CAJ | 학술논문

目的回顾性分析采用CAV(CTX+ THP+ VCR)/IE(IFO+VP-1 6)交替方案联合手术和/或放疗治疗儿童中危型横纹肌肉瘤(rhabdomyosarcoma,RMS)的疗效和生存.方法对初治儿童中危型RMS患儿采用CAV/IE交替方案诱导化疗4～6个疗程,随后根据肿瘤缩小情况采取手术和/或放疗,局部治疗后继续化疗,总疗程数8～10程.计算诱导化疗后手术切除的肿瘤组织坏死率.结果 2003年6月至2012年3月期间,共38例患儿纳入研究;中位年龄5岁(1.4～18.0岁);胚胎型31例(81.6％),主要起病部位为脑膜旁区域15例(39.5％).诱导化疗有效率为89.5％(34/38).接受局部治疗27例.9例患儿诱导化疗后手术并行肿瘤组织坏死率计算,6例肿瘤坏死率≥90％,3例肿瘤坏死率＜90％.化疗不良反应主要是骨髓抑制,对症处理均能恢复.中位随访时间22个月(4.5～97.7个月).全组患儿3年总生存率和无事件生存率分别为59.4％和47.3％.将全组患儿分为化疗联合局部治疗组(27例)和单纯化疗组(11例),患儿的3年总生存率分别为72.2％和33.3％(P=0.001),3年无事件生存率分别为53.3％和33.3％(P=0.025).行肿瘤组织坏死率计算的9例患儿中,组织坏死率在90％～100％的6例患儿均存活,组织坏死率＜90％的3例患儿均死亡.结论采用CAV/IE交替化疗方案治疗儿童中危型横纹肌肉瘤有效率较高、组织坏死率较高,毒性可耐受.积极、规范的综合治疗有助于进一步提高患儿的长期生存率.
목적 회고성분석채용CAV(CTX+ THP+ VCR)/IE(IFO+VP-1 6)교체방안연합수술화/혹방료치료인동중위형횡문기육류(rhabdomyosarcoma,RMS)적료효화생존.방법 대초치인동중위형RMS환인채용CAV/IE교체방안유도화료4～6개료정,수후근거종류축소정황채취수술화/혹방료,국부치료후계속화료,총료정수8～10정.계산유도화료후수술절제적종류조직배사솔.결과 2003년6월지2012년3월기간,공38례환인납입연구;중위년령5세(1.4～18.0세);배태형31례(81.6％),주요기병부위위뇌막방구역15례(39.5％).유도화료유효솔위89.5％(34/38).접수국부치료27례.9례환인유도화료후수술병행종류조직배사솔계산,6례종류배사솔≥90％,3례종류배사솔＜90％.화료불량반응주요시골수억제,대증처리균능회복.중위수방시간22개월(4.5～97.7개월).전조환인3년총생존솔화무사건생존솔분별위59.4％화47.3％.장전조환인분위화료연합국부치료조(27례)화단순화료조(11례),환인적3년총생존솔분별위72.2％화33.3％(P=0.001),3년무사건생존솔분별위53.3％화33.3％(P=0.025).행종류조직배사솔계산적9례환인중,조직배사솔재90％～100％적6례환인균존활,조직배사솔＜90％적3례환인균사망.결론 채용CAV/IE교체화료방안치료인동중위형횡문기육류유효솔교고、조직배사솔교고,독성가내수.적겁、규범적종합치료유조우진일보제고환인적장기생존솔.
Objective To analyze the efficacy of alternating Cyclophosphamide/Pirarubicin/Vincristine (CAV) with Ifosfamide/Etoposide (IE) on patients with intermediate-risk Rhabdomyosarcoma (RMS).Methods Children with intermediate-risk RMS were studied.They first received chemotherapy regimen of alternating CAV with IE for 4-6 cycles.Then,local therapies (surgery and/or radiotherapy) were selectively given depending on tumor response.Total chemotherapy included 8-10 cycles.The tumor cell necrosis rate of surgical specimens was calculated after neoadjuvant chemotherapy.Results Thirty-eight children (1.4-18.0,mean 5 years old) with intermediate-risk RMS were treated in this institution between June 2003 and March 2012.The rate of response to neoadjuvant chemotherapy was 89.5％,including CR (n =12),GPR (n =6) and PR (n =16).Twenty-seven patients underwent local treatments,12 underwent surgery,7 underwent radiotherapy and the other 8 underwent both.Nine patients underwent second look operation and the tumor cell necrosis rate in the surgical specimens was ≥90％ in 6 patients and ＜90％ in 3 patients.The main side effect was bone marrow suppression,which was reversible.The median follow-up period was 22 months (range,4.5 to 97.7 months).Three-year overall survival (OS) and event-free survival (EFS) of all patients were 59.4％ and 47.3％,respectively.Three-year OS of patients with and without local treatment were 72.2％ and 33.3％,respectively (P =0.001).Three-year EFS of patients with and without local treatment were 53.3％ and 33.3％,respectively (P =0.025).All the 6 patients with tumor cell necrosis rate ≥90％ were still alive,but all the 3 patients with tumor cell necrosis rate ＜90％ died after relapse.Conclusions The chemotherapy regimen of alternating CAV with IE is effective for intermediate-risk RMS,showing a high rate of tumor cell necrosis and tolerable toxicities.Positive and standard comprehensive treatment could improve the survival of patients with RMS.