Clinicopathologic features and prognostic factors of desmoid-type fibromatosis: An analysis of 107 cases

Objective To study the clinicopathological features and risk factors for the recurrence of desmoid-type fibromatosis(aggressive fibromatosis). Methods Clinical data about 107 desmoid-type fibromatosis patients who admitted to Chinese PLA General Hospital from 2005 to 2015 were collected. The clinicopathological features and the expressions of P53 and β-catenin were used to analyze the factors influencing the prognosis of desmoid-type fibromatosis retrospectively. Results Of the 107 cases, 49 males (45.8%) and 58 females (54.2%) were included with mean age of 27.6 years (ranging from 1 to 78 years). The tumors located at upper extremity in 8 cases, lower extremity in 26 cases, trunk in 62 cases, head and neck in 11 cases. The mean diameter of the tumors was 8.9 cm (ranging from 1.2 to 30.0 cm). The median follow-up time was 6.4 years (ranging from 3.1 to 11.0 years). Of the 107 cases, 51 (47.7%) patients recurred after surgical resection. The recurrence develped in 2 to 70 months with median of 12 months. Univariate analysis revealed that surgical margin (P=0.000), tumor location (P=0.001), nuclear β-catenin (P=0.026) and P53(P=0.001) expression were the factors influencing tumor recurrence. Multivariate analysis revealed that surgical margin (P=0.000), tumor location (P=0.003) and P53 expression (P=0.000) were independent prognostic factors. Conclusion Positive surgical margin is a risk factor for the recurrence of desmoid-type fibromatosis. The expression of P53 is a poor prognostic factor, suggesting further treatment after surgery.