Abstract:

Objective　To investigate the clinical characteristics，treatment strategies，and prognosis of aggressive fibromatosis in children. Methods　A retrospective analysis was performed in 27 cases of aggressive fibromatosis from January 2007 to January 2018 in Beijing Children’s Hospital，Capital Medical University. Results　There were 16 males and 11 females in 27 cases of aggressive fibromatosis，with a median age of 65（35，96） months at diagnosis. Tumor locations were as follows: abdominal wall（n＝3），abdominal cavity（n＝5），and extra-abdominal（n＝19）. Preoperative imaging examination showed that the median maximum diameter of primary tumors was 9.8（5.3，12.1） cm. Upfront surgery was performed in all of the cases，and 10 cases received adjuvant chemotherapy combined with methotrexate and vinorelbine. Among the 27 cases，9 cases survived without disease，15 cases survived with disease，and 3 cases died of tumor recurrence or progression. The 5-year overall survival and event-free survival rates were 85.8% and 32.6%，respectively. Further analysis of survival showed that positive surgical margin was associated with tumor recurrence. Conclusion　Aggressive fibromatosis is a rare，intermediate tumor in children. It is easy to relapse after incomplete surgical resection. Chemotherapy plays a role in controlling the growth of tumors. Most of the children have good prognosis，but some of them may be disabled or even die because of persistent，fast-growing tumors.

Key words: aggressive fibromatosis, clinical characteristics, treatment strategy, prognosis

摘要：

目的　分析儿童侵袭性纤维瘤的临床特征及预后情况，以提高临床认识及诊疗水平。方法　回顾性分析2007年1月至2018年1月首都医科大学附属北京儿童医院收治的27例原发性侵袭性纤维瘤患儿临床资料，并进行随访。结果　27例患儿中，男16例、女11例，中位发病年龄65（35，96）个月，原发肿瘤部位分型中，腹壁型3例、腹内型5例、腹外型19例。术前影像学检查提示原发肿瘤最大径中位数为9.8（5.3，12.1） cm。所有患儿初诊时均接受手术治疗，10例接受化疗（甲氨喋呤+长春碱类）。27例患儿中，无病生存9例，带瘤生存15例，死亡3例。5年总体存活率85.8%，5年无事件存活率32.6%。生存分析结果显示，手术切缘阳性与肿瘤复发相关。结论　儿童侵袭性纤维瘤是一种罕见的中间性肿瘤，手术切除效果不佳者极易复发，化疗对控制肿瘤的增长有一定作用。多数患儿预后良好，但部分患儿可因肿瘤持续增长而致残甚至死亡。

关键词: 侵袭性纤维瘤, 临床特征, 治疗策略, 预后情况