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目的总结较少见的儿童非典型临床表现神经母细胞瘤(neuroblastoma,NB)的临床特点及治疗经验。方法回顾分析中山大学孙逸仙纪念医院2011年8月至2012年12月收治的2例儿童非典型临床表现NB的病例资料及诊疗情况。结果 2例非典型临床表现NB患儿初诊时仅骨髓涂片可见典型的呈“菊花团样”堆积的NB细胞,未发现有骨髓外的实质性瘤块;而且除此之外,2例患儿均有血清神经元特异性烯醇化酶(NSE),尿香草杏仁酸(VMA)及外周血NB微小残留病灶(MRD)的增高;2例起病时有骨髓浸润或骨的浸润。2例患儿均完成9个疗程的高危诱导方案化疗,例1在诱导化疗结束后获得完全缓解,因经济原因未予巩固方案,随访6个月后骨髓复发;例2在整个诱导方案结束后获得部分缓解,最后由于经济原因放弃进一步治疗。结论由于儿童非典型临床表现NB缺乏实质性瘤块,所以化疗是目前唯一有效的治疗方式。即使采用强化疗,非典型临床表现NB的预后仍较差,尤其是存在骨转移的患儿。
Objective To summarize the clinical features and treatment experience of the less common childhood atypical neuroblastoma (NB). Methods A retrospective analysis of Zhongshan Hospital Sun Yat-sen Memorial Hospital from August 2011 to December 2012 admitted to the two cases of children with atypical clinical manifestations of NB cases and treatment of cases. Results 2 cases of atypical clinical manifestations NB children newly diagnosed with bone marrow smear showed typical “chrysanthemum like” accumulation of NB cells, found no extramedullary solid tumor mass; and in addition, 2 Serum neuron-specific enolase (NSE), urinary vanillin (VMA) and NB minimal residual disease (MRD) were increased in all cases. Two cases had infiltration of bone marrow or infiltration of bone marrow at the time of onset. In both cases, 9 courses of high-risk induction chemotherapy were completed. In case 1, complete remission was achieved at the end of induction chemotherapy. Unconsolidated patients were excluded due to economic reasons. The bone marrow recurred after 6 months of follow-up. Example 2 After the entire induction program was completed Get partial relief, and finally give up further treatment for economic reasons. Conclusion As the clinical manifestation of atypia in children NB lack of substantial tumor mass, chemotherapy is currently the only effective treatment. Even with intensive chemotherapy, the prognosis of atypical clinical manifestations, NB, is still poor, especially in children with bone metastases.