Recurrent perineal aggressive angiomyxoma: dilemma in diagnosis and outcome of this rare disease

Aggressive angiomyxoma (AAM) is a very rare, benign, locally infiltrative mesenchymal tumour with a high chance of recurrence following surgical excision. In the male population, it is so rare that less than only 50 cases have been reported so far. We present a case of a large recurrent perineal AAM in a man who presented with swelling in the perineal region following surgical excision 3 years ago. After evaluation, the diagnostic dilemma of a possible perineal hernia or recurrence remained. Surgical exploration ruled out hernia and the tumour was excised with difficulty. Immunohistochemical examination showed tumour cells with diffuse nuclear positivity for oestrogen receptor and patchy cytoplasmic positivity for desmin (A2). Histological and immunohistochemical features confirmed the diagnosis. Being very rare, AAMs need to be considered as a differential diagnosis of pelvic/perineal tumours among males. With no standardised therapy for AAM, complete resection would be the goal of therapy.