Ding Zheyu,He Min,Xu Bin,et al.Application of "clinical observation" strategy for adrenal congenital neuroblastoma[J].Journal of Clinical Pediatric Surgery,2025,(12):1153-1157.[doi:10.3760/cma.j.cn101785-202412012-011]
“临床观察”策略在肾上腺先天性神经母细胞瘤中的应用
- Title:
- Application of "clinical observation" strategy for adrenal congenital neuroblastoma
- Keywords:
- Congenital Neuroblastoma; Adrenal Glands; Clinical Observation; Clinical Protocols; Treatment Outcome; Program Evaluation
- 摘要:
- 目的 初步总结肾上腺先天性神经母细胞瘤的临床及预后特征,评估采取"临床观察"策略的安全性及可行性。方法 本研究为回顾性病例系列研究,自2014年12月起,浙江大学医学院附属儿童医院对于肾上腺先天性神经母细胞瘤采用"临床观察"的治疗策略,仅对其中存在危险因素的患儿采取手术和(或)化疗干预。总结所有患儿的临床特征、治疗过程及预后等相关信息,分析患儿预后不良的危险因素。结果 83例中,72例在发病初期采取"临床观察"的策略,其中50例逐步消退(消退率69.4%);22例在"临床观察"过程中肿瘤进展,给予手术和(或)化疗干预;72例均无事件生存。另外11例于发病初期即采取干预措施,10例为肾上腺肿瘤合并肝脏弥漫性转移,其中4例死亡,3年总体生存率(overall survival,OS)为60.0%(6/10)。生存分析结果显示,肝脏弥漫性转移是此类患儿预后不良的危险因素(P＜0.05)。结论 肾上腺先天性神经母细胞瘤整体预后较好,具有较高的自行消退率,采用"临床观察"策略有一定安全性。肝脏弥漫性转移是此类患儿预后不良的危险因素,对存在肝脏弥漫性转移的患儿建议及时积极治疗。
- Abstract:
- Objective This study focuses on the clinical and prognostic characteristics of adrenal CNB and evaluates the safety and feasibility of "clinical observation" strategy. Methods A "clinical observation"-first strategy was adopted for adrenal CNB since December 2014.Only children with risk factors underwent immediate surgical and/or chemotherapeutic intervention,analyzing and summarizing clinical characteristics,treatment processes,and prognostic outcomes of the pediatric cohort,as well as risk factors for poor prognosis. Results Among the 83 patients,72 initially underwent "clinical observation." And 50 cases (69.4%) showed gradual regression while 22 experienced tumor progression during observation and subsequently underwent surgery and/or chemotherapy.All 72 children achieved event-free survival (EFS).The remaining 11 children received immediate intervention and 10 of them had adrenal tumors with diffuse liver metastases.Four children died with a 3-year overall survival (OS) rate of 60.0%.Univariate survival analysis identified diffuse liver metastasis as a significant prognostic risk factor (P<0.05). Conclusions Adrenal CNB has demonstrated favorable clinical outcomes and a high spontaneous regression rate.The above "clinical observation" strategy is safe.Diffuse liver metastasis is a critical prognostic risk factor and aggressive interventions should be promptly implemented.
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备注/Memo
收稿日期:2024-12-3。
基金项目:浙江省"尖兵""领雁"研发攻关计划项目(2024C03181)
通讯作者:王金湖,Email:wjh@zju.edu.cn