儿童非横纹肌肉瘤类软组织肉瘤的临床特点及预后

Clinical features and outcome of pediatric non-rhabdomyosarcoma soft tissue sarcomas

目的 分析儿童非横纹肌肉瘤类软组织肉瘤(NRSTS)的临床特点、治疗及预后影响因素。方法 回顾性分析2012年8月—2022年8月确诊并在我院接受规范治疗的NRSTS患儿的临床资料,通过Kaplan-Meier法分析患儿的预后及影响因素。结果 共纳入49例患儿,男27例,女22例,中位诊断年龄52.7 (0.8~149.6)个月。病理亚型有14种,包括婴儿型纤维肉瘤9例(18.4%)、肾外横纹肌样瘤8例(16.3%)、炎性肌纤维母细胞瘤7例(14.3%)、肝未分化胚胎性肉瘤7例(14.3%)、其他病理亚型18例(36.7%)。在多学科综合治疗下,初治完全缓解43例,部分缓解1例,有效率89.8%。复发15例(30.6%),中位复发时间14 (3~40)个月,复发后中位生存时间16个月。中位随访时间37(4~125个月),3年OS为(74.9±6.6)%,3年EFS为(59.4±7.3)%。Ⅰ+Ⅱ期、Ⅲ+Ⅳ期的3年OS分别为(94.4±5.4)%vs(56.9±10.4)%(P=0.003),3年EFS分别为(72.9±9.6)%vs(46.2±10.3)%(P=0.042)。COG危险度分组低危组和中高危组3年OS分别为100%vs(62.0±9.2)%(P=0.009)。结论 儿童NRSTS治疗以多学科综合治疗为主,完全手术切除是预后良好的关键,COG中高危组预后不佳,复发患儿预后较差。

Objective To analyze the clinical features,treatment and prognosis of pediatric nonrhabdomyosarcoma soft tissue tumors.Methods 49 NRSTS patients were admitted to the Pediatric Hematological Oncology Ward from August 2012 to August 2022.Their clinical features,treatment and prognosis were retrospectively analyzed.In the univariable analysis survival was estimated with the KaplaneMeier method,and the log-rank test was used to compare the survival for patient subgroups.Results There were 27 boys and 22 girls in the enrolled 49 childrens.The mean age at initial diagnosis was 52.7( 0.8-149.6) months.There were 14 pathological types,including 9 patients with infantile fibrosarcoma,8 with extrarenal rhabdoid tumor,7 with inflammatory myofibroblastoma,7 with undifferentiated embryonal sarcoma of liver and 18 with other pathological types.43 patients achieved complete remission and 1 patient achieved partial remission after initial treatment,the overall response rate was 89.8%.The relapse occurred in 15 patients,within 3-40( median 14) months after patients first diagnosis,the median overall survival after relapse was 16 months.The follow-up period ranged from 4 to 125 months,with a median follow-up of 37 months.The 3-year overall survival rate of all the children was( 74.9 ± 6.6) %,and the 3-year event-free survival rate was( 59.4 ± 7.3) %.The 3-year OS rate of children with Stage Ⅰ+Ⅱ and stage Ⅲ+Ⅳ were( 94.4 ± 5.4) % vs( 56.9 ± 10.4) %,respectively( P =0.003).The 3-year EFS rate of children with Stage Ⅰ+Ⅱ and stage Ⅲ+Ⅳ were( 72.9 ± 9.6) % vs( 46.2 ± 10.3) %,respectively( P = 0.042).The 3-year OS rate of children in COG low and median/high-risk were 100% vs( 62.0 ± 9.2) %,respectively( P = 0.009).Conclusions NRSTS need multidisciplinary treatment.Complete resection of tumor remains the most important modality of treatment and is directly related to prognosis.Children with COG median/high-risk had poor outcomes.The outcome of patients with recurrent NRSTS is poor.