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目的本研究回顾性分析中危神经母细胞瘤患者的临床特点和治疗结果。方法以美国儿童肿瘤组织(Children’s Ocology Group,COG)神经母细胞瘤分层标准为基础,但略有不同。中危神经母细胞瘤定义:A:年龄0～21岁无MYCN基因扩增的3期患者;B:年龄0～548 d、无MYCN基因扩增的4期患者。分析中山大学肿瘤防治中心儿童肿瘤科2004年1月至2012年12月收治的中危神经母细胞瘤患者临床资料。治疗采用手术+化疗±放疗±异维甲酸。化疗方案CAV/EP交替4～6个疗程。结果共22例神经母细胞瘤患者纳入分析。中位年龄:2(7个月~10)岁,3期18例,4期4例。最常见的起病部位为肾上腺和腹膜后。中位随访49.5个月,2例复发。复发后2例挽救化疗后存活。4例进展死亡,共18例存活。5年总生存率和无事件生存率分别是:(81.6±8.3)%和(60.4±11.2)%。结论中危神经母细胞瘤患者经手术和中等强度化疗可达到较高的总生存率和无事件生存率。中危神经母细胞瘤患者复发后积极挽救治疗仍有生存机会。
Objective This study retrospectively analyzed the clinical features and treatment outcome of patients with neuroblastoma. Methods Based on the Children’s Ocular Group (COG) neuroblastoma stratification criteria, but slightly different. Definition of intermediate-risk neuroblastoma: A: Stage 3 patients with no MYCN gene amplification ranging in age from 0 to 21 years; B: Stage 4 patients aged 0-548 days without MYCN gene amplification. To analyze the clinical data of patients with moderate-risk neuroblastoma admitted to Department of Oncology, Cancer Center of Sun Yat-sen University from January 2004 to December 2012. Treatment with surgery + chemotherapy ± radiotherapy ± isotretinoin. Chemotherapy program CAV / EP alternating 4 to 6 courses. Results A total of 22 patients with neuroblastoma were included in the analysis. Median age: 2 (7 months to 10 years), stage 3 18 cases, stage 4 4 cases. The most common sites of onset are adrenal and retroperitoneal. The median follow-up was 49.5 months and 2 patients relapsed. Two patients survived after salvage chemotherapy. 4 cases of death, a total of 18 cases of survival. The 5-year overall and event-free survival rates were (81.6 ± 8.3)% and (60.4 ± 11.2)%, respectively. Conclusion The patients with moderate-risk neuroblastoma can achieve higher overall survival and event-free survival after operation and moderate-intensity chemotherapy. In patients with moderate-risk neuroblastoma relapse after active rescue treatment still have a chance of survival.