儿童1-2期神经母细胞瘤的临床研究

临床儿科杂志 ›› 2014, Vol. 32 ›› Issue (5): 410-412.

儿童1-2期神经母细胞瘤的临床研究

潘慈，张安安，叶启东，周敏，薛惠良，陈静，罗长缨，沈树红，王坚敏，汤燕静，汤静燕

上海交通大学医学院附属上海儿童医学中心血液肿瘤科（上海　200127）

收稿日期:2014-03-17 出版日期:2014-05-15 发布日期:2014-05-15

Long-term follow-up of stage 1-2 neuroblastoma

Pan Ci,，Zhang Anan，Ye Qidong，Zhou Min, Hue Huiliang, Chen Jing, Luo Changyin, Shen Shuhong, Wang Jiangmin，Tang Yanjing, Tang Jingyan

(Department of Hematology and Oncology, Shanghai Children’s Medical Center, Shanghai JiaoTong University, School of Medicine, Shanghai 200127, China)

Received:2014-03-17 Published:2014-05-15 Online:2014-05-15

摘要/Abstract

摘要：

　目的　分析1-2期神经母细胞瘤（NB）的临床特征、诊疗方案及远期疗效，为进一步改进治疗方案提供循证依据。方法　回顾性分析1998年6月至2010年12月初诊并接受治疗的神经母细胞瘤1-2期患儿的临床资料。结果　NB患儿 237例，1期24例，2期25例，1-2期患儿占所有NB患儿的 20.7%（49/237）。诊断时的中位年龄25个月（2周~9岁），男29例，女20例。31例（63.6%）患儿因体检或影像学检查意外发现肿块。原发部位以后纵隔和腹部多见，分别为21例（42.9%）、22例（44.9%）。病理类型为预后良好者40例（81.6％）。尿香草苦杏仁酸（VMA）正常者32例（91.4％）。进行检测的患儿血LDH升高均不到正常值5倍。N-MYC扩增1例。10例患儿进入低危组治疗，仅行手术治疗。39例患儿进入中危组治疗，进行了手术及化疗综合治疗。所有49例患儿治疗结束时均获得非常好的部分缓解（VGPR），中位随访时间为60个月（22~148个月），未满5年失访者9例，中位随访时间为3个月（1~32个月）。全组2年、3年、5年无事件生存率（EFS）及生存率（OS）均为100%。结论　1-2期神经母细胞瘤预后良好，应进一步减少化疗强度和时间。

Abstract: Objective To evaluate the clinical features, treatment scheme and long-term outcomes of stage 1-2 childhood Neuroblastoma (NB). Methods The retrospective study included 49 newly diagnosed NB stage 1-2 patients from June 1998 to December 2010. Clinical data and long-term outcomes were analyzed. Results Twenty-four patients with stage 1 NB and twenty-five patients with stage 2 NB were found among all 237 patients with NB enrolled in this study. The median age at diagnosis was 25 months（２ week to９ year old），29 males and 20 females. 31 patients (63.6%) without symptoms were discovered with tumor by physical or imaging examination. Thorax and abdomen were the most common sites of primary tumor (21 and 22 cases, accounting for 42.9% and 44.9% of all patients, respectively) .40 (81.6%) NB patients had favorable pathology classification. One patient was of MYCN amplification status. Urine vanilla mandelic acid was normal in 32 (91.4%) patients, and serum lactate dehydrogenase was less than five times of the normal value in all patients. Ten NB patients were treated according to the low-risk protocol who received surgery alone.Thirty-nine patients were treated according to intermediate-risk protocol who received both surgery and chemotherapy. All the patients achieved very good partial remission (VGPR) (100%).The median follow-up period was 60 months （22 months to148months）。Nine patients were lost after a follow up of 3 months in median. The 2-、3-、5-year event free survival (EFS) and overall survial (OS) of all 49 patients was 100%. Conclusions The prognosis for neuroblastoma of stage 1-2 in this study was with 100% survival, which provides opportunity for further reduction of dosage and/or duration of episodes in chemotherapy.

潘慈，张安安，叶启东，周敏，薛惠良，陈静，罗长缨，沈树红，王坚敏，汤燕静，汤静燕. 儿童1-2期神经母细胞瘤的临床研究[J]. 临床儿科杂志, 2014, 32(5): 410-412.

Pan Ci,Zhang Anan,Ye Qidong,Zhou Min,Hue Huiliang,Chen Jing,Luo Changyin,Shen Shuhong,Wang Jiangmin,Tang Yanjing,Tang Jingyan. Long-term follow-up of stage 1-2 neuroblastoma [J]. , 2014, 32(5): 410-412.

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