[Clinical analysis of 26 children with postoperative residual or recurrent fibrosarcoma].

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.