Clinical features and prognosis of neuroblastoma with intraspinal extension in children / 中华实用儿科临床杂志

ABSTRACT

Objective:To explore the clinical features, risks of recurrence and prognosis of neuroblastoma (NB) with intraspinal extension in children, and to perform a long-term follow-up to monitor their health problems.Methods:Clinical data of 22 children with NB with intraspinal extension treated in Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2007 to December 2019 were retrospectively analyzed, including gender, age at diagnosis, clinical stage, and risks of recurrence.They were divided into non-recurrent group and recurrent group, and the survival was analyzed by Kaplan-Meier method.Results:(1)Fifteen(68.2%) children had motor nervous symptoms as the initial symptoms, including lower limb pain, weakness, and limited mobility, and 10(45.5%) showed moderate-to-severe nervous compression.(2)Neuronal enolase(NSE) (<200 μg/L), lactate dehydrogenase(LDH) (≤500 U/L) and vanillylmandelic acid(VMA) were in the normal range in most of children in the non-recurrence group, and a single site was involved.(3)Tumor lesions were not completely resected in the majority of children in the recurrent group, and their NSE (≥200 μg/L), LDH (>500 U/L) and VMA (more than 3 times higher) were abnormally higher, or suffered multisite pyramidal metastasis.(4)The median survival time (MST) was 119.4 months for children in the non-recurrent group, while it was only 25.3 months for those in the recurrent group.The 3-year overall survival (OS) rate was (95.5±6.4)% for the non-recurrent group, compared to only (20.0±17.9)% for children in the recurrent group ( χ2=9.387, P=0.002). Likewise, the 3-year event-free survival (EFS) rate for children in the non-recurrent group was (94.1±5.7)%, whereas it was only (20.0±17.9)% for children in the recurrent group( χ2=29.700, P<0.001). (5)Eleven of 22 children had long-term health problems, especially motor nerve function defects and scoliosis. Conclusions:Motor neurological, and moderate-to-severe neurological compression predominates are the main symptoms of NB children with intraspinal extension at the initial diagnosis.Patients who exhibit intradural tumor remnants, higher NSE, LDH, or VMA levels, and intradural extension with multiple sites of cone metastases are prone to recurrence.Once relapsed, the prognosis is extremely poor.The prognosis of NB with intraspinal extension in children is related to the severity of the initial neurological impairment, the duration of tumor compression, and the treatment regimen.Early diagnosis and intervention may reduce the risk of long-term health problems in children.