儿童颅内原发性非生殖细胞瘤性生殖细胞肿瘤疗效分析

目的 探讨儿童颅内原发性非生殖细胞瘤性生殖细胞肿瘤（NGGCT）的临床特征,及其预后影响因素。方法 回顾性分析2008年11月至2019年6月在本中心接受放疗的40例NGGCT患儿的临床资料,90%患儿接受全脑全脊髓放疗,所有患儿接受铂类为基础的化疗。采用Kaplan‐Meier曲线分析生存情况,采用log‐rank检验分析预后相关因素。结果 原发部位以松果体、鞍区/鞍上、基底节为主;中位发病年龄为108个月（20~204个月）;中位随访时间33个月（8~131个月）,3、5年总生存率均为82.0%;3、5年无进展生存率分别为78.6%和73.0%。单因素分析发现甲胎蛋白（AFP）升高（P=0.02）,初诊年龄>10岁（P=0.006）,初诊时有转移（P<0.001）,肿瘤病理学类型中含有绒毛膜癌、卵黄囊瘤和/或胚胎性癌成分（P=0.036）是患儿独立的不良预后因素。结论 患儿AFP升高、初诊年龄>10岁、肿瘤转移播散、病理类型是儿童颅内NGGCT独立的不良预后因素。其整体预后差于生殖细胞瘤,需要多学科合作强化治疗以提高生存。

Analysis of therapeutic effect of pediatric patients with intracranial primary non‐germinomatous germ cell tumors

Objective To investigate the clinical features of pediatric patients with intracranial primary non‐germinomatous germ cell tumors (NGGCT) and evaluate the treatment outcomes and prognostic factors of NGGCT. Methods Clinical data of 40 children with NGGCT who were treated with radiotherapy (RT) at our department between November 2008 and June 2019 were retrospectively analyzed. Ninety percent of them received craniospinal irradiation (CSI). All children received platinum‐based chemotherapy. Survival analysis was conducted using the Kaplan‐Meier estimate. The prognostic factors were analyzed by log‐rank test. Results The primary sites were pineal gland, sellar / suprasellar region and basal ganglia. The median age of onset was 108 months (20‐204 months). The median follow‐up time was 33 months (8‐131 months), and the 3‐year and 5‐year overall survival (OS) rates were 82.0%. The 3‐year and 5‐year progression‐free survival (PFS) rates were 78.6% and 73.0%. Univariate analysis showed that increased alpha‐fetoprotein (AFP) (P=0.02), age at first diagnosis>10 years (P=0.006), metastasis at first diagnosis (P<0.001), and the pathological type (choriocarcinoma, yolk sac tumor and / or embryonal carcinoma) (P=0.036) were independent adverse prognostic factors. Conclusions Increased AFP, age>10 years at first diagnosis, tumor metastasis and pathological type were independent adverse prognostic factors of NGGCT. The overall prognosis of NGGCT children is worse than that of their counterparts with germinoma, and multidisciplinary intensive therapy is needed to improve survival.