婴儿型纤维肉瘤16例

Infantile fibrosarcoma: 16 case reports

目的总结婴儿纤维肉瘤(IFS)的临床特点,并分析影响IFS预后的高危因素。方法回顾性分析2013年10月至2019年12月首都医科大学附属北京同仁医院儿科收治的16例IFS患儿的临床资料,应用SPSS 21.0软件进行统计学分析,分析其临床特点,并分析影响预后的高危因素。结果1.共16例患儿,中位发病年龄0.13岁(0~4.92岁),男童多见(14/16例,占87.5%)。2.原发肿瘤部位:10例位于四肢,3例位于头颈部,3例位于躯干部。3.治疗方案:以手术联合化疗为主。化疗方案为"长春新碱+环磷酰胺+放线菌素D",15例化疗患儿中,12例对化疗敏感,3例对化疗不敏感。14例行肿瘤切除手术治疗,2例仅行活检。4.中位随访3.5年,3例患儿家长放弃治疗后死亡,13例患儿均完全缓解,其中2例复发(2/16例,12.5%),5年总生存(OS)率为70%。按病理分级分组,G1+G2级患儿5年OS率100%,G3级患儿的5年OS率为30%,差异有统计学意义(χ^(2)=4.853,P=0.028)。结论IFS总体预后良好。肿瘤病理分级是影响预后的重要因素,术前化疗有利于完全切除肿瘤,存在高危因素的患儿建议术后进行化疗。

Objective To summarize the clinical characteristics of infantile fibrosarcoma(IFS)and analyze the high risk factors that affect IFS prognosis.Methods Totally,16 patients were diagnosed IFS in Department of Pedia-trics,Beijing Tongren Hospital,Capital Medical University from October 2013 to December 2019.The clinical features and the high risk factors were analyzed by using SPSS 21.0 software.Results(1)The median age at diagnosis was 0.13 years(0 to 4.92 years),predominantly in males(14/16 cases,87.5%).(2)Focus site:10 cases were located in the extremities,3 cases in the head and neck,and 3 cases in the trunk.(3)Treatment plan was mainly surgical combined chemotherapy.The initial treatment was"Vincristine+Cyclophosphamide+Actinomycin D".Among the 15 cases receiving chemotherapy,3 cases were insensitive to chemotherapy and 12 cases were sensitive to chemotherapy.There were 14 cases of tumor resection and 2 cases of biopsy.(4)The median follow-up time was 3.5 years,and 3 patients′parents gave up the treatment due to the progress of the disease after chemotherapy and died.The remaining 13 cases were completely relieved until the follow-up period,of which 2 cases relapsed(2/16 cases,12.5%)and the overall 5-year survival rate was about 70%.Grouped by pathological grade,the total 5-year survival rate of children with G1 and G2 was 100%,and G3 was only 30%,with statistically significant differences(χ^(2)=4.853,P=0.028).Conclusions The overall prognosis of IFS is favorable.Tumor grade and lesion size are important factors that affect the prognosis.Adjuvant chemotherapy is beneficial to complete tumor resection,and chemotherapy is recommended for children with high risk factors.