Aggressive angiomyxoma in African women: A report of two cases

Abstract

BACKGROUND: Aggressive angiomyxoma (AAM) is a rare soft tissue tumour usually of the perineum. There is no report in the surgical literature of a description of AAM in black Africans. OBJECTIVE: To report the first description of AAM in black Africa women in the surgical literature and to highlight the value of special immunostains in the complete characterization these rare tumours. METHODS: Case one was a 38-year-old pre-menopausal woman who presented with a five-year history of a painless mass in the left buttock extending to the left side of the perineum with recent ulceration. Clinical examination revealed a pale and febrile woman with an ulcerated 60 x 40 cm mass distorting the left gluteal region and the left side of the perineum. In case two, a 28-year old woman reported for the assessment of pedunculated mass arising from the right labium major that has been present for four years. Clinical examination revealed a 19x 15.5cm well-circumscribed mass in the perineum. The mass was completely covered by thickened hairy skin and attached to the right labium majored by a short thick stalk that measured 5cm x 7cm in size. Both tumours were excised via incisions in the perineum. RESULTS: In both cases the histopathology of the surgical specimens was reported as bland hypocellular tumours with spindle and stellate cells that lacked mitotic activity consistent with a diagnosis of an aggressive angiomyxoma. CONCLUSION: The clinical and histopathological features of the tumours described in this report are consistent with a diagnosis of aggressive angiomyxoma. To the best of our knowledge this is the first ever report of AAM in black African women.