颈部淋巴结转移的神经母细胞瘤患儿单中心10年临床特征及生存分析

目的总结伴颈部淋巴结转移的神经母细胞瘤(NB)患儿的临床特征,分析生存状况,提高对此类患儿的认识。方法回顾性分析2007年4月至2017年5月首都医科大学附属北京儿童医院血液肿瘤病房确诊的伴颈部淋巴结转移的NB患儿。按照治疗时间,即2007年4月1日至2011年12月31日和2012年1月1日至2017年5月31日分为2个阶段,进行治疗效果和生存率分析,随访时间至2018年1月31日。结果1.共225例伴有颈部淋巴结远处转移患儿(占NB住院患儿的为37.3%),其中男125例(55.6%),女100例(44.4%);中位发病年龄37个月(2~147个月),≤18个月27例(12.1%)。原发瘤灶位于腹膜后肾上腺区174例(77.3%),位于后纵隔48例(21.3%)。原发于盆腔、椎管及胸廓各1例。仅伴颈部淋巴结转移(4N期)的患儿33例(14.7%);同时伴骨髓转移139例(62.1%)、骨转移159例(71.0%)。高危组患儿208例(91.2%),中低危组17例(7.5%)。2.共200例患儿于接受系统治疗和随访,随访时间为2~123个月,中位随访时间23个月。所有患儿预计3年无事件生存(EFS)率为52.0%,总生存(OS)率为56.4%。5年EFS为39.7%,OS为46.5%。2007年4月1日至2011年12月31日住院期间患儿3年EFS为49.6%,OS为54.4%;5年EFS为39.5%,OS为44.7%。2012年1月1日至2017年5月31日住院患儿,3年EFS为58.1%,OS为57.3%;5年EFS为48.7%,OS为46.8%。4N期NB患儿3年EFS为71.2%,OS为82.2%,5年EFS为61.5%,OS为76.7%。结论颈部淋巴结是NB远处转移的常见部位,多数同时伴骨髓和骨骼转移。仅伴颈部淋巴结转移(4N期)患儿预后较好,但仍低于国外报道,可能与患儿就诊时病情较重,高危组患儿比例高等有关。

Clinical features and survival analysis of neuroblastoma with cervical lymph node metastasis in children:su-mmary of 10 years in single center

Objective To improve the awareness of neuroblastoma(NB)with cervical lymph node metastasis in children patients by summarizing the clinical features of such patients and analyzing their survival situation.Methods The research analyzed the medical records of 225 patients with cervical lymph node metastasis of NB from April 1,2007 to May 31,2017 in Beijing Children′s Hospital,Capital Medical University.The treatment were divided into 2 phases according to treatment time(one from April 1,2007 to December 31,2011 and the other from January 1,2012 to May 31,2017).The survival situation and treatment effect in each phase were analyzed.The following up time ended at Jan 1,2018.Results(1)Clinical features:total 225 cases admitted in the research with about 37.3%of all the NB patients.One hundred girls(44.4%)and 125 boys(55.6%).The age from 2 months to 147 months,the me-dian age of patients was 37 months.The age under 18 months were 27 cases(12.1%).The majority primary site of tumor was located in retroperitoneal(174 cases,77.3%)and mediastinum(48 cases,21.3%).There were 33 cases which metastasic site confined to cervical lymph node(4N stage,14.7%);139 cases(62.1%)with bone marrow metastasis,159 cases(71.0%)with bone metastasis.The number of patients in high-risk group was 208 cases(91.2%),while the low-risk and the medium-risk group was 17 cases(7.5%).(2)Treatment effect and survival analysis:a total of 200 cases received systemic treatment and followed-up in our hospital.The follow up time from 2 months to 123 months,the median follow up time was 23 months.The 3 year overall survival(OS)rate of all the patients was 56.4%,the 3 year event free survival(EFS)rate was 52.0%.While the 5-year OS was 46.5%,and the 5-year EFS was 39.7%.According to the first phase of treatment(April 1,2007 to December 31,2011),the 3-year EFS was 49.6%,and the 3-year OS was 54.4%,the 5-year EFS was 39.5%,and the 5-year OS was 44.7%.According to the second phase of treatment(January 1,2012 to May 31,2017),the 3-year EFS was 58.1%,and the 3-year OS was 57.3%.The 5-year EFS was 48.7%,and the 5-year OS was 46.8%.About the 4N patients,the 3-year EFS was 71.2%,and the 3-year OS was 82.2%.The 5-year EFS was 61.5%,and the 5-year OS was 76.7%.Conclusions Cervical lymph node is one of the most common distant metastasis of NB.More than half of the patient with bone or bone marrow metastasis.The prognosis of patients′metastatic disease limited to cervical lymph nodes(4N stage),but still worse than the foreigner haver reported.The probable reason is the proportion of high-risk patients in the center is higher than the foreign research.