Eight children with desmoplastic small round cell tumor and literature review
10.3760/cma.j.cn101070-20200220-00208
- VernacularTitle:儿童促结缔组织增生性小圆细胞肿瘤8例并文献复习
- Author:
Jingjing TANG
1
;
Jingyan TANG
;
Ci PAN
;
Yijin GAO
;
Yali HAN
;
Wenting HU
;
Jing ZHANG
;
Min ZHOU
;
Minzhi YIN
Author Information
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
- Keywords:
Desmoplastic small round cell tumor;
Multimodal therapy;
Prognosis;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2021;36(14):1108-1110
- CountryChina
- Language:Chinese
-
Abstract:
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.
