Infantile fibrosarcoma: 16 case reports / 中华实用儿科临床杂志

ABSTRACT

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 ( χ2=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.