Efficacy and prognosis after radiotherapy in pediatric atypical teratoid / rhabdoid tumors
10.3760/cma.j.cn113030-20231211-00193
- VernacularTitle:儿童非典型性畸胎样/横纹肌样瘤放疗疗效及预后分析
- Author:
Wenfang TANG
1
;
Wenqi FAN
;
Yiyuan LI
;
Renhua ZHOU
;
Dongqing LU
;
Qing ZHOU
;
Mawei JIANG
Author Information
1. 上海交通大学医学院附属新华医院肿瘤科，上海 200092
- Keywords:
Central nervous system neoplasms;
Atypical teratoid / rhabdomyoma tumor;
Radiotherapy;
Craniospinal irradiation;
Progression-free survival;
Overall surviva
- From:
Chinese Journal of Radiation Oncology
2024;33(6):511-517
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical efficacy and prognostic factors of comprehensive treatment for atypical teratoid / rhabdomyoma tumor (AT/RT).Methods:Clinical data of children diagnosed with AT/RT who underwent radiotherapy in Department of Oncology of Xinhua Hospital affiliated to Shanghai Jiao Tong University School of Medicine from November 2012 to September 2022 were retrospectively analyzed. Pearson Chi-square test or continuous-corrected Chi-square test or Fisher's exact probability method were used for inter-group comparison of categorical variables. Independent sample t-test or Wilcoxon rank-sum test were used for inter-group comparison of continuous variables. Kaplan-Meier method was used to calculate the 1-year and 2-year overall survival (OS) rate and progression free survival (PFS) rate. Univariate and multivariate Cox regression analyses were employed to determine relevant prognostic factors. Results:A total of 45 patients were included, with a male/female ratio of 1.65:1, including 27 children aged ≥3 years old. All patients received surgery and radiotherapy in which 39 patients received chemotherapy, 41 craniospinal irradiation (CSI), and 4 whole brain or focal radiation therapy. The median follow-up was 28 (13.5, 49) months. A total of 14 patients died after comprehensive treatment. The 1-year OS rate was 80.0% and the PFS rate was 71.1%. The 2-year OS rate was 75.5% and the PFS rate was 65.7%. Survival prognostic analysis showed negative imaging assessment after radiotherapy ( HR=0.087, 95% CI: 0.011-0.697, P=0.022) was a favorable factor for PFS. The primary tumor<4.8 cm ( HR=0.221, 95% CI: 0.052-0.935, P=0.040) and CSI ( HR=0.085, 95% CI: 0.011-0.651, P=0.018) were favorable factors for OS. In subgroup analysis, CSI also improved OS in children aged ≥3 years ( HR=0.014, 95% CI: 0-0.470, P=0.017), but there was no significant difference in PFS. In children without cerebrospinal fluid dissemination, negative radiographic results after radiotherapy ( HR=0.066, 95% CI: 0.009-0.481, P=0.007; HR=0.076, 95% CI: 0.008-0.695, P=0.024, respectively) and CSI (HR=0.105, 95% CI: 0.012-0.937, P=0.044; HR=0.054, 95% CI: 0.005-0.629, P=0.020, respectively) were favorable factors for PFS and OS in children, and the primary tumor<4.8 cm also suggested a longer OS ( HR=0.094, 95% CI: 0.013-0.690, P=0.020). Conclusions:Comprehensive treatment including radiotherapy improves clinical prognosis of children with AT/RT. Our study shows that negative imaging results after radiotherapy are associated with PFS improvement. The primary tumor<4.8 cm and CSI are favorable factors for OS. CSI is also a significantly positive prognostic factor in children aged ≥3 years and those without cerebrospinal fluid dissemination.
