背景：恶性生殖细胞肿瘤（MGCT）可以在颅内或颅内发展。这些患者在化疗后可能会发展为畸胎瘤综合征（GTS）。关于MGCT患儿GTS的临床特征和结局的报道有限。
方法：我们回顾性收集了数据，包括我们系列中5例患者的临床特征和结局,以及通过MGCTs的文献回顾选择的93例儿科患者.本研究旨在分析MGCT发展为GTS的儿科患者的生存结果和后续事件的危险因素。
结果：性别比例为1.09（男/女）。总的来说,52例(53.1%)患者有颅内MGCTs。与颅外GCT患者相比，颅内GCT的患者更年轻，主要是男孩，MGCT和GTS之间的间隔较短，并且GTS主要发生在初始位点(所有p<0.001)。95例患者（96.9%）存活。然而,GTS复发（n=14），GTS进展（n=9），MGCT复发(n=19)导致无事件生存率(EFS)大幅下降。多变量分析表明，这些事件的唯一重要危险因素是GTS切除不完全以及GCT和GTS的位置不同。没有任何风险的患者的5年EFS为78.8%±7.8%，而那些有任何风险的人有41.7%±10.2%(p<0.001)。
结论：对于具有高风险特征的患者，应尽一切努力密切监测，完全删除，并在病理上证明任何新出现的肿块，以指导相关治疗。可能需要进一步研究将风险因素纳入治疗策略以优化辅助治疗。
Malignant germ cell tumors (MGCTs) can develop either extracranially or intracranially. Growing teratoma syndrome (GTS) may develop in these patients following chemotherapy.
Reports on the clinical characteristics and outcomes of GTS in children with MGCTs are limited.
We retrospectively collected the data, including the clinical characteristics and outcomes of five patients in our series and 93 pediatric patients selected through a literature review of MGCTs. This study aimed to analyze survival outcomes and risk factors for subsequent events in pediatric patients with MGCTs developing GTS.
The sex ratio was 1.09 (male/female). In total, 52 patients (53.1%) had intracranial MGCTs. Compared with patients with extracranial GCTs, those with intracranial GCTs were younger, predominantly boys, had shorter intervals between MGCT and GTS, and had GTS mostly occurring over the initial site (all p < 0.001). Ninety-five patients (96.9%) were alive. However, GTS recurrence (n = 14), GTS progression (n = 9), and MGCT recurrence (n = 19) caused a substantial decrease in event-free survival (EFS). Multivariate analyses showed that the only significant risk factors for these events were incomplete GTS resection and different locations of GCT and GTS. Patients without any risk had a 5-year EFS of 78.8% ± 7.8%, whereas those with either risk had 41.7% ± 10.2% (p < 0.001).
For patients with high-risk features, every effort should be made to closely monitor, completely remove, and pathologically prove any newly developed mass to guide relevant treatment. Further studies incorporating the risk factors into treatment strategies may be required to optimize adjuvant therapy.
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