Aggressive angiomyxoma of the pelvis surgical management in a case with delayed diagnosis

Introduction and importanceAggressive angiomyxoma is characterized as a non-capsulated soft mass with the ability to progress to surrounding tissues but without metastasis to distant tissues. Slowing tumor extension leading delayed tumor diagnosis, expression of different types of hormonal receptors, therapeutic ineffectiveness of noninvasive treatment approaches and misdiagnosis have remained as the major challenges for managing this tumor.Case presentationHerein, we described a case of aggressive angiomyxoma located in the posterior of the uterus and vagina that as successfully managed surgically to remove tumor mass followed by gonadotropin-releasing hormone (GnRH) agonist to prevent tumor recurrence.Clinical discussionSurgical resection is the treatment of choice in aggressive angiomyxoma with complete success rate, however despite such successfulness, about two-thirds of patients experienced postoperative recurrence rate that could be prevented by hormone-based therapy especially GnRH agonist.ConclusionAggressive angiomyxoma is a rare tumor with locally invasive behavior. As misdiagnosis is common imaging like MRI with DWI should be considered. The best treatment is surgical resection by experienced surgeons in tertiary referral hospitals. Even with complete resection, the recurrence rate is high. So adjuvant medical treatment seems to be necessary.