汤梦婕; 袁晓军; 安霞; 张勤; 盛琦; 谈珍

doi:10.3760/cma.j.issn.2096-5400.2019.03.004

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转移性神经母细胞瘤伴骨和骨髓转移的治疗疗效及预后分析

中华转移性肿瘤杂志, 2019,02(3) : 18-24. DOI: 10.3760/cma.j.issn.2096-5400.2019.03.004

摘要

目的

由于儿童转移性神经母细胞瘤(neuroblastoma, NB)预后较差，通过分析上海交通大学医学院附属新华医院收治的转移性NB患儿临床特点，从而评估其疗效和预后。

方法

回顾性分析2007年1月1日—2014年12月1日于上海交通大学附属新华医院于收治的75例转移性NB患儿的临床特征、实验室检查、临床分期、危险度分组、病理分型、治疗方案和治疗疗效及并发症。中位随访时间56.0(1.1～133.3)个月。

结果

在75例转移性NB患儿中，男性52例(69.3%)，女性23例(30.7%)。中位诊断年龄为39.1个月。中危组17例(22.7%)，极高危组58例(77.3%)。52例(69.3%)伴有骨髓转移，45例(60.0%)伴有骨转移，30例(40%)伴有淋巴结转移。患儿5年总体生存率(OS)为(45.3±5.7)%，5年无事件生存率(EFS)为(31.1±5.6)%；中危组和极高危组的5年OS分别为(80.7±12.7)%和(43.0±6.5)%(P<0.001)。患儿骨髓在2个疗程内获得完全缓解的5年OS为(64.3±9.1)%，在3个疗程后获得完全缓解的5年OS为(40.0±11.0)%(P＝0.017)；患儿单部位骨转移的5年OS为(66.7±11.1)%，多发骨转移患儿5年OS为(33.3±9.1)%(P＝0.045)。多因素分析显示，骨转移(HR: 2.2, 95%CI 1.1～4.8, P＝0.028)是NB患者的独立预后因素。

结论

骨和骨髓是儿童神经母细胞瘤最常见的转移部位，骨髓2疗程内完全缓解以及单部位骨转移患儿的预后较好。N-MYC基因扩增阳降低了年龄小于18个月龄患儿的生存率，确诊时血乳酸脱氢酶、神经烯醇化酶、铁蛋白、尿VMA等指标对转移性NB的预后无显著影响。

引用本文: 汤梦婕, 袁晓军, 安霞, 等. 转移性神经母细胞瘤伴骨和骨髓转移的治疗疗效及预后分析 [J] . 中华转移性肿瘤杂志, 2019, 02(3) : 18-24. DOI: 10.3760/cma.j.issn.2096-5400.2019.03.004.

参考文献导出: Endnote NoteExpress RefWorks NoteFirst 医学文献王

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神经母细胞瘤(Neuroblastoma, NB)是儿童最常见的颅外肿瘤，15岁以下儿童中发病率每年约10.54/100000例，90%的患儿小于5岁^[1]。确诊时伴有转移是神经母细胞瘤的主要高危因素之一，其临床特点、不同转移部位对预后的影响并未被广泛地研究^[2]。本研究通过回顾性分析我院收治的初发转移性NB患者，从而评估转移性NB患儿临床特点、疗效及预后。

贡献者信息

汤梦婕

上海交通大学医学院附属新华医院小儿血液肿瘤科　200092

袁晓军

上海交通大学医学院附属新华医院小儿血液肿瘤科　200092

安霞

江苏省淮安市妇幼保健院小儿内科　223002

张勤

上海交通大学医学院附属新华医院小儿血液肿瘤科　200092

盛琦

上海交通大学医学院附属新华医院小儿血液肿瘤科　200092

谈珍

上海交通大学医学院附属新华医院小儿血液肿瘤科　200092

通信作者

袁晓军

上海交通大学医学院附属新华医院小儿血液肿瘤科　200092

Email：xhxjyuan@hotmail.com

关键词

神经母细胞瘤; 转移; 儿童; 临床特征; 预后;

利益冲突

利益冲突　所有作者均声明不存在利益冲突。

历史

出版日期：2019-09-30

收稿日期：2019-06-25

本文编辑

李子卓

Original Article

Outcome and prognostic factors of children with bone marrow or bone metastatic neuroblastoma

Mengjie Tang, Xiaojun Yuan, Xia An, Qin Zhang, Qi Sheng, Zhen Tan

Published 2019-09-30

Cite as Chin J Metastatic Cancer, 2019, 02(3): 18-24. DOI: 10.3760/cma.j.issn.2096-5400.2019.03.004

Abstract

Objective

The presence of metastases is the risk factors in patients with neuroblastoma (NB). The aim of this study was to evaluate the clinical features and outcomes of metastatic NB treated in Xinhua hospital.

Methods

We analyzed 75 NB patients with metastases from January 2007 to December 2014 retrospectively in our hospital, including clinical manifestations, laboratory tests, stage, risk groups, pathology subtype, treatment protocol, outcome and complications were analyzed. Median follow-up time was 56.0 months with a minimum of 1.1 months and a maximum of 133.3 months.

Results

In all 75 NB patients with metastasis, fifty-two were male (69.3%) and twenty-three female (30.7%), middle age at diagnosis was 39.1 months. Seventeen cases (22.7%) were intermediate risk group, fifty-eight (77.3%) were in very high risk group. Fifty-two cases (69.3%) had metastasis with bone marrow at diagnosis, forty-five (60.0%) with bone and thirty (40.0%) with lymph nodes. The 5-year overall survival (OS) and event-free survival (EFS) were (45.3±5.7)% and (31.1±5.6)% respectively. The 5 year OS in intermediate risk and very high risk group were (80.7±12.7)% and (43.0±6.5)%(P<0.001). The 5-year OS in early and late bone marrow response groups were (64.3±9.1)% and (40.0±11.0)%(P=0.017), the 5-year OS in single bone and multiple bone metastases were (66.7±11.1)% and (33.3±9.1)% respectively (P=0.045). The multivariate analysis revealed that patients with bone metastasis (HR: 2.2, 95%CI 1.1-4.8, P=0.028) could be regarded as independent poor prognostic factors.

Conclusions

The most common metastatic sites of NB were bone marrow and bone, children with complete remission of bone marrow less than 2 courses or with single bone metastasis had a better prognosis. N-MYC gene amplification reduced the survival rate of the children younger than 18 months, the parameters such as serum lactate dehydrogenase, neuron-specific enolase, ferritin, and urine VMA at the time of diagnosis had no independent prognostic value.

Key words:

Neuroblastoma; Metastasis; Children; Clinical features; Prognosis

Contributor Information

Mengjie Tang