[Analysis of clinical characteristics and prognosis of non-rhabdomyosarcoma soft tissue sarcomas in 72 children].
AI interpretation is pending for this paper.
Open original publication →What the AI sees
Not AI summarized yet.
Research significance
Pending deeper interpretation.
Source abstract
Objective: To investigate the clinical characteristics, treatment, and prognosis factor of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) in children. Methods: In this retrospective cohort study, clinical data (including age, sex, pathological type, surgical resection status, risk stratification, protocols, and survival time) of 81 children with pathologically confirmed NRSTS initially treated at Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine from January 2015 to December 2024 were collected. Survival rates was analyzed by risk group, resection completeness, and other factors. Old and new protocols (changed around 2021) were compared for survival rates. Survival analysis and univariate analysis were performed using the Kaplan-Meier method; intergroup comparison of survival rates was conducted using the Log-rank test and multivariate analysis was performed using the Cox proportional hazards regression model. Results: A total of 72 children with NRSTS were enrolled, including 38 males (53%) and 34 females (47%), with the age at initial diagnosis of 6.5 (3.7, 9.9) years. A total of 16 histological subtypes were identified, with synovial sarcoma being the most common (14 children (19%)). Of 47 children with genetic testing, 34 (72%) carried class1 pathogenic variants. Among all enrolled children, 21 children (29%) were stratified into low-risk group, 31 children (43%) into intermediate-risk group, and 20 children (28%) into high-risk group. With a follow-up time of 66.0 (28.3, 79.8)months, 23 events were observed, including 20 relapses or progressions, 2 second malignancies, and 1 treatment-related death; 12 deaths reported. The estimated 5-year events free survival (EFS) and overall survival (OS) rates were 66.5% (95%CI 55.1%-77.9%) and 85.0% (95%CI 76.4%-93.6%), respectively. The 5-year EFS and OS rates were 85.7% (95%CI 70.9%-100.0%) and 100.0% in the low-risk group, 58.2% (95%CI 39.8%-76.6%) and 84.8% (95%CI 70.7%-98.9%) in the intermediate-risk group, and 59.2% (95%CI 37.5%-80.9%) and 69.3% (95%CI 48.8%-89.8%) in the high-risk group. Prognostic analysis indicated that incomplete resection independently predicted poorer prognosis in children (EFS rate: HR=5.46, 95%CI 2.17-13.70, P<0.001; OS rate: HR=10.17, 95%CI 2.93-35.29, P<0.001). Among 12 low-risk children before 2021, 9 received postoperative chemoradiotherapy; among 9 children after 2021, 7 underwent observation after complete resection. The 3-year EFS and OS rates both had no statically difference between the two groups (both P>0.05). Conclusions: Surgery remains the cornerstone of treatment for pediatric NRSTS, and complete resection is the most important prognostic determinant. Most low-risk children can be cured without adjuvant chemotherapy or radiotherapy.