Department of Pediatric Surgery, Maternal and Child Health Hospital of Hubei Province, Tongji Medical College, Huazhong University of Science and Technology, Wuhan City, China.
Front Endocrinol (Lausanne). 2022 Sep 12;13:977105. doi: 10.3389/fendo.2022.977105. eCollection 2022.
To investigate the clinicopathological characteristics and long-term survival outcomes of pediatric adrenal malignancies.
This study retrospectively analyzed children with pathologically confirmed pediatric adrenal malignancies from Surveillance, Epidemiology, and End Results Database from 2000 to 2019. Kaplan-Meier curve was used to assess the overall survival (OS) and cancer-special survival (CSS), and the Log-Rank method was used to calculate statistical differences. Cox proportional hazards model and Fine-and-Grey model were used to calculate the hazard ratio (HR) of all-cause mortality risk and the sub-distribution HR (sHR) of disease-specific mortality risk, respectively, and their corresponding 95% confidence intervals (CI).
1601 children were included in the study in which 1335 (83.4%) neuroblastoma, 151 (9.4%) ganglioneuroblastoma, 89 (5.6%) adrenocortical carcinoma, and 26 (1.6%) were diagnosed with other types malignancies. Metastatic disease accounted for the largest proportion (69.3%), and the proportion of metastases diagnosed by neuroblastoma was higher than that of adrenocortical carcinoma and ganglioneuroblastoma (73.9% vs. 45.7% vs. 47.2%). The 5-year OS and CSS of all cohort were 69.5% and 70.5%, respectively. Adrenal cortical carcinoma had the worst prognosis, with 5-year OS and CSS of 52.5% and 53.1%, respectively. Patients in recent years had no better OS and CSS than in previous years at diagnosis. The tumor stage remained the main prognostic predictor. Compared to metastatic adrenal tumors, the risk of all-cause mortality (adjusted HR: 0.12, 95% CI: 0.06-0.25, < 0.001) and the risk of disease-specific mortality (adjusted sHR: 0.11, 95% CI: 0.05-0.25, <0.001) was significantly lower for patients with localized diseases. Additionally, higher age, adrenal cortical carcinoma, and lack of complete tumor resection are independent risk factors for poor prognosis. Furthermore, it was found that the prognosis of patients who received chemotherapy was worse than those who did not, mainly because the former mostly had metastasis at the presentation and complete resection of the tumor cannot be achieved.
The clinicopathological characteristics of pediatric adrenal malignancies have not changed significantly in the past two decades, while the prognosis of patients has improved. Early diagnosis of disease and complete resection of local tumors are the keys to improving prognosis.
探讨儿童肾上腺恶性肿瘤的临床病理特征和长期生存结局。
本研究回顾性分析了 2000 年至 2019 年期间，经病理证实为儿童肾上腺恶性肿瘤的患者，这些患者均来自监测、流行病学和最终结果数据库。采用 Kaplan-Meier 曲线评估总生存（OS）和癌症特异性生存（CSS），采用对数秩检验计算统计学差异。Cox 比例风险模型和 Fine-and-Grey 模型分别计算全因死亡率风险的风险比（HR）和疾病特异性死亡率的亚分布 HR（sHR）及其相应的 95%置信区间（CI）。
本研究共纳入 1601 例患儿，其中神经母细胞瘤 1335 例（83.4%），神经节母细胞瘤 151 例（9.4%），肾上腺皮质癌 89 例（5.6%），其他类型恶性肿瘤 26 例（1.6%）。转移疾病占比最大（69.3%），神经母细胞瘤转移的比例高于肾上腺皮质癌和神经节母细胞瘤（73.9% vs. 45.7% vs. 47.2%）。全队列的 5 年 OS 和 CSS 分别为 69.5%和 70.5%。肾上腺皮质癌的预后最差，5 年 OS 和 CSS 分别为 52.5%和 53.1%。与前几年相比，近年诊断的患者 OS 和 CSS 并没有改善。肿瘤分期仍然是主要的预后预测因素。与转移性肾上腺肿瘤相比，局部疾病患者的全因死亡率（调整 HR：0.12，95%CI：0.06-0.25，<0.001）和疾病特异性死亡率（调整 sHR：0.11，95%CI：0.05-0.25，<0.001）风险显著降低。此外，年龄较大、肾上腺皮质癌和肿瘤不完全切除是预后不良的独立危险因素。此外，研究还发现，接受化疗的患者预后较差，主要是因为前者在就诊时多有转移，且无法完全切除肿瘤。
过去 20 年，儿童肾上腺恶性肿瘤的临床病理特征无明显变化，而患者的预后有所改善。早期诊断疾病和完全切除局部肿瘤是改善预后的关键。
