儿童神经母细胞瘤无化疗/放疗组远期随访报告

摘要

Objectives To analyze the clinical features and long-term follow-up results of neuroblastoma (NB) without chemotherapy and radiotherapy, and to provide evidence for further improvement of treatment. Methods The clinical data of children diagnosed with NB who received operation during January 2005 to December 2015 was retrospectively analyzed, and the long-term follow-up results were evaluated. Results In 57 cases of NB, 43 cases (81.1％) were in stage 1, 8 cases were in stage 2 and 2 cases were in stage 4S. The median age at diagnose was 7 months (11 days - 10 years and 11 months). There were 47/51 cases had the pathological type with a good prognosis (accounting for 92.2％). FISH was detected in 1/49 case which had the amplification at greater than 10 copies. 56/57 cases underwent surgical resection of the primary tumor, 50 cases of which were completely resected, and 3 cases had very good partial remission after tumor resection. The abdominal mass was found in the uterus in 1 case, and surgical operation was not performed and the imaging was regularly checked for follow-up , and the mass subsided completely at 7-month-old. The median follow-up time was 36 months (4 - 99 months). Five children were lost to follow-up and the median time of follow-up was 19 months (4 - 45 months). One child in stage 4S relapsed at 1 year of follow-up, 2 cases in stage 1 relapsed at 6 months of follow-up. Five years event free survival rates (EFS) in all patients were 94.6％, and overall survival rate (OS) of the 5 years was 100％. Conclusions Children younger than 18 months without MYCN amplification in the stages 1 and 2 are safe by surgical treatment alone with good prognosis. Simple surgical treatment can also be extended to all age groups of NB without MYCN amplification in the stages 1 or 2.%目的 分析神经母细胞瘤(NB)无化疗/放疗组患儿的临床特点及远期随访结果,为进一步改进治疗方案提供循证依据.方法 回顾分析2005年1月至2015年12月明确诊断为NB,且采取术后观察患儿的临床资料,评估远期随访结果.结果 NB患儿57例,1期43例(81.1％),2期8例,4S期2例;诊断中位年龄7个月(11天～10岁11个月);男30例,女27例.原发部位后腹膜和肾上腺37例(64.9％).47/51例病理分型为预后良好型(占92.2％).1/49例FISH检测到MYCN扩增大于10个拷贝.56/57例患儿接受手术切除原发肿瘤,其中50例完整切除,3例肿瘤切除术后达非常好的部分缓解;1例患儿宫内发现腹部肿块,出生后未手术定期影像学随访,肿块在7个月时完全消退.随访中位时间36个月(4～99个月);失访5例,失访患儿的随访中位时间19个月(4～45个月).1例4S期患儿随访1年时复发,2例1期患儿均在随访6个月时原位复发.全组5年无事件生存率94.6％,5年总生存率100％.结论 年龄小于18个月不伴MYCN扩增的1期和2期患儿单纯手术治疗安全、预后良好,单纯手术治疗可适当扩增至更大范围,如不伴MYCN扩增的全年龄组1期和2期NB.