神经母细胞瘤并骨转移患儿临床特征及预后相关因素的单中心分析

目的分析伴骨转移神经母细胞瘤(neuroblastoma,NB)患儿的临床特征及预后相关因素,总结临床诊疗经验,以提高伴骨转移NB患儿的生存率。方法以2013年12月至2020年12月重庆医科大学附属儿童医院肿瘤外科收治的伴骨转移NB患儿为研究对象,收集并总结患儿临床资料及预后情况。随访时间截至2021年3月31日。结果共收集97例NB患儿,男68例,女29例,男女比例为2.4∶1;中位年龄为49.4个月。首发症状:发热47例(48.5%),骨痛38例(39.2%),腹痛或腹胀29例(29.9%),咳嗽15例(15.5%);伴骨转移的NB患儿存在多类骨转移(62.9%),且合并骨髓转移(73.2%),整体预后差,1年生存率为93.6%,5年生存率仅20.2%。经生存分析发现,女性、伴多类骨转移、肿瘤位于腹部、首诊时LDH测定值大于660 U/L以及术中肿瘤残留是预后不佳的影响因素,其中手术切除范围是独立预后影响因素。结论伴骨转移的NB患儿临床表现多样,其预后受诸多因素影响,肿瘤复发或进展是主要的致死原因。对于此类患儿,建议术中尽可能完全切除肿瘤,以改善预后。

Single-center analysis of clinical features and prognosis of neuroblastoma children with bone metastasis

Objective To analyze the clinical features and prognostic factors of neuroblastoma(NB)children with bone metastasis.Methods The clinical data of 97 cases of NB children with bone metastasis treated in the Department of surgical Oncology,Children's Hospital of Chongqing Medical University from December 2013 to December 2020 were retrospectively analyzed to compare the influence of different factors on the prognosis of the children.Results Among the 97 cases,there were 68 males and 29 females.The male to female ratio was 2.4:1,and the median age was 49.4 months.The common first symptoms were fever(48.5%),bone pain(39.2%),abdominal pain or abdominal distension(29.9%).The children with bone metastasis were prone to multiple bone metastasis(62.9%)and bone marrow metastasis(73.2%).The overall prognosis of NB children with bone metastasis was poor,with a 1-year overall survival rate of 93.6% and a 5-year overall survival rate of 20.2%.Survival analysis showed that female children,patients with multiple bone metastases,tumors located in the abdomen,LDH value greater than 660U/L at first diagnosis,and intraoperative tumor residue often suggested poor prognosis,among which surgical resection range was an independent prognostic factor.Conclusion NB children with bone metastasis have diverse clinical manifestations,and their prognosis is affected by many factors,and tumor recurrence or progression is the main cause of death.For such children,it is recommended that the tumor be removed as completely as possible intraoperatively to improve the prognosis.