Li Yi,Dai Shuyang,Zhan Yong,et al.Correlation analysis of clinical features and outcomes of infantile fibrosarcoma[J].Journal of Clinical Pediatric Surgery,,():641-647.[doi:10.3760/cma.j.cn101785-202204074-007]
Correlation analysis of clinical features and outcomes of infantile fibrosarcoma
- Keywords:
- Infantial Fibrosarcoma; Prognosis; Surgical Procedures; Operative; Child
- Abstract:
- Objective To explore the clinical characteristics and therapeutic outcomes of infantile fibrosarcoma (IFS) and explore the related prognostic factors at a single center. Methods Between January 2010 and January 2022,the relevant clinical data were collected from 23 IFS children.Kaplan-Meier analysis was performed for calculating the values of overall survival (OS) and event-free survival (EFS).Univariate analysis was performed with Log-rank test and multivariate analysis by Cox regression. Results With a male-to-female ratio of 1.86:1,the age was from birth to 46 months.Tumor locations included head & neck (n=4),trunk (n=13) and extremities (n=6).Lung metastasis (n=1) was detected at an initial diagnosis.Seventeen children had tumors with a diameter >5 cm and 11 cases tested positive for ETS variant transcription factor 6 (ETV6)-neurotrophic receptor tyrosine kinase 3 (NTRK3) gene fusion.Primary resection (n=16) and biopsy (n=7) were performed.According to the staging scheme of Intergroup Rhabdomyosarcoma Study (IRS),the clinical stages were Ⅰ(n=9),Ⅱ(n=4),Ⅲ(n=9) and Ⅳ(n=1).Among 5 cases on neoadjuvant chemotherapy,efficacy was indefinite (n=3).Five cases taking oral larotrectinib showed a promising efficacy.At the end of follow-up, 6 of 23 cases had been followed for less than 3 years,and 9 less for 5 years.The 3-year OS was (15/17,88.24%) and the 5-year OS was (12/14,85.71%); the 3-year EFS (11/17, 64.71%) and the 5-year EFS rate was (9/14, 64.29%). Univariate analysis revealed that primary resection at an initial diagnosis was associated with a favorable prognosis for IFS (χ2=4.938,P=0.026).Multivariate Cox regression analysis revealed that neither the initial surgical method nor the postoperative IRS stage were independent prognostic factors for IFS (P>0.05). Conclusions The overall prognosis of IFS is favorable.Primary resection at an initial diagnosis is beneficial for the survival of IFS children.The effectiveness of neoadjuvant chemotherapy for IFS requires further investigations.Larotrectinib represents a potential effective therapy for refractory IFS children with NTRK gene fusion.
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Memo
收稿日期:2022-4-24。
基金项目:唐仲英基金会项目(ZSBK0070)
通讯作者:董瑞,Email:rdong@fudan.edu.cn