目的:肾上腺皮质癌(ACC)是一种罕见的肾上腺内分泌肿瘤,在儿童中预后较差。我们旨在进行一项基于人群的队列研究,以预测ACC儿科患者的总体生存率(OS)。
方法:我们使用了监测,流行病学,和最终结果(SEER)数据库对1975年至2018年间诊断为ACC的儿科患者进行回顾性队列研究。我们检查了人口统计特征,肿瘤分期和大小,治疗方案,和生存结果。Kaplane-Meier估计用于基于几个参数生成存活曲线。为了比较生存曲线,采用对数秩检验。Cox比例风险回归用于确定与OS相关的变量。此外,我们创建了一个列线图来预测儿科ACC患者的总生存期.
结果:共确定了143例小儿ACC患者。女性受影响最大(60.8%)。整体1年,3年,5年生存率为75.0%,57.6%,所有患者占53.7%,分别。与老年患者(5-19岁)相比,年轻患者(≤4岁)显示出更多的积极特征,包括局部疾病的可能性更高(29.4%vs.14%,P<0.001),肿瘤小于10厘米(23.1%vs.14.7%,P<0.001),并改善总生存率(5年OS89.6%vs.27.7%,P<0.001)。诊断时的年龄,SEER阶段,在该模型中,手术是OS的重要独立预测因子,根据Cox比例风险回归的结果。之后,我们开发了一个用于预测ACC儿童OS的列线图。4岁以上的患者死亡的可能性更高。此外,SEER阶段越高,死亡风险越高。没有做手术的患者的生存率比做手术的患者差。
结论:我们的研究表明,诊断时的年龄,SEER阶段,和手术被发现是小儿ACC总生存率的最重要预测因素。这些发现有助于现有的知识体系,并强调持续研究的重要性,以增进我们对儿科ACC的理解并改善患者护理。
OBJECTIVE: Adrenocortical carcinoma (ACC) is an uncommon adrenal gland endocrine tumor that has a poor prognosis in children. We aimed to conduct a population-based cohort study to predict overall survival (OS) in pediatric patients with ACC.
METHODS: We used the Surveillance, Epidemiology, and End Results (SEER) database to conduct a retrospective cohort research on pediatric patients diagnosed with ACC between 1975 and 2018. We examined demographic characteristics, tumor stage and size, treatment options, and survival results. Kaplane-Meier estimations were used to generate survival curves based on several parameters. To compare survival curves, the log-rank test was applied.Cox proportional-hazards regression was used to determine the variables related with OS. In addition, we created a nomogram to predict overall survival in pediatric ACC patients.
RESULTS: A total of 143 pediatric ACC patients were identified. Females were the most impacted (60.8%). Overall 1 year, 3 year, and 5 year survival rates were 75.0%, 57.6%, and 53.7% for all patients, respectively. In comparison to older patients (5-19 years), younger patients (≤ 4 years) were shown to have more positive characteristics, including a higher likelihood of local disease (29.4% vs. 14%, P < 0.001), tumors less than 10 cm (23.1% vs. 14.7%, P < 0.001), and improved overall survival (5 year OS 89.6% vs. 27.7%, P < 0.001). Age at diagnosis, SEER stage, and surgery were significant independent predictors of OS in this model, according to the results of Cox proportional hazard regression. After that, we developed a nomogram for predicting OS in children with ACC. Patients older than 4 years old had a higher chance of dying. Furthermore, the higher the SEER stage, the higher the risk of death. Patients who do not have surgery have a worse survival rate than those who do.
CONCLUSIONS: Our study revealed that age at diagnosis, SEER stage, and surgery were found to be the most important predictors of the overall survival of pediatric ACC. These findings contribute to the existing body of knowledge and emphasize the importance of continued research to advance our understanding of pediatric ACC and improve patient care.