Individualized management of Aggressive Angiomyxoma: A case report

Aggressive Angiomyxoma (AAM) is a rare benign soft tissue tumor of uncertain differentiation mainly found in vulvovaginal region, female pelvis and perineum of reproductive age women. The term was given to reflect locally infiltrative behavior, high tendency for locally and also multiple or late recurrence and existing of myxoid and vascular components. Primary surgical excision is the traditional first line of therapy, although it could be difficult to reach negative resection margins because of its infiltrative nature and broad extension of huge tumor masses in pelvis. Nowadays extensive mutilating surgery is not supported any longer. Furthermore hormonal treatment with GnRH is a new auspicious therapeutic approach, especially to avoid often required extensive, maybe mutilating surgery in these young women. This is based on the fact that AAM occurs predominantly in premenopausal women and estrogen and progesterone receptors are commonly expressed in the tumor. We report a case of an 18-year-old female with a hormone dependent AAM in pelvis treated with primary surgery. In recurrent diseases Leuprorelin was recommended twice to avoid mutilating surgery and a great response was shown. In stable disease another surgery was performed because of increased postmenopausal symptoms. The hormonal treatment was withdrawn 3 month after operation and until today, 3 years later, no sign of recurrence has been evident. There is no existing consensus in duration of hormonal treatment with GnRH agonists or with regard to add-back therapy, but our individualized management has shown great response with good quality of life.