Department of Oncology Surgery, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, Beijing, 100045, China.
Medical Imaging Center, Beijing Children's Hospital, National Center for Children's Health, Capital Medical University, Beijing, 100045, China.
Eur J Med Res. 2023 Oct 9;28(1):411. doi: 10.1186/s40001-023-01381-3.
To summarize the clinical characteristics of children with adrenocortical carcinoma (ACC) and preliminarily explore the indications for and efficacy of neoadjuvant chemotherapy in certain patients.
The data of 49 children with adrenocortical tumors (ACT) in the past 15 years were retrospectively analyzed, and after pathology assessment using Weiss system grading, 40 children diagnosed with ACC were included. Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 and three-dimensional (3D) reconstruction of contrast-enhanced computed tomography data were used to evaluate the response to neoadjuvant chemotherapy.
Forty patients (17 males, 23 females) with ACC were enrolled. Abnormal hormone levels were common in children with ACC (n = 31), and in terms of clinical presentation, sexual precocity was the most common (n = 14, 35.0%), followed by Cushing's syndrome (n = 12, 30.0%). Seven of 40 children received neoadjuvant chemotherapy due to a maximum lesion diameter greater than 10 cm (n = 4), invasion of surrounding tissues (n = 2), intravenous tumor thrombus (n = 2), and/or distant metastasis (n = 2); 2 patients achieved partial response, and 5 had stable disease according to the RECIST 1.1 standard. Furthermore, 3D tumor volume reconstruction was performed in 5 children before and after neoadjuvant chemotherapy. Tumor volumes were significantly reduced in all 5 children, with a median volume reduction of 270 (interquartile range, IQR 83, 293) (range: 49-413) ml. After surgery with/without chemotherapy, the 5-year overall survival rate for all children was 90.0% (95% CI-confidence interval 80.0-100.0%), and the 5-year event-free survival rate was 81.5% (95% CI 68.0-97.7%).
In the diagnosis and treatment of pediatric ACC, a comprehensive endocrine evaluation is necessary to facilitate early diagnosis. Surgery and chemotherapy are important components of ACC treatment, and neoadjuvant chemotherapy should be considered for children with ACC who meet certain criteria, such as a large tumor, distant metastases, or poor general condition.
总结儿童肾上腺皮质癌（adrenocortical carcinoma，ACC）的临床特征，并初步探讨某些患者接受新辅助化疗的适应证和疗效。
回顾性分析过去 15 年 49 例肾上腺皮质肿瘤（adrenocortical tumors，ACT）患儿的临床资料，采用 Weiss 系统分级病理评估后，纳入 40 例诊断为 ACC 的患儿。采用实体瘤疗效评价标准 1.1（Response Evaluation Criteria in Solid Tumors，RECIST）1.1 和增强 CT 三维（three-dimensional，3D）重建评估新辅助化疗的疗效。
共纳入 40 例 ACC 患儿（男 17 例，女 23 例）。患儿常伴有激素水平异常（n=31），临床表现以性早熟最为常见（n=14，35.0%），其次为库欣综合征（n=12，30.0%）。因最大病灶直径>10 cm（n=4）、侵犯周围组织（n=2）、静脉内肿瘤栓子（n=2）和/或远处转移（n=2）等原因，7 例患儿接受了新辅助化疗，根据 RECIST 1.1 标准，2 例患儿达到部分缓解，5 例疾病稳定。此外，5 例患儿在新辅助化疗前后进行了 3D 肿瘤体积重建，所有患儿的肿瘤体积均明显缩小，中位数体积缩小 270（四分位间距 IQR 83，293）ml（范围：49-413 ml）。手术后行化疗或单纯手术治疗，所有患儿的 5 年总生存率为 90.0%（95%可信区间 80.0-100.0%），无事件生存率为 81.5%（95%可信区间 68.0-97.7%）。
在儿童 ACC 的诊断和治疗中，需要进行全面的内分泌评估以促进早期诊断。手术和化疗是 ACC 治疗的重要组成部分，对于符合一定标准的 ACC 患儿，如肿瘤较大、远处转移或一般状况较差等，应考虑新辅助化疗。
