RESUMEN
Objective To summarize the clinical manifestations,pathological character,diagnosis and treatment of aggressive angiomyxoma (AAM).Methods A computer-based online search of PubMed database and CHKD database was undertaken for literature about AAM published from all the relevant documents with the key words of aggressive angiomyxon.According to the condition 210 articles were analyzed.All the articles were analyzed about natural history,clinical manifestation,diagnosis,pathological character,treatment and prognosis of AAM.Results A total of 282 cases in well-documented articles had been reported,among which 64 were male and 218 were female,with male to female ratio of 1:3.4.The age of the patients from 1 to 83 years(mean 40.38 years).The most common sites were the perineum,genital tract and soft tissue in pelvic cavity in females and the scrotum,spermatic cord and groin in males.None of the cases could be accurately diagnosed as AAM preoperatively.The minimum diameter of the tumors was 1 cm,and the maximum was 60 cm.All the specimens showed typical pathological features of AAM as reported previously.Immunohistochemistry indicated that AAM tended to be strongly positive for vimentin,CD34,Desmin,estrogen receptor,progesterone receptor but mostly negative for S-100 and Ki-67 and Actin.The medical history was from 1 month to 20 years.The recurrence of the postoperative follow-up was 2 months to 20 years.The diagnosis depended on pathological examination.Conclusions AAM is a sort of unusual soft connective tissue tumor.It is a kind of unknown cause,slow progression,locally invasive,easy to recur after tumor resection.Long-term follow-up is quite necessary because of the high rate of local recurrence.