儿童促结缔组织增生性小圆细胞肿瘤8例并文献复习  

Eight children with desmoplastic small round cell tumor and literature review

作　　者：唐京京 汤静燕[1] 潘慈[1] 高怡瑾[1] 韩亚丽[1] 胡文婷 张婧 周敏[1] 殷敏智[2] Tang Jingjing;Tang Jingyan;Pan Ci;Gao Yijin;Han Yali;Hu Wenting;Zhang Jing;Zhou Min;Yin Minzhi(Department of Hematology and Oncology,Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine,Shanghai 200127,China;Department of Pathology,Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine,Shanghai 200127,China;Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]上海交通大学医学院附属上海儿童医学中心血液肿瘤科,200127 [2]上海交通大学医学院附属上海儿童医学中心病理科,200127 [3]郑州大学第一附属医院儿科,450052

出　　处：《中华实用儿科临床杂志》2021年第14期1108-1110,共3页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的分析促结缔组织增生性小圆细胞肿瘤(DSRCT)患儿的临床特点、治疗模式及预后,并归纳国际研究进展。方法采用回顾性研究,研究对象为1999年1月至2019年8月上海交通大学医学院附属上海儿童医学中心收治的8例DSRCT患儿,总结其临床特征、诊治过程及随访结果,采用Kaplan-Meier生存分析法计算生存率。结果8例患儿中男6例,女2例;7例原发腹盆腔,1例原发骶尾部;8例就诊时存在临近组织/器官/淋巴结不同程度浸润,其中4例(50%)同时有远处转移,包括远处淋巴结、肝、肺、骨转移。8例均接受化疗,其中3例接受腹盆腔+瘤窝增量放疗,2例接受自体造血干细胞移植。随访至2020年2月15日,中位随访时间59个月,3例死亡,5例存活(2例完全缓解,1例反复复发,2例部分缓解仍在治疗中)。中位复发时间为14.5个月,3年无复发生存率为(30.0±17.5)%、总体生存率为(51.4±20.4)%。结论50%的DSRCT患儿就诊时已有远处转移;虽经过化疗、积极的手术切除、腹盆腔放疗和干细胞移植等多模式治疗,但预后仍较差。Objective To analyze the clinical characteristics,therapeutic modalities and prognosis of desmoplastic small round cell tumor(DSRCT)in children,and to summarize the international research progress.Methods A total of 8 children with DSRCT admitted to Shanghai Children′s Medical Center,Shanghai Jiaotong University,School of Medicine,from January 1999 to August 2019 were retrospectively studied.The clinical characteristics,consultation process and follow-up results were summarized,and the Kaplan-Meier survival analysis method was used to calculate the survival rate.Results Among these 8 cases,there were 6 male children and 2 female children.Seven cases originated in the abdomen and pelvis,and 1 case originated in the sacral region.All cases had infiltrate surrounding tissues or viscera,and 4 cases(50%)had extra-peritoneal metastasis,including distant lymph node metastasis,liver,lung and bone metastasis.All patients received chemotherapy,among which 3 patients received radiotherapy,and 2 patients received autologous hematopoietic stem cell transplantation.The medical follow-up was continued to February 15,2020,with the median follow-up period being 59 months.Three cases died and 5 cases survived(2 cases in complete remission,1 case in recurrent relapse,2 cases in partial remission still under treatment).The median relapse time was 14.5 months,the 3-year relapse-free survival rate was(30.0±17.5)%,and 3-year overall survival was(51.4±20.4)%.Conclusions Half of DSRCT had distant metastasis;the prognosis was poor despite the aggressive multimodality therapeutic approaches,such as chemotherapy,cytoreductive surgery,and whole abdominopelvic radiotherapy and stem cell transplantation.

关 键 词：促结缔组织增生性小圆细胞肿瘤 综合疗法 预后 儿童 

分 类 号：R738.6[医药卫生—肿瘤]