横纹肌肉瘤42例综合治疗及疗效评估

Study on 42 Cases of Rhabdomyosarcoma in Children with Integrated Treatment

目的分析儿童横纹肌肉瘤(RMS)的临床资料及疗效评估。方法回顾性分析本院儿科病房2005年8月-2010年11月收治的42例RMS患儿的临床资料。男24例,女18例;中位数年龄6岁。对其肿瘤原发部位、临床分期、病理分型、治疗方法及效果进行统计分析,尤其是125I放射性粒子组织间植入术及自体外周血造血干细胞移植(auto-PBSCT)治疗对RMS的疗效。结果1.首发症状:临床表现主要为眼球突出及眼睑肿胀占52.38%(22/42例),鼻塞及鼻出血占26.19%(11/42例),跛行、关节肿痛占4.76%(2/42例),面颊及腹部包块占16.67%(7/42例)。2.原发于头颈部39例,原发于四肢2例,原发于腹部1例。37例头颈部有明确病理分型:胚胎型占89.18%(33/37例),腺胞型占5.42%(2/37例),多形型和混合型各占2.70%(均为1/37例);腹部及四肢原发RMS其病理分型为胚胎型占66.67%(2/3例),腺胞型占33.33%(1/3例)。经统计学分析,不同原发部位的病理分型差异有统计学意义(P<0.01)。3.Ⅲ期原发于头颈部3例患儿进行auto-PBSCT,至2011年10月术后分别随访26、30及35个月,2例患儿获完全缓解,1例患儿术后6个月复发,复发后1 a死亡。4.头颈部8例患儿行125I放射性粒子组织间植入,随访至2011年10月,随访时间为(45.00±17.43)个月,中位随访时间为43个月,病情达到完全缓解5例,部分缓解2例,死亡1例,总生存率为87.5%(7/8例)。5.全部患儿随访至2011年10月,中位随访时间38个月(10～80个月),放弃治疗4例,获随访38例。7例复发,5例因脑转移死亡,总生存率达86.84%(33/38例),完全缓解率达86.42%(26/38例),带瘤生存率达21.21%(7/33例)。结论头颈部是儿童横纹肌肉瘤多发部位,病理分型以胚胎型为主。包括125I放射性粒子组织间植入及auto-PBSCT等多种综合治疗方法对于提高肿瘤缓解率具有一定的临床意义。

Objective To analyze the clinical data and evaluate the treatment of rhabdomyosarcoma（RMS） in children. Methods Retrospective analysis was performed on 42 cases （24 boys, 18 grils）with RMS in children from Aug. 2005 to Nov. 2010 in Pediatrics of Beijing Tongren Hospital. The median age was 6 years old. Their primary sites of RMS, clinical stages, pathological types, therapy and curative outcomes were analyzed, especially the therapy with t251 particles implants into its location and auto - peripheral blood stem cell transplantation （auto- PBSCT）. Results 1. Primary symptoms included exophthalmos and eyelid swell, occupying 52.38% （22/42 cases）, snuffle and nose blood accounting for 26.19% （ 11/42 cases） , gimp and arthralgia was 4.76% （2/42 cases） ,and jowl and belly mass was 16.67% （7/ 42 eases）. 2. There were 39 cases of RMS with head and neck, with abdomen in 1 case, and RMS with limb in 2 cases. The pathological types of 37 head and neck cases were： embryo type 89.18% （33/37 cases）, adenocytc type 5.42% （2/37 cases）, pathology mutiformity type 2.70% （ 1/37 cases）, and commix type 2.70% （ 1/37 cases）. The pathological types of abdomen and limb cases were ： embryo type 66.67% （2/3 cases）, and adenocyte type 33.33% （ 1/3 cases）. The pathology data were of statistical value between diflerent primary sites （P 〈 0.01 ）. 3. Three cases of RMS in stage m were treated with auto - PBSCT. Average follow - up time was 26 months, 30 months and 35 months until Oct. 2011 ;2 cases after auto - PBSCT reached complete remission（CR） , 1 case recurred about 6 mouths after auto - PBSCT and died about 1 year after reeun＇ing. 4. Eight cases with head and neck treated with t251 particles implanted into its location. The average follow - up time was （45.00 ± 17.43 ） months and median follow - up time was 43 mouths. Five cases had CR, 2 cases had partial remission（PR） , 1 case was died. Total survival rate was 87.5% （7/8）. 5. The median follow - up time was 38 mouths in 42 cases with RMS until Oct. 2011. Four cases gave up, 38 cases received follow -up. Seven cases recurred, 5 cases died for cerebral metastasis, and total survival rate was 86.84% （33/38 cases）. CR rate was 86.42% （26/38 cases） ; PR was 21.21% （7/33 cases）. Conclusions Head and neck are very common sites of RMS in children and the embryo type is the most common pathological type of RMS. Comprehensive treatment can reach a higher level of therapeutic effect, and auto - PBSCT therapy in particular is of clinical value for increasing the remission rate of RMS.