儿童Ⅳ期神经母细胞瘤远期随访报告  

Report on Long-term follow-up of stage Ⅳ neuroblastoma

作　　者：汤静燕[1] 潘慈[1] 刘茵[1] 徐敏[1] 薛惠良[1] 陈静[1] 董璐[1] 周敏[1] 顾龙君[1] 

机构地区：[1]上海交通大学医学院附属上海儿童医学中心血液/肿瘤科

出　　处：《中国小儿血液与肿瘤杂志》2009年第2期66-70,共5页Journal of China Pediatric Blood and Cancer

摘　　要：目的总结分析儿童Ⅳ期神经母细胞瘤(NB)诊断治疗方案的远期疗效及影响预后的因素,为方案的改进提供依据。方法研究对象为1996年4月1日至2005年12月31日新诊断的Ⅳ期NB患儿,年龄1个月至15岁,共69例。其中Ⅳ期67例,Ⅳs期2例。根据年龄将患儿分为中危组和高危组,综合治疗方案包括根据分组采用不同强度化疗、完成化疗后全顺式维甲酸诱导分化治疗及适时肿瘤根治性切除术,起病年龄>1岁的患儿在停药前造血干细胞支持下超剂量化疗(ASCT),部分术后有残留者接受局部放疗。结果16/69例在接受44疗程后好转中放弃治疗。53例按计划治疗>4个疗程,22例接受了ASCT(中危组3例,高危组19例)。获得非常好的部分缓解(very good partial remission,VGPR)49例(92.45%),获得部分缓解(PR)4例(7.55%)。中位随访期28个月(7个月至79个月)。末次随访时在VGPR中13例(24.53%),中位VGPR时间为38个月。带病生存病情稳定5例。疾病进展、复发或已死亡35例(66.04%)。2年总体生存率(OS)和VGPR生存率(VGPRS)分别为64.4%和60.4%,3年OS和VGPRS分别为43.9%和41.2%,4年OS和VGPRS分别为19.8%和14.4%。肿瘤原发于后腹膜、骨髓转移对预后的影响达统计学有效水平,P值均<0.05。结论Ⅳ期NB在强烈化疗等综合治疗下远期预后仍然极差,原发于后腹膜并有骨髓转移者预后更差,需进一步探索研究新的治疗手段,以改善其预后。Objective To evaluate the long-term outcomes of childhood stage Ⅳ neuroblastoma （NB） and the factors correlate with the prognosis. Find the evidence for revising the protocol and improving the prognosis by comprehensive protocols for diagnosis and treatment. Methods Total 69 newly diagnosed NB patients aged form 1 month to 15-year-old from April 1996 to December 2005 were divided into medium and high risk groups depending on age, Comprehensive protocol includes delayed and/or second tumor resection, intensive chemotherapy mainly composed of cell cycle nonspecific drugs and 13-cis-retinoid for inducing cell differentiation. ASCT was given at the end of therapy for older than 12 months group. Results Of the 69 patients, 53 cases were treated as planned. 22 of them received ASCT. Of the 53 patients, 49 （92. 45% ） achieved very good partial remission （ VGPR）, 4 （7.55%） achieved partial remission （PR） after surgery and/or chemotherapy. After a median 28 month （range 7 to 79 months） follow up, 13 eases （24. 53% ） remained VGPR （median 38 months） and 5 survived with stable disease. The survival rate（SR） was 33. 96%. 35 cases（66. 04% ） died of relaple and disease progression. 2 year OS and VGPRS were 64.4% and 60. 4% respectively, 43.9% and 41.2% for 3 year, only 19.8% and 14.4% for 4 year. Statistical analysis showed that tumor originated from the retroperitoneal space and bone marrow involvement were prognostic factors, P 〈 0. 05. Conclusion Stage Ⅳ NB is still in poor prognosis even with comprehensive therapy including intensive chemotherapy. Tumor originated from the retroperitoneal space and with bone marrow infiltration is related to poor prognosis.

关 键 词：神经母细胞瘤 Ⅳ期 预后 儿童 

分 类 号：R739.4[医药卫生—肿瘤] R681.705[医药卫生—临床医学]