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目的探讨31例神经母细胞瘤(NB)患儿的诊治特点。方法分析31例确诊的NB患儿的临床资料。结果 31例NB患儿共15例(48.4%)血乳酸脱氮酶增高;VMA值增高仅8例(25.8%);8例行N-MYC基因检测,4例阴性患儿(Ⅰ期3例、Ⅱ2A期1例)存活,4例阳性者为Ⅳ期,其中3例已死亡;31例患儿中11例完全缓解(CR)(其中10例Ⅰ期和1例Ⅱ2A期患儿);2例非常好的部分缓解(VGPR)(均为ⅣS期患儿);4例部分缓解(PR)(为Ⅳ期存活患儿);死亡14例(1例Ⅲ期患儿,13例Ⅳ期患儿)。结论患儿的VMA值正常不能排除神经母细胞瘤;多部位骨髓穿刺,可提高NB诊断率;肿瘤组织N-MYC基因扩增数可作为NB患儿重要预后因素;婴儿期NB治疗效果佳;Ⅳ期患儿预后差,个体化综合治疗有望改善预后。
Objective To investigate the diagnosis and treatment of 31 children with neuroblastoma (NB). Methods Clinical data of 31 confirmed children with NB were analyzed. Results There were 15 cases (48.4%) with elevated lactate dehydrogenase in 31 children with NB, only 8 cases (25.8%) with increased VMA, 8 cases with N-MYC and 4 cases with negative , 1 case of stage Ⅱ 2A) survived, 4 cases were positive stage Ⅳ, of which 3 cases had died; 11 cases of complete remission (CR) in 11 cases (10 cases of stage Ⅰ and 1 case of stage Ⅱ 2A); 2 (VGPR) (all children with stage IVS); 4 patients with partial remission (PR) (survived by stage IV); 14 patients died (1 patient with stage III disease, 13 patients with stage IV disease child). Conclusion The normal VMA in children can not rule out neuroblastoma. Multi-site bone marrow biopsy can improve the diagnostic rate of NB. The number of N-MYC gene in tumor can be used as an important prognostic factor in NB. Stage IV children with poor prognosis, individualized comprehensive treatment is expected to improve prognosis.