Aggressive angiomayxoma in men: Case report and systematic review.

Introduction: Aggressive angiomyxoma is a rare benign mesenchymal tumor and occurs rarely in males. This study aimed to review all the cases of AAM in men in the English literature up to September 2020 and investigate the clinical, histochemical, and radiological characteristics of AAM and discuss the best treatment choices according to available data. Methods: A comprehensive search of the PubMed, Google Scholar, and Embase databases up to September 2020 was performed looking for reported cases of male patients with AAM. The search excluded articles in languages other than English, reported female cases, and superficial angiomyxoma cases. Results: Among the 97 patients, the mean age was 48.2 years with an incidence peak between 40 and 60 years. The sites commonly involved were the scrotum (42.3%). On ultrasound, the tumor was hypoechoic (85.7%) with a well-defined margin (100%), whereas on MRI, most cases were isointense on T1-weighted images (53.8%), and hyperintense on T2-weighted images (85.7%). Immunohistochemistry revealed that the tumor tended to be positive for vimentin (100%), CD34 (63.4%), ER (50%), and PR (53.3%) while S-100 showed 91% negativity. Wide and complete surgical excision was conducted in most cases (72%), and follow-up duration ranged from 1 month to 144 months with a recurrence rate of 11.8%. Conclusion: Although the occurrence of AAM is rare in men, consideration should be taken in the differential diagnosis of a mass in the genitourinary region. According to our review, the most decisive immunohistochemistry profile is the positivity of Vimentin and CD34 with the negativity of S-100. Although hormonal treatment is controversial, we suggest a novel algorithm for the management of aggressive angiomyxoma.