Deep (Aggressive) Angiomyxoma of Vulva: A Case Report

Abstract

Aggressive angiomyxoma (AAM) is rare, locally aggressive mesenchymal tumor that has a high propensity for local recurrence
that exceeds 35%. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in women of the reproductive age
group. It presents clinically mostly as a vulval polyp and simulates a vulvar abscess, bartholin gland cyst, vaginal cyst or an
inguinal hernia. As a result, complete excision is preferable if this can be accomplished without undo morbidity. In instances
where complete removal would be associated with high morbidity or when preservation of fertility is desired, incomplete removal
may be acceptable if the risks of recurrence and additional surgical interventions are understood. All patients require long-term
follow-up. Gross examination usually reveals a large mass, commonly >10 cm and not infrequently >20 cm. AAMs generally
have fairly uniform, low to moderate cellularity, and they contain relatively small, stellate-shaped and spindled cells, set in a
loosely collagenous, myxomatousstroma with scattered vessels of varying caliber and a variety of entrapped regional structures.
We report a case of AAM in a 40-year-old female presenting with a vulval mass on right labia majora.