神经母细胞瘤规范化诊治的初步经验

Treatment results of neuroblastoma with a standardization protocol

目的总结按日本研究会方案治疗神经母细胞瘤的经验体会。方法2000年至2006年间诊治新发小儿神经母细胞瘤98例，年龄12d～144个月，经影像学、24h尿香草扁桃酸、骨穿和手术病理确诊。按INKS分期，Ⅰ期3例、Ⅱ期13例、Ⅲ期31例、Ⅳ期48例、Ⅳs期3例。治疗方案按日本研究会神经母细胞瘤综合治疗方案98年修订版进行。结果本组术前新辅助化疗缓解率为49．2％（29／59），手术肉眼完整切除率为60．8％。总体2年生存率为73．4％，总体5年生存率为47．8％，其中Ⅲ期分别为75．0%和58．8％，Ⅳ期分别为64．1％和20．0％。结论规范化治疗是提高神经母细胞瘤治疗效果的重要手段。日本研究会方案是较为可行的方案之一，可有效提高患儿的生存率。肿瘤肉眼完整切除是神经母细胞瘤综合治疗的重要部分，外科医师参与并掌握治疗方案，是规范化治疗得以执行的保证。

Objective To summarize our experience on the treatment of neuroblastoma on the basis of the Japanese Study Group Protocol. Methods Ninety-eight newly diagnosed patients with neuroblastoma were included in this study from January 2000 to December 2006. The age of the patients was from 12 days to 144 months. The diagnosis was made by imageology, quantization of urine VMA for 24 hours, bone marrow biopsy and postoperative pathological diagnosis. The staging of patients was determined by INSS system as follows： 3 cases in stage Ⅰ, 13 in Ⅱ, 31 in Ⅲ, 48 in Ⅳ and 3 in Ⅳs. The treatment scheme was on the basis of the Japanese study group protocol which was modified in 1998. Results The remission rate after the new assistant chemotherapy was 49. 2%. Gross total rsection rate of this group was 60. 8%. The overall survival rates of 2 and 5 years in this group were 73. 4% and 47. 8% respectively. Among the total of the above-mentioned, survival rates of patients in stage Ⅲ were 75% and 58. 8% respectively, and that in stage Ⅳ were 64. 1 % and 20% respectively. Conclusions Standardized treatment is an important way to improve the survival rate of neuroblastoma. Japanese study group protocol may be of significant value in increasing the remission rate. Since gross total resection of the tumor takes an important role in the neuroblastoma treatment, surgeons should join in the clinical practice of the polychemotherapy and master the whole protocol.