神经母细胞瘤副肿瘤综合征临床分析

Analysis of paraneoplastic syndromes in neuroblastoma

目的总结儿童神经母细胞瘤(NB)副肿瘤综合征的临床特点,提高对其认识。方法通过整理分析我科近年收治的合并有副肿瘤综合征NB患儿的临床资料,并回顾相关文献,归纳总结NB副肿瘤综合征的特点。结果近年我科收治的68例NB患儿中有9例(13%)伴有副肿瘤综合征的表现;神经系统受累4例(44%),消化系统2例(22%),心血管系统2例(22%),血液系统1例(骨髓未受累,11%);组织学分类:神经母细胞瘤6例,神经节母细胞瘤3例;人血丙种球蛋白和(或)糖皮质激素治疗可缓解副肿瘤综合症;随诊期2例复发,2年生存率71%。结论 NB副肿瘤综合征可涉及多个系统,但以神经系统最常见;免疫治疗可缓解副肿瘤综合征;合并有副肿瘤综合征的NB患儿预后似较好。

Objective To summarize the characteristic of neuroblastoma＇ s paraneoplastic syndrome. Methods Collected the clinical data of NB patients with paraneoplastic syndrome, and review the literatures from Pubmed. Results Among 68 NB patients admitted in our institution during the past 3 years, there were 9 patients （13%） represented with paraneoplastic syndrome, in whom 4 patients （44%） involved nervous system, 2 patients （22%） withcardiovascular system, 2 patients （22%） with digestive system, and 1 patient （11%） with hematological system. On histological classification, 6 patients were diagnosed with neuroblastoma, 3 patients ganglioneuroblastoma. Paraneoplastic syndromes could be relieved to some extent, after inmmnoglobulin and/or glucocorticoids therapy, 2 patients relapsed, 2-year overall survival was 71%. Conclusions Paraneoplastic syndromes in NB may involve nmhiple systems, nervous system was the most common. Most paraneoplastic syndrome lesponded to inmmne therapy. Paraneoplastic syndromes might be a favorite prognostic factor.