儿童神经源性纵隔肿瘤的规范性综合诊治经验OA北大核心
Standardized comprehensive diagnostic and therapeutic experiences of neurogenic mediastinal tumors in children
目的 分析儿童神经源性纵隔肿瘤的临床特征、治疗方法及预后,探讨规范性诊治经验.方法 回顾性分析复旦大学附属儿科医院心胸外科2014年1月至2023年12月收治的神经源性纵隔肿瘤患儿资料,对其临床特征、术前影像学及实验室检查结果、治疗方案、预后情况进行分析.结果 共197例神经源性纵隔肿瘤患儿接受手术治疗,均完成手术切除,其中胸腔镜切除55例,开胸手术142例(含胸腔镜中转开胸7例);无一例围手术期死亡.术后病理:良性43例、恶性154例;神经母细胞瘤81例,节细胞神经母细胞瘤67例,节细胞神经瘤32例,神经鞘瘤7例,神经纤维瘤4例,原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)6例.术后随访1~5年,5年总生存率为98.48%,5年免于复发率为92.22%.结论 儿童神经源性纵隔肿瘤以恶性居多,通常局限于胸部,完全切除的可能性较大,接受规范性综合诊治患儿术后生存率良好.
Objective To explore the clinical characteristics,treatment protocols and outcomes of pedi-atric mediastinal neurogenic tumors and summarize standardized diagnostic and therapeutic experiences.Methods From January 2014 to December 2023,the relevant clinical data were retrospectively reviewed for 197 children with neurogenic tumors admitted into Children's Hospital of Fudan University.Their clinical character-istics,preoperative imaging and laboratory examinations,treatment protocols and outcomes were analyzed.Re-sults All of whom received completed surgical resection or near-total resection.The procedures included thora-coscopy(n=55)and thoracotomy(n=142,including 7 converting cases from thoracoscopy into thoracotomy).There was no perioperative mortality.Postoperative pathology was benign(n=43)and malignant(n=154),including neuroblastoma(n=81),ganglioneuroblastoma(n=67),ganglioneuroma(n=32),Schwannoma(n=7),neurofibroma(n=4)and primitive neuroectodermal tumor(PNET,n=6).Postoperative follow-up period was(1-5)year.And 5-year overall survival rate was 98.48%and 5-year recurrence-free survival rate 92.22%.Conclusions Pediatric mediastinal neurogenic tumors are mostly malignant and usually confined to chest with a high likelihood of complete resection and decent postoperative survival rates.
张文波;黄家熙;石奇琪;张惠锋;陈纲;叶明;仇万山
复旦大学附属儿科医院心胸外科,上海 201102复旦大学附属儿科医院心胸外科,上海 201102复旦大学附属儿科医院心胸外科,上海 201102复旦大学附属儿科医院心胸外科,上海 201102复旦大学附属儿科医院心胸外科,上海 201102复旦大学附属儿科医院心胸外科,上海 201102复旦大学附属中山医院儿科中心,上海 201102
肿瘤,纵隔神经源性肿瘤神经母细胞瘤外科手术儿童
Neoplasms,MediastinumNeurogenic TumorNeuroblastomaSurgical Procedures,Oper-ativeChild
《临床小儿外科杂志》 2025 (5)
415-418,4
2024年医疗服务与保障能力提升(国家临床重点专科建设)项目(1000015Z15508000000) National Clinical Key Specialty Construction Project(10000015Z15508000000)
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