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A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies
Published on: July 28, 2020
[Clinical analysis of 26 children with postoperative residual or recurrent fibrosarcoma]
1Department of Hematology and Oncology, Shanghai Children's Medical Center Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China (is working on the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China).
Insights
Pediatric fibrosarcoma patients treated with the SCMC-RS-99 regimen show good long-term survival. Younger children and those without recurrence have a significant survival advantage.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Genetics
Background:
- Fibrosarcoma in children presents challenges in achieving complete resection and preventing recurrence.
- Understanding long-term outcomes and prognostic factors is crucial for optimizing treatment strategies.
Purpose of the Study:
- To evaluate the long-term outcomes and identify prognostic factors for pediatric patients with postoperative residual or recurrent fibrosarcoma.
- To assess the impact of age, disease status, and treatment on survival rates.
Main Methods:
- Retrospective analysis of 26 pediatric fibrosarcoma patients treated with the SCMC-RS-99 regimen and radical resection.
- Kaplan-Meier analysis for overall survival (OS) and event-free survival (EFS).
- Univariate and multivariate analyses using Log-Rank test and Cox proportional hazards models.
Main Results:
- The 5-year OS and EFS rates were 86% and 77%, respectively.
- Postoperative residual fibrosarcoma and younger age (<3 years) were associated with significantly better 5-year EFS rates.
- Multivariate analysis indicated no significant impact of age >3 years, advanced stage, or recurrence on EFS.
Conclusions:
- Pediatric fibrosarcoma patients treated with the SCMC-RS-99 regimen demonstrate favorable long-term survival.
- Younger children and those without recurrence exhibit a significant survival advantage.
- Timely radical tumor resection and chemotherapy contribute to good remission rates and survival outcomes.
Abstract:
Objective: To evaluate the long-term outcomes and prognostic factors of postoperative residual or recurrent fibrosarcoma in children. Methods: Clinical data of 26 patients continually admitted to Shanghai Children's Medical Center between April 2004 and February 2019 with postoperative residual or recurrent fibrosarcoma were analyzed retrospectively. All patients were treated with Shanghai Children's Medical Center-rhabdomyosarcoma-1999 (SCMC-RS-99) regimen and timely radical tumor resection. Before chemotherapy, according to the surgery and imaging examination, 26 patients were divided into 2 groups: postoperative residual group and postoperative recurrent group. Clinical features and long-term follow-up results of patients were summarized. Kaplan-Meier analysis was used to evaluate the overall survival (OS) and event-free survival (EFS) rates, Log-Rank test and Cox proportional hazards models were used for univariate and multivariate prognostic analysis of factors including age (<3 years or 3-18 years old), gender, primary tumor site, postoperative stage, disease status, ETS variant 6 (ETV6) gene and chemotherapy drugs. Results: Among 26 cases, 13 were male and 13 were female, 17 cases were in postoperative residual group and 9 cases were in postoperative recurrent group. Until the last follow-up at December 31, 2019, the median follow-up time was 73 months (ranged from 10 to 188 months).The 5-year OS and EFS rates were (86±7)% and (77±9)%. Univariate analysis showed that, the 5-year EFS rate of postoperative residual group was significantly higher than that of the postoperative recurrent group ((94±5)% vs.(63±16)%,χ(2)=5.106,P=0.024), the 5-year EFS rate of patients <3 years old was significantly higher than that of patients 3-18 years old ((94±5)% vs. (62±17)%, χ(2)=6.507, P=0.011). Gender (χ(2)=0.445), primary tumor site (χ(2)=0.258), postoperative stage (χ(2)=3.046), ETV6 gene (χ(2)=1.496), and whether doxorubicin-containing drugs in chemotherapy (χ(2)=1.692) did not exhibit significant impact on 5-EFS rate (all P>0.05). Age, postoperative stage and disease status were included in COX proportional risk model for multivariate analysis, which showed that age >3 years old (HR=8.95, 95%CI 0.73-109.50, P=0.086), stage Ⅲ-Ⅳ (HR=16.50, 95%CI 0.84-321.40, P=0.065) and postoperative recurrence (HR=10.60, 95%CI 0.84-134.30, P=0.068) had no significant impact on EFS rate. Conclusion: Children with postoperative residual or postoperative recurrent fibrosarcoma still had good remission rate and long-term survival, especially young children without recurrence have a significant survival advantage.
