先天性哑铃形神经母细胞瘤3例疗效分析  

Clinical analysis of congenital dumbbell neuroblastoma in three children

作　　者：金惠明[1] 武志祥[1] JIN Huiming;WU Zhixiang(Department of Pediatric Surgery,Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院小儿外科,上海200092

出　　处：《临床儿科杂志》2018年第12期939-941,共3页Journal of Clinical Pediatrics

摘　　要：目的探讨先天性哑铃形神经母细胞瘤(NB)的治疗。方法回顾分析1990年4月—2014年5月收治的3例以出生后下肢瘫痪为表现的先天性哑铃形NB患儿的临床资料。结果 3例患儿均为男性,就诊时出生14～53天,入院后均行椎板切除减压、硬脊膜外NB切除术。经病理检查证实为NB。术后均未行化疗、放疗。例1患儿为腹膜后NB行经腹切除,8个月后肝脏转移死亡;例2患儿经椎管术后,家长放弃治疗,5年后获随访,腹膜后NB自然消退;例3患儿经椎管术后,N-myc基因检测无扩增,观察随访14个月,腹膜后NB自然消退。结论椎板切除减压、硬脊膜外NB切除为先天性哑铃形NB的治疗选择之一。若N-myc基因无扩增,腹膜后NB不需切除和化疗,可定期随访。Objective To explore the treatment of congenital dumbbell neuroblastoma(NB).Method The clinical data of 3 congenital dumbbell NB patients presenting with postnatal lower extremity paralysis from April 1990 to May 2014 were retrospectively analyzed.Results All 3 children were male and the age at admission was 14-53 days.Vertebral laminectomy for decompression and extradural intra-vertebral canal tumor resection were performed in all patients.Then they were confirmed to have NB by pathological examination and had no chemotherapy or radiotherapy after operation.Case 1 underwent retroperitoneal NB transabdominal resection and died 8 months later after the surgery due to hepatic metastasis.In case 2,the parents abandoned any treatment after vertebral laminectomy and was found that the rest of the tumor was regressed spontaneously at five years follow-up.In case 3,there was no amplification of N-myc gene after the surgery,and the retroperitoneal neuroblastoma showed spontaneous regression after 14 months.Conclusions Vertebral laminectomy for decompression and extradural intra-vertebral canal tumor resection is one of the treatment options for congenital dumbbell NB.For retroperitoneal NB patients without N-myc gene amplification,resection and chemotherapy are not required,but regular follow-up may be scheduled.

关 键 词：先天性肿瘤 哑铃形神经母细胞瘤 治疗 

分 类 号：R739.4[医药卫生—肿瘤]