Aggressive Angiomyxoma of the Vulva: A Case Report

BACKGROUND: Aggressive angiomyxoma is a rare, slowly growing, soft tissue tumor found usually in women of reproductive age. It is a benign tumor with locally infiltrative growth and a high recurrence rate. It is difficult to make a definitive diagnosis before histological examination. Surgical excision is suggested. Long-term follow-up is necessary. CASE: We report a case of a 46-year-old woman who had an enormous aggressive angiomyxoma of the vulva with a length of 28 cm. The patient was admitted for excessive bleeding from the tumor which had been present for less than one year without treatment. Complete surgical excision was performed. Conventional histological examination showed it to be an aggressive angiomyxoma; the pedicle base was tumor free. Immunohistochemical staining was positive for desmin, CD34 and estrogen receptor (IRS 4) but negative for progesterone receptor. Ki67 immunoreactivity was present in < 1 %. CONCLUSION: We present a rare case of an enormous aggressive angiomyxoma, describe the histological characteristics of aggressive angiomyxoma and explain various treatment approaches. More investigations are needed to clarify the cause and optimal therapeutic treatment of this disease.