Clinical characteristics and prognosis of high-risk neuroblastoma patients with skeletal metastases at different sites
10.3760/cma.j.cn101070-20220629-00792
- VernacularTitle:不同骨骼转移部位高危神经母细胞瘤患儿的临床特征与预后
- Author:
Hongjun FAN
1
;
Cheng HUANG
;
Yuchen ZHOU
;
Shuai ZHU
;
Chao DUAN
;
Wen ZHAO
;
Qian ZHAO
;
Xisi WANG
;
Dawei ZHANG
;
Mei JIN
;
Xiaoli MA
;
Yan SU
Author Information
1. 国家儿童医学中心（北京），首都医科大学附属北京儿童医院，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，北京 100045
- Keywords:
Neuroblastoma;
High risk;
Skeletal metastasis;
Prognosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2023;38(5):331-338
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical characteristics and prognostic factors of high-risk neuroblastoma (HR-NB) patients with skeletal metastasis.Methods:The clinical features of 336 newly treated HR-NB patients with skeletal metastases admitted to the Department of Medical Oncology of Beijing Children′s Hospital, Capital Medical University from January 2007 to December 2018 were analyzed retrospectively.Kaplan-Meier method was used for the survival analysis, and Log- Rank test was used for univariate prognosis analysis.The Cox model was used to analyze the multifactorial prognostic analysis. Results:A total of 336 HR-NB patients were recruited, involving 188 males and 148 females with the median age of onset of at 43 (4-148) months.Skeletal metastases affected the viscerocranium (89 cases, 26.5%), neurocranium (193 cases, 57.4%), vertebrae (298 cases, 88.7%), sternum and ribs (183 cases, 54.5%), pelvis (270 cases, 80.4%), upper limbs (182 cases, 54.2%) and lower limbs (240 cases, 71.4%). The 5-year event-free survival (EFS) rate and overall survival (OS) rate were (30.4±2.7)% and (41.3±2.9)%, respectively.Univariate analysis showed a significantly lower 5-year OS rate in skeletal metastatic HR-NB patients with poor prognostic classification, the morphology of neuroblastoma (stroma-poor) and ganglioneuroblastoma (intermixed), high index of mitosis-karyorrhexis index, lactate dehydrogenase≥587 U/L, serum ferritin≥92 μg/L, MYCN amplification and 1p loss of heterozygosity, and metastases in the viscerocranium, neurocranium, vertebrae, sternum and ribs, pelvis, upper limbs and lower limbs (all P<0.05). The 5-year OS rate of HR-NB patients with all 7 regions of skeletal metastases was only (14.2±5.9)%, which was significantly lower than that in patients with a single region metastasis or multi-region metastases[(66.0±10.2)% vs.(43.6±3.4)%, χ2=45.722, P<0.05]. Cox multifactorial analysis showed that MYCN amplification ( HR=4.165, 95% CI: 2.356-7.363) and the viscerocranium metastasis ( HR=2.560, 95% CI: 1.519-4.315) were the independent risk factors affecting the prognosis of HR-NB patients with skeletal metastases (all P<0.05). Conclusions:The prognosis is extremely poor in HR-NB patients with multiple skeletal metastases at the initial diagnosis.The amplification of MYCN and the viscerocranium metastasis are the poor prognostic factors for HR-NB patients with skeletal metastases.
