142例侵袭性纤维瘤病的临床特点及预后分析

Objective Aggressive fibromatosis is a rare kind of soft tissue tumor and was evaluated by few large studies. This study was to evaluate the clinical characteristics and identify the prognostic factors of this disease. Methods One hundred and forty-two patients with aggressive fibromatosis treated from January 1983 to August 2009 in Tianjin Medical University Cancer Hospital were retrospectively reviewed.The prognostic value of clinical and treatment factors was analyzed. Univariate analysis was performed with Log-rank test and Multivariate analysis was performed with Cox regression model. Results The follow-up rate is 93.7% and the median follow up time was 54 months (range,6 -208 months). Sixty-three patients had a minimum follow up time of 5 years and 6 patients had a minimum follow up time of 10 years. The male/female ratio was 1/1.84. The disease was most popular in women aged from 18 to 35 years old. The disease frequently occurred in the trunk (55.6%) and extremity (31.7%). All patients received surgery,and 46 received radiotherapy. The 5-year and 10-year local recurrence rates were 24. 4% and 31.1%,respectively. The 5-year and 10-year overall survival rates were both 99. 3%. Univariate analysis revealed that factors correlated with local recurrence were tumor size ( χ2 = 4. 37, P = 0. 037 ) and margin status (χ2 = 12. 36,P =0. 002). Multivariate analysis revealed that margin status was an independent risk factor (RR = 2. 219; χ2 = 9. 47,P = 0. 002) and radiotherapy was an independent protective factor ( RR = 0. 360;χ2 = 4. 95, P = 0. 026 ) for disease recurrence. When radiotherapy was delivered, the 10-year local recurrence rate decreased from 70. 1% to 20. 7% in patients with positive margin ( χ2 = 4. 22, P = 0. 040 )and decreased from 19.8% to 10.4% (χ2= 0.90, P= 0.344) in patients with negative margin.Conclusions Radical resection is the mainstay of treatment for aggressive fibromatosis. Postoperative radiotherapy can reduce the recurrent rate for patients with positive margin. In patients with negative margin,the role of radiotherapy should to be further evaluated in large clinical trials.

The clinical characteristics and prognostic factors of aggressive fibromatosis in 142 patients