Objective: The aim was to describe the clinical features of extracranial germ cell tumors (GCTs) in pediatrics and study the clinical risk factors related to survival for malignant germ cell tumors (MGCTs) in order to optimize therapeutic options. Methods: The clinical data of children with extracranial GCTs in three children’s medical centers in Shanghai were retrospectively analyzed. Results: In total, 1007 cases of extracranial GCTs diagnosed between 2010 and 2019 were included in this study, including teratomas (TERs) 706 (70.11%) and MGCTs 301 (29.89%). There were twice as many TER cases as MGCT cases. Approximately 50% of children with GCTs were <3 years old (43.39% for TERs, 67.13% for MGCTs). GCTs in children of different ages show differences in tumor anatomical locations and pathological subtypes. The 5-year event-free survival (EFS) and overall survival (OS) of all patients with MGCTs were 82.33% (95% CI, 77.32%, 86.62%) and 94.13% (95% CI, 90.02%, 96.69%), respectively. The multivariate Cox regression analysis identified a primary site in the mediastinum and alpha fetoprotein (AFP) levels ≥10,000 ng/mL as independent adverse prognostic factors (p< 0.0.0001, χ2= 23.6638,p= 0.0225, χ2= 5.2072.). There were no significant differences in OS among children receiving various chemotherapy regimens, such as the BEP, PEB, JEB and other regimens (VBP/VIP and AVCP/IEV) (p< 0.05). Conclusions: The clinical features of GCTs in Chinese pediatrics are similar to those reported in children in Europe and America. The age distribution of pathological types and primary sites in GCTs reflect the developmental origin of type I and type II GCTs transformed from mismigration primordial germ cells (PGCs). Optimizing the current platinum-based chemotherapy regimens and exploring the treatment strategies for MGCTs of the mediastinum are future research directions.
目的：旨在描述儿童颅外生殖细胞肿瘤（GCTs）的临床特征，并研究恶性生殖细胞肿瘤（MGCTs）生存相关的临床危险因素，以优化治疗方案。方法：回顾性分析上海三家儿童医疗中心儿童颅外GCTs的临床资料。结果：本研究共纳入2010年至2019年间诊断的1007例颅外GCTs，其中畸胎瘤（TERs）706例（70.11%），MGCTs 301例（29.89%）。TER病例数约为MGCT的两倍。约50%的GCT患儿年龄<3岁（TERs为43.39%，MGCTs为67.13%）。不同年龄儿童的GCTs在肿瘤解剖部位和病理亚型上存在差异。所有MGCT患者的5年无事件生存率（EFS）和总生存率（OS）分别为82.33%（95% CI，77.32%，86.62%）和94.13%（95% CI，90.02%，96.69%）。多因素Cox回归分析确定纵隔原发部位和甲胎蛋白（AFP）水平≥10,000 ng/mL为独立不良预后因素（p<0.0001，χ²=23.6638；p=0.0225，χ²=5.2072）。接受不同化疗方案（如BEP、PEB、JEB及其他方案（VBP/VIP和AVCP/IEV））的患儿OS无显著差异（p<0.05）。结论：中国儿童GCTs的临床特征与欧美报道的儿童病例相似。GCTs病理类型和原发部位的年龄分布反映了由错误迁移的原始生殖细胞（PGCs）转化而来的I型和II型GCTs的发育起源。优化当前以铂类为基础的化疗方案，探索纵隔MGCTs的治疗策略是未来的研究方向。
肿瘤(癌症)患者之家