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.