婴儿期尤文肉瘤家族肿瘤临床特征及预后分析

Clinical characteristics and prognosis of infants with Ewing sarcoma family tumors in a single-center

目的总结婴儿尤文肉瘤(ESFT)临床特征及预后分析。方法回顾性分析2008年1月—2022年12月于首都医科大学附属北京儿童医院血液肿瘤中心诊断并规律治疗的婴儿期ESFT的临床资料,总结临床特征及评估预后,随访至2023年2月28日。结果共7例患儿纳入分析,占我院所有ESFT病例的5.7%(7/123)。男5例,女2例,中位发病年龄为10个月,从发病至确诊的中位时间为1.5个月。原发于骨骼者共4例,软组织者3例。原发肿瘤最大直径平均值分别为8.4cm和4cm。部分切除者5例,2例完整切除。全部患儿接受了化疗,平均化疗周期为14.4个。中位放疗年龄为20.5个月。7例患儿中,5例存活,其中4例疾病完全缓解状态,1例长期带瘤生存,2例死亡。结论婴儿ESFT是一种罕见的疾病,软组织起源的肿瘤较大年龄段ESFT相对多见,婴儿期原发肿瘤相对小,婴儿ESFT需要多学科联合治疗。病初诊断时转移期、对化疗的敏感性可能是不良预后的影响因素。

Objective To summarize the clinical features and prognostic analysis of infants with Ewing sarcoma family tumors(ESFT).Methods We retrospectively analyzed the clinical data of infants with ESFT treated in Hematology Oncology Center,Beijing Children′s Hospital from January 2008 to December 2022 and summarized the clinical characteristics and assess the prognosis,with follow-up up to February 28,2023.A total of 7 infants were included in our study,representing 5.7%of all children with ESFT admitted to our center during the same period.The median age at presentation was 10 months and the median time from presentation to diagnosis was 1.5 months in 5 males and 2 females.The tumor originated from the bone in 4 cases and soft tissue in 3 cases.The maximum diameter of the primary tumor averages 8.4cm and 4cm respectively.Partial resection was performed in 5 cases and complete resection was performed in 2 cases.All children received chemotherapy with a mean of 14.4 chemotherapy cycles.4 children received local radiotherapy.The median age of radiotherapy was 20.5 months.Of the seven children,five survived,four of whom were in complete remission,one was in long-term survival with tumor,and two died.Conclusions ESFT in infants is rare.Tumors of soft tissue origin are relatively common in ESFT at older ages.Tumors are relatively small in infants.ESFT in infants still requires combined multidisciplinary treatment.The stage of metastasis at the initial diagnosis and sensitivity to chemotherapy might be key factors for poor prognosis.