旨在评估资源匮乏国家主要在三级护理中心接受治疗的儿童颅外恶性生殖细胞肿瘤 (MGCT) 的临床病理特征、治疗和结果。前瞻性维护的 14 岁以下儿童接受治疗的数据对 1994 年 5 月至 2023 年 1 月的颅外 MGCT 患者特征、管理、无事件生存期 (EFS) 和总生存期 (OS) 以及影响生存的因素进行了分析。事件定义为死亡、复发和进展。进行多变量逻辑回归分析以确定独立预测不良结果的因素。纳入了 177 名儿童（37% 男性），发病时中位 (IQR) 年龄为 30 个月（范围 2-168 个月）。该队列由 87 例 (49%) 性腺外病例和 90 例 (51%) 性腺病例组成。 48 例 (27%) 患者就诊时疾病已发生转移，其中肺部是最常见的部位。 119 例（67%）接受了新辅助化疗（NACT），最终 162/177 例（92%）接受了原发肿瘤切除术。内胚窦肿瘤 (EST) 是 141 名儿童 (73%) 中最常见的组织学亚型。 22 名 (12%) 患者死亡，5 年 OS 为 84.7% (95% CI 78.3-91.1)。 25 名患者出现复发，另外 5 名患者出现进展，5 年 EFS 为 69.9%（95% CI 62.5-77.3）。 III 期 (p = 0.05)、IV 期 (p = 0.006) 和性腺外部位 (p = 0.05) 与较差的 EFS 显着相关。患有 MGCT 的儿童具有良好的结果，5 年 OS 为 84.7%，EFS 为69.9%。 III 期和 IV 期疾病以及性腺外部位是不良结果的独立预测因素。© 2024。作者获得 K C Chaudhuri 博士基金会的独家许可。

To assess the clinico-pathological features, management and outcomes, amongst extracranial malignant germ cell tumors (MGCTs) in children treated primarily at a tertiary care center in a resource-challenged nation.The prospectively maintained data for children below 14 y of age treated for extracranial MGCT from May 1994 to January 2023 was analyzed for patient characteristics, management, event-free survival (EFS) and overall survival (OS) and factors effecting survival. Events was defined as death, recurrence and progression. Multivariate logistic regression analysis was performed to identify the factors independently predicting unfavorable outcomes.One hundred and seventy-seven children (37% males) with a median (IQR) age at presentation of 30 mo (range 2-168 mo) were included. The cohort consisted of 87 (49%) extra-gonadal and 90 (51%) gonadal cases. Disease was metastatic at presentation in 48 (27%) with lungs being the most common site. Neoadjuvant chemotherapy (NACT) was given to 119 (67%) and finally 162/177 (92%) had undergone resection of the primary tumor. Endodermal sinus tumor (EST) was the commonest histological subtype in 141 children (73%). Twenty-two (12%) patients had died giving a 5-y OS of 84.7% (95% CI 78.3- 91.1). Recurrence occurred in 25 patients, and an additional 5 patients had progression giving a 5-y EFS of 69.9% (95% CI 62.5- 77.3). Stage III (p = 0.05), Stage IV (p = 0.006) and extra-gonadal site (p = 0.05) were significantly associated with poorer EFS.Children with MGCT have a favorable outcome with 5-y OS of 84.7% and EFS of 69.9%. Stage III and IV disease and extra-gonadal sites were independent predictors of a poor outcome.© 2024. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.