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儿童神经节细胞瘤及神经节神经母细胞瘤混杂型的治疗经验

Treatment of ganglioneuroma and ganglioneuroblastoma intermixed in children
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摘要 目的总结治疗儿童神经节细胞瘤及神经节神经母细胞瘤混杂型的经验。方法回顾性分析2009年5月—2019年11月在广州市妇女儿童医疗中心诊治的神经节细胞瘤及神经节神经母细胞瘤混杂型病例。采用Mann-Whitney U检验、卡方检验和费希尔精确检验进行比较各指标,采用Kaplan-Meier进行生存分析、Log-rank比较生存曲线。结果本研究共纳入88例患儿,中位年龄为54.7(13.7~192.0)个月。肿瘤原发于颈部(2例)、胸部(54例)、胸腹(2例)、腹部(24例)、盆腔(6例)。INSS分期1期、2A、2B、3期、4期分别为58例、9例、10例、9例、2例。所有患儿均接受手术切除,术中失血量中位数为50(2~4700)mL,术中输注红细胞中位数为1.(0~7)单位、输注血浆中位数为100(0~1700)mL。5例(5.7%)患儿发生了并发症。20例(22.7%)有肿瘤残留,其中11例肿瘤残留大于2cm,2例残留小于2cm,另外7例未记录具体残留组织大小。术后病理确诊神经节细胞瘤32例(36.4%),神经节神经母细胞瘤混杂型56例(63.6%)。神经节细胞瘤患儿发病年龄较神经节神经母细胞瘤混杂型患儿大[69.1(47.4~88.3)个月vs 49.6(32.1~68.9)个月,P=0.005]。中位随访时间为23.5(0.0~89.0)个月,2例(2.3%)复发、3例(3.4%)死亡、9例(10.2%)失访。术后肿瘤残留对无事件生存率及总体生存率没有显著影响(P=0.691,P=0.128)。结论神经节细胞瘤及神经节神经母细胞瘤混杂型总体治疗效果良好,手术治疗安全有效,术后肿瘤残留对临床结局可能无显著影响。 Objective This study aims to summarize the treatment experience of ganglioneuroma(GN)and ganglioneuroblastoma intermixed(GNBI)patients in a single institution.Methods Patients diagnosed with GN and GNBI between May 2009 and November 2019 at Guangzhou Women and Children′s Medical Center were enrolled.Mann-Whitney U test,Chi-square test and Fisher′s exact test were used for comparison.Kaplan-Meier method was used for survival analysis and Log-rank was used to compare survival curves.Results Eighty-eight patients were included in this study.The median age was 54.7(13.7~192.0)months old.The primary tumors were in the neck(2 cases),chest(54 cases),chest and abdomen(2 cases),abdomen(24 cases)and pelvis(6 cases).According to the International Neuroblastoma Staging System(INSS),there were 58 cases of stage 1,9 cases of stage 2A,10 cases of stage 2B,9 cases of stage 3,and 2 cases of stage 4.All patients underwent surgical resection.Median blood loss was 50(2~4700)mL the median intraoperative transfusion of red blood cells was 1(0~7)units,and the median plasma transfusion was 100(0~1700)mL.Five(5.7%)patients developed operative complications.Twenty(22.7%)patients had residual tumor,including 11 cases with residual tumors larger than 2cm,2 cases with residual tumors smaller than 2cm,and the 7 cases with no specific residual tissue size recorded.Surgical pathology confirmed 32(36.4%)cases of GN and 56(63.6%)cases of GNBI.The age of onset of GN children was older than that of GNBI children(69.1,47.4~88.3 months vs 49.6,32.1~68.9 months,P=0.005).During the follow-up period of 23.5(0.0~89.0)months,there were 2 cases of disease recurrences,3 cases of dead and 9 cases of lost to follow-up.The presence of residual tumor had no significant impact on the EFS and OS rates(P=0.691,P=0.128,respectively).Conclusions The surgical outcomes of GN and GNBI are excellent.Residual tumor does not influence the overall outcome.
作者 郑曼娜 李嘉豪 李乐 谭天宝 潘静 胡超 杨纪亮 沈远超 吴慧莹 牛会林 徐涛 张晓红 陈希文 鹿连伟 邹焱 杨天佑 ZHENG Manna;LI Jiahao;LI Le;TAN Tianbao;PAN Jing;HU Chao;YANG Jiliang;SHEN Yuanchao;WU Huiying;NIU Huilin;XU Tao;ZHANG Xiaohong;CHEN Xiwen;LU Lianwei;ZOU Yan;YANG Tianyou(Department of Paediatric Oncological Surgery,Guangzhou Women and Children′s Medical Center,Guangzhou Medical University,Guangzhou 510623,China;Department of Thoracic Surgery,Guangzhou Women and Children′s Medical Center,Guangzhou Medical University,Guangzhou 510623,China;Department of Radiology,Guangzhou Women and Children′s Medical Center,Guangzhou Medical University,Guangzhou 510623,China;Department of Pathology,Guangzhou Women and Children′s Medical Center,Guangzhou Medical University,Guangzhou 510623,China;Department of Hematology and Oncology,Guangzhou Women and Children′s Medical Center,Guangzhou Medical University,Guangzhou 510623,China)
出处 《中国小儿血液与肿瘤杂志》 CAS 2023年第2期113-117,共5页 Journal of China Pediatric Blood and Cancer
基金 国家自然科学基金青年基金(编号:81602199)。
关键词 神经节细胞瘤 神经节神经母细胞瘤混杂型 儿童 手术 Ganglioneuroma Ganglioneuroblastoma intermixed Children Surgery
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