Abstract：
Adrenocortical carcinoma (ACC) is a rare but aggressive pediatric endocrine tumor. However, there is no recent US national report on the management or outcomes of pediatric ACC. We aimed to examine the clinical characteristics, current management strategies, and outcomes of pediatric ACC.
In this retrospective National Cancer Database study between 2004 and 2019, children (<18 y) with ACC were included. Overall survival was examined by means of Kaplan-Meier method, log-rank tests, and Cox regression modeling.
Seventy-eight children with ACC were included. The median age was 10 y, the median tumor size was 10.2 cm, and 35.9% had metastasis at diagnosis. Most patients underwent surgical treatment (84.6%), 56.4% received chemotherapy, and 7.7% received radiation. The 1-, 3-, and 5-y overall survival rates were 87.0%, 62.0%, and 60.1%, respectively. In unadjusted analysis, surgical treatment was associated with improved overall survival (log-rank test, P < 0.001). In multivariable Cox regression, metastasis at diagnosis was associated with inferior overall survival (hazard ratio: 2.72, 95% confidence interval: 1.15-6.40, P = 0.02), when adjusting for age, tumor size, receipt of surgical treatment, and chemotherapy. In patients with nonmetastatic ACC, increasing age was associated with inferior overall survival (hazard ratio: 1.12, 95% confidence interval: 1.00-1.24, P = 0.04), when adjusting for tumor size, receipt of surgical treatment, and chemotherapy.
Most children with ACC in the USA undergo surgical treatment with about half of these also receiving chemotherapy. Metastasis at diagnosis was independently associated with inferior overall survival; in patients with nonmetastatic ACC, increasing age was independently associated with inferior overall survival.
In this retrospective National Cancer Database study between 2004 and 2019, children (<18 y) with ACC were included. Overall survival was examined by means of Kaplan-Meier method, log-rank tests, and Cox regression modeling.
Seventy-eight children with ACC were included. The median age was 10 y, the median tumor size was 10.2 cm, and 35.9% had metastasis at diagnosis. Most patients underwent surgical treatment (84.6%), 56.4% received chemotherapy, and 7.7% received radiation. The 1-, 3-, and 5-y overall survival rates were 87.0%, 62.0%, and 60.1%, respectively. In unadjusted analysis, surgical treatment was associated with improved overall survival (log-rank test, P < 0.001). In multivariable Cox regression, metastasis at diagnosis was associated with inferior overall survival (hazard ratio: 2.72, 95% confidence interval: 1.15-6.40, P = 0.02), when adjusting for age, tumor size, receipt of surgical treatment, and chemotherapy. In patients with nonmetastatic ACC, increasing age was associated with inferior overall survival (hazard ratio: 1.12, 95% confidence interval: 1.00-1.24, P = 0.04), when adjusting for tumor size, receipt of surgical treatment, and chemotherapy.
Most children with ACC in the USA undergo surgical treatment with about half of these also receiving chemotherapy. Metastasis at diagnosis was independently associated with inferior overall survival; in patients with nonmetastatic ACC, increasing age was independently associated with inferior overall survival.
摘要：
背景：肾上腺皮质癌（ACC）是一种罕见但侵袭性的儿科内分泌肿瘤。然而,美国近期没有关于小儿ACC的治疗或结局的国家报告.我们的目的是检查临床特征，当前的管理策略，和儿科ACC的结果。
方法：在2004年至2019年的这项回顾性国家癌症数据库研究中，纳入了患有ACC的儿童（<18岁）。通过Kaplan-Meier方法检查总生存期，对数秩测试，和Cox回归建模。
结果：纳入了78名ACC患儿。平均年龄为10岁，中位肿瘤大小为10.2cm，35.9%在诊断时发生转移。大多数患者接受了手术治疗（84.6%），56.4%接受化疗,7.7%接受了辐射。1-,3-,5-y总生存率为87.0%，62.0%,和60.1%，分别。在未经调整的分析中，手术治疗与总生存率改善相关(log-rank检验,P<0.001)。在多变量Cox回归中，诊断时转移与低总生存率相关(风险比:2.72,95%置信区间:1.15-6.40,P=0.02),当调整年龄时，肿瘤大小,接受手术治疗，和化疗。在非转移性ACC患者中，年龄增长与整体生存率低相关(风险比:1.12,95%置信区间:1.00-1.24,P=0.04),当调整肿瘤大小时，接受手术治疗，和化疗。
结论：美国大多数ACC患儿接受手术治疗，其中约一半还接受化疗。诊断时的转移与低总生存率独立相关；在非转移性ACC患者中，年龄增长与整体生存率低下独立相关.
方法：在2004年至2019年的这项回顾性国家癌症数据库研究中，纳入了患有ACC的儿童（<18岁）。通过Kaplan-Meier方法检查总生存期，对数秩测试，和Cox回归建模。
结果：纳入了78名ACC患儿。平均年龄为10岁，中位肿瘤大小为10.2cm，35.9%在诊断时发生转移。大多数患者接受了手术治疗（84.6%），56.4%接受化疗,7.7%接受了辐射。1-,3-,5-y总生存率为87.0%，62.0%,和60.1%，分别。在未经调整的分析中，手术治疗与总生存率改善相关(log-rank检验,P<0.001)。在多变量Cox回归中，诊断时转移与低总生存率相关(风险比:2.72,95%置信区间:1.15-6.40,P=0.02),当调整年龄时，肿瘤大小,接受手术治疗，和化疗。在非转移性ACC患者中，年龄增长与整体生存率低相关(风险比:1.12,95%置信区间:1.00-1.24,P=0.04),当调整肿瘤大小时，接受手术治疗，和化疗。
结论：美国大多数ACC患儿接受手术治疗，其中约一半还接受化疗。诊断时的转移与低总生存率独立相关；在非转移性ACC患者中，年龄增长与整体生存率低下独立相关.
