53例单纯手术治疗极低危组、低危组神经母细胞瘤患儿临床特征及近期预后分析  

Clinical characteristics and short-term prognosis analysis of 53 cases of neuroblastoma treated by surgery alone

作　　者：简冰林 苏雁[1] 秦红[2] 陈诚豪[3] 王生才[4] 蒋持怡 张诗晗 于彤[5] 伏利兵[6] 曾骐[3] 王焕民[2] 倪鑫[4] 马晓莉[1] JIAN Binglin;SU Yan;QIN Hong;CHEN Chenghao;WANG Shengcai;JIANG Chiyi;ZHANG Shihan;YU Tong;FU Libing;ZENG Qi;WANG Huanmin;NING Xi;MA Xiaoli(Medical Oncology Department, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children, Ministry of Education;Department of Surgical Oncology, Beijing Children′s Hospital, Capital Medical University, Beijing 100045, China;Department of Thoracic,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;Department of Otorhinolaryngology Head and Neck Surgery,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;Department of Imaging Center,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China;Department of Pathology,Beijing Children′s Hospital,Capital Medical University,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院肿瘤内科,北京市儿童血液肿瘤重点实验室,儿科重大疾病研究教育部重点实验室,北京100045 [2]首都医科大学附属北京儿童医院肿瘤外科,北京100045 [3]首都医科大学附属北京儿童医院胸外科,北京100045 [4]首都医科大学附属北京儿童医院耳鼻咽喉头颈外科,北京100045 [5]首都医科大学附属北京儿童医院影像科,北京100045 [6]首都医科大学附属北京儿童医院病理科,北京100045

出　　处：《中国小儿血液与肿瘤杂志》2021年第4期238-242,共5页Journal of China Pediatric Blood and Cancer

基　　金：首都卫生发展专项(首发2018-2-2095);国家科技重大专项(2017ZX09304029004)。

摘　　要：目的探讨神经母细胞瘤(NB)极低危组、低危组(<18个月)患儿仅行单纯手术治疗的临床特征、疗效及其近期预后,为应用单纯手术切除治疗极低危组、低危组NB患儿的意义提供临床依据。方法回顾性分析2016年12月1日—2019年12月31日于北京儿童医院就诊的极低危组和低危组NB仅行单纯手术治疗未行放、化疗治疗患儿的临床信息,分析该组患儿临床特征及近期预后。结果53例患儿入组,极低危组51例,低危组2例。初诊时中位年龄14.1(0.1~141.5)个月。原发瘤灶以腹膜后肾上腺最多见(47.2%)。病理分型:节细胞型神经母细胞瘤30例(56.6%)、神经母细胞瘤型23例(43.4%)。2例患儿血清NSE>100 ng/mL(3.8%),14例高于正常但<100 ng/mL(26.4%,14/53);50例行血清LDH检测,3例患儿血清LDH>500 U/L(6%);40例患儿病初行尿VMA/HVA检测,19例升高(47.5%)。NMYC基因扩增、1p36、11q23缺失均阴性。手术治疗:49例行局部瘤灶切除,3例行原发瘤灶切除+局部淋巴结清扫术,1例行分期肿瘤切除。53例患儿平均随访时间为21.9个月,52例(98.1%)病情稳定无复发。结论极低危组、低危组(<18个月)NB患儿具有良好的生物学特征,通过完整的瘤灶切除和术后定期随访能使患儿获得良好预后,可避免因过度治疗对患儿生活质量带来不利影响。Objective To explore the clinical manifestations,curative effect and short-term prognosis of children with neuroblastoma(NB)in the very low-risk and low-risk groups(<18 months)who only received surgery,without radiotherapy and chemotherapy,so as to provide clinical evidence for the application of surgery alone in the treatment of NB in very low-risk group and low-risk group.Methods Clinical data of children with very low-risk and low-risk group NB who were only treated with surgery,without radiotherapy and chemotherapy in Beijing Children′s Hospital from Dec 2016 to Dec 2019 were collected.The clinical characteristics and short-term prognosis of them were analyzed retrospectively.Results A total of 53 children were included,including 51 cases of very low-risk group and 2 cases of low-risk group.The median age at initial diagnosis was 14.1(ranged from 0.1 to 141.5)month.The primary tumor was most located in the retroperitoneum/adrenal glands(47.2%).In pathology,there were 30 cases of ganglioneuroblastoma(GNB)and 23 cases of NB.2/53 cases had NSE>100 ng/ml,14/53 cases had NSE higher than normal but<100ng/ml,3/50 cases had LDH>500 U/L and 19/40 cases had elevated VMA/HVA at initial diagnosis.NMYC gene,1p36 and 11q23 were all negative in these cases.Among 53 children,49 underwent local excision,3 received primary tumor resection and local lymph node dissection,1 received staged tumor resection.53 children were successfully followed up,the average follow-up time was 21.9 months.52 cases(98.1%)were in stable condition without recurrence during the follow-up period.Conclusions Children with NB in the very low-risk group and low-risk group less than 18 months have good biological characteristics.Complete tumor resection and regular follow-up can acheive good prognosis and avoid the adverse effects of overtreatment on children.

关 键 词：神经母细胞瘤 极低危及低危组 手术 临床特征 近期预后 

分 类 号：R73[医药卫生—肿瘤]