Liu Denghui,Tang Xianglian,Huang Zhao,et al.Clinical features of neuroblastoma in children with concurrent paraneoplastic syndrome[J].Journal of Clinical Pediatric Surgery,2021,20(04):348-353.[doi:10.12260/lcxewkzz.2021.04.009]
伴副肿瘤综合征儿童神经母细胞瘤临床特征分析
- Title:
- Clinical features of neuroblastoma in children with concurrent paraneoplastic syndrome
- Keywords:
- Paraneoplastic Syndrome; Neuroblastome; Child
- 分类号:
- R726;R73
- 摘要:
- 目的 探讨伴有副肿瘤综合征儿童神经母细胞瘤的临床特点、治疗策略及预后。方法 回顾性分析湖南省儿童医院2010年1月至2019年12月收治的9例伴副肿瘤综合征神经母细胞瘤患者的临床资料，包括一般资料、副肿瘤综合征症状、肿瘤情况、治疗策略及预后。随访截至2020年6月1日。结果 9例中男5例，女4例；起病年龄为19.0~48.2个月，出现副肿瘤综合征症状至确诊神经母细胞瘤后开始治疗时间为2.1~5.7个月；神经系统受累5例，消化系统受累2例，心血管系统受累1例，血液系统受累1例（骨髓未受累）；组织学分类：神经母细胞瘤3例，其中预后良好型2例，预后不良型1例；节细胞性神经母细胞瘤6例，其中结节型2例，混杂型4例。国际神经母细胞瘤分期系统（International Neuroblastoma Staging System，INSS）临床分期：Ⅱ期7例，Ⅲ期2例。美国儿童肿瘤协作组（Children’s Oncology Group，COG）危险度分组：低危组3例，中危组6例。肿瘤N-MYC基因均无扩增；采用手术、系统化疗和（或）间断大剂量人血丙种球蛋白、糖皮质激素治疗等综合治疗可缓解副肿瘤综合征；所有患者获有效随访，随访时间23.0~67.5个月，平均随访（34.5±8.2）个月，无事件生存率66.7%，总体生存率100%。结论 副肿瘤综合征在儿童神经母细胞瘤中可累及多个系统，但以神经系统最为常见，尽早切除原发肿瘤病灶、系统化疗及免疫抑制等综合治疗可有效缓解副肿瘤综合征，伴副肿瘤综合征的儿童神经母细胞瘤预后良好。
- 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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备注/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