摘要
目的总结原发于躯干及四肢的儿童横纹肌肉瘤(RMS)的临床特征、治疗效果及预后相关因素,以进一步提高对此类RMS的认识。方法回顾性分析2010年7月—2017年7月在北京儿童医院血液肿瘤中心诊治、原发瘤灶位于躯干及四肢的RMS患儿的临床资料及治疗疗效。治疗方式为化学治疗、手术和放射治疗。结果本研究共收集16例原发瘤灶位于躯干及四肢的RMS患儿,占同期总RMS患儿的10.7%,男性9例,女性7例,中位发病年龄46.1个月。原发瘤灶位于躯干者7例(胸背软组织3例,臀部2例,肛周赘生物2例),原发于四肢者9例(上肢6例,下肢3例);3例患儿存在远处转移:肺转移2例,颅内转移1例;区域淋巴结转移8例。病理分型:胚胎型9例,腺泡型7例;临床分期:Ⅱ期2例,Ⅲ期11例,Ⅳ期3例。所有患儿均采取手术联合化学治疗,其中3例患儿行二次手术治疗,16例患儿中13例给予放射治疗(总剂量:36～50.4Gy)。16例患儿的中位随访时间为26.1±7.1个月,1例死亡,3例完全缓解,9例病情稳定,3例病情进展,总生存率为94%(15/16)。四肢组及躯干组RMS患儿2年无事件生存率分别为48%及50%。结论躯干及四肢为RMS预后不良部位,原发瘤灶位于四肢的RMS患儿病理分型以腺泡型多见,且病初局部浸润及淋巴结受累比例高,整体预后较差,病初手术切除程度对预后有明显影响。
Objective Summarize clinical characteristics, therapeutic effect and the related factors of prognosis in childhood rhabdomyosareoma（RMS） of the extremity and trunk, to further improve the recognization of these kind of RMS. Methods RMS in Hematology Oncology Center of Beijing Children＇s Hospital, primary location in the extremity and trunk, from July 2010 to July 2017, were analyzed retrospectively. Treatment including chemotherapy, surgery and radiotherapy, and the therapeutic effect were analyzed. Results 16 cases with RMS of the extremity and trunk were collected, accounted for 10.7% of all RMS during the same period in our center, 9 boys , 7 girls, the median age at 46. 1 months. Tumor primary location： 7 in the trunk, 9 in the extremity; There were distant metastasis in 3 cases, including 2 cases of pulmonary and 1 case of intracranial metastasis, and 8 cases of regional lymph node metastasis; 9 emhryonal RMS, 7 alveolar RMS; Clinical stage [[ for 2 cases, stage m for 11 cases, and stage IV for 3 cases; All patients were treated by surgery and chemotherapy, 3 children underwent secondary surgery, and 13 patients were treated with radiotherapy （total dose 36 -50. 4 Gy）. The median follow-up time of 16 children was 26.1 ±7.1 months, 1 death, 3 complete remission, 9 stable, 3 progression, and the OS was 94 % （15/16）. 2 years EFS was 48% and 50% for extremity and trunk of tumor origin respectively. Conclusions Trunk and extremity were not the tumor＇ s primary location of good prognosis, alveolar type was more than embryonal in the extremity of tumor origin, the rate of regional lymph node metastasis was high, and the prognosis was poor, surgery in the beginning of treatment is significant for the prognosis.
作者
赵倩
金眉
张大伟
赵文
王希思
段超
马晓莉
ZHAO Qian;JIN Mei;ZHANG Dawei;ZHAO Wen;WANG Xisi;DUAN Chao;MA Xiaoli.(Beijing Key Laboratory of Pediatric Hematology Oncolog;National Discipline of Pediatrics, Ministry of Educatio;MOE Key Laboratory of Major Diseases in Childre;Hematology Oncology Center, Beijing Children＇ s Hospital, Capital Medical University, National Center for Children＇ s Health, Beijing 100045, China)
出处
《中国小儿血液与肿瘤杂志》
CAS
2018年第3期127-131,共5页
Journal of China Pediatric Blood and Cancer
关键词
横纹肌肉瘤
儿童
躯干
四肢
疗效
预后
Rhabdomyosarcoma
children
Trunk
Extremity
Therapeutic efficacy
Prognosis
