Objective: To discuss the clinical experiences of aggressive angiomyxoma (AAM) by summarizing clinical information of patients with this disease in China.

Methods: Chinese articles concerning AAM were retrospectively reviewed and analyzed.

Results: A total of 93 cases in 44 well-documented articles had been reported, among which 11 were male and 82 were female, with a male to female ratio of 1:7.45. The age of the females varied from 14 to 55 years (mean [SD], 37.17 [10.39] years; median, 38.5 years), whereas that of the males ranged from 15 to 65 years (mean [SD], 44.75 [17.61] years; median, 55 years), which was significantly higher than that of the females (t = 2.082, P = 0.041). The most common sites were the perineum in females and the scrotum in males. None of the cases could be accurately diagnosed as AAM preoperatively. The mean minimum diameter of the tumors was 4.62 (3.51) cm, and the maximum was 9.12 (8.23) cm. All the specimens showed typical pathological features of AAM as reported previously. Immunohistochemistry indicated that AAM tended to be strongly positive for vimentin, smooth-muscle actin, and CD34 but mostly negative for S-100 and CD68. The duration of the postoperative follow-up was 3 to 96 months (mean [SD], 28.5 [18.86] months; median, 9.5 months) in 44 cases, with a recurrence rate of 31.82%.

Conclusions: Aggressive angiomyxoma must be considered in the differential diagnosis of any female with an asymptomatic perineal mass. A complete margin-free excision should be achieved to avoid recurrence. Long-term follow-up is quite necessary because of the high rate of local recurrence.