合治疗后仍有较高的生存率,初治时仅手术未行辅助化疗者,复发再治疗后的生存率明显优于初治时已接受化疗的患儿。

Objective To study the survival and prognostic factors for the recurrent extracranial malignant germ cell tumors(MGCTs)in children,and to explore feasible salvage treatment.Methods A retrospective study.Pediatric patients with recurrent extracranial MGCTs diagnosed in Shanghai Children′s Medical Center between January 2010 and January 2020 were retrospectively recruited.Comprehensive treatment regimens included surgery,chemotherapy and radiation.Kaplan-Meier survival analysis and Cox regression model were employed to analyze the survival and prognostic factors for children with recurrent extracranial MGCTs.Results A total of 172 children with extracranial MGCTs were treated,including 21(12.2%)recurrent cases.The median time of MGCT recurrence after the end of the first treatment was 11 months.Finally,19 patients were recruited after excluding 2 non-eligible cases,including 10 boys and 9 girls with the age at recurrence of 26(8-170)months.The follow-up time was 57(13-122)months.Salvage chemotherapy,complete resection and radiotherapy were performed in 16,14 and 4 patients,respectively.The 4-year overall survival(4yr-OS)rate was(82.5±9.2)%(19 cases).The 4yr-OS rate was significantly higher in patients managed with surgery but without adjuvant chemotherapy at the initial treatment(13 cases)than those managed with chemotherapy at the initial treatment(6 cases)[(92.3±7.4)%vs.(60.0%±21.9)%,P=0.002].Univariant and Cox multivariant regression analyses showed that failure to achieve the normal range of alpha fetoprotein after 3 cycles of chemotherapy significantly influenced the survival of recurrent extracranial MGCTs.Conclusions For patients with recurrent extracranial MGCTs,comprehensive treatment approaches like complete surgical resection,chemotherapy,and radiotherapy offer a favorable survival rate.Specifically,recurrent and re-treated patients who initially received surgery alone without adjuvant chemotherapy have a higher survival rate compared to those who received chemotherapy during the initial treatment.