摘要目的 探讨儿童颅内原发性非生殖细胞瘤性恶性生殖细胞肿瘤(NGMGCTs)的治疗方法及其预后.方法 回顾性纳入2006年1月至2019年3月上海交通大学医学院附属新华医院小儿神经外科收治的43例儿童颅内原发性NGMGCTs患者,均行手术切除肿瘤.术后35例采用铂剂为基础的化疗和(或)放疗.随访方法包括临床随访和影像学随访.采用Kaplan-Meier方法和多因素Cox回归分析探讨患儿生存期的影响因素.结果 43例患儿中,23例(53.5％)达到肿瘤全切除,14例(32.6％)行次全切除,6例(13.9％)予部分切除.未成熟畸胎瘤、混合性生殖细胞肿瘤以及卵黄囊瘤的中位无进展生存期(PFS)分别为4.86年、4.24年以及0.79年(P=0.002),中位总体生存期(OS)分别为6.68年、5.88年以及1.03年(P<0.001).2例绒毛膜癌患儿中,1例死于围手术期并发症,1例术后55 d死于肿瘤复发.多因素Cox回归分析结果显示,肿瘤病理学类型(HR:0.26,95％CI:0.05～1.28,P=0.010)、血清甲胎蛋白(AFP)水平(HR:0.06,95％CI:0.01～0.55,P=0.013)以及是否进行完整的辅助治疗(HR:3.38,95％CI:0.99～11.56,P=0.050)为影响患儿OS的危险因素;而术后是否进行完整的辅助治疗(HR:4.72,95％CI:1.54～14.47,P=0.007)和病理学类型(HR:0.62,95％CI:0.19～2.05,P=0.010)为影响患儿PFS的危险因素.结论 NGMGCTs患儿中,未成熟畸胎瘤和混合性生殖细胞肿瘤预后最好,卵黄囊瘤患儿的预后次之,绒毛膜癌患儿的预后最差.手术切除结合辅助治疗是治疗NGMGCTs的主要方式.肿瘤的病理学类型是影响NGMGCTs患儿的预后因素.术后接受完整的辅助治疗能改善NGMGCTs的预后,而血清AFP>100 ng/ml为NGMGCTs预后不良的一个因素.

abstractsObjective To investigate the treatment and prognosis of primary pediatric intracranial nongerminomatous malignant germ cell tumors ( NGMGCTs ) . Methods A total of 43 children with primary intracranial NGMGCTs who underwent tumor resection at Department of Pediatric Neurosurgery, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine from January 2006 to March 2019 were enrolled. Only 35 cases received adjuvant chemotherapy and/or radiotherapy. The clinical and imaging follow-up were performed after operation. Kaplan-Meier method and multiple Cox regression analysis were used to analyze influencing factors of overall survival (OS) and progression-free survival (PFS). Results Of 34 patients,total resection were achieved in 23 cases (53. 5％),subtotal resection in 14 cases (32. 6％) and partial resection in 6 cases ( 13. 9％). The median PFS of patients with immmature teratomas,mixed germ cell tumors and yolk sac tumors were 4. 86 years, 4. 24 years and 0. 79 years, respectively(P=0. 002). The median OS of patients with immmature teratomas,mixed germ cell tumors and yolk sac tumors were 6. 68 years,5. 88 years and 1. 03 years, respectively(P<0. 001). One patient with choriocarcinoma died of perioperative complication, the other died of postoperative recurrence. Cox regression analysis showed that pathology(HR:0. 26,95％CI:0. 05 -1. 28,P=0. 010),serum AFP(HR:0. 06,95％CI:0. 01-0. 55,P=0. 013) and complete adjuvant therapy( HR:3. 38,95％CI:0. 99-11. 56, P=0. 050)are influencing factors of OS in patients with NGMGCTs,while complete adjuvant therapy(HR:4. 72,95％CI:1. 54-14. 47,P=0. 007) and pathology(HR:0. 62,95％CI:0. 19-2. 05,P=0. 010) was associated with PFS. Conclusions For NGMGCTs,the prognosis of immature teratoma and mixed germ cell tumors were the best, followed by yolk sac tumors, while choriocarcinoma was the worst. NGMGCTs patients were treated with surgery and adjuvant chemotherapy and/or radiotherapy. The prognosis of NGMGCTs was associated with pathology. Complete postoperative adjuvant therapy may improve the prognosis of primary pediatric NGMGCTs,while serum AFP>100 ng/ml may predict poor outcome.