The treatment and prognosis of primary pediatric intracranial nongerminomatous malignant germ cell tumors

Objective 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. Key words: Brain neoplasms; Child; Treatment; Prognosis; Nongerminomatous malignant germ cell tumors