基于国家癌症中心数据库分析，采用腺瘤切除术治疗局限性小儿肾上腺皮质癌的生存特征。
Survival characteristics of localized pediatric adrenocortical carcinoma managed with adenectomy: A national cancer center database analysis.
机构信息
College of Medicine, University of Oklahoma, 940 Stanton L Young Blvd #357, Oklahoma City, OK, 73104, USA.
Department of Urology, University of Oklahoma, 920 Stanton L. Young Blvd, WP 2140, Oklahoma City, OK, 73104, USA.
出版信息
J Pediatr Urol. 2021 Oct;17(5):735.e1-735.e6. doi: 10.1016/j.jpurol.2021.06.005. Epub 2021 Jun 9.
INTRODUCTION
Pediatric adrenocortical carcinoma (ACC) is a rare malignancy, encompassing less than 0.2% of all childhood malignancies. Due to the scarcity of this diagnosis, it is often managed according to guidelines established for adults, as there is a lack of reliable evidence regarding optimal adjuvant treatment options for pediatric patients. It is our aim to identify recent treatment trends as well as clinical and tumor characteristics and their impact on overall survival.
METHODS
Using the National Cancer Data Base (NCDB), this study identified 49 patients under 18 years old with localized ACC (M0) undergoing adrenalectomy from 2004 to 2017. Kaplan-Meier analysis was performed to determine overall survival (OS) from patient characteristics and treatments received. Comparison of survival was performed using the log rank test.
RESULTS
The median age of our cohort was 3 years old with a slight female predominance of 61%. The median tumor size was 9.4 cm, and patients older than 4 years were significantly (p = 0.03) more likely to present with larger tumors (11.33 cm vs 8.76 cm). Adjuvant treatment in the form of systemic therapy was administered in 20 of 49 (41%) patients and radiation therapy in 2 of 49 (4%) patients. Three-year OS for patients 4 years old and younger was 92.6% vs 61.8% for those older than 4 years (p = 0.002). Patients presenting with tumor size ≥9 cm had worse three-year OS compared to those with tumors <9 cm (95.24% vs 67.1% respectively, p = 0.02, Fig. 1). In patients with tumors ≥ 9 cm, younger children age 0-4 years had significantly (p = 0.04) higher OS rates than older children age 5-17 years.
CONCLUSIONS
ACC is a rare pediatric malignancy with a female predominance. Those older than 4 years and those with presenting tumor size ≥9 cm have decreased overall survival rates after adrenalectomy for localized disease. Additionally, children older than 4 have poorer prognosis, even after controlling for larger tumor size. This is the largest contemporary series of localized pediatric ACC to date. However, multi-institutional prospective cohort or randomized-controlled trials are necessary to better evaluate relevant prognostic factors and the role of adjuvant therapies following adrenalectomy.
简介
儿科肾上腺皮质癌（ACC）是一种罕见的恶性肿瘤，占儿童所有恶性肿瘤的比例不到 0.2%。由于这种诊断很少见，因此通常根据为成年人制定的指南进行治疗，因为对于儿科患者的最佳辅助治疗选择缺乏可靠的证据。我们的目标是确定最近的治疗趋势以及临床和肿瘤特征及其对总生存率的影响。
方法
本研究使用国家癌症数据库（NCDB），从 2004 年至 2017 年期间，共确定了 49 名年龄在 18 岁以下、接受肾上腺切除术的局部 ACC（M0）患者。通过 Kaplan-Meier 分析确定患者特征和治疗方法对总生存率（OS）的影响。使用对数秩检验比较生存率。
结果
我们的队列中位年龄为 3 岁，女性略多（61%）。肿瘤中位大小为 9.4cm，4 岁以上患者的肿瘤明显更大（p=0.03）（11.33cm 比 8.76cm）。49 例患者中有 20 例（41%）接受了全身性辅助治疗，2 例（4%）接受了放疗。4 岁及以下患者的 3 年 OS 为 92.6%，4 岁以上患者为 61.8%（p=0.002）。肿瘤大小≥9cm 的患者 3 年 OS 较差，与肿瘤<9cm 的患者相比（分别为 95.24%和 67.1%，p=0.02，图 1）。肿瘤大小≥9cm 的患者中，0-4 岁的儿童与 5-17 岁的儿童相比，OS 率显著更高（p=0.04）。
结论
ACC 是一种罕见的儿科恶性肿瘤，以女性为主。4 岁以上和肿瘤大小≥9cm 的患者，在局部疾病接受肾上腺切除术治疗后，总生存率降低。此外，4 岁以上的儿童即使在控制肿瘤较大的情况下，预后也较差。这是迄今为止最大的局部儿科 ACC 当代系列研究。然而，需要多机构前瞻性队列或随机对照试验来更好地评估相关预后因素和肾上腺切除术后辅助治疗的作用。
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