Liu Denghui,Tang Xianglian,Huang Zhao,et al.Clinical features of neuroblastoma in children with concurrent paraneoplastic syndrome[J].Journal of Clinical Pediatric Surgery,,20():348-353.[doi:10.12260/lcxewkzz.2021.04.009]
Clinical features of neuroblastoma in children with concurrent paraneoplastic syndrome
- Keywords:
- Paraneoplastic Syndrome; Neuroblastome; Child
- CLC:
- R726;R73
- Abstract:
- Objective To explore the clinical characteristics, treatment strategies and prognoses of neuroblastoma (NB) in children with paraneoplastic syndrome.Methods From January 2010 to December 2019, clinical data were retrospectively analyzed for 9 NB children with concurrent paraneoplastic syndrome.And analysis was performed for clinical data, symptoms of paraneoplastic syndrome, thrapy methods and prognosis.Results 9 patients presented with paraneoplastic syndrome.There were 5 boys and 4 girls with a median onset age of 32.0(19.0-48.2) months.The period from an occurrence of paraneoplastic syndrome symptoms to starting treatment upon a definite diagnosis was 2.1 to 5.7 months.The involved system was nervous (n=5), digestive (n=2), cardiovascular (n=1) and hematological (n=1).The histopathological classification was neurolastoma (n=3), including differentiated (n=2) and poorly differentiated (n=1) type.And the pathological type was ganglion cell neuroblastoma (n=6), including nodular (n=2) and mixed (n=4).INSS was Ⅱ (n=7) and Ⅲ (n=2).Clinical risk was low(n=3) and intermediate (n=6) by Children’s Oncology Group(COG).N-MYC was not amplified.Resection of primary tumor was followed by systemic chemotherapy and/or immunotherapy with intermittent high-dose intravenous immunoglobulin, glucocorticoid impact.Paraneoplastic syndromes became markedly relieved.The average effective follow-up period was (34.5±8.2) (23.0-67.5) months, event-free survival rate 66.7% and overall survival rate 100%.Conclusion Paraneoplastic syndromes in NB may involve multiple systems and nervous system is the most common target.Most cases of paraneoplastic syndrome responded to tumor resection, systemic chemotherapy and immunosuppression.Often NB children with concurrent paraneoplastic syndrome have an excellent prognosis.
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Memo
收稿日期:2020-09-21。
基金项目:湖南省儿童医院2020年度院级科研基金资助项目;湖南省自然科学基金-面上项目(编号:2018JJ2210)
通讯作者:李勇,Email:liyongpuwaike@163.com