Abstract: Objective　To retrospectively analyze the clinical features，treatment and outcome of 6 children with infantile fibrosarcoma（IFS） who were hospitalized in the Peking University First Hospital，in order to provide evidence for a universal protocol of this very rare childhood tumor nationwide.Methods　From September 2012 to February 2018，six children with IFS were admitted to the Hematological Oncology Ward，Department of Pediatrics of Peking University First Hospital.The clinical data of IFS patients were collected，including age，gender，tumor size，primary site，pathology and results of ETV6-NTRK3 fusion gene test.The clinical data，disease staging，treatment protocol，efficacy evaluation and follow-up of chemotherapy were reviewed retrospectively.Results　The disease occurred in very young patients（all under the age of two years），predominantly in males（5∶1），and mainly in limbs（4/6）．ETV6-NTRK3 transcript was present in two patients.Postoperative staging was as follows：two patients were classified as IRS stage Ⅰ，two as IRS stage Ⅱ，one as IRS stage Ⅲ and one as IRS stage Ⅳ.Two patients had initial surgery and postoperative chemotherapy，one of who received radiotherapy；four received adjuvant chemotherapy and delayed operation.Two cases underwent direct surgical resection of the tumors，and the remaining four cases received preoperative chemotherapy，all with response，with the volume of tumors decreased by 66% to 93%.After a median follow-up of 35 months（range 13-77），all patients survived disease-free.Conclusion　Overall prognosis of IFS is favorable.Conservative surgery remains the mainstay treatment.When the initial operation fails to completely remove the tumor，patients should receive adjuvant chemotherapy and delay the surgery after tumor reduction.Adjuvant chemotherapy combined with conservative surgery approach is effective and feasible without significant long-term functional or cosmetic impairment.

Key words: Infantile fibrosarcoma；Chemoradiotherapy, adjuvant；Conservative surgery；Signs and symptoms；Diagnosis

摘要： 目的　回顾性分析北京大学第一医院收治的6例婴儿型纤维肉瘤患儿的临床特点、治疗方法和结局，为国内形成统一的治疗方案提供依据。方法　2012年9月—2018年2月北京大学第一医院儿科血液肿瘤病房共收治6例婴儿型纤维肉瘤患儿。收集患儿的临床资料，包括年龄、性别、肿瘤大小、起病部位、病理和ETV6-NTRK3融合基因检测结果。回顾性分析6例患儿的临床资料、疾病分期和治疗方案、化疗效果评估及随访。结果　6例患儿起病年龄均<2岁，男性发病多于女性（5∶1），原发部位多见于四肢（4/6）。2例患儿检测ETV6-NTRK3融合基因均为阳性。疾病分期：横纹肌肉瘤研究协作组（IRS）Ⅰ期2例、IRSⅡ期2例、IRSⅢ期1例、IRSⅣ期1例。2例行初次手术及术后化疗，其中1例行联合放疗；4例行辅助化疗并延期手术。2例直接行手术切除肿物，其余4例术前化疗全部有效，肿瘤体积缩小66%~93%。随访截止时间为2019年2月，随访中位时间35个月（13~77个月），6例患儿均无病生存。结论　婴儿型纤维肉瘤总体预后良好。保守手术仍然是主要治疗手段。初次手术无法完整切除肿瘤时应行辅助化疗并延期手术。辅助化疗联合保守手术治疗安全有效且能保留受累肢体功能及外形。

关键词: 婴儿型纤维肉瘤；放化疗, 辅助；保守手术；体征和症状；诊断