Abstract
Aggressive angiomyxoma (AA) is a rare mesenchymal, locally aggressive tumour that mainly involves the vulval region of young females. It is characteristically a soft unencapsulated and poorly circumscribed mass, with a smooth external surface. The microscopic appearance is characterized by a myxoid, glistening cut surface and indistinct outlines. Although it is a slow-growing tumour, AA has a marked tendency to local recurrence. Surgical resection is the main treatment modality with a good prognosis, however long-term follow-up is necessary due to its high local recurrence rate. Alternative treatment options include hormonal treatment, due to the presence of estrogen and progesterone receptors in tumour cells. Hormonal therapies with tamoxifen, raloxifene or GnRH analogues have been shown to reduce the tumour size and help to achieve nearcomplete resection in large tumours. There are no guidelines in the postoperative management of AA. However, due to the high recurrence rate and potential morbidity associated with undiagnosed recurrences, several authors have recommended periodic evaluations up to 15 years after initial treatment.
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