摘要
目的总结单中心胸部软组织肉瘤患儿的临床特征,治疗及预后。方法回顾性分析2008年6月—2020年5月31日在首都医科大学附属北京儿童医院诊治的原发于胸部的软组织肉瘤,包括尤文肉瘤、横纹肌肉瘤和非横纹肌肉瘤类软组织肿瘤患儿临床资料,分析其临床病理特征,治疗过程和预后相关因素。结果53例患儿中,男27例,女26例,中位诊断年龄是92(13~193)个月。胸壁肿瘤24例(45.3%),胸腔内肿瘤29例(54.7%)。最常见的病理类型是尤文肉瘤,占54.7%。53例患者中1例诊断后回当地治疗,1例患者化疗1疗程后放弃治疗。52例患儿接受化疗,平均化疗11(1~26)个疗程,46例患儿接受原发瘤灶手术切除,47例患儿接受放疗,平均放疗剂量34.9Gy。系统治疗并随访51例患儿的生存分析显示3年总生存率(OS)是(60.0±7.7)%,其中尤文肉瘤3年OS是(64.3±9.9)%,横纹肌肉瘤3年OS是(27.8±14.8)%,其他肉瘤三年OS是(90.0±9.5)%。接受综合治疗(手术+放疗+化疗)的患儿的预后明显优于单纯手术和或化疗的患儿[3年OS:(65.4±8.1)%vs(28.6%±17.1)%,χ^(2)=12.33,P<0.001;3年EFS:(55.4±0.81)%vs 0.0%,χ^(2)=28.99,P<0.001]。单因素分析显示肿瘤部位在胸腔内,病理类型为横纹肌肉瘤,发生远处转移的患儿3年OS明显降低(P<0.05)。原发部位位于胸腔的横纹肌肉瘤患儿病死率100%,尤文肉瘤病死率46.6%,其他肉瘤患儿病死率14.2%,三组有明显统计学差异(χ^(2)=11.56,P<0.01)。结论儿童及青少年胸腔内横纹肌肉瘤预后极差。诊断时伴有远处转移及肿瘤位置发生在胸腔内是影响胸部软组织肉瘤患儿预后的不良因素。
Objective To summarize the clinical characteristics,treatment response and prognostic analysis of soft tissue sarcoma in children and adolescents.Methods The clinical features(sex,age,metastatic site,etc.)of 53 newly diagnosed chest soft tissue sarcoma admitted to Medical Oncology Department,Beijing Children's Hospital from June 2008 to May 2020 were analyzed retrospectively.Results There were 27 males and 26 females,the age at onset was 92 months(13~193months).24 cases was chest wall tumors(45.3%)and 29 cases was intrathoracic tumors(54.7%).The most common pathological type is Ewing's sarcoma(54.7%).One of the 53 patients returned to local treatment after diagnosis,and one patient gave up treatment after one cycles of chemotherapy.52 children received chemotherapy,with an average of 11(1~26)cycles,46 children received surgical resection of the primary tumor,and 47 children received radiotherapy,with an average radiation dose of 34.9 Gy.The survival analysis of 51 children with systematic treatment and follow-up showed that the 3-year overall survival(OS)was 60.0%±7.7%,of which the 3-year OS for Ewing's sarcoma was 64.3%±9.9%,the 3-year OS for rhabdomyosarcoma was 27.8%±14.8%,the 3-year OS of other sarcomas is 90.0%±9.5%.The prognosis of children who received comprehensive treatment(surgery+radiotherapy+chemotherapy)was significantly better than those received surgery or chemotherapy alone(3-year OS:65.4%±8.1%vs.28.6%±17.1%,χ^(2)=12.33;3-year EFS:55.4%±0.81%to 0,χ^(2)=28.99,P<0.001).A significant association with poor prognosis was found in the patients with intrathoracic tumors,rhabdomyosarcoma,or distant metastasis.The mortality of children with intrathoracic rhabdomyosarcoma was 100%,intrathoracic Ewing's sarcoma was 46.6%,and intrathoracic other sarcomas was 14.2%,there were significant statistical differences between the three groups(χ^(2)=11.56,P<0.01).Conclusions The prognosis of intrathoracic rhabdomyosarcoma is very poor.Distant metastasis and intrathoracic tumors are adverse factors affecting the prognosis of patients with chest soft tissue sarcoma.
作者
张诗晗
马晓莉
于洁
徐娜
赵文
段超
于彤
伏利兵
曾骐
张大伟
ZHANG Shihan;MA Xiaoli;YU Jie;XU Na;ZHAO Wen;DUAN Chao;YU Tong;FU Libing;ZENG Qi;ZHANG Dawei(Medical Oncology Department,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing,China.Beijing Key Laboratory of Pediatric Hematology Oncology,Key Laboratory of Major Diseases in Children,Ministry of Education,Beijing 100045,China;Department of Thoracic Surgery,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;Department of Radiology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;Department of Pathology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)
出处
《中国小儿血液与肿瘤杂志》
CAS
2022年第3期163-168,共6页
Journal of China Pediatric Blood and Cancer
基金
北京市医院管理中心“登峰”计划专项(DFL20191201)。
关键词
儿童及青少年
胸部
软组织肉瘤
预后
Children and adolescents
Chest
Soft tissue sarcoma
Prognosis
