A case report of aggressive angiomyxoma of vulva: first appearance deceives many

Aggressive angiomyxoma (AA) of the vulva is a rare, benign tumour of mesenchymal origin. It has marked tendency for local recurrence but rarely metastasize. We report a case of 35-years old, nulliparous women presented with bilateral, large vulval masses. Local examination revealed approximately 20×20 cm mass arising from right labia majora, nodular, firm, non-tender with overlying skin intact. Left vulval mass was approximately 15×10 cm arising from labia minora, nodular, firm, non-tender. Inguinal lymph nodes were not palpable. After thorough evaluation, she underwent excision of bilateral vulval masses with reconstruction. Histopathology revealed tumour composed of ovoid to spindle-shaped cells in a fibrous and myxomatous stroma with loosely interspersed collagen fibres suggestive of aggressive angiomyxoma. On immunohistochemistry, the tumour cells were focally positive for smooth muscle actin and desmin. Stains for acid-fast bacilli and fungus did not reveal any organisms. Her postoperative period was uneventful with good recovery. She is on follow up and no recurrence has been seen so far in follow-up period of 15 months. AA should be kept in mind as one of the differential diagnoses in evaluation of large vulval masses. Keeping this rare entity in mind and relevant investigations may help in reducing the misdiagnosis and avoiding any mutilating surgery. Multidisciplinary approach can be crucial in management of such cases.