Aggressive Angiomyxoma of the Vulva: Dramatic Response to Gonadotropin-releasing Hormone Agonist Therapy

Background: Aggressive angiomyxoma (AA) is a vulvovaginal mesenchymal neoplasm with a marked tendency to local recurrence but which usually does not metastasise. Most cases exhibit positive immunohistochemical staining with oestrogen receptor (ER) and, or, progesterone receptor (PR).

Case: We report a case of AA which exhibited positive immunohistochemical staining with ER and in which radiological examination following resection showed extensive residual tumour. The patient was commenced on gonadotropin-releasing hormone (GnRH) agonist therapy which resulted in complete radiological remission with replacement by scar tissue. The patient is currently maintained on a GnRH agonist.

Conclusion: The present case, together with several others reported in the literature, suggests that GnRH agonists may be of value in managing cases of AA, either primary or recurrent, which are not amenable to surgical excision. These agents may also be used to effect a reduction in size, so that more conservative surgery can be undertaken.