13例小儿横纹肌肉瘤疗效分析  

Response to therapy of 13 children with rhabdomyosarcoma

作　　者：徐莹[1] 唐锁勤[1] 王建文[1] 刘英[1] 刘立真[1] 

机构地区：[1]解放军总医院小儿内科,北京100853

出　　处：《中国当代儿科杂志》2008年第2期163-166,共4页Chinese Journal of Contemporary Pediatrics

摘　　要：目的分析小儿横纹肌肉瘤疗效,特别是大剂量化疗的效果。方法对该院1998年1月至2005年10月收治的13例横纹肌肉瘤患儿临床资料进行分析。男8例,女5例;年龄7个月至12岁;依据美国横纹肌肉瘤研究组(IRS)的分期标准Ⅰ期2例、Ⅱ期2例、Ⅲ期3例、Ⅳ期6例。所有患儿均经病理活检确诊,其中胚胎型12例,腺泡型1例。1例手术,1例手术加放疗,1例手术加化疗,10例手术加放疗及化疗。2002年前的病例化疗以VDCA、VAC和VadrC为主;2002年后采取了美国COG治疗横纹肌肉瘤的化疗方案,Ⅲ期采取CDV+IE方案治疗,Ⅳ期采用CT+VAC或CT+VAC+VCT方案治疗。结果10例接受手术加放疗及化疗联合治疗的患儿2年生存率为60%,另外3例没有接受联合治疗的患儿全部死亡。2002年后患儿生存率(60%,3/5)高于2002年前生存率(37.5%,3/8)。患儿2年生存率2002年前为37.5%(3/8),2002年后为60%(3/5),平均为46.2%(6/13)。结论儿童横纹肌肉瘤病理类型以胚胎型为主,恶性程度高。晚期病例采取手术联合放、化疗,特别是大剂量化疗可取得较好疗效。Objective To study the clinical response to comprehensive therapy in children with rhabdomyosarcoma. Methods Clinical data of 13 children （8 males and 5 females ） with rhabdomyosarcoma from January 1998 through October 2005 were retrospectively studied. Their ages ranged from 7 months to 12 years. The 13 Cases of rhabdomyosarcoma consisted of 2 cases in stage I, 2 cases in stage II, 3 cases in stage Ⅲ, and 6 cases in stage IV. Rhabdomyosarcoma was confirmed by biopsy, 12 cases （92.3%） presenting as embryonal type and 1 as alveolar type in histology. One patient underwent surgery treatment alone, one patient received surgery plus local radiation treatment, one patient received surgery plus chemotherapy and 10 patients were administered with a combination of surgery, local radiation treatment and chemotherapy. The chemotherapy protocol before 2002 was VDCA, VAC or VadrC. After 2002, the COG protocol was employed, with CDV ＋ IE for stage III, and CT ＋ VAC or CT ＋ VAC ＋ VCT for stage IV patients. Results The 2-year overall survival was 60% in the 10 patients who received a combination of surgery, local radiation treatment and chemotherapy, but the three patients died without receiving combination therapy. The 2-year overall survival in the 13 patients was 46.2%. The 2-year overall survival of the patients after 2002 （60%, 3/5） was higher than that before 2002 （37.5% , 3/8 ）. Conchlsions Embryonal rhabdomyosarcoma dominates the histology type in children, which is highly malignant. A combination therapy of surgery, local radiation and chemotherapy can result in a satisfactory therapeutic effect in children with rhabdomyosarcoma.

关 键 词：横纹肌肉瘤 治疗 儿童 

分 类 号：R738[医药卫生—肿瘤]