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中国循证儿科杂志

中国循证儿科杂志 ›› 2023, Vol. 18 ›› Issue (2): 106-109.DOI: 10.3969/j.issn.1673-5501.2023.02.005

• 论著 • 上一篇    下一篇

非高危神经母细胞瘤患儿预后病理分型的回顾性队列研究

黄程1,苏雁1,张诗晗1,蒋持怡1,何乐健2,曾骐3,王焕民4,马晓莉1,倪鑫5   

  1. 首都医科大学附属北京儿童医院北京，100045，1 儿童肿瘤中心肿瘤内科，北京市儿童血液肿瘤重点实验室，儿科重大疾病研究教育部重点实验室；2 病理科；3 胸外科；4 儿童肿瘤中心肿瘤外科；5 耳鼻咽喉头颈外科
  • 收稿日期:2021-12-19 修回日期:2023-05-15 出版日期:2023-04-25 发布日期:2023-05-19
  • 通讯作者: 倪鑫

Prognostic pathological staging in children with non-high-risk neuroblastoma: A retrospective case-control study

HUANG Cheng1, SU Yan1, ZHANG Shihan1, JIANG Chiyi1, HE Lejian2, ZENG Qi3, WANG Huanmin4, MA Xiaoli1, NI Xin5   

  1. Beijing Children’s Hospital, Capital Medical University Beijing 100045,China，1 Medical Oncology Department, Pediatric Oncology Center，Beijing Key Laboratory of Pediatric Hematology Ocology, Key Laboratory of Major Diseases in Children,Ministry of Education; 2 Pathology Department; 3 Thoracic Surgery Department; 4 Surgical Oncology Department of Pediatric Oncology Center; 5 Otolaryngology, Head and Neck Surgery Department
  • Received:2021-12-19 Revised:2023-05-15 Online:2023-04-25 Published:2023-05-19
  • Contact: NI Xin, email: nixin@bch.com.cn

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摘要： 背景:神经母细胞瘤（NB）是最常见的儿童颅外实体肿瘤，临床表现及预后差异较大。 目的:探讨非高危NB患儿不同预后病理分型的临床特点和预后。 设计:回顾性队列研究。 方法:回顾性纳入2007年3月至2020年12月首都医科大学附属北京儿童医院肿瘤内科病房收治的初诊非高危NB患儿，根据INPC分类系统分为预后良好（FH）组和预后不良（UH）组，分析不同预后病理分型的临床特点和预后。生存分析采用KaplanMeier方法。 主要结局指标:3年和5年总体生存率（OS）和无事件生存率（EFS）。 结果:445例患儿纳入分析，FH组313例，<18月龄164例（52.4%），原发部位以纵隔最多见（52.7%），远处转移52例(16.6%)；UH组132例，<18月龄25例（18.9%），原发部位以腹膜后最多见（49.2%），远处转移10例(7.6%)。两组年龄分布、原发瘤灶部位和远处转移发生率差异均有统计学意义，性别、瘤灶最大直径和LDH水平差异均无统计学意义。中位随访时间34（0.16~166）月。FH组和UH组3年EFS分别为96.4%和92%，5年EFS分别为95.4%和87.8%，差异均有统计学意义（χ2分别为1.63和4.75，P分别为0.046和0.029）。FH组和UH组3年OS分别为98.3%和98.4%，5年OS分别为98%和98.4%，差异无统计学意义（χ2分别为0.76和0.54，P分别为1.53和0.82）。 结论:非高危NB患儿中，UH患儿较FH患儿发病年龄更大、原发瘤灶更多位于腹膜后、远处转移发生率更低。FH患儿较UH患儿有着更好的EFS，可考虑减轻化疗强度。

关键词: 神经母细胞瘤, 病理分型, 预后良好, 预后不良, 生存分析

Abstract: Background：Neuroblastoma is the most common extracranial solid tumor in children, and its clinical presentation and prognosis vary widely. Objective：To investigate the clinical features and prognostic analysis of different international neuroblastoma pathology classification in children with nonhighrisk neuroblastoma. Design：Retrospective cohort study. Methods：We retrospectively collected clinical data of nonhighrisk neuroblastoma children admitted to the medical oncology ward at Beijing Children's Hospital, Capital Medical University from March 2007 to December 2020. We divided the children into favorable histology（FH）group and unfavorable histology（UH）group according to the INPC classfication system. We analyzed the clinical features of the different international neuroblastoma pathology classification and performed a prognostic analysis. Survival analysis was carried out using the KaplanMeier method. Main outcome measures：3year and 5year overall survival(OS) and eventfree survival(EFS) rates. Results：A total of 445 children were included in the clinical data, including 313 children in the FH group. There were 164 cases (52.4%) younger than 18 months of age; the mediastinum was the most common site of origin (52.7%), with distant metastases in 52 cases (16.6%). In the UH group, there were 132 cases, with 25 cases (18.9%) younger than 18 months of age; the primary site was most common in the retroperitoneum (49.2%) and 10 cases (7.6%) had distant metastases. The differences in age distribution, primary site and incidence of distant metastases between the two groups were statistically significant, while the differences in gender, maximum diameter of the tumour and LDH level were not statistically significant. The median followup time was 34 (0.16166) months. The 3year EFS was 96.4% and 92% in the FH and UH groups, respectively, and 5year EFS was 95.4% and 87.8%, respectively, with statistically significant differences (χ2was 1.63 and 4.75, P was 0.046 and 0.029 , respectively). The 3year OS was 98.3% and 98.4% in the FH and UH groups, respectively, and 5year OS was 98% and 98.8%, respectively, with no statistically significant difference (χ2was 0.76 and 0.54, P was 1.53 and 0.82, respectively). Conclusions：Among children with nonhighrisk neuroblastoma, patients in the UH group are older than those in the FH group, have more retroperitoneal primary tumours and a lower incidence of distant metastases. Children with FH have better OS and EFS than children with UH and may be considered for less intensive chemotherapy.

Key words: Neuroblastoma, International Neuroblastoma Pathology Classification, favourable histology, Unfavorable histology, Prognostic analysis