Mucoepidermoid carcinoma of the vulva: A distinct pathological and clinical entity

Unlike their major salivary gland counterparts, primary mucoepidermoid carcinoma of the vulva is extremely rare tumor. Here, we describe an unusual case of MEC arising from vulva and a short review of literature. This report describes an interesting and unusual case of mucoepidermoid carcinoma of vulva. A 42-year female presented with approx. 3.5 x 3.0 cm size painless polypoidal growth on right labia majora. CT scan abdomen & pelvis showed 30 x 27 cm size well defined lobulated polypoidal lesion is noted in right side of labia majora. Wide local excision had done with margin. Histological evaluation revealed a typical MEC with negative margin. Our tumor board has decided to keep on close follow-up because of the aggressive behaviour of such type of tumour. Follow-up at 6 months has revealed no evidence of recurrence. This is, to our knowledge, the first report of MEC arising from vulva in India. We report a very rare case of primary mucoepidermoid carcinoma of the vulva and this is the first case reported in India. Treatment strategy for MEC vulva yet to decide and patients should be closely followed-up for life to rule out late recurrence.